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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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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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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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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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Vanoli A, Albarello L, Uncini S, Fassan M, Grillo F, Di Sabatino A, Martino M, Pasquali C, Milanetto AC, Falconi M, Partelli S, Doglioni C, Schiavo-Lena M, Brambilla T, Pietrabissa A, Sessa F, Capella C, Rindi G, La Rosa S, Solcia E, Paulli M. Neuroendocrine Tumors (NETs) of the Minor Papilla/Ampulla: Analysis of 16 Cases Underlines Homology With Major Ampulla NETs and Differences From Extra-Ampullary Duodenal NETs. Am J Surg Pathol 2019; 43:725-736. [PMID: 30913089 DOI: 10.1097/pas.0000000000001234] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
Neuroendocrine tumors (NETs) of the minor papilla/ampulla (MIPA) are rare and poorly studied. Only individual case reports and no comprehensive analysis are available from the literature. We collected 16 MIPA NETs and investigated their clinicopathologic and immunohistochemical features, including markers such as somatostatin, pancreatic polypeptide, gastrin, serotonin, MUC1, cytokeratin 7, and somatostatin receptors type 2A and 5. The median age at diagnosis was 57.5 years, and the female-to-male ratio was 2.2:1. The median NET size was 1.45 cm, and most (94%) were low-grade (G1) tumors. Similarly to what was observed in the major ampulla, 3 histotypes were found: (i) ampullary-type somatostatin-producing tumors (ASTs, 10 cases), characterized by somatostatin expression in most tumor cells, focal-to-extensive tubulo-acinar structures, often with psammoma bodies, MUC1 reactivity, and no or rare membranous reactivity for somatostatin receptor type 2A; (ii) gangliocytic paragangliomas (3 cases), characterized by the coexistence of 3 tumor cell types: epithelioid, often reactive for pancreatic polypeptide, ganglion-like cells, and S100 reactive sustentacular/stromal cells; and (iii) ordinary nonfunctioning NETs (3 cases), resembling those more commonly observed in the extra-ampullary duodenum. Comparable histotypes could also be recognized among the 30 MIPA NETs from the literature. No NET-related patient death among MIPA cases was observed during a median follow-up of 38 months; however, MIPA ASTs showed lymph node metastases and invasion of the duodenal muscularis propria or beyond in 44% and 40% of cases, respectively. In conclusion, MIPA NETs closely resemble tumors arising in the major ampulla, with predominance of ASTs.
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Affiliation(s)
- Alessandro Vanoli
- Department of Molecular Medicine, Unit of Anatomic Pathology, University of Pavia
- Anatomic Pathology, IRCCS San Matteo Hospital Foundation, Pavia
| | | | - Stefania Uncini
- Department of Molecular Medicine, Unit of Anatomic Pathology, University of Pavia
| | - Matteo Fassan
- Department of Medicine (DIMED), Surgical Pathology & Cytopathology Unit
| | - Federica Grillo
- Department of Surgical Science and Integrated Diagnostics (DISC), Pathology Unit, University of Genoa
- San Martino Hospital, Genoa
| | - Antonio Di Sabatino
- First Department of Internal Medicine, IRCCS San Matteo Hospital Foundation, University of Pavia
| | - Michele Martino
- First Department of Internal Medicine, IRCCS San Matteo Hospital Foundation, University of Pavia
| | - Claudio Pasquali
- Department of Surgery 1 Pancreatic and Endocrine Digestive Surgical Unit, University of Padua, Padua
| | - Anna C Milanetto
- Department of Surgery 1 Pancreatic and Endocrine Digestive Surgical Unit, University of Padua, Padua
| | - Massimo Falconi
- Pancreatic Surgery Unit, Pancreas Translational & Clinical Research Center, San Raffaele Scientific Institute, "Vita-Salute" University
| | - Stefano Partelli
- Pancreatic Surgery Unit, Pancreas Translational & Clinical Research Center, San Raffaele Scientific Institute, "Vita-Salute" University
| | | | | | - Tatiana Brambilla
- Pathology Unit, Humanitas Research Hospital, Humanitas University, Rozzano, Milan
| | - Andrea Pietrabissa
- Department of Clinical-Surgical, Diagnostic and Pediatric Sciences, Unit of General Surgery 2, University of Pavia and IRCCS San Matteo Hospital Foundation, Pavia
| | - Fausto Sessa
- Department of Medicine and Surgery, Anatomic Pathology Unit, University of Insubria, Varese
| | - Carlo Capella
- Department of Medicine and Surgery, Anatomic Pathology Unit, University of Insubria, Varese
| | - Guido Rindi
- Institute of Anatomic Pathology
- Rome ENETS Center of Excellence, University Hospital Foundation A. Gemelli IRCCS-Catholic University of Sacred Heart, Rome, Italy
| | - Stefano La Rosa
- Service of Clinical Pathology, Institute of Pathology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Enrico Solcia
- Department of Molecular Medicine, Unit of Anatomic Pathology, University of Pavia
| | - Marco Paulli
- Department of Molecular Medicine, Unit of Anatomic Pathology, University of Pavia
- Anatomic Pathology, IRCCS San Matteo Hospital Foundation, Pavia
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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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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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Abstract
A case of gangliocytic paraganglioma of the second portion of the duodenal loop is presented. The tumor was polypoid and, histologically, composed of mature ganglion cells, spindle cells and epithelial-like cells. Immunocytochemical examination demonstrated the presence of neurofilament 200 K and S-100 protein only in the first two types of cells; all the cells were positive for neuron-specific enolase. The reaction for cytokeratin was negative in all neoplastic components. According to morphologic and immunocytochemical findings, we suggest a hamartomatous nature of this entity.
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Affiliation(s)
- S Dante
- Istituto di Anatomia e Istologia Patologica I, Università di Padova, Italia
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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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11
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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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Kang GE, Kim H, Lee JK, Kim DH, Jeong BN, Jang JH, Yeo SM, Sohn KR. Simultaneous Manifestation of Gangliocytic Paraganglioma and Heterotopic Pancreas of Ampulla of Vater Treated by Endoscopic Resection. ACTA ACUST UNITED AC 2016. [DOI: 10.15279/kpba.2016.21.4.232] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022]
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Abstract
Over the past three decades, Immunohistochemistry has materially changed the practice of diagnostic surgical pathology. Foundational observations in this field were critical to a reasoned assessment of both the risks and opportunities that immunohistochemistry afforded the surgical pathologist, and our current practice draws heavily on those early assessments. As we collectively look to and acknowledge those who recognized the value of this technique and who helped guide its development as a companion to (not a replacement for) histomorphologic evaluation, we are drawn to those whose mastery of detail and ability to draw common patterns from seemingly unrelated phenomena helped define the diagnostic power of immunohistochemistry. The focus of this review is on one individual, Dr. Juan Rosai, whose contributions transcend the simple linkage of molecular observations to morphology, recognizing novel patterns in both form and color (the latter often the lovely shades of diaminobenzidine), seemingly viewing our diagnostic world at times through an entirely different lens. By looking at Dr. Rosai's early work in this field, reviewing a selection of his seminal observations, particularly in the Immunohistochemistry of thyroid and thymic neoplasia, revisiting how his special insight is often guided by the work of the early masters of morphology, and how his mentorship of others has helped shaped academic surgical pathology practice, perhaps we can get a glimpse through that lens.
