scholarly journals Somatostatinoma of the Vater's papilla in a patient with von Recklinghausen's disease

2007 ◽  
Vol 64 (3) ◽  
pp. 219-222 ◽  
Author(s):  
Radoje Colovic ◽  
Marjan Micev ◽  
Nikica Grubor ◽  
Vladimir Radak
Keyword(s):  
Endocrine Cells ◽  
Von Recklinghausen’S Disease ◽  
Type B ◽  
Von Recklinghausen's Disease ◽  
Recklinghausen's Disease ◽  
D Cells ◽  
Histology And Immunohistochemistry ◽  
Vater’S Papilla ◽  
Recklinghausen’S Disease ◽  
Vater's Papilla

Background. Somatostatinomas of the gastrointestinal tract secret hormone somatostatin which can cause "inhibitory syndrome" comprising diabetes mellitus, cholelithiasis and steatorrheic diarrhea. It is also secreted by the D cells of Langerhans's islands of the pancreas as well as endocrine cells of the stomack, small bowel, salivary glands and parafollicular cells of the thiroid gland. Somatostatinomas of the digestive tract appear within the pancreas and duodenum. Patients suffering from von Recklinghausens's disease are paticularly prone to the somatostatinomas of the duodenum. Case report. In this paper we presented a 51-year old female patient with von Recklinghausen's disease in whom, during the investigation for obstructive jaundice, tumor of the Vater's papilla was found. The patient was submitted to Whipple's duodenopancreatectomy. Histology and immunohistochemistry discovered type B glandular carcinoid tumor with strong antisomatostatin and mild antigastrin immunoreactivity. The patient stayed symptom-free more than four years now. Conclusion. Patients with von Recklinghausen's disease should be examined for other tumors, particularly carcinoids of the duodenum and papilla, especially if the signs of cholestasis are present.

Which cells are inducer or target of neurofibromas in von Recklinghausen's disease?

1986 ◽  
Vol 48 (3) ◽  
pp. 491-499
Author(s):  
Shuhei IMAYAMA ◽  
Yutaka YASHIMA ◽  
Atsushi IRIKI ◽  
Emiko SATO ◽  
Harukuni URABE
Keyword(s):  
Von Recklinghausen’S Disease ◽  
Von Recklinghausen's Disease ◽  
Recklinghausen's Disease ◽  
Recklinghausen’S Disease

Concurrence of Marfan's syndrome and von Recklinghausen's disease with the development of malignant schwannoma.

1987 ◽  
Vol 49 (3) ◽  
pp. 460-464
Author(s):  
Kazumoto KATAGIRI ◽  
Tomohito HONDA ◽  
Satoshi ITAMI ◽  
Etsuji MATSUNAGA ◽  
Hiroshi SHINKAI ◽  
...  
Keyword(s):  
Malignant Schwannoma ◽  
Von Recklinghausen’S Disease ◽  
Marfan’S Syndrome ◽  
Von Recklinghausen's Disease ◽  
Marfan's Syndrome ◽  
Recklinghausen's Disease ◽  
Recklinghausen’S Disease

Neural sheath tumors of major nerves

1994 ◽  
Vol 81 (3) ◽  
pp. 362-373 ◽  
Author(s):  
Thomas R. Donner ◽  
Rand M. Voorhies ◽  
David G. Kline
Keyword(s):  
Motor Function ◽  
Complete Resolution ◽  
Von Recklinghausen’S Disease ◽  
Total Removal ◽  
Von Recklinghausen's Disease ◽  
Plexiform Neurofibromas ◽  
Pain Syndromes ◽  
Recklinghausen's Disease ◽  
Recklinghausen’S Disease

✓ Over a 22-year period, operations were performed on 263 patients for 288 primary benign tumors of major peripheral nerves. The tumors included 85 schwannomas, 197 neurofibromas, and six plexiform neurofibromas. Total removal was achieved in 83 of the 85 schwannomas, and 76 of these patients were available for follow-up evaluation. Motor function either improved or was unchanged in 87% of these patients and 85% of those with pain in the distribution of the involved nerve had either total or partial resolution of their symptoms. Of the neurofibromas, 123 occurred in 121 patients without von Recklinghausen's disease. All tumors within this group were completely excised using a fascicular approach to the tumor. Of the 99 patients available for follow-up evaluation, 90% had either improved or unchanged motor function and 88% had partial or complete resolution of pain syndromes. Fifty-nine patients with von Recklinghausen's disease had 80 tumors removed: 74 fusiform tumors (58 of which were completely removed) and six plexiform tumors. Forty-eight of the 58 patients with gross total removal of fusiform tumors were available for follow-up evaluation, of whom 83% had improved or unchanged motor function and 74% had partial or complete resolution of pain syndromes. All six patients with plexiform tumors had progression of symptoms postoperatively. One brachial plexus schwannoma recurred and was re-excised without subsequent recurrence at the 5-year follow-up evaluation. Several incompletely excised plexiform neurofibromas have recurred with a symptomatic presentation.

NEUROFIBROMATA SIMULATING A PENIS AND LABIOSCROTAL GONADS IN A GIRL WITH VON RECKLINGHAUSEN'S DISEASE

PEDIATRICS ◽  
1966 ◽  
Vol 37 (3) ◽  
pp. 456-459
Author(s):  
Frederic M. Kenny ◽  
George H. Fetterman ◽  
Chawalit Preeyasombat
Keyword(s):  
Ambiguous Genitalia ◽  
Von Recklinghausen’S Disease ◽  
Von Recklinghausen's Disease ◽  
Histologic Diagnosis ◽  
Unilateral Ovariectomy ◽  
Recklinghausen's Disease ◽  
Recklinghausen’S Disease

A 15-year-old girl is described, who had ambiguous genitalia from birth. Enlargement of the clitoris, and labioscrotal masses were present; these were shown to be due to unusually situated neurofibromata. This is the first reported instance of neurofibromata masquerading as a phallus and testes. Prior to the correct histologic diagnosis of the neurofibromata, she underwent two exploratory laparotomies, and unilateral ovariectomy in an attempt to explain the etiology of her ambiguous genitalia.

Primary hepatic somatostatinoma developed in a patient with von Recklinghausen's disease

2006 ◽  
Vol 41 (4) ◽  
pp. 389-391 ◽  
Author(s):  
Yoshihiko Morisawa ◽  
Atsushi Tanaka ◽  
Takatsugu Yamamoto ◽  
Satoko Uegaki ◽  
Yoriyuki Takamori ◽  
...  
Keyword(s):  
Von Recklinghausen’S Disease ◽  
Von Recklinghausen's Disease ◽  
Recklinghausen's Disease ◽  
Recklinghausen’S Disease
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