Panniculite nodulaire et tumeur carcinoïde intracanalaire d’un pancréas divisum

Outtas, O.; Barthet, Marc; Troyer, Jérémy De; Franck, F.; García, Stéphane

doi:10.1016/s0151-9638(04)93641-1

Cited by 24 publications

(9 citation statements)

References 12 publications

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“…In our case, although the size of the carcinoid tumor was small and the tumor was hormonally inactive, the concomitant pancreas divisum led to an early diagnosis because obstruction of the minor papilla due to the carcinoid tumor caused recurrent abdominal pain and resulted in dilatation of the main pancreatic duct. Of the eight reported cases of carcinoid tumors involving the minor papilla in the literature, five cases have been associated with pancreas divisum and presented with recurrent abdominal pain or pancreatitis [3-7]. …”

Section: Discussionmentioning

confidence: 99%

“…Tumors of the minor papilla of the duodenum are very rare; the majority of tumors of the minor papilla of the duodenum are neuroendocrine tumors (NETs), such as somatostatinomas and carcinoid tumors [1-7]. Carcinoid tumors arise from enterochromaffin cells, and gastrointestinal carcinoids are usually located in the appendix, ileum, stomach, and rectum.…”

Section: Introductionmentioning

confidence: 99%

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Carcinoid tumor of the minor papilla in complete pancreas divisum presenting as recurrent abdominal pain

Kim

2010

BMC Gastroenterol

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BackgroundTumors of the minor papilla of the duodenum are extremely rare, and they are mostly neuroendocrine tumors, such as somatostatinomas and carcinoid tumors. However, true incidence of carcinoid tumors in minor papilla might be much higher, because patients with minor papillary tumors usually remain asymptomatic. We report a very unusual case of carcinoid tumor in a patient with complete pancreas divisum with a review of the literature.Case presentationA 56-year-old female patient was referred for evaluation of pancreatic duct dilatation noted on abdominal ultrasonography and computerized tomography. She complained of intermittent epigastric pain for 6 months. A MRCP and ERCP revealed complete pancreas divisum with dilatation of the main pancreatic duct. On duodenoscopy, a small, yellows, subepithelial nodule was visualized at the minor papilla; biopsy of this lesion revealed a carcinoid tumor. She underwent a pylorus-preserving pancreaticoduodenectomy. The histologic evaluation showed a single nodule, 1 cm in diameter, in the submucosa with duodenal and vascular invasion and metastasis to the regional lymph nodes.ConclusionAlthough the size of the carcinoid tumor was small and the tumor was hormonally inactive, the concomitant pancreas divisum led to an early diagnosis, the tumor had aggressive behavior. Carcinoid tumors of the minor papilla should be included in the differential diagnosis of recurrent abdominal pain or pancreatitis of unknown cause.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Carcinoid tumor of the minor papilla in complete pancreas divisum presenting as recurrent abdominal pain

Kim

2010

BMC Gastroenterol

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“…Pancreatic panniculitis has a high mortality rate unless the underlying pancreatic abnormality is reversed [8]. Several case studies have shown resolution of panniculitis in patients after surgical intervention or medical management of their underlying pancreatic disease [3,13]. Prognosis is worse in cases of pancreatic panniculitis associated with pancreatic tumour [14].…”

Section: Discussionmentioning

confidence: 99%

Panniculitis in a patient presenting with a pancreatic tumour and polyarthritis: a case report

Chee

2009

J Med Case Rep

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IntroductionPanniculitis is a rare manifestation of pancreatic disease. Rarer still is the association of panniculitis, pancreatitic disease and polyarthritis. A literature search revealed less than five cases of pancreatic panniculitis associated with pancreatic tumour and polyarthritis.Case presentationAn 84-year-old Caucasian man presented with epigastric pain, weight loss, polyarthritis and multiple discharging nodules. A computed tomography scan revealed a mass in the head of the pancreas. Histology of the cutaneous lesions confirmed the diagnosis of pancreatic panniculitis.ConclusionPancreatic panniculitis can clinically present in many ways to clinicians across a broad scope of specialties. Knowledge and understanding of the association between panniculitis and polyarthritis with pancreatic disease may aid rapid diagnosis and management.

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“…The anomaly is caused by absent or incomplete fusion of the ventral (main or Wirsung) and dorsal (marginal or Santorini) ducts (Kanth et al, 2014) and results in coexistence of two ampullary systems: the ventral duct drains the pancreatic head through the major ampulla, while the dorsal duct drains the pancreatic body and tail through the minor ampulla (Ferri et al, 2019). Ampullary neoplasms are rare, comprising 7% of all periampullary malignancies (Adsay et al, 2012), and association of theses tumors with pancreas divisum is considered an episodic event (Singh et al, 2003;Outtas et al, 2004;Kim et al, 2010). Their occurrence has been reported in families with hereditary cancer syndromes, such as Familial Adenomatous Polyposis (Pérez-Cuadrado-Robles et al, 2019) and Neurofibromatosis type 1 (NF1) (Tewari et al, 2014).…”

Section: Introductionmentioning

confidence: 99%

Synchronous Periampullary Tumors in a Patient With Pancreas Divisum and Neurofibromatosis Type 1

et al. 2020

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Introduction: In this study, we describe for the first time a Neurofibromatosis type 1 patient with pancreas divisum, multiple periampullary tumors and germline pathogenic variants in NF1 and CFTR genes.Case report: A 62-year-old female NF1 patient presented with weakness, choluria, nausea, and diffuse abdominal pain to an emergency room service. Magnetic resonance imaging revealed an abdominal mass involving the periampullary region and pancreas divisum. After surgical resection, three synchronous neoplasms were detected including two ampullary tumors (adenocarcinoma of the major ampulla and a neuroendocrine tumor of the minor ampulla) and a gastrointestinal stromal tumor (GIST). Germline multigene panel testing (MGPT) identified two pathogenic heterozygous germline variants: NF1 c.838del and CFTR c.1210-34TG[12]T [5].Conclusion: This is the first report of a Neurofibromatosis type 1 patient with pancreas divisum and multiple periampullary tumors harboring pathogenic germline variants in NF1 and CFTR genes. The identification of two germline variants and a developmental anomaly in this patient may explain the unusual and more severe findings and underscores the importance of comprehensive molecular analyses in patients with complex phenotypes.

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Panniculite nodulaire et tumeur carcinoïde intracanalaire d’un pancréas divisum

Cited by 24 publications

References 12 publications

Carcinoid tumor of the minor papilla in complete pancreas divisum presenting as recurrent abdominal pain

Carcinoid tumor of the minor papilla in complete pancreas divisum presenting as recurrent abdominal pain

Panniculitis in a patient presenting with a pancreatic tumour and polyarthritis: a case report

Synchronous Periampullary Tumors in a Patient With Pancreas Divisum and Neurofibromatosis Type 1

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