Human immunodeficiency virus-related Epstein-Barr virus-associated smooth muscle tumours: South African experience from Chris Hani Baragwanath Academic Hospital.

Autor: Pather, Sugeshnee, Wainwright, Rosalind D., Sahid, Faieza, Mashele, Thembi, van den Berg, Eunice J., Mohanlal, Reena D.
Předmět:
Zdroj: Southern African Journal of Infectious Diseases; Dec2017, Vol. 32 Issue 4, p115-118, 4p, 4 Color Photographs, 1 Chart
Abstrakt: Background: Despite the rampant human immunodeficiency (HIV) epidemic in Africa, there is a paucity of published data of HIV-related Epstein-Barr virus-associated smooth muscle tumours (EBV-SMT) from Africa. Methods: We embarked on retrospective documentation of the clinicopathological features of confirmed HIV-related EBV-SMT over a 5-year time frame at the largest hospital in Africa. All haematoxylin and eosin stained tissue sections, immunohistochemistry and EBVin situhybridisation (ISH) investigations were reviewed in conjunction with clinical data. Results: Fourteen (n= 14) EBV-SMT were confirmed in 13 patients (age range: 10–53 years). Five paediatric patients and a predominance of females (70%) were evident in this series. All patients were HIV seropositive and CD4 counts ranged from 1 to 1331 cells/ul (median 355 cells/ul; mean 442 cells/ul). Tumour-associated pain was a common symptom in the paediatric age group, while neurological symptoms were frequent in the adults due to paraspinal cervicothoracic involvement. Unusual topography, multifocality (n = 5) and smooth muscle morphology in association with round cell features (n = 3) were evident. Immunoexpression of desmin (n = 12), SMA (n = 12) and h-Caldesmon (n = 8) were consistent findings and positive EBV ISH nuclear signaling was demonstrated within all of these tumours. Treatment included antiretroviral therapy, surgical resection, radiation and/or palliative therapy. Conclusion: HIV-associated EBV-SMT are rare tumours that may develop in paediatric or adult patients. A female predominance and multifocal topographic involvement may be evident. AIDS-related co-morbidities are likely to contribute to mortality; and, when these tumours occur in paraspinal regions, debilitating neurological morbidity may manifest. [ABSTRACT FROM PUBLISHER]
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