Fibroma mole gigante vulvar: relato de caso / Giant soft vulvar fibroma: case report

Fibroma mole, ou pólipo fibroepitelial, é uma lesão de proporções geralmente reduzidas, de cor hiperpigmentada ou igual à da pele, localizando-se frequentemente na face, pescoço, tronco e regiões intertriginosas. É um tumor classificado como benigno e pode acometer tanto homens quanto mulheres em idade reprodutiva e depois da quarta década de vida. Ocorre principalmente em obesos, diabéticos e durante a gestação. Com menor frequência, podem alcançar dimensões que excedem 5 cm. Seu crescimento pode ser lento ou rápido e comumente são assintomáticos, mas podem promover sangramentos por conta de ulcerações decorrentes de traumas repetidos. Apresentamos neste relato um fibroma mole, gigante, de localização vulvar, com 11 cm de comprimento, 11 cm de largura e 5 cm de espessura, pesando 500 g.

Giant soft vulvar fibroma is a fibroepithelial polyp lesion with generally reduced proportions, with a hyperpigmented color or similar to that of the skin, frequently located on the face, neck, trunk and intertriginous regions. It is a tumor classified as benign, can affect both men and women, of reproductive age and after the fourth decade, mainly obese, diabetic and during pregnancy. However, less frequently, they can reach dimensions that exceed 5 cm, may have a slow or accelerated evolution. They are commonly asymptomatic, but bleeding may be present due to ulcerations resulting from repeated trauma. In the current study, we describe a giant soft fibroma with a vulvar location measuring 11 cm in length, 11 cm in width, 5 cm in thickness and weighing 500 grams.

Carcinoma primario de células de Merkel vulvar

Las células de Merkel se describieron originalmente en el estrato basal de la epidermis, con propiedades neuroendocrinas. El carcinoma de células de Merkel de la vulva es una neoplasia extremadamente rara y altamente agresiva. Existen pocos casos de estos tumores, la mayoría de los cuales han sido considerados tumores neuroendocrinos. El origen histológico y la etiología de esta enfermedad son controvertidas. Debido a su rareza en esta localización, no está claro si se comporta de manera diferente a los carcinomas de piel similares en otras localizaciones. Se presenta un caso de carcinoma primario de células de Merkel vulvar. El examen de biopsia por escisión reveló una tumoración de 4 x 3 centímetros en el tercio posterior del labio mayor izquierdo de la vulva sin afectación de los ganglios linfáticos. La paciente fue sometida a vulvectomía radical y disección bilateral de ganglios linfáticos inguinales. La evaluación histológica postoperatoria no mostró metástasis regionales ni distantes.

Merkel cells were originally described in the stratum basale of the epidermis with neuroendocrine properties. Merkel cell carcinoma of the vulva is an extremely rare and highly aggressive neoplasm. There are few cases of these tumors, most of which have been considered neuroendocrine tumors. The histologic origin and etiology of this disease are controversial. It is known for his aggressive behavior and propensity for early diffusion. Because of its rarity in this location, it is unclear whether it behaves differently from similar skin carcinomas in other locations. A case of primary vulvar Merkel cell carcinoma is presented. Excisional biopsy examination revealed a 4 x 3-centimeter tumor in the posterior third of the left labium majus of the vulva without lymph node involvement. The patient underwent radical vulvectomy and bilateral inguinal lymph node dissection. Postoperative histological evaluation showed no regional or distant metastases.

Sarcoma epitelioide de tipo proximal de vulva

El sarcoma epitelioide es un tipo raro de tumor mesenquimal. El sarcoma epitelioide de tipo proximal de vulva es un tumor maligno extremadamente raro de los tejidos blandos, con frecuentes recurrencias por su comportamiento agresivo. Debido a que sus características son similares a otras lesiones benignas, esto lleva a retrasos diagnósticos y terapéuticos. Las características histológicas de este tumor incluyen células epitelioides prominentes y aparición de rasgos rabdoides con marcadas atipias nucleares. Debido a su baja incidencia, no existen algoritmos diagnósticos basados en la evidencia ni recomendaciones publicadas para el tratamiento. El pronóstico es generalmente malo. Se presenta un caso de sarcoma epitelioide de tipo proximal de vulva.

