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Albers MB, Sevcik M, Wiese D, Manoharan J, Rinke A, Jesinghaus M, Bartsch DK. Characteristics, therapy, and outcome of rare functioning pancreatic neuroendocrine neoplasms. Sci Rep 2024; 14:18507. [PMID: 39122816 PMCID: PMC11316089 DOI: 10.1038/s41598-024-68290-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/10/2024] [Accepted: 07/22/2024] [Indexed: 08/12/2024] Open
Abstract
Functioning pancreatic neuroendocrine neoplasms other than insulinomas and gastrinomas (rf-pNENs) are exceptionally rare tumours. Thus, their characteristics and long-term prognosis have not been well defined. This article aims to present data and experience from a single institution concerning this topic. Twelve of 216 (5.5%) patients with pNENs operated between 2002 and 2022 in the ENETS Centre of Excellence Marburg had rf-pNENs and their data were retrospectively analysed. We identified three vasoactive intestinal polypeptide producing pNENs, four glucagonomas and five calcitoninomas. The tumour could be visualised by preoperative imaging in all 12 patients, and six patients had distant metastases at the time of diagnosis. The tumour was located in the pancreatic tail in nine patients and the median tumour size was 82 (range 12-220) mm. Eleven patients underwent tumour resections (two robotic, nine conventional), nine of which were R0. After a median follow-up of 75 (range 1-247) months, six patients were alive, five of whom had no evidence of disease. All patients who remained disease-free had an initial R0 resection of the primary tumour and no initial liver involvement. This study sheds light on the distinct characteristics and outcomes of these exceedingly rare tumours, offering insights for improved understanding and management.
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Affiliation(s)
- Max B Albers
- Department of Visceral, Thoracic and Vascular Surgery, University Hospital Marburg, Philipps University Marburg, Marburg, Germany.
| | - Martina Sevcik
- Department of Visceral, Thoracic and Vascular Surgery, University Hospital Marburg, Philipps University Marburg, Marburg, Germany
| | - Dominik Wiese
- Department of Visceral, Thoracic and Vascular Surgery, University Hospital Marburg, Philipps University Marburg, Marburg, Germany
| | - Jerena Manoharan
- Department of Visceral, Thoracic and Vascular Surgery, University Hospital Marburg, Philipps University Marburg, Marburg, Germany
| | - Anja Rinke
- Department of Internal Medicine, Division of Gastroenterology and Endocrinology, University Hospital Marburg, Philipps University Marburg, Marburg, Germany
| | - Moritz Jesinghaus
- Institute of Pathology, University Hospital Marburg, Philipps University Marburg, Marburg, Germany
| | - Detlef K Bartsch
- Department of Visceral, Thoracic and Vascular Surgery, University Hospital Marburg, Philipps University Marburg, Marburg, Germany
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Suteetorn S, Kitisin K, Wanpiyarat N, Kunjan S, Snabboon T. VIPoma: An Unusual Cause of Chronic Diarrhea. ACTA MEDICA (HRADEC KRALOVE) 2024; 67:64-68. [PMID: 39434673 DOI: 10.14712/18059694.2024.22] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 10/23/2024]
Abstract
Chronic diarrhea is a significant challenge in clinical practice because of its high prevalence and various causes. Comprehensive clinical assessment and stepwise laboratory approach are crucial for an accurate diagnosis. This report presents a case of an adult woman who experienced chronic watery diarrhea, complicated by renal impairment and multiple electrolyte imbalances, including hypokalemia, hypophosphatemia, and metabolic acidosis. The diagnosis of a vasoactive intestinal polypeptide-secreting tumor (VIPoma) with liver metastases was confirmed by elevated serum levels of a vasoactive intestinal polypeptide (VIP) and imaging findings of a pancreatic mass with multiple hepatic lesions. Preoperative management, including fluid rehydration, electrolyte correction, and somatostatin analog therapy, significantly improved her clinical symptoms. Subsequent surgical tumor removal and radiofrequency ablation of the hepatic lesions resulted in complete resolution of symptoms and normalized VIP levels. This case emphasizes the importance of early recognition of this rare tumor in patients with chronic diarrhea to improve clinical outcomes.
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Affiliation(s)
- Sutharin Suteetorn
- Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
- Department of Medicine, Queen Savang Vadhana Memorial Hospital, Chonburi province, Thailand
| | - Krit Kitisin
- Department of Surgery, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
| | - Natcha Wanpiyarat
- Department of Pathology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
| | - Supaksorn Kunjan
- Center for Medical Diagnostic Laboratories, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
| | - Thiti Snabboon
- Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
- Excellence Center in Diabetes, Hormone and Metabolism, King Chulalongkorn Memorial Hospital, Thai Red Cross Society, Bangkok, Thailand.
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Giampatzis V, Kotsiari C, Bostantzis P, Chrisoulidou A, Fotiadou A, Loti S, Papantoniou S, Papadopoulou P. Pancreatic Vasoactive Intestinal Peptide-Producing Tumor as a Rare Cause of Acute Diarrhea and Severe Hypokalemia. J Med Cases 2023; 14:307-316. [PMID: 37868325 PMCID: PMC10586331 DOI: 10.14740/jmc4141] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/24/2023] [Accepted: 08/19/2023] [Indexed: 10/24/2023] Open
Abstract
Pancreatic vasoactive intestinal peptide-producing tumor (VIPoma) is a rare functional neuroendocrine tumor most commonly presenting with watery diarrhea and electrolyte abnormalities that include hypokalemia, hypercalcemia and metabolic acidosis. This type of tumor has usually insidious clinical behavior that is characterized by chronic secretory diarrhea, lasting usually from months to years before diagnosis, not responsive to usual medical or dietary treatment approaches. Given the resemblance of VIPoma with other more common causes of chronic watery diarrhea, the final diagnosis is often delayed and the tumors are usually large and metastatic at the time of detection. Our case of pancreatic VIPoma demonstrates an unusual clinical course for this type of tumor with acute refractory diarrhea and rapid deterioration of patient's clinical and biochemical status that required emergent in-hospital diagnosis and treatment. Our patient is a 45-year-old woman who presented with abrupt, watery diarrhea during the past 24 h before admission accompanied with severe hypokalemia as well as hyponatremia, hyperglycemia and hypercalcemia. Despite aggressive management with fluid administration and electrolyte replenishment, no significant improvement in patient's symptoms and electrolyte imbalance was observed. After exclusion of other causes of acute diarrhea from the medical history and the laboratory tests, the clinical suspicion of a functional neuroendocrine tumor was raised. After the establishment of final diagnosis of pancreatic VIPoma with biochemical tests and magnetic resonance imaging (MRI), somatostatin analogues were prescribed and the patient underwent distal pancreatectomy and splenectomy with no signs of lymph node and splenic metastases. Few days after the surgical resection of the tumor, the patient readmitted to our hospital with tarry stools and severe anemia. The abdominal computed tomography (CT) revealed a retroperitoneal cystic lesion. The gastrointestinal bleeding gradually recessed after endoscopic hemostasis of duodenal ulcer lesions whereas the cystic lesion (postoperative lymphocele) was successfully drained under CT-guidance before discharge. After almost 10 years postoperatively, the patient is still asymptomatic with no signs of relapse or metastasis of the disease in the periodic laboratory and imaging follow-up. In conclusion, pancreatic VIPoma can sometimes manifest symptoms of abrupt onset and rapid progression that require high clinical suspicion, appropriate diagnostic workup and aggressive management.
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Affiliation(s)
| | | | | | - Alexandra Chrisoulidou
- Endocrinology Department, Theageneio Anticancer Hospital, Alexandrou Symeonidi 2, Thessaloniki 54639, Greece
| | - Aimilia Fotiadou
- Endocrinology Department, Theageneio Anticancer Hospital, Alexandrou Symeonidi 2, Thessaloniki 54639, Greece
| | - Soultana Loti
- Department of Radiology, General Hospital of Kavala, Greece
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First NJ, Pedreira-Lopez J, San-Silvestre MRF, Parrish KM, Lu XH, Gestal MC. Bordetella spp. utilize the type 3 secretion system to manipulate the VIP/VPAC2 signaling and promote colonization and persistence of the three classical Bordetella in the lower respiratory tract. Front Cell Infect Microbiol 2023; 13:1111502. [PMID: 37065208 PMCID: PMC10090565 DOI: 10.3389/fcimb.2023.1111502] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/29/2022] [Accepted: 02/01/2023] [Indexed: 03/31/2023] Open
Abstract
Introduction Bordetella are respiratory pathogens comprised of three classical Bordetella species: B. pertussis, B. parapertussis, and B. bronchiseptica. With recent surges in Bordetella spp. cases and antibiotics becoming less effective to combat infectious diseases, there is an imperative need for novel antimicrobial therapies. Our goal is to investigate the possible targets of host immunomodulatory mechanisms that can be exploited to promote clearance of Bordetella spp. infections. Vasoactive intestinal peptide (VIP) is a neuropeptide that promotes Th2 anti-inflammatory responses through VPAC1 and VPAC2 receptor binding and activation of downstream signaling cascades. Methods We used classical growth in vitro assays to evaluate the effects of VIP on Bordetella spp. growth and survival. Using the three classical Bordetella spp. in combination with different mouse strains we were able to evaluate the role of VIP/VPAC2 signaling in the infectious dose 50 and infection dynamics. Finally using the B. bronchiseptica murine model we determine the suitability of VPAC2 antagonists as possible therapy for Bordetella spp. infections. Results Under the hypothesis that inhibition of VIP/VPAC2 signaling would promote clearance, we found that VPAC2-/- mice, lacking a functional VIP/VPAC2 axis, hinder the ability of the bacteria to colonize the lungs, resulting in decreased bacterial burden by all three classical Bordetella species. Moreover, treatment with VPAC2 antagonists decrease lung pathology, suggesting its potential use to prevent lung damage and dysfunction caused by infection. Our results indicate that the ability of Bordetella spp. to manipulate VIP/VPAC signaling pathway appears to be mediated by the type 3 secretion system (T3SS), suggesting that this might serve as a therapeutical target for other gram-negative bacteria. Conclusion Taken together, our findings uncover a novel mechanism of bacteria-host crosstalk that could provide a target for the future treatment for whooping cough as well as other infectious diseases caused primarily by persistent mucosal infections.
