Sarcomatosis of leptomeninges, brain and spinal cord coexisting with von Recklinghausen's neurofibromatosis. A case report and review of the literature.
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.
Słowiński
J
J
Department of Neuropathology, Jagiellonian University, Collegium Medicum, Kraków.
Adamek
D
D
Krygowska-Wajs
A
A
Rudzińska
M
M
Kałuza
J
J
D001099:Arachnoid / Q000473:pathology*; D001932:Brain Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D017253:Neurofibromatoses / Q000150:complications* / Q000175:diagnosis; D012509:Sarcoma / Q000150:complications* / Q000175:diagnosis / Q000473:pathology*; D013116:Spinal Cord / Q000473:pathology*; D013120:Spinal Cord Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000473:pathology*