|
1
|
Zhao Z, Guo Y, Liu L, Zhang L, Li S, Yang J. Primary non-functional pancreatic paraganglioma: A case report and review of the literature. J Int Med Res 2022; 50:3000605221143023. [PMID: 36562124 PMCID: PMC9793047 DOI: 10.1177/03000605221143023] [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] [Indexed: 12/24/2022] Open
Abstract
Primary pancreatic paragangliomas are rare. They are mainly non-functional tumours that lack typical clinical manifestations. Definite diagnosis relies on histopathology and immunohistochemistry, and the main treatment is surgery. We report here a case of primary, non-functional, pancreatic paraganglioma in a 49-year-old woman. The tumour was approximately 5.0 × 3.2 ×4.7 cm in size and located in the pancreatic neck and body. We undertook 3D laparoscopic complete resection of the tumour. The patient developed a pancreatic fistula (biochemical leak) post-surgery, but she recovered and was discharged from hospital 11 days after surgery. We describe this case study and briefly summarize previous related reports.
Collapse
Affiliation(s)
- Zhanxue Zhao
- Medical College of Soochow University, Suzhou 215123, Jiangsu
Province, China,Department of General Surgery, Qinghai Provincial People's
Hospital, Xining 810007, Qinghai Province, China
| | - Yan Guo
- Department of Pathology, Qinghai Provincial People's Hospital,
Xining 810007, Qinghai Province, China
| | - Linxun Liu
- Department of General Surgery, Qinghai Provincial People's
Hospital, Xining 810007, Qinghai Province, China
| | - Linming Zhang
- Department of MRI, Qinghai Provincial People's Hospital, Xining
810007, Qinghai Province, China
| | - Shuai Li
- Department of Clinical Pharmacy, Affiliated Hospital of Qinghai
University, Xining 810007, Qinghai Province, China
| | - Jinyu Yang
- Department of General Surgery, Qinghai Provincial People's
Hospital, Xining 810007, Qinghai Province, China,Jinyu Yang, Department of General Surgery,
Qinghai Provincial People's Hospital, Xining, Qinghai 810007, China.
| |
Collapse
|
|
2
|
Kim M, Aploks K, Vargas-Pinto S, Dong X. RET T244I Germline Variant Mutation in a Patient with Pancreatic Paraganglioma and Primary Hyperparathyroidism. Int J Endocrinol Metab 2022; 20:e121056. [PMID: 36407031 PMCID: PMC9661538 DOI: 10.5812/ijem-121056] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/10/2021] [Revised: 05/14/2022] [Accepted: 05/16/2022] [Indexed: 11/16/2022] Open
Abstract
INTRODUCTION Paragangliomas are rare neuroendocrine tumors that arise from chromaffin cells. Often termed extra-adrenal pheochromocytomas, these tumors vary with regards to their functionality, location, and malignant potential. Mutations in the RET proto-oncogene are associated with multiple endocrine neoplasia syndrome type 2 (MEN-2) and paragangliomas. The phenotypes of the individual mutations are documented to help determine prognosis. CASE PRESENTATION We report a case of a 64-year-old man with a history of parathyroid adenoma who developed a pancreatic retroperitoneal paraganglioma. Despite having laboratory evidence of excess circulating catecholamines, the patient's only presenting symptom was hip pain. The patient underwent resection, and histologic findings were consistent with paraganglioma with lymph node metastasis. Genetic testing revealed a variant of uncertain significance within the RET gene [c.731C>T (p.T244I)]. CONCLUSIONS Paragangliomas are rare extra-adrenal neuroendocrine tumors that can be associated with germline mutations. Our patient was diagnosed with a pancreatic paraganglioma associated with a RET T244I mutation. Identifying patients with germline mutations is important for documenting phenotypic presentations of RET gene variants of uncertain significance, which will allow physicians to provide proper management and surveillance of paragangliomas and other associated tumors.
