Neurofibrossarcoma retroperitoneal primário

Michelle Lucinda Gonçalves De Oliveira,Gaspar De Jesus Lopes Filho

doi:10.1590/s0100-69912003000500013

Michelle Lucinda Gonçalves De Oliveira, Gaspar De Jesus Lopes Filho

Open Access

https://doi.org/10.1590/s0100-69912003000500013

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Abstract

Malignant schwannoma or neurofibrosarcoma usually grows from the peripheral nerves and rarely from the retroperitoneum. When found in the retroperitoneum, it usually has a worse prognosis. This kind of tumor seems to be resistant to adjuvant therapy and the best treatment is radical surgery. We report a case of a primary retroperitoneal malignant schwannoma, in a 64 year-old man, treated surgically.

Highlights

Malignant schwannoma or neurofibrosarcoma usually grows from the peripheral nerves and rarely from the retroperitoneum
When found in the retroperitoneum, it usually has a worse prognosis. This kind of tumor seems to be resistant to adjuvant therapy and the best treatment is radical surgery
We report a case of a primary retroperitoneal malignant schwannoma, in a 64 year-old man, treated surgically

Summary

Introduction

Malignant schwannoma or neurofibrosarcoma usually grows from the peripheral nerves and rarely from the retroperitoneum. O neurofibrossarcoma, também conhecido como schwannoma maligno, é um tumor primário da bainha neural, originado do neuroectoderma, que, usualmente, surge dos nervos periféricos e, raramente, do retroperitônio. O schwannoma maligno retroperitoneal, na ausência da neurofibromatose, é raro, representando menos de 2% de todos os tumores primitivos que ocorrem nesta região. Em torno de 4% dos pacientes com esta doença apresentam schwannoma maligno e, quando ocorre, esta associação sugere um pior prognóstico[2 ].

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Conclusion