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Affiliation(s)
- Nicholas J. First
- Department of Microbiology and Immunology, Louisiana State University (LSU) Health Sciences Center at Shreveport, Shreveport, LA, United States
| | - Jose Pedreira-Lopez
- Department of Microbiology and Immunology, Louisiana State University (LSU) Health Sciences Center at Shreveport, Shreveport, LA, United States
| | - Manuel R. F. San-Silvestre
- Department of Microbiology and Immunology, Louisiana State University (LSU) Health Sciences Center at Shreveport, Shreveport, LA, United States
| | - Katelyn M. Parrish
- Department of Microbiology and Immunology, Louisiana State University (LSU) Health Sciences Center at Shreveport, Shreveport, LA, United States
| | - Xiao-Hong Lu
- Department of Pharmacology, Toxicology, and Neuroscience, Louisiana State University Health Sciences Center at Shreveport, Shreveport, LA, United States
| | - Monica C. Gestal
- Department of Microbiology and Immunology, Louisiana State University (LSU) Health Sciences Center at Shreveport, Shreveport, LA, United States
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Marcal LP, Chuang HH, Tran Cao HS, Halperin DM. Pancreatic Neuroendocrine Tumors. ONCOLOGIC IMAGING : A MULTIDISCIPLINARY APPROACH 2023:197-217. [DOI: 10.1016/b978-0-323-69538-1.00014-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/06/2025]
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Angelousi A, Koumarianou A, Chatzellis E, Kaltsas G. Resistance of neuroendocrine tumours to somatostatin analogs. Expert Rev Endocrinol Metab 2023; 18:33-52. [PMID: 36651768 DOI: 10.1080/17446651.2023.2166488] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/29/2022] [Accepted: 01/05/2023] [Indexed: 01/13/2023]
Abstract
INTRODUCTION A common feature shared by most neuroendocrine tumors (NETs) is the expression on their surface of somatostatin receptors (SSTRs) that are essential for their pathophysiological regulation, diagnosis, and management. The first-generation synthetic somatostatin analogs (SSAs), octreotide and lanreotide, constitute the cornerstone of treatment for growth hormone secreting pituitary adenomas and functioning, progressive functioning, and non-functioning gastro-entero-pancreatic (GEP-NETs). SSAs exert their mechanism of action through binding to the SSTRs; however, their therapeutic response is frequently attenuated or diminished by the development of resistance. The phenomenon of resistance is complex implicating the presence of additional epigenetic and genetic mechanisms. AREAS COVERED We aim to analyze the molecular, genetic, and epigenetic mechanisms of resistance to SSA treatment. We also summarize recent clinical data related to the development of resistance on conventional and non-conventional modes of administration of the first-generation SSAs and the second-generation SSA pasireotide. We explore mechanisms used to counteract the resistance to SSAs using higher doses or more frequent mode of administration of SSAs and/or combination treatments. EXPERT OPINION There is considerable heterogeneity in the development of resistance to SSAs that is tumor-specific necessitating the delineation of the underlying pathophysiological processes to further expand their therapeutic applications.
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Affiliation(s)
- Anna Angelousi
- First Department of Internal Medicine, Unit of Endocrinology, Laikon General hospital, National and Kapodistrian University of Athens, Athens, Greece
| | - Anna Koumarianou
- Hematology Oncology Unit, Fourth Department of Internal Medicine, Attikon University Hospital, National and Kapodistrian University of Athens, Athens, Greece
| | - Eleftherios Chatzellis
- Endocrinology Diabetes and Metabolism Department, 251 Hellenic Air Force and VA General Hospital, Athens, Greece
| | - Gregory Kaltsas
- First Propaedeutic Department of Internal Medicine, Endocrine Unit, National and Kapodistrian University of Athens, Athens, Greece
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Brown G, Monaghan AM, Fristedt R, Ramsey E, Al-Mrayat M, Rajak R, Armstrong T, Takhar A. Metastatic mixed VIPoma/PPoma-induced diarrhoea causing renal failure. Endocrinol Diabetes Metab Case Rep 2022; 2022:22-0231. [PMID: 36001009 PMCID: PMC9422256 DOI: 10.1530/edm-22-0231] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/06/2022] [Accepted: 07/26/2022] [Indexed: 11/29/2022] Open
Abstract
Summary Vasoactive intestinal peptide-secreting tumours (VIPomas) are an extremely rare form of functional pancreatic neuroendocrine tumour with an estimated annual incidence of 1 in 10 million. Associated tumour hypersecretion of other peptides, including pancreatic polypeptide (PPomas), may also be seen. These malignancies classically present with a defined triad of refractory diarrhoea, hypokalaemia and metabolic acidosis known as Verner-Morrison syndrome. Diagnosis is frequently delayed, and the majority of patients will have metastatic disease at presentation. Symptoms are usually well controlled with somatostatin analogue administration. Here we report a case of metastatic mixed VIPoma/PPoma-induced diarrhoea causing renal failure so severe that ultrafiltration was required to recover adequate renal function. Learning points Profuse, watery diarrhoea is a common presenting complaint with a multitude of aetiologies. This, combined with the rarity of these tumours, makes diagnosis difficult and frequently delayed. A functional neuroendocrine tumour should be suspected when diarrhoea is unusually extreme, prolonged and common causes have been promptly excluded. These patients are likely to be profoundly unwell on presentation. They are extremely hypovolaemic with dangerous electrolyte and metabolic abnormalities. Aggressive initial rehydration and electrolyte replacement are imperative. A somatostatin analogue should be commenced as soon as the diagnosis is suspected. This is an extreme example of Verner-Morrison syndrome. We are unaware of another case where renal failure secondary to diarrhoea and dehydration was so severe that renal replacement therapy was required to restore adequate renal function, further emphasising how critically unwell these patients can be. Both the primary tumour and metastases showed a remarkably good and rapid response to somatostatin analogue administration. Cystic change and involution were noted on repeat imaging within days. Prior to his illness, this patient was extremely high functioning with no medical history. His diagnosis was an enormous psychological shock, and the consideration and care for his psychological well-being were a crucial part of his overall management. It highlights the importance of a holistic approach to cancer care and the role of the clinical nurse specialist within the cancer multidisciplinary team.
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Affiliation(s)
- George Brown
- Department of Hepatobiliary & Pancreatic Surgery, University Hospital Southampton, Southampton, UK
| | - Anthony Mark Monaghan
- Department of Hepatobiliary & Pancreatic Surgery, University Hospital Southampton, Southampton, UK
| | - Richard Fristedt
- Department of Hepatobiliary & Pancreatic Surgery, University Hospital Southampton, Southampton, UK
| | - Emma Ramsey
- Department of Hepatobiliary & Pancreatic Surgery, University Hospital Southampton, Southampton, UK
| | - Ma’en Al-Mrayat
- Department of Endocrinology, University Hospital Southampton, Southampton, UK
| | - Rushda Rajak
- Department of Cellular Pathology, University Hospital Southampton, Southampton, UK
| | - Thomas Armstrong
- Department of Hepatobiliary & Pancreatic Surgery, University Hospital Southampton, Southampton, UK
| | - Arjun Takhar
- Department of Hepatobiliary & Pancreatic Surgery, University Hospital Southampton, Southampton, UK
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Ruggeri RM, Altieri B, Grossrubatcher E, Minotta R, Tarsitano MG, Zamponi V, MIsidori A, Faggiano A, Colao AM. Sex differences in carcinoid syndrome: A gap to be closed. Rev Endocr Metab Disord 2022; 23:659-669. [PMID: 35292889 DOI: 10.1007/s11154-022-09719-8] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 03/04/2022] [Indexed: 12/17/2022]
Abstract
The incidence of neuroendocrine neoplasms and related carcinoid syndrome (CS) has markedly increased over the last decades and women seem to be more at risk than men for developing CS. Nevertheless, very few studies have investigated sex differences in clinical presentation and outcomes of CS. However, as per other tumours, sex might be relevant in influencing tumour localization, delay in diagnosis, clinical outcomes, prognosis and overall survival in CS. The present review was aimed at evaluating sex differences in CS, as they emerge from an extensive search of the recent literature. It emerged that CS occurs more frequently in female than in male patients with NENs and women seem to have a better prognosis and a slight advantage in overall survival and response to therapy. Moreover, the disease likely impacts differently the quality of life of men and women, with different psychological and social consequences. Nevertheless, sex differences, even if partially known, are deeply underestimated in clinical practice and data from clinical trials are lacking. There is urgent need to increase our understanding of the sex-related differences of CS, in order to define tailored strategies of management of the disease, improving both the quality of life and the prognosis of affected patients.
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Affiliation(s)
- Rosaria M Ruggeri
- Department of Clinical and Experimental Medicine, Unit of Endocrinology, University of Messina, Messina, Italy.
| | - Barbara Altieri
- Division of Endocrinology and Diabetes, Department of Internal Medicine I, University Hospital, University of Würzburg, Würzburg, Germany
| | | | - Roberto Minotta
- Department of Clinical Medicine and Surgery, Endocrinology Unit, University Federico II, Naples, Italy
| | | | - Virginia Zamponi
- Endocrinology Unit, Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, Sapienza University of Rome, Rome, Italy
| | - Andrea MIsidori
- Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy
| | - Antongiulio Faggiano
- Endocrinology Unit, Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, Sapienza University of Rome, Rome, Italy
| | - Anna Maria Colao
- Department of Clinical Medicine and Surgery, Endocrinology Unit, University Federico II, Naples, Italy
- Cattedra Unesco "Educazione Alla Salute E Allo Sviluppo Sostenibile", University Federico II, Naples, Italy
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Una Cidon E. Vasoactive intestinal peptide secreting tumour: An overview. World J Gastrointest Oncol 2022; 14:808-819. [PMID: 35582098 PMCID: PMC9048535 DOI: 10.4251/wjgo.v14.i4.808] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/17/2021] [Revised: 05/15/2021] [Accepted: 03/25/2022] [Indexed: 02/06/2023] Open
Abstract
Vasoactive intestinal peptide (VIP) secreting tumour (VIPoma) is a rare functional neuroendocrine tumour that typically arises from pancreatic islet cells. These present as sporadic, solitary pancreatic neoplasias with an estimated incidence of one in ten million individuals per year. Only around 5% of VIPomas are associated with multiple endocrine neoplasia type I syndrome. Excessive VIP secretion produces a clinical syndrome characterized by refractory watery diarrhoea, hypokalemia and metabolic acidosis. These coupled with elevated plasma levels of VIP are diagnostic. The majority of VIPomas are malignant and have already metastasized at the time of diagnosis (60%). Metastases occur most frequently in the liver, or regional lymph nodes, lungs, kidneys and bones. Some reports of skin metastases have been documented. Complete surgical resection continues to be the only potentially curative treatment. However, when the neoplasia cannot be excised completely, surgical debulking may provide palliative benefit. Other palliative options have included recently the peptide receptor radionuclide therapy which has shown to be effective and well-tolerated. This article will review all aspects of pancreatic VIPomas highlighting aspects such as clinical presentation, diagnosis and management.
