Skip to main content
SpringerLink
Log in

Intravascular lymphomatosis: a clinicopathological study of three cases

  • Original Papers
  • Clinical Oncology
  • Published:
Journal of Cancer Research and Clinical Oncology Aims and scope Submit manuscript

Abstract

In patients with intravascular lymphomatosis (IL) a broad spectrum of neuro-psychiatric disorders including dementia, focal neurological signs and seizures has been reported. Clinical diagnosis is difficult since neuroimaging findings are nonspecific. The clinical histories, biopsy and autopsy findings of three patients with IL, one with brain biopsy, are described. Two of them presented with rapidly progressive, fluctuating dementia. The third patient suffered from seizures followed by aphasia. Histology revealed large-cell lymphoma of the B cell type. The prognosis of IL, in general, is poor.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  • Abe S, Shimbo S, Saito S, Kohno M, Nishiyama A, Kumanishi T (1988) An immunohistochemical study on “neoplastic angioendotheliosis”: demonstration of B lymphocyte markers in the neoplastic cells. Acta Neuropathol (Berl) 75:313–316

    Google Scholar 

  • Angelis de LM, Yahalamo J, Heinemann MH, Cirrincione C, Thaler HT, Krol G (1990) Primary CNS lymphoma: combined treatment with chemotherapy and radiotherapy. Neurology 40:80–86

    PubMed  Google Scholar 

  • Ansbacher L, Low N, Beck D, Boarini D, Jacobi C, Cancilla PA (1981) Neoplastic angioendotheliomatosis: a clinical pathological entity with multifocal presentation: case report. J Neurosurg 54:412–415

    PubMed  Google Scholar 

  • 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

    PubMed  Google Scholar 

  • Bergmann N, Breining H, Kuchelmeister K, Blasius S, Kindl HJ (1992) Intravaskuläre Lymphomatose (neoplastische Angioendotheliomatose)—eine Kasuistik. Verh Dtsch Ges Pathol 76:520

    Google Scholar 

  • Bhawan J, Wolff SM, Ucci AA, Bhan AK (1985) Malignant lymphoma and malignant angioendotheliomatosis: one disease. Cancer 55:570–576

    PubMed  Google Scholar 

  • Bloomfield CD, Arthur DC, Frizzera G, Levine EG, Peterson BA, Gajl-Peczalska KJ (1983) Nonrandom chromosome abnormalities in lymphoma. Cancer Res 43:2975–2984

    PubMed  Google Scholar 

  • Carroll TJ, Schelper RL, Goeken JA, Kemp JD (1985) Neoplastic angioendotheliomatosis: immunopathologic evidence for intravascular malignant lymphoma. Lab Invest 52:12A-13A

    Google Scholar 

  • 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

    PubMed  Google Scholar 

  • Clark WC, Dohan FC, Moss T, Schweitzer JB (1991) Immunocytochemical evidence of lymphocytic derivation of neoplastic cells in malignant angioendotheliomatosis. J Neurosurg 74:757–762

    PubMed  Google Scholar 

  • Croisile B, Tommasi M, Jouvet A, Truffert A, Trillet M (1990) Lymphome malin endovasculaire à grandes cellules. Rev Neurol (Paris) 146:3:184–190

    Google Scholar 

  • Daniel SE, Rudge P, Scaravilli F (1987) Malignant angioendotheliosis involving the nervous system: support for a lymphoid origin of the neoplastic cells. J Neurol Neurosurg Psychiatry 50:1173–1177

    PubMed  Google Scholar 

  • Davey D, Munn R, Smith LW, Cibull ML (1990) Angiotropic lymphoma. Presentation in uterine vessels with cytogenetic studies. Arch Pathol Lab Med 114:879–882

    PubMed  Google Scholar 

  • Dolman CL (1986) Immunohistochemistry of so called “neoplastic angioendotheliosis”. Acta Neuropathol (Berl) 72:197–199

    Google Scholar 

  • Domizio P, Hall PA, Cotter P (1989) Angiotropic large cell lymphoma (ALCL): morphological, immunohistochemical and genotypic studies with analysis of previous reports. Hematol Oncol 7:195–206

    PubMed  Google Scholar 

  • Drlicek M, Grisold W, Liszka U, Hitzenberger P, Machacek E (1991) Angiotropic lymphoma (malignant angioendotheliomatosis) presenting with rapidly progressive dementia. Acta Neuropathol (Berl) 82:533–535