Collapse
Affiliation(s)
- Minha Kim
- Division of Surgical Oncology, Nuvance Health, Danbury, CT, USA
- Department of General Surgery, Danbury Hospital, Danbury, CT, USA
| | - Krist Aploks
- Division of Surgical Oncology, Nuvance Health, Danbury, CT, USA
- Department of General Surgery, Danbury Hospital, Danbury, CT, USA
| | - Susana Vargas-Pinto
- Division of Surgical Oncology, Nuvance Health, Danbury, CT, USA
- Department of General Surgery, Danbury Hospital, Danbury, CT, USA
| | - Xiang Dong
- Division of Surgical Oncology, Nuvance Health, Danbury, CT, USA
- Department of General Surgery, Danbury Hospital, Danbury, CT, USA
- Corresponding Author: Division of Surgical Oncology, Nuvance Health, Danbury, CT, USA.
| |
Collapse
|
|
3
|
Lanke G, Stewart JM, Lee JH. Pancreatic paraganglioma diagnosed by endoscopic ultrasound-guided fine needle aspiration: A case report and review of literature. World J Gastroenterol 2021; 27:6322-6331. [PMID: 34712035 PMCID: PMC8515802 DOI: 10.3748/wjg.v27.i37.6322] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/06/2021] [Revised: 06/28/2021] [Accepted: 09/03/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Pancreatic paragangliomas (PPGL) are rare benign neuroendocrine neoplasms but malignancy can occur. PPGL are often misdiagnosed as pancreatic neuroendocrine tumor or pancreatic adenocarcinoma.
CASE SUMMARY We reviewed 47 case reports of PPGL published in PubMed to date. Fifteen patients (15/47) with PPGL underwent endoscopic ultrasound-guided fine needle aspiration (EUS-FNA). Only six (6/15) were correctly diagnosed as PPGL. All patients with PPGL underwent surgical resection except three (one patient surgery was aborted because of hypertensive crisis, two patients had metastasis or involvement of major vessels). Our patient remained on close surveillance as she was asymptomatic.
CONCLUSION Accurate preoperative diagnosis of PPGL can be safely achieved by EUS-FNA with immunohistochemistry. Multidisciplinary team approach should be considered to bring the optimal results in the management of PPGL.
Collapse
Affiliation(s)
- Gandhi Lanke
- Department of Gastroenterology, Hepatology and Nutrition, MD Anderson Cancer Center, Lubbock, TX 79407, United States
| | - John M Stewart
- Pathology-lab Medicine Division, MD Anderson Cancer Center, Houston, TX 77030, United States
| | - Jeffrey H Lee
- Department of Gastroenterology, Hepatology and Nutrition, MD Anderson Cancer Center, Houston, TX 77030, United States
| |
Collapse
|
|
4
|
Jiang CN, Cheng X, Shan J, Yang M, Xiao YQ. Primary pancreatic paraganglioma harboring lymph node metastasis: A case report. World J Clin Cases 2021; 9:8071-8081. [PMID: 34621864 PMCID: PMC8462202 DOI: 10.12998/wjcc.v9.i27.8071] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/24/2021] [Revised: 05/19/2021] [Accepted: 08/05/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Primary pancreatic paragangliomas are extremely rare tumors. Limited by the diagnostic efficacy of histopathological examination, their malignant behavior is thought to be associated with local invasion or metastasis, with only four malignant cases reported in the literature to date. As pancreatic paragangliomas share similar imaging features with other types of pancreatic neuroendocrine neoplasms, they are difficult to diagnose accurately without the support of pathological evidence. As primary pancreatic paragangliomas are rare, especially those accompanied by lymph node metastasis, there is currently no consensus on treatment. Herein, we report a case of primary pancreatic paraganglioma with lymph node metastasis.
