Malignant endocrine tumor of the pancreas associated with von Recklinghausen's disease

We describe a case of malignant endocrine tumor of the pancreas associated with von Recklinghausen's disease. A 66-year-old woman with familial von Recklinghausen's disease was admitted to our hospital with weight loss and lower abdominal pain. Endoscopy revealed a submucosal tumor in the...

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Veröffentlicht in:Journal of gastroenterology 2002-01, Vol.37 (1), p.59-67
Hauptverfasser: Fujisawa, Takashi, Osuga, Tatsuya, Maeda, Mitsuo, Sakamoto, Norikazu, Maeda, Tetsuo, Sakaguchi, Kazuhiko, Onishi, Yutaka, Toyoda, Masanori, Maeda, Hiroaki, Miyamoto, Katsufumi, Kawaraya, Nobuaki, Kusumoto, Chosei, Nishigami, Takashi
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container_title Journal of gastroenterology
container_volume 37
creator Fujisawa, Takashi
Osuga, Tatsuya
Maeda, Mitsuo
Sakamoto, Norikazu
Maeda, Tetsuo
Sakaguchi, Kazuhiko
Onishi, Yutaka
Toyoda, Masanori
Maeda, Hiroaki
Miyamoto, Katsufumi
Kawaraya, Nobuaki
Kusumoto, Chosei
Nishigami, Takashi
description We describe a case of malignant endocrine tumor of the pancreas associated with von Recklinghausen's disease. A 66-year-old woman with familial von Recklinghausen's disease was admitted to our hospital with weight loss and lower abdominal pain. Endoscopy revealed a submucosal tumor in the second part of the duodenum. Contrast-enhanced computed tomography showed a heterogeneous low-density tumor, measuring 20 x 15 mm, in the head of the pancreas, and metastatic lymphadenopathy. Angiography showed faint tumor staining without encasement or apparent increase in vascularity. Pancreatoduodenectomy was performed. Macroscopically, there was a solid tumor, measuring 20 x 18 mm, in the head of the pancreas. Histologically, a malignant endocrine tumor was shown, with direct invasion to the duodenum, and lymph node metastases. This is the thirteenth case of pancreatic malignant tumor and the fourth case of pancreatic endocrine tumor associated with von Recklinghausen's disease reported in the world literature.
doi_str_mv 10.1007/s535-002-8135-x
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subjects Aged
Antineoplastic Combined Chemotherapy Protocols - therapeutic use
Combined Modality Therapy
Duodenal Neoplasms - diagnosis
Duodenal Neoplasms - therapy
Duodenoscopy
Endocrine Gland Neoplasms - complications
Endocrine Gland Neoplasms - diagnosis
Endocrine Gland Neoplasms - pathology
Endocrine Gland Neoplasms - therapy
Female
Humans
Lymphatic Metastasis
Neoplasm Invasiveness
Neurofibromatosis 1 - complications
Pancreatic Neoplasms - complications
Pancreatic Neoplasms - diagnosis
Pancreatic Neoplasms - pathology
Pancreatic Neoplasms - therapy
Pancreaticoduodenectomy
title Malignant endocrine tumor of the pancreas associated with von Recklinghausen's disease
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