Morphological spectrum of peripheral nerve sheath tumors: a series of 126 cases.

By convention, soft tissue tumors include the peripheral nerve sheath tumor (PNST) although they arise from neuroectoderm. PNSTs display a wide spectrum of morphology and biological behavior. Malignant transformation in benign tumors, especially neurofibroma, is an acknowledged phenomenon.This article describes clinico-pathological features of 126 PNSTs with their morphological variants and those associated with neurofibromatosis 1 (NF1) encountered in a single center over a 6-year period. Benign PNSTs were more common [118 (93.65%)] when compared with their malignant counterparts [8 (6.34%)]. Histological variants including plexiform schwannomas and diffuse, plexiform and pigmented forms of neurofibromas, though rare, were observed. Eleven (8.73%) patients with NF1 showed a strong association with plexiform neurofibroma. A malignant transformation in pre-existing neurofibroma was seen in 1 patient with NF1. This series highlights the clinico-pathological spectrum of PNSTs, their morphological variants and the incidence of associated NF1. The importance of detecting plexiform variants lies in their vastly different prognostic implications particularly when occurring in the setting of NF1.

Muir Torre syndrome: a case report.

Muir Torre Syndrome (MTS) is a rare syndrome complex of association of multiple sebaceous skin tumors with visceral malignancies. Till date a total of 205 cases have been reported in the world literature and to the best of our knowledge this is the first case report from the Indian subcontinent. Multiple, synchronous and metachronous, tiny sebaceous lesions is a hallmark of MTS along with multiple primary carcinomas at different sites, the commonest being gastro-intestinal tract cancers. The visceral cancers occur at a relatively young age. They are low-grade, non-aggressive and have a good prognosis. Awareness of this rare entity is essential. A case report of a 55-year-old male presenting with multiple sebaceous skin tumors, colonic cancer and positive family history in younger brother is presented.