Sarcomatosis of leptomeninges, brain and spinal cord coexisting with von Recklinghausen's neurofibromatosis. A case report and review of the literature.

Abstract:

:A case of 56-year-old male with sarcomatosis of leptomeninges as well as of the brain and spinal cord coexisting with Recklinghausen's neurofibromatosis is presented. Neurological and neurophysiological symptoms of the disease resembled those of amyotrophic lateral sclerosis (ALS). Patient died 7 months after onset of the initial symptoms. The post-mortem examination revealed neoplastic infiltration of the leptomeninges of brain and spinal cord. Histologically sarcomatosis of the leptomeninges was diagnosed and immunohistochemical analysis of the neoplastic infiltrates can indicate fibrohistiocytic origin of the neoplasm, suggesting also a probable contribution of perineurial cells in the pathogenesis of the tumor. On the grounds of the performed immunohistochemical study together with a review of the literature, the differential diagnosis of malignant mesenchymal tumors of the CNS is discussed with a special regard to their histogenesis.

journal_name

Folia Neuropathol

journal_title

Folia neuropathologica

authors

Słowiński J,Adamek D,Krygowska-Wajs A,Rudzińska M,Kałuza J

subject

Has Abstract

pub_date

1995-01-01 00:00:00

pages

135-40

issue

3

eissn

1641-4640

issn

1509-572X

journal_volume

33

pub_type

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