Multiple Epstein-Barr virus-associated subcutaneous angioleiomyomas in a patient with acquired immunodeficiency syndrome

Abstract

Tumours of smooth muscle origin, either solitary or multiple, are occasionally found in immunocompromised patients, particularly in children with acquired immunodeficiency syndrome (AIDS). Most of the reported AIDS-associated leiomyomatous neoplasms have been found in the visceral organs, and the tumour cells all possessed the Epstein-Barr virus (EBV) genome. Here we present a 32-year-old-man with AIDS who developed three skin nodules on his lower left extremity. No other tumorous lesions were found using computed tomography scans. Two of the three nodules were resected for pathological examination. Histologically, both tumours were well circumscribed and located in the subcutis. The tumours were composed of interlacing fascicles of spindle-shaped cells with prominent vasculature and lymphocytic infiltration. No pleomorphism, mitosis or necrosis was seen. Immunohistochemically, the tumour cells were reactive to smooth muscle actin and desmin. Angioleiomyoma was diagnosed. EBV-encoded small RNAs were also demonstrated in the nucleus of the tumour cells by in situ hybridization but no EBV receptor (CD21) or latent membrane protein (LMP)-1 was found in the tumour cells. No human herpesvirus (HHV)-8 genome was detected in the lesion using polymerase chain reaction analysis. The results of this study indicated that EBV containing subcutaneous angioleiomyoma was another neoplasm that must be considered in patients with human immunodeficiency virus infection who develop skin nodules. The role of EBV in the pathogenesis of this unique neoplasm is still unknown.

AIDS-Related EBV-Associated Smooth Muscle Tumors: A Review of 64 Published Cases

“…Some cases described a mild to moderate increase in cellularity and a second population of small round cells with irregular nuclear contours that displayed a smooth muscle phenotype [26, 29, 48]. Other features that have been described include a hemangiopericytic pattern with dilated and branching capillaries and chronic inflammatory cells including intratumoral T lymphocytes [22, 26, 29, 35, 36, 48]. In some cases, tumors appeared to originate from vessel walls, which the authors believed might explain the multiplicity of these tumors [26, 48].…”

Extra-uterine myoid tumours in patients with acquired immunodeficiency syndrome: a clinicopathological reappraisal

“…They are documented often, but not always, in association with Epstein–Barr virus (EBV) infection 4–6 . Occurring in unusual anatomical locations, these EBV‐associated MTs (EBV‐positive MTs) are also typified by multifocality, phenotypic heterogeneity and intratumoral T cell lymphocytic infiltration 7–13 . Since the original discovery, the classification of EBV‐positive smooth muscle tumours (EBV‐positive SMTs) has been plagued by ill‐defined and inconsistent diagnostic criteria 14 .…”

Infratentorial angioleiomyoma: a new location for a rare neoplastic entity

“…The association of these tumors with immunodeficiency, in particular, AIDS and EpsteinBarr virus, deserves consideration. 2 Although the HIV status of our patient is unknown, his immunohis tochemical reaction for EpsteinBarr virus was negative. Antibody testing for these viral entities should probably be considered once a diagnosis has been confirmed.…”