Multifocal Histologically Malignant Epstein–Barr Virus–Associated Smooth Muscle Tumor in a Pediatric Transplant Patient With an Indolent Course

Epstein–Barr virus–associated smooth muscle tumors (EBV-SMTs) are rare lesions that occur in immunocompromised patients. Dural involvement appears to be less common in organ transplant recipients than in HIV patients. Due to the paucity of reported cases following organ transplantation, the natural...

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Bibliographic Details
Published in:International journal of surgical pathology 2014-04, Vol.22 (2), p.186-189
Main Authors: Kazmi, Syed A. Jaffar, Aizenberg, Michele R., Harper, James L., McComb, Rodney D.
Format: Article
Language:English
Subjects:
Child
Epstein-Barr Virus Infections - pathology
Female
Herpesvirus 4, Human - isolation & purification
Human immunodeficiency virus
Humans
Immunocompromised Host
Kidney Transplantation
Muscle Neoplasms - pathology
Muscle Neoplasms - surgery
Muscle Neoplasms - virology
Muscle, Smooth - pathology
Muscle, Smooth - surgery
Treatment Outcome
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Summary:Epstein–Barr virus–associated smooth muscle tumors (EBV-SMTs) are rare lesions that occur in immunocompromised patients. Dural involvement appears to be less common in organ transplant recipients than in HIV patients. Due to the paucity of reported cases following organ transplantation, the natural history of these lesions is unclear. We describe an 8-year-old female who presented with adrenal, small bowel, and intracranial tumors 6 years following renal transplantation. Histopathological analysis revealed a highly cellular, mitotically active, smooth muscle neoplasm without necrosis. The tumor stained diffusely for smooth muscle actin and myosin. In situ hybridization for EBV-encoded RNA was diffusely positive. Following gross total resection, antiviral therapy, and a reduction in immunosuppression, the patient is tumor-free at 3 years follow-up. In patients with compromised immune systems, it is important to recognize this unique form of SMT because, even when there are multiple lesions, the prognosis may be excellent.
ISSN:1066-8969
1940-2465
DOI:10.1177/1066896913494793