EBV-positive low-grade marginal zone lymphoma in the breast with massive amyloid deposition arising in a heart transplant patient: A report of an unusual case.

According to the 2008 World Health Organization classification, low-grade lymphomas arising in transplant recipients are not considered as specific types of PTLD. Most such cases are not associated with EBV infections, although rare reports of post-transplant marginal zone lymphoma have been described. We describe the case of an 18-yr-old female with history of heart transplant who developed a breast mass, but was otherwise completely asymptomatic. Surgical excision of the mass and histopathologic examination showed a low-grade B-cell lymphoma most consistent with marginal zone lymphoma with massive amyloid deposition; furthermore, numerous tumor cells were positive for EBV by in situ hybridization for EBV-encoded RNA. The patient was treated with reduction in immunosuppression, and no additional lesions developed. This case describes an atypical presentation of post-transplant low-grade B-cell lymphoma, unusual in its location, histopathologic features, and association with EBV, thereby adding to the rare previous accounts of such an entity, suggesting the need to include post-transplant marginal zone lymphomas in the current classification of PTLD, and helping in determining the optimal treatment modalities for such tumors.