Epithelioid sarcoma is a rare type of mesenchymal tumor. Proximal-type epithelioid sarcoma of the vulva is an extremely rare malignant tumor of the soft tissues with frequent recurrences due to its aggressive behaviour. Because its characteristics are similar to other benign lesions, this leads to diagnostic and therapeutic delays. Histologic features of this tumor include prominent epithelioid cells and the appearance of rhabdoid features with marked nuclear atypia. Due to its low incidence, there are no evidence-based diagnostic algorithms or published recommendations for treatment. The prognosis is generally poor. A case of proximal type epithelioid sarcoma of the vulva is presented.

Pleomorphic hyalinizing angiectatic tumor of the vulva: literature review based on a rare presentation

Pleomorphic hyalinizing angiectatic tumor (PHAT) of soft tissues is a rare, non-metastatic tumor of unknown etiology and uncertain behavior, which may recur locally. There are few reports on this condition, and due to the rarity of the disease, its lineage has not yet been fully elucidated. The present study aims to report the case of an unusual entity observed for the first time in vulval topography. A female patient, 83 years old, presented with a tumor in the vulvar region that had evolved for approximately 4 months. Magnetic resonance imaging showed an expansive perineal formation of 8.5 × 3.5 cm, and a hemivulvectomy with a flap rotation was performed. The review of the slides revealed a mesenchymal lesion without significant atypia, which was richly vascularized. In the areas of interest, the immunohistochemical (IHC) study demonstrated positivity for CD34, estrogen, and progesterone receptors; it was negative for the other tested markers. Morphological findings associated with the IHC staining panel supported the diagnosis of PHAT. The main morphological features of PHAT are clusters of ectatic vessels of different sizes that show deposits of subendothelial and intraluminal fibrin. Fusiform and pleomorphic cells randomly arranged in leaves or long fascicles intermingle these vessels. It is essential to recognize this entity and consider it among the differential diagnoses of a mesenchymal lesion, given the wide variety of entities that comprise this group of lesions.

High-Grade Transformation in Adenoid Cystic Carcinoma of the Bartholin Gland: Case Report / Transformação de alto grau em carcinoma adenoide cístico de glândula de Bartholin: relato de caso

Abstract Introduction In the present study, we report a case of primary adenoid cystic carcinoma (ACC) of the Bartholin gland with high-grade transformation (HGT). Adenoid cystic carcinoma of the Bartholin gland is a rare tumor and HGT has only been reported in head and neck tumors. Case Report A 77-year-old woman with a non-ulcerated vulvar lesion on the topography of the right Bartholin gland. The patient was submitted to tumor resection followed by V-Y island flap and adjuvant radiotherapy. The histopathological examination revealed primary ACC of the Bartholin gland, with areas of HGT and extensive perineural invasion. The immunohistochemical study with p53 showed a diffuse and strong positive reaction in areas with HGT. After 24 months of follow-up, the patient presented distant metastases and died, despite having undergone to chemotherapy. Conclusion As far as we know, this case is the first description in the literature of HGT in ACC of the Bartholin gland, and HGT appears to be associated with tumor aggressiveness.

Resumo Introdução Este estudo relata o caso de um carcinoma adenoide cístico (CAC) de glândula de Bartholin com transformação de alto grau. O CAC de glândula de Bartholin é um tumor raro, e sua transformação de alto grau é relatada somente em tumores de cabeça e pescoço. Relato de caso Paciente de 77 anos de idade, do sexo feminino, com lesão vulvar não ulcerada na topografia da glândula de Bartholin direita. A paciente foi submetida a ressecção do tumor e realização de retalho em V-Y, seguidas de radioterapia adjuvante. O exame histopatológico revelou CAC primário de glândula de Bartholin, com áreas de transformação de alto grau e invasão perineural. O estudo imunohistoquímico com p53 mostrou reação positiva difusa e intensa em áreas com transformação de alto grau. Após 24 meses de seguimento, a paciente apresentou metástases à distância e faleceu, apesar de ter sido submetida a quimioterapia. Conclusão Pelo que sabemos, este caso é a primeira descrição na literatura de transformação de alto grau em CAC de glândula de Bartholin, e a transformação de alto grau parece estar associada à agressividade do tumor.

