[en] We report two cases of intravascular malignant lymphomatosis (IML) with a clinical expression limited to the central nervous system. The first patient presented with signs of cerebral, cerebellar and spinal cord involvement. The second had an isolated involvement of the spinal cord. In both cases the diagnosis was made at post-mortem examination; pre-mortem examination of biopsy tissue from peripheral nerve and muscle in the first case, spleen and liver in the second were unhelpful for the diagnosis of lymphoma. We review the published literature on IML, its ante-mortem diagnosis and treatment.
Disciplines :
Neurology Neurosciences & behavior
Author, co-author :
Vandenheede, Michel; Centre Hospitalier Régionale de la Citadelle (Liège) - CHR CITADELLE > Department of Neurology
Dioh, Alioune ; Centre Hospitalier Universitaire de Liège - CHU > Neurologie CHR
Baumann T.P., Hurwitz N., Karamitopolou-Diamantis E., Probst A., Herrmann R. (2000) Diagnosis and treatment of intravascular lymphomatosis. Arch. Neurol 57:374-377.
Bequet D., Guillamo J.S., Mokhtari K. (2000) Confrontation de la Salpêtrière. Syndrome confusionnel, troubles du langage, troubles moteurs des membres inférieurs à rechutes chez un homme de 48 ans. Rev. Neurol. (Paris) 156:821-827.
Billé J., Billé-Turc F., Lehmann G., Gambarelli D., Padovani R. (1995) Un cas anatomo-clinique de démence au cours d'un lymhome endovasculaire à grandes cellules. Rev. Neurol. (Paris) 151:576-579.
Bots G.Th.An. (1974) Angioendotheliomatosis of central nervous system. Acta Neuropathol. (Berl) 28:75-78.
Butori C., Perrin C.H., Chevallier A. (1997) Diagnosis of intravascular lymphoma on a muscular biopsy. Ann. Patho 17:340-342.
Croisile B., Tommasi M., Jouvet A., Truffert A., Trillet M. (1990) Lymphome malin endovasculaire à grandes cellules. Rev. Neurol. (Paris) 146:184-190.
Curtis J.L., Warnock M.L., Conrad D.J., Helfend L.K., Boushey H.A. (1991) Intravascular (angiotropic) large-cell lymphoma ("malignant angioendotheliomatosis") with small vessel pulmonary vascular obstruction and hypercalcemia. West. J. Med 155:72-76.
Demirer T., David H.D., Aboulafia D.M. (1994) Four varied cases of intravascular lymphomatosis and a literature review. Cancer 73:1738-1745.
DiGuiseppe J.A., Nelson W.G., Seifter E.J., Boiknott J.K., Mann R.B. (1994) Intravascular lymphomatosis : A clinicopathologic study of 10 cases and assessment of response to chemotherapy. J. Clin. Oncol 12:2573-2579.
Dolman C.L., Sweeney V.P., Magil A. (1979) Neoplastic angioendotheliomatosis: The case of the missed primary?. Arch. Neurol 36:5-7.
Dubas F., Saint-Andre J.P., Poulard Barthelaiz A., Delestre F., Emile J. (1990) Intravascular malignant lymphomatosis (so-called malignant angioendotheliomatosis): A case confined to the lumbosacral spinal cord and nerve roots. Clin. Neuropathol 9:115-120.
Domizio P., Hall P.K., Cotter F., Amiel S., Tucker J. (1989) Angiotropic large cell lymphoma (ALCL) : Morphological, immunohistochemical and genotypic studies with analysis of previous reports. Hematol. Oncol 7:195-206.
Glass J., Hochberg F., Miller D. (1993) Intravascular lymphomatosis: A systemic disease with neurologic manifestations. Cancer 71:3156-3164.
Hamada K., Hamada T., Satoh M., Tashiro K., Katoh I. (1991) Two cases of neoplastic angio-endotheliomatosis presenting with myelopathy. Neurology 41:1139-1140.
Harris C.P., Sigman J.D., Jaeckle K.A. (1994) Intravascular malignant lymphomatosis : Amelioration of neurologic symptoms with plasmapheresis. Ann. Neurol 35:357-359.
Krieger C., Robitaille Y., Jothy S. (1982) Intravascular malignant histiocytosis mimicking central nervous system vasculitis : An immunopathologic diagnostic approach. Ann. Neurol 12:489-492.
Kuvliev E., Glamour T., Shekar R., West B.C. (1999) Angiotropic large cell lymphoma presenting as fever of unknown origin. Am. J. Med. Sci 317:266-268.
Lacomis D., Smith T.W., Long R.R. (1992) Angiotropic lymphoma (intravascular large cell lymphoma) presenting with cauda equina syndrome. Clin. Neurol. Neurosurg 94:311-315.
Levin K.H., Lutz G. (1996) Angiotropic large-cell lymphoma with peripheral nerve and skeletal muscle involvement : Early diagnosis and treatment. Neurology 47:1009-1011.