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Affiliation(s)
- Esther Una Cidon
- Department of Medical Oncology, University Hospitals Dorset, Bournemouth BH7 7DW, Dorset, United Kingdom
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Vemuri KR, Balcerek MI, McGowan TE, Love A, Chapman PR. VIPoma:
an unsuspecting culprit of severe secretory diarrhoea in a human immunodeficiency virus‐infected patient. Intern Med J 2022; 52:125-129. [DOI: 10.1111/imj.15655] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/11/2021] [Revised: 06/16/2021] [Accepted: 06/29/2021] [Indexed: 11/27/2022]
Affiliation(s)
- Kanthi R. Vemuri
- Department of Infectious Diseases Royal Brisbane and Women's Hospital Brisbane Queensland Australia
- Herston Infectious Diseases Institute (HeIDI) Metro North Hospitals and Health Services Brisbane Queensland Australia
| | - Matthew I. Balcerek
- Department of Endocrinology and Diabetes Royal Brisbane and Women's Hospital Brisbane Queensland Australia
| | - Tom E. McGowan
- Department of Infectious Diseases Royal Brisbane and Women's Hospital Brisbane Queensland Australia
| | - Amanda Love
- Department of Endocrinology and Diabetes Royal Brisbane and Women's Hospital Brisbane Queensland Australia
| | - Paul R. Chapman
- Department of Infectious Diseases Royal Brisbane and Women's Hospital Brisbane Queensland Australia
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Cai Y, Liu Z, Jiang L, Ma D, Zhou Z, Ju H, Zhu Y. Patterns of Lymph Node Metastasis and Optimal Surgical Strategy in Small (≤20 mm) Gastroenteropancreatic Neuroendocrine Tumors. Front Endocrinol (Lausanne) 2022; 13:871830. [PMID: 35937823 PMCID: PMC9350735 DOI: 10.3389/fendo.2022.871830] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/08/2022] [Accepted: 05/24/2022] [Indexed: 11/30/2022] Open
Abstract
BACKGROUND Regional lymph node metastasis (LNM) is crucial for planning additional lymphadenectomy, and is directly correlated with poor prognosis in gastroenteropancreatic neuroendocrine tumors (GEP-NETs). However, the patterns of LNM for small (≤20 mm) GEP-NETs remain unclear. This population-based study aimed at evaluating LNM patterns and identifying optimal surgical strategies from the standpoint of lymph node dissemination. METHODS This retrospective cohort study retrieved data from the Surveillance, Epidemiology, and End Results (SEER) 18 registries database for 17,308 patients diagnosed as having localized well-differentiated GEP-NETs ≤ 20 mm between January 1, 2004, and December 31, 2017. The patterns of LNM were characterized in 6,622 patients who underwent extended resection for adequate lymph node harvest. RESULTS Of 6,622 patients with localized small GEP-NETs in the current study, 2,380 (36%) presented with LNM after regional lymphadenectomy. Nodal involvement was observed in approximately 7.4%, 49.1%, 13.6%, 53.7%, 13.8%, 7.8%, and 15.4% of gastric (g-), small intestinal (si-), appendiceal (a-), colonic (c-), rectal (r-), non-functional pancreatic (nfp-), and functional pancreatic (fp-) NETs ≤ 20 mm. Patients with younger age, larger tumor size, and muscularis invasion were more likely to present with LNM. Additional lymphadenectomy conferred a significant survival advantage in NETs (≤10 mm: HR, 0.47; 95% CI, 0.33-0.66; p < 0.001; 11-20 mm: HR, 0.54; 95% CI, 0.34-0.85; p = 0.008) and fp-NETs ≤ 20 mm (HR, 0.08; 95% CI, 0.02-0.36; p = 0.001), as well as g-NETs (HR, 0.39; 95% CI, 0.16-0.96; p = 0.041) and c-NETs of 11-20 mm (HR, 0.07; 95% CI, 0.01-0.48; p = 0.007). Survival benefits of additional lymphadenectomy were not found in a-NETs, r-NETs, and nfp-NETs with a small size. CONCLUSIONS Given the increased risk for nodal metastasis, primary tumor resection with regional lymphadenectomy is a potential optimal surgical strategy for si-NETs and fp-NETs ≤ 20 mm, as well as g-NETs and c-NETs of 11-20 mm. Local resection is an appropriate and reliable surgical approach for a-NETs, r-NETs, and nfp-NETs ≤ 20 mm.
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Affiliation(s)
| | | | | | | | | | - Haixing Ju
- *Correspondence: Yuping Zhu, ; Haixing Ju,
| | - Yuping Zhu
- *Correspondence: Yuping Zhu, ; Haixing Ju,
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Patel N, Das P, Jain D. Systemic Manifestations of Gastrointestinal Tract Diseases and Systemic Diseases Involving the Gastrointestinal Tract. SURGICAL PATHOLOGY OF THE GASTROINTESTINAL SYSTEM 2022:521-572. [DOI: 10.1007/978-981-16-6395-6_14] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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Abstract
Extracellular calcium is normally tightly regulated by parathyroid hormone (PTH), 1,25-dihydroxyvitamin D, as well as by calcium ion (Ca++) itself. Dysregulated PTH production leading to hypercalcemia occurs most commonly in sporadic primary hyperparathryoidism (PHPT) but may also result from select genetic mutations in familial disorders. Parathyroid hormone-related protein shares molecular mechanisms of action with PTH and is the most common cause of hypercalcemia of malignancy. Other cytokines and mediators may also cause resorptive hypercalcemia once bone metastases have occurred. Less commonly, extrarenal production of calcitriol can occur in malignancies and in infectious and noninfectious inflammatory conditions and can cause hypercalcemia.
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Affiliation(s)
- David Goltzman
- Calcium Research Laboratory, Department of Medicine and Physiology, McGill University, Research Institute of the McGill University Health Centre, Glen Site, 1001 Decarie Boulevard, Room EM1.3220, Montreal, Quebec H4A 3J1, Canada.
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14
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Simonsen JK, Rejnmark L. Endocrine Disorders with Parathyroid Hormone-Independent Hypercalcemia. Endocrinol Metab Clin North Am 2021; 50:711-720. [PMID: 34774242 DOI: 10.1016/j.ecl.2021.07.002] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
The most common causes of hypercalcemia are primary hyperparathyroidism and malignancy, constituting 80% to 90% of all cases. Although less common, several nonparathyroid endocrine disorders are associated with hypercalcemia. The most well described is hyperthyroidism, although the reported prevalence of hypercalcemia in hyperthyroid patients varies depending on applied method for measuring serum calcium levels. Also, adrenal insufficiency, pheochromocytoma, and vasoactive intestinal polypeptide are associated with hypercalcemia. These are differential diagnoses when assessing the hypercalcemic patient for whom common causes have been excluded. Further investigation is needed regarding hypothyroidism; acromegaly, hyperprolactinemia, gonadal dysfunction, and diabetes are not associated with hyperthyroidism.
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Affiliation(s)
- Jo Krogsgaard Simonsen
- Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Palle Juul-Jensens Boulevard 165, Aarhus N 8200, Denmark.
| | - Lars Rejnmark
- Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Palle Juul-Jensens Boulevard 165, Aarhus N 8200, Denmark
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15
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Brugel M, Walter T, Goichot B, Smith D, Lepage C, Do Cao C, Hautefeuille V, Rebours V, Cadiot G, de Mestier L. Efficacy of treatments for VIPoma: A GTE multicentric series. Pancreatology 2021; 21:1531-1539. [PMID: 34404601 DOI: 10.1016/j.pan.2021.08.001] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/09/2021] [Revised: 07/28/2021] [Accepted: 08/04/2021] [Indexed: 12/11/2022]
Abstract
BACKGROUND Vasoactive intestinal peptide-secreting tumor (VIPoma) is a very rare, life-threatening, functioning pancreatic neuroendocrine tumor (pNET). The efficacy of antitumor therapies against functioning symptoms and tumor burden have been poorly described in VIPoma. OBJECTIVE Describe the impact of treatments on the secretory syndrome, tumor burden and survival in patients with VIPoma. METHODS We retrospectively reviewed the records of patients with VIPoma treated in seven French expert centers between 1990 and 2016. Diagnostic of VIPoma was reassessed using strict criteria. We evaluated the antisecretory efficacy (>50 % decrease of daily bowel movements), and antitumor efficacy (RECIST 1.1) of all treatments received. RESULTS Twenty-two patients were included. pNETs were mostly metastatic (77 %) and classified as grade 2 (83 %). Median follow-up was 78.2 months. Surgical excision of nonmetastatic VIPoma effectively controlled the secretory syndrome. Although 4/5 patients had metastatic recurrences, all patients were alive after median post-operative follow-up of 171 months. Among the 87 treatments received for metastatic VIPoma, curative-intent surgery (n = 14), somatostatin analogs alone (n = 11), chemotherapy (n = 23), transarterial liver embolization (TALE) (n = 14), everolimus (n = 10) and sunitinib (n = 7) achieved, respectively, 100 %, 67 %, 83 %, 50 %, 20 % and 100 % antisecretory efficacy. The 5-year OS rate was 63.6 %, with pejorative impact of higher Ki-67 index (P = 0.045) and higher plasma VIP concentration (P = 0.025). CONCLUSIONS Surgical resection of localized VIPoma is effective but rarely curative. For metastatic VIPoma, curative-intent surgery, chemotherapy and sunitinib are the therapeutic options that best combined antitumor and antisecretory efficacies.
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Affiliation(s)
- Mathias Brugel
- Department of Hepato-Gastroenterology and Digestive Oncology, Robert-Debré Hospital and Reims-Champagne-Ardenne University, Reims, France.
| | - Thomas Walter
- Department of Digestive Oncology, ENETS Centre of Excellence, Hospices Civils de Lyon, Lyon, France
| | - Bernard Goichot
- Department of Internal Medicine, Endocrinology & Nutrition, Hôpital Hautepierre, Strasbourg University Hospital, France
| | - Denis Smith
- Department of Hepato-Gastroenterology and Digestive Oncology, Haut-Lévêque Hospital, Bordeaux University Hospital, Pessac, France
| | - Côme Lepage
- Department of Hepato-Gastroenterology and Digestive Oncology, Dijon University Hospital, Dijon, France
| | - Christine Do Cao
- Department of Endocrinology, Hôpital Claude-Huriez, Lille University Hospital, Lille, France
| | - Vincent Hautefeuille
- Department of Hepato-Gastroenterology and Digestive Oncology, Amiens University Hospital, Amiens, France
| | - Vinciane Rebours
- Department of Gastroenterology-Pancreatology, ENETS Centre of Excellence, Hôpital Beaujon and Université de Paris Nord, Clichy, France
| | - Guillaume Cadiot
- Department of Hepato-Gastroenterology and Digestive Oncology, Robert-Debré Hospital and Reims-Champagne-Ardenne University, Reims, France
| | - Louis de Mestier
- Department of Gastroenterology-Pancreatology, ENETS Centre of Excellence, Hôpital Beaujon and Université de Paris Nord, Clichy, France
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16
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Dhillon H, Dowling D, Alexander S. A neuroendocrine cause for refractory symptoms in coeliac disease. JGH Open 2021; 5:1314-1315. [PMID: 34816018 PMCID: PMC8593779 DOI: 10.1002/jgh3.12664] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/03/2021] [Revised: 09/15/2021] [Accepted: 09/20/2021] [Indexed: 11/08/2022]
Abstract
Neuroendocrine tumours while rare, need to be considered in patients with chronic diarrhoea. Reported herein is a case of vasoactive intestinal peptideoma in a patient with refractory diarrhoea following a diagnosis of coeliac disease.
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Affiliation(s)
- Hardesh Dhillon
- Department of Gastroenterology University Hospital Geelong, Barwon Health Geelong Victoria Australia
| | - Damian Dowling
- Department of Gastroenterology University Hospital Geelong, Barwon Health Geelong Victoria Australia.,School of Medicine Deakin University Geelong Victoria Australia
| | - Sina Alexander
- Department of Gastroenterology University Hospital Geelong, Barwon Health Geelong Victoria Australia.,School of Medicine Deakin University Geelong Victoria Australia
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17
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Shiozaki H, Shirai Y, Horiuchi T, Yasuda J, Furukawa K, Onda S, Gocho T, Shiba H, Ikegami T. Feasible laparoscopic distal pancreatectomy for pancreatic neuroendocrine tumors. Mol Clin Oncol 2021; 14:111. [PMID: 33903817 DOI: 10.3892/mco.2021.2273] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/10/2020] [Accepted: 02/15/2021] [Indexed: 11/05/2022] Open
Abstract
Pancreatic neuroendocrine tumor (PNET) cases are increasing; however, the treatment indication and procedure remain unestablished. The present study evaluated the indication, feasibility and safety of laparoscopic distal pancreatectomy (LDP) with our technique for PNET. A total of 13 patients with insulinoma and nonfunctional PNET <2 cm in diameter who underwent LDP and 13 patients with any size of PNET who underwent open distal pancreatectomy (ODP) between October 2009 and June 2019 were retrospectively reviewed and compared. The median age of patients was 45 (33-61) years, and 14 (54%) patients were male. The median follow-up periods were 70 months for the LDP group and 46 months for the ODP group. The tumor diameter of the patients who underwent LDP for PNET was 18±9 mm compared with 37±25 mm for those who underwent ODP. The operation time, estimated blood loss, and complication were 290.2±115 vs. 337±131 min (P=0.338), 122±172 vs. 649±693 ml (P=0.019) and 31 vs. 54% (P=0.234), respectively. Pancreatic fistula developed in 8% of patients who underwent LDP compared with 31% who underwent ODP (P=0.131). Notably, the postoperative hospitalization period was significantly shorter in the LDP group (11±7 vs. 21±13 days; P=0.022). Tumor grade of 2017 World Health Organization classification (G1/G2/G3/NEC/unknown) was 9/2/0/0/2 for the LDP group compared with 5/5/0/3/0 for the ODP group. Furthermore, lymph node metastasis was detected in only 1 patient who underwent ODP, for whom the maximum tumor diameter was 70 mm and was classified as G2. In addition, 2 patients in the ODP group developed postoperative lung and liver metastases. LDP for PNETs of <2 cm in selected patients can be safely performed; however, the extent of lymph node dissection needs to be clarified.
