Neurofibromatosis-1 and Ampullary Gangliocytic Paraganglioma Causing Biliary and Pancreatic Obstruction

Castoldi, Laura; De, P.; Marini, A.; Ferrero, Stefano; Luca, Valeria Midolo De; Tiberio, G

doi:10.1385/ijgc:29:2:093

Cited by 23 publications

(8 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Major clinical symptoms of the disease include abdominal pain, nausea, vomiting, and gastrointestinal bleeding. Biliary obstruction has rarely been reported as a devastating complication [ 6 , 7 ]. Histopathological diagnosis of the disease should require a confirmation of three identical components comprising epithelioid cells, spindle-shaped cells, and ganglion-like cells [ 1 , 6 - 8 ].…”

Section: Discussionmentioning

confidence: 99%

Duodenal gangliocytic paraganglioma showing lymph node metastasis: A rare case report

et al. 2010

View full text Add to dashboard Cite

We describe a case of duodenal gangliocytic paraganglioma showing lymph node metastasis. A 61-year-old Japanese man underwent pylorus preserving pancreaticoduodenectomy to remove a tumor at the papilla of Vater. The section of the tumor extending from the mucosa to submucosa of the duodenum was sharply demarcated, solid, and white-yellowish. Neither necrosis nor hemorrhage was present. Histological examination confirmed the immunohistochemical identification of three components comprising epithelioid cells, spindle-shaped cells, and ganglion-like cells. Epithelioid cells showed positive reactivity for synaptophysin, somatostatin, and CD56. In contrast, spindle-shaped cells showed positive reactivity for S-100 protein, but not for synaptophysin, somatostatin or CD56. Furthermore, we found lymph node metastasis despite lack of bcl-2 and p53 expression. In addition to the rarity of the tumor, we are describing here the present case suggests the malignant potency of the tumor despite lack of acceptable prognostic indicators for neuroendocrine tumor.

show abstract

Section: Discussionmentioning

confidence: 99%

Duodenal gangliocytic paraganglioma showing lymph node metastasis: A rare case report

et al. 2010

View full text Add to dashboard Cite

show abstract

“…In NF1, the SOM-NETs typically occur in the duodenum (Mao et al 1995 Castoldi et al 2001, Cappelli et al 2004, Fendrich et al 2004. In a review, the reported occurrence of duodenal and pancreatic SOM-NETs in NF1 was 43.2 and 20.8% respectively (Soga & Yakuwa 1999).…”

Section: Endocrine-related Cancermentioning

confidence: 99%

Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity

Garbrecht¹,

Anlauf²,

Schmitt³

et al. 2008

Endocrine Related Cancer

166

128

View full text Add to dashboard Cite

Somatostatin-producing neuroendocrine tumors (SOM-NETs) of the duodenum and pancreas appear to be heterogeneous. To determine their clinicopathological profiles, respective data were analyzed on a series of 82 duodenal and 541 pancreatic NETs. In addition, the clinical records of 821 patients with duodenal or pancreatic NETs were reviewed for evidence of a somatostatinoma syndrome. Predominant or exclusive expression of somatostatin was found in 21 (26%) duodenal and 21 (4%) pancreatic NETs. They were classified as sporadic (nZ31) or neurofibromatosis type 1 (NF1)-associated duodenal NETs (nZ3), gangliocytic paragangliomas (GCPGs; nZ6), or poorly differentiated neuroendocrine carcinomas (pdNECs; nZ2). In addition, five duodenal and four pancreatic SOM-NETs were found in five patients with multiple endocrine neoplasia type 1 (MEN1). Metastases occurred in 13 (43%) patients with sporadic or NF1-associated SOM-NETs, but in none of the duodenal or pancreatic MEN1-associated SOM-NETs or GCPGs. Sporadic advanced (stage IV) SOM-NETs were more commonly detected in the pancreas than in the duodenum. None of the patients (including the 821 patients for whom only the clinical records were reviewed) fulfilled the criteria of a somatostatinoma syndrome. Our data show that somatostatin expression is not only seen in sporadic NETs but may also occur in GCPGs, pdNECs, and hereditary NETs. Surgical treatment is effective in most duodenal and many pancreatic SOM-NETs. MEN1-associated SOM-NETs and GCPGs follow a benign course, while somatostatin-producing pdNECs are aggressive neoplasms. The occurrence of the so-called somatostatinoma syndrome appears to be extremely uncommon.

show abstract

“…It is seen more frequently in men than in women, with a sex ratio of 1 : 6, and the average age of diagnosis is 54 (17-83 years) [3,6,7]. Although the most frequently reported type of occurrences are sporadic, four reported cases were associated with type I neurofibromatosis [8,9]. GP is a submucosal tumor that is usually located in the second portion of the duodenum, exactly in the periampullar region (89% of cases), as in this case.…”

Section: Discussionmentioning

confidence: 99%

“…In 95% of the cases, the tumor is symptomatic, with 60% of patients presenting with melena [18][19][20] or abdominal pain [21]. Obstruction of the upper digestive tract [6] and obstructive jaundice are uncommon [8,18,22]. Endoscopic examination shows a single pedunculated or a sessile polypoid mass with a smooth or an ulcerated surface [3,18].…”

Section: Discussionmentioning

confidence: 99%

Duodenal gangliocytic paraganglioma with lymph node metastasis and an 8-year follow-up

Barret¹,

Rahmi²,

Huyen³

et al. 2012

European Journal of Gastroenterology & Hepatology

View full text Add to dashboard Cite

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.

show abstract

Neurofibromatosis-1 and Ampullary Gangliocytic Paraganglioma Causing Biliary and Pancreatic Obstruction

Cited by 23 publications

References 17 publications

Duodenal gangliocytic paraganglioma showing lymph node metastasis: A rare case report

Duodenal gangliocytic paraganglioma showing lymph node metastasis: A rare case report

Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity

Duodenal gangliocytic paraganglioma with lymph node metastasis and an 8-year follow-up

Contact Info

Product

Resources

About