Summary
An 87-year-old male presented with rapidly progressive dementia, disorder of consciousness and myoclonus, suggesting Creutzfeldt-Jakob disease, but with nonspecific EEG and negative CT findings. Autopsy revealed a malignant angiotropic lymphoma with accumulation of tumor cells within small vessels of the brain and visceral organs. Immunohistochemistry disclosed a large cell lymphoma of B cell type.
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Ansbacher L, Low N, Beck D, Boarini D, Jacoby C, Cancilla PA (1981) Neoplastic angioendotheliosis: a clinico pathological entity with multifocal presentation; case report. J Neurosurg 54:412–415
Ben-Ezra J, Sheibani K, Kendrick FE, Winberg CD, Rappaport H (1986) Angiotropic large cell lymphoma of the prostate gland: an immunohistochemical study. Hum Pathol 17:964–967
Carroll TJ, Schelper RL, Goeken JA, Kemp JD (1986) Neoplastic angioendotheliomatosis: immunopathologic and morphologic evidence for intravascular malignant lymphomatosis. Am J Clin Pathol 85:169–175
Daniel SE, Rudge P, Scaravilli F (1987) Malignant angioendotheliomatosis involving the nervous system: support for a lymphoid origin of the neoplastic cells. J Neurol Neurosurg Psychiatry 50:1173–1177
Davey DD, Munn R, Smith LW, Cibull ML (1990) Angiotropic lymphoma. Presentation in uterine vessels with cytogenetic studies. Arch Pathol Lab Med 114:879–882
Dolman CL, Sweeny VP, Magil A (1979) Neoplastic angioendotheliosis: the case of a missed primary? Arch Neurol 36:5–7
Dozic S, Suvakovic V, Cvetkovic D, Jevtovic D, Skender M (1990) Neoplastic angioendotheliomatosis (NAE) of the CNS in a patient with AIDS subacute encephalitis, diffuse leukencephalopathy and meningo-cerebral cryptococcosis. Clin Neuropathol 9:284–289
Ferry JA, Harris NL, Picker LJ, Weinberg DS, Rosales RK, Tapia J, Richardson EP Jr (1988) Intravascular lymphomatosis (malignant angioendotheliomatosis). A B cell neoplasm expressing surface homing receptors. Mod Pathol 1:444–452
Fredericks RK, Walker FO, Elster A, Challa V (1991) Angiotropic intravascular large cell lymphoma (malignant angioendotheliomatosis): report of a case and review of the literature. Surg Neurol 35:218–223
Fulling KH, Gersell DJ (1983) Neoplastic angioendotheliomatosis. Histologic, immunohistochemical and ultrastructural findings in two cases. Cancer 51:1107–1118
Kayano H, Katayama I (1990) Primary hepatic lymphoma presenting as intravascular lymphomatosis. Arch Pathol Lab Med 114:580–584
Kitagawa M, Matsubara O, Song SY, Kurashima C, Okeda R, Kasuga T (1985) Neoplastic angioendotheliosis. Immunohistochemical and electron microscopic findings in three cases. Cancer 56:1134–1143
Mori S, Itoyama S, Mohri N, Shibuya A, Hirose T, Takanashi R, Oshimi K, Mizoguchi H, Epstein AE (1985) Cellular characteristics of neoplastic angioendotheliosis an immunohistological marker study of 6 cases. Virchows Arch [A] 407:167–175
Ojeda VJ, Scopa J, Cala LA, Kakulas BA (1984) Multifocal progressive neurological disorder caused by neoplastic angioendotheliosis. Med J Aust 141:246–249
Otrakji CL, Voigt W, Amador A, Nadji M, Gregorios JB (1988) Malignant angioendotheliomatosis—A true lymphoma: a case of intravascular malignant lymphomatosis studied by southern blot hybridization analysis. Hum Pathol 19:475–478
Pardo CA, Glass JD, Ruggio D, Mann RB, Raymond LA, Troncoso JC (1990) Intravascular malignant lymphomatosis: a clinicopathological study (abstract). J Neuropathol Exp Neurol 49:240
Petito CK, Gottilieb GJ, Dougherty JH, Petito FA (1978) Neoplastic angioendotheliosis: Ultrastructural study and review of the literature, Ann Neurol 3:393–399
Pfleger L, Tappeiner J (1959) Zur Kenntnis der systemisierten Endotheliomatose der cutanen Blutgefäße. Hautarzt 10:359–363
Raroque HG, Mandler RN, Griffey MS, Orrison WW, Kornfeld M (1990) Neoplastic angioendotheliomatosis. Arch Neurol 47:929–930
Sepp N, Schuler G, Romani N, Geissler N, Gattringer C, Burg G, Bartram CR, Fritsch P (1990) “Intravascular lymphomatosis” (angioendotheliomatosis): evidence for a T-cell origin in two cases. Hum Pathol 21:1051–1058
Sheibani K, Battifora H, Winberg CD, Burke JS, Ben-Ezra J, Ellinger GM, Quigley NS, Fernandez BB, Morrow D, Rappaport H (1986) Further evidence that “malignant angioendotheliomatosis” is an angiotropic large-cell lymphoma. N Engl J Med 314:943–948
Stroup RM, Sheibani K, Moncada A, Purdy LJ, Battifora H (1990) Angiotropic (intravascular) large cell lymphoma. Cancer 66:1781–1788
Wick MR, Mills SE, Scheithauer BW, Cooper PH, Davitz MA, Parkinson K (1986) Reassessment of malignant “angioendotheliomatosis”: evidence in favor of its reclassification as “intravascular lymphomatosis”. Am J Surg Pathol 10:112–123
Yousem SA, Colby TV (1990) Intravascular lymphomatosis presenting in the lung. Cancer 65:349–353
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Drlicek, M., Grisold, W., Liszka, U. et al. Angiotropic lymphoma (malignant angioendotheliomatosis) presenting with rapidly progressive dementia. Acta Neuropathol 82, 533–535 (1991). https://doi.org/10.1007/BF00293391
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DOI: https://doi.org/10.1007/BF00293391