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Intravascular lymphomatosis (neoplastic angioendotheliosis) of the central nervous system: case report and literature review

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Summary

A 63-year-old woman developed gradual slowness, recurrent multifocal deficits, severe constitutional symptoms and hypopituitarism which progressed to death over 2.5 months. Elevation in lactico dehydrogenases was the main biological abnormality. Necropsy showed an intravascular malignant proliferation which proved to be a lymphoma of B-lineage. In order to better define diagnostic criteria, we reviewed previously reported cases of intravascular lymphomatosis of the central nervous system. A strategy for establishing the diagnosis is proposed.

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References

  1. Pfleger L, Tappeiner J: Zür Kenntnis der systemisierten Endotheliomatose der cutanen Blutgefässe (Reticuloendotheliose?). Hautarzt 10: 359–363, 1959

    CAS  PubMed  Google Scholar 

  2. Case Records of the Massachusetts General Hospital: N Engl J Med 315: 874–885, 1986

    Google Scholar 

  3. Mori S, Takanashi M, Motosi T: Neoplastic angioendotheliosis with B lymphocytes markers: a case report. Jpn J Cancer Res 76: 208–212, 1985

    CAS  PubMed  Google Scholar 

  4. Sheibani K, Battigora H, Winberg CD, Burke JS, Ben Ezra J: Further evidence that malignant angioendotheliomatosis is an angiotropic large cell lymphoma. N Engl J Med 314: 943–948, 1986

    CAS  PubMed  Google Scholar 

  5. Wich MR, Mills SE, Scheithauer BW, Cooper PH, Davitz MA, Parkinson K: Reassessment of malignant ‘angioendotheliomatosis’. Evidence in favor of its reclassification as ‘intravascular lymphomatosis’. Am J Surg Pathol 10: 112–123, 1986

    Google Scholar 

  6. Petito CK, Gottlieb GJ, Dougherty JH, Petito FA: Neoplastic angioendotheliosis: ultrastructural study and review of the literature. Ann Neurol 3: 393–399, 1977

    Google Scholar 

  7. Sunohara N, Mukoyama M, Satoyoshi E: Neoplastic angioendotheliosis of the central nervous system. J Neurol 231: 14–19, 1984

    Article  CAS  PubMed  Google Scholar 

  8. Tappeiner J, Pfleger L: Angioendotheliomatosis proliferans systemisata. Ein klinisch und pathologisch neues Kronicheutsbild. Hautarzt 14: 67–70, 1963

    CAS  PubMed  Google Scholar 

  9. Arnn ET, Yam LT, Li CH: Systemic angioendotheliomatosis presenting with hemolytic anemia. Am J Clin Pathol 80: 246–251, 1983

    CAS  PubMed  Google Scholar 

  10. Remberger K, Nawrath-Koll I, Gokel JM: Systemic angioendotheliomatosis of the lung. Path Res Pract 182: 265–270, 1987

    CAS  PubMed  Google Scholar 

  11. Wick MR, Banks PM, Mc Donald TH: Angioendotheliomatosis of the nose with fatal systemic dissemination. Cancer 48: 2510–2517, 1981

    CAS  PubMed  Google Scholar 

  12. Ansell J, Bhawan J, Cohen S, Sullivan J, Sherman D: Histiocytic lymphoma and malignant angioendotheliomatosis. One disease or two? Cancer 50: 1506–1512, 1982

    CAS  PubMed  Google Scholar 

  13. Bhawan J, Wolff JM, Ucci AA, Bhan AK: Malignant lymphoma and malignant angioendotheliomatosis: one disease. Cancer 55: 570–576, 1985

    CAS  PubMed  Google Scholar 

  14. Braverman JM, Lerner AB: Diffuse malignant proliferation of vascular endothelium. Arch Dermatol 84: 22–30, 1961

    Google Scholar 

  15. Crosby TW, Chou SM, Gutresht J: Neoplastic angioendotheliosos. J Neuropathol Exp Neurol (Abstr) 35: 361, 1976

    Google Scholar 

  16. Dolman CL: Immuno-histochemistry of so called «neoplastic angioendotheliosis’. Acta Neuropathol (Berl) 72: 197–199, 1986

    CAS  Google Scholar 

  17. Fievez M, Fievez C, Hustin J: Proliferation systematized angioendotheliomatosis. Arch Dermatol 104: 320–324, 1971

    Article  CAS  PubMed  Google Scholar 

  18. Haber H, Harris-Jones JN, Wells AL: Intravascular endothelioma (endothelioma in situ, systemic endotheliomatosis). J Clin Path 17: 608–611, 1964

    CAS  PubMed  Google Scholar 

  19. Husain MM, Sun CN, White HJ: Neoplastic angioendotheliosis. J Neuropathol Exp Neurol (Abstr) 38: 322, 1979

    Google Scholar 

  20. Kageyama Y, Kawamuka J, Miyazki T, Iikuni K, Koto A: A case of multiple tumor-cell embolism to the central nervous system simulating neoplastic angioendotheliosis. Clin Neurol (Tokyo) 23: 110–116, 1983

