Epstein-Barr virus-associated adrenal smooth muscle tumors and disseminated diffuse large B-cell lymphoma in a child with common variable immunodeficiency: a case report and review of the literature.

摘要: This article reports the clinical and histological features in a 7-year-old girl affected by common variable immunodeficiency (CVID) who developed multiple Epstein-Barr virus-associated tumors, represented bilateral adrenal smooth muscle tumors (EBV-SMT) multifocal diffuse large B-cell lymphoma. The EBV-SMTs showed compatible with benign or at least low-malignant potential neoplasm. A peculiar feature observed both was occurrence of numerous lymphocytes intermingled spindle cells, which consisted CD3+ CD5+ T-cells, predominant cytotoxic CD8+ component. Interestingly, EBV status differed neoplasms, since were negative for LMP1 positive EBER, whereas lymphoma expressed markers. Furthermore, EBV-LMP1 deletion only EBV-SMTs, thus indicating that these consequence 2 distinct, EBV-dependent transformations. Similarly, lymphocyte clonality assay also different clonal bands sites (skin nasal cavity), suggesting development intratumoral mutations. Finally, authors review all 127 previously reported EBV-SMT, discussion their pathological features.