Rare vulvar lesions: A case series

@#Mass of the vulva include both benign and malignant lesions. Benign mass lesions of the vulva include tumors, hamartomas, cysts, infectious disorders, and non-neoplastic epithelial disorders. Some are rare mass lesions. Here, we presented three rare mass lesions of the vulva. The first case presented with the complaint of vulvar lesion since childhood, while the two cases presented in reproductive age and perimenopausal age. All three cases presented as mass lesions in the vulva with pain or uneasiness during work. In all these cases, excision was done. On histopathological examination, the lesions have different diagnoses which are common in other parts of the body, but rarely present at the vulva. A definitive diagnosis of a vulvar mass lesion is difficult to make, especially in the case of rare mass lesion. Histopathology is the principal tool of diagnosis.

Tumor de células granulares de vulva: un raro tumor genital benigno / Granular cell tumor of vulva: a rare benign genital tumor

Resumen ANTECEDENTES: El tumor de células granulares de la vulva es poco común y de origen neurogénico. Afecta, principalmente, a mujeres entre 60 y 70 años y es más frecuente en la raza negra. CASO CLÍNICO: Paciente de 63 años, con una lesión vulvar indolora y no pruriginosa, en crecimiento. En la exploración se observó una lesión excrecente de 2.5 cm en la región superior del labio mayor izquierdo, dura, vascularizada y con infiltración a 2 cm de profundidad. No se palparon adenopatías sospechosas. Luego del reporte de la biopsia, sugerente de tumor de células granulares, se practicó una escisión completa, con márgenes libres. El estudio inmunohistoquímico se reportó positivo para CD68, S100 y TFE3 lo que confirmó el diagnóstico. Puesto que el índice proliferativo (Ki67) fue inferior al 5% y los márgenes quirúrgicos estaban libres, no se requirió tratamiento adyuvante. La paciente permanece en seguimiento y sin signos de recidiva. CONCLUSIÓN: Si bien los tumores de células granulares de la vulva son poco comunes y casi siempre benignos, deben incluirse en el diagnóstico diferencial de una tumoración vulvar. La inmunohistoquímica es la herramienta más útil para el diagnóstico preciso y su tratamiento de elección es la escisión local amplia, por su tendencia a la recurrencia local.

Abstract BACKGROUND: Granular cell tumor of the vulva is rare and neurogenic in origin. It mainly affects women between 60 and 70 years of age and is more frequent in black women. CLINICAL CASE: A 63-year-old woman with a painless, non-pruritic, growing vulvar lesion. Examination revealed a 2.5 cm excrescent lesion in the upper region of the left labium majus, hard, vascularized and infiltrated to a depth of 2 cm. No suspicious lymph nodes were palpated. After the biopsy report, suggestive of granular cell tumor, complete excision was performed, with free margins. The immunohistochemical study was positive for CD68, S100 and TFE3 which confirming the diagnosis. Since the proliferative index (Ki67) was less than 5% and the surgical margins were clear, adjuvant treatment was not required. The patient remains in follow-up with no signs of recurrence. CONCLUSION: Although granular cell tumors of the vulva are rare and almost always benign, they should be included in the differential diagnosis of a vulvar tumor. Immunohistochemistry is the most useful tool for accurate diagnosis and their treatment of choice is wide local excision because of their tendency for local recurrence.

Tumor de células granulares de la vulva: caso clínico / Granular cell tumor of the vulva: a clinical case

El tumor de células granulares es una neoplasia de la piel y los tejidos blandos muy poco frecuente, benigna y de crecimiento lento, pero con altas tasa de recurrencia. La localización más frecuente en el aparato genital es en la vulva. Se debe realizar diagnóstico diferencial con otras lesiones vulvares, debido al manejo distinto de esta tumoración. El diagnóstico generalmente es histológico, ya que clínicamente es muy difícil diferenciarlo de otras tumoraciones a nivel vulvar. El tratamiento recomendado es la exéresis quirúrgica, con márgenes de seguridad. En caso de bordes afectos, se recomienda realizar una reescisión, por la frecuencia de recurrencia local y porque ésta puede ser el primer indicador de una conducta agresiva. Presentamos un caso clínico a nivel vulvar, con el objetivo de destacar la importancia de realizar un diagnóstico correcto, para un buen manejo clínico y seguimiento. Las formas malignas suponen menos del 3% de estos tumores y son muy agresivas

The granular cell tumor is a neoplasm of the skin and soft tissue very rare, benign and slow growing, but with high recurrence rate. The most frequent location in the genital tract is in the vulva. Differential diagnosis should be made with other vulvar lesions, due to differences in the management of this tumor. The diagnosis is usually histological, since it is clinically difficult to differentiate it from other tumors at the vulvar level. The recommended treatment is surgical excision, with safety margins. In case of affected borders, it is recommended to perform a resection, due to the local recurrence and this may be the first indicator of aggressive behavior. We present a clinical case at the vulvar level, in order to emphasize the importance of making a correct diagnosis, for a good clinical management and follow-up. Malignant forms account for less than 3% of these tumors and are very aggressive.

Granulomatose linfomatóide-relato de caso / Lymphomatoid granulomatosis -case report

Objetivo: descrever um caso com manifestaçõs clínicas e achados histopatológicos de Granulomatose Linromatóide (GL), uma rara doença linfoproliferativa das células B, angiocêntrica e angiodestrutiva associada ao vírus Epstein-Barr (EBV). A apresentação clínica mais comum é no pulmão, podendo acometer qualquer órgão, sendo os achados extra-pulmonares as principais manifestações nestes casos. Relato do caso: mulher de 65, com lesão cutânea vulvar e que evoluiu com quadro neurológico associado a lesão expansiva envolvendo parênquima cerebelar, sem alterações pulmonares. Considerações Finais: pelo seu prognóstico e capacidade de ser confundida com outras vasculites a GL impõe-se como um importante diagnóstico diferencial dessas doenças.

lntroduction: this report describe a case of Lymphomatoid Granulomatosis (LG,s a rare linfoproliferative disorder of B cell showing angiocentric growth and destructive behaviour caused by Epstain-Barr virus (EBV). The predominant clinical presentations are confined to the pumonary system, however ; extra-pumonary manifestations can sometimes be the main feature of the dieseses. Method: here we report a 65 year old female, with a skin vulvar lesion, and another in cerebelus, without pulmonary disease. Conclusion: the LG can mimics systemic vasculits and is a diagnostic challenge.