Neurofibrosarcoma of skin and subcutaneous tissues.

Abstract:

:In this report, we describe 13 cases of primary neurofibrosarcoma of the skin. The tumor presumably arises from small cutaneous nerves, is locally aggressive, and has a potential for metastasis. Characteristic histopathologic features include proliferating atypical spindle cells with slender wavy and pointed nuclei; hypocellular areas with loose, myxoid stroma; and areas of organoid organization such as palisading, whorly, storiform, and tactile body-like formations. The S-100 stain is positive in about 60% of cases. In the current series, most tumors arose in deep dermis and were grade 2 malignant lesions with a moderate degree of cytologic atypia and 2 or fewer mitoses in 10 high-power fields. Three patients died of their malignant lesion. Only two tumors metastasized. Of the 10 patients who had local recurrence, 5 had multiple recurrent lesions. Neurofibrosarcoma should be considered in the differential diagnosis of malignant tumors of the skin. A complete surgical resection of the primary tumor with adequate margins of surrounding normal-appearing tissue is advised.

journal_name

Mayo Clin Proc

journal_title

Mayo Clinic proceedings

authors

Dabski C,Reiman HM Jr,Muller SA

doi

10.1016/s0025-6196(12)65011-3

subject

Has Abstract

pub_date

1990-02-01 00:00:00

pages

164-72

issue

2

eissn

0025-6196

issn

1942-5546

pii

S0025-6196(12)65011-3

journal_volume

65

pub_type

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