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Affiliation(s)
- Hironori Shiozaki
- Department of Surgery, The Jikei University School of Medicine, Minato-ku, Tokyo 105-8461, Japan
| | - Yoshihiro Shirai
- Department of Surgery, The Jikei University School of Medicine, Minato-ku, Tokyo 105-8461, Japan
| | - Takashi Horiuchi
- Department of Surgery, The Jikei University School of Medicine, Minato-ku, Tokyo 105-8461, Japan
| | - Jungo Yasuda
- Department of Surgery, The Jikei University School of Medicine, Minato-ku, Tokyo 105-8461, Japan
| | - Kenei Furukawa
- Department of Surgery, The Jikei University School of Medicine, Minato-ku, Tokyo 105-8461, Japan
| | - Shinji Onda
- Department of Surgery, The Jikei University School of Medicine, Minato-ku, Tokyo 105-8461, Japan
| | - Takeshi Gocho
- Department of Surgery, The Jikei University School of Medicine, Minato-ku, Tokyo 105-8461, Japan
| | - Hiroaki Shiba
- Department of Surgery, The Jikei University School of Medicine, Minato-ku, Tokyo 105-8461, Japan
| | - Toru Ikegami
- Department of Surgery, The Jikei University School of Medicine, Minato-ku, Tokyo 105-8461, Japan
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18
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Sánchez-Salazar SM, Torres-Alzate S, Muñoz-Cortés VM, Builes-Barrera CA, Gutiérrez-Montoya JI, Román-González A. VIPoma: a rare cause of diarrhea. A case report. REVISTA DE LA FACULTAD DE MEDICINA 2021. [DOI: 10.15446/revfacmed.v69n3.81603] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022] Open
Abstract
Introduction: Vasoactive intestinal peptide-secreting tumor (VIPoma) is a rare functional pancreatic neuroendocrine tumor (F-PNET) characterized by secretory diarrhea, hypokalemia, and hypochlorhydria. Its low incidence and high risk of malignancy pose a clinical challenge that requires a high degree of clinical suspicion.
Case presentation: A 61-year-old woman visited the emergency department of a tertiary care hospital in Medellín, Colombia, due to chronic diarrhea (7 months) that led to dehydration, renal failure, metabolic acidosis, and hypokalemia. As a result, a treatment based on loperamide, intravenous fluids and broad-spectrum antibiotics was started. In addition, chromogranin A levels of 477 ug/L (<100) were reported, while an abdominal MRI showed a 33x30mm mass in the head and uncinate process of the pancreas, so outpatient surgical management was decided. However, three days after discharge, and due to the persistence of clinical signs, the patient was admitted to another hospital (also a tertiary care hospital), where, given the high suspicion of VIPoma, and once the diarrhea was solved, the mass was removed (Whipple procedure) without any complication. Finally, the diagnosis was confirmed based on serum vasoactive intestinal peptide levels (930 pg/mL (RV<75)) and the pathology report (PNET tumor grade 2). Two years after the surgery, the patient was asymptomatic, and no residual lesions or metastases were evident in a control MRI.
Conclusion: Late diagnosis of VIPoma is associated with worsened quality of life, severe complications, and high prevalence of metastasis. Therefore, it should be suspected in patients with chronic secretory diarrhea that is not caused by an infection, since early diagnosis and timely treatment can contribute to achieving better survival rates in these patients.
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19
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Hu Y, Ye Z, Wang F, Qin Y, Xu X, Yu X, Ji S. Role of Somatostatin Receptor in Pancreatic Neuroendocrine Tumor Development, Diagnosis, and Therapy. Front Endocrinol (Lausanne) 2021; 12:679000. [PMID: 34093445 PMCID: PMC8170475 DOI: 10.3389/fendo.2021.679000] [Citation(s) in RCA: 33] [Impact Index Per Article: 8.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/10/2021] [Accepted: 04/27/2021] [Indexed: 12/02/2022] Open
Abstract
Pancreatic neuroendocrine tumors (pNETs) are rare and part of the diverse family of neuroendocrine neoplasms (NENs). Somatostatin receptors (SSTRs), which are widely expressed in NENs, are G-protein coupled receptors that can be activated by somatostatins or its synthetic analogs. Therefore, SSTRs have been widely researched as a diagnostic marker and therapeutic target in pNETs. A large number of studies have demonstrated the clinical significance of SSTRs in pNETs. In this review, relevant literature has been appraised to summarize the most recent empirical evidence addressing the clinical significance of SSTRs in pNETs. Overall, these studies have shown that SSTRs have great value in the diagnosis, treatment, and prognostic prediction of pNETs; however, further research is still necessary.
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Affiliation(s)
- Yuheng Hu
- Department of Pancreatic Surgery, Fudan University Shanghai Cancer Center, Shanghai, China
- Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
- Shanghai Pancreatic Cancer Institute, Shanghai, China
- Pancreatic Cancer Institute, Fudan University, Shanghai, China
| | - Zeng Ye
- Department of Pancreatic Surgery, Fudan University Shanghai Cancer Center, Shanghai, China
- Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
- Shanghai Pancreatic Cancer Institute, Shanghai, China
- Pancreatic Cancer Institute, Fudan University, Shanghai, China
| | - Fei Wang
- Department of Pancreatic Surgery, Fudan University Shanghai Cancer Center, Shanghai, China
- Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
- Shanghai Pancreatic Cancer Institute, Shanghai, China
- Pancreatic Cancer Institute, Fudan University, Shanghai, China
| | - Yi Qin
- Department of Pancreatic Surgery, Fudan University Shanghai Cancer Center, Shanghai, China
- Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
- Shanghai Pancreatic Cancer Institute, Shanghai, China
- Pancreatic Cancer Institute, Fudan University, Shanghai, China
| | - Xiaowu Xu
- Department of Pancreatic Surgery, Fudan University Shanghai Cancer Center, Shanghai, China
- Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
- Shanghai Pancreatic Cancer Institute, Shanghai, China
- Pancreatic Cancer Institute, Fudan University, Shanghai, China
| | - Xianjun Yu
- Department of Pancreatic Surgery, Fudan University Shanghai Cancer Center, Shanghai, China
- Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
- Shanghai Pancreatic Cancer Institute, Shanghai, China
- Pancreatic Cancer Institute, Fudan University, Shanghai, China
- *Correspondence: Xianjun Yu, ; Shunrong Ji,
| | - Shunrong Ji
- Department of Pancreatic Surgery, Fudan University Shanghai Cancer Center, Shanghai, China
- Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
- Shanghai Pancreatic Cancer Institute, Shanghai, China
- Pancreatic Cancer Institute, Fudan University, Shanghai, China
- *Correspondence: Xianjun Yu, ; Shunrong Ji,
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20
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Eng K, Wiseman D, Van Uum S, Quan D, Sanatani M, Mujoomdar A. Percutaneous Cryoablation of Recurrent Pancreatic Mass for Life-Threatening Pancreatic VIPoma Syndrome: A Case Report. Cardiovasc Intervent Radiol 2020; 44:163-166. [PMID: 32914368 DOI: 10.1007/s00270-020-02630-2] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/11/2020] [Accepted: 08/14/2020] [Indexed: 11/29/2022]
Abstract
We present the case of a 73-year-old female who underwent percutaneous cryoablation for recurrent life-threatening pancreatic vasoactive intestinal polypeptide-producing tumor (VIPoma) following a pancreaticoduodenectomy and chemotherapy 5 years earlier. She presented with profuse watery diarrhea causing severe electrolyte and acid-base abnormalities, along with acute kidney injury. Cryoablation was successful in treating her profound symptoms, completely reversing her clinical course. The patient has made a successful recovery for the last 1.5 years since the procedure.
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Affiliation(s)
- Kevin Eng
- Division of Interventional Radiology, Department of Medical Imaging, London Health Sciences Centre, Western University, London, ON, Canada
| | - Daniele Wiseman
- Division of Interventional Radiology, Department of Medical Imaging, London Health Sciences Centre, Western University, London, ON, Canada
| | - Stan Van Uum
- Division of Endocrinology, Department of Medicine, St. Joseph's Health Care, Western University, London, ON, Canada
| | - Douglas Quan
- Division of General Surgery, Department of Surgery, London Health Sciences Centre, Western University, London, ON, Canada
| | - Michael Sanatani
- Division of Medical Oncology, London Regional Cancer Program, Western University, London, ON, Canada
| | - Amol Mujoomdar
- Division of Interventional Radiology, Department of Medical Imaging, London Health Sciences Centre, Western University, London, ON, Canada.
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21
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Abstract
OBJECTIVE. Multiple endocrine neoplasia (MEN) syndromes are autosomal-dominant genetic disorders that predispose two or more organs of the endocrine system to tumor development. Although the diagnosis relies on clinical and serologic findings, imaging provides critical information for surgical management with the ultimate goal of complete tumor resection. CONCLUSION. This article reviews abdominal neoplasms associated with the various subtypes of MEN syndromes, with a focus on clinical presentation and characteristic imaging features.
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22
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Shankar N, Linzay C, Rowe K. Vasoactive intestinal peptide-oma causing refractory diarrhea in a young woman. Proc (Bayl Univ Med Cent) 2020; 33:641-643. [PMID: 33100555 DOI: 10.1080/08998280.2020.1778963] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022] Open
Abstract
Vasoactive intestinal peptide (VIP)-secreting neuroendocrine tumors are an exceptionally rare cause of chronic diarrhea. We describe a 36-year-old woman presenting with a 2-year history of severe diarrhea and electrolyte derangements ultimately diagnosed with VIPoma.
