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Miyazaki T, Takemura N, Mihara F, Ito K, Miyazaki H, Igari T, Kokudo N. Surgical treatment for a rare case of duodenal gangliocytic paraganglioma accompanied with lymph node metastasis. Clin J Gastroenterol 2022; 15:712-716. [PMID: 35513688 DOI: 10.1007/s12328-022-01636-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/20/2021] [Accepted: 04/15/2022] [Indexed: 10/18/2022]
Abstract
We report a rare case of duodenal gangliocytic paraganglioma accompanied with lymph node metastasis. An asymptomatic 58-year-old woman was admitted for the evaluation of abnormal liver enzyme test results. Abdominal computed tomography and upper gastrointestinal endoscopy revealed a 27-mm pedunculated tumor of the papilla of Vater. Adenocarcinoma of the papilla with muscularis propria invasion was suspected; therefore, pancreaticoduodenectomy with lymph node dissection was performed. There were no perioperative complications. A definitive diagnosis of gangliocytic paraganglioma of the papilla of Vater with lymph node metastases was confirmed by pathological examinations, which is a rare condition. The patient was discharged on her 16th postoperative day, and no recurrence or metastases were identified during the 18-month follow-up period. We herein discuss the surgical treatment for this rare case and present a review of related literature.
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Affiliation(s)
- Toko Miyazaki
- Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Nobuyuki Takemura
- Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan.
| | - Fuminori Mihara
- Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Kyoji Ito
- Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Hideki Miyazaki
- Pathology Division of Clinical Laboratory, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Toru Igari
- Pathology Division of Clinical Laboratory, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Norihiro Kokudo
- Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
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Choi H, Choi JW, Ryu DH, Park S, Kim MJ, Yoo KC, Woo CG. Ampullary gangliocytic paraganglioma with lymph node metastasis: A case report with literature review. Medicine (Baltimore) 2022; 101:e29138. [PMID: 35475800 PMCID: PMC9276348 DOI: 10.1097/md.0000000000029138] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/04/2022] [Accepted: 03/04/2022] [Indexed: 01/04/2023] Open
Abstract
RATIONALE Gangliocytic paraganglioma (GP) is a rare tumor that mostly develops in the duodenum and is composed of the following 3 cell types: epithelioid endocrine, spindle-like, and ganglion-like cells. It manifests as symptoms such as abdominal pain, gastrointestinal bleeding, and weight loss; however, occasionally, it is incidentally detected on endoscopic or radiologic examinations. Although GP is usually benign, it can metastasize to the lymph nodes, and distant metastases have been reported in some cases. PATIENT CONCERNS A 46-year-old woman presented with anemia on health surveillance examination. She had no other specific symptoms, and her physical examination did not reveal any abnormal finding. DIAGNOSIS Endoscopic ultrasound-guided fine-needle aspiration biopsy was performed, and the endoscopist obtained samples from the inner side of the ampullary mass. Pathological examination suggested GP or a neuroendocrine tumor. INTERVENTIONS Initially, we planned transduodenal ampullectomy with lymph node excision. However, there was severe fibrosis around the duodenum, and an examination of a frozen biopsy sample from the periduodenal lymph node showed atypical cells in the lymph node. Therefore, we performed pylorus-preserving pancreaticoduodenectomy with lymph node dissection. OUTCOMES The final pathological diagnosis was GP located in the ampulla of Vater. The GP showed lymphovascular and perineural invasion and invaded the duodenal wall. Furthermore, 4 out of 18 harvested lymph nodes showed metastasis. LESSONS We described a case of GP confined to the ampulla with regional lymph node metastasis and reviewed published literature on ampullary GP with lymph node metastasis.
