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Takada Y, Ishikawa T, Yamao K, Mizutani Y, Iida T, Uetsuki K, Nakamura M, Furukawa K, Yamamura T, Kawashima H. Endoscopic Papillectomy for Ampullary Gangliocytic Paraganglioma: A Case Series and Literature Review. Intern Med 2025; 64:1151-1159. [PMID: 39343575 DOI: 10.2169/internalmedicine.4102-24] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/01/2024] Open
Abstract
Objective Gangliocytic paraganglioma (GP) significantly affects patients' quality of life. However, studies on endoscopic papillectomy (EP) for ampullary GP are limited. We therefore evaluated the safety and efficacy of EP for treating ampullary GP. Methods We retrospectively reviewed the clinicopathological characteristics of patients with GP who underwent EP at Nagoya University Hospital and conducted a literature survey. Results We enrolled six patients with a median tumor diameter of 17 mm. Complications related to EP were observed in three patients: two experienced bleeding, one had mild acute pancreatitis, and one had perforation (duplicate patients included), all of whom improved conservatively. Five resected specimens were confined to the submucosal layer, and one was beyond the submucosal layer. All patients were monitored without surgery, and no disease recurrence was observed after a median follow-up of 73 months. A literature review identified 14 patients, and additional surgery due to a positive vertical margin after EP revealed lymph node metastasis in 2 patients. There was no disease recurrence or death. Conclusion EP led to good long-term outcomes and effectively treated ampullary GP. Considering the potential for lymph node metastasis, additional surgery is recommended if the tumor exceeds the submucosal layer.
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Affiliation(s)
- Yoshihisa Takada
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Japan
| | - Takuya Ishikawa
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Japan
| | - Kentaro Yamao
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Japan
| | - Yasuyuki Mizutani
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Japan
| | - Tadashi Iida
- Department of Endoscopy, Nagoya University Hospital, Japan
| | - Kota Uetsuki
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Japan
| | | | - Kazuhiro Furukawa
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Japan
| | - Takeshi Yamamura
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Japan
| | - Hiroki Kawashima
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Japan
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2
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Rami G, Ali MM, Meriem H, Sarra S, Alia Z, Mohamed BS. When thoracic trauma does more good than harm: About an incidental finding of a gangliocytic paraganglioma of the ampulla of Vater. Clin Case Rep 2024; 12:e9026. [PMID: 39605851 PMCID: PMC11599427 DOI: 10.1002/ccr3.9026] [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: 02/22/2024] [Revised: 03/31/2024] [Accepted: 04/05/2024] [Indexed: 11/29/2024] Open
Abstract
Gangliocytic paragangliomas are benign neuro-endocrine tumors of the ampulla of Vater. Their preoperative diagnosis is hampered by the low yield of biopsies and non-specific imaging. Their management relies then on resection. But the type of resection is controversial. Radical resection is indicated in case of lymph node involvement.
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Affiliation(s)
- Guizani Rami
- Department of General Surgery BLa Rabta HospitalTunisTunisia
| | | | - Hsairi Meriem
- Department of General Surgery BLa Rabta HospitalTunisTunisia
| | - Saad Sarra
- Department of General Surgery BLa Rabta HospitalTunisTunisia
| | - Zehani Alia
- Department of HistologyLa Rabta hospitalTunisTunisia
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Viñas T, Gomis Á, Ruiz de la Cuesta E, Gracia E, Jover R, Shalygina S, Ramia JM, Carbonell S. Jejunal gangliocytic paraganglioma. GASTROENTEROLOGIA Y HEPATOLOGIA 2024; 47:501-503. [PMID: 38072360 DOI: 10.1016/j.gastrohep.2023.12.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 07/26/2023] [Revised: 11/20/2023] [Accepted: 12/04/2023] [Indexed: 01/01/2024]
Affiliation(s)
- Tamara Viñas
- Servicio de Cirugía General, Hospital General Universitario Dr. Balmis, Alicante, España; ISABIAL, Instituto de Investigación Sanitaria y Biomédica, Alicante, España.
| | - Álvaro Gomis
- Servicio de Cirugía General, Hospital General Universitario Dr. Balmis, Alicante, España
| | - Emilio Ruiz de la Cuesta
- Servicio de Cirugía General, Hospital General Universitario Dr. Balmis, Alicante, España; ISABIAL, Instituto de Investigación Sanitaria y Biomédica, Alicante, España
| | - Ester Gracia
- Servicio de Cirugía General, Hospital General Universitario Dr. Balmis, Alicante, España; ISABIAL, Instituto de Investigación Sanitaria y Biomédica, Alicante, España
| | - Rodrigo Jover
- Servicio de Aparato Digestivo, Hospital General Universitario Dr. Balmis, Alicante, España; Universidad Miguel Hernández, Sant Joan d'Alacant, Alicante, España
| | - Svetlana Shalygina
- Servicio de Anatomía Patológica, Hospital General Universitario Dr. Balmis, Alicante, España
| | - José Manuel Ramia
- Servicio de Cirugía General, Hospital General Universitario Dr. Balmis, Alicante, España; ISABIAL, Instituto de Investigación Sanitaria y Biomédica, Alicante, España; Universidad Miguel Hernández, Sant Joan d'Alacant, Alicante, España
| | - Silvia Carbonell
- Servicio de Cirugía General, Hospital General Universitario Dr. Balmis, Alicante, España; ISABIAL, Instituto de Investigación Sanitaria y Biomédica, Alicante, España
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4
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Lin YS, Cao C, Cooper WA, Cheung VKY. Primary gangliocytic paraganglioma of the lung. Pathology 2024; 56:423-426. [PMID: 37863709 DOI: 10.1016/j.pathol.2023.07.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/28/2023] [Revised: 06/19/2023] [Accepted: 07/09/2023] [Indexed: 10/22/2023]
Affiliation(s)
- Yee Sing Lin
- Tissue Pathology and Diagnostic Oncology, NSW Health Pathology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia; School of Medical Science, University of New South Wales, Kensington, NSW, Australia.
| | - Christopher Cao
- Department of Cardiothoracic Surgery, Royal Prince Alfred Hospital, Camperdown, NSW, Australia; Sydney Medical School, University of Sydney, Sydney, NSW, Australia
| | - Wendy A Cooper
- Tissue Pathology and Diagnostic Oncology, NSW Health Pathology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia; Sydney Medical School, University of Sydney, Sydney, NSW, Australia; School of Medicine, Western Sydney University, Campbelltown, NSW, Australia
| | - Veronica Ka-Yan Cheung
- Tissue Pathology and Diagnostic Oncology, NSW Health Pathology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia
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Cai W, Hu W, Fang T. Endoscopic papillectomy combined with endoscopic retrograde cholangio-pancreatography for duodenal gangliocytic paraganglioma: A case report. Medicine (Baltimore) 2023; 102:e36662. [PMID: 38115359 PMCID: PMC10727581 DOI: 10.1097/md.0000000000036662] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/18/2023] [Accepted: 11/23/2023] [Indexed: 12/21/2023] Open
Abstract
RATIONALE Gangliocytic paraganglioma is a rare tumor that can occur in several organs throughout the body. Gangliocytic paraganglioma of the main duodenal papilla is even rarer. This study analyzes and discusses the endoscopic management of a case of gangliocytic paraganglioma of the main duodenal papilla and reviews the relevant literature. It is hoped that this study will increase clinicians' awareness of this disease. PATIENT CONCERNS Electron endoscopy reveals a duodenal main papillary tumor, and the patient desires further clarification of the nature of the tumor and the next step in the treatment plan. DIAGNOSES Duodenal gangliocytic paraganglioma. INTERVENTIONS As the patient lesion was located in the main duodenal papilla, we successfully performed endoscopic minimally invasive treatment of the tumor by endoscopic papillectomy combined with endoscopic retrograde cholangiopancreatography. OUTCOMES The patient was discharged after the postoperative removal of the nasobiliary drain and returned to the hospital 2 months later to have the biliary stent removed; the patient was in good general condition at follow-up. LESSONS For duodenal main papillary tumor, we need to be alert to the possibility of gangliocytic paraganglioma. Since the tumor is located in the submucosa of the juxta-abdominal region, the preoperative biopsy positivity rate is low, and the tumor is often adjacent to or involves the biliopancreatic duct, endoscopic resection combined with endoscopic retrograde cholangiopancreatography can be considered for diagnosis and treatment.
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Affiliation(s)
- Wenpin Cai
- Department of Gastroenterology, The Second Affiliated Hospital of Fujian Medical University, Quanzhou, Fujian, P.R. China
| | - Weitao Hu
- Department of Gastroenterology, The Second Affiliated Hospital of Fujian Medical University, Quanzhou, Fujian, P.R. China
| | - Taiyong Fang
- Department of Gastroenterology, The Second Affiliated Hospital of Fujian Medical University, Quanzhou, Fujian, P.R. China
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Liao X, Zhang D. Groove pancreatitis due to periampullary gangliocytic paraganglioma with lymph node metastasis. Pathology 2023; 55:429-432. [PMID: 36328810 DOI: 10.1016/j.pathol.2022.08.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/15/2022] [Revised: 07/26/2022] [Accepted: 08/01/2022] [Indexed: 03/08/2023]
Affiliation(s)
- Xiaoyan Liao
- Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, NY, USA
| | - Dongwei Zhang
- Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, NY, USA; Department of Pathology and Laboratory Medicine, Indiana University, Indianapolis, IN, USA.