CASE SUMMARY A mass located in the pancreatic body was incidentally discovered on computed tomography in a 41-year-old Tibetan man. Distal pancreatectomy was subsequently performed and a 4.1 cm × 4.2 cm tumor was found embedded in the body of the pancreas during surgery. Histological examination confirmed the characteristics of paraganglioma in which the neoplastic chief cells were arranged in a classic Zellballen pattern under hematoxylin-eosin staining. Further, immunohistochemistry demonstrated that the sustentacular cells in the tumor tissue were positive for S-100 protein, and neoplastic cells and pancreatic draining lymph nodes were positive for chromogranin A and synaptophysin; thus, the presence of lymph node metastasis (two of the eight resected pancreatic draining lymph nodes) was also confirmed. A diagnosis of primary pancreatic paraganglioma with lymph node metastasis was finally established. The patient remained disease-free for 1 year after the surgery.
CONCLUSION A definite diagnosis of pancreatic paraganglioma mainly depends on postoperative histopathological and immunohistochemical examinations. Surgical resection may be the first treatment of choice for patients with primary pancreatic paraganglioma that has metastasized to the lymph nodes.
Collapse
Affiliation(s)
- Cui-Nan Jiang
- Department of Hepatopancreatobiliary Surgery, Chengdu Third People’s Hospital, Chengdu 610031, Sichuan Province, China
| | - Xiao Cheng
- Department of Pathology, Chengdu Third People’s Hospital, Chengdu 610031, Sichuan Province, China
| | - Jing Shan
- Department of Gastroenterology, Chengdu Third People’s Hospital, Chengdu 610031, Sichuan Province, China
| | - Mei Yang
- Department of Gastroenterology, Chengdu Third People’s Hospital, Chengdu 610031, Sichuan Province, China
| | - Yu-Qing Xiao
- Department of Hepatopancreatobiliary Surgery, Chengdu Third People’s Hospital, Chengdu 610031, Sichuan Province, China
| |
Collapse
|
|
5
|
Aaquist T, Dembic M, Thomassen M, de Stricker K, Bertelsen M, Christensen LG, Mortensen MB, Detlefsen S. Synchronous detection of pancreatic adenocarcinoma and paraganglioma in a Whipple resection specimen. Pathol Res Pract 2021; 226:153590. [PMID: 34454393 DOI: 10.1016/j.prp.2021.153590] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/18/2021] [Accepted: 08/14/2021] [Indexed: 01/17/2023]
Abstract
We report a case of a pancreatic ductal adenocarcinoma (PDAC) presenting synchronously with a paraganglioma (PGL) in a Whipple reaction specimen. The patient was a 72-year-old female with a history of breast and vulvar cancer. The simultaneous occurrence of two synchronous tumours in the pancreas was striking. Due to the presence of PGL and multiple meta- and synchronous tumours, the patient was referred to genetic counselling. Tumour tissue from the vulvar carcinoma, the PDAC and the PGL was analysed by targeted next-generation sequencing (NGS) of 161 cancer-related genes and by whole exome sequencing (WES). Peripheral blood was also examined by NGS and WES. These genetic analyses revealed germline polymorphisms in AXIN2 (NM_004655.4:c 0.2272 G>A; p.Ala758Thr), BRCA2 (NM_000059.3:c.9976 A>T; p.Lys3326Ter), NCOR1 (NM_006311.4:c 0.6544 G>A; p.Ala2182Thr) and SPTA1 (NM_003126.3:c 0.373 G>A; p.Ala125Thr) and somatic mutations of KRAS (NM_033360.3;c 0.35 G>A; p.Gly12Asp) and TP53 (NM_000546.5; c.602delT; p.Leu201CysfsTer46) in the PDAC and of TP53 (NM_000546.5; c 0.733 G>A; p.Gly245Ser) and TERT (NM_198253.2; c.-124 C>T; promotor region) in the vulvar carcinoma. Breast carcinoma tissue was not available for genetic analysis. The results of the genetic analyses did not explain the presence of multiple tumours in this patient, despite a slightly increased risk of breast cancer associated with the identified BRCA2 polymorphism. To our knowledge, this is the first report of the synchronous occurrence of PDAC and PGL. This case emphasizes the importance of thorough macroscopic examination of pancreatic resection specimens, as coexisting neoplasms may otherwise be missed.
