ABSTRACT
Resumen ANTECEDENTES: El angiomixoma agresivo profundo perineal es un tumor mesenquimatoso de muy limitada aparición que se origina debido a un crecimiento tumoral del tejido conjuntivo que se expande a pesar de su naturaleza benigna; se caracteriza por un comportamiento agresivo. CASO CLÍNICO: Paciente de 38 años, con un nódulo genital de 3 cm en el labio mayor izquierdo, con sospecha clínica de quiste de la glándula de Bartolino. Durante la intervención para su drenaje se objetivó una tumoración blanda, de aspecto mesenquimal, pediculado. El análisis histológico confirmó que se trataba de un angiomixoma agresivo profundo perineal. Posterior al estudio de extensión negativo, se completó la vulvectomía simple izquierda, con un posoperatorio favorable. CONCLUSIONES: La obtención de una biopsia inicial es decisiva, sobre todo en casos de tumores mesenquimales agresivos, como el angiomixoma agresivo perineal profundo. De esta manera puede establecerse un plan de tratamiento individual en función del diagnóstico histológico definitivo.
Abstract BACKGROUND: Aggressive deep perineal angiomyxoma is a mesenchymal tumor of very limited occurrence that originates due to a tumorous growth of connective tissue that expands despite its benign nature; it is characterized by an aggressive behavior. CLINICAL CASE: 38-year-old patient with a 3 cm genital nodule on the left labium majus, with clinical suspicion of Bartholin's gland cyst. During the intervention for its drainage, a soft, mesenchymal, pedunculated tumor was observed. Histological analysis confirmed that it was an aggressive deep perineal angiomyxoma. Following a negative extension study, a simple left vulvectomy was completed, with a favorable postoperative course. CONCLUSIONS: Obtaining an initial biopsy is critical, especially in cases of aggressive mesenchymal tumors, such as deep perineal aggressive angiomyxoma. In this way an individual treatment plan can be established based on the definitive histologic diagnosis.
ABSTRACT
Los tumores vulvares son un desafío diagnóstico en la práctica clínica por las múltiples etiologías subyacentes. CASO CLÍNICO: Adolescente de 13 años que presentó dos nódulos vulvares de un mes de evolución, asociados a leve dolor. Ecografía preoperatoria sospecha un quiste de inclusión epidérmica, el que se confirmó con biopsia excisional.
Vulvar tumors are a diagnostic challenge in clinical practice due to the multiple underlying etiologies. CLINICAL CASE: A 13-year-old adolescent who presented two vulvar nodules of one month of evolution, associated with mild pain. Preoperative ultrasound suspected an epidermal inclusion cyst, which was confirmed with excisional biopsy.
Subject(s)
Humans , Female , Adolescent , Vulvar Diseases/complications , Vulvar Diseases/diagnosis , Epidermal Cyst/complications , Epidermal Cyst/diagnosis , Vulvar Diseases/surgery , Epidermal Cyst/surgeryABSTRACT
Primary vulvar Ewing sarcoma (ES)/PNET is an uncommonly documented tumor, especially with molecular results. A 10-year-old girl presented with left vulvar swelling, a year ago. Her abdominopelvic ultrasound revealed a 12 cm × 8 cm sized, mixed echogenic blood-fi lled lesion in the left vulva; radiologically considered as a hematoma. Vulvectomy revealed a multinodular grey-brown tumor, microscopically comprising malignant round cells. Immunohistochemically, tumor cells diffusely expressed MIC2/ CD99 and Fli1 and subsequently displayed EWSR1 rearrangement, confi rming diagnosis of ES/PNET. Subsequently, PET-CT scan revealed residual local lesion with lung metastases. The patient was induced on EFT 2001 chemotherapy protocol. Three months after chemotherapy completion, there was no metabolically active disease on PET scan. Four months later, MRI disclosed recurrent primary and metastatic pulmonary lesions. She was planned for scar excision and adjuvant radiotherapy, but unfortunately defaulted further treatment. This forms the eighth case of primary vulvar ES/PNET confi rmed with molecular cytogenetic result, underscoring therapeutic value of objective diagnosis in such cases.