Tumor de Buschke-Löwenstein en una niña. A propósito de un caso

El tumor de Buschke-Löwenstein es un condiloma acuminado gigante, benigno, cuyas lesiones clínicas son mayores de 10 cm, por lo que el tratamiento de elección debería ser siempre quirúrgico. Se presenta el caso de una niña de un año con condiloma gigante en la vulva, tumor de Buschke-Löwenstein de rápida evolución, a quien se le practicó resección quirúrgica satisfactoria. Se revisó la literatura respecto a su diagnóstico y tratamiento.

Buschke-Löwenstein tumor is a benign giant condyloma acuminatum, whose clinical lesions are larger than 10 cm, so the treatment of choice should always be surgical. We present the case of a one-year-old girl with giant condyloma of the vulva, a rapidly evolving Buschke-Löwenstein tumor, who underwent successful surgical resection. The literature was reviewed regarding its diagnosis and treatment.

Linfadenectomía inguinal por video endoscopia (VEIL) para el manejo de cáncer vulvar en el Instituto Nacional de Cancerología (Bogotá, Colombia) / Video endoscopic inguinal lymphadenectomy (VEIL) for the management of vulvar cancer at the Instituto Nacional de Cancerología (Bogotá, Colombia)

Resumen El cáncer de vulva representa el 2% al 5% de todos los cánceres ginecológicos a nivel mundial, dentro de los cuales, el más frecuente es el carcinoma escamocelular seguido por el melanoma. La cirugía es la piedra angular en el tratamiento de la neoplasia vulvar, y la evaluación del estado ganglionar es un poderoso predictor de supervivencia. La linfadenectomía inguinofemoral hace parte de las estrategias de tratamiento. La aproximación quirúrgica mínimamente invasiva por video endoscopia (VEIL) es una alternativa a la vía abierta que disminuye la morbilidad postoperatoria como dehiscencia de la herida quirúrgica, linfocele, linfedema, infección y deterioro psicosexual. A continuación, se describe la técnica quirúrgica VEIL como abordaje novedoso en Colombia para el manejo de la neoplasia vulvar.

Abstract Vulvar cancer represents 2% to 5% of all gynecological cancers worldwide, of which the most common is squamous cell carcinoma followed by melanoma. Surgery is the cornerstone in the treatment of vulvar neoplasia, and evaluation of lymph node status is a powerful predictor of survival. Inguinofemoral lymphadenectomy is part of the treatment strategies. The minimally invasive surgical approach by video endoscopy (VEIL) is an alternative to the open approach that reduces postoperative morbidity such as dehiscence of the surgical wound, lymphocele, lymphedema, infection, and psychosexual deterioration. The following describes the surgical technique of VEIL, as a novel approach in Colombia for the management of this cancer.

Carcinoma basocelular na vulva: um relato de caso de neoplasia cutânea em área especial / Basal cell carcinoma of the vulva: a case report of cutaneous neoplasia in a special area

O carcinoma basocelular (CBC) é o câncer de pele mais comum.¹ Entre os fatores de risco para seu desenvolvimento estão a exposição a radiações ionizantes e não ionizantes, alguns produtos químicos e cicatrizes prévias.² Porém, o fator mais importante é a exposição à radiação ultravioleta, o que explica a maior incidência dessa neoplasia em áreas fotoexpostas. O CBC em áreas não expostas é incomum. O objetivo deste relato é descrever um caso de CBC recorrente na vulva, demonstrar a importância do exame dermatológico em áreas incomuns e relatar a aplicação da cirurgia micrográfica de Mohs.