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Affiliation(s)
- Nagasri Shankar
- Department of Internal Medicine, Baylor University Medical Center, Dallas, Texas
| | - Catherine Linzay
- Division of Gastroenterology, Baylor University Medical Center, Dallas, Texas
| | - Kyle Rowe
- Division of Gastroenterology, Baylor University Medical Center, Dallas, Texas
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23
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Gomes-Porras M, Cárdenas-Salas J, Álvarez-Escolá C. Somatostatin Analogs in Clinical Practice: a Review. Int J Mol Sci 2020; 21:ijms21051682. [PMID: 32121432 PMCID: PMC7084228 DOI: 10.3390/ijms21051682] [Citation(s) in RCA: 132] [Impact Index Per Article: 26.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/31/2019] [Revised: 02/22/2020] [Accepted: 02/25/2020] [Indexed: 12/14/2022] Open
Abstract
Somatostatin analogs are an invaluable therapeutic option in the diagnosis and treatment of somatotropinomas, thyrotropinomas, and functioning and non-functioning gastroenteropancreatic neuroendocrine tumors. They should also be considered an effective and safe therapeutic alternative to corticotropinomas, gonadotropinomas, and prolactinomas resistant to dopamine agonists. Somatostatin analogs have also shown to be useful in the treatment of other endocrine diseases (congenital hyperinsulinism, Graves’ orbitopathy, diabetic retinopathy, diabetic macular edema), non-endocrine tumors (breast, colon, prostate, lung, and hepatocellular), and digestive diseases (chronic refractory diarrhea, hepatorenal polycystosis, gastrointestinal hemorrhage, dumping syndrome, and intestinal fistula).
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Affiliation(s)
- Mariana Gomes-Porras
- Department of Endocrinology, “La Paz” University Hospital. Paseo de la Castellana, 261, 28046 Madrid, Spain;
| | - Jersy Cárdenas-Salas
- Department of Endocrinology, “Fundación Jiménez-Diaz” University Hospital. Av. de los Reyes Católicos, 2, 28040 Madrid, Spain;
| | - Cristina Álvarez-Escolá
- Department of Endocrinology, “La Paz” University Hospital. Paseo de la Castellana, 261, 28046 Madrid, Spain;
- Correspondence: ; Tel.: +34-917-277-209
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24
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Abstract
Vasoactive intestinal peptide-secreting tumors (VIPomas) are a group of rare neuroendocrine tumors, which cause a typical syndrome of watery diarrhea. Most of these tumors are found in the pancreas and are usually detected at a later stage. Although curative resection is not possible in most of these tumors, both symptom and tumor control can be achieved by a multidimensional approach, to enable a long survival of most patients. There are no clear-cut guidelines for the management of VIPomas because of the rarity of this neoplasm and lack of prospective data. In this review, we discuss the available evidence on the clinical features and management of these rare tumors.
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25
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Abdullayeva L. VIPoma: Mechanisms, clinical presentation, diagnosis and treatment (Review). WORLD ACADEMY OF SCIENCES JOURNAL 2019. [DOI: 10.3892/wasj.2019.22] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/06/2025]
Affiliation(s)
- Leila Abdullayeva
- Department of Clinical Anatomy and Operative Surgery, Joint Stock Company ‘National Medical University’, A05H2A0 Almaty, Kazakhstan
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26
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Diagnostic and Management Challenges in Vasoactive Intestinal Peptide Secreting Tumors: A Series of 15 Patients. Pancreas 2019; 48:934-942. [PMID: 31268974 DOI: 10.1097/mpa.0000000000001347] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
OBJECTIVES Vasoactive intestinal peptide-secreting tumors (VIPomas) are rare functioning neuroendocrine tumors often characterized by a difficult-to-control secretory syndrome and high potential to develop metastases. We hereby present the characteristics of 15 cases of VIPomas and provide a recent literature review. METHODS This was a retrospective data analysis of 15 patients with VIPoma from 3 different centers and literature research through PubMed database during the last 10 years. RESULTS Fifteen patients with VIPomas (9 with hepatic metastases at diagnosis) with watery diarrhea and raised VIP levels were studied. Ten patients (67%) had grade 2 tumors, 6 of 15 had localized disease and underwent potentially curative surgery, whereas the remaining 9 received multiple systemic therapies; 3 patients died during follow-up. The median overall survival was 71 months (range, 41-154 months). Patients who were treated with curative surgery (n = 7) had longer median overall survival compared with patients who were treated with other therapeutic modalities (44 vs 33 months). CONCLUSIONS The management of VIPomas is challenging requiring the application of multiple treatment modalities. Patients who underwent surgical treatment with curative intent appear to have higher survival rate. Central registration and larger prospective studies are required to evaluate the effect of currently employed therapies in these patients.
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Briganti V, Cuccurullo V, Di Stasio GD, Mansi L. Gamma Emitters in Pancreatic Endocrine Tumors Imaging in the PET Era: Is there a Clinical Space for 99mTc-peptides? Curr Radiopharm 2019; 12:156-170. [DOI: 10.2174/1874471012666190301122524] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/28/2018] [Revised: 06/15/2018] [Accepted: 10/19/2018] [Indexed: 12/18/2022]
Abstract
Background:
Pancreatic Neuroendocrine Tumors (PNETs) are rare neoplasms, sporadic or
familial, even being part of a syndrome. Their diagnosis is based on symptoms, hormonal disorders or
may be fortuitous. The role of Nuclear Medicine is important, mainly because of the possibility of a
theranostic strategy. This approach is allowed by the availability of biochemical agents, which may be
labeled with radionuclides suitable for diagnostic or therapeutic purposes, showing almost identical
pharmacokinetics. The major role for radiopharmaceuticals is connected with radiolabeled Somatostatin
Analogues (SSA), since somatostatin receptors are highly expressed on some of the neoplastic
cell types.
Discussion:
Nowadays, in the category of radiolabeled SSA, although 111In-pentetreotide, firstly
commercially proposed, is still used, the best choice for diagnosis is related to the so called DOTAPET
radiotracers labeled with 68-Gallium (Ga), such as 68Ga-DOTATATE, 68Ga-DOTANOC, and
68Ga-DOTATOC. More recently, labeling with 64-Copper (Cu) (64Cu-DOTATATE) has also been
proposed. In this review, we discuss the clinical interest of a SAA (Tektrotyd©) radiolabeled with
99mTc, a gamma emitter with better characteristics, with respect to 111Indium, radiolabeling Octreoscan
©. By comparing both pharmacokinetics and pharmacodynamics of Octreoscan©, Tektrotyd©
and PET DOTA-peptides, on the basis of literature data and of our own experience, we tried to highlight
these topics to stimulate further studies, individuating actual clinical indications for all of these
radiotracers.
Conclusion:
In our opinion, Tektrotyd© could already find its applicative dimension in the daily practice
of NETs, either pancreatic or not, at least in centers without a PET/CT or a 68Ga generator. Because
of wider availability, a lower cost, and a longer decay, compared with respect to peptides labeled
with 68Ga, it could be also proposed, in a theranostic context, for a dosimetry evaluation of patients
undergoing Peptide Receptor Radionuclide Therapy (PRRT), and for non-oncologic indications
of radiolabelled SSA. In this direction, and for a more rigorous cost/effective evaluation, more precisely
individuating its clinical role, further studies are needed.
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Affiliation(s)
- Vittorio Briganti
- Nuclear Medicine Unit - Azienda Ospedaliero Universitaria Careggi Firenze, Italy
| | - Vincenzo Cuccurullo
- Nuclear Medicine Unit, Department of Clinical and Experimental Medicine "F.Magrassi, A.Lanzara" – Università della Campania "Luigi Vanvitelli", Napoli, Italy
| | - Giuseppe Danilo Di Stasio
- Nuclear Medicine Unit, Department of Clinical and Experimental Medicine "F.Magrassi, A.Lanzara" – Università della Campania "Luigi Vanvitelli", Napoli, Italy
| | - Luigi Mansi
- Health and Development Section, Interuniversitary Research Center for Sustainable Development, Napoli-Roma, Italy
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28
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Zamir MA, Hakim W, Yusuf S, Thomas R. Imaging of Pancreatic-Neuroendocrine Tumours: An Outline of Conventional Radiological Techniques. Curr Radiopharm 2019; 12:135-155. [DOI: 10.2174/1874471012666190214165845] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/12/2018] [Revised: 09/19/2018] [Accepted: 10/30/2018] [Indexed: 01/18/2023]
Abstract
IIntroduction:
Pancreatic Neuroendocrine Tumours (p-NETs) are an important disease entity
and comprise of peptide-secreting tumours often with a functional syndrome.
:
Accounting for a small percentage of all pancreatic tumours, they have a good overall survival rate
when diagnosed early, with surgery being curative. The role of nuclear medicine in the diagnosis and
treatment of these tumours is evident. However, the vast majority of patients will require extensive imaging
in the form of conventional radiological techniques. It is important for clinicians to have a fundamental
understanding of the p-NET appearances to aid prompt identification and to help direct management
through neoplastic staging.
Methods:
This article will review the advantages and disadvantages of conventional radiological techniques
in the context of p-NETs and highlight features that these tumours exhibit.
Conclusion:
Pancreatic neuroendocrine tumours are a unique collection of neoplasms that have markedly
disparate clinical features but similar imaging characteristics. Most p-NETs are small and welldefined
with homogenous enhancement following contrast administration, although larger and less welldifferentiated
tumours can demonstrate areas of necrosis and cystic architecture with heterogeneous
enhancement characteristics.
:
Prognosis is generally favourable for these tumours with various treatment options available. However,
conventional radiological techniques will remain the foundation for the initial diagnosis and staging of
these tumours, and a grasp of these modalities is extremely important for physicians.
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Affiliation(s)
- Muhammad Affan Zamir
- Hammersmith Hospital, Imperial College Healthcare NHS Trust, London W12 0HS, United Kingdom
| | - Wasim Hakim
- Hammersmith Hospital, Imperial College Healthcare NHS Trust, London W12 0HS, United Kingdom
| | - Siraj Yusuf
- Hammersmith Hospital, Imperial College Healthcare NHS Trust, London W12 0HS, United Kingdom
| | - Robert Thomas
- Hammersmith Hospital, Imperial College Healthcare NHS Trust, London W12 0HS, United Kingdom
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Krainev AA, Mathavan VK, Klink DF, Saxe JM, Ong GKB, Murphy JK. Pancreatic neuroendocrine microadenomatosis presenting as a functional VIPoma. J Surg Case Rep 2019; 2019:rjz196. [PMID: 31275551 PMCID: PMC6598297 DOI: 10.1093/jscr/rjz196] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/15/2019] [Revised: 05/03/2019] [Accepted: 06/05/2019] [Indexed: 01/14/2023] Open
Abstract
Pancreatic microadenomas are benign tumors of neuroendocrine origin less than 5 mm in size. Whereas most microadenomas are non-functional; a few rare functional pancreatic microadenomas have been described in the setting of multiple endocrine neoplasia type one (MEN-1). In this report, we describe a unique case of multiple functional microadenomas of the pancreatic head in a patient who presented with persistent secretory diarrhea, refractory hypokalemia, metabolic acidosis and elevated plasma vasoactive intestinal peptide (VIP) levels. Following extensive serologic, radiographic and endoscopic work up, our patient underwent open pancreaticoduodenectomy with subsequent resolution of diarrheal symptoms and electrolyte abnormalities on postoperative follow up.
