Retrocaval mass in patient with von Recklinghausen disease. Case report

Cavallaro, Giuseppe and Crocetti, D. and Pedullà, Giuseppe and Giustini, Sandra and Letizia, C. and De Toma, Giorgio (2012) Retrocaval mass in patient with von Recklinghausen disease. Case report. Il giornale di chirurgia, 33 (1/2). pp. 26-30. ISSN 1971-145X

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Abstract

Type I Neurofibromatosis (NF1) is an autosomal-dominant inheritable disorder, with an incidence of 1:3,000, and a prevalence of 1:4,000 to 5,000. Pathogenesis is based on mutations of the NF1 gene, a tumor suppressor gene encoding a cytoplasmic protein named neurofibromin that controls cellular proliferation. Patients affected by NF1 typically present with cutaneous neurofibromas, cafè au lait spots and eye involvement, but they can also be affected by various visceral tumors, such as neurofibromas (nodular or plexiform type), gastrointestinal stromal tumors or endocrine tumors, such as pheochromocytomas. Visceral neurofibromas are often asymptomatic but when growing in size they may present with pain, palpable abdominal mass, symptoms secondary to bowel obstruction or main vessels compression, and even gastrointestinal bleeding when mucosa or submucosa are involved. In these cases surgery becomes mandatory in order to remove all neoplastic tissue. The Authors describe a case of a young man affected by NF1 with associated retrocaval abdominal mass with compression and displacement of the inferior vena cava, thus requiring a complex surgical procedure.

Item Type:	Article
Uncontrolled Keywords:	Neurofibromatosis - Abdominal mass - Surgery
Subjects:	600 Tecnologia - Scienze applicate > 610 Medicina e salute (Classificare qui la tecnologia dei servizi medici) > 617 Rami vari della medicina; Chirurgia > 617.4 Chirurgia secondo il Sistema e Regioni
Depositing User:	Nadia Del Gobbo
Date Deposited:	15 Jun 2016 15:51
Last Modified:	15 Jun 2016 15:51
URI:	http://eprints.bice.rm.cnr.it/id/eprint/7803

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