Liow K., Asmar P., Liow M. (2000) Intravascular lymphomatosis : Contribution of cerebral MRI findings to diagnosis. J. Neuroimaging 10:116-118.
Liszka U., Drlicek M., Hitzenberger P. (1994) Intravascular lymphomatosis : A clinicopathological study of three cases. J. Cancer Res. Clin. Oncol 120:164-168.
Molina A., Lombard C., Donlon T. (1990) Immunohistochemical and cytogenetic studies indicate that malignant angioendotheliomatosis is a primary intravascular (angiotropic) lymphoma. Cancer 66:474-479.
Mori S., Takanashi M., Motoji T., Shiozaki H., Oshimi K. (1985) Neoplastic angioendotheliosis with B lymphocyte markers on neoplastic cells : A case report. Jpn. J. Cancer Res 76:208-212.
Nakahara T., Saito T., Muroi A., Sugiura Y., Ogata M. (1999) Intravascular lymphomatosis presenting as an ascending cauda equina : Conus medullaris syndrome : Remission after biweekly CHOP therapy. J. Neurol. Neurosurg. Psychiatry 67:403-406.
Ojeda V.J. (1983) Neoplastic angioendotheliosis of spinal cord. Case report. Acta Neuropathol. (Berl) 62:164-166.
Otrakji C.L., Voigt W., Amador A., Nadji M., Gregorios J.B. (1988) Malignant angioendotheliomatosis - A true lymphoma : A case of intravascular malignant lymphomatosis studied by Southern blot hybridisation analysis. Hum. Pathol 19:475-478.
Pellat J., Tillier J.N., Henin D., Gaulier A. (1993) Epilepsie tardive et syndrome démentiel d'évolution subaiguë chez un homme de 56 ans. Rev. Neurol. (Paris) 149:65-70.
Petito C.K., Geoffrey J., Gottlieb G.J. (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. Hautarz 10:359-363.
Prayson R.A. (1996) Angiotropic large cell lymphoma : Simultaneous peripheral nerve and skeletal muscle involvement. Pathology 28:25-27.
Rose C., Staumont D., Jouet J.-P. (1999) Successful autologous bone marrow transplantation in intravascular lymphomatosis. British Journal of Haematology 105:313-320.
Roux S., Grossin M., De Bandt M. (1995) Angiotropic large cell lymphoma with mononeuritis multiplex mimicking systemic vasculitis. J. Neurol. Neurosurg. Psychiatry 58:363-366.
Shanks J.H., Harris M., Howat A.J., Freemont A.J. (1997) Angiotropic lymphoma with endocrine involvement. Histopathology 31:161-166.
Shibuya A., Saito S., Tanaka S. (1983) A case of malignant angioendotheliomatosis : Association with malignant histiocytosis. Journal of Japanese Society of Reticuloendotherial System 23:197-205.
Sleater J.P., Segal G.H., Scott M.D. (1994) Intravascular (angiotropic) large cell lymphoma : Determination of monoclonality by polymerase chain reaction on paraffin-embedded tissues. Mod. Pathol 7:593-598.
Stahl R.L., Chan W., Duncan A. (1991) Malignant angioendotheliomatosis presenting as disseminated intravascular coagulopathy. Cancer 68:2319-2323.
Stroup R.M., Sheibani K., Moncada A., Purdy L.J., Battifora H. (1990) Angiotropic (intravascular) large cell lymphoma : A clinico-pathologic study of seven cases with unique clinical presentations. Cancer 66:1781-1788.
Suzuki S., Koizumi Y. (1997) Angiotropic lymphoma diagnosed by muscle biopsy. Intern. Med 36:304-307.
Vieren M., Sciot R., Robberecht W. (1999) Intravascular lymphomatosis of the brain : A diagnostic problem. Clinical Neurology and Neurosurgery 101:33-36.
Vital C., Heraud A., Coquet M., Julien M., Maupetit J. (1989) Acute mononeuropathy with angiotropic lymphoma. Acta Neuropatho. (Berl.) 78:105-107.
Waring W.S., Wharton S.B., Grant R., McIntyre M. (1999) Angiotropic large B-cell lymphoma with clinical features resembling subacute combined degeneration of the cord. Clinical Neurology and Neurosurgery 101:275-279.
Wick M.R., Stacey E.M. (1991) Intravascular lymphomatosis : Clinicopathologic features and differential diagnosis. Semin. Diagn. Pathol 8:91-101.
Wick M.R., Mills S.E., Scheithauer B.W., Cooper P.H., Davitz M.A. (1986) Reassessment of malignant "angioendotheliomatosis". Evidence in favor of its reclassification as "intravascular lymphomatosis". Am. J. Surg. Pathol 10:112-123.
Willemze R., Kruyswijk M.R.J., De Bruin C.D. (1987) Angiotropic (intravascular) large cell lymphoma of the skin previously classified as malignant angioendotheliomatosis. Br. J. Dermatol 116:393-399.