Basal cell carcinoma (BCC) is the most common skin cancer¹. Among the risk factors for its development are exposure to ionizing and non-ionizing radiation, some chemicals, and previous scars.² However, the most important factor is exposure to ultraviolet radiation, which explains the higher incidence of this neoplasm in photo-exposed areas.¹ BCC in unexposed areas is uncommon. The purpose of this report is to describe a case of recurrent BCC in the vulva, to demonstrate the importance of dermatological examination in unusual areas, and to report the application of Mohs micrographic surgery

Leiomioma vulvar de localización en la glándula de Bartolino: un dilema diagnóstico / Vulvar leiomyoma located in Bartholin's gland: a challenging clinical case

Resumen ANTECEDENTES: Los leiomiomas son tumores benignos que se originan en las fibras musculares lisas. La mayor parte de los leiomiomas del aparato genital femenino se localizan en el útero. La localización extrauterina es excepcional, la incidencia del leiomioma vulvar es del 0.07%. Estos tumores del músculo liso suelen ser indoloros, de crecimiento lento y pueden aparecer en mujeres de cualquier edad. OBJETIVO: Reportar un caso de leiomioma vulvar que, por su baja incidencia y similitud clínica con otras lesiones, como el quiste de la glándula de Bartolino, pueden conducir a un diagnóstico erróneo inicial. Además, efectuar una revisión bibliográfica que aporte conocimiento a su diagnóstico y tratamiento. CASO CLÍNICO: Paciente de 52 años, con una masa vulvar de años de evolución y un diagnóstico equívoco inicial de quiste de la glándula de Bartolino. La paciente fue intervenida mediante escisión quirúrgica y estudio histopatológico posterior. El diagnóstico definitivo fue de leiomioma vulvar con diferenciación simplástica. CONCLUSIONES: En contraposición con sus homólogos uterinos, la localización vaginal del leiomioma es excepcional. Si bien hasta ahora no ha podido demostrarse la efectividad de la biopsia preoperatoria se aconseja su toma porque podría tratarse de una masa de características malignas, lo que modificaría el tratamiento y su urgencia. La actitud quirúrgica es, quizá, la más acertada porque permite, además de un diagnóstico anatomopatológico definitivo, un tratamiento curativo. Se propone la escisión completa, mediante una incisión elipsoidal en la piel y asegurar la extirpación completa de la pseudocápsula.

Abstract BACKGROUND: Leiomyomas are benign tumors that originate in smooth muscle fibers. Most leiomyomas of the female genital tract are located in the uterus. Extrauterine localization is exceptional; the incidence of vulvar leiomyoma is 0.07%. These smooth muscle tumors are usually painless, slow growing and can occur in women of any age. OBJECTIVE: To report a case of vulvar leiomyoma that because of its low incidence and clinical similarity to other lesions, such as Bartholin's gland cyst, may lead to an initial misdiagnosis. In addition, a review of the literature should be carried out in order to contribute to its diagnosis and treatment. CLINICAL CASE: A 52-year-old patient with a vulvar mass of years of evolution and an initial misdiagnosis of Bartholin's gland cyst. The patient was operated by surgical excision and subsequent histopathological study. The definitive diagnosis was vulvar leiomyoma with symplastic differentiation. CONCLUSIONS: In contrast to its uterine counterparts, the vaginal location of leiomyoma is exceptional. Although the effectiveness of a preoperative biopsy has not been demonstrated so far, it is advisable to take a biopsy because it could be a mass with malignant characteristics, which would change the treatment and its urgency. The surgical approach is perhaps the most appropriate because it allows, in addition to a definitive anatomopathological diagnosis, a curative treatment. Complete excision is proposed, by means of an ellipsoidal incision in the skin and ensuring complete excision of the pseudocapsule.