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Affiliation(s)
- Paul E Swanson
- Cumming School of Medicine, Calgary Laboratory Services, Calgary, Alberta.
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Abstract
Transmission electron microscopy (TEM) was introduced by Ruska and Knoll as a laboratory technique in 1933. Thereafter, several decades passed before the methods required for its optimal implementation were fully developed. Early uses of TEM were in Botany, rather than in Medicine; however, isolated publications did catalog the ultrastructural characteristics of several individual human tumor types. Finally, in 1968, Rosai and Rodriguez authored an important article, introducing the concept that TEM could be used for the differential diagnosis of histologically similar neoplasms. This publication heralded the steadily increasing application of TEM in anatomic pathology over the following decade, including continuing contributions by Dr. Juan Rosai. This brief review summarizes his influence on clinical electron microscopy, and lists some of the lesions for which that procedure is still a useful means of analysis.
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Affiliation(s)
- Mark R Wick
- Division of Surgical Pathology and Cytopathology, University of Virginia Health System, Charlottesville, Virginia.
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Zhuang Z, Yang C, Ryska A, Ji Y, Hou Y, Graybill SD, Bullova P, Lubensky IA, Klöppel G, Pacak K. HIF2A gain-of-function mutations detected in duodenal gangliocytic paraganglioma. Endocr Relat Cancer 2016; 23:L13-6. [PMID: 27130043 PMCID: PMC4899235 DOI: 10.1530/erc-16-0148] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/03/2016] [Accepted: 04/18/2016] [Indexed: 11/08/2022]
Affiliation(s)
- Zhengping Zhuang
- Surgical Neurology BranchNational Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA
| | - Chunzhang Yang
- Neuro-Oncology BranchNational Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
| | - Ales Ryska
- The Fingerland Department of PathologyCharles University Medical Faculty Hospital, Hradec Kralove, Czech Republic
| | - Yuan Ji
- Department of PathologyZhongshan Hospital, Fudan University, Shanghai, China
| | - Yingyong Hou
- Department of PathologyZhongshan Hospital, Fudan University, Shanghai, China
| | - Sky D Graybill
- San Antonio Military Medical CenterFort Sam Houston, Texas, USA
| | - Petra Bullova
- Section on Medical NeuroendocrinologyEunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA Department of Molecular Medicine Institute of Virology, Slovak Academy of Sciences, Bratislava, Slovakia
| | - Irina A Lubensky
- Cancer Diagnosis ProgramDivision of Cancer Treatment and Diagnosis, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
| | - Günter Klöppel
- Consultation Center for Pancreas and Neuroendocrine TumorsDepartment of Pathology, Technical University Munich, Munich, Germany
| | - Karel Pacak
- Section on Medical NeuroendocrinologyEunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA
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16
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Duval C, Isaac S, Lifante JC, Decaussin-Petrucci M. [A rare tumor of the Ampulla of Vater]. Ann Pathol 2015; 35:528-30. [PMID: 26586086 DOI: 10.1016/j.annpat.2015.06.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/01/2015] [Revised: 05/30/2015] [Accepted: 06/14/2015] [Indexed: 11/28/2022]
Affiliation(s)
- Caroline Duval
- Service d'anatomo-pathologie, hôpital Lyon Sud, hospices Civils de Lyon, université Lyon I, 69495 Pierre Bénite cedex, France
| | - Sylvie Isaac
- Service d'anatomo-pathologie, hôpital Lyon Sud, hospices Civils de Lyon, université Lyon I, 69495 Pierre Bénite cedex, France
| | - Jean-christophe Lifante
- Service de chirurgie endocrinienne, hôpital Lyon Sud, Hospices Civils de Lyon, Université Lyon I, 69495 Pierre Bénite cedex, France
| | - Myriam Decaussin-Petrucci
- Service d'anatomo-pathologie, hôpital Lyon Sud, hospices Civils de Lyon, université Lyon I, 69495 Pierre Bénite cedex, France.
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Hernández AG, Lanuza EDA, Matias AC, Huertas RP, Rodriguez KMG, Perez PG, Mompean FO. Large gangliocytic paraganglioma of the duodenum: A rare entity. World J Gastrointest Surg 2015; 7:170-173. [PMID: 26328037 PMCID: PMC4550844 DOI: 10.4240/wjgs.v7.i8.170] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/24/2015] [Revised: 05/14/2015] [Accepted: 06/08/2015] [Indexed: 02/06/2023] Open
Abstract
Gangliocytic paragangliomas are rare tumors that almost exclusively occur within the second portion of the duodenum. Although these tumors generally have a benign clinical course, they have the potential to recur or metastasize to regional lymph nodes. The case report presented here describes a 57-year-old female patient with melena, progressive asthenia, anemia, and a mass in the second-third portion of the duodenum that was treated by local excision. The patient was diagnosed with a friable bleeding tumor. The histologic analysis showed that the tumor was a 4 cm gangliocytic paraganglioma without a malignant cell pattern. In the absence of local invasion or distant metastasis, endoscopic resection represents a feasible, curative therapy. Although endoscopic polypectomy is currently considered the treatment of choice, it is not recommended if the size of the tumor is > 3 cm and/or there is active or recent bleeding. Patients diagnosed with a gangliocytic paraganglioma should be closely followed-up for possible local recurrence.
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Okubo Y, Nemoto T, Wakayama M, Tochigi N, Shinozaki M, Ishiwatari T, Aki K, Tsuchiya M, Aoyama H, Katsura K, Fujii T, Nishigami T, Yokose T, Ohkura Y, Shibuya K. Gangliocytic paraganglioma: a multi-institutional retrospective study in Japan. BMC Cancer 2015; 15:269. [PMID: 25886293 PMCID: PMC4399421 DOI: 10.1186/s12885-015-1308-8] [Citation(s) in RCA: 34] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/15/2014] [Accepted: 03/31/2015] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Gangliocytic paraganglioma (GP) is an extremely rare benign tumor that commonly arises from the second part of the duodenum. Since GP exhibit neither prominent mitotic activity nor Ki-67 immunoreactivity, this tumor is often misdiagnosed as neuroendocrine tumor (NET) G1 (carcinoid tumor). However, patients with GP may have a better prognosis than patients with NET G1. This fact emphasizes the importance of differentiating GP from NET G1, but few studies have reported the epidemiology and histopathology of GP because of its rarity. To differentiate GP from NET G1 with ease, we conducted a multi-institutional retrospective study analyzing the morphometric and immunohistochemical features of this tumor. METHODS Since only a limited number of patients with GP could be identified in our institute, we conducted a multi-institutional retrospective study of GP in Japan, which was approved by the Ethics Committee of our medical institute. The obtained tissue sections underwent detailed morphometric and immunohistochemical analyses. Additionally, to differentiate GP from NET G1 with ease, immunohistochemical findings were compared. RESULTS In our examination of 12 cases of duodenal GP, we found that epithelioid cells of GP exhibited positive reactivity for progesterone receptor and pancreatic polypeptide, whereas tumor cells of NET G1 were completely negative reactivity for both. Additionally, although GP is considered to be an extremely rare NET, we found that four (40.0%) of the ten patients at our institute with duodenal NET G1 actually had GP. CONCLUSIONS Although GP is regarded as a rare NET, our results suggest that it accounts for a substantial percentage of duodenal NETs. Additionally, confirmation of immunoreactivity for progesterone receptor and pancreatic polypeptide can assist in differentiating GP from NET G1.