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Affiliation(s)
- Alexei A Krainev
- St. Vincent Hospital General Surgery Residency Program, St. Vincent Hospital Indianapolis, St. Vincent Ortho Center 2001 West 86th Street, Indianapolis, IN, USA
| | - Viney K Mathavan
- St. Vincent Hospital Indianapolis/North Indy Surgical Group, Indianapolis, IN, USA
| | - Daniel F Klink
- St. Vincent Hospital Indianapolis, Department of Pathology/AmeriPath Anatomic Pathology Services, Indianapolis, IN, USA
| | - Jonathan M Saxe
- St. Vincent Hospital General Surgery Residency Program, St. Vincent Hospital Indianapolis, St. Vincent Ortho Center 2001 West 86th Street, Indianapolis, IN, USA
| | - Gabie K B Ong
- St. Vincent Hospital General Surgery Residency Program, St. Vincent Hospital Indianapolis, St. Vincent Ortho Center 2001 West 86th Street, Indianapolis, IN, USA
| | - Jamie K Murphy
- St. Vincent Hospital General Surgery Residency Program, St. Vincent Hospital Indianapolis, St. Vincent Ortho Center 2001 West 86th Street, Indianapolis, IN, USA
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Farina DA, Krogh KM, Boike JR. Chronic Diarrhea Secondary to Newly Diagnosed VIPoma. Case Rep Gastroenterol 2019; 13:225-229. [PMID: 31123451 PMCID: PMC6514521 DOI: 10.1159/000494554] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/27/2018] [Accepted: 10/15/2018] [Indexed: 11/23/2022] Open
Abstract
Vasoactive intestinal polypeptide-secreting tumors (VIPoma) are a rare pancreatic neuroendocrine tumor that can cause chronic diarrhea with 1 case per 10 million people per year. Diagnosis is made based on a combination of laboratory evaluation (serum VIP level), imaging findings (functional positron emission tomography-computed tomography [PET-CT]), and histological analysis (chromogranin A stain). We present a case of a male with 6 months of diarrhea and malaise who was found to have significant kidney injury and hypokalemia requiring admission to the medical intensive care unit. Subsequent laboratory evaluation while admitted eventually showed a low stool osmotic gap (–11 mOsm/kg) consistent with secretory diarrhea, in addition to significantly elevated VIP levels at 940 pg/mL (normal <75). Cross-sectional imaging with functional Gallium-68 dotatate PET-CT confirmed metastatic functional neuroendocrine tumor indicative of a VIPoma. Pathology on subsequent metastatic liver lesion aspiration was consistent with a well-differentiated VIPoma, and symptoms dramatically improved following initiation of octreotide therapy.
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Affiliation(s)
- Domenico A Farina
- Department of Medicine, Northwestern University, Chicago, Illinois, USA
| | - Katrina M Krogh
- Department of Pathology, Northwestern University, Chicago, Illinois, USA
| | - Justin R Boike
- Division of Gastroenterology and Hepatology, Northwestern University, Chicago, Illinois, USA
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31
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Clinicopathological features and surgical outcomes of resected functional pancreatic neuroendocrine tumors: a single institution experience. JOURNAL OF PANCREATOLOGY 2019. [DOI: 10.1097/jp9.0000000000000018] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022] Open
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Guilmette J, Nosé V. Paraneoplastic syndromes and other systemic disorders associated with neuroendocrine neoplasms. Semin Diagn Pathol 2019; 36:229-239. [PMID: 30910348 DOI: 10.1053/j.semdp.2019.03.002] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/19/2023]
Abstract
Neuroendocrine paraneoplastic syndromes (PNS) consist of metabolic disorders that accompany benign and malignant neoplasms but remain unrelated to mass effects or invasion by the primary tumor or its metastases. The underlying pathogenesis responsible for PNS usual clinical presentation relies on aberrant production of protein hormones, proteins and other substances by the tumor. Prompt recognition of characteristic signs and symptoms combined with serological identification of key substances may result in early diagnosis of PNS and its underlying malignancy. For these reasons, healthcare professionals should familiarize themselves with tumor-induced hypercalcemia, syndrome of inappropriate antidiuretic hormone, carcinoid syndrome, virilisation syndrome, gynecomastia, acromegaly, Cushing syndrome, osteogenic osteomalacia, tumor-induced hypoglycemia, necrolytic migratory erythema, and watery diarrhea, hypokalemia and achlorydria syndrome. Medical awareness for PNS can improve patient outcomes through earlier administration of cancer therapy and treatment, better symptomatic relief and prolong overall survival.
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Affiliation(s)
- Julie Guilmette
- Department of Pathology, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA 02114-2696, United States; Department of Pathology, Charles-Lemoyne Hospital, Greenfield Park, Quebec, Canada
| | - Vânia Nosé
- Department of Pathology, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA 02114-2696, United States.
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Chen C, Zheng Z, Li B, Zhou L, Pang J, Wu W, Zheng C, Zhao Y. Pancreatic VIPomas from China: Case reports and literature review. Pancreatology 2019; 19:44-49. [PMID: 30391116 DOI: 10.1016/j.pan.2018.10.007] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/17/2018] [Revised: 10/08/2018] [Accepted: 10/18/2018] [Indexed: 01/30/2023]
Abstract
Vasoactive intestinal polypeptide-secreting tumors (VIPomas) are rare neuroendocrine tumors that often present as watery diarrhea, hypokalemia, and achlorhydria or hypochlorhydria. In this study, we present our institutional experience of diagnosis and treatment of VIPomas, along with a review of the Chinese literature since 1980. Patient #1, diagnosed in 1984 and with intact clinical records, shows the natural history of this disease. Patient #2, diagnosed in 2015, shows the results of evaluation by nuclear medicine techniques and the outcomes of standardized treatment. Comprehensive review of 41 cases allows evaluation of clinical characteristics, treatments and outcomes of pancreatic VIPoma patients. All patients presented with watery diarrhea. The average stool volume reached 3247 mL per day. Average serum VIP level was 839.3 ng/L. Twelve of the 41 cases were reported to have metastases at diagnosis. Somatostatin receptor scintigraphy and 18FDG PET-CT are efficient methods for detection of VIPoma. Surgical excision can promptly alleviate hormonal symptoms.
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Affiliation(s)
- Chuyan Chen
- Department of General Surgery, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Zhibo Zheng
- Department of General Surgery, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Binglu Li
- Department of General Surgery, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.
| | - Liangrui Zhou
- Department of Pathology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Junyi Pang
- Department of Pathology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Wenming Wu
- Department of General Surgery, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Chaoji Zheng
- Department of General Surgery, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Yupei Zhao
- Department of General Surgery, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
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Neoplasms of the Neuroendocrine Pancreas: An Update in the Classification, Definition, and Molecular Genetic Advances. Adv Anat Pathol 2019; 26:13-30. [PMID: 29912000 DOI: 10.1097/pap.0000000000000201] [Citation(s) in RCA: 71] [Impact Index Per Article: 11.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
This review focuses on discussing the main modifications of the recently published 2017 WHO Classification of Neoplasms of the Neuroendocrine Pancreas (panNEN). Recent updates separate pancreatic neuroendocrine tumors into 2 broad categories: well-differentiated pancreatic neuroendocrine tumors (panNET) and poorly differentiated pancreatic neuroendocrine carcinoma (panNEC), and incorporates a new subcategory of "well-differentiated high-grade NET (G3)" to the well-differentiated NET category. This new classification algorithm aims to improve the prediction of clinical outcomes and survival and help clinicians select better therapeutic strategies for patient care and management. In addition, these neuroendocrine neoplasms are capable of producing large quantity of hormones leading to clinical hormone hypersecretion syndromes. These functioning tumors include, insulinomas, glucagonomas, somatostatinomas, gastrinomas, VIPomas, serotonin-producing tumors, and ACTH-producing tumors. Although most panNENs arise as sporadic diseases, a subset of these heterogeneous tumors present as parts on inherited genetic syndromes, such as multiple endocrine neoplasia type 1, von Hippel-Lindau, neurofibromatosis type 1, tuberous sclerosis, and glucagon cell hyperplasia and neoplasia syndromes. Characteristic clinical and morphologic findings for certain functioning and syndromic panNENs should alert both pathologists and clinicians as appropriate patient management and possible genetic counseling may be necessary.
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35
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Ausania F, Senra Del Rio P. Lymphadenectomy in pancreatic neuroendocrine neoplasms: Why are we still debating? Pancreatology 2018; 18:855-861. [PMID: 30253923 DOI: 10.1016/j.pan.2018.09.005] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/07/2018] [Revised: 08/26/2018] [Accepted: 09/18/2018] [Indexed: 02/07/2023]
Abstract
Pancreatic Neuroendocrine Neoplasms (PNEN) are rare tumours exhibiting very heterogeneous behaviour. For these reasons, studies with high level of evidence are lacking. Whether lymphadenectomy should be performed for PNEN is a matter of debate. In this review, we perform a critical analysis of the available literature regarding the clinical significance of lymphnode metastases, the importance of lymphadenectomy, and the implications on disease-specific survival.
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Affiliation(s)
- F Ausania
- HPB Surgery, Hospital Alvaro Cunqueiro, Complejo Hospitalario Universitario de Vigo, Spain.
| | - P Senra Del Rio
- HPB Surgery, Hospital Alvaro Cunqueiro, Complejo Hospitalario Universitario de Vigo, Spain
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36
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Abstract
Peptide hormones represent a major class of hormones that are made from amino acids by specialized endocrine glands. The maturation of bioactive hormones take place in the rough endoplasmic reticulum and Golgi apparatus, where preprohormones are proteolytically cleaved into prohormones, and subsequently into mature peptide hormones. Once the bioactive hormones are released into the circulation, they interact with receptors located on the plasma membrane of target cells, and initiate intracellular signaling pathways to regulate physiological processes including energy metabolism, growth, stress, and reproduction. However, excessive amount of circulating peptide hormones often associates with the presence of tumors. Section 2 discusses 10 peptide hormones as tumor markers and their clinical application in aiding the diagnosis of tumors as well as monitoring the disease process.
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Affiliation(s)
- Qian Sun
- National Institutes of Health, Bethesda, MD, United States
| | - Zhen Zhao
- National Institutes of Health, Bethesda, MD, United States.
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37
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ElGuindy YM, Javadi S, Menias CO, Jensen CT, Elsamaloty H, Elsayes KM. Imaging of secretory tumors of the gastrointestinal tract. Abdom Radiol (NY) 2017; 42:1113-1131. [PMID: 27878636 DOI: 10.1007/s00261-016-0976-4] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
Gastrointestinal secretory tumors, or gastroenteropancreatic neuroendocrine tumors, encompass a wide array of endocrine cell tumors. The significance of these tumors lies in their ability to alter physiology through hormone production as we well as in their malignant potential. Functioning tumors may present earlier due to symptomatology; conversely, non-functioning tumors are often diagnosed late as they reach large sizes, causing symptoms secondary to local mass effect. Imaging aids in the diagnosis, staging, and prognosis and provides key information for presurgical planning. Although most of these tumors are sporadic, some are associated with important syndromes and associations, knowledge of which is critical for patient management. In this article, we provide an overview of secretory and neuroendocrine tumors of the GI tract and pancreas.
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Nilubol N, Freedman EM, Quezado MM, Patel D, Kebebew E. Pancreatic Neuroendocrine Tumor Secreting Vasoactive Intestinal Peptide and Dopamine With Pulmonary Emboli: A Case Report. J Clin Endocrinol Metab 2016; 101:3564-3567. [PMID: 27583474 PMCID: PMC5052354 DOI: 10.1210/jc.2016-2051] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
CONTEXT The vasoactive intestinal peptide-secreting neuroendocrine tumor (VIPoma) is a very rare pancreatic tumor. We report the first case of a patient with VIPoma that co-secreted dopamine and had pulmonary emboli. CASE DESCRIPTION A 67-year-old woman presented with 2 months of watery diarrhea, severe generalized weakness,6.8 kg of weight loss, a facial rash, and hypokalemia. Colonoscopy did not reveal the cause of the chronic diarrhea. Initial biochemical testing showed markedly elevated serum vasoactive intestinal peptide (VIP) and pancreatic polypeptide. Computed tomography scan of the abdomen and pelvis revealed a 5.4-cm distal pancreatic mass. Octreoscan showed an intense uptake in the area of the pancreatic mass. Incidental pulmonary emboli were found and treated. Additional biochemical testing revealed a markedly elevated urinary dopamine level. The patient received preoperative α-blockade and octreotide. She underwent a successful laparoscopic distal pancreatectomy. Postoperative urinary dopamine and pancreatic polypeptide were within normal limits. Serum VIP decreased by half but remained elevated. Pathology confirmed a grade 1 pancreatic neuroendocrine tumor without lymph node metastasis. The patient's symptoms resolved and no longer required octreotide. Metastatic workup including computed tomography, F18-fluorodeoxglucose positron emission tomography, and Ga68-DOTATATE scans were negative during 4 years of follow-up. CONCLUSIONS VIPoma is a rare subtype of pancreatic neuroendocrine tumor that can secrete dopamine and can be associated with thromboembolism.