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7
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Stan-Ilie M, Şandru V, Plotogea OM, Rînja E, Pavel C, Constantinescu G, Negreanu L, Paduraru DN, Bolocan A, Andronic O, Davitoiu D, Bălan GG, Constantinescu A. Duodenal Gangliocytic Paragangliomas-Case Series and Literature Review. Life (Basel) 2023; 13:597. [PMID: 36983753 PMCID: PMC10058500 DOI: 10.3390/life13030597] [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: 01/10/2023] [Revised: 02/12/2023] [Accepted: 02/16/2023] [Indexed: 02/23/2023] Open
Abstract
Duodenal gangliocytic paragangliomas are rare neuroendocrine tumors primarily localized in the periampullary area. Though mostly asymptomatic, they can present with various symptoms, most often jaundice, anemia and abdominal pain. The present paper is a case series report, describing our personal experience with patients presenting to the Emergency Unit with different symptoms due to duodenal gangliocytic paraganglioma. Endoscopic resection is safe and indicated in most of the cases, being also associated with lower medical costs. EUS plays a central role in the pre-resection management and in surveillance, and immunostaining is decisive to ascertain the tumor histologic origin. In addition to reporting our experience, we researched the literature regarding these rare tumors and performed a comprehensive review.
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Affiliation(s)
- Madalina Stan-Ilie
- Department of Gastroenterology, Clinical Emergency Hospital of Bucharest, 105402 Bucharest, Romania
- Department 5, “Carol Davila” University of Medicine and Pharmacy, 050474 Bucharest, Romania
| | - Vasile Şandru
- Department of Gastroenterology, Clinical Emergency Hospital of Bucharest, 105402 Bucharest, Romania
- Department 5, “Carol Davila” University of Medicine and Pharmacy, 050474 Bucharest, Romania
| | - Oana-Mihaela Plotogea
- Department of Gastroenterology, Clinical Emergency Hospital of Bucharest, 105402 Bucharest, Romania
- Department 5, “Carol Davila” University of Medicine and Pharmacy, 050474 Bucharest, Romania
| | - Ecaterina Rînja
- Department of Gastroenterology, Clinical Emergency Hospital of Bucharest, 105402 Bucharest, Romania
- Department 5, “Carol Davila” University of Medicine and Pharmacy, 050474 Bucharest, Romania
| | - Christopher Pavel
- Department of Gastroenterology, Clinical Emergency Hospital of Bucharest, 105402 Bucharest, Romania
| | - Gabriel Constantinescu
- Department of Gastroenterology, Clinical Emergency Hospital of Bucharest, 105402 Bucharest, Romania
- Department 5, “Carol Davila” University of Medicine and Pharmacy, 050474 Bucharest, Romania
| | - Lucian Negreanu
- Department 5, “Carol Davila” University of Medicine and Pharmacy, 050474 Bucharest, Romania
- Department of Gastroenterology, University Emergency Hospital Bucharest, 050098 Bucharest, Romania
| | - Dan Nicolae Paduraru
- Department 5, “Carol Davila” University of Medicine and Pharmacy, 050474 Bucharest, Romania
- Department of Surgery, University Emergency Hospital Bucharest, 050098 Bucharest, Romania
| | - Alexandra Bolocan
- Department 5, “Carol Davila” University of Medicine and Pharmacy, 050474 Bucharest, Romania
- Department of Surgery, University Emergency Hospital Bucharest, 050098 Bucharest, Romania
| | - Octavian Andronic
- Department 5, “Carol Davila” University of Medicine and Pharmacy, 050474 Bucharest, Romania
- Department of Surgery, University Emergency Hospital Bucharest, 050098 Bucharest, Romania
| | - Dragos Davitoiu
- Department 5, “Carol Davila” University of Medicine and Pharmacy, 050474 Bucharest, Romania
- Department of Surgery, University Emergency Hospital Bucharest, 050098 Bucharest, Romania
| | - Gheorghe G. Bălan
- Department of Gastroenterology, “Grigore T. Popa” University of Medicine and Pharmacy, 700115 Iași, Romania
| | - Alexandru Constantinescu
- Department 5, “Carol Davila” University of Medicine and Pharmacy, 050474 Bucharest, Romania
- Department of Gastroenterology, University Emergency Hospital Bucharest, 050098 Bucharest, Romania
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Jain V, Selvakumar B, Varshney VK, Vishwajeet V, Taywade S, Agarwal L, Yadav T, Pandey R. Gangliocytic Paraganglioma of the Duodenum: A Masquerader. Ochsner J 2023; 23:251-256. [PMID: 37711472 PMCID: PMC10498948 DOI: 10.31486/toj.23.0010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/16/2023] Open
Abstract
Background: Gangliocytic paraganglioma (GP) is a rare tumor that most commonly arises from the duodenum and is characterized pathologically by 3 cell types: epithelioid, spindle, and ganglion cells. GP is often difficult to differentiate from a neuroendocrine tumor on the basis of preoperative imaging, and the diagnosis is based on final histopathologic and immunohistochemical analysis. Case Report: We report the case of a 28-year-old male who presented with pain in the abdomen, bilious vomiting, and weight loss. Imaging showed a mass involving the first and second part of the duodenum that was likely a neuroendocrine or gastrointestinal stromal tumor. He underwent robotic-assisted pancreatoduodenectomy, and the final pathology report identified GP with lymph node metastasis. The patient was doing well at 1-year follow-up. Conclusion: GP is often a histologic surprise as most cases are diagnosed in postoperative histopathology. While GP has a more benign course than a neuroendocrine tumor, radical surgical resection is warranted in cases of diagnostic dilemma, suspicion of malignancy, or lymph node metastasis. Robotic-assisted pancreatoduodenectomy is a feasible option.
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Affiliation(s)
- Vishu Jain
- Department of Surgical Gastroenterology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
| | - B Selvakumar
- Department of Surgical Gastroenterology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
| | - Vaibhav Kumar Varshney
- Department of Surgical Gastroenterology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
| | - Vikarn Vishwajeet
- Department of Pathology and Laboratory Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
| | - Sameer Taywade
- Department of Nuclear Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
| | - Lokesh Agarwal
- Department of Surgical Gastroenterology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
| | - Taruna Yadav
- Department of Radiodiagnosis, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
| | - Rakesh Pandey
- Department of Nuclear Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
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9
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Lugo-Fagundo E, Weisberg EM, Fishman EK. Distinct cases of gangliocytic paraganglioma in the duodenum: Two case reports. Radiol Case Rep 2022; 17:2855-2858. [PMID: 35711738 PMCID: PMC9194760 DOI: 10.1016/j.radcr.2022.02.048] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/26/2022] [Revised: 02/14/2022] [Accepted: 02/14/2022] [Indexed: 11/19/2022] Open
Abstract
Gangliocytic paraganglioma (GP) is a rare, benign neuroendocrine tumor that commonly arises in the second portion of the duodenum. Despite its favorable prognosis, there have been instances of lymph node and liver metastasis as well as 1 reported fatal case. The immunohistochemical and morphological resemblance between GP and neuroendocrine tumor G1 makes it critical to properly recognize and differentiate between the 2. In this article, we present 2 distinct cases of GP: a 70-year-old male with a GP tumor in the ampulla, and a 46-year-old male with a GP near the ampulla whose tumor was excised using a robotic Whipple procedure. We focus on optimizing diagnosis and management through the application of radiological modalities and pathological analysis.
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Miyazaki T, Takemura N, Mihara F, Ito K, Miyazaki H, Igari T, Kokudo N. Surgical treatment for a rare case of duodenal gangliocytic paraganglioma accompanied with lymph node metastasis. Clin J Gastroenterol 2022; 15:712-716. [PMID: 35513688 DOI: 10.1007/s12328-022-01636-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/20/2021] [Accepted: 04/15/2022] [Indexed: 10/18/2022]
Abstract
We report a rare case of duodenal gangliocytic paraganglioma accompanied with lymph node metastasis. An asymptomatic 58-year-old woman was admitted for the evaluation of abnormal liver enzyme test results. Abdominal computed tomography and upper gastrointestinal endoscopy revealed a 27-mm pedunculated tumor of the papilla of Vater. Adenocarcinoma of the papilla with muscularis propria invasion was suspected; therefore, pancreaticoduodenectomy with lymph node dissection was performed. There were no perioperative complications. A definitive diagnosis of gangliocytic paraganglioma of the papilla of Vater with lymph node metastases was confirmed by pathological examinations, which is a rare condition. The patient was discharged on her 16th postoperative day, and no recurrence or metastases were identified during the 18-month follow-up period. We herein discuss the surgical treatment for this rare case and present a review of related literature.
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Affiliation(s)
- Toko Miyazaki
- Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Nobuyuki Takemura
- Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan.
| | - Fuminori Mihara
- Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Kyoji Ito
- Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Hideki Miyazaki
- Pathology Division of Clinical Laboratory, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Toru Igari
- Pathology Division of Clinical Laboratory, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Norihiro Kokudo
- Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
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Oriolo L, Vasta G, Plessi C, Marandola C, Grillo LR, Briganti V. Gangliocytic paraganglioma of Meckel diverticulum: An unusual leading point for ileocecal intussusception in an infant. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2022. [DOI: 10.1016/j.epsc.2022.102334] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
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12
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Choi H, Choi JW, Ryu DH, Park S, Kim MJ, Yoo KC, Woo CG. Ampullary gangliocytic paraganglioma with lymph node metastasis: A case report with literature review. Medicine (Baltimore) 2022; 101:e29138. [PMID: 35475800 PMCID: PMC9276348 DOI: 10.1097/md.0000000000029138] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/04/2022] [Accepted: 03/04/2022] [Indexed: 01/04/2023] Open
Abstract
RATIONALE Gangliocytic paraganglioma (GP) is a rare tumor that mostly develops in the duodenum and is composed of the following 3 cell types: epithelioid endocrine, spindle-like, and ganglion-like cells. It manifests as symptoms such as abdominal pain, gastrointestinal bleeding, and weight loss; however, occasionally, it is incidentally detected on endoscopic or radiologic examinations. Although GP is usually benign, it can metastasize to the lymph nodes, and distant metastases have been reported in some cases. PATIENT CONCERNS A 46-year-old woman presented with anemia on health surveillance examination. She had no other specific symptoms, and her physical examination did not reveal any abnormal finding. DIAGNOSIS Endoscopic ultrasound-guided fine-needle aspiration biopsy was performed, and the endoscopist obtained samples from the inner side of the ampullary mass. Pathological examination suggested GP or a neuroendocrine tumor. INTERVENTIONS Initially, we planned transduodenal ampullectomy with lymph node excision. However, there was severe fibrosis around the duodenum, and an examination of a frozen biopsy sample from the periduodenal lymph node showed atypical cells in the lymph node. Therefore, we performed pylorus-preserving pancreaticoduodenectomy with lymph node dissection. OUTCOMES The final pathological diagnosis was GP located in the ampulla of Vater. The GP showed lymphovascular and perineural invasion and invaded the duodenal wall. Furthermore, 4 out of 18 harvested lymph nodes showed metastasis. LESSONS We described a case of GP confined to the ampulla with regional lymph node metastasis and reviewed published literature on ampullary GP with lymph node metastasis.
