title: [Primary diffuse leptomeningeal leiomyomatosis].
abstract: A female patient aged 28 fell ill with manifestations of sensory aphasia, headache and signs of cerebral compression. Except for a bilateral papilledema there were no further pathological clinical and paraclinical findings. Later on, amaurosis, deafness, anosmia and generalized muscular hypotonia developed. The nuclear magnetic resonance image revealed a major accumulation of contrast medium in the leptomeninx. Biopsy demonstrated a mesenchymal neoplasm in the leptomeninx. After a strong rise in intracranial pressure, the patient died from a bulbar brain syndrome. Microscopy revealed a diffuse neoplasm limited to the leptomeninx of brain and spinal cord as well as the immediate neighbourhood of small cortical vessels which, by morphological criteria, was classified as low-grade malignant. With the aid of electron microscopy, the tumour cells could be identified as descendants of smooth muscle cells.
authors.affiliation: Institut für Pathologie (Rudolf-Virchow-Haus), Humboldt-Universität zu Berlin, Deutschland.
authors.forename: W
authors.initials: W
authors.lastname: Jänisch
authors.forename: J
authors.initials: J
authors.lastname: Janda
authors.forename: I
authors.initials: I
authors.lastname: Link
mesh_terms: D000328:Adult; D001344:Autopsy; D001706:Biopsy; D005260:Female; D006801:Humans; D018231:Leiomyomatosis / Q000175:diagnosis / Q000473:pathology*; D009682:Magnetic Resonance Spectroscopy; D008577:Meningeal Neoplasms / Q000175:diagnosis / Q000473:pathology* / Q000648:ultrastructure; D008854:Microscopy, Electron; D015899:Tomography, Emission-Computed, Single-Photon
vernacular_title: Primäre diffuse leptomeningeale Leiomyomatose.