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Affiliation(s)
- Naris Nilubol
- Endocrine Oncology Branch (N.N., E.M.F., D.P., E.K.) and Laboratory of Pathology (M.M.Q.), Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892
| | - Esther M Freedman
- Endocrine Oncology Branch (N.N., E.M.F., D.P., E.K.) and Laboratory of Pathology (M.M.Q.), Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892
| | - Martha M Quezado
- Endocrine Oncology Branch (N.N., E.M.F., D.P., E.K.) and Laboratory of Pathology (M.M.Q.), Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892
| | - Dhaval Patel
- Endocrine Oncology Branch (N.N., E.M.F., D.P., E.K.) and Laboratory of Pathology (M.M.Q.), Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892
| | - Electron Kebebew
- Endocrine Oncology Branch (N.N., E.M.F., D.P., E.K.) and Laboratory of Pathology (M.M.Q.), Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892
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Dréanic J, Lepère C, El Hajjam M, Gouya H, Rougier P, Coriat R. Emergency therapy for liver metastases from advanced VIPoma: surgery or transarterial chemoembolization? Ther Adv Med Oncol 2016; 8:383-7. [PMID: 27583030 DOI: 10.1177/1758834016656495] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/14/2023] Open
Abstract
VIPoma is a rare neuroendocrine tumor (NET) with a high potential to develop hepatic metastases and poor prognosis. The primitive tumor is nonsymptomatic and usually localized within the pancreas. Liver metastasis drives the prognosis and induces profuse watery diarrhea or renal failure. We herein present severe renal failure or diarrhea in two patients hospitalized in intensive care justifying emergency treatment of liver metastasis. The two patients experienced severe diarrhea due to a hypersecretion of vasoactive intestinal peptide (VIP) from liver metastasis released into the blood circulation. Therapeutic management was discussed and liver transarterial chemoembolization (TACE) was performed with chemotherapy-loaded embospheres, which cause necrosis of tumor lesions. TACE controlled the hormonal syndrome and made patients eligible for curative surgery. Tumor necrosis occurred and VIP levels collapsed. Surgery was performed in one of the two cases after TACE and the patient was considered in remission. Both patients were still alive after 3 years of follow up. Thus, TACE is feasible and appears to be an effective emergency treatment in patients with a VIP-hormonal syndrome due to liver metastases. Despite the biological disorder due to the hormonal secretion, an aggressive approach is warranted in VIP liver metastasis.
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Affiliation(s)
- Johann Dréanic
- Gastroenterology and Endoscopy Unit, Hôpital Cochin, 27, Rue du Faubourg, Saint Jacques F75014, Paris, France
| | - Céline Lepère
- Department of Digestive Oncology, Hôpital Européen Georges Pompidou, Université Paris Descartes, Sorbonne Paris Cité, Faculté de Médecine, AP-HP, Paris, France
| | - Mostafa El Hajjam
- Department of Radiology, Hôpital Ambroise Paré, Université Paris Ouest Versailles, AP-HP, Boulogne, France
| | - Hervé Gouya
- Department of Radiology, Hôpital Cochin, Université Paris Descartes, Sorbonne Paris Cité, Faculté de Médecine, AP-HP, Paris, France
| | - Philippe Rougier
- Department of Digestive Oncology, Hôpital Européen Georges Pompidou, Université Paris Descartes, Sorbonne Paris Cité, Faculté de Médecine, AP-HP, Paris, France
| | - Romain Coriat
- Department of Gastroenterology and Endoscopy, Hôpital Cochin, Université Paris Descartes, Sorbonne Paris Cité, Faculté de Médecine, AP-HP, Paris, France
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40
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Concomitant Case of Intraductal Papillary Mucinous Neoplasm of the Pancreas and Functioning Pancreatic Neuroendocrine Tumor (Vasoactive Intestinal Polypeptide-Producing Tumor): First Report. Pancreas 2016; 45:e24-5. [PMID: 27295534 DOI: 10.1097/mpa.0000000000000578] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
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41
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Pancreatic neuroendocrine tumors: Challenges in an underestimated disease. Crit Rev Oncol Hematol 2016; 101:193-206. [PMID: 27021395 DOI: 10.1016/j.critrevonc.2016.03.013] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/17/2015] [Revised: 02/24/2016] [Accepted: 03/09/2016] [Indexed: 12/20/2022] Open
Abstract
Pancreatic neuroendocrine tumours (PanNETs) are considered a relatively unusual oncologic entity. Due to its relative good prognosis, surgery remains the goal standard therapy not only in localized disease but also in the setting of locally or metastatic disease. Most of the patients are diagnosed in metastatic scenario, where multidisciplinary approach based on surgery, chemotherapies, liver-directed and/or molecular targeted therapies are commonly used. Owing to a deeper molecular knowledge of this disease, these targeted therapies are nowadays widely implemented, being the likely discovery of predictive biomarkers that would allow its use in other settings. This review is focused on describing the different classifications, etiology, prognostic biomarkers and multidisciplinary approaches that are typically used in PanNET.
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Zhang X, Zhou L, Liu Y, Li W, Gao H, Wang Y, Yao B, Jiang D, Hu P. Surgical resection of vasoactive intestinal peptideoma with hepatic metastasis aids symptom palliation: A case report. Exp Ther Med 2016; 11:783-787. [PMID: 26997993 PMCID: PMC4774344 DOI: 10.3892/etm.2016.3019] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/10/2014] [Accepted: 02/05/2015] [Indexed: 12/18/2022] Open
Abstract
Vasoactive intestinal peptideoma (VIPoma) is a rare pancreatic endocrine tumor associated with a well-defined clinical syndrome characterized by watery diarrhea, hypokalemia and metabolic acidosis. In adults, VIPoma is most commonly found in the pancreas, with 80% of the tumors occurring in the body and tail and 20% occurring in the pancreatic head. VIPomas can represent a significant diagnostic challenge due to their nonspecific clinical presentation, which can result in the misdiagnosis of a VIPoma as another condition, such as laxative overdose or a carcinoid secreting tumor. Surgical clearance of the tumor is the first-line treatment, even in cases with metastasis. The present study describes the case of a patient who presented with chronic watery diarrhea and hypokalemia due to a tumor in the pancreatic head, which was confirmed to contain immunoreactive vasoactive intestinal polypeptide via immunohistochemistry. A hepatic metastasis lesion was diagnosed following computed tomography. Stable control of symptoms was achieved after surgery and drug treatment. The study additionally reviews the clinical, histological, radiological and diagnostic features of the condition, as well as the therapeutic modalities that can be used to treat VIPoma in the pancreatic head with hepatic metastasis.
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Affiliation(s)
- Xiaomei Zhang
- Department of Endocrinology, Peking University International Hospital, Beijing 102206, P.R. China
| | - Lingli Zhou
- Department of Endocrinology, Peking University People's Hospital, Beijing 100044, P.R. China
| | - Ying Liu
- Department of Internal Medicine, First Hospital, Dandong, Liaoning 118000, P.R. China
| | - Wei Li
- Department of Surgical Medicine, First Hospital, Dandong, Liaoning 118000, P.R. China
| | - Hongkai Gao
- Department of Surgical Medicine, General Hospital of the Chinese People's Armed Police Forces, Beijing 100039, P.R. China
| | - Yunan Wang
- Department of Internal Medicine, First Hospital, Dandong, Liaoning 118000, P.R. China
| | - Baoting Yao
- Department of Internal Medicine, First Hospital, Dandong, Liaoning 118000, P.R. China
| | - Daming Jiang
- Department of Internal Medicine, Central Hospital, Dandong, Liaoning 118000, P.R. China
| | - Peijun Hu
- Department of Gastroenterology, Beijing First Hospital of Integrated Chinese and Western Medicine, Beijing 100026, P.R. China
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43
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Sakr M. Pancreatic Neuroendocrine Tumors. HEAD AND NECK AND ENDOCRINE SURGERY 2016:367-382. [DOI: 10.1007/978-3-319-27532-1_14] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/06/2025]
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44
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Fujiya A, Kato M, Shibata T, Sobajima H. VIPoma with multiple endocrine neoplasia type 1 identified as an atypical gene mutation. BMJ Case Rep 2015; 2015:bcr-2015-213016. [PMID: 26564120 DOI: 10.1136/bcr-2015-213016] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
A 47-year-old man presented with persistent diarrhoea and hypokalaemia. CT revealed 4 pancreatic tumours that appeared to be VIPomas, because the patient had an elevated plasma vasoactive intestinal polypeptide level. MRI showed a low-intensity area in the pituitary suggestive of a pituitary tumour, and a parathyroid tumour was detected by ultrasonography and 99Tc-MIBI scintigraphy. Given these results, the patient was diagnosed with multiple endocrine neoplasia type 1 (MEN1) and scheduled for surgery. MEN1 is an autosomal dominant disorder associated with MEN1 mutations. Genetic testing indicated that the patient had a MEN1 gene mutation; his 2 sons had the same mutations. Most MEN1 tumours are benign, but some pancreatic and thymic tumours could become malignant. Without treatment, such tumours would result in earlier mortality. Despite its rarity, we should perform genetic testing for family members of patients with MEN1 to identify mutation carriers and improve the patients' prognosis.
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Affiliation(s)
- Atsushi Fujiya
- Department of Diabetology and Nephrology, Ogaki Municipal Hospital, Ogaki, Japan
| | - Makoto Kato
- Department of Diabetology and Nephrology, Ogaki Municipal Hospital, Ogaki, Japan
| | - Taiga Shibata
- Department of Diabetology and Nephrology, Ogaki Municipal Hospital, Ogaki, Japan
| | - Hiroshi Sobajima
- Department of Diabetology and Nephrology, Ogaki Municipal Hospital, Ogaki, Japan
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45
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Pancreatic Neuroendocrine Tumors: an Update. Indian J Surg 2015; 77:395-402. [PMID: 26722203 DOI: 10.1007/s12262-015-1360-2] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/24/2015] [Accepted: 09/30/2015] [Indexed: 02/06/2023] Open
Abstract
Pancreatic neuroendocrine tumors (pNETs) are rare and comprise only 1-2 % of all pancreatic neoplastic disease. Although the majority of these tumors are sporadic (90 %), pNETs can arise in the setting of several different hereditary genetic syndromes, most commonly multiple endocrine neoplasia type 1 (MEN1). The presentation of pNETs varies widely, with over 60 % having malignant distant disease at the time of initial diagnosis involving the liver or other distant sites. Functioning pNETs represent approximately 10 % of all pNETs, secrete a variety of peptide hormones, and are responsible for several clinical syndromes caused by profound hormonal derangement. Surgery remains the cornerstone of therapy and the only curative approach. It should be pursued for localized disease and for metastatic lesions amenable to resection. Multimodality therapies, including liver-directed therapies and medical therapy, are gaining increasing favor in the treatment of advanced pNETs. Their utility is multifold and spans from ameliorating symptoms of hormonal excess (functional pNETs) to controlling the local and systemic disease burden (non-functional pNETs). The recent introduction of target molecular therapy has promising results especially for the treatment of progressive well-differentiated G1/G2 tumor. In this review, we summarize the current knowledge and give an update on recent advancements made in the therapeutic strategies for pNETs.