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Affiliation(s)
- Hanlim Choi
- Department of Surgery, Chungbuk National University College of Medicine, Cheongju, Korea
- Department of Surgery, Chungbuk National University Hospital, Cheongju, Korea
| | - Jae-Woon Choi
- Department of Surgery, Chungbuk National University College of Medicine, Cheongju, Korea
- Department of Surgery, Chungbuk National University Hospital, Cheongju, Korea
| | - Dong Hee Ryu
- Department of Surgery, Chungbuk National University College of Medicine, Cheongju, Korea
- Department of Surgery, Chungbuk National University Hospital, Cheongju, Korea
| | - Sungmin Park
- Department of Surgery, Chungbuk National University College of Medicine, Cheongju, Korea
- Department of Surgery, Chungbuk National University Hospital, Cheongju, Korea
| | - Myung Jo Kim
- Department of Surgery, Chungbuk National University College of Medicine, Cheongju, Korea
- Department of Surgery, Chungbuk National University Hospital, Cheongju, Korea
| | - Kwon Cheol Yoo
- Department of Surgery, Chungbuk National University College of Medicine, Cheongju, Korea
- Department of Surgery, Chungbuk National University Hospital, Cheongju, Korea
| | - Chang Gok Woo
- Department of Pathology, Chungbuk National University Hospital, Cheongju, Korea
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13
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Laville D, Godfraind C, Dal Col P, Vassal F, Boutet C, Péoc’h M, Forest F. A 56‐year‐old woman with back pain and lower limbs weakness. Brain Pathol 2022; 32:e13045. [PMID: 35075739 PMCID: PMC9616081 DOI: 10.1111/bpa.13045] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/06/2021] [Revised: 12/03/2021] [Accepted: 12/09/2021] [Indexed: 11/26/2022] Open
Affiliation(s)
- David Laville
- Department of Pathology North Hospital University Hospital of Saint Etienne Saint Etienne Cedex 2 France
| | - Catherine Godfraind
- Department of Pathology Hospital Gabriel Montpied University Hospital of Clermond‐Ferrand Clermont‐Ferrand France
| | - Pierre Dal Col
- Department of Pathology North Hospital University Hospital of Saint Etienne Saint Etienne Cedex 2 France
| | - François Vassal
- Department of Neurosurgery North Hospital University Hospital of Saint Etienne Saint Etienne Cedex 2 France
| | - Claire Boutet
- Department of Radiology North Hospital University Hospital of Saint Etienne Saint Etienne Cedex 2 France
| | - Michel Péoc’h
- Department of Pathology North Hospital University Hospital of Saint Etienne Saint Etienne Cedex 2 France
- Corneal Graft Biology, Engineering and Imaging Laboratory BiiGC EA2521 Federative Institute of Research in Sciences and Health Engineering Faculty of Medicine Jean Monnet University Saint‐Etienne France
| | - Fabien Forest
- Department of Pathology North Hospital University Hospital of Saint Etienne Saint Etienne Cedex 2 France
- Corneal Graft Biology, Engineering and Imaging Laboratory BiiGC EA2521 Federative Institute of Research in Sciences and Health Engineering Faculty of Medicine Jean Monnet University Saint‐Etienne France
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14
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Li J, Wang LP, Zhu PS. Is Gangliocytic Paraganglioma Designated as a Subtype of Composite Paragangliomas and Originated From Pancreas Islet? A Case Report and Review of Literature. Front Endocrinol (Lausanne) 2022; 13:847632. [PMID: 35299959 PMCID: PMC8922442 DOI: 10.3389/fendo.2022.847632] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/03/2022] [Accepted: 01/24/2022] [Indexed: 12/02/2022] Open
Abstract
Gangliocytic paraganglioma (GP) is quite rare, and origin and entity remain to be elucidated. A 51-year-old man presented with GP as a sessile polyp with a smooth surface that measured about 1 cm in diameter in the descending portion of duodenum. Pathological examination displayed that a neoplasm was predominantly located in the submucosa and infiltrated mucosa focally. The tumor consisted of epithelioid, ganglion-like, and spindle cells admixing in a haphazard way. The epithelioid cells resembled paraganglioma in cytological and architectural features. The ganglion-like cells were scattered and merged with the bland spindle cells in fascicular clusters, which resembled ganglioneuroma. Synaptophysin (Syn), microtubule-associated protein-2 (MAP-2), and chromogranin A (CgA) were positive in the epithelioid and ganglion-like cells in variety, and neurofilament (NF) staining highlighted the ganglion-like cells. S-100 and SOX-10 were positive in the spindle cell proliferation and around the epithelioid cells. Progesterone receptor (PR) was positive in the epithelioid cells. The polyp was resected, and no adjuvant therapy was given. The patient remained with no recurrence in 2 years' follow-up. Origin of GP is presumed to be related to pancreas islet. GP is distinguished from neuroendocrine tumor (NET) G1 and designated as paraganglioma-ganglioneuroma, a kind of composite paragangliomas.
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Fontana F, Piacentino F, Ossola C, Pascarella RD, Franchi C, Curti M, Coppola A, Basile A, Saverio SD, Carcano G, Venturini M. Gangliocytic paraganglioma leading to duodeno-jejunal intussusception: A case report. Radiol Case Rep 2021; 16:3977-3981. [PMID: 34729128 PMCID: PMC8545659 DOI: 10.1016/j.radcr.2021.09.056] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/05/2021] [Revised: 09/19/2021] [Accepted: 09/20/2021] [Indexed: 02/07/2023] Open
Abstract
The intussusception of the small bowel is rarely encountered in adult patients and is frequently associated with a lead point that is often malignant. In a 69-year-old female patient with an episode of gastrointestinal (GI) bleeding, computed tomography (CT) showed a duodenal-jejunal intussusception caused by an intraluminal mass. Open polypectomy and reduction of intussusception were performed and the diagnosis of gangliocytic paraganglioma was made at pathological evaluation. It would be important to consider neoplasms like gangliocytic paraganglioma in the setting of adult small bowel intussusception.
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Affiliation(s)
- Federico Fontana
- Diagnostic and Interventional Radiology Department, Ospedale di Circolo, ASST dei Sette Laghi, Varese 21100, Italy
- School of Medicine and Surgery, Università degli Studi dell'Insubria, Varese 21100, Italy
| | - Filippo Piacentino
- Diagnostic and Interventional Radiology Department, Ospedale di Circolo, ASST dei Sette Laghi, Varese 21100, Italy
| | - Christian Ossola
- School of Medicine and Surgery, Università degli Studi dell'Insubria, Varese 21100, Italy
| | | | - Caterina Franchi
- School of Medicine and Surgery, Università degli Studi dell'Insubria, Varese 21100, Italy
| | - Marco Curti
- School of Medicine and Surgery, Università degli Studi dell'Insubria, Varese 21100, Italy
| | - Andrea Coppola
- Diagnostic and Interventional Radiology Department, Ospedale di Circolo, ASST dei Sette Laghi, Varese 21100, Italy
| | - Antonio Basile
- Department of Medical and Surgical Sciences and Advanced Technologies, Radiodiagnostic and Radiotherapy Unit, University Hospital "Policlinico-Vittorio Emanuele", Catania 95123, Italy
| | - Salomone Di Saverio
- School of Medicine and Surgery, Università degli Studi dell'Insubria, Varese 21100, Italy
- Department of General Surgery, ASUR Marche, AV5, Hospital of San Benedetto del Tronto, San Benedetto del Tronto 63074, Italy
| | - Giulio Carcano
- School of Medicine and Surgery, Università degli Studi dell'Insubria, Varese 21100, Italy
- Surgery Department, Ospedale di Circolo, ASST dei Sette Laghi, Varese 21100, Italy
| | - Massimo Venturini
- Diagnostic and Interventional Radiology Department, Ospedale di Circolo, ASST dei Sette Laghi, Varese 21100, Italy
- School of Medicine and Surgery, Università degli Studi dell'Insubria, Varese 21100, Italy
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Abstract
The latest WHO classification cleanly divides gastrointestinal neuroendocrine neoplasms into neuroendocrine tumor (NET; well-differentiated, any grade) and neuroendocrine carcinoma (NEC; poorly differentiated, high-grade by definition), along with mixed neuroendocrine-non-neuroendocrine neoplasms. NECs are always aggressive, with multiple mutations; they are treated with chemotherapy. NETs have widely different presentations, behavior, and management depending on site of origin. Esophageal examples are vanishingly rare. Most gastric and appendiceal tumors are indolent, as are many colonic and rectal tumors. The duodenum is home to some unusual variants of NET, and jejunal/ileal NETs frequently metastasize, which impacts their staging and clinical management.