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Affiliation(s)
- Hanlim Choi
- Department of Surgery, Chungbuk National University College of Medicine, Cheongju, Korea
- Department of Surgery, Chungbuk National University Hospital, Cheongju, Korea
| | - Jae-Woon Choi
- Department of Surgery, Chungbuk National University College of Medicine, Cheongju, Korea
- Department of Surgery, Chungbuk National University Hospital, Cheongju, Korea
| | - Dong Hee Ryu
- Department of Surgery, Chungbuk National University College of Medicine, Cheongju, Korea
- Department of Surgery, Chungbuk National University Hospital, Cheongju, Korea
| | - Sungmin Park
- Department of Surgery, Chungbuk National University College of Medicine, Cheongju, Korea
- Department of Surgery, Chungbuk National University Hospital, Cheongju, Korea
| | - Myung Jo Kim
- Department of Surgery, Chungbuk National University College of Medicine, Cheongju, Korea
- Department of Surgery, Chungbuk National University Hospital, Cheongju, Korea
| | - Kwon Cheol Yoo
- Department of Surgery, Chungbuk National University College of Medicine, Cheongju, Korea
- Department of Surgery, Chungbuk National University Hospital, Cheongju, Korea
| | - Chang Gok Woo
- Department of Pathology, Chungbuk National University Hospital, Cheongju, Korea
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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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Sekine M, Miyatani H, Matsumoto K, Kashima H, Koito Y, Miura T, Takahashi Y, Tsuboi R, Ishii T, Fujiwara J, Uehara T, Urayoshi S, Yuhashi K, Asano T, Sagihara N, Matsumoto S, Mashima H. Gangliocytic Paraganglioma with Carcinoma of the Ampulla of Vater. Intern Med 2018; 57:2663-2668. [PMID: 29780117 PMCID: PMC6191592 DOI: 10.2169/internalmedicine.0464-17] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/15/2017] [Accepted: 01/10/2018] [Indexed: 11/06/2022] Open
Abstract
The patient was a "73" -year-old woman who visited our hospital with the chief complaint of weight loss. Upper gastrointestinal endoscopy revealed an enlarged ampulla of Vater, and a biopsy led to a diagnosis of Group "4" gastric carcinoma; suspicious of adenocarcinoma. There were no findings suggesting invasion into the muscle layer of duodenum, despite tumor mass formation being observed in the sphincter of Oddi. We performed endoscopic papillectomy for both diagnostic and therapeutic purposes. Pathologically, a well-differentiated adenocarcinoma existed in the superficial layer of the mucous membrane of the papilla of Vater, and gangliocytic paraganglioma was present in the deep portion. The resected margins of both lesions were negative.
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Affiliation(s)
- Masanari Sekine
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Hiroyuki Miyatani
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Keita Matsumoto
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Hitomi Kashima
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Yudai Koito
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Takaya Miura
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Yuko Takahashi
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Rumiko Tsuboi
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Takehiro Ishii
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Junichi Fujiwara
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Takeshi Uehara
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Shunsuke Urayoshi
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Kazuhito Yuhashi
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Takeharu Asano
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Noriyoshi Sagihara
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Satohiro Matsumoto
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
| | - Hirosato Mashima
- Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan
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Okubo Y, Yoshioka E, Suzuki M, Washimi K, Kawachi K, Kameda Y, Yokose T. Diagnosis, Pathological Findings, and Clinical Management of Gangliocytic Paraganglioma: A Systematic Review. Front Oncol 2018; 8:291. [PMID: 30101131 PMCID: PMC6072869 DOI: 10.3389/fonc.2018.00291] [Citation(s) in RCA: 25] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/24/2018] [Accepted: 07/11/2018] [Indexed: 12/11/2022] Open
Abstract
Background: Although gangliocytic paraganglioma (GP) is considered a rare benign neuroendocrine tumor, cases of mortality have been reported. Occasionally, GP is misdiagnosed as neuroendocrine tumor G1, which is associated with a poorer prognosis than GP. To avoid such misdiagnoses, it is important to understand the clinicopathological characteristics of GP. Thus, herein, we discuss the current literature on the clinicopathological characteristics of GP. Methods: We conducted a systematic review in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. PubMed and Japana Centra Revuo Medicina searches were used to identify papers describing GP. Inclusion criteria included confirmation of epithelioid, spindle-shaped, and ganglion-like cells in the main article and/or figures and whether the paper was cited in other studies of GP. Data were collected on age, sex, site of the primary lesion, tumor size, treatment, prognosis, lymph node metastasis (LNM), depth of tumor invasion, rate of preoperative diagnosis, and clinical symptoms. Results: In total, 162 papers containing 263 cases of GP met the criteria. The mean age at diagnosis was 53.5 years. The male-to-female ratio was 157:104. The mean tumor size was 25.7 mm. The predominant site of the primary tumor was the duodenum (89.7%). The most common clinical sign of GP was gastrointestinal bleeding (47.9%). Other signs and symptoms of GP included abdominal pain (44.7%), anemia (20.3%), incidental findings (12.9%), nausea (6.9%), weight loss (5.5%), general fatigue (5.1%), jaundice (4.6%), and incidental autopsy findings (5.1%). LNM was observed in 11.4% of patients. Liver metastasis was observed in 1.1% of patients. Depth of tumor invasion (penetrating beyond the submucosal layer or sphincter of Oddi) was by far the most significant risk factor for LNM in patients with GP. This suggests, along with histological heterogeneity, that GP may have hamartomatous characteristics. Furthermore, immunohistochemical expression of progesterone receptor and pancreatic polypeptide were useful in distinguishing between GP and neuroendocrine tumor G1, even in small biopsy specimens. Conclusions: We reveal the clinicopathological characteristics of GP, including risk factors for LNM, differential diagnostic approaches, and improvements in the clinical management of this tumor.In addition, GP may have hamartomatous characteristics.