Tumor Fibro-histiocítico Plexiforme de Vulva: Relato de Caso / Vulva Plexiform Fibrohistiocytic Tumor: Case Report / Tumor Fibrohistiocítico Plexiforme de la Vulva: Reporte de Caso

Introdução: O tumor fibro-histiocítico plexiforme é uma condição clínica rara, com cerca de 150 casos descritos na literatura. Relato do caso: Paciente, 23 anos, sexo feminino, portadora de lesão nodular em região vulvar sem características de malignidade em exames de ultrassonografia. Realizou acompanhamento clínico, tendo apresentado aumento do tamanho da lesão inicial, sendo optado então por realização de ressecção cirúrgica da lesão. Em estudo histopatológico, evidenciou-se lesão compatível com tumor fibro-histiocítico plexiforme, com margens cirúrgicas livres. No seguimento, a paciente foi encaminhada para avaliação oncológica com a intenção de realizar exames complementares de rastreio e descartar diagnósticos morfologicamente semelhantes. Diante dos exames complementares confirmando o diagnóstico inicial e sem evidência de doença metastática após excisão cirúrgica completa com margens livres, foi iniciado seguimento clínico. Conclusão: Relatar o caso é de extrema importância para divulgar a apresentação clínica, etapas diagnósticas, tratamento proposto e compartilhar informações acerca de evolução clínica apresentada.

Introduction: Plexiform fibrohistiocytic tumor is a rare clinical condition, with about 150 cases described in the literature. Case report: 23-years-old, female patient with nodular lesion in vulva without malignancy characteristics on ultrasound image. She underwent clinical follow-up and after an increase in the size of the lesion, surgical resection was opted. Histopathological findings suggested plexiform fibrohistiocytic tumor, with free surgical margins. In follow up, she was referred to oncological evaluation for screening complementary exams and rule out morphologically similar diagnoses. After the complementary exams confirming the initial diagnosis of plexiform fibrohistiocytic tumor and without evidence of metastatic disease after complete surgical excision with free margins, the patient is in clinical follow-up. Conclusion: Reporting the case is extremely important to disseminate the clinical presentation, diagnostic steps, proposed treatment and share information about the clinical evolution presented.

Introducción: El tumor fibrohistiocítico plexiforme es una condición clínica poco frecuente, con unos 150 casos descritos en la literatura. Reporte del caso: Paciente de 23, sexo feminino, años con lesión nodular en una región vulvar sin características malignas en los exámenes de ultrasonido. Se sometió a un seguimiento clínico, habiendo presentado un aumento en el tamaño de la lesión inicial, y se decidió realizar la resección quirúrgica de la lesión. En un estudio histopatológico, se evidenció una lesión compatible con tumor fibrohistiocítico plexiforme con márgenes quirúrgicos libres. Fue referida para la evaluación oncológica, realizó pruebas complementarias para el cribado y para descartar diagnósticos morfológicamente similares. En vista de las pruebas complementarias que confirman el diagnóstico inicial y sin evidencia de enfermedad metastásica después de una escisión quirúrgica completa con márgenes libres, se inició el seguimiento clínico. Conclusión: Informar del caso es extremadamente importante para difundir la presentación clínica, etapas diagnósticas, tratamiento propuesto y compartir información sobre la evolución clínica presentada.

State of the art in vulvar cancer imaging / Estado da arte na avaliação radiológica do carcinoma da vulva

Abstract Vulvar carcinoma is an uncommon tumor that predominantly affects postmenopausal women. Currently, there is no screening procedure for vulvar carcinoma; in most cases, it is diagnosed only when symptoms appear. The most widely used staging system is that developed by the International Federation of Gynecology and Obstetrics. Lymph node status is the most important prognostic factor. We searched the PubMed/Medline database to identify relevant English-language articles on vulvar cancer, with a special focus on its imaging evaluation. Magnetic resonance imaging is useful for local and nodal staging, as well as facilitating the planning of surgical interventions and radiotherapy. Computed tomography or positron-emission tomography/computed tomography can play an important role in nodal and distant disease assessment, whereas ultrasound is often used for image-guided biopsies. Imaging is pivotal for staging and treatment planning in vulvar carcinoma.

