Sarcoma. Vulva cancer
Soft tissue sarcomas make up fewer than 2% of vulvar malignancies. They occur over a wide age range, including the pediatric population, and usually appear as a rapidly enlarging and painful mass. Most tumors on the vulva are related to the leiomyosarcoma group, although the fibrous histiocytomas, rhabdomyosarcomas, hemangiosarcomas, and the newly described epithelioid sarcomas may also occur on the vulva. The prognosis for such lesions is variable depending on the biologic character of the individual sarcoma, but may well be related to hematogenous metastasis. Radical vulvectomy with groin dissection has yielded the lowest incidence of recurrent disease, but many patients die rapidly.
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Superficial perineal leiomyosarcomas are rare and may be more aggressive than superficial leiomyosarcomas in general. The tumor is well differentiated and shows immunoreactivity for smooth muscle “-actin and “-desmin.
The natural history of vulvar leiomyosarcomas is characterized by an indolent protracted course and frequent local recurrence, followed by distant fatal metastases. Surgery, chemotherapy, and radiotherapy achieve palliation rather than cure.
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Dermatofibrosarcoma protuberans of the vulva is an uncommon low-grade sarcoma of dermal origin. Although wide excision is the treatment of choice, microscopic tumor projections beyond the central tumor Nodule explain the tumor’s propensity for local recurrence. Frozen sections of margins may be useful to ensure complete resection.
Epithelioid sarcoma typically involves extremities of young men and may be confused histologically with various benign and malignant processes. Epithelioid sarcoma of the vulva is a soft tissue malignancy arising from the tenosynovial tissue and can present as a painless lump of the vulva. The suggested mode of treatment can range from wide excision to radical vulvectomy with groin Node dissection.