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Affiliation(s)
- Yoichiro Okubo
- Department of Surgical Pathology, Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo, 143-8541, Japan.
| | - Tetsuo Nemoto
- Department of Surgical Pathology, Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo, 143-8541, Japan.
| | - Megumi Wakayama
- Department of Surgical Pathology, Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo, 143-8541, Japan.
| | - Naobumi Tochigi
- Department of Surgical Pathology, Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo, 143-8541, Japan.
| | - Minoru Shinozaki
- Department of Surgical Pathology, Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo, 143-8541, Japan.
| | - Takao Ishiwatari
- Department of Surgical Pathology, Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo, 143-8541, Japan.
| | - Kyoko Aki
- Department of Surgical Pathology, Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo, 143-8541, Japan.
| | - Masaru Tsuchiya
- Division of General and Gastroenterological Surgery, Department of Surgery (Omori), Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo, 143-8541, Japan.
| | - Hajime Aoyama
- Department of Pathology and Oncology, University of the Ryukyus, 59, Nishihara-cho, Nakagami-gun, Okinawa, 903-0214, Japan.
| | - Kanade Katsura
- Department of Pathology, Japanese Red Cross Kyoto Daini Hospital, 355-5, Jokyo-ku, Kyoto, 602-8026, Japan.
| | - Takeshi Fujii
- Department of Pathology, Toranomon Hospital, 2-2-2 Toranomon, Minato-ku, Tokyo, 105-8470, Japan.
| | - Takashi Nishigami
- Department of Pathology, Steel Memorial Hirohata Hospital, 3-1, Himeji, Hyogo, 671-1122, Japan.
| | - Tomoyuki Yokose
- Department of Pathology, Kanagawa Cancer Center, 1-1-2, Nakao, Asahi-ku, Yokohama, Kanagawa, 245-0815, Japan.
| | - Yasuo Ohkura
- Department of Pathology, Kyorin University School of Medicine, 6-20-2, Shinkawa, Mitaka, 181-8611, Japan.
| | - Kazutoshi Shibuya
- Department of Surgical Pathology, Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo, 143-8541, Japan. .,Department of Dermatology, Peking University First Hospital, Beijing, China.
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Loftus TJ, Kresak JL, Gonzalo DH, Sarosi GA, Behrns KE. Duodenal gangliocytic paraganglioma: A case report and literature review. Int J Surg Case Rep 2015; 8C:5-8. [PMID: 25600615 PMCID: PMC4353939 DOI: 10.1016/j.ijscr.2015.01.003] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/12/2014] [Accepted: 01/03/2015] [Indexed: 02/02/2023] Open
Abstract
Differentiation between GP and GIST alters treatment algorithms. Primary management of duodenal GP consists of resection with negative margins. Surveillance alone is safe and effective following resection with negative margins. For regionally advanced disease, consider adjuvant radiotherapy. Introduction Gangliocytic paraganglioma is a rare tumor that is most commonly located in the duodenum. At presentation, it may be confused with a gastrointestinal stromal tumor (GIST), but distinguishing between these tumors is critical because the natural history and treatment of these two tumors differs markedly. Duodenal gangliocytic paraganglioma typically exhibits benign behavior with occasional regional lymph node metastasis and no reports of tumor associated deaths. Recurrence after resection is rare. Presentation of case A 50 year-old male presented with melena and hemoglobin concentration of 4.6 g/dl. Esophagogastroduodenoscopy demonstrated a submucosal mass in the third portion of the duodenum with no active bleeding. CT scan identified no regional lymphadenopathy or distant metastasis. The tumor was resected through a longitudinal duodenotomy with negative margins. Discussion Endoscopic resection of duodenal gangliocytic paraganglioma appears to be safe and effective when tumor may be removed in its entirety by this method. If the tumor is not suspended by a stalk or there is suspicion for regional lymph node disease then surgical management is preferred. Radiation oncologists at high volume centers have endorsed utilization of adjuvant radiotherapy to the postsurgical bed in cases involving lymph node metastasis. Utilization of chemotherapy for management of this disease has not been reported. Conclusion Localized duodenal gangliocytic paragangliomas are best managed by resection with negative margins. In cases in which the tumor is resected with negative margins, it appears to be safe to embark on a course of surveillance and forego adjuvant therapy.
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Affiliation(s)
- Tyler J Loftus
- Department of Surgery, the University of Florida College of Medicine, Gainesville, FL 32610, USA
| | - Jesse L Kresak
- Department of Pathology, the University of Florida College of Medicine, Gainesville, FL 32610, USA
| | - David H Gonzalo
- Department of Pathology, the University of Florida College of Medicine, Gainesville, FL 32610, USA
| | - George A Sarosi
- Department of Surgery, the University of Florida College of Medicine, Gainesville, FL 32610, USA
| | - Kevin E Behrns
- Department of Surgery, the University of Florida College of Medicine, Gainesville, FL 32610, USA.
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20
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Gucer H, Mete O. Endobronchial gangliocytic paraganglioma: not all keratin-positive endobronchial neuroendocrine neoplasms are pulmonary carcinoids. Endocr Pathol 2014; 25:356-8. [PMID: 23912549 DOI: 10.1007/s12022-013-9258-7] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]
Affiliation(s)
- Hasan Gucer
- Department of Pathology, University Health Network, 200 Elizabeth Street, 11th floor, Toronto, ON, M5G 2C4, Canada
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21
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Rafiullah, Tanimu S, Onitilo AA. Gangliocytic paraganglioma, a rare ampullary tumour treated with open transduodenal resection and sphincteroplasty. BMJ Case Rep 2014; 2014:bcr-2013-202941. [PMID: 25100803 DOI: 10.1136/bcr-2013-202941] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022] Open
Abstract
Gangliocytic paragangliomas are rare neuroendocrine tumours residing in the gastrointestinal tract, most commonly the periampullary region. Most are benign tumours with a low malignancy rate. We report a 50-year-old man who presented with acute onset of left-upper quadrant abdominal pain with radiation to the back. An intraluminal mass at the junction of the duodenum with normal pancreatic/hepatic parenchyma was discovered on abdominal CT. Following upper endoscopy and MRI revealing a periampullary lesion, fine-needle aspiration and biopsies were. Immunohistochemistry was positive for synaptophysin and S100HU, consistent with gangliocytic paraganglioma. The benign nature of this tumour and unique anatomy of a separate opening of the pancreatic and common bile ducts led to transduodenal excision with sphincteroplasty, thereby avoiding extensive surgery. Surveillance CT every 6 months and upper endoscopy initially every 6 months (now yearly) revealed no evidence of endoscopic or histological recurrence at 3 years follow-up.