    CAS  Google Scholar 

  21. Kauh YC, Mc Farland JP, Carnabuci GC, Luscombe HA: Malignant proliferating angioendotheliomatosis. Arch Dermatol 116: 803–806, 1980

    CAS  PubMed  Google Scholar 

  22. Madara J, Shane J, Scarlato M: Systemic endotheliomatosis: a case report. J Clin Path 28: 476–482, 1975

    CAS  PubMed  Google Scholar 

  23. Scott PWB, Silvers DN, Helwig EB: Proliferating angioendotheliomatosis. Arch Pathol 99: 323–326, 1975

    CAS  PubMed  Google Scholar 

  24. Shtern RD, Likhachev YP: Endotheliomatosis as a systemic tumor affection. Arch Pathol 25: 35–40, 1963

    CAS  Google Scholar 

  25. Sparling TG, Rosen DA, Corbett WEN, Galbraith PR: Microvascular-closure in malignant histiocytosis. Can Med Assoc J 120: 47–54, 1979

    CAS  PubMed  Google Scholar 

  26. Abe S, Shimbo Y, Saito T, Khono M, Nishiyama A, Kumanishi T: An immunohistochemical study on ‘neoplastic angioendotheliosis’: demonstration of B lymphocyte markers in the neoplastic cells. Acta Neuropathol 75: 313–316, 1988

    Article  CAS  PubMed  Google Scholar 

  27. Ansbacher L, Low N, Beck D, Boarini D, Jacoby C, Cancilla PA: Neoplastic angioendotheliosis: a clinico-pathological entity with multifocal presentation; case report. J Neurosurg 54: 412–415, 1981

    CAS  PubMed  Google Scholar 

  28. Beal MF, Fisher CM: Neoplastic angioendotheliosis. J Neurol Sci 53: 359–375, 1982

    Article  CAS  PubMed  Google Scholar 

  29. Bots TAM: Angioendotheliomatosis of the central nervous system. Acta Neuropathol (Berl) 28: 75–78, 1974

    CAS  Google Scholar 

  30. Carroll TJ, Schelper RL, Goeken JA, Kemp JD: Noming: malignant angioendotheliomatosis. N Engl J Med 315: 895, 1986

    PubMed  Google Scholar 

  31. Chen P, Gaetjens E, Sher J, Somasundaram M, Lee TK, How SW, Cho NH: Malignant angioendotheliomatosis manifesting as ascending spinal cord dysfunction. NY State J Med 87: 470–472, 1987

    CAS  Google Scholar 

  32. Daniel SE, Rodge P, Scaravilli F: Malignant angioendotheliomatosis involving the nervous system: support for a lymphoid origin of the neoplastic cells. J Neurol Neurosurg Psychiatry 50: 1173–1177, 1987

    CAS  PubMed  Google Scholar 

  33. Dolman CL, Sweeney VP, Magil A: Neoplastic angioendotheliosis. The case of missed primary? Arch Neurol 36: 5–7, 1979

    CAS  PubMed  Google Scholar 

  34. Fulling KH, Gersell DJ: Neoplastic angioendotheliomatosis. Histologic, immunohistochemical and ultrastructural findings in two cases. Cancer 51: 1107–1118, 1983

    CAS  PubMed  Google Scholar 

  35. Kermarec J, Canioni D, Michiels JF, Kullmann B, Borg M, Martin P: L'angioendothéliomatose (maligne) systémique. A propos d'une observation avec étude ultrastructurale et histo-immunologique. Ann Pathol (Paris) 4: 61–67, 1984

    CAS  Google Scholar 

  36. Kitagawa M, Matsubaro O, Song SY, Kurashima C, Okeda R, Kasuga T: Neoplastic angioendotheliosis. Cancer 56: 1134–1143, 1985

    CAS  PubMed  Google Scholar 

  37. Knight RSG, Anslow P, Theaker JM: Neoplastic angioendotheliosis: a case of subacute dementia with unusual cerebral CT appearances and a review of the literature. J Neurol Neurosurg Psychiatry 50: 1022–1028, 1987

    CAS  PubMed  Google Scholar 

  38. Krieger C, Robitaille Y, Jothy S, Elleker G: Intravascular malignant histiocytosis mimicking central nervous system vasculitis: an immunopathological diagnostic approach. Ann Neurol 12: 489–492, 1982

    Article  CAS  PubMed  Google Scholar 

  39. Lewitt PA, Forno LS, Brant-Zawadzki M: Neoplastic angioendotheliosis: a case with spontaneous regression and radiographic appearance of cerebral arteritis. Neurology 33: 39–44, 1983

    CAS  PubMed  Google Scholar 

  40. Nakamura T, Watanabe M, Hotchi M, Fusimori N, Mizono M: Neoplastic angioendotheliomatosis. Report of two autopsy cases with special reference to the origin of atypical cells. Acta Pathol Jpn 37: 1337–1346, 1987