Resumo O carcinoma da vulva é um tumor incomum que afeta predominantemente mulheres em menopausa. Atualmente, não existe um teste de rastreio para o carcinoma da vulva, e a maioria dos casos é diagnosticada com o aparecimento de sintomas. O sistema de estadiamento mais frequentemente utilizado é o da International Federation of Gynecology and Obstetrics. O fator prognóstico mais importante é o estadiamento ganglionar. Neste trabalho foram efetuadas pesquisas na base de dados PubMed/Medline, considerando-se textos pertinentes em língua inglesa sobre carcinoma da vulva, com especial ênfase na avaliação radiológica. A ressonância magnética é útil para a avaliação local e ganglionar e pode ajudar no planejamento cirúrgico e/ou de radioterapia; a tomografia computadorizada ou a tomografia com emissão de pósitrons/tomografia computadorizada pode ser importante na apreciação ganglionar e na doença a distância; e a ultrassonografia é, por vezes, utilizada para orientar procedimentos de intervenção, como a biópsia. A radiologia é fundamental no estadiamento e planejamento do tratamento do carcinoma da vulva.

Dermatofibrosarcoma protuberans vulvar: tres casos clínicos de localización inusual / Dermatofibrosarcoma protuberans vulvar: three clinical cases of localization unusual

RESUMEN El dermatofibrosarcoma protuberans (DFSP) es un sarcoma fusocelular de grado intermedio de malignidad con máxima incidencia en adultos entre 20 y 40 años y de localización habitualmente troncular (cabeza, cuello extremidades superiores). Se presentan tres casos de DFSP de localización excepcional a nivel vulvar. Las pacientes fueron tratadas con vulvectomía respetando márgenes de seguridad pero, debido a la idiosincrasia del tumor, presentaron recidivas locales que precisaron de una nueva cirugía. En su posterior seguimiento no presentaron recaídas y se encuentran libres de enfermedad.

ABSTRACT Dermatofibrosarcoma protuberans (DFSP) is an intermediate grade spindle-cell sarcoma with a highest incidence in adults between 20 and 40 years old and a trunk location (head, neck and arms). We introduce three case reports of vulvar DFSP considered unusual because of their location. The patients were conducted a free-margin vulvectomy but, due to the nature of the tumor, local reappearances required a second surgery. In the subsequent follow-up they did not suffer from any tumor relapse and they are currently disease-free.

Fibroadenoma vulvar: a propósito de un caso clínico / Vulvar fibroadenoma: about a clinical case

RESUMEN El fibroadenoma vulvar es un tumor benigno poco frecuente con muy pocos casos descritos en la literatura. Por lo general, se diagnostica entre los 20 y 70 años. Tiene un buen pronóstico y rara vez recurre después de la escisión. Presentamos un caso de fibroadenoma vulvar, en una mujer de 33 años, manifestado como un tumor localizado, bien definido de partes blandas en el labio mayor izquierdo. La lesión fue extirpada completamente y la histología confirmó un fibroadenoma con marcador de estrógeno positivo.

ABSTRACT Vulvar fibroadenoma is a rare benign tumor with few cases reported in the literature. It usually occurs between 20 and 70 years of age. It has a good prognosis and rarely recurs following excision. We present a case of vulvar fibroadenoma as a localized, well-defined soft tissue mass in the left labium majus of a 33-year-old woman. The mass was excised completely, and histology demonstrated a fibroadenoma positive for estrogen receptor.

Progress of radiotherapy for vulvar cancer / 国际肿瘤学杂志

Radiation therapy has a major role in curative treatment of vulvar cancer patients including preoperative radiotherapy, radical radiotherapy and postoperative radiotherapy. Radiation therapy with concurrent chemotherapy may be able to achieve better clinical outcomes, but the rate of acute and chronic toxicities may be higher. So we should develop individual radiotherapy and chemotherapy program, strictly limit the dose and avoid treatment interruption. It is recommended to apply intensity-modulated radiotherapy, which is supe-rior to conventional radiotherapy in dose and clinical efficacy. Interstitial brachytherapy can be used selectively, but it requires a high level of surgical experience, and the doses to target and organs at risk need to be further discussed.

