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HHV8/EBV-associated germinotropic lymphoproliferative disorder (GLD) is a challenging diagnosis given its rarity, the particular clinical presentation, and the lack of expression of markers usually used in establishing hematopoietic lineage. We report a new case of HHV8/EBV GLD in an immunocompetent 78-year-old woman. The diagnosis was made in an incidentally discovered lymphadenopathy. Histological examination showed a nodular lymphoid proliferation centered by aggregates of atypical plasmablastic cells admixed with small lymphoid cells. Tumor cells were strongly positive with EMA, HHV8, LMP1, CD38, CD138, and kappa light chains. They were negative with common lymphoma-associated markers (CD20, CD3, CD15, CD30, CD10, and bcl2). In situ hybridization confirmed the monotypic kappa light chains and the EBV infection (EBER+). A polyclonal pattern of Ig gene rearrangement was detected by PCR analysis. In the adjacent lymph node parenchyma, some germinal centers mimicked Castleman disease. In this case, the differential diagnosis was discussed with an early stage of large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease. The clinical presentation, the immunophenotype, and the molecular results helped to make the accurate diagnosis. Through the review of the nine previously reported cases in literature, we discuss the clinical and pathologic features and the differential diagnosis of HHV8/EBV GLD.

Highlights Isolated pancreatic metastasis from renal cell carcinoma is relatively rare and it's usually seen in clear cell renal cell carcinoma (CCRCC), but its occurrence from chromophobe renal cell carcinoma is extremely rare. Pancreatic location is often diagnosed during routine surveillance imaging for the primary lesion. Patients in whom the pancreas is the only metastatic site and who are fit enough to undergo pancreatic surgery appear to be good candidates for the surgical treatment chemotherapy can improve the quality of life but not survival. It is necessary a long- term follow-up for patients treated for tumors with known low-grade metastatic potential and relatively good prognosis such as chromophobe renal cell carcinoma.

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