Epstein-Barr Virus-Associated Adrenal Smooth Muscle Tumors andDisseminated Diffuse Large B-Cell Lymphoma in a Child With Common Variable Immunodeficiency: A Case Report and Review of the Literature
| Autor: | Alessandro Ubiali, Giulia Petrilli, Richard Fabian Schumacher, Paolo Cabassa, Roberto Paracchini, Fabio Facchetti, Luisa Lorenzi |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2014 |
| Předmět: |
Pathology
medicine.medical_specialty Epstein-Barr Virus Infections Herpesvirus 4 Human Lymphocyte Adrenal Gland Neoplasms Biology medicine.disease_cause Pathology and Forensic Medicine Fatal Outcome hemic and lymphatic diseases medicine Neoplasm Humans Child Smooth Muscle Tumor Common variable immunodeficiency medicine.disease Epstein–Barr virus Lymphoma medicine.anatomical_structure Common Variable Immunodeficiency Surgery Female Lymphoma Large B-Cell Diffuse Anatomy CD5 Diffuse large B-cell lymphoma CD8 |
| Popis: | This article reports the clinical and the histological features in a 7-year-old girl affected by common variable immunodeficiency (CVID) who developed multiple Epstein–Barr virus–associated tumors, represented by bilateral adrenal smooth muscle tumors (EBV-SMT) and multifocal diffuse large B-cell lymphoma. The EBV-SMTs showed features compatible with a benign or at least a low-malignant potential neoplasm. A peculiar feature observed in both EBV-SMTs was the occurrence of numerous lymphocytes intermingled with the spindle cells, which consisted of CD3+ CD5+ T-cells, with a predominant cytotoxic CD8+ component. Interestingly, EBV status differed in the neoplasms, since the EBV-SMTs were negative for LMP1 and positive for EBER, whereas the B-cell lymphoma expressed both EBV markers. Furthermore, EBV-LMP1 deletion was positive only in the EBV-SMTs, thus indicating that these tumors were the consequence of 2 distinct, EBV-dependent transformations. Similarly, lymphocyte clonality assay also showed different clonal bands in different sites (skin and nasal cavity), suggesting the development of intratumoral mutations. Finally, the authors review all 127 previously reported EBV-SMT, with discussion of their clinical and pathological features. |
| Databáze: | OpenAIRE |
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