[Von Recklinghausen type I neurofibromatosis and neuroendocrine tumor (somatostatinoma) in a 50-year-old woman].

METHODS

A 50-year-old woman has had diffuse abdominal symptoms for approximately 2 weeks. For 30 years a von Recklinghausen's neurofibromatosis has been known.

METHODS

Clinically and chemically there was a cholestasis (alkaline phosphatase 244 U/l, gamma GT 83 U/l) with uneventful values for transaminases and bilirubin. The hepatitis serology (A, B, C) as well as the AMA were negative. Somatostatin with 73 ng/l was slightly increased. Ultrasonography revealed a low-grade intrahepatic cholestasis, the ductus pancreaticus was extended to 9 mm, while endoscopic retrograde cholangiopancreatography showed an extended pancreatic duct without inflamed changes as well as an extended intra- and extrahepatic gall duct system without detecting a stone. The oesophagogastroduodenoscopy showed a polypoid tumor 3 cm above the Papilla Vateri which is part of a neuroendocrine tumor of the carcinoid type, immunoreactive towards somatostatin.

METHODS

In the framework of the surgical intervention carried out by extirpation of the polypoid tumor above the Papilla Vateri by segment excision with a duodeno-duodenostomy. Within a period of 15 months, the patient was free from a tumor relapse or metastasis.

CONCLUSIONS

Carcinoid tumors should always be considered in patients who have von Recklinghausen's neurofibromatosis in combination with abdominal pain in the duodenal area, especially if cholestasis parameters and bilirubin are high or if gastrointestinal bleeding occurs.