Collapse
Affiliation(s)
- Trine Aaquist
- Department of Pathology, Odense University Hospital, Odense, Denmark; Odense Pancreas Center (OPAC), Odense University Hospital, Odense, Denmark; Department of Clinical Research, Faculty of Health Sciences, University of Southern Denmark, Odense, Denmark
| | - Maja Dembic
- Department of Clinical Genetics, Odense University Hospital, Odense, Denmark; Department of Mathematics and Computer Science (IMADA), University of Southern Denmark, Odense, Denmark
| | - Mads Thomassen
- Department of Clinical Research, Faculty of Health Sciences, University of Southern Denmark, Odense, Denmark; Department of Clinical Genetics, Odense University Hospital, Odense, Denmark
| | - Karin de Stricker
- Department of Pathology, Odense University Hospital, Odense, Denmark
| | - Mette Bertelsen
- Department of Clinical Genetics, Rigshospitalet, Copenhagen, Denmark
| | | | - Michael Bau Mortensen
- Odense Pancreas Center (OPAC), Odense University Hospital, Odense, Denmark; Department of Clinical Research, Faculty of Health Sciences, University of Southern Denmark, Odense, Denmark; Department of Surgery, Odense University Hospital, Odense, Denmark
| | - Sönke Detlefsen
- Department of Pathology, Odense University Hospital, Odense, Denmark; Odense Pancreas Center (OPAC), Odense University Hospital, Odense, Denmark; Department of Clinical Research, Faculty of Health Sciences, University of Southern Denmark, Odense, Denmark.
| |
Collapse
|
|
6
|
Wang W, Qin Y, Zhang H, Chen K, Liu Z, Zheng S. A rare case of retroperitoneal paraganglioma located in the neck of the pancreas: a case report and literature review. Gland Surg 2021. [PMID: 33968704 DOI: 10.21037/gs] [Citation(s) in RCA: 62] [Impact Index Per Article: 15.5] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
Abstract
Paraganglioma (PGL) is an uncommon tumor located in the head, neck and abdomen. The majority of the tumor is benign and the patient has no obvious clinical symptoms. However, PGL located in the pancreas is rather rare and tends to mimic Castleman's disease, pancreatic neuroendocrine tumors and pancreatic primary tumor. Herein, we reported a patient with PGL that occurred in the neck of the pancreas. A 75-year-old Chinese female presented to our hospital with a complaint of upper abdomen pain for two weeks and she had good past health. The laboratory findings and physical examination were all normal. Preoperative computed tomography (CT) and magnetic resonance imaging revealed a tumor located in the neck of the pancreas and a tentative diagnosis of Castleman's disease or PGL was made. We resected the tumor by laparoscopic surgery. Postoperative pathology and immunohistochemistry confirmed that the tumor was a PGL. The patient was recovered well after a postoperative follow-up of 6 months. PGL located in the neck of the pancreas is difficult to be diagnosed accurately and clinicians have difficulties in distinguishing PGL from Castleman's disease, pancreatic neuroendocrine tumors and pancreatic primary tumor. Fifteen cases were listed to show the characters of PGL located in the pancreas and we also presented the difference among PGL, Castleman's disease and pancreatic neuroendocrine tumor. We showed our experience of treating such a rare tumor hoping to help clinicians correctly diagnose and treat PGL.
