PMID: 2508207Sep 1, 1989Paper

Abdominal localizations of von Recklinghausen's neurofibromatosis: apropos of a false uterine leiomyosarcoma

Revue médicale de Bruxelles
J NortierJ Sternon

Abstract

This article reports the surprising history of a patient presenting a von Recklinghausen neurofibromatosis. Ten years after a full program of radio- and chemotherapy for a so-called invasive uterine leiomyosarcoma, the diagnosis of intestinal neurofibromatosis is finally established and the patient is surgically cured. The authors discuss the difficulties of the histologic diagnosis and the aid of the recent techniques of immunohistochemical markers. The second part of the article provides the actual clinical classification of the different types of neurofibromatosis with their diagnostic approach. It also offers a review of the literature concerning the intestinal location of the disease and the potential risks of cancer formation.

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