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Affiliation(s)
- Rafiullah
- Department of Internal Medicine, Saint Clair's Hospital, Weston, Wisconsin, USA
| | - Sabo Tanimu
- Department of Gastroenterology, Marshfield Clinic, Weston, Wisconsin, USA
| | - Adedayo A Onitilo
- Department of Oncology/Hematology, Marshfield Clinic, Weston, Wisconsin, USA
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22
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Yang JI, Choi JS, Lee GH, Kim BW, Moon SJ, Kang MS, Ahn HJ. A case of ampullary gangliocytic paraganglioma. Korean J Intern Med 2014; 29:375-8. [PMID: 24851073 PMCID: PMC4028528 DOI: 10.3904/kjim.2014.29.3.375] [Citation(s) in RCA: 15] [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] [Received: 10/04/2013] [Revised: 11/11/2013] [Accepted: 01/24/2014] [Indexed: 12/22/2022] Open
Abstract
Gangliocytic paragangliomas (GPs) are rare tumors of the duodenum, presenting as single sessile or pedunculated polypoid masses. Clinical manifestations of duodenal GPs can vary from an incidental finding at endoscopy to frequent upper gastrointestinal bleeding caused by mucosal ulceration and abdominal pain. GPs are considered benign, but the disease can recur and spread to regional lymph nodes. A 41-year-old female presented with abdominal pain. Upper gastrointestinal endoscopy revealed a subepithelial tumor of the ampulla of Vater in the second portion of the duodenum. The tumor was resected using the endoscopic mucosal resection technique. The tumor was diagnosed as benign GP of the duodenum using histological and immunohistochemical staining procedures.
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Affiliation(s)
- Ju-Il Yang
- Division of Gastroenterology, Department of Internal Medicine, Inje University Busan Paik Hospital, Busan, Korea
| | - Jung-Sik Choi
- Division of Gastroenterology, Department of Internal Medicine, Inje University Busan Paik Hospital, Busan, Korea
| | - Ga-Hee Lee
- Division of Gastroenterology, Department of Internal Medicine, Inje University Busan Paik Hospital, Busan, Korea
| | - Byeong-Woo Kim
- Division of Gastroenterology, Department of Internal Medicine, Inje University Busan Paik Hospital, Busan, Korea
| | - Seok-Jun Moon
- Division of Gastroenterology, Department of Internal Medicine, Inje University Busan Paik Hospital, Busan, Korea
| | - Mi Seon Kang
- Department of Pathology, Inje University Busan Paik Hospital, Busan, Korea
| | - Hyo Jung Ahn
- Department of Pathology, Inje University Busan Paik Hospital, Busan, Korea
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23
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Sathyamurthy A, Choudhary A, Ng D, Okponobi S, Diaz-Arias A, Grewal A, Hammoud GM. Obstructive jaundice due to a rare periampullary tumor. World J Gastrointest Oncol 2013; 5:195-197. [PMID: 24137522 PMCID: PMC3796670 DOI: 10.4251/wjgo.v5.i10.195] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/20/2013] [Revised: 08/06/2013] [Accepted: 09/04/2013] [Indexed: 02/05/2023] Open
Abstract
Gangliocytic paraganglioma is a rare neuroendocrine tumor predominantly arising in the second part of the duodenum with rare local recurrence or metastasis to regional lymph nodes. A 92-year-old female presented with obstructive jaundice. On exam she had pale conjunctiva and icteric sclera. Abdominal examination revealed tenderness in the upper abdomen. Laboratory data was consistent with obstructive jaundice. Computed tomography of the abdomen revealed a dilated gall bladder and a common bile duct (CBD) with no evidence of liver lesions or pancreatic head mass. Endoscopic ultrasonography revealed a 1 cm isoechoic submucosal nodule at the periampullary area, dilated CBD (9 mm), a prominent pancreatic duct (4.1 mm) and a hydropic gall bladder with no stones. Endoscopic retrograde cholangiopancreaticography was performed to relieve obstruction and showed a 1 cm periampullary mass which underwent an en-bloc snare resection. Histopathology analyses with immunohistochemical stains were positive for cytokeratin, synaptophysin, S-100 protein, neuron specific enolase and negative for actin and desmin consistent with periampullary gangliocytic paraganglioma. Periampullary gangliocytic paraganglioma is a rare benign tumor of the small bowel. Common presentation includes abdominal pain and obstructive jaundice which should be included in differential diagnosis of obstructive jaundice. Endoscopic resection is a curative therapy in the absence of local invasion or distant metastasis.
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24
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Papp R, Baracs J, Papp A, Tornóczki T, Vincze Á, Horváth ÖP, Kelemen D. Ganglioneuroma in the papilla of Vater with neurofibromatosis type 1: report of a case. Surg Today 2012; 43:675-7. [DOI: 10.1007/s00595-012-0367-6] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/16/2011] [Accepted: 01/31/2012] [Indexed: 11/30/2022]
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25
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PDX-1, CDX-2, TTF-1, and CK7: a reliable immunohistochemical panel for pancreatic neuroendocrine neoplasms. Am J Surg Pathol 2012; 36:737-43. [PMID: 22498824 DOI: 10.1097/pas.0b013e31824aba59] [Citation(s) in RCA: 60] [Impact Index Per Article: 4.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
Neuroendocrine tumors (NETs) occur in virtually all sites of the body. As NETs arising in different organs share similar morphologic features, distinguishing metastatic from primary NETs on the basis of morphologic grounds alone is difficult. Pancreatic duodenal homeobox 1 (PDX-1) is a Hox-type transcription factor that is essential for both exocrine and endocrine pancreatic differentiation and maintenance of β-cell function. We investigated PDX-1 as an immunohistochemical (IHC) marker in primary pancreatic NETs. Eighty primary NETs [25 pancreatic, 29 bronchopulmonary, and 26 in the gastrointestinal (GI) tract] and 13 metastatic NETs in the liver were studied. Clinical and radiologic data were reviewed to confirm the stated primary sites. IHC analysis for PDX-1, CDX-2, thyroid transcription factor-1 (TTF-1), keratin 7 (CK7), and keratin 20 (CK20) was performed, and the results were based on review blinded to the primary sites. PDX-1 was seen in 18 of 25 (72%) pancreatic NETs; in contrast, only 3 of 29 (10%) bronchopulmonary NETs and 1 of 26 (4%) GI NETs were positive. PDX-1 was therefore 93% specific and 72% sensitive for pancreatic NETs. TTF-1 was expressed only in bronchopulmonary NETs; all other NETs were negative for TTF-1. CK7 was also very specific (92%) and moderately sensitive (66%) for bronchopulmonary NETs. CDX-2 was seen in 22 of 26 (85%) cases of GI NETs and in only 1 of 51 (2%) cases of extra-GI NETs. Thus, CDX-2 was 98% specific and 85% sensitive for GI NETs. In terms of metastatic NETs found in the liver, PDX-1 was positive in 5 of 5 cases of metastatic pancreatic NETs and 2 of 2 cases of metastatic duodenal NETs. PDX-1 is highly specific, with very good overall diagnostic accuracy for pancreatic NETs. An IHC panel including PDX-1, CDX-2, TTF-1, CK7, and CK20 may be useful in distinguishing NETs of pancreatic origin from other primaries.
