Epstein-Barr Virus–Associated Smooth Muscle Tumor of the Iris in a Patient With Transplant: A Case Report and Review of the Literature

Abstract Epstein-Barr virus infection has been linked to the development of smooth muscle tumors in immunocompromised patients with organ transplants and acquired immunodeficiency syndrome. A 52-year-old female recipient of a renal transplant presented with enlarging masses of the left iris. Incisional biopsy of the mass revealed a smooth muscle tumor of the iris. Epstein-Barr virus infection was confirmed by in situ hybridization for Epstein-Barr virus–encoded, small RNA in tumor cells. Eight months after total iridectomy the patient was free of disease. Although the prognosis and classification of Epstein-Barr virus–associated smooth muscle tumors are controversial, mortalities caused by these tumors are rare.

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Epstein-Barr Virus–Associated Smooth Muscle Tumor

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2015-0120-rs ◽

2016 ◽

Vol 140 (7) ◽

pp. 718-722 ◽

Cited By ~ 22

Author(s):

Jyoti Dekate ◽

Runjan Chetty

Keyword(s):

Smooth Muscle ◽

Virus Infection ◽

Epstein Barr Virus ◽

Smooth Muscle Tumor ◽

Epstein Barr Virus Infection ◽

Barr Virus ◽

Epstein Barr ◽

Barr Virus Infection ◽

Uncommon Neoplasm ◽

Muscle Tumor

Immunodeficient individuals are prone to develop a number of opportunistic infections and unique neoplasms. Epstein-Barr virus–associated smooth muscle tumor is an uncommon neoplasm associated with immunodeficiency. It has been described in patients infected with human immunodeficiency virus, in the posttransplant setting, and in those with congenital immunodeficiency. Different anatomic sites can be involved by Epstein-Barr virus–associated smooth muscle tumor, and even multiple locations can contain these unique lesions within the same patient. The presence of variable numbers of intratumoral lymphocytes and primitive round cell areas are the unique defining features for this tumor. Histopathologic features may vary considerably in terms of cellular atypia, mitotic activity, and necrosis, with no correlation to the clinical behavior. Demonstration of Epstein-Barr virus infection by in situ hybridization within tumor cell remains critical for the diagnosis. The mechanism for Epstein-Barr virus infection of progenitor cells and neoplastic transformation has been an area of interest and conjecture. Different treatment strategies are proposed according to underlying disease status. This paper reviews the clinicopathologic features of this uncommon neoplasm with detailed discussion of the role of Epstein-Barr virus in the pathogenesis.

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Epstein-Barr Virus Infection-Associated Smooth-Muscle Tumors in Patients with AIDS

Clinical Infectious Diseases ◽

10.1086/429830 ◽

2005 ◽

Vol 40 (10) ◽

pp. 1521-1528 ◽

Cited By ~ 57

Author(s):

C. Suankratay ◽

S. Shuangshoti ◽

A. Mutirangura ◽

V. Prasanthai ◽

S. Lerdlum ◽

...

Keyword(s):

Smooth Muscle ◽

Virus Infection ◽

Epstein Barr Virus ◽

Epstein Barr Virus Infection ◽

Barr Virus ◽

Smooth Muscle Tumors ◽

Epstein Barr ◽

Barr Virus Infection

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Epstein-Barr Virus-Associated Smooth Muscle Tumor of the Basal Ganglia in an HIV+ Child: Case Report and Review of the Literature

Pediatric and Developmental Pathology ◽

10.1007/s10024-003-7079-2 ◽

2004 ◽

Vol 7 (2) ◽

pp. 198-203 ◽

Cited By ~ 28

Author(s):

Shimareet Kumar ◽

Mariarita Santi ◽

Gilbert Vezina ◽

Tena Rosser ◽

Roma S. Chandra ◽

...

Keyword(s):

Smooth Muscle ◽

Basal Ganglia ◽

Epstein Barr Virus ◽

Smooth Muscle Tumor ◽

Barr Virus ◽

Smooth Muscle Tumors ◽

Immunodeficiency Virus ◽

Epstein Barr ◽

Mass Lesions ◽

Muscle Tumor

We describe the clinicopathologic features of an Epstein-Barr virus (EBV)-associated smooth muscle tumor arising in the basal ganglia of a 10-year-old human immunodeficiency virus (HIV)-positive child. Only a few cases of intracranial smooth muscle tumors are reported in the literature and virtually all of these have been extra-axial, involving the dura or sinuses in HIV+ adults. Our case underscores the need to include an EBV-associated smooth muscle tumor in the differential diagnosis when evaluating intracranial mass lesions in immunodeficient children.

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