Giant vulvo-abdominal aggressive angiomyxomas, report of two cases

Aggressive angiomyxoma is a rare type of soft tissue tumour. It has traditionally been described as a low-grade myxomatous neoplasm of the vulvovaginal, perineal and pelvic regions in women, and has a high rate of recurrence because of its infiltrative potential and locoregional extension. Its pathogenesis and histogenesis are poorly understood, but it is proposed that chromosome 12 alterations and fibroblastic/myofibroblastic tumours are involved in its origin. Around 250 cases have been reported to date in the western literature, some of them with unusual presentations. The biggest reported tumour measures 26 cm × 21 cm × 6 cm. The following article describes two case reports of this neoplasm, which were studied at Mexico's General Hospital “Dr. Eduardo Liceaga” over period of one year. Both case reports state that the tumours are considerably bigger than the ones currently reported (66 cm × 60 cm × 20 cm and 50 cm × 46 cm × 17 cm). We shall focus on histopathology and end with an analysis and discussion. Resumen: El angiomixoma profundo o agresivo es una entidad poco frecuente, agrupada dentro de las neoplasias del estroma genital. Se describe clásicamente como una neoplasia mixomatosa de bajo grado de las regiones vulvovaginal, perineal y pélvica en mujeres, con una tasa de recurrencia alta debido a su potencial de infiltración y extensión locoregional; con patogénesis e histogénesis pobremente entendidas, postulándose alteraciones en el cromosoma 12 y origen fibroblástico/miofibroblástico. La literatura occidental reporta poco más de 250 casos a la fecha, algunos de ellos con presentaciones inusuales, el caso informado de mayor tamaño es de 26 × 21 × 6 cm. A continuación se describen dos casos de dicha neoplasia estudiados en el Hospital General de México “Dr. Eduardo Liceaga”, en el periodo de un año, ambos de tamaños considerablemente mayores a los reportados actualmente (66 × 60 × 20 cm y 50 × 46 × 17 cm) y se hace enfoque en su estudio histopatológico, finalizando con un análisis y discusión sobre el tema. Keywords: Angiomyxoma, Vulvar neoplasms, Palabras clave: Angiomixoma, Neoplasias vulvares