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Affiliation(s)
- Raul S Gonzalez
- Department of Pathology, Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, MA 02215, USA; Harvard Medical School, Boston, MA, USA.
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18
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Gangliocytic Paraganglioma of Ampulla Masquerading as a Neuroendocrine Tumor. J Gastrointest Cancer 2020; 52:776-779. [PMID: 32895898 DOI: 10.1007/s12029-020-00507-8] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
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Ahmed M. Gastrointestinal neuroendocrine tumors in 2020. World J Gastrointest Oncol 2020; 12:791-807. [PMID: 32879660 PMCID: PMC7443843 DOI: 10.4251/wjgo.v12.i8.791] [Citation(s) in RCA: 130] [Impact Index Per Article: 26.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/31/2019] [Revised: 05/26/2020] [Accepted: 07/18/2020] [Indexed: 02/05/2023] Open
Abstract
Gastrointestinal neuroendocrine tumors are rare slow-growing tumors with distinct histological, biological, and clinical characteristics that have increased in incidence and prevalence within the last few decades. They contain chromogranin A, synaptophysin and neuron-specific enolase which are necessary for making a diagnosis of neuroendocrine tumor. Ki-67 index and mitotic index correlate with cellular proliferation. Serum chromogranin A is the most commonly used biomarker to assess the bulk of disease and monitor treatment and is raised in both functioning and non-functioning neuroendocrine tumors. Most of the gastrointestinal neuroendocrine tumors are non-functional. World Health Organization updated the classification of neuroendocrine tumors in 2017 and renamed mixed adenoneuroendocrine carcinoma into mixed neuroendocrine neoplasm. Gastric neuroendocrine tumors arise from enterochromaffin like cells. They are classified into 4 types. Only type I and type II are gastrin dependent. Small intestinal neuroendocrine tumor is the most common small bowel malignancy. More than two-third of them occur in the terminal ileum within 60 cm of ileocecal valve. Patients with small intestinal neuroendrocrine tumors frequently show clinical symptoms and develop distant metastases more often than those with neuroendocrine tumors of other organs. Duodenal and jejuno-ileal neuroendocrine tumors are distinct biologically and clinically. Carcinoid syndrome generally occurs when jejuno-ileal neuroendocrine tumors metastasize to the liver. Appendiceal neuroendocrine tumors are generally detected after appendectomy. Colonic neuroendocrine tumors generally present as a large tumor with local or distant metastasis at the time of diagnosis. Rectal neuroendocrine tumors are increasingly being diagnosed since the implementation of screening colonoscopy in 2000. Gastrointestinal neuroendocrine tumors are diagnosed and staged by endoscopy with biopsy, endoscopic ultrasound, serology of biomarkers, imaging studies and functional somatostatin scans. Various treatment options are available for curative and palliative treatment of gastrointestinal neuroendocrine tumors.
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Affiliation(s)
- Monjur Ahmed
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Thomas Jefferson University, Philadelphia, PA 19107, United States
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Taggart MW, Foo WC, Lee SM. Tumors of the Gastrointestinal System Including the Pancreas. ONCOLOGICAL SURGICAL PATHOLOGY 2020:691-870. [DOI: 10.1007/978-3-319-96681-6_12] [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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Katayama T, Iwasaki E, Minami K, Fukuhara S, Kanai T. Transient hypotension during endoscopic resection of gangliocytic paraganglioma with ampullary involvement. VideoGIE 2020; 5:26-28. [PMID: 31922078 PMCID: PMC6945226 DOI: 10.1016/j.vgie.2019.09.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/01/2022] Open
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Abstract
BACKGROUND Gangliocytic paraganglioma (GP) is rare and difficult to be differentiated from other periampullary neoplasms. The clinical characteristics and optimal treatment of periampullary GPs have not been clarified. METHODS The data pool for the analysis comprised of cases of periampullary GP encountered in our institution and sporadic cases reported in the English literature. RESULTS A total of 117 cases with periampullary GP were studied, including 2 from our institute, and among them, duodenal GP was the most common (53.0%). GP size ranged from 0.7 to 19.0 cm, with a median of 2.2 cm. The most common presenting symptom for overall periampullary GPs was epigastric pain in 49.5% cases, followed by gastrointestinal bleeding in 35.4% cases. Most (84.1%) of the periampullary GPs were benign, whereas 15.9% were malignant. Metastasis was noted in 26.3% of periampullary GPs, with 14.5% showing lymph node metastasis and 1.8% showing liver metastasis. Of the periampullary GP cases included, 30.1% were treated with pancreaticoduodenectomy, 40.6% with local excision, and 17.7% with endoscopic resection. CONCLUSIONS Periampullary GP should be considered as a tumor with malignant potential. Endoscopic resection is the treatment of choice for most of the duodenal GPs, whereas pancreaticoduodenectomy is recommended for GPs with possible malignancy, such as large size, with submucosal invasion, or pancreatic GP.
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Ebi M, Ochiai T, Sugiyama T, Yamamoto K, Adachi K, Yamaguchi Y, Tamura Y, Izawa S, Hijikata Y, Yamamoto S, Funaki Y, Ogasawara N, Sasaki M, Kasugai K. Endoscopic resection of gangliocytic paraganglioma of the duodenum: a case report. Clin J Gastroenterol 2019; 13:203-208. [PMID: 31535284 DOI: 10.1007/s12328-019-01043-0] [Citation(s) in RCA: 3] [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: 08/08/2019] [Accepted: 09/01/2019] [Indexed: 11/26/2022]
Abstract
Duodenal gangliocytic paragangliomas are extremely rare. A 79-year-old woman underwent gastrointestinal endoscopy for screening prior to resection of gallbladder carcinoma. Gastrointestinal endoscopy revealed a 5-mm submucosal tumor in the second portion of the duodenum. Contrast-enhanced computed tomography revealed no tumor or metastasis. Endoscopic ultrasonography revealed low echo pattern of the tumor. Histopathological examination of the biopsy specimen revealed proliferation of three types of cells (epithelioid cells, spindle cells, and ganglion cells). Immunohistochemical examination revealed that the tumor was positive for S-100 and synaptophysin. The preoperative diagnosis was gangliocytic paraganglioma. The tumor was completely resected by endoscopic mucosal resection (EMR). In conclusion, an early stage of gangliocytic paraganglioma of the duodenum could be resected using EMR.
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Affiliation(s)
- Masahide Ebi
- Departments of Gastroenterology, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, 480-1195, Japan.
| | - Tomoko Ochiai
- Departments of Gastroenterology, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, 480-1195, Japan
| | - Tomoya Sugiyama
- Departments of Gastroenterology, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, 480-1195, Japan
| | - Kazuhiro Yamamoto
- Departments of Gastroenterology, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, 480-1195, Japan
| | - Kazunori Adachi
- Departments of Gastroenterology, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, 480-1195, Japan
| | - Yoshiharu Yamaguchi
- Departments of Gastroenterology, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, 480-1195, Japan
| | - Yasuhiro Tamura
- Departments of Gastroenterology, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, 480-1195, Japan
| | - Shinya Izawa
- Departments of Gastroenterology, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, 480-1195, Japan
| | - Yasutaka Hijikata
- Departments of Gastroenterology, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, 480-1195, Japan
| | - Sayuri Yamamoto
- Departments of Gastroenterology, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, 480-1195, Japan
| | - Yasushi Funaki
- Departments of Gastroenterology, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, 480-1195, Japan
| | - Naotaka Ogasawara
- Departments of Gastroenterology, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, 480-1195, Japan
| | - Makoto Sasaki
- Departments of Gastroenterology, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, 480-1195, Japan
| | - Kunio Kasugai
- Departments of Gastroenterology, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, 480-1195, Japan
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Yang TW, Peng CM, Tsai MC. An Unusual Cause of Acute Pancreatitis in a Young Woman. Gastroenterology 2019; 157:619-621. [PMID: 30615872 DOI: 10.1053/j.gastro.2018.11.080] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/30/2018] [Revised: 11/24/2018] [Accepted: 11/28/2018] [Indexed: 12/02/2022]
Affiliation(s)
- Tzu-Wei Yang
- Division of Gastroenterology, Chung Shan Medical University Hospital, Taiwan; Institute of Medicine, School of Medicine of Chung Shan Medical University, Taiwan; School of Medicine of Chung Shan Medical University, Taiwan
| | - Cheng-Ming Peng
- Department of Surgery, Chung Shan Medical University Hospital, Taiwan; Institute of Medicine, School of Medicine of Chung Shan Medical University, Taiwan; School of Medicine of Chung Shan Medical University, Taiwan
| | - Ming-Chang Tsai
- Division of Gastroenterology, Chung Shan Medical University Hospital, Taiwan; Institute of Medicine, School of Medicine of Chung Shan Medical University, Taiwan; School of Medicine of Chung Shan Medical University, Taiwan
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25
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Yeo GE, Kwon HJ, Kim JH, Chang HK, Park JG. A Incidentally Diagnosed Duodenal Subepithelial Mass: Gangliocytic Paraganglioma Treated by Endoscopic Mucosal Resection. THE KOREAN JOURNAL OF HELICOBACTER AND UPPER GASTROINTESTINAL RESEARCH 2019. [DOI: 10.7704/kjhugr.2019.19.2.132] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
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Okubo Y. Gangliocytic Paraganglioma Is Often Misdiagnosed as Neuroendocrine Tumor G1. Arch Pathol Lab Med 2019; 141:1309. [PMID: 28968157 DOI: 10.5858/arpa.2017-0102-le] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Affiliation(s)
- Yoichiro Okubo
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa, Japan
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27
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Ko JK, Park DH, Hwang HS. A Case of Concurrent Ampullary Adenoma and Gangliocytic Paraganglioma at the Minor Papilla Treated with Endoscopic Resection. Clin Endosc 2019; 52:382-386. [PMID: 30974934 PMCID: PMC6680003 DOI: 10.5946/ce.2018.198] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/31/2018] [Accepted: 02/03/2019] [Indexed: 01/09/2023] Open
Abstract
A gangliocytic paraganglioma is a benign tumor of the digestive system with a very low incidence. The tumor is histopathologically characterized by a triphasic pattern consisting of epithelioid, ganglion, and spindle-shaped Schwann cells. In most cases, it occurs in the second portion of the duodenum near the ampulla of Vater. We report a case of a gangliocytic paraganglioma occurring at the minor duodenal papilla (a rare location) with a concurrent adenoma of the ampulla of Vater. Both lesions were treated simultaneously using endoscopic resection. Additionally, we have presented a literature review.