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Affiliation(s)
- Yoichiro Okubo
- Department of Pathology, Kanagawa Cancer Center, Kanagawa, Japan
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Pancreatic gangliocytic paraganglioma harboring lymph node metastasis: a case report and literature review. Diagn Pathol 2017; 12:57. [PMID: 28764742 PMCID: PMC5540335 DOI: 10.1186/s13000-017-0648-x] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/26/2017] [Accepted: 07/20/2017] [Indexed: 12/20/2022] Open
Abstract
Background Gangliocytic paraganglioma (GP) is a rare neuroendocrine neoplasm, which occurs mostly in the periampullary portion of the duodenum; the majority of the reported cases of duodenal GP has been of benign nature with a low incidence of regional lymph node metastasis. GP arising from the pancreas is extremely rare. To date, only three cases have been reported and its clinical characteristics are largely unknown. Case presentation A nodule located in the pancreatic head was incidentally detected in an asymptomatic 68-year-old woman. Computed tomography revealed 18-, 8-, and 12-mm masses in the pancreatic head, the pancreatic tail, and the left adrenal gland, respectively. Subsequent genetic examination revealed an absence of mutations in the MEN1 and VHL genes. Macroscopically, the tumor located in the pancreatic head was 22 mm in size and displayed an ill-circumscribed margin along with yellowish-white color. Microscopically, it was composed of three cell components: epithelioid cells, ganglion-like cells, and spindle cells, which led to the diagnosis of GP. The tumor was accompanied by a peripancreatic lymph node metastasis. The tumor in the pancreatic tail was histologically classified as a neuroendocrine tumor (NET) G1 (grade 1, WHO 2010), whereas the tumor in the left adrenal gland was identified as an adrenocortical adenoma. The patient was disease-free at the 12-month follow-up examination. Conclusions Pancreatic GP is associated with a higher incidence of metastasis and larger tumor size than duodenal GPs, suggesting that the primary organ of GP is an important prognostic factor.