Collapse
Affiliation(s)
- Wenchao Wang
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Yunsheng Qin
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Huifang Zhang
- Department of Pathology, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Kangjie Chen
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Zhengtao Liu
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Shusen Zheng
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| |
Collapse
|
|
7
|
Wang W, Qin Y, Zhang H, Chen K, Liu Z, Zheng S. A rare case of retroperitoneal paraganglioma located in the neck of the pancreas: a case report and literature review. Gland Surg 2021; 10:1523-1531. [PMID: 33968704 DOI: 10.21037/gs-20-758] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Paraganglioma (PGL) is an uncommon tumor located in the head, neck and abdomen. The majority of the tumor is benign and the patient has no obvious clinical symptoms. However, PGL located in the pancreas is rather rare and tends to mimic Castleman's disease, pancreatic neuroendocrine tumors and pancreatic primary tumor. Herein, we reported a patient with PGL that occurred in the neck of the pancreas. A 75-year-old Chinese female presented to our hospital with a complaint of upper abdomen pain for two weeks and she had good past health. The laboratory findings and physical examination were all normal. Preoperative computed tomography (CT) and magnetic resonance imaging revealed a tumor located in the neck of the pancreas and a tentative diagnosis of Castleman's disease or PGL was made. We resected the tumor by laparoscopic surgery. Postoperative pathology and immunohistochemistry confirmed that the tumor was a PGL. The patient was recovered well after a postoperative follow-up of 6 months. PGL located in the neck of the pancreas is difficult to be diagnosed accurately and clinicians have difficulties in distinguishing PGL from Castleman's disease, pancreatic neuroendocrine tumors and pancreatic primary tumor. Fifteen cases were listed to show the characters of PGL located in the pancreas and we also presented the difference among PGL, Castleman's disease and pancreatic neuroendocrine tumor. We showed our experience of treating such a rare tumor hoping to help clinicians correctly diagnose and treat PGL.
Collapse
Affiliation(s)
- Wenchao Wang
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Yunsheng Qin
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Huifang Zhang
- Department of Pathology, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Kangjie Chen
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Zhengtao Liu
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Shusen Zheng
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| |
Collapse
|
|
8
|
Konukiewitz B, von Hornstein M, Jesinghaus M, Steiger K, Weichert W, Detlefsen S, Kasajima A, Klöppel G. Pancreatic neuroendocrine tumors with somatostatin expression and paraganglioma-like features. Hum Pathol 2020; 102:79-87. [PMID: 32668277 DOI: 10.1016/j.humpath.2020.07.004] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/13/2020] [Revised: 07/06/2020] [Accepted: 07/07/2020] [Indexed: 02/07/2023]
Abstract
A small fraction of pancreatic neuroendocrine tumors (PanNETs) shows a solid, paraganglioma-like (PG-like) histology. We wanted to know whether these PanNETs have a special hormone expression and are related to paragangliomas (PGs)/pheochromocytomas (PCs). We screened a series of 48 surgically resected PanNETs for their histological growth patterns and their association with expression of islet hormones. The PanNETs were divided into PG-like and non-PG-like tumors and immunohistochemically monitored for the expression of islet hormones, cytokeratins, and S100. The results were correlated to histological pattern, lymph node status, and data in 28 PGs/PCs, including 2 PGs attached to the pancreas. All PanNETs, in contrast to PGs/PCs, were cytokeratin positive. A PG-like growth pattern was identified in 9 of 48 PanNETs and correlated with somatostatin expression. Only half of the non-PG-like PanNETs also contained somatostatin-positive cells. Eight of 28 PGs/PCs expressed somatostatin, mostly in individual cells. PG-like PanNETs and non-PG-like PanNETs infiltrated the adjacent pancreatic tissue, whereas 2 to the pancreas-associated PGs were well demarcated. Lymph node metastases were detected in 58%, 39%, 57%, and 53% of the somatostatin-producing, somatostatin-negative, PG-like, and non-PG-like PanNETs, respectively. PG-like PanNETs, in contrast to PG/PCs, are characterized by the expression of cytokeratin and somatostatin, the development of lymph node metastasis, and the infiltration into pancreatic parenchyma. Non-PG-like PanNETs may also express somatostatin and show lymph node metastases to the same extent. A literature review of cases reported as PG of the pancreas reveals that only a small fraction of these tumors probably represents true pancreatic PGs.