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Affiliation(s)
- Jun Kwon Ko
- Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Do Hyun Park
- Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Hee Sang Hwang
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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28
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Nagose VB, Jadhav VA. Gangliocytic Paraganglioma of Dorsolumbar Spine: A Rare Tumor at Rare Site. Asian J Neurosurg 2019; 14:907-910. [PMID: 31497126 PMCID: PMC6702988 DOI: 10.4103/ajns.ajns_80_19] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022] Open
Abstract
Spinal paragangliomas are rare benign tumors. The gangliocytic paragangliomas (GP) of spine are even rarer. The GPs are almost exclusively seen in duodenum. In spine, the usual site of affection is cauda equina region. The involvement of other spinal levels is far less often and includes thoracic and cervical region. We report this case of GP involving the conus medullaris region and reaching up to the upper part of cauda equina. The prognosis of spinal GP is excellent after total excision, as it is classified under WHO Grade I tumor. The patient made a full recovery after tumor removal. Clinical, radiological, and pathological characteristics of this rare tumor are discussed here along with.
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Affiliation(s)
- Vaishali Baburao Nagose
- Department of Pathology, Dr. Ulhas Patil Medical College and Hospital, Jalgaon, Maharashtra, India.,Department of Pathology, Mamata Medical College, Khammam, Telangana, India
| | - Varsha Ashok Jadhav
- Department of Pathology, Mamata Medical College, Khammam, Telangana, India.,Department of Pathology, Apollo Institute of Medical Sciences and Research, Chittoor, Andhra Pradesh, India
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Abstract
CONTEXT.— Duodenal epithelial polyps are reported in 1.5% to 3% of individuals referred for upper endoscopy. Most duodenal epithelial polyps are asymptomatic and nonneoplastic; however, a small subset is neoplastic and may progress to adenocarcinoma. Recent advances in immunohistochemical and molecular techniques have helped further characterize these polyps, shedding light on their origin, classification, and risk of progression to adenocarcinoma. OBJECTIVE.— To provide a comprehensive clinicopathologic review of nonneoplastic and neoplastic duodenal epithelial polyps, with particular emphasis on recent developments in classification schemes and risk stratification based upon immunohistochemical and molecular profiles. DATA SOURCES.— This review is based on peer-reviewed literature and the authors' experiences. CONCLUSIONS.— In this review we provide an update on the clinicopathologic, immunohistochemical, and molecular features of duodenal epithelial polyps and discuss the surveillance recommendations and treatment options available. Particular attention should be placed on recognizing duodenal adenomas with intestinal, gastric, and serrated phenotype, as they have an increased risk of malignant transformation if not completely excised.
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Affiliation(s)
- Katrina Collins
- From the Department of Pathology & Laboratory Medicine, Hartford Hospital, Hartford, Connecticut
| | - Saverio Ligato
- From the Department of Pathology & Laboratory Medicine, Hartford Hospital, Hartford, Connecticut
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Bakshi N, Rao S, Dhawan S, Singla V. Duodenal gangliocytic paraganglioma: A rare cause for gastrointestinal polyp. INDIAN J PATHOL MICR 2018; 61:580-582. [PMID: 30303155 DOI: 10.4103/ijpm.ijpm_479_17] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Duodenal gangliocytic paragangliomas are rare neoplasms often arising in the duodenum in close proximity to the ampulla of Vater. These neoplasms are considered to have a benign behavior with lymph node metastases being a rare phenomenon and distant metastases even more so. Although a standardized treatment has not been determined, a margin-free tumor resection seems to be the best treatment modality. We report herein the case of a 36-year-old female who presented with abdominal pain and was found to have a polyp in the second part of duodenum which was excised endoscopically. Histopathology and immunohistochemistry revealed characteristic features of this rare tumor.
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Affiliation(s)
- Neha Bakshi
- Department of Histopathology, Sir Ganga Ram Hospital, New Delhi, India
| | - Seema Rao
- Department of Histopathology, Sir Ganga Ram Hospital, New Delhi, India
| | - Shashi Dhawan
- Department of Histopathology, Sir Ganga Ram Hospital, New Delhi, India
| | - Vikas Singla
- Institute of Liver Gastroenterology and Pancreatico-Biliary Sciences, Sir Ganga Ram Hospital, Rajender Nagar, New Delhi, India
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Duodenal Gangliocytic Paraganglioma Requiring a Pancreaticoduodenectomy: A Case Report and Review of the Literature. Case Rep Surg 2018; 2018:6292789. [PMID: 30356398 PMCID: PMC6178180 DOI: 10.1155/2018/6292789] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/15/2018] [Accepted: 08/02/2018] [Indexed: 02/06/2023] Open
Abstract
Introduction Duodenal gangliocytic paragangliomas (GPs) are a subclass of duodenal neuroendocrine neoplasms and are exceedingly rare. They have been associated with an indolent behavior; however, they can rarely metastasize. Radical resection like a pancreaticoduodenectomy is sometimes indicated. We hereby present a case requiring major surgery and perform a literature search about this disease. Presentation of Case A 49-year-old Caucasian female, who presented with an upper gastrointestinal bleed, was found to have a hypodense mass in the second/third portion of the duodenum. A biopsy of the mass during upper endoscopy was inconclusive. A pancreaticoduodenectomy was recommended based on the high suspicion for a duodenal adenocarcinoma and was performed successfully. Her final pathology revealed a duodenal gangliocytic paraganglioma. Discussion The majority of duodenal GPs present as gastrointestinal bleeds while others less commonly present with anemia, abdominal pain, duodenal obstructive symptoms, pancreatitis, or abnormal incidental findings on axial abdominal imaging. Duodenal GPs were initially viewed as benign tumors of the duodenum; however, there have been increasing incidence reports of hematogenous and lymphatic metastasis. Appropriate treatment of duodenal GPs is still controversial and ranges from local endoscopic submucosal resection to major radical surgery. Conclusion Duodenal GPs are very rare tumors of the second portion of the duodenum presenting with upper gastrointestinal bleeding and local symptoms of surrounding organs. Local or radical resection is usually recommended to prevent bleeding and the minor risk of metastatic spread.
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Sekine M, Miyatani H, Matsumoto K, Kashima H, Koito Y, Miura T, Takahashi Y, Tsuboi R, Ishii T, Fujiwara J, Uehara T, Urayoshi S, Yuhashi K, Asano T, Sagihara N, Matsumoto S, Mashima H. Gangliocytic Paraganglioma with Carcinoma of the Ampulla of Vater. Intern Med 2018; 57:2663-2668. [PMID: 29780117 PMCID: PMC6191592 DOI: 10.2169/internalmedicine.0464-17] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/15/2017] [Accepted: 01/10/2018] [Indexed: 11/06/2022] Open
Abstract
The patient was a "73" -year-old woman who visited our hospital with the chief complaint of weight loss. Upper gastrointestinal endoscopy revealed an enlarged ampulla of Vater, and a biopsy led to a diagnosis of Group "4" gastric carcinoma; suspicious of adenocarcinoma. There were no findings suggesting invasion into the muscle layer of duodenum, despite tumor mass formation being observed in the sphincter of Oddi. We performed endoscopic papillectomy for both diagnostic and therapeutic purposes. Pathologically, a well-differentiated adenocarcinoma existed in the superficial layer of the mucous membrane of the papilla of Vater, and gangliocytic paraganglioma was present in the deep portion. The resected margins of both lesions were negative.
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Affiliation(s)
- Masanari Sekine
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Hiroyuki Miyatani
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Keita Matsumoto
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Hitomi Kashima
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Yudai Koito
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Takaya Miura
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Yuko Takahashi
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Rumiko Tsuboi
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Takehiro Ishii
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Junichi Fujiwara
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Takeshi Uehara
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Shunsuke Urayoshi
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Kazuhito Yuhashi
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Takeharu Asano
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Noriyoshi Sagihara
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Satohiro Matsumoto
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Hirosato Mashima
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
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Colle E, Zouhry I, Colignon N, Mourra N. Paraampullary gangliocytic paraganglioma. Clin Res Hepatol Gastroenterol 2018; 42:291-293. [PMID: 28943235 DOI: 10.1016/j.clinre.2017.08.002] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/03/2017] [Revised: 08/09/2017] [Accepted: 08/11/2017] [Indexed: 02/04/2023]
Affiliation(s)
- Elise Colle
- Pathology department, hôpital Saint-Antoine, AP-HP, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France
| | - Ilyass Zouhry
- Pathology department, hôpital Saint-Antoine, AP-HP, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France
| | - Nikias Colignon
- Radiology department, hôpital Saint-Antoine, AP-HP, Paris, France
| | - Najat Mourra
- Pathology department, hôpital Saint-Antoine, AP-HP, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France.