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Cathcart SJ, Sasson AR, Kozel JA, Oliveto JM, Ly QP. Duodenal gangliocytic paraganglioma with lymph node metastases: A case report and comparative review of 31 cases. World J Clin Cases 2017; 5:222-233. [PMID: 28685135 PMCID: PMC5480070 DOI: 10.12998/wjcc.v5.i6.222] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/05/2016] [Revised: 02/14/2017] [Accepted: 04/24/2017] [Indexed: 02/05/2023] Open
Abstract
Gangliocytic paraganglioma (GP) is a rare tumor of uncertain origin most often located in the second portion of the duodenum. It is composed of three cellular components: Epithelioid endocrine cells, spindle-like/sustentacular cells, and ganglion-like cells. While this tumor most often behaves in a benign manner, cases with metastasis are reported. We describe the case of a 62-year-old male with a periampullary GP with metastases to two regional lymph nodes who was successfully treated with pancreaticoduodenectomy. Using PubMed, EMBASE, EBSCOhost MEDLINE and CINAHL, and Google Scholar, we searched the literature for cases of GP with regional lymph node metastasis and evaluated the varying presentations, diagnostic workup, and disease management of identified cases. Thirty-one cases of GP with metastasis were compiled (30 with at least lymph node metastases and one with only distant metastasis to bone), with age at diagnosis ranging from 16 to 74 years. Ratio of males to females was 19:12. The most common presenting symptoms were abdominal pain (55%) and gastrointestinal bleeding or sequelae (42%). Twenty-five patients underwent pancreaticoduodenectomy. Five patients were treated with local resection alone. One patient died secondary to metastatic disease, and one died secondary to perioperative decompensation. The remainder did well, with no evidence of disease at follow-up from the most recent procedure (except two in which residual disease was deliberately left behind). Of the 26 cases with sufficient histological description, 16 described a primary tumor that infiltrated deep to the submucosa, and 3 described lymphovascular invasion. Of the specific immunohistochemistry staining patterns studied, synaptophysin (SYN) stained all epithelioid endocrine cells (18/18). Neuron specific enolase (NSE) and SYN stained most ganglion-like cells (7/8 and 13/18 respectively), and S-100 stained all spindle-like/sustentacular cells (21/21). Our literature review of published cases of GP with lymph node metastasis underscores the excellent prognosis of GP regardless of specific treatment modality. We question the necessity of aggressive surgical intervention in select patients, and argue that local resection of the mass and metastasis may be adequate. We also emphasize the importance of pre-surgical assessment with imaging studies, as well as post-surgical follow-up surveillance for disease recurrence.
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Papaconstantinou D, Machairas N, Damaskou V, Zavras N, Kontopoulou C, Machairas A. Duodenal gangliocytic paraganglioma, successfully treated by local surgical excision-a case report. Int J Surg Case Rep 2017; 32:5-8. [PMID: 28213067 PMCID: PMC5312508 DOI: 10.1016/j.ijscr.2017.01.046] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/08/2016] [Revised: 01/09/2017] [Accepted: 01/17/2017] [Indexed: 12/14/2022] Open
Abstract
Duodenal gangliocytic paraganglioma is a rare tumor usually with benign behavior. Resection of the tumor, either endoscopically or surgically is the treatment of choice. Complete removal of the mass offers cure and long-term survival. Introduction Duodenal gangliocytic paragangliomas are rare neoplasms often arising in proximity to the major duodenal papilla of Vater. These neoplasms are considered to have a benign behavior with lymph node metastases being a rare phenomenon and distant metastatic disease even more so. Resection of the tumor is the only definitive therapy. Presentation of case A 67 year old male presented to a referring hospital with symptoms of fatigue and malaise. Evaluation with CT imaging revealed a 3.1 cm intraluminal mass situated grossly at the junction of the third with the fourth portion of the duodenum. The tumor was found to be situated near the ampulla of Vater and was excised through a longitudinal duodenotomy followed by myotomy of the sphincter of Oddi. Discussion Complete resection of duodenal gangliocytic paragangliomas by surgical or endoscopic means is the only potential cure. Endoscopic removal is the first option and is both safe and adequate. Ηowever, localized excision may be utilized instead in those cases in which endoscopic removal is not possible or cannot achieve negative margins. Recurrent disease after complete resection is unlikely. Conclusion Cases of duodenal gangliocytic paragangliomas are best managed with endoscopic resection. However, local surgical excision remains as a second-choice procedure. Adjuvant chemotherapy and radiotherapy are unnecessary after complete excision.
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Affiliation(s)
- Dimetrios Papaconstantinou
- 3rd Department of Surgery, University General Hospital "ATTIKON", Medical School, National and Kapodistrian University of Athens, Greece
| | - Nikolaos Machairas
- 2nd Department of Surgery, General Hospital "Laiko", Medical School, National and Kapodistrian University of Athens, Greece
| | - Vasileia Damaskou
- 2nd Department of Pathology, University General Hospital "ATTIKON", Medical School, National and Kapodistrian University of Athens, Greece
| | - Nikolaos Zavras
- Pediatric Surgery Department, University General Hospital "ATTIKON", Medical School, National and Kapodistrian University of Athens, Greece.
