Epstein-Barr virus-associated leiomyosarcoma with cutaneous involvement in an African child with human immunodeficiency virus: a case report and review of the literature

Abstract

Epstein-Barr virus (EBV)-associated smooth muscle tumors (SMTs) are infrequently encountered lesions restricted to immunocompromised patients. However, they represent the second most common tumor in children with human immunodeficiency virus (HIV) infection. We report a case of a progressively enlarging abdominal mass with cutaneous involvement in an HIV-infected, 4-year-old African girl in Malawi with clinical acquired immunodeficiency syndrome on highly active antiretroviral therapy. Analysis of an excisional specimen revealed a well-differentiated leiomyosarcoma and subsequent studies revealed diffuse nuclear positivity for Epstein-Barr virus early RNAs in lesional cells. We present a report of this case and provide a summary of the literature regarding SMTs in pediatric HIV-infected patients. In addition, we draw attention to the cutaneous manifestations of SMTs in immunosuppressed populations. We highlight EBV-related SMTs as a potential cutaneous complication of HIV infection in children and immunosuppressed patients populations as a whole.

Contemporary management and classification of hepatic leiomyosarcoma

“…13 The single case of EBV leiomyosarcoma in this series illustrates typical features of this disease; it is a rare tumour 14 characterized by its development following a period of immunosuppression. This occurs in two settings in, respectively, patients with human immunodeficiency virus (HIV) infection [15][16][17] and in those with immunosuppression following solid organ transplantation. [18][19][20][21][22][23] Immunosuppression appears to be the key regulator of leiomyosarcoma EBV disease progression.…”

Epstein-Barr Virus–Associated Smooth Muscle Tumor

“…4,16 These observations have led to the proposal of a model suggesting that EBV infects the smooth muscle cells directly by attaching to CD21 and thereby facilitating and promoting replication within these cells. 17 However, in cases of PT-SMT, examination for CD21 has yielded negative results, suggesting an alternative, as yet unidentified route of EBV integration. 3,18,19 In such cases, fusion of smooth muscle cells with an infected B cell prior to tumor proliferation has been postulated.…”

Epstein-Barr virus associated hepatic smooth muscle tumor in a patient with acquired immunodeficiency syndrome

“…[ 9 , 10 ] This role is consistent with the roles of viruses in other associations between opportunistic oncogenic infections and cancers, including correlations between HHV-8 and Kaposi's sarcoma; EBV and non-Hodgkin lymphomas as well as nasopharyngeal carcinoma; human papillomavirus (HPV) and anogenital cancers; and hepatitis B/C and liver cancers. [ 3 , 11 ] In contrast, EBV is rarely associated with SMTs in immunocompetent patients. HIV-SMT multiplicity is common, with an inclination to the CNS, including cranial and spinal epidural locations, and can arise in extra-CNS locations such as the liver, lung, vocal cords and adrenal glands.…”

“…[ 2 ] Such tumours must be differentiated from various lesions with the same appearance, including Kaposi's sarcoma, myopericytoma, and gastrointestinal stromal cell tumour (GIST). [ 3 ] With respect to therapy, surgical resection is the main treatment. In patients with immunodeficiency, improving immune status is significant for defending against other viruses.…”