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26
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Nuño-Guzmán CM, Arróniz-Jáuregui J, Alvarez-López F, Corona JL, Cerda-Camacho F, Rostro R, Gutiérrez-Manjarrez JI. Obstructing gangliocytic paraganglioma in the third portion of the duodenum. Case Rep Gastroenterol 2012; 6:489-95. [PMID: 22855666 PMCID: PMC3409504 DOI: 10.1159/000341586] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/08/2023] Open
Abstract
Gangliocytic paragangliomas are infrequent tumors almost exclusively found in the second portion of the duodenum. An unusual case of a gangliocytic paraganglioma in the third portion of the duodenum with obstructive symptoms is herein reported. A 16-year-old male patient presented with epigastric pain, postprandial plenitude and reflux. A barium swallow failed to demonstrate abnormalities. Endoscopy showed a pedunculated submucosal tumor, originating at the third duodenal portion and causing partial obstruction. Biopsy was not performed due to the risk of bleeding. CT scan demonstrated a polypoid lesion. Through a transmesocolic approach and an anterior duodenotomy, resection of the tumor was performed. No lymph node or other organ affection was found. Histologic examination revealed a gangliocytic paraganglioma. Immunohistochemical examination was performed. Gangliocytic paragangliomas originating in the third or fourth portion of the duodenum, as in the present case, are extremely rare. Characteristic histologic features including epithelioid cells, spindle-shaped cells and ganglion-like cells were met. The majority of cases manifest with a similar benign behavior. Local resection of the tumor is recommended for these cases. An infrequent case of a gangliocytic paraganglioma located in the third portion of the duodenum, with a less common clinical presentation, is herein reported.
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Affiliation(s)
- Carlos M Nuño-Guzmán
- Department of General Surgery, Antiguo Hospital Civil de Guadalajara 'Fray Antonio Alcalde', Guadalajara, Mexico
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27
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Duodenal gangliocytic paraganglioma with lymph node metastasis and an 8-year follow-up: a case report. Eur J Gastroenterol Hepatol 2012; 24:90-4. [PMID: 22081010 DOI: 10.1097/meg.0b013e32834dfdfa] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
Abstract
Gangliocytic paraganglioma (GP) is a rare tumor, usually located in the second part of the duodenum. On pathological examination, GP is characterized by the association of the histological features of paragangliomas, ganglioneuromas, and carcinoid tumors. Classical clinical presentations are upper gastrointestinal bleeding and abdominal pain. Preoperative diagnosis is difficult because of the submucosal site of the tumor, with usually negative mucosal biopsies. Endoscopic ultrasound helps establish the diagnosis and allows lymph node staging, which will guide the choice of the treatment. If GPs usually follow a benign course, metastatic spread to regional lymph nodes treated by surgical resection alone has been reported. We report a case of GP with lymph node metastases treated by duodenopancreatectomy with long-term disease-free survival, suggesting that surgical resection is a reasonable approach for metastatic GPs.
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28
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Abstract
This article reviews the most common and characteristic mesenchymal lesions found in the gastrointestinal tract and intraabdominal location in a pattern-based approach: spindle and epithelioid tumors (gastrointestinal stromal tumor, schwannoma, glomus tumor, leiomyoma and leiomyosarcoma, inflammatory fibroid polyp, perineurioma, melanoma, calcifying fibrous tumor, sclerosing mesenteritis, mesenteric fibromatosis, and inflammatory myofibroblastic tumor), and clear and granular tumors (clear cell sarcoma, granular cell tumor, gangliocytic paraganglioma, and ganglioneuroma). Information includes gross and histologic features, diagnosis and differential diagnosis, and histologic and other diagnostic techniques, including immunohistochemistry related to projected patient outcome, along with prognosis, staging, and treatment.
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Affiliation(s)
- Dora Lam-Himlin
- Mayo Clinic Arizona, 13400 East Shea Boulevard, Scottsdale, AZ 85259, USA
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29
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30
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Okubo Y, Wakayama M, Nemoto T, Kitahara K, Nakayama H, Shibuya K, Yokose T, Yamada M, Shimodaira K, Sasai D, Ishiwatari T, Tsuchiya M, Hiruta N. Literature survey on epidemiology and pathology of gangliocytic paraganglioma. BMC Cancer 2011; 11:187. [PMID: 21599949 PMCID: PMC3141762 DOI: 10.1186/1471-2407-11-187] [Citation(s) in RCA: 69] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/24/2010] [Accepted: 05/20/2011] [Indexed: 12/20/2022] Open
Abstract
BACKGROUND Although gangliocytic paraganglioma (GP) has generally been regarded as a neuroendocrine tumor, its origin remains unclear. We therefore aimed to investigate the details of this disease by carefully analyzing and extracting common features of the disease as presented in selected publications. METHODS We searched for English and Japanese cases of GP using the PubMed and IgakuChuoZasshi databases on August 2010. We then extracted and sampled raw data from the selected publications and performed appropriate statistical analyses. Additionally, we evaluated the expression of hormone receptors based on our previously reported case. RESULTS 192 patients with GP were retrieved from the databases. Patient ages ranged from 15 y to 84 y (mean: 52.3 y). The gender ratio was 114:76 (male to female, 2 not reported). Maximum diameter of the tumors ranged from 5.5 mm to 100 mm (mean: 25.0 mm). The duodenum (90.1%, 173/192) was found to be the most common site of the disease. In 173 patients with duodenal GP, gastrointestinal bleeding (45.1%, 78/173) was found to be the most common symptom of the disease, followed by abdominal pain (42.8%, 74/173), and anemia (14.5%, 25/173). Rate of lymph node metastasis was 6.9% (12/173). Our statistical analysis indicated that significant differences were found for gender between GP within the submucosal layer and exceeding the submucosal layer. Furthermore, our immunohistochemical evaluation showed that both epithelioid and pancreatic islet cells showed positive reactivity for progesterone receptors. CONCLUSIONS Our literature survey revealed that there were many more cases of GP exceeding the submucosal layer than were expected. Meanwhile, our statistical analyses and immunohistochemical evaluation supported the following two hypotheses. First, vertical growth of GP might be affected by progesterone exposure. Second, the origin of GP might be pancreatic islet cells. However, it is strongly suspected that our data have been affected by publication bias and to confirm these hypotheses, further investigation is required.
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Affiliation(s)
- Yoichiro Okubo
- Department of Surgical Pathology, Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-Ku, Tokyo, 143-8541, Japan
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31
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Parray FQ, Lone IM, Chowdri NA, Wani I, Wani MA, Gulzar GM, Thakur N. Emergency pancreaticoduodenectomy in duodenal paraganglioma: case report. ISRN SURGERY 2011; 2011:268674. [PMID: 22084750 PMCID: PMC3195982 DOI: 10.5402/2011/268674] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 02/22/2011] [Accepted: 03/28/2011] [Indexed: 11/23/2022]
Abstract
Duodenal gangliocytic paraganglioma (DGP) is a rare tumor that characteristically occurs in the second part of duodenum. These appear as submucosal masses that protrude into the lumen of a duodenum. Gastrointestinal bleeding is the commonest manifestation of DGP. Metastatic spread to regional lymph nodes occurs rarely. Surgical resection is the treatment of choice for DGP. A case of a DGP is reported in young female who presented with a recurrent upper gastrointestinal bleeding. Upper gastrointestinal endoscopy (UGIE) documented a mass in the ampullary region with ulceration in its middle which was bleeding. Recurrent gastrointestinal bleeding necessitated an emergency pancreaticoduodenectomy. Histopathology of specimen documented gangliocytic paraganglioma.