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Okubo Y, Yoshioka E, Suzuki M, Washimi K, Kawachi K, Kameda Y, Yokose T. Diagnosis, Pathological Findings, and Clinical Management of Gangliocytic Paraganglioma: A Systematic Review. Front Oncol 2018; 8:291. [PMID: 30101131 PMCID: PMC6072869 DOI: 10.3389/fonc.2018.00291] [Citation(s) in RCA: 25] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/24/2018] [Accepted: 07/11/2018] [Indexed: 12/11/2022] Open
Abstract
Background: Although gangliocytic paraganglioma (GP) is considered a rare benign neuroendocrine tumor, cases of mortality have been reported. Occasionally, GP is misdiagnosed as neuroendocrine tumor G1, which is associated with a poorer prognosis than GP. To avoid such misdiagnoses, it is important to understand the clinicopathological characteristics of GP. Thus, herein, we discuss the current literature on the clinicopathological characteristics of GP. Methods: We conducted a systematic review in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. PubMed and Japana Centra Revuo Medicina searches were used to identify papers describing GP. Inclusion criteria included confirmation of epithelioid, spindle-shaped, and ganglion-like cells in the main article and/or figures and whether the paper was cited in other studies of GP. Data were collected on age, sex, site of the primary lesion, tumor size, treatment, prognosis, lymph node metastasis (LNM), depth of tumor invasion, rate of preoperative diagnosis, and clinical symptoms. Results: In total, 162 papers containing 263 cases of GP met the criteria. The mean age at diagnosis was 53.5 years. The male-to-female ratio was 157:104. The mean tumor size was 25.7 mm. The predominant site of the primary tumor was the duodenum (89.7%). The most common clinical sign of GP was gastrointestinal bleeding (47.9%). Other signs and symptoms of GP included abdominal pain (44.7%), anemia (20.3%), incidental findings (12.9%), nausea (6.9%), weight loss (5.5%), general fatigue (5.1%), jaundice (4.6%), and incidental autopsy findings (5.1%). LNM was observed in 11.4% of patients. Liver metastasis was observed in 1.1% of patients. Depth of tumor invasion (penetrating beyond the submucosal layer or sphincter of Oddi) was by far the most significant risk factor for LNM in patients with GP. This suggests, along with histological heterogeneity, that GP may have hamartomatous characteristics. Furthermore, immunohistochemical expression of progesterone receptor and pancreatic polypeptide were useful in distinguishing between GP and neuroendocrine tumor G1, even in small biopsy specimens. Conclusions: We reveal the clinicopathological characteristics of GP, including risk factors for LNM, differential diagnostic approaches, and improvements in the clinical management of this tumor.In addition, GP may have hamartomatous characteristics.
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Affiliation(s)
- Yoichiro Okubo
- Department of Pathology, Kanagawa Cancer Center, Kanagawa, Japan
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Carlini M, Appetecchia M. Neuroendocrine Tumors: a Nosologic Framework. Updates Surg 2018. [DOI: 10.1007/978-88-470-3955-1_1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]
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Chai SM, Brown IS, Kumarasinghe MP. Gastroenteropancreatic neuroendocrine neoplasms: selected pathology review and molecular updates. Histopathology 2017; 72:153-167. [DOI: 10.1111/his.13367] [Citation(s) in RCA: 29] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Affiliation(s)
- Siaw M Chai
- PathWest Laboratory Medicine; Queen Elizabeth II Medical Centre; Perth Australia
| | - Ian S Brown
- Envoi Pathology; Kelvin Grove; Queensland Australia
| | - M Priyanthi Kumarasinghe
- PathWest Laboratory Medicine; Queen Elizabeth II Medical Centre; Perth Australia
- School of Pathology and Laboratory Medicine; University of Western Australia; Perth Australia
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Okubo Y, Kasajima R, Suzuki M, Miyagi Y, Motohashi O, Shiozawa M, Yoshioka E, Washimi K, Kawachi K, Kameda Y, Yokose T. Risk factors associated with the progression and metastases of hindgut neuroendocrine tumors: a retrospective study. BMC Cancer 2017; 17:769. [PMID: 29145818 PMCID: PMC5693490 DOI: 10.1186/s12885-017-3769-4] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/06/2017] [Accepted: 11/09/2017] [Indexed: 12/14/2022] Open
Abstract
BACKGROUND The worldwide incidence of neuroendocrine tumors (NETs) has increased remarkably, with the hindgut being the second most common site for such tumors. However, the mechanisms underlying progression and metastasis of hindgut NETs are unclear. A retrospective study was conducted to elucidate these mechanisms. METHODS Clinicopathological data of cases of hindgut NET between April 1996 and September 2015 were analyzed, retrospectively. Patients with neuroendocrine carcinoma were excluded. Formalin-fixed paraffin-embedded tissues of hindgut NET cases were subjected to detailed morphometric and immunohistochemical analyses. Statistical analyses were performed using the non-parametric Mann-Whitney U test, Spearman's correlation coefficient, and chi-squared test. Multivariate logistic regression analysis was conducted as appropriate for the data set. RESULTS Fifty-six hindgut NET cases were considered. Microvessel density and lymphatic microvessel density were identified as significant risk factors for venous and lymphatic invasion. There was a positive correlation between microvessel density and the maximum tumor diameter. Multivariate logistic regression analysis revealed that the maximum tumor diameter alone was an independent predictor of lymph node metastasis, whereas lymphovascular invasion and MVD was not the predictor of lymph node metastasis. There were no significant correlations between the Ki-67 labeling index and any of the parameters evaluated including age, sex, the maximum tumor diameter, venous invasion, lymphatic invasion, microvessel density, lymphatic microvessel density, and lymph node metastasis. CONCLUSIONS Angiogenic mechanisms may play important roles in the progression of hindgut NET. Otherwise, the maximum tumor diameter alone was an independent predictor of lymph node metastasis in hindgut NETs. Moreover, our study raises the question of whether the presence of lymphovascular invasion, in endoscopically obtained hindgut NET tissues, is an absolute indication for additional surgery or not.
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Affiliation(s)
- Yoichiro Okubo
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Rika Kasajima
- Molecular Pathology and Genetics Division, Kanagawa Cancer Center Research Institute, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Masaki Suzuki
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Yohei Miyagi
- Molecular Pathology and Genetics Division, Kanagawa Cancer Center Research Institute, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Osamu Motohashi
- Department of Gastroenterology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Manabu Shiozawa
- Department of Gastrointestinal Surgery, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Emi Yoshioka
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Kota Washimi
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Kae Kawachi
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Yoichi Kameda
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Tomoyuki Yokose
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
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Pancreatic gangliocytic paraganglioma harboring lymph node metastasis: a case report and literature review. Diagn Pathol 2017; 12:57. [PMID: 28764742 PMCID: PMC5540335 DOI: 10.1186/s13000-017-0648-x] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/26/2017] [Accepted: 07/20/2017] [Indexed: 12/20/2022] Open
Abstract
Background Gangliocytic paraganglioma (GP) is a rare neuroendocrine neoplasm, which occurs mostly in the periampullary portion of the duodenum; the majority of the reported cases of duodenal GP has been of benign nature with a low incidence of regional lymph node metastasis. GP arising from the pancreas is extremely rare. To date, only three cases have been reported and its clinical characteristics are largely unknown. Case presentation A nodule located in the pancreatic head was incidentally detected in an asymptomatic 68-year-old woman. Computed tomography revealed 18-, 8-, and 12-mm masses in the pancreatic head, the pancreatic tail, and the left adrenal gland, respectively. Subsequent genetic examination revealed an absence of mutations in the MEN1 and VHL genes. Macroscopically, the tumor located in the pancreatic head was 22 mm in size and displayed an ill-circumscribed margin along with yellowish-white color. Microscopically, it was composed of three cell components: epithelioid cells, ganglion-like cells, and spindle cells, which led to the diagnosis of GP. The tumor was accompanied by a peripancreatic lymph node metastasis. The tumor in the pancreatic tail was histologically classified as a neuroendocrine tumor (NET) G1 (grade 1, WHO 2010), whereas the tumor in the left adrenal gland was identified as an adrenocortical adenoma. The patient was disease-free at the 12-month follow-up examination. Conclusions Pancreatic GP is associated with a higher incidence of metastasis and larger tumor size than duodenal GPs, suggesting that the primary organ of GP is an important prognostic factor.
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Sánchez Torrijos Y, León Montañés R, Mejías Manzano MÁ. Gangliocytic paraganglioma: an unusual cause of upper gastrointestinal bleeding. REVISTA ESPANOLA DE ENFERMEDADES DIGESTIVAS 2017; 109:605-606. [PMID: 28715900 DOI: 10.17235/reed.2017.4808/2016] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
Gangliocytic paraganglioma is an uncommon tumor, which is usually located in the ampulla of Vater, and may get confused in the differential diagnosis with ampuloma or GIST. Here we present a case of a patient with upper gastrointestinal bleeding as a predominant symptom, objectified by endoscopy an ulcerative polypoid mass at the juxtapilar level, with histological result of gangliocítica paraganglioma after multiple biopsies, and finally surgical resection. The interest of this case is the difficulty for diagnosing, the different treatments and prognosis that this implies, especially for the tumor location, as surgery can lead to great complications.
