title: Malignant endocrine tumor of the pancreas associated with von Recklinghausen's disease.
abstract: 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.
authors.affiliation: Department of Internal Medicine, Harima Hospital of Ishikawajima-Harima Heavy Industries, Health Insurance Society, Aioi, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Fujisawa
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Osuga
authors.forename: Mitsuo
authors.initials: M
authors.lastname: Maeda
authors.forename: Norikazu
authors.initials: N
authors.lastname: Sakamoto
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Maeda
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Sakaguchi
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Onishi
authors.forename: Masanori
authors.initials: M
authors.lastname: Toyoda
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Maeda
authors.forename: Katsufumi
authors.initials: K
authors.lastname: Miyamoto
authors.forename: Nobuaki
authors.initials: N
authors.lastname: Kawaraya
authors.forename: Chosei
authors.initials: C
authors.lastname: Kusumoto
authors.forename: Takashi
authors.initials: T
authors.lastname: Nishigami
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003131:Combined Modality Therapy; D004379:Duodenal Neoplasms / Q000175:diagnosis / Q000628:therapy; D004384:Duodenoscopy; D004701:Endocrine Gland Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D005260:Female; D006801:Humans; D008207:Lymphatic Metastasis; D009361:Neoplasm Invasiveness; D009456:Neurofibromatosis 1 / Q000150:complications*; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D016577:Pancreaticoduodenectomy