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Affiliation(s)
- Fazl Q Parray
- Department of General Surgery, SKIMS, Srinagar, Kashmir 190011, India
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32
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Abstract
In this essay, the role of the neural crest in the development of the vertebrate embryo is briefly described. The techniques used to document the neural crest origin of various cell types and the tumors arising from them are discussed, with emphasis on Le Douarin's quail-chick chimera model. The current dogma on the origin of the cells of the diffuse endocrine system is presented, and some personal conjectures based on the microscopic appearances of various types of normal, vestigial and neoplastic human tissues are offered to the reader as 'food for thought.'
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Affiliation(s)
- Juan Rosai
- International Center for Oncologic Pathology Consultations, Centro Diagnostico Italiano, Milan, Italy.
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Ogata S, Horio T, Sugiura Y, Aiko S, Aida S. Duodenal gangliocytic paraganglioma with regional lymph node metastasis and a glandular component. Pathol Int 2010; 61:104-7. [PMID: 21255188 DOI: 10.1111/j.1440-1827.2010.02620.x] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Gangliocytic paraganglioma (GP) is generally considered to be a benign periampullary lesion, although it is unclear whether it should be classified as a hamartoma or as a neoplasm. Here, we present a GP case with lymph node metastasis. A 16-year-old boy complained of exertional dyspnea. Upper endoscopy and imaging studies revealed a polypoid ampullary tumor. Pancreaticoduodenectomy with lymph node dissection was performed due to swelling of peripancreatic lymph nodes. Histologically, the tumor consisted of three cell types: epithelioid; spindle; and ganglion cells. In addition to these typical components of GP, a distinct glandular component was also present. There was substantial invasion of tumor cells into the lymphovascular vessels, associated with lymph node metastases. These lymph node metastases were histologically similar to the primary tumor. To judge from these findings GP may be a true neoplasm with metastatic capacity. Pre- and intraoperative investigations for lymph node or distant metastases are required for adequate resection of this kind of tumor.
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Affiliation(s)
- Sho Ogata
- Department of Pathology, Laboratory Medicine Surgery, National Defense Medical College, Saitama, Japan.
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Rowsell C, Coburn N, Chetty R. Gangliocytic paraganglioma: a rare case with metastases of all 3 elements to liver and lymph nodes. Ann Diagn Pathol 2010; 15:467-71. [PMID: 21036639 DOI: 10.1016/j.anndiagpath.2010.07.009] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/20/2010] [Revised: 07/27/2010] [Accepted: 07/27/2010] [Indexed: 02/07/2023]
Abstract
Gangliocytic paragangliomas are a rare but well-described neuroendocrine tumor with a proclivity for the duodenal area. It is usually a locally infiltrative lesion with a good prognosis. Rare cases that spread to lymph nodes have been documented. A 52-year-old female was found to have a duodenal tumor, enlarged regional lymph nodes, and multiple small nodules in the liver. The duodenal tumor was a classic triphasic gangliocytic paraganglioma. Two regional lymph nodes and one of the nodules in the liver were noted to contain all 3 elements constituting a gangliocytic paraganglioma. This is the second case of gangliocytic paraganglioma with liver spread and is also noteworthy because of the presence of all 3 elements in the metastases. The presence of lymph node and liver metastases should not result in overtreatment as such behavior is not necessarily fraught with a bad clinical outcome. It is probably best to regard such cases as having "uncertain malignant potential" and advocate careful follow-up.
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Affiliation(s)
- Corwyn Rowsell
- Department of Pathology, Sunnybrook Health Sciences Centre, Toronto M4N 3M5, Canada
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35
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Chong Y, Cho MY. First Report of a Gangliocytic Paraganglioma Arising in a Tailgut Cyst. KOREAN JOURNAL OF PATHOLOGY 2010. [DOI: 10.4132/koreanjpathol.2010.44.4.435] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Affiliation(s)
- Yosep Chong
- Department of Pathology, Yonsei University Wonju College of Medicine, Wonju, Korea
| | - Mee-Yon Cho
- Department of Pathology, Yonsei University Wonju College of Medicine, Wonju, Korea
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36
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Ohtsuki Y, Watanabe R, Kimura M, Okamoto T, Murakami S, Mizukami Y, Takeji M, Okada Y, Hayashi Y, Lee GH, Furihata M. Immunohistochemical and electron microscopic studies of a case of duodenal gangliocytic paraganglioma. Med Mol Morphol 2009; 42:245-9. [PMID: 20033372 DOI: 10.1007/s00795-009-0442-2] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/09/2009] [Accepted: 02/17/2009] [Indexed: 11/25/2022]
Abstract
Gangliocytic paraganglioma (GPG) is a rare tumor, occurring almost exclusively in the duodenum. In the present case, a submucosal tumor 2.5 x 2 x 1.5 cm in size was located on the anal side of the papilla of Vater, with clear margins and without capsule on cut-surface examination. Tumor cells included three types of cells: ganglion-like cells (GCs), endocrine cells (ECs), and Schwann cells (SCs). The GCs were large with eccentric nuclei with large nucleoli and clear abundant cytoplasm. ECs were detected in small nests, surrounded by bundles of SCs. Immunohistochemically, GCs were positive for synaptophysin, neuron-specific enolase (NSE), and CD56. ECs were positive for chromogranin A, NSE, somatostatin, pancreatic polypeptide, and CD56, and were associated with S100 protein-positive SCs. On fine structural examination, ECs contained numerous membrane-bounded secretory granules, 250-450 nm in diameter, in their cytoplasm, surrounded by a branched, complex basal lamina. SCs possessed basal lamina along their long interlacing cytoplasmic processes. The histogenesis of GPG most likely involves proliferation and differentiation of pluripotent stem cells in the duodenal crypts in the duodenum as a true tumor, although it is also possible that the retroperitoneal components of both GCs and SCs proliferate, together with ECs, from ventral primordial tissue of the pancreas in the duodenum. The immunohistochemical and ultrastructural findings of a case of GPG are reported, focusing on three major cellular components: GCs, ECs, and SCs.
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Affiliation(s)
- Yuji Ohtsuki
- Division of Pathology, Matsuyama-shimin Hospital, Matsuyama, Ehime 790-0067, Japan.
