Final Diagnosis -- Posttransplant Lymphoproliferative Disease, Burkitt's Lymphoma-like (Left Neck Mass)


FINAL DIAGNOSIS:

Left neck mass, excision:
POST-TRANSPLANT LYMPHOPROLIFERATIVE DISEASE,
MONOMORPHOUS-TYPE.

NOTE: The histology resembles that of a malignant lymphoma, small non-cleaved cell, Burkitts-like.

CONTRIBUTOR'S NOTE:

Post-transplant lymphoproliferative disorders (PTLD) are generally Epstein-Barr virus associated lymphoid expansions with a variety of histologic appearances, ranging from lymph node hyperplasia resembling infectious mononucleosis to monoclonal neoplasms. The monoclonal varieties are usually not classifiable according to the accepted typologies of non-Hodgkin's lymphoma, but occasionally PTLD will manifest as a lymphoma histologically andcontain a genetic abnormality characteristic of the equivalent non-PTLD lymphoma. Unlike many cases of PTLD, such lesions do not regress upon withdrawal of immunosuppression.

The current case is an example of such a lesion. The tumor has the histologic appearance of Burkitt's lymphoma, and a chromosomal translocation was found which is typical of conventional Burkitt's lymphoma. The translocation, t(8;14), juxtaposes portions of the MYC oncogene at 8q24 and the immunoglobulin heavy chain gene at 14q32. The patient was treated with chemotherapy appropriate for Burkitt's lymphoma, and is currently alive without evidence of residual tumor.

REFERENCES:

Nalesnik MA, Jaffe R, Starzl TE, Demetris AJ, Porter K, Burnham JA, Makowka L, Ho M, Locker J. The pathology of posttransplant lymphoproliferative disorders occurring in the setting of cyclosporine A-prednisone immunosuppression. Am J Pathol 1988;133:173-192.

Locker J, Nalesnik M: Molecular genetic analysis of lymphoid tumors arising after organ transplantation. Am J Pathol 1989;135:977-987.

Swerdlow SH. Post-transplant lymphoproliferative disorders: A morphologic, phenotypic and genotypic spectrum of disease. Histopathol 1992;20:373-385.

Knowles DM, Cesarman E, Chadburn A, Frizzera G, Chen J, Rose EA, Michler RE. Correlative morphologic and molecular genetic analysis demonstrates three distinct categories of posttransplantation lymphoproliferative disorders. Blood 1995;85:552-565.

Contributed by Paul S. Dickman, M.D., Lance Cohen, M.D., and Charles A. Richert, M.D.


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