Collapse
Affiliation(s)
- Björn Konukiewitz
- Department of Pathology, Technical University of Munich, 81675, Munich, Germany.
| | | | - Moritz Jesinghaus
- Department of Pathology, Technical University of Munich, 81675, Munich, Germany.
| | - Katja Steiger
- Department of Pathology, Technical University of Munich, 81675, Munich, Germany.
| | - Wilko Weichert
- Department of Pathology, Technical University of Munich, 81675, Munich, Germany.
| | - Sönke Detlefsen
- Department of Pathology, Odense University Hospital, 5000, Odense, Denmark.
| | - Atsuko Kasajima
- Department of Pathology, Technical University of Munich, 81675, Munich, Germany.
| | - Günter Klöppel
- Department of Pathology, Technical University of Munich, 81675, Munich, Germany.
| |
Collapse
|
|
9
|
Mamilla D, Manukyan I, Fetsch PA, Pacak K, Miettinen M. Immunohistochemical distinction of paragangliomas from epithelial neuroendocrine tumors-gangliocytic duodenal and cauda equina paragangliomas align with epithelial neuroendocrine tumors. Hum Pathol 2020; 103:72-82. [PMID: 32668278 DOI: 10.1016/j.humpath.2020.07.010] [Citation(s) in RCA: 28] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/10/2020] [Revised: 07/06/2020] [Accepted: 07/07/2020] [Indexed: 01/08/2023]
Abstract
Distinction of paraganglioma (PGL) from epithelial neuroendocrine tumors (NETs) can be difficult as they can mimic each other by nested architecture and expression of neuroendocrine markers. In this study, we examined differential diagnostic markers in 262 PGLs (142 adrenal pheochromocytomas and 120 extra-adrenal PGLs), 9 duodenal gangliocytic PGLs and 3 cauda equina PGLs, and 286 NETs (81 GI, 78 pancreatic, 42 thoracic, 37 medullary thyroid carcinomas, and 48 high-grade NETs including 32 small cell carcinomas of lung). While keratin expression was nearly uniform in NETs with the exception of few tumors, extensive keratin expression was seen in only one PGL (<1%) and focal expression in 5% PGLs. GATA3 was present in >90% of PGLs but only in 2% of NETs, usually focally. Tyrosine hydroxylase (TH) was expressed in >90% of adrenal, abdominal, and thoracic PGLs but only in 37% of head and neck PGLs, reflecting their variable catecholamine synthesis. Focal or occasional extensive TH-expression was detected in 10% of NETs. CDX2 was a helpful discriminator seen in 28% of pancreatic and most GI NETs but in no PGLs. SOX10 detected sustentacular cells in 85% of PGLs and 7% of NETs, whereas GFAP detected sustentacular cells mainly in PGLs of neck and was absent in NETs. Duodenal gangliocytic PGLs (n = 9) and all cauda equina PGLs (n = 3) expressed keratins, lacked GATA3, showed no or minimal TH expression as some NETs, and contained SOX10 and S100 protein-positive spindle cells negative for GFAP. Ganglion-like epithelioid cells were keratin-positive and negative for TH and SOX10 differing from true ganglion cells. We conclude that duodenal gangliocytic and cauda equina PGLs have a NET-like immunoprofile and differ from ordinary PGLs. NETs can be distinguished from PGLs by their expression of keratins and general lack of GATA3, TH, and GFAP-positive sustentacular cells, and sometimes by expression of CDX2 or TTF1.
Collapse
Affiliation(s)
- Divya Mamilla
- Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child, Health and Human Development, National Institutes of Health, Bethesda, MD, 20892, USA
| | - Irena Manukyan
- National Cancer Institute, Laboratory of Pathology, Bethesda, MD, 20892, USA
| | - Patricia A Fetsch
- National Cancer Institute, Laboratory of Pathology, Bethesda, MD, 20892, USA
| | - Karel Pacak
- Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child, Health and Human Development, National Institutes of Health, Bethesda, MD, 20892, USA
| | - Markku Miettinen
- National Cancer Institute, Laboratory of Pathology, Bethesda, MD, 20892, USA.
| |
Collapse
|
|
10
|
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.
Collapse
|
|
11
|
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.
Collapse
|