Serous Cystic Neoplasms of the Whole Pancreas in a Patient with von Hippel-Lindau Disease

Abstract

We describe here a case of von Hippel-Lindau (VHL) disease with a serous cystic neoplasm of the whole pancreas. The patient was a 35-year-old woman suffering from a palpable abdominal tumor. She had a history of hemangioblastomas of the cerebellum. CT revealed large solid tumors in the pancreatic head and body, and multiple cystic lesions in the whole pancreas as well as a right renal tumor. When endoscopic retrograde cholangiopancreatography (ERCP) was performed, bleeding from the duodenal papilla was detected. Since she had some distinguishing clinical features, the diagnosis of VHL disease was made. The preoperative diagnosis of the pancreatic lesion was serous cystic neoplasms with hemosuccus pancreaticus and total pancreatectomy was performed. Histological examination of the specimen revealed serous cystic neoplasms which occupied the entire pancreas. VHL cases operated on for serous cystic neoplasms of the entire pancreas are very rare.

Wirsungorrhagia in cystic mucinous pancreasic tumor

“…Как правило, причиной вирсунгоррагии являются хронический панкреатит и ложные аневризмы бассейна чревного ствола и ветвей верхней брыжеечной артерии [11]. В англоязычной литературе (MEDLINE) описано лишь 6 наблюдений кистозных опухолей поджелудочной железы, проявляющихся кровотечением в просвет протока поджелудочной железы: серозная цистаденома -у 3 больных, муцинозная цистаденома -у 2, внутрипротоковая папиллярно-муцинозная опухоль -у 1 [3][4][5][6][7][8].…”

“…Клиническая симптоматика муцинозной опухоли нередко отсутствует или носит неспецифический характер, что объясняет ее частое выявление в качестве случайной находки. Вирсунгоррагия -кровотечение в проток поджелудочной железы и далее в двенадцатиперстную кишку вследствие аррозии прилежащего к опухоли артериального сосуда -является казуистическим осложнением [2][3][4][5][6][7][8].…”

Role of cortical sparing adrenalectomy and novel variant of mutation in patient with von Hippel-Lindau disease

“…Pancreatic cysts and serous cyst neoplasm occur in 17–56% patients with VHL. [6] Neuroendocrine pancreatic tumors are rare and are reported in 8–17% of patients with VHL. [6] These lesions may be malignant and metastasize to regional lymph nodes.…”

“…[6] Neuroendocrine pancreatic tumors are rare and are reported in 8–17% of patients with VHL. [6] These lesions may be malignant and metastasize to regional lymph nodes. [78] Based on pre-operative imaging alone, it is very difficult to differentiate pancreatic neuroendocrine tumors from serous cyst neoplasm.…”

Pancreatic cyst development: insights from von Hippel-Lindau disease

“…One study [10] and numerous case-reports [12,13,17-23] have recorded clinical problems, of which compression of the biliary tract was most frequently reported (Table 1). Intervention was indicated in only 3% of VHL patients [10].…”