    Google Scholar 

  • Elner VM, Hidayat AA, Charles NC et al. (1986) Neoplastic angioendotheliomatosis: a variant of malignant lymphoma: immunohistochemical and ultrastructural observations in three cases. Ophthalmology 93:1237–1242

    PubMed  Google Scholar 

  • Ferry JA, Harris NL, Picker LJ, Weinberg DS, Rosales RK, Tapia J, Richardson EP (1988) Intravascular lymphomatosis (malignant angioendotheliomatosis). Mod Pathol 1:444–452

    PubMed  Google Scholar 

  • Hamada K, Hamada T, Satoh M, Tashiro K, Katoh I, Naganuma M, Shima K, Ogata A, Nagashima K (1991) Two cases of neoplastic angioendotheliomatosis presenting with myelopathy. Neurology 41:1139–1140

    PubMed  Google Scholar 

  • Haneke E, Vigneswaran N (1988) Lymphome malin intravasculaire (angioendotheliomatose systematisee proliferante). Ann Dermatol Venereol 115:1221–1223

    PubMed  Google Scholar 

  • Heafield MTE, Carey M, Williams AC, Cullen M (1993) Neoplastic angioendotheliomatosis: a treatable “vascular dementia” occurring in an immunosuppressed transplant patient. Clin Neuropathol 12:102–106

    PubMed  Google Scholar 

  • Hochberg FH, Miller CD (1988) Primary central nervous system lymphoma. J Neurosurg 68:835–853

    PubMed  Google Scholar 

  • Jellinger KA, Paulus W (1992) Primary central nervous system lymphoma—an update. J Cancer Res Clin Oncol 119:7–27

    PubMed  Google Scholar 

  • Keahey TM, Guerry D, Tuthill RJ, Bondi EE (1982) Malignant angioendotheliomatosis proliferans treated with doxorubicin. Arch Dermatol 118:512

    PubMed  Google Scholar 

  • 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

    PubMed  Google Scholar 

  • Kraus J, Stöss H, Gramatzki M (1991) Angioendotheliotropes Lymphom—eine ungewöhnliche solitäre ossäre Manifestation. Fallbeschreibung. Verh Dtsch Ges Pathol 75:536

    Google Scholar 

  • Kumanishi T, Washiyama K, Saito T, Nishiyama A, Abe S, Tanaka T (1986) Primary malignant lymphoma of the the brain: an immunohistochemical study of eight cases using a panel of monoclonal and heterologous antibodies. Acta Neuropathol (Berl) 71:190–196

    Google Scholar 

  • 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 six cases. Virchows Arch [A] 407:167–175

    Google Scholar 

  • Nakamura T, Watanabe M, Hotchi M, Fujimori N, Mizuno M (1987) Neoplastic angioendotheliomatosis: results of two autopsy cases with special reference to the origin of atypical cells. Acta Pathol Jpn 37:1337–1346

    PubMed  Google Scholar 

  • Neuwelt EA, Frenkel ED, Gumerlok MK, Braziel M, Dana B, Hill SA (1986) Development in the diagnosis and treatment of primary CNS lymphomas. Cancer 58:1609–1620

    PubMed  Google Scholar 

  • Nishikawa K, Sekiyama S, Suzuki T, Ito Y, Matsukawa W, Tamai H, Yoshida F (1991) A case of angiotropic large cell manifesting nephrotic syndrome treated successfully with combination chemotherapy. Nephron 58:479–482

    PubMed  Google Scholar 

  • Otrakji CL, Voigt W, Amador A, Nadji M, Gregorios J (1988) Malignant angioendotheliomatosis—a true lymphoma: a case of intravascular malignant lymphomatosis studied by Southern blot hybridization analysis. Hum Pathol 19:475–478

    PubMed  Google Scholar 

  • Pierard GE, Soyeur-Broux M, Fridman F, Sadzot B, Brassinne M de la, Moonen G, Boniver J (1988) Lymphome angiotrope de type angioendotheliomatose proliferative systemique. Ann Dermatol Venereol 115:333–336

    PubMed  Google Scholar 

  • Pfleger L, Tappeiner J (1959) Zur Kenntnis der systemisierten Endotheliomatose der cutanen Blutgefäße. Hautarzt 10:359–363