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40
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Cathcart SJ, Sasson AR, Kozel JA, Oliveto JM, Ly QP. Duodenal gangliocytic paraganglioma with lymph node metastases: A case report and comparative review of 31 cases. World J Clin Cases 2017; 5:222-233. [PMID: 28685135 PMCID: PMC5480070 DOI: 10.12998/wjcc.v5.i6.222] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/05/2016] [Revised: 02/14/2017] [Accepted: 04/24/2017] [Indexed: 02/05/2023] Open
Abstract
Gangliocytic paraganglioma (GP) is a rare tumor of uncertain origin most often located in the second portion of the duodenum. It is composed of three cellular components: Epithelioid endocrine cells, spindle-like/sustentacular cells, and ganglion-like cells. While this tumor most often behaves in a benign manner, cases with metastasis are reported. We describe the case of a 62-year-old male with a periampullary GP with metastases to two regional lymph nodes who was successfully treated with pancreaticoduodenectomy. Using PubMed, EMBASE, EBSCOhost MEDLINE and CINAHL, and Google Scholar, we searched the literature for cases of GP with regional lymph node metastasis and evaluated the varying presentations, diagnostic workup, and disease management of identified cases. Thirty-one cases of GP with metastasis were compiled (30 with at least lymph node metastases and one with only distant metastasis to bone), with age at diagnosis ranging from 16 to 74 years. Ratio of males to females was 19:12. The most common presenting symptoms were abdominal pain (55%) and gastrointestinal bleeding or sequelae (42%). Twenty-five patients underwent pancreaticoduodenectomy. Five patients were treated with local resection alone. One patient died secondary to metastatic disease, and one died secondary to perioperative decompensation. The remainder did well, with no evidence of disease at follow-up from the most recent procedure (except two in which residual disease was deliberately left behind). Of the 26 cases with sufficient histological description, 16 described a primary tumor that infiltrated deep to the submucosa, and 3 described lymphovascular invasion. Of the specific immunohistochemistry staining patterns studied, synaptophysin (SYN) stained all epithelioid endocrine cells (18/18). Neuron specific enolase (NSE) and SYN stained most ganglion-like cells (7/8 and 13/18 respectively), and S-100 stained all spindle-like/sustentacular cells (21/21). Our literature review of published cases of GP with lymph node metastasis underscores the excellent prognosis of GP regardless of specific treatment modality. We question the necessity of aggressive surgical intervention in select patients, and argue that local resection of the mass and metastasis may be adequate. We also emphasize the importance of pre-surgical assessment with imaging studies, as well as post-surgical follow-up surveillance for disease recurrence.
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Affiliation(s)
- Yoichiro Okubo
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa, 241-8515, Japan.
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Papaconstantinou D, Machairas N, Damaskou V, Zavras N, Kontopoulou C, Machairas A. Duodenal gangliocytic paraganglioma, successfully treated by local surgical excision-a case report. Int J Surg Case Rep 2017; 32:5-8. [PMID: 28213067 PMCID: PMC5312508 DOI: 10.1016/j.ijscr.2017.01.046] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/08/2016] [Revised: 01/09/2017] [Accepted: 01/17/2017] [Indexed: 12/14/2022] Open
Abstract
Duodenal gangliocytic paraganglioma is a rare tumor usually with benign behavior. Resection of the tumor, either endoscopically or surgically is the treatment of choice. Complete removal of the mass offers cure and long-term survival. Introduction Duodenal gangliocytic paragangliomas are rare neoplasms often arising in proximity to the major duodenal papilla of Vater. These neoplasms are considered to have a benign behavior with lymph node metastases being a rare phenomenon and distant metastatic disease even more so. Resection of the tumor is the only definitive therapy. Presentation of case A 67 year old male presented to a referring hospital with symptoms of fatigue and malaise. Evaluation with CT imaging revealed a 3.1 cm intraluminal mass situated grossly at the junction of the third with the fourth portion of the duodenum. The tumor was found to be situated near the ampulla of Vater and was excised through a longitudinal duodenotomy followed by myotomy of the sphincter of Oddi. Discussion Complete resection of duodenal gangliocytic paragangliomas by surgical or endoscopic means is the only potential cure. Endoscopic removal is the first option and is both safe and adequate. Ηowever, localized excision may be utilized instead in those cases in which endoscopic removal is not possible or cannot achieve negative margins. Recurrent disease after complete resection is unlikely. Conclusion Cases of duodenal gangliocytic paragangliomas are best managed with endoscopic resection. However, local surgical excision remains as a second-choice procedure. Adjuvant chemotherapy and radiotherapy are unnecessary after complete excision.
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Affiliation(s)
- Dimetrios Papaconstantinou
- 3rd Department of Surgery, University General Hospital "ATTIKON", Medical School, National and Kapodistrian University of Athens, Greece
| | - Nikolaos Machairas
- 2nd Department of Surgery, General Hospital "Laiko", Medical School, National and Kapodistrian University of Athens, Greece
| | - Vasileia Damaskou
- 2nd Department of Pathology, University General Hospital "ATTIKON", Medical School, National and Kapodistrian University of Athens, Greece
| | - Nikolaos Zavras
- Pediatric Surgery Department, University General Hospital "ATTIKON", Medical School, National and Kapodistrian University of Athens, Greece.
| | - Christine Kontopoulou
- Second Department of Radiology, University General Hospital "ATTIKON", Medical School, National and Kapodistrian University of Athens, Greece
| | - Anastasios Machairas
- 3rd Department of Surgery, University General Hospital "ATTIKON", Medical School, National and Kapodistrian University of Athens, Greece
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Sherazadishvili A, Leslie K. Duodenal gangliocytic paraganglioma with lymph node metastasis: A systematic case review. INTERNATIONAL JOURNAL OF HEPATOBILIARY AND PANCREATIC DISEASES 2017. [DOI: 10.5348/ijhpd-2017-74-ra-10] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/12/2022]
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Matsubayashi H, Ishiwatari H, Matsui T, Fujie S, Uesaka K, Sugiura T, Okamura Y, Yamamoto Y, Ashida R, Ito T, Sasaki K, Ono H. Gangliocytic Paraganglioma of the Minor Papilla of the Duodenum. Intern Med 2017; 56:1029-1035. [PMID: 28458307 PMCID: PMC5478562 DOI: 10.2169/internalmedicine.56.7812] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/14/2022] Open
Abstract
A duodenal polyp was found during a health check of a 71-year-old asymptomatic man. Duodenoscopy demonstrated a pedunculated, smooth-surfaced tumor of 18 mm in size, protruding from the minor papilla. Endoscopic ultrasonography demonstrated a homogeneously low-echoic submucosal tumor. Enhanced computed tomography and magnetic resonance imaging demonstrated a well-enhanced duodenal tumor without obvious metastasis. A tumor biopsy revealed a well-differentiated neuroendocrine tumor, and laparotomic transduodenal polypectomy with regional lymph node dissection was performed. The histology of the surgical specimen revealed gangliocytic paraganglioma consisting of three cell types: endocrine, ganglion, and spindle cells. There has been no recurrence in >5 years after surgery.
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Affiliation(s)
| | | | - Toru Matsui
- Division of Endoscopy, Shizuoka Cancer Center, Japan
| | - Shinya Fujie
- Division of Endoscopy, Shizuoka Cancer Center, Japan
| | - Katsuhiko Uesaka
- Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center, Japan
| | - Teiichi Sugiura
- Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center, Japan
| | - Yukiyasu Okamura
- Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center, Japan
| | - Yusuke Yamamoto
- Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center, Japan
| | - Ryo Ashida
- Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center, Japan
| | - Takaaki Ito
- Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center, Japan
| | - Keiko Sasaki
- Division of Pathology, Shizuoka Cancer Center, Japan
| | - Hiroyuki Ono
- Division of Endoscopy, Shizuoka Cancer Center, Japan
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Okubo Y, Okubo T, Okubo Y, Ishiwatari T. Neuroendocrine Differentiation in Breast Cancer: Clinicopathological Significance of Bcl-2 Positive Solid Papillary Carcinoma. Case Rep Med 2016; 2016:9501410. [PMID: 28105053 PMCID: PMC5220427 DOI: 10.1155/2016/9501410] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2016] [Revised: 11/22/2016] [Accepted: 11/24/2016] [Indexed: 12/14/2022] Open
Abstract
Solid papillary carcinoma (SPC) is considered a rare malignant breast tumor. Maluf and Koerner first reported this disease entity as a special type of ductal carcinoma in situ with several characteristic histopathological features, including low-grade cellular atypia, intracellular or extracellular mucin deposition, and solid papillary growth pattern, as well as neuroendocrine differentiation. The present paper describes a case of SPC with bcl-2 expression, which is known as a marker for malignancy of neuroendocrine tumors. Interestingly, despite bcl-2 expression being a poor prognostic indicator of neuroendocrine tumors, the patient with this tumor has achieved long-term survival (approximately 6 years) at the time of writing this report. Because previous investigators reported that bcl-2 expression might play a role in the inhibition of the development of breast cancer, we suggest that bcl-2 expression might reflect a good prognosis in patients with SPC, rather than being a poor prognostic indicator, as it is in several types of neuroendocrine tumor. However, to confirm this hypothesis, further investigation is required.
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Affiliation(s)
- Yoichiro Okubo
- Department of Pathology, Kanagawa Cancer Center, 2-3-2 Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515, Japan
| | - Takuji Okubo
- Department of Internal Medicine, Okubo Internal Medicine/Surgery Clinic, 1282 Ohaza, Oita, Oita Prefecture 870-1151, Japan
| | - Yoshimi Okubo
- Department of Surgery, Okubo Internal Medicine/Surgery Clinic, 1282 Ohaza, Oita, Oita Prefecture 870-1151, Japan
| | - Takao Ishiwatari
- Department of Pathology, Chiba Cytopathological Center, 5-27-8 Nishifuna, Funabashi, Chiba 273-0031, Japan
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Okubo Y, Yokose T, Motohashi O, Miyagi Y, Yoshioka E, Suzuki M, Washimi K, Kawachi K, Nito M, Nemoto T, Shibuya K, Kameda Y. Duodenal Rare Neuroendocrine Tumor: Clinicopathological Characteristics of Patients with Gangliocytic Paraganglioma. Gastroenterol Res Pract 2016; 2016:5257312. [PMID: 28096810 PMCID: PMC5209618 DOI: 10.1155/2016/5257312] [Citation(s) in RCA: 28] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/13/2016] [Accepted: 11/23/2016] [Indexed: 12/14/2022] Open
Abstract
Gangliocytic paraganglioma (GP) has been regarded as a rare benign tumor that commonly arises from the second part of the duodenum. As GP does not exhibit either prominent mitotic activity or Ki-67 immunoreactivity, it is often misdiagnosed as neuroendocrine tumor (NET) G1. However, the prognosis might be better in patients with GP than in those with NET G1. Therefore, it is important to differentiate GP from NET G1. Moreover, our previous study indicated that GP accounts for a substantial, constant percentage of duodenal NETs. In the present article, we describe up-to-date data on the clinicopathological characteristics of GP and on the immunohistochemical findings that can help differentiate GP from NET G1, as largely revealed in our new and larger literature survey and recent multi-institutional retrospective study. Furthermore, we would like to refer to differential diagnosis and clinical management of this tumor and provide intriguing information about the risk factors for lymph node metastasis on GP.
