Overall Management > Uncommon, Morbid Complications

Meeting Abstract

Human immunodeficiency virus (HIV) associated smooth muscle tumors with central nervous system (CNS) involvement: A report of four cases and review of the literature

A. M. Demopoulos, L. Abrey, M. L. Rosenblum, R. J. O'Reilly, M. Bilsky, J. Raizer

Memorial Sloan-Kettering Cancer Center, New York, NY

Patients with Human Immunodeficiency virus (HIV) are at increased risk for the development of certain neoplasms, including Kaposi's sarcoma and high grade B-cell non-Hodgkin's lymphoma (NHL).
Leiomyoma (LM) and leiomyosarcoma (LMS) are frequent complications of HIV infection in children, but are rare in adults.
In the pediatric population, these tumors typically involve the lungs or gastrointestinal tract, and CNS involvement is unusual.
We report four cases of EBV-associated LM/LMS in adult patients with immunodeficiency syndromes (three with AIDS and one organ transplant recipient) and review clinical characteristics of the 11 patients previously reported in the literature.
In the fifteen HIV positive adult CNS LM/LMS tumors reported to date, there were six women and the median age was 35.
The median CD4 count was 23, with a range of 2 - 125 cells/uL.
Most patients had an AIDS defining infection prior to diagnosis.
However, there was one patient who tested negative for HIV one month prior to diagnosis and seroconverted two months after surgery.
Pain was the most distinctive presenting complaint, occurring in all but three patients.
Facial pain was frequent and resembled trigeminal neuralgia, while lesions within the spinal canal presented with radiculopathy.
Lesions were dural-based, whether occurring in the spinal canal or in the intracranial compartment.
Most were associated with vascular structures such as venous sinuses or adjacent arteries.
On imaging studies, all tumors enhanced with gadolinium and resembled meningiomas or schwannomas.
Resection was usually sufficient therapy.
EBV DNA was detected in 9/10 samples studied.
One patient received liposomal doxorubicin at a dose of 20 mg/m2 every three to four weeks over one year without surgery.
This patient is stable and symptom free five years after presentation.

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