    PubMed  Google Scholar 

  • Raroque HG, Mandler RN, Orrison WW, Kornfeld M (1990) Neoplastic angioendotheliomatosis. Arch Neurol 47:929–930

    PubMed  Google Scholar 

  • Reinglass JL, Muller J, Wissman S, Wellman H (1977) Central nervous system angioendotheliosis. A treatable multi infarct dementia. Stroke 8:218–221

    PubMed  Google Scholar 

  • Sheibani K, Battifora H, Winberg CD, Burke JS, Ben-Ezra J, Ellinger GM, Quigley NJ, Fernandez BB, Morrow D, Rappaport HN (1986) Further evidence that “malignant angioendotheliomatosis” is an angiotropic large-cell lymphoma. N Engl J Med 314:943–948

    PubMed  Google Scholar 

  • Smadja D, Mas JL, Fallet-Bianco C, Meyniard O, Sicard D, de Recondo J, Rondot P (1991) Intravascular lymphomatosis (neoplastic angioendotheliosis) of the central nervous system: case report and literature review. J Neurooncol 11:171–181

    PubMed  Google Scholar 

  • Stroup RM, Sheibani K, Moncada A, Purdy J, Battifora H (1990) Angiotropic (intravascular) large cell lymphoma. A clinicopathological study of seven cases with unique presentations. Cancer 66:1781–1788

    PubMed  Google Scholar 

  • Theaker JM, Gatter KC, Esiri MM, Easterbrook P (1986) Neoplastic angioendotheliosis—further evidence supporting a lymphoid origin. Histopathology 10:1261–1270

    PubMed  Google Scholar 

  • Vacquero J, Martinez R, Rossi E, Lopez R (1984) Primary cerebral lymphoma: the “ghost tumor”. Case report. J Neurosurg 60:174–176

    PubMed  Google Scholar 

  • Wick MR, Mills SE, Scheithauer BW, Cooper PH, Davitz MA, Parkinson K (1986) Reassessenent of malignant “angioendotheliomatosis” evidence in favor for its reclassification as “intravascular lymphomatosis”. Am J Surg Pathol 2:112–123

    Google Scholar 

  • Willemze R, Kruyswijk MR, Bruin CD de, Meijer CJ, Berkel W van (1987) Angiotropic (intravascular) large cell lymphoma of the skin previously classified as malignant angioendotheliomatosis. Br J Dermatol 116:393–399

    PubMed  Google Scholar 

  • Williams DB, Lyons MK, Yanagihara T, Colgan JP, Banks PM (1991) Cerebral angiotropic large cell lymphoma (neoplastic angioendotheliosis): therapeutic considerations. J Neurol Sci 103:16–21

    PubMed  Google Scholar 

  • Wrotnowski U, Mills SE, Cooper PH (1985) Malignant angioendotheliomatosis. An angiotropic lymphoma? Am J Clin Pathol 83:244–248

    PubMed  Google Scholar 

  • Yousem SA, Colby TV (1990) Intravascular lymphomatosis presenting in the lung. Cancer 65:349–355

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pathology and Bacteriology, Psychiatric Hospital Baumgartner Hoehe, Baumgartner Höhe 1, 1145, Vienna, Austria

    Ulrike Liszka & Markus Drlicek

  2. Department of Neurology, Kaiser Franz Josef Spital, Vienna, Austria

    Peter Hitzenberger & Wolfgang Grisold

  3. Ludwig Boltzmann Institute for Clinical Neurobiology, Vienna, Austria

    Peter Hitzenberger & Wolfgang Grisold

  4. Department of Pathology, Kaiser Franz Josef Spital, Vienna, Austria

    Ernst Machacek

  5. Department of Pathology, University of Innsbruck, Austria

    Hans Mayer

  6. Department of Neurology, University of Innsbruck, Austria

    Günther Stockhammer

Authors
  1. Ulrike Liszka
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Markus Drlicek
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Peter Hitzenberger
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Ernst Machacek
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Hans Mayer
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Günther Stockhammer
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Wolfgang Grisold
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Liszka, U., Drlicek, M., Hitzenberger, P. et al. Intravascular lymphomatosis: a clinicopathological study of three cases. J Cancer Res Clin Oncol 120, 164–168 (1994). https://doi.org/10.1007/BF01202196

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01202196

Key words

Search

Navigation