7 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)186-189
Number of pages4
JournalInternational Journal of Surgical Pathology
Volume22
Issue number2
DOIs
StatePublished - Apr 2014

Fingerprint

Smooth Muscle Tumor
Human Herpesvirus 4
Pediatrics
Transplants
Muscle Neoplasms
Smooth Muscle Myosins
Neoplasms
Immunocompromised Host
Organ Transplantation
Natural History
Kidney Transplantation
Immunosuppression
In Situ Hybridization
Antiviral Agents
Smooth Muscle
Actins
Immune System
Necrosis
HIV
RNA

Keywords

  • EBV
  • dural tumor
  • pediatric tumor
  • smooth muscle tumor
  • transplant

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

Cite this

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title = "Multifocal histologically malignant epstein-barr virus-associated smooth muscle tumor in a pediatric transplant patient with an indolent course",
abstract = "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.",
keywords = "EBV, dural tumor, pediatric tumor, smooth muscle tumor, transplant",
author = "Kazmi, {Syed A Jaffar} and {Aizenberg Ansari}, {Michele R} and Harper, {James Lloyd} and McComb, {Rodney D}",
year = "2014",
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T1 - Multifocal histologically malignant epstein-barr virus-associated smooth muscle tumor in a pediatric transplant patient with an indolent course

AU - Kazmi, Syed A Jaffar

AU - Aizenberg Ansari, Michele R

AU - Harper, James Lloyd

AU - McComb, Rodney D

PY - 2014/4

Y1 - 2014/4

N2 - 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.

AB - 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.

KW - EBV

KW - dural tumor

KW - pediatric tumor

KW - smooth muscle tumor

KW - transplant

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