Epstein-Barr virus-associated smooth muscle tumor of the tonsil
ABSTRACT Smooth muscle tumors of the tonsil are rare. Recently, the occurrence of Epstein-Barr virus-associated smooth muscle tumor (EBV-SMT) has been increasingly recognized in immunocompromised patients, mainly post-transplantation and AIDS patients. The clinicopathologic features of EBV-SMT are different from conventional smooth muscle tumors. To the best of our knowledge, EBV-SMT involving the tonsil in an AIDS patient has not been reported. A 27-year-old man presented with a 2.2cm right tonsillar mass six months after AIDS diagnosis. The tumor was composed of a cellular proliferation of oval to spindle-shaped cells with mitotic count up to 10 in 10 high-power fields. The diagnosis of EBV-SMT was confirmed by in situ hybridization for EBV-encoded RNA (EBER) transcripts. Synchronous lesions were also detected in the liver and peritoneum by an abdominal computed tomographic scan. EBV-SMT should be included in the differential diagnoses of a mesenchymal tumor in immunocompromised patients, and in the differential diagnoses of a smooth muscle tumor occurring in uncommon sites including the tonsil.
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ABSTRACT: Rare Epstein–Barr virus (EBV)+ smooth muscle tumours (SMT) manifest typically under immunosuppression. Three major subtypes are known: human immunodeficiency virus-associated (HIV-SMT), after transplantation (PTSMT) or associated with congenital immunodeficiency syndromes (CI-SMT). So far, there are no analyses which compare the clinico-pathological characteristics of all three subtypes. Case reports and case series on these three tumour types were collected (1990–2012). Meta-data analysis was performed for identification of similarities and differences. A total of 73 HIV-SMT, 66 PTSMT and 9 CI-SMT were evaluated. There was a slight female predominance (55–67%). Children were affected nearly equally in HIV-SMT (33%) and PTSMT (35%), while all CI-SMT occurred in children. HIV-SMT manifested preferentially in the central nervous system, gut/liver, skin, lungs/larynx/pharynx and adrenal glands. PTSMT were predominantly found in the liver, lungs/larynx/pharynx, gut/spleen and brain. CI-SMT were often found in lungs/larynx, brain, liver, adrenal glands and spleen. Antecedent EBV+ lymphoproliferations manifested more often in PTSMT. In all three tumour subtypes, survival analyses did not show any significant differences regarding surgical therapeutic approaches, the occurrence of multiple tumours, tumour size or sarcoma-like histological features. HIV-SMT had the poorest overall survival, which might be attributed to HIV-associated infectious complications.European Journal of Cancer 09/2014; 50(14). DOI:10.1016/j.ejca.2014.06.006 · 4.82 Impact Factor