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37
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Giuseppe A, Silvia C, Ambrogio F. Duodenal Periampullary Gangliocytic Paraganglioma: Report of Two Cases with Immunohistochemical and Ultrastructural Study. Ultrastruct Pathol 2009. [DOI: 10.1080/01913120120824] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022]
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38
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Witkiewicz A, Galler A, Yeo CJ, Gross SD. Gangliocytic paraganglioma: case report and review of the literature. J Gastrointest Surg 2007; 11:1351-4. [PMID: 17653595 DOI: 10.1007/s11605-007-0217-9] [Citation(s) in RCA: 57] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/15/2007] [Accepted: 06/12/2007] [Indexed: 01/31/2023]
Abstract
Gangliocytic paraganglioma is a rare tumor, which occurs nearly exclusively in the second portion of the duodenum. Generally, this tumor has a benign clinical course, although rarely, it may recur or metastasize to regional lymph nodes. Only one case with distant metastasis has been reported. We present a case of duodenal gangliocytic paraganglioma treated first by local resection followed by pylorus-preserving pancreaticoduodenectomy. Examination of the first specimen revealed focal nuclear pleomorphism and mitotic activity, in addition to the presence of three characteristic histologic components: epithelioid, ganglion, and spindle cell. In the subsequent pancreaticoduodenectomy specimen, there was no residual tumor identified in the periampullary area, but metastatic gangliocytic paraganglioma was present in two of seven lymph nodes. This case report confirms the malignant potential of this tumor. We review the published literature on gangliocytic paragangliomas pursuing a malignant course. We conclude that surgical therapy of these neoplasms should not be limited to local resection, as disease recurrence, lymph node involvement, and rarely distant metastasis may occur.
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Affiliation(s)
- Agnes Witkiewicz
- Department of Pathology, Thomas Jefferson University, Philadelphia, PA, USA
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39
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Morita T, Tamura S, Yokoyama Y, Onishi T, Kuratani Y, Mizuta H, Onishi S. Endoscopic resection of a duodenal gangliocytic paraganglioma. Dig Dis Sci 2007; 52:1400-4. [PMID: 17415650 DOI: 10.1007/s10620-006-9370-3] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/02/2006] [Accepted: 04/01/2006] [Indexed: 12/09/2022]
Abstract
A gangliocytic paraganglioma (GP) is an extremely rare neurogenic tumor nearly located in the second portion of the duodenum and has been regarded as benign. We report a case of duodenal GP in a 53-year-old man. The GP arose in the second portion of the duodenum and was shown by histological examination to consist of epithelioid cells, spindle cells, and ganglion-like cells. An endoscopic ultrasonography showed a round, well-demarcated, inhomogeneous, submucosal tumor in the 3rd-4th layer. We resected it by endoscopic mucosal resection.
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Affiliation(s)
- Tomoko Morita
- Department of Gastroenterology and Hepatology, Kochi Medical School, Kohasu, Kochi, Japan.
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40
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Loew BJ, Lukens FJ, Navarro F, Roy M, Mattia A, Howell DA. Successful endoscopic resection of a gangliocytic paraganglioma of the minor papilla in a patient with pancreas divisum and pancreatitis (with video). Gastrointest Endosc 2007; 65:547-50. [PMID: 17145056 DOI: 10.1016/j.gie.2006.07.019] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/27/2006] [Accepted: 07/18/2006] [Indexed: 02/08/2023]
Affiliation(s)
- Burr J Loew
- Division of Gastroenterology and Pancreaticobiliary Center, Departments of Medicine and Pathology, Maine Medical Center, Portland, ME, USA
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41
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42
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Paláu MA, Merino MJ, Quezado M. Corticotropin-producing pulmonary gangliocytic paraganglioma associated with Cushing's syndrome. Hum Pathol 2006; 37:623-6. [PMID: 16647961 DOI: 10.1016/j.humpath.2005.12.006] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/11/2005] [Revised: 11/23/2005] [Accepted: 12/05/2005] [Indexed: 10/24/2022]
Abstract
Gangliocytic paraganglioma (GP) is an unusual tumor in the wide spectrum of neuroendocrine neoplasms. The biologic behavior and histogenesis of this tumor is uncertain because, despite its benign histologic features, there have been reports of metastases to regional lymph nodes and distant metastases. Although most of these tumors have been reported in the duodenum, examples of GP arising in extra-ampullary sites have been well documented. Herein, we report the case of a lung GP corticotropin producing and presenting as Cushing's syndrome. After removal of the tumor, there was a significant drop in cortisol and corticotropin levels. To our knowledge, this is the first report of a GP causing Cushing's syndrome.
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Affiliation(s)
- Mauricio A Paláu
- Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA.
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43
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Plaza JA, Vitellas K, Marsh WL. Duodenal gangliocytic paraganglioma: a radiological-pathological correlation. Ann Diagn Pathol 2005; 9:143-7. [PMID: 15944956 DOI: 10.1016/j.anndiagpath.2005.02.004] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Abstract
Duodenal gangliocytic paraganglioma is a rare tumor that characteristically occurs in the second portion of the duodenum and typically presents with gastrointestinal bleeding. Gangliocytic paragangliomas have a characteristic triphasic microscopic appearance with epithelioid cells, spindle cells, and ganglion cells, resulting in a complex histology with features of paraganglioma, carcinoid, and ganglioneuroma. Duodenal gangliocytic paragangliomas have an excellent prognosis after surgical resection but metastatic spread to regional lymph nodes and recurrence may rarely occur. We report a case of duodenal gangliocytic paraganglioma and discuss the radiological and pathological differential diagnosis of this rare entity.
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Affiliation(s)
- Jose Antonio Plaza
- Department of Pathology, The Ohio State University Medical Center, Columbus, OH 43210, USA
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44
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Nwakakwa V, Kahaleh M, Bennett A, Berg C, Brock A, Wick MR, Yeaton P. EMR of ampullary gangliocytic paragangliomas. Gastrointest Endosc 2005; 62:318-22. [PMID: 16047009 DOI: 10.1016/s0016-5107(05)01580-4] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
Affiliation(s)
- Victor Nwakakwa
- Digestive Health Center, Department of Pathology, University of Virginia Health System, Charlottesville, 22908, USA
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45
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Wong A, Miller AR, Metter J, Thomas CR. Locally advanced duodenal gangliocytic paraganglioma treated with adjuvant radiation therapy: case report and review of the literature. World J Surg Oncol 2005; 3:15. [PMID: 15740625 PMCID: PMC554089 DOI: 10.1186/1477-7819-3-15] [Citation(s) in RCA: 37] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/17/2004] [Accepted: 03/01/2005] [Indexed: 12/14/2022] Open
Abstract
BACKGROUND: Gangliocytic paraganglioma are rare neoplasms that predominantly arise in periampulary region. Though considered benign the disease can spread to regional lymphatics. CASE PRESENTATION: A 49 year old woman presented with melena and was found to have a periampullary mass. Endoscopic evaluation and biopsy demonstrated a periampullary paraganglioma. The tumor was resected with pylorus-preserving pancreaticoduodenectomy and was found to represent a gangliocytic paraganglioma associated with nodal metastases. In a controversial decision, the patient was treated with adjuvant external beam radiation therapy. She is alive and well one year following resection. The authors have reviewed the current literature pertaining to this entity and have discussed the biologic behavior of the tumor as well as the rationale for treatment strategies employed. CONCLUSION: Paraganglioma is a rare tumor that typically resides in the gastrointestinal tract and demonstrates low malignant potential. Due to rarity of the disease there is no consensus on the adjuvant treatment even though nearly 5% of the lesions demonstrate the malignant potential.