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Affiliation(s)
- Yoichiro Okubo
- Department of Pathology, Kanagawa Cancer Center, 2-3-2 Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515, Japan
| | - Tomoyuki Yokose
- Department of Pathology, Kanagawa Cancer Center, 2-3-2 Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515, Japan
| | - Osamu Motohashi
- Department of Gastroenterology, Kanagawa Cancer Center, 2-3-2 Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515, Japan
| | - Yohei Miyagi
- Molecular Pathology and Genetics Division, Kanagawa Cancer Center Research Institute, 2-3-2 Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515, Japan
| | - Emi Yoshioka
- Department of Pathology, Kanagawa Cancer Center, 2-3-2 Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515, Japan
| | - Masaki Suzuki
- Department of Pathology, Kanagawa Cancer Center, 2-3-2 Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515, Japan
| | - Kota Washimi
- Department of Pathology, Kanagawa Cancer Center, 2-3-2 Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515, Japan
| | - Kae Kawachi
- Department of Pathology, Kanagawa Cancer Center, 2-3-2 Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515, Japan
| | - Madoka Nito
- Department of Thoracic Surgery, Kanagawa Cancer Center, 2-3-2 Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515, Japan
| | - Tetsuo Nemoto
- Department of Surgical Pathology, Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-Ku, Tokyo 143-8541, Japan
| | - Kazutoshi Shibuya
- Department of Surgical Pathology, Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-Ku, Tokyo 143-8541, Japan
| | - Yoichi Kameda
- Department of Pathology, Kanagawa Cancer Center, 2-3-2 Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515, Japan
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Okubo Y, Motohashi O, Nakayama N, Nishimura K, Kasajima R, Miyagi Y, Shiozawa M, Yoshioka E, Suzuki M, Washimi K, Kawachi K, Nito M, Kameda Y, Yokose T. The clinicopathological significance of angiogenesis in hindgut neuroendocrine tumors obtained via an endoscopic procedure. Diagn Pathol 2016; 11:128. [PMID: 27821179 PMCID: PMC5100185 DOI: 10.1186/s13000-016-0580-5] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/06/2016] [Accepted: 11/03/2016] [Indexed: 12/20/2022] Open
Abstract
BACKGROUND As the World Health Organization grading system for gastroenteropancreatic-neuroendocrine tumors (GEP-NETs) may not always correlate with tumor progression, it is imperative that other independent predictors of tumor progression be established. To identify such predictors, we conducted a retrospective histopathological study of hindgut NETs, obtained from endoscopic procedures, and used statistical analyses to evaluate predictive factors. METHODS We first obtained clinicopathological data of cases of hindgut NETs. Tissue sections from tumor samples were prepared and subjected to pathological examination. In particular, we calculated the microvessel density (MVD) and lymphatic microvessel density (LMVD) values, and performed appropriate statistical analyses. RESULTS A total of 42 cases of hindgut NETs were selected for the study, 41 from the rectum and 1 from the sigmoid colon. Based on the Ki-67 labeling index, 34 cases were classified as NET G1 tumors and 8 as NET G2 tumors. MVD values ranged from 1.4/mm2 to 73.9/mm2 and LMVD values from 0/mm2 to 22.9/mm2. MVD and LMVD were identified as risk factors for venous and lymphatic invasion of hindgut NETs. Moreover, MVD positively correlated with the maximum diameter of the tumor. CONCLUSIONS Tumor progression of NETs may cause angiogenesis and lymphangiogenesis, via an unknown mechanism, as well as lymphovascular invasion. Angiogenesis likely plays an important role in occurrence and progression in the initial phase of hindgut NETs.
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Affiliation(s)
- Yoichiro Okubo
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Osamu Motohashi
- Department of Gastroenterology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Norisuke Nakayama
- Department of Gastroenterology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Ken Nishimura
- Department of Gastroenterology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Rika Kasajima
- Molecular Pathology and Genetics Division, Kanagawa Cancer Center Research Institute, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Yohei Miyagi
- Molecular Pathology and Genetics Division, Kanagawa Cancer Center Research Institute, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Manabu Shiozawa
- Department of Gastrointestinal Surgery, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Emi Yoshioka
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Masaki Suzuki
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Kota Washimi
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Kae Kawachi
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Madoka Nito
- Department of Thoracic Surgery, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Yoichi Kameda
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Tomoyuki Yokose
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
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Paasch C, Hünerbein M, Theissig F. Endoscopic resection of a periampullary gangliocytic paraganglioma of the duodenum: A case report. Int J Surg Case Rep 2016; 29:39-43. [PMID: 27810610 PMCID: PMC5094203 DOI: 10.1016/j.ijscr.2016.10.060] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2016] [Accepted: 10/25/2016] [Indexed: 02/07/2023] Open
Abstract
The gangliocytic paraganglioma was resected endoscopically. Immunohistochemical examination reveal S100, synaptophysin and VIP expression. Introduction The gangliocytic paraganglioma (GP) is an extremely rare neuroendocrine tumour originating from the second part of the duodenum. Generally GP shows benign clinical behaviour. The GP is typically characterized by consisting of three tumour components: the epithelioid, the spindle-shaped and the ganglion-like cells. Presentation of case We present a female patient at the age of 65, who underwent a routine gastroscopy due to known gastro-oesophageal reflux. Accidentally a 2 × 1,5 cm sized mass of unknown entity was revealed in the duodenum. The magnet resonance tomography neither detected distant metastasis nor any local lymphadenopathy. After endosonographically guided punctures of the submucosal mass, a malignant tumour could not be diagnosed thus the decision to perform an endoscopical resection was made and successfully conducted. Immunohistochemical examination revealed a total resected GP. Discussion In literature malignant transformation with distant metastasis and local recurrences has been described. Furthermore the clinical manifestation and location varies. The GP has often been misdiagnosed as a neuroendocrine tumour (NET) G1. Conclusion Due to published cases of metastasising GPs, we recommend a long term follow-up. In a non-metastatic stage the endoscopic resection should be the therapy of choice in order to prevent unnecessary major surgical interventions. In this case report we will discuss the clinical appearance, behaviour and differential diagnosis of GP.
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Affiliation(s)
- Christoph Paasch
- Department of General, Visceral and Cancer Surgery, HELIOS Klinikum Berlin-Buch, Schwanebecker Chaussee 50, 13125 Berlin, Germany.
| | - Michael Hünerbein
- Department of General, Visceral and Cancer Surgery, HELIOS Klinikum Berlin-Buch, Schwanebecker Chaussee 50, 13125 Berlin, Germany.
| | - Franz Theissig
- Institute of Pathology, HELIOS Klinikum Emil von Behring, Walterhöferstraße 11, 14165 Berlin, Germany.
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Ribeiro I, Fernandes C, Fernandes S, Proença L, Silva J, Ponte A, Rodrigues J, Carvalho J. Endoscopic resection as a treatment for duodenal gangliocytic paraganglioma. GASTROENTEROLOGIA Y HEPATOLOGIA 2016; 39:605-606. [PMID: 26547609 DOI: 10.1016/j.gastrohep.2015.07.010] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 06/03/2015] [Revised: 07/28/2015] [Accepted: 07/31/2015] [Indexed: 11/12/2022]
Affiliation(s)
- Iolanda Ribeiro
- Department of Gastroenterology, Centro Hospitalar Vila Nova Gaia, Portugal.
| | - Carlos Fernandes
- Department of Gastroenterology, Centro Hospitalar Vila Nova Gaia, Portugal
| | - Sónia Fernandes
- Department of Gastroenterology, Centro Hospitalar Vila Nova Gaia, Portugal
| | - Luísa Proença
- Department of Gastroenterology, Centro Hospitalar Vila Nova Gaia, Portugal
| | - Joana Silva
- Department of Gastroenterology, Centro Hospitalar Vila Nova Gaia, Portugal
| | - Ana Ponte
- Department of Gastroenterology, Centro Hospitalar Vila Nova Gaia, Portugal
| | - Jaime Rodrigues
- Department of Gastroenterology, Centro Hospitalar Vila Nova Gaia, Portugal
| | - João Carvalho
- Department of Gastroenterology, Centro Hospitalar Vila Nova Gaia, Portugal
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50
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Caballero Rodríguez E, Arencibia Pérez B, Hernández Hernández G. Bowel obstruction secondary to yeyunal intussusception due to gangliocytic paraganglioma. REVISTA ESPANOLA DE ENFERMEDADES DIGESTIVAS 2016; 108:842-843. [PMID: 27756144 DOI: 10.17235/reed.2016.4185/2015] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
Gangliocytic paraganglioma is an uncommon entity. The jejunum location is extremely rare: there are only five cases reported in medical literature. We present a case of gangliocytic paraganglioma.
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