Pancreatic Neuroendocrine Tumor in the Setting of Dorsal Agenesis of the Pancreas

Nassif, Samih; Ponchiardi, Cecilia; Sachs, Teviah E.

doi:10.1155/2016/3801962

Cited by 11 publications

(10 citation statements)

References 13 publications

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“…The mechanism of tumorigenesis in ADP patients is unclear, but chronic pancreatitis caused by the ADP itself could be one of the risk factors for neoplasia [2]. Thus, the association of tumorigenesis with ADP indicates that every ADP patient should be observed with a focus on the early detection of pancreatic neoplasia [4,5,22,23]. According to literature reports and our own study, the clinical features of SPTs in patients with ADP are similar to those observed in patients without ADP [12,13,21,24,25].…”

Section: Discussionsupporting

confidence: 54%

Pancreatic Head Sparing Surgery for Solid Pseudopapillary Tumor in Patients with Agenesis of the Dorsal Pancreas

Shyr

Chen

et al. 2021

Preprint

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Background: This study aimed to clarify the feasibility and justification of pancreatic head sparing (PHS) enucleation for patients with agenesis of the dorsal pancreas (ADP) associated with a solid pseudopapillary tumor (SPT).Methods: Data of the SPT patients with and without ADP, including clinical presentations, surgical options, and surgical and survival outcomes, were recruited for comparison.Results: A total of 31 patients with SPTs were included, 3 of whom displayed ADP and underwent PHS enucleation. Surgical complications were comparable between the groups. Overall, the 5-year and 10-year disease-free survival rates were 100% and 90%, respectively. The 20-year and 25-year overall survival rates were 100% and 66.7%, respectively. Only one patient (3.2%) developed tumor recurrence 7.3 years after pancreatectomy for an SPT with lymph node involvement, and the patient survived 24.5 years after the initial operation. No tumor recurrence occurred in any patient with ADP after PHS enucleation.Conclusion: The association between tumorigenesis and ADP suggests that every congenital ADP patient should be observed for the early detection of pancreatic neoplasia. PHS enucleation seems to be feasible and justifiable for SPT patients with ADP in terms of surgical and survival outcomes, and this approach could be recommended to avoid pancreatic insufficiency.

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Section: Discussionsupporting

confidence: 54%

Pancreatic Head Sparing Surgery for Solid Pseudopapillary Tumor in Patients with Agenesis of the Dorsal Pancreas

Shyr

Chen

et al. 2021

Preprint

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“…A young woman with ADP was reported to have asymptomatic insulin-dependent diabetes mellitus and already had severe retinal lesions at the time of presentation [15]. Other studies reported ADP combined with ampullary tumors, and the pathological types included cystic adenocarcinoma, solid pseudopapillary tumors, intraductal papillary mucinous neoplasms, neuroendocrine tumors and cholangiocarcinoma [16,17]. Some patients with pancreatic pseudocysts were also found [18].The surgical treatment of these types of patients is no different from that for patients with normal pancreatic development, but after pancreatic head resection, due to the lack of the tail of the pancreas, postoperative pancreatic exocrine insufficiency and exogenous insulin dependence will be needed.…”

Section: Discussionmentioning

confidence: 99%

Two cases of agenesis of the dorsal pancreas and a review of the literature

Mei

Cao

2020

BMC Gastroenterol

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Background Agenesis of the dorsal pancreas (ADP) is a very rare disease with no specific symptoms, and the pathogenesis is not clear. Some patients will be accompanied by other diseases, such as pancreatic tumor or pancreatitis. But most cases are very atypical and difficult to distinguish. Some syndromes of pancreatic exocrine insufficiency are common in patients with ADP. Here, we report two cases of ADP and summarize the clinical features, diagnosis, and treatment of ADP. Case presentation Case A is a 65-year-old Chinese woman who presented with abdominal pain accompanied by nausea, bloating and acid reflux. The enhanced abdominal CT scan found nothing meaningful except the absence of the body and tail of the pancreas. The diagnosis was considered as gastrointestinal dysfunction cause by exocrine pancreatic insufficiency and recovered after symptomatic treatment. Case B is a 61-year-old Chinese woman who presented with abdominal pain accompanied by fever, vomiting and bloating. The abdominal CT showed multiple stones in the gallbladder, and the body and tail of the patient’s pancreas were absent. She was diagnosed with cholelithiasis and recovered after laparoscopic cholecystectomy. Conclusion Agenesis of the dorsal pancreas (ADP) is a rare congenital disease with an unclear pathogenesis that presents multiple symptoms. It should be considered when the patients have non-specific, persistent and unexplained symptoms such as bloating or uncontrolled blood sugar. Imaging examination is helpful for diagnosis. And it does not require surgical intervention unless it accompanies other diseases, EPI need to be considered when the non-specific gastrointestinal symptoms appear.

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“…PDA association with pancreatic tumours is extremely rare, the PDAC being the most frequent1; NET developed in PDA is even rarer, with only two cases reported as far as the authors know, including the one described in this manuscript 10…”

Section: Discussionmentioning

confidence: 73%

Neuroendocrine tumour in pancreatic dorsal agenesis: a rare association

Rodrigues

Oliveira²,

Oliveira

et al. 2017

BMJ Case Reports

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Pancreatic dorsal agenesis (PDA) is a rare congenital anomaly, usually asymptomatic, that can present with abdominal pain, pancreatitis, diabetes mellitus and jaundice. Pancreatic tumours in PDA background are extremely rare, and when they occur are mainly pancreatic ductal adenocarcinoma. We present a case of a 48-year-old female patient with incidental detection of a 26×20 mm nodular lesion of the cephalic pancreas on ultrasound. Surgery was performed and gross examination revealed PDA with a tumour developed around the Wirsung duct. Histology showed a neuroendocrine tumour G1 with neural and vascular invasion. Two and half years later, the patient is alive and without tumour relapse. Awareness of the association of PDA and pancreatic tumours is fundamental in order to develop personalised follow-up programmes.

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Pancreatic Neuroendocrine Tumor in the Setting of Dorsal Agenesis of the Pancreas

Cited by 11 publications

References 13 publications

Pancreatic Head Sparing Surgery for Solid Pseudopapillary Tumor in Patients with Agenesis of the Dorsal Pancreas

Pancreatic Head Sparing Surgery for Solid Pseudopapillary Tumor in Patients with Agenesis of the Dorsal Pancreas

Two cases of agenesis of the dorsal pancreas and a review of the literature

Neuroendocrine tumour in pancreatic dorsal agenesis: a rare association

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