Multifocal Epstein-Barr virus-associated smooth muscle tumor in adults with AIDS: case report and review of the literature.
OBJECTIVE
The aim of this study was to provide a systematic review of Epstein-Barr virus-associated smooth muscle tumors (EBV-SMT) in human immunodeficiency virus (HIV)-infected adults, focusing on clinical and histopathologic features and outcome.


METHODS
A literature search was performed using Medline, Embase and the Cochrane Library.


RESULTS
We reviewed 35 cases including our case of a patient with a progressive multifocal EBV-SMT. Patients were mainly men (n = 24) with a mean age of 35.5 years. Median CD4 count was 21/mm(3). Main locations were brain (n = 12), liver (n = 8), spinal cord (n = 7) and adrenal gland (n = 6). The tumors were multifocal in 34% of cases, whereas analysis of clonality showed different clones in tumors from different sites. Treatment included removal surgery in 17 cases and/or radiotherapy in 9 and therapeutic abstention in 4. Mean follow-up after diagnosis was 12.3 months. Nine patients died during this period essentially from opportunistic infection and only 2 from the disease.


CONCLUSIONS
EBV-SMT should be added to the list of virally induced tumors in severely immunocompromised HIV-infected adults. Multifocality of independent tumor clones, especially in liver, brain, spinal cord and adrenal gland, and a slow disease progression seem to be the key features of these tumors, the treatment of which remains poorly defined.
Gallien
S
S
Department of Infectious Disease, Saint Louis Hospital and University Paris 7, Paris, France. sebastien_gallien@yahoo.fr
Zuber
B
B
Polivka
M
M
Lagrange-Xelot
M
M
Thiebault
J-B
JB
Bertheau
P
P
Gray
F
F
Molina
J-M
JM
D017088:AIDS-Related Opportunistic Infections / Q000473:pathology / Q000821:virology*; D000328:Adult; D018791:CD4 Lymphocyte Count; D020031:Epstein-Barr Virus Infections / Q000473:pathology / Q000628:therapy / Q000821:virology*; D015658:HIV Infections / Q000235:genetics / Q000821:virology; D015497:HIV-1 / Q000235:genetics / Q000302:isolation & purification; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000302:isolation & purification; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D012367:RNA, Viral / Q000235:genetics; D012509:Sarcoma / Q000473:pathology / Q000628:therapy / Q000821:virology*; D018235:Smooth Muscle Tumor / Q000473:pathology / Q000628:therapy / Q000821:virology*; D014057:Tomography, X-Ray Computed; D016862:Tuberculoma, Intracranial / Q000175:diagnosis; D014376:Tuberculosis / Q000150:complications / Q000175:diagnosis / Q000628:therapy
D012367:RNA, Viral