BACKGROUND:

Aggressive angiomyxoma of the vulva is a very rare mesenchymal neoplasm, which has a preference for the pelvic and perineal regions. It is defined to be aggressive because of its tendency to infiltrate and local recurrence. The treatment of choice is surgical excision.

CLINICAL CASE:

A 39-year-old female, who started with a 6 cm right vulvar tumor, soft, mobile, non-painful, without ulcerations, classified as a cyst of the Bartholin's gland; surgical resection of the lesion was performed without incidents or complications. In the Oncology Surgery service (Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán), a computerized axial tomography was requested, wich evidenced a heterogeneous, hypodense, vascularized tumor with cystic component that displaced the vaginal canal, uterus and bladder, with infiltration in soft tissue and skin, of 15 x 10 x 28 cm on the right upper lip. The incisional biopsy established of deep aggressive angiomyxoma diagnosis. In December 2016 the vulvoperineal tumor was extirpated. The Department of Pathological Anatomy reported a tumor of 30 x 25 x 7 cm, with estrogen and progesterone positive receptors, and intensity +++ in more than 80%. The patient received adjuvant treatment with tamoxifen. Currently the patient is asymptomatic and under follow-up.

CONCLUSIONS:

The use of antiestrogenic agents has been shown to decrease tumor size. This protocol requires less aggressive surgical approaches. Given the tendency of these tumors to recur, it is necessary to provide long-term follow-up considering the treatment with antiestrogens as a maintenance therapy.

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