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Affiliation(s)
- Adrian Wong
- Department of Radiation Oncology, School of Medicine, University of Texas Health Science Center @ San Antonio, San Antonio, TX, 78229, USA
| | - Alexander R Miller
- Division of Surgical Oncology, Cancer Therapy & Research Center, San Antonio, TX, 78229, USA
| | - John Metter
- Department of Pathology, Methodist Hospital, San Antonio, TX, 78229, USA
| | - Charles R Thomas
- Department of Radiation Oncology, School of Medicine, University of Texas Health Science Center @ San Antonio, San Antonio, TX, 78229, USA
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46
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Weinrach DM, Wang KL, Blum MG, Yeldandi AV, Laskin WB. Multifocal presentation of gangliocytic paraganglioma in the mediastinum and esophagus. Hum Pathol 2004; 35:1288-91. [PMID: 15492999 DOI: 10.1016/j.humpath.2004.07.013] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
Gangliocytic paraganglioma (GP) is a rare, typically benign tumor that shows neuroectodermal (neurosustentacular or Schwannian and neuronal) and neuroendocrine differentiation. Once thought to arise exclusively in the periampullary region as a solitary lesion, recent reports have documented both origin of GP in a variety of extra-duodenal sites as well as synchronous multifocal presentation of the tumor. Herein, we describe the first reported case of simultaneous occurrence of GP in the superior mediastinum and esophagus. A mass in the mid-distal esophagus and a separate mass in the superior mediastinum at the thoracic inlet were found in a 58-year-old woman by computed tomography scan. Subsequent biopsy of the superior mediastinal mass showed nests of epithelioid tumor cells coexisting with ganglioneuromatous elements, whereas biopsy of the esophageal mass showed nests of epithelioid cells with interspersed ganglion cells. The epithelioid tumor cells showed diffuse immunohistochemical expression of keratin (CAM 5.2), chromogranin, and synaptophysin supporting true neuroendocrine differentiation; ganglion cells expressed S-100 protein and neurofilament protein; and the spindled elements expressed S-100 protein, neurofilament protein, and glial fibrillary acidic protein indicating Schwannian differentiation. The finding of another GP occurring outside the periampullary region bolsters the argument for a stem cell origin of this unusual tumor.
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Affiliation(s)
- David M Weinrach
- Department of Pathology, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA
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47
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Ghouti L, Moutardier V, Hassoun J, Delpero JR. Paragangliome gangliocytique péri-ampullaire traité par ampullectomie chirurgicale. ACTA ACUST UNITED AC 2004; 28:494-6. [PMID: 15243329 DOI: 10.1016/s0399-8320(04)94970-6] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
We report the case of a 40-Year-old woman with duodenal gangliocytic paraganglioma of the ampulla of Vater. Preoperative diagnosis was periampullar tumor. Final diagnosis of duodenal gangliocytic paragangioma of the ampulla of Vater with negative margins was made by histological and immunohistochemical study of surgical ampullectomy specimen. This case report stresses the yield of immunohostochemical study in the diagnosis of duodenal gangliocytic paraganglioma, a rare entity.
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Affiliation(s)
- Laurent Ghouti
- Service de chirurgie digestive, Institut Paoli-Calmettes, Marseille.
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48
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Kee AR, Forrest CH, Brennan BA, Papadimitriou JM, Glancy RJ. Gangliocytic paraganglioma of the bronchus: a case report with follow-up and ultrastructural assessment. Am J Surg Pathol 2003; 27:1380-5. [PMID: 14508400 DOI: 10.1097/00000478-200310000-00011] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
We report a case of gangliocytic paraganglioma of bronchus. A 54-year-old woman underwent bronchoscopy following two episodes of right lower lobe pneumonia over the previous 5 months with unresolved chest radiographic changes. A computerized tomographic scan showed a right lower lobe endobronchial lesion, and at bronchoscopy there was a mass partly occluding the lumen of the bronchus. The biopsy and subsequent bronchoscopic resection showed a tumor with morphologic, immunohistochemical, and ultrastructural features of paragangliomatous, gangliocytic, and Schwann cell differentiation consistent with a gangliocytic paraganglioma. The lesion was treated conservatively with bronchoscopic resection and laser therapy. Histopathologic examination of recurrent tumor at 6 months showed features consistent with paraganglioma. Ten months after initial diagnosis, there was no bronchoscopic evidence of residual tumor. The occurrence of gangliocytic paraganglioma in diverse sites gives cause for the reappraisal of the histogenesis of this fascinating lesion. The variable morphology of this lesion may be an expression of the potential for divergent differentiation of a pluripotent stem cell.
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Affiliation(s)
- Ai-Rene Kee
- Department of Histopathology, Fremantle Hospital, Fremantle, Australia
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49
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Mahdavi A, Silberberg B, Malviya VK, Braunstein AH, Shapiro J. Gangliocytic paraganglioma arising from mature cystic teratoma of the ovary. Gynecol Oncol 2003; 90:482-5. [PMID: 12893225 DOI: 10.1016/s0090-8258(03)00272-5] [Citation(s) in RCA: 29] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022]
Abstract
BACKGROUND Gangliocytic paraganglioma is a rare neoplasm involving the small intestine, stomach, and spinal cord. Ovarian gangliocytic paraganglioma has not been reported in the medical literature. CASE A 55-year-old caucasian woman underwent exploratory laparotomy, total abdominal hysterectomy, and bilateral salpingo-oophorectomy for evaluation of a persistent right adnexal mass. Microscopic examination of the right ovary revealed a mature cystic teratoma with a mural nodule consistent with gangliocytic paraganglioma. As there was no evidence of significant pleomorphism or neoplastic infiltration, surgical staging was not performed. CONCLUSION Gangliocytic paraganglioma may arise from ovarian cystic teratoma. Although most cases of gangliocytic paragangliomas are benign, surgical staging and retroperitoneal lymphadenectomy may be required if histopathology is suggestive of invasive disease or if enlarged lymph nodes are noted.
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Affiliation(s)
- Ali Mahdavi
- Division of Gynecologic Oncology, Providence Hospital and Medical Centers, 16001 West Nine Mile Road, Southfield, MI 48075, USA.
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50
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Sundararajan V, Robinson-Smith TM, Lowy AM. Duodenal gangliocytic paraganglioma with lymph node metastasis: a case report and review of the literature. Arch Pathol Lab Med 2003; 127:e139-41. [PMID: 12653602 DOI: 10.5858/2003-127-e139-dgpwln] [Citation(s) in RCA: 50] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
A case of duodenal gangliocytic paraganglioma (DGP) in a 67-year-old woman is presented. The DGP arose in the second part of the duodenum. Although most of the reported cases of DGP are considered benign, in the present case, we found regional lymph nodes containing metastatic tumor. Previous reports have documented metastases containing only epithelioid cells. The current case demonstrates metastatic tumor in regional lymph nodes containing all 3 of the DGP components (spindle cells, ganglion-like cells, and epithelioid cells).
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Affiliation(s)
- Vanitha Sundararajan
- Department of Pathology and Laboratory Medicine, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
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