Source: Eur Urol  |  Posted 5 years ago

By Maggie Schwarz

WASHINGTON, D.C. -- May 10, 2006 -- An unusual, soft fibrous vaginal tumor was first reported in the 1990s and has been seen in a total of 7 pre-pubertal girls, according to case studies presented here.

Albert Altchek, MD, clinical professor of obstetrics, gynecology and reproductive science, Mt. Sinai School of Medicine, New York, New York, reported his finding here at the 54[^]th[] Annual Clinical Meeting of the American College of Obstetricians and Gynecologists (ACOG).

The 7 patients ranged in age from 4 to 11 years.

"We don't know what caused it," said Dr. Altchek, "but 6 of the 7 patients were Ashkenazi Jewish."

"The tumor seems to grow in relation to the adrenal gland," Dr. Altchek said. "It's not possible to excise it entirely, since it extends into the adjacent area, an extension of the pelvic floor. It causes no pain and grows rapidly. "

The tumors were deep, asymptomatic, unilateral, poorly circumscribed and 3 to 6 cm in size. They had overlying peau d'orange skin and developed in a few months. Five were right sided and 2 were left sided, the presenter said.

Simultaneous surgery with negative results included inguinal exploration, vaginoscopy and biopsies of cervix and vagina.

Grossly, the tumor appeared fibrous, with shiny 4-mm particles of fat. Histology results showed dense fibrous tissue rich in elastic fibers infiltrating mature adipose tissue. There were no mitoses. The bland stromal cells were variably positive for CD34 but negative for muscle markers and progesterone receptors. Estrogen receptors were positive in 4 of the girls.

Electron microscopy was confirmatory, Dr. Altchek said. Magnetic resonance imaging revealed that the labial mass extended into the contiguous pelvic floor and persisted after surgery. One case had a local small recurrence within a few months. Tissue culture karyotype done in 3 of the cases was 46,XX. In 2 cases addition of dehydroepiandrosterone sulfate to the culture speeded mitosis.

Serum adrenal hormones in 3 patients showed physiological increase. Dr. Altchek said he consider the tumors to be a variety of fibromatosis, possibly responsive to adrenal hormones.

[Presentation title: Prepubertal Unilateral Soft Fibrous Tumor of Labium Majus Without Palpable Border in Girls. Abstract P. 20S]