EBV-associated smooth muscle tumor

Nabeel Hamoui

Northwestern Memorial Hospital

Ellen C. Benya, MD

Children's Memorial Hospital

Abstract

Two year old with history of liver transplant and persistently elevated LFT's

Keywords

1563 Leiomyosarcoma EBV-associated-smooth muscle tumor (SMT), published in the Journal of Pediatric Gastroenterology and Nutrition, 2012


Publication Date: 2009-11-18

History

Two year old female with history of cryptogenic cirrhosis and end-stage liver disease s/p orthotopic liver transplantation.

Presents with persistently elevated LFTs and positive EBV PCR. Liver US ordered for further evaluation

Findings

US findings- Two well-defined, heterogenous, relatively hypoechoic lesions in the transplanted liver.

CT findings- New low density right posterior and left medial hepatic lobe lesions with relatively ill-defined margins and no definite peripheral rim enhancement. There is also stable splenomegaly.

PET/CT- Increased metabolic activity in both liver lesions

Diagnosis

Epstein-Barr virus (EBV) associated smooth muscle tumor (SMT)

DDx

EBV associated SMT, PTLD, abscess/phlegmon

Discussion

General Features:

  • Rare but recognized disease found primarily in immunocompromised patients (e.g. AIDS, post-transplant patients)
  • Identical appearance to leiomyosarcoma (diagnosis is based on clinical history)

Etiology:

  • Association between EBV and post-transplant smooth muscle tumors has been documented and appears to be similar to the development of lymphoproliferative disorders, wherein EBV-infected T lymphocytes induce B-lymphocyte transformation
  • EBV has a direct role in the development of smooth muscle tumors and that Epstein–Barr nuclear antigen-2 (EBNA-2) protein is consistently expressed in smooth muscle cells in immunocompromised individuals. EBV has not been implicated in leiomyosarcoma (LMS) arising in immunocompetent individuals, indicating that sporadic LMS may be biologically different from the LMS arising in the setting of EBV infection and immunodeficiency.

Epidemiology:

  • Very rare overall, with prevalence of 0.1-0.5% in immunocompromised patients
  • Children and adolescents are the population of predilection
  • EBV-SMT are rarely diagnosed before 3 years post surgery

Treatment:

References

  1. Deyrup AT, et al. Epstein-Barr virus-associated smooth muscle tumors are distinctive mesenchymal tumors reflecting multiple infection events: a clinicopathologic and molecular analysis of 29 tumors from 19 patients. Am J Surg Pathol. 2006 Jan;30(1):75-82.
  2. Norton KI, et al. Leiomyosarcoma of the kidney in an HIV-infected child. Pediatr Radiol. 1997; 27: 557-558.
  3. Rogatsch H, et al. Epstein-Barr virus-associated multicentric leiomyosarcoma in an adult patient after heart transplantation: case report and review of the literature. Am J Surg Pathol. 2000 Apr;24(4):614-21.
  4. Smets BB, et al. Unusual evolution of an EB virus-associated leiomyosarcoma occurring after liver transplantation. Pedatr Transplant 2001; 5: 365-369.
  5. Timmon CF, et al. Epstein-Barr virus-associated leiomyosarcomas in liver transplantation recipients. Origin from either donor or recipient tissue. Cancer. 1995 Oct 15;76(8):1481-9.

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