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46
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Pancreatic Tumor Presenting as Diarrhea. J Gastrointest Cancer 2015; 47:454-457. [PMID: 26453447 DOI: 10.1007/s12029-015-9770-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/22/2022]
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47
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Chen Y, Shi D, Dong F, Han SG, Qian ZH, Yang LI, Wang Y, Yu RS, Li QH, Fu YB. Multiple-phase spiral CT findings of pancreatic vasoactive intestinal peptide-secreting tumor: A case report. Oncol Lett 2015; 10:2351-2354. [PMID: 26622850 DOI: 10.3892/ol.2015.3615] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/30/2014] [Accepted: 07/28/2015] [Indexed: 01/27/2023] Open
Abstract
The present study reports a case of pancreatic vasoactive intestinal peptide-secreting tumor (VIPoma), of 2.2 cm in diameter, arising from the region of the uncus of the pancreas with liver metastases in a 50-year-old patient, which demonstrated unusual multiple-phase spiral computed tomography (MPSCT) results. The pancreatic lesion was isodense compared with the pancreatic parenchyma. During the hepatic artery phase and portal venous phase, the mass was hypodense compared with the enhanced pancreas, with the mean CT attenuation (HU) values being 56 HU and 66 HU, respectively. During the hepatic parenchymal phase, the mass became hyperdense with the mean CT attenuation values being 74 HU. The process of contrast-enhanced MPSCT demonstrated progressive strengthening. Pancreaticoduodenectomy and wedge resection of a number of the liver metastases was performed. There are only a limited number of studies reporting CT findings of pancreatic VIPoma and no MPSCT findings have been described previously.
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Affiliation(s)
- Ying Chen
- Department of Radiology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China
| | - Dan Shi
- Department of Radiology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China
| | - Fei Dong
- Department of Radiology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China
| | - Shu-Gao Han
- Department of Radiology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China
| | - Zi-Hua Qian
- Department of Radiology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China
| | - L I Yang
- Department of Radiology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China
| | - Ying Wang
- Department of Radiology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China
| | - Ri-Sheng Yu
- Department of Radiology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China
| | - Qing-Hai Li
- Department of Radiology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China
| | - Yan-Biao Fu
- Department of Pathology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China
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48
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Apodaca-Torrez FR, Triviño M, Lobo EJ, Goldenberg A, Triviño T. Extra-pancreatic vipoma. ABCD-ARQUIVOS BRASILEIROS DE CIRURGIA DIGESTIVA 2015; 27:222-3. [PMID: 25184777 PMCID: PMC4676380 DOI: 10.1590/s0102-67202014000300015] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 03/19/2013] [Accepted: 03/11/2014] [Indexed: 12/14/2022]
Affiliation(s)
| | - Marcello Triviño
- Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil
| | - Edson José Lobo
- Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil
| | - Alberto Goldenberg
- Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil
| | - Tarcísio Triviño
- Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil
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49
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Qiao Z, Zhang J, Jin X, Huo L, Zhu Z, Xing H, Li F. 99mTc-HYNIC-TOC imaging in the evaluation of pancreatic masses which are potential neuroendocrine tumors. Clin Nucl Med 2015; 40:397-400. [PMID: 25706799 DOI: 10.1097/rlu.0000000000000756] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
PURPOSE The aim of this investigation was to determine the accuracy of the findings and the diagnoses of Tc-hydrazinonicotinyl-Tyr3-octreotide scan (Tc-HYNIC-TOC imaging) in patients with pancreatic masses which were potential neuroendocrine tumors. METHODS Records of total 20 patients with pancreatic masses were retrospectively reviewed. All of the patients had been revealed by abdominal contrast CT and possibility of neuroendocrine tumors could not be excluded by CT imaging before Tc-HYNIC-TOC imaging. Tc-HYNIC-TOC imaging was performed at 1 and 4 hours post-tracer injection, and SPECT/CT images of the abdomen were also acquired. The image findings were compared to final diagnoses which were made from pathological examination. RESULTS Among all 20 pancreatic masses evaluated, there were 16 malignant lesions which included 1 ductal adenocarcinoma and 15 neuroendocrine tumors. Tc-HYNIC-TOC imaging identified 14 of 15 pancreatic neuroendocrine tumors and excluded 4 of 5 lesions which were not neuroendocrine tumors. The overall sensitivity, specificity, and accuracy was therefore 93.3% (14 of 15), 80% (4 of 5), and 90.0% (18 of 20), respectively, in our patient population. CONCLUSION Tc-HYNIC-TOC imaging provides reasonable accuracy in the evaluation pancreatic mass suspected to be neuroendocrine tumors.
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Affiliation(s)
- Zhen Qiao
- From the Peking Union Medical College Hospital Beijing, Beijing, China
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50
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Marcucci G, Cianferotti L, Beck-Peccoz P, Capezzone M, Cetani F, Colao A, Davì MV, degli Uberti E, Del Prato S, Elisei R, Faggiano A, Ferone D, Foresta C, Fugazzola L, Ghigo E, Giacchetti G, Giorgino F, Lenzi A, Malandrino P, Mannelli M, Marcocci C, Masi L, Pacini F, Opocher G, Radicioni A, Tonacchera M, Vigneri R, Zatelli MC, Brandi ML. Rare diseases in clinical endocrinology: a taxonomic classification system. J Endocrinol Invest 2015; 38:193-259. [PMID: 25376364 DOI: 10.1007/s40618-014-0202-6] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/19/2014] [Accepted: 10/17/2014] [Indexed: 02/05/2023]
Abstract
PURPOSE Rare endocrine-metabolic diseases (REMD) represent an important area in the field of medicine and pharmacology. The rare diseases of interest to endocrinologists involve all fields of endocrinology, including rare diseases of the pituitary, thyroid and adrenal glands, paraganglia, ovary and testis, disorders of bone and mineral metabolism, energy and lipid metabolism, water metabolism, and syndromes with possible involvement of multiple endocrine glands, and neuroendocrine tumors. Taking advantage of the constitution of a study group on REMD within the Italian Society of Endocrinology, consisting of basic and clinical scientists, a document on the taxonomy of REMD has been produced. METHODS AND RESULTS This document has been designed to include mainly REMD manifesting or persisting into adulthood. The taxonomy of REMD of the adult comprises a total of 166 main disorders, 338 including all variants and subtypes, described into 11 tables. CONCLUSIONS This report provides a complete taxonomy to classify REMD of the adult. In the future, the creation of registries of rare endocrine diseases to collect data on cohorts of patients and the development of common and standardized diagnostic and therapeutic pathways for each rare endocrine disease is advisable. This will help planning and performing intervention studies in larger groups of patients to prove the efficacy, effectiveness, and safety of a specific treatment.
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Affiliation(s)
- G Marcucci
- Head, Bone Metablic Diseases Unit, Department of Surgery and Translational Medicine, University of Florence, Viale Pieraccini 6, 50139, Florence, Italy.
| | - L Cianferotti
- Head, Bone Metablic Diseases Unit, Department of Surgery and Translational Medicine, University of Florence, Viale Pieraccini 6, 50139, Florence, Italy
| | - P Beck-Peccoz
- Department of Clinical Sciences and Community Health, University of Milan and Endocrine Unit, Fondazione IRCCS Ca' Granda, Milan, Italy
| | - M Capezzone
- Section of Endocrinology and Metabolism, Department of Internal Medicine, Endocrinology and Metabolism and Biochemistry, University of Siena, Policlinico Santa Maria alle Scotte, Siena, Italy
| | - F Cetani
- Unit of Endocrinology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy
| | - A Colao
- Dipartimento di Medicina Clinica e Chirurgia, Università Federico II di Napoli, Naples, Italy
| | - M V Davì
- Section D, Department of Medicine, Clinic of Internal Medicine, University of Verona, Verona, Italy
| | - E degli Uberti
- Section of Endocrinology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy
| | - S Del Prato
- Section of Metabolic Diseases and Diabetes, Department of Endocrinology and Metabolism, University of Pisa, Pisa, Italy
| | - R Elisei
- Unit of Endocrinology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy
| | - A Faggiano
- Dipartimento di Medicina Clinica e Chirurgia, Università Federico II di Napoli, Naples, Italy
| | - D Ferone
- Endocrinology, Department of Internal Medicine and Medical Specialties and Center of Excellence for Biomedical Research, IRCCS AOU San Martino-IST, University of Genoa, Genoa, Italy
| | - C Foresta
- Department of Medicine and Centre for Human Reproduction Pathology, University of Padova, Padua, Italy
| | - L Fugazzola
- Department of Clinical Sciences and Community Health, University of Milan and Endocrine Unit, Fondazione IRCCS Ca' Granda, Milan, Italy
| | - E Ghigo
- Division of Endocrinology, Diabetology and Metabolism Department of Medical Sciences, University Hospital Città Salute e Scienza, Turin, Italy
| | - G Giacchetti
- Division of Endocrinology, Azienda Ospedaliero-Universitaria, Ospedali Riuniti Umberto I-GM Lancisi-G Salesi, Università Politecnica delle Marche, Ancona, Italy
| | - F Giorgino
- Section of Internal Medicine, Endocrinology, Andrology and Metabolic Diseases, Department of Emergency and Organ Transplantation, University of Bari Aldo Moro, Bari, Italy
| | - A Lenzi
- Chair of Endocrinology, Section Medical Pathophysiology, Food Science and Endocrinology, Department Exp. Medicine, Sapienza University of Rome, Policlinico Umberto I, Rome, Italy
| | - P Malandrino
- Endocrinology, Department of Clinical and Molecular Biomedicine, Garibaldi-Nesima Medical Center, University of Catania, Catania, Italy
| | - M Mannelli
- Endocrinology Unit, Department of Experimental and Clinical Biomedical Sciences, University of Florence, Florence, Italy
| | - C Marcocci
- Department of Endocrinology and Metabolism, University of Pisa, Pisa, Italy
| | - L Masi
- Department of Orthopedic, Metabolic Bone Diseases Unit AOUC-Careggi Hospital, Largo Palagi, 1, Florence, Italy
| | - F Pacini
- Section of Endocrinology and Metabolism, University of Siena, Siena, Italy
| | - G Opocher
- Familial Cancer Clinic and Oncoendocrinology, Veneto Institute of Oncology, IRCCS, Padua, Italy
- Department of Medicine DIMED, University of Padova, Padova, Italy
| | - A Radicioni
- Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy
| | - M Tonacchera
- Unit of Endocrinology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy
| | - R Vigneri
- Department of Clinical and Molecular Biomedicine, University of Catania, and Humanitas Catania Center of Oncology, Catania, Italy
| | - M C Zatelli
- Section of Endocrinology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy
| | - M L Brandi
- Head, Bone Metablic Diseases Unit, Department of Surgery and Translational Medicine, University of Florence, Viale Pieraccini 6, 50139, Florence, Italy.
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