Primary Cutaneous Giant Cell Plasmacytoma in an Organ Transplant Recipient: A Rare Presentation of a Posttransplant Lymphoproliferative Disorder

Abstract

Posttransplant lymphoproliferative disorder (PTLD) is comprised of a spectrum of lymphoid diseases, ranging from early lesions, such as plasmacytic hyperplasia, to monomorphic neoplasms, including plasmacytoma-like lesions. Although PTLD may involve a variety of organs, primary cutaneous PTLD is rare. We report a unique case of Epstein-Barr virus (EBV)-positive primary cutaneous giant cell plasmacytoma developed 5 years after renal/pancreatic transplant in a 55-year-old male patient. The patient received local radiotherapy without reduction in immunosuppression and responded well. A review of the literature identified additional 49 cases of primary cutaneous B-cell PTLD, including 18 cases of plasmacytoma-like lesions. Primary cutaneous B-cell PTLD usually presents years after transplantation, has male preponderance, tends to occur on extremities, is frequently EBV-associated, and predicts a favorable clinical outcome. Unlike PTLD in general, in which EBV-positive cases usually occur earlier than EBV-negative ones, the longer presentation interval in the cutaneous PTLD seems to be uncorrelated to EBV status. Compared with other subtypes of cutaneous B-cell PTLD, plasmacytoma-like lesions have an increased male preponderance and tendency to present on the extremities. Although the majority of cases have been treated with reduction of immunosuppression, antiviral therapy and/or local radiotherapy, and a few with chemotherapy, the best therapeutic intervention for primary cutaneous B-cell PTLD remains to be further investigated with the analysis of more reported cases and large clinical trials.

Cutaneous plasmacytoma-like posttransplant lymphoproliferative disorder after renal transplantation with response to imiquimod 5% cream and reduced immunosuppression

“…Primary cutaneous plasmacytoma-like PTLD is extremely rare, with fewer than 20 published cases in the literature 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13. This scarcity of information poses prognostic and therapeutic uncertainty.…”

“…Most reported cases of plasmacytoma-like PTLD presented as nodules in the skin 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13. Very few cases manifested as erythematous plaques or ulcerations, and the majority were EBV + 3 , 7 .…”

Pathologic Diagnosis of Cutaneous Lymphomas

“…134 Posttransplant B-cell lymphoproliferative disorders represent a spectrum of lymphoid tissue disease, ranging from early lesions, such as plasmacytic hyperplasia, to monomorphic neoplasm. 136 They usually manifest years after transplantation, are frequently EBV linked, and are associated with a favorable clinical outcome. 136 Methotrexate-associated B-cell lymphoproliferative disease presents with ulcerating or generalized skin lesions.…”

“…136 They usually manifest years after transplantation, are frequently EBV linked, and are associated with a favorable clinical outcome. 136 Methotrexate-associated B-cell lymphoproliferative disease presents with ulcerating or generalized skin lesions. Histologically, it shares features with PCDLBCL-LT. 137 In cases with a reduced staining for CD79a and marked tumor cell polymorphism, this diagnosis should be taken into account.…”

Cutaneous Lymphomas

“…Fifty cases of primary cutaneous B-cell posttransplant lymphoproliferative disorder were recently reviewed. 73 They usually manifest several years after transplantation, show a male preponderance, tend to occur on extremities, are frequently EBV associated, and were associated with a favorable clinical outcome. Histologically, some of these cases show plasmacytomalike features.…”