Abstract • A 60-year-old white man presented with aphasia, seizures, paraparesis, and incontinence. His serologic and hematologic profiles were unremarkable. His cerebrospinal fluid showed pleocytosis, increased daily central nervous system IgG synthesis, increased myelin basic protein, and negative cytology and cultures. Cerebral computed tomography exhibited multiple areas of hypodensity but spinal computed tomography and myelography showed no abnormalities. Cranial and spinal magnetic resonance imaging revealed areas of increased signal on T2-weighted images. The use of gadolinium—pentetic acid on T1-weighted images delineated smaller areas of cortical enhancement with surrounding rim of decreased signal. Brain biopsy showed intravascular malignant cells positive for leukocyte common antigen and B-cell markers. The diagnosis was neoplastic angioendotheliomatosis (intravascular lymphomatosis). To our knowledge, this is the first report on the use of both cranial and spinal magnetic resonance imaging in this condition. References 1. Bhawan J. Angioendotheliomatosis proliferans systemica: an angiotropic neoplasm of lymphoid origin . Semin Diagn Pathol . 1987;4:18-27. 2. Petito CK, Gottlieb GS, Dougherty JH, Petito FA. Neoplastic angioendotheliomatosis: ultrastructural study and review of the literature . Ann Neurol . 1978;3:393-399.Crossref 3. Kitagawa M, Matsubara O, Song S, Kurashime C, Okeda R, Kasuga T. Neoplastic angioendotheliomatosis: immunohistochemical and electron microscopic findings in three cases . Cancer 1987;56:1134-1143.Crossref 4. Sheibani K, Battifora H, Winberg CD, et al. Further evidence that 'malignant angioendotheliomatosis' is an angiotropic large cell lymphoma . N Engl J Med . 1986;314:943-948.Crossref 5. Daniel SE, Rudge B, Scaravilli S. Malignant angioendotheliomatosis involving the nervous system: support for a lymphoid origin of neoplastic cells . J Neurol Neurosurg Psychiatry . 1987;50:1173-1177.Crossref 6. Bhawan J, Wolff SM, Ucci AA, Bhan AK. Malignant lymphoma and malignant angioendotheliomatosis: one disease . Cancer 1985;55:570-576.Crossref 7. Schelper RL, Olson SP, Carrol TS, Hart MN, Witters E. Studies of the endothelial origin of cells in systemic angioendotheliomatosis and other vascular lesions of the brain and meninges using Ulex europaeus lectin stains . Clin Neuropathol . 1986;5:231-237. 8. Carroll TS, Schelper RL, Goeken JA, Kemp JD. Neoplastic angioendotheliomatosis: immunopathologic malignant lymphomatosis . Am J Clin Pathol . 1986;85:169-175. 9. Mori S, Takanashi M, Motoji T, Shiozaki H, Oshimi K, Mizoguehi H. Neoplastic angioendotheliomatosis with B lymphocyte markers on neoplastic cells: a case report . Gann . 1985;76:208-212. Abstract. 10. Brew BJ, O'Sullivan DJ, Darveniza P, et al. Neoplastic angioendotheliomatosis . Clin Exp Neurol . 1987;23:119-126. 11. Elner VM, Hidayot AA, Charles NC, et al. Neoplastic angioendotheliomatosis . Ophthalmology . 1986;93:1237-1245.Crossref 12. Ansbacher L, Low N, Beck D, Boarini D, Jacoby C, Cancilla PA. Neoplastic angioendotheliomatosis: a clinicopathological entity with multifocal presentation: case report . J Neurosurg . 1985;54:412-415.Crossref 13. Otrakji CL, Voigt W, Amador A, Nadji M, Gregorios J. Malignant angioendotheliomatosis, a true lymphoma: a case of intravascular malignant lymphomatosis studied by southern blot hybridization analysis . Hum Pathol . 1988;19:475-478.Crossref 14. Reinglass JL, Muller J, Wissman S, Wollman A. Central nervous system angioendotheliomatosis: a treatable multiple infarct dementia . Stroke . 1977;8:218-221.Crossref 15. Schonfeld SM, Pinto RS, Aeksic S, Pearson J. Cerebral angioendotheliomatosis: a report of 2 cases and review of the literature . AJNR . 1985;6:437-441. 16. Chapman JE, Loy WA. Cerebral angioendotheliomatosis with hemispheric symptoms of carotid arterial origin . J Vase Surg . 1985;2:281-284.Crossref 17. Keahy TM, DuPont G IV, Tuhill RJ, Bondi EE. Malignant angioendotheliomatosis proliferans treated with doxurubicin . Arch Dermatol . 1982;118:512-514.Crossref
Archives of Neurology – American Medical Association
Published: Aug 1, 1990
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