    CAS  PubMed  Google Scholar 

  41. Ojeda VJ: Neoplastic angioendotheliosis of the spinal cord. Case report. Acta Neuropathol (Berl) 62: 164–166, 1983

    CAS  Google Scholar 

  42. Otrakji CL, Voigt W, Amador A, Nadji M, Gregorios JB: Malignant angioendotheliomatosis. A true lymphoma: a case of intravascular malignant lymphomatosis studied by southern blot hybridization analysis. Human Pathol 19: 475–478, 1988

    CAS  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  44. Strouth JC, Donahue S, Ross A, Aldred A: Neoplastic angioendotheliosis. Neurology 15: 644–648, 1965

    CAS  PubMed  Google Scholar 

  45. Tanaka M, Yamagushi H, Uchiyama T, Hirai S: An autopsy case of malignant lymphoma mimicking neoplastic angioendotheliosis. Clin Neurol (Tokyo) 22: 507–513, 1982

    CAS  Google Scholar 

  46. Wick MR, Sheithauer BW, Okazaki H, Thomas JE: Cerebral angioendotheliomatosis. Arch Pathol Lab Med 106: 342–346, 1982

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  48. Yamamura Y, Akamizu H, Hirata T, Kito S, Hamada T: Malignant lymphoma presenting with neoplastic angioendotheliosis of the CNS. Clin Pathol 2: 62–68, 1983

    CAS  Google Scholar 

  49. Carroll TJ, Schelper RL, Goeken JA, Kemp KD: Neoplastic angioendotheliomatosis: immunopathologic and morphologic evidence for intravascular malignant lymphomatosis. Am J Clin Pathol 85: 169–175, 1986

    PubMed  Google Scholar 

  50. Mori S, Itoyama S, Mohri N, Shibuya A, Hirose T, Takanashi R, Oshimi K: Cellular characteristics of neoplastic angioendotheliosis. Virchows Arch (Pathol Anat) 407: 167–175, 1985

    CAS  Google Scholar 

  51. Ojeda VJ, Spagnolo DV: Neoplastic angioendotheliosis is, in fact, intravascular malignant lymphoma. Acta Neuropathol (Berl) 72: 203, 1986

    CAS  Google Scholar 

  52. Ferry JA, Harris NL, Picker LJ, Weinberg DS, Rosales RK, Tapia J, Richardson EP: Intravascular lymphomatosis (Malignant angioendotheliomatosis). A B-cell neoplasm expressing surface homing receptors. Modern Pathology, Vol 1, 6: 444–452, 1988

    CAS  PubMed  Google Scholar 

  53. Cooper HS: Lecting as probes in histochemistry and immunohistochemistry: the peanut (Arachis hypogagea) Pectin. Hum Pathol 15: 904, 1984

    CAS  PubMed  Google Scholar 

  54. Baster JD, Harris AW, Tomkins GM, Cohen M: Glucocorticoid receptors in lymphoma cells in culture: relationship of glucocorticoid killing activity. Science 2: 189–191, 1971

    Google Scholar 

  55. Singh A, Strobos RJ, Singh BJ, Rothballer AB, Reddy V, Puljic S, Poon TP: Steroid-induced remissions in CNS lymphoma. Neurology 32: 1267–1271, 1982

    CAS  PubMed  Google Scholar 

  56. Chamberlain MC, Levin VA: Adjuvant chemotherapy for primary lymphoma of the central nervous system. Neurology 38: 210, 1988

    Google Scholar 

  57. Dastur DC: The pathology and pathogenesis of tuberculous encephalopathy and myeloradiculopathy: a comparison with allergic encephalomyelitis. Child's Nerv Syst 2: 13–19, 1986

    Article  CAS  Google Scholar 

  58. Mas JL, Louarn F, Degos JD: Angéites non spécifiques et système nerveux central. Rev Neurol (Paris) 139: 467–484, 1983

    CAS  Google Scholar 

  59. Calabrese LH, Mallek JA: Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature and proposal for diagnostic criteria. Medicine 67: 20–39, 1987

    Google Scholar 

  60. Biller J, Challa VR, Toole JF, Howard VV: Non bacterial thrombotic endocarditis. A neurological perspective of clinicopathologic correlations of 99 patients. Arch Neurol 39: 95–98, 1982

    CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Services de Neurologie, CHS Sainte-Anne, Centre Raymond Garcin, 1 rue Cabanis, 75674, Paris Cedex 14, France

    Didier Smadja, Jean-Louis Mas, Jean de Recondo & Pierre Rondot

  2. d'Anatomie Pathologique, CHS Sainte-Anne, Centre Raymond Garcin, 1 rue Cabanis, 75674, Paris Cedex 14, France

    Catherine Fallet-Bianco

  3. Département de Médecine Interne, Groupe Hospitalier Cochin Port-Royal, 27 rue du Faubourg Saint Jacques, 75674, Paris Cedex 14, France

    Olivier Meyniard & Didier Sicard

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  1. Didier Smadja
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  2. Jean-Louis Mas
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  6. Jean de Recondo
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Smadja, D., Mas, JL., Fallet-Bianco, C. et al. Intravascular lymphomatosis (neoplastic angioendotheliosis) of the central nervous system: case report and literature review. J Neuro-Oncol 11, 171–180 (1991). https://doi.org/10.1007/BF02390177

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