| | - Christine Kontopoulou
- Second Department of Radiology, University General Hospital "ATTIKON", Medical School, National and Kapodistrian University of Athens, Greece
| | - Anastasios Machairas
- 3rd Department of Surgery, University General Hospital "ATTIKON", Medical School, National and Kapodistrian University of Athens, Greece
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Sherazadishvili A, Leslie K. Duodenal gangliocytic paraganglioma with lymph node metastasis: A systematic case review. INTERNATIONAL JOURNAL OF HEPATOBILIARY AND PANCREATIC DISEASES 2017. [DOI: 10.5348/ijhpd-2017-74-ra-10] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/12/2022]
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Park HK, Han HS. Duodenal Gangliocytic Paraganglioma With Lymph Node Metastasis. Arch Pathol Lab Med 2016; 140:94-8. [PMID: 26717062 DOI: 10.5858/arpa.2014-0456-rs] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
Gangliocytic paraganglioma is a rare tumor that occurs most commonly in the second portion of the duodenum. It is characterized by its triphasic cellular differentiation: epithelioid neuroendocrine cells, spindle cells with Schwann cell differentiation, and ganglion cells. Most gangliocytic paragangliomas are considered benign and are amenable to local excision. However, to our knowledge, 23 cases with lymph node metastasis have been reported, 1 case of bone metastasis, and 2 cases of liver metastases. Predictive factors that have been suggested for lymph node metastasis include size (larger than 2 cm), young age, and tumors exceeding the submucosal layer. Our objective was to review the clinical features, the histopathologic characteristics, and the differential diagnosis of gangliocytic paraganglioma and to discuss the value of the predictive factors for lymph node metastasis.
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Affiliation(s)
- Hyung Kyu Park
- From the Department of Pathology, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul, Republic of Korea
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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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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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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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15
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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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16
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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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17
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Ghassemi KA, Cortina G, Reber HA, Farrell JJ. Complete Resection of Ampullary Paragangliomas Confined to the Submucosa on Endoscopic Ultrasound May Be Best Achieved by Radical Surgical Resection. Case Rep Gastroenterol 2009; 3:169-174. [PMID: 21103270 PMCID: PMC2988952 DOI: 10.1159/000210438] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/12/2023] Open
Abstract
Paragangliomas of the gastrointestinal tract generally are benign tumors usually found in the second portion of the duodenum. We present a case of paraganglioma of the ampulla of Vater confined to the submucosa on endoscopic ultrasound examination. This was initially treated by endoscopic resection, followed by pancreaticoduodenectomy after local resection margins were positive. Histopathology showed a well-differentiated ampullary paraganglioma confined to the submucosa, but with involvement of one regional lymph node. Only 25 prior cases of paraganglioma at the ampulla of Vater have been reported, and nine of these have demonstrated local or distant metastases. Because of their malignant potential, ampullary paragangliomas should be treated with radical resection if the goal is to achieve complete resection, even if preoperative imaging shows local confinement.
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Affiliation(s)
- Kevin A Ghassemi
- Department of Medicine, UCLA School of Medicine, Los Angeles, Calif., USA
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18
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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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19
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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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20
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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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21
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Bucher P, Mathe Z, Bühler L, Chilcott M, Gervaz P, Egger JF, Morel P. Paraganglioma of the ampulla of Vater: a potentially malignant neoplasm. Scand J Gastroenterol 2004; 39:291-5. [PMID: 15074402 DOI: 10.1080/00365520310007503] [Citation(s) in RCA: 38] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
Paragangliomas are rare tumours originating from neuroectodermic remnants and are usually considered as benign. We present two cases of paraganglioma of the ampulla of Vater that were treated surgically by pancreaticoduodenectomy. In one case, histopathology revealed malignant characteristics of the tumour with invasion of the pancreas and simultaneous duodenal lymph-node metastases. Both patients had a favourable outcome without disease recurrence at 40 and 44 months postoperatively. Only 21 cases of ampullary paraganglioma have been reported in the literature, 7 of them with malignant characteristics. In conclusion, paragangliomas of the ampulla of Vater have malignant potential. Surgical therapy of these tumours should not be limited to local resection, as disease recurrence and lymph node involvement have been reported. We propose that paragangliomas of the ampulla of Vater should be operated by cephalic pancreaticoduodenectomy, which allows long-term and disease-free survival.
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Affiliation(s)
- P Bucher
- Clinic of Visceral and Transplantation Surgery, Dept. of Surgery, University Hospital of Geneva, Geneva, Switzerland.
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