Progress of radiotherapy for vulvar cancer / 国际肿瘤学杂志

Radiation therapy has a major role in curative treatment of vulvar cancer patients including preoperative radiotherapy,radical radiotherapy and postoperative radiotherapy. Radiation therapy with concur-rent chemotherapy may be able to achieve better clinical outcomes,but the rate of acute and chronic toxicities may be higher. So we should develop individual radiotherapy and chemotherapy program,strictly limit the dose and avoid treatment interruption. It is recommended to apply intensity-modulated radiotherapy,which is supe-rior to conventional radiotherapy in dose and clinical efficacy. Interstitial brachytherapy can be used selectively, but it requires a high level of surgical experience,and the doses to target and organs at risk need to be further discussed.

Uso do imiquimode para tratamento da doença de Paget extramamária: série de quatro casos / Imiquimod use for the treatment of extramammary Paget disease: series of four cases

Introdução: A doença de Paget extramamária é constituída por adenocarcinoma da pele em áreas de glândulas apócrinas. Trata-se de neoplasia rara cujo tratamento cirúrgico aparenta resultados satisfatórios. Devido às altas taxas de recorrência, entretanto, as cirurgias são potencialmente mutilantes. O imiquimode é imunoestimulador tópico utilizado no tratamento de verrugas anogenitais e carcinomas in situ. Seu uso tem sido descrito na literatura científica para terapia da doença de Paget extramamária com resultados satisfatórios. Objetivo: Descrever a evolução de quatro casos de doença de Paget extramamária tratados com imiquimode, para avaliação da evolução e resposta terapêutica. Métodos: Foi realizado estudo retrospectivo em serviço de dermatologia da cidade de São Paulo com revisão de prontuários de todos os pacientes com diagnóstico de doença de Paget extramamária e tratados com imiquimode de janeiro de 2011 a julho de 2018. Resultados: Foram incluídos quatro pacientes, três com lesão vulvar e um com lesão em bolsa escrotal. Duas mulheres evoluíram com resolução total da doença, uma não apresentou alteração, e o homem evolui com regressão de 70% da lesão, sendo submetido à exérese cirúrgica de área consideravelmente menor do que a da lesão inicial. Conclusões: O imiquimode se apresenta como método terapêutico válido no tratamento da doença de Paget extramamária.

Introduction: Extramammary Paget disease consists of a cutaneous adenocarcinoma in areas of apocrine glands. It is a rare neoplasia, and its surgical treatment yields satisfactory results. However, due to the high recurrence rates, surgeries are potentially disfiguring. Imiquimod is a topical immune-stimulant used for the treatment of anogenital warts and in situ carcinomas. Its use has been described in the scientific literature for the treatment of Extramammary Paget disease with satisfactory results. Objective: Describe the course of four cases of Extramammary Paget disease treated with imiquimod, to evaluate evolution and therapeutic response. Methods: A retrospective study was performed in a service of dermatology in the city of São Paulo, with the review of medical records of all patients diagnosed with Extramammary Paget disease and treated with imiquimod from January 2011 to July 2018. Results: Four patients were included, three with vulvar lesion and one with scrotal lesion. Two women presented completed resolution of the disease, one did not respond and the man progressed with 70% regression of the lesion, having undergone surgical excision of a considerably smaller area than the initial lesion. Conclusions: Imiquimod presents as a valid therapeutic modality for the treatment of Extramammary Paget disease.

Fibroadenoma de la vulva. A propósito de un caso / Vulvar Fibroadenoma with regards

El fibroadenoma vulvar es una lesión extremadamente rara. Se presenta como una masa unilateral, de tamaño variable, asintomático y ocasionalmente asociado a otras lesiones vulvares o extravulvares y existe gran controversia sobre su origen. Estas lesiones necesitan del examen histopatológico para llegar al diagnóstico definitivo. El comportamiento de este tumor es similar al mamario. La escisión generalmente tiene un buen pronóstico y rara vez recidiva. Presentamos un caso donde se diagnosticó histopatológicamente esta lesión en una mujer de 26 años de edad con masa tumoral en la vulva, a la que se le realizó excéresis quirúrgica.

Vulvar fibroadenoma is extremely rare. It presents as a unilateral mass of variable size, asymptomatic and occasionally associated to other vulvar and extra-vulvar lesions. There is a great controversy about their origin. These lesions need a histo-pathological exam to reach a concluding diagnosis. Its behavior is similar to the breast tumor. Its removal generally has a good prognosis and there is rarely a relapse. This is a case of a 26 year old woman who was histo-pathologically diagnosed a vulvar tumor. A surgical removal was performed.

Adenoma pleomórfico de vulva / Vulvar Pleomorphic Adenoma

El adenoma pleomórfico (AP) es el tumor benigno más prevalente de las glándulas salivales mayores; sin embargo, esta no es su localización anatómica exclusiva. Las células mioepiteliales, tapizan la membrana basal de las porciones terminales de diferentes tejidos glandulares exocrinos incluyendo los salivales, lacrimales, mamarios y sudoríparos; por esta razón, no es insólito que este tumor se origine en tejidos glandulares diferentes a los salivales. El adenoma pleomórfico de la vulva es un hallazgo infrecuente, con pocos casos reportados en la literatura, siendo este caso el primero reportado en Colombia y Latinoamérica(AU)

Pleomorphic Adenoma is the most prevalent benign tumor among the major salivary glands; nevertheless, this is not the exclusive anatomic localization. Myoepithelial cells cover the basal membrane of the terminal portions of different exocrine glandular tissues including the salivary glands, lacrimal glands, mammary tissue and sweat glands. Consequently, it is not rare that this kind tumor originate in tissues different from the salivary glands. The vulvar pleomorphic adenoma is an uncommon finding, with few reported cases in literature. Actuality this the first case reported in Colombia and Latin America(AU)

Vulvar hemangioma: case report / Hemangioma vulvar: relato de caso

Abstract Hemangioma is a benign neoplasm that may affect the vulva, and it can cause functional or emotional disability. This article reports the case of a 52-year-old female patient with a history of a genital ulcer for the past 3 years and who had undergone various treatments with creams and ointments. The patient was biopsied and diagnosed with vulvar hemangioma and was subsequently submitted to surgical excision of the lesion. We emphasize the importance of following the steps of the differential diagnosis and proceeding with a surgical approach only if necessary.

Resumo O hemangioma é uma neoplasia benigna que pode afetar a vulva e pode causar incapacidade funcional ou emocional. Este artigo relata o caso de uma paciente de 52 anos com história de úlcera genital nos últimos 3 anos, submetida a diversos tratamentos com cremes e pomadas. A paciente foi biopsiada e diagnosticada com hemangioma vulvar e subsequentemente submetida a excisão cirúrgica da lesão. Ressaltamos a importância de seguir as etapas do diagnóstico diferencial e proceder a uma abordagem cirúrgica somente se necessário.

Prognostic impact of reduced tumor-free margin distance on long-term survival in FIGO stage IB/II vulvar squamous cell carcinoma / 부인종양

OBJECTIVE: We aimed to identify the minimum tumor-free margin distance conferring long-term oncological safety in patients diagnosed with International Federation of Gynecology and Obstetrics (FIGO) stage IB/II vulvar squamous cell carcinoma (VSCC). METHODS: This was a retrospective cohort study in patients with stage IB/II VSCC treated at a single institution in Turin, Italy. The main aim was to identify the minimum tumor-free margin distance that confers oncological safety in early-stage VSCC. Patients were divided in groups according to tumor-free histological margin distance to compare survival outcomes. Overall survival (OS), disease-specific survival (DSS), and recurrence rate (RR) were estimated by the Kaplan-Meier method for the newly proposed and the currently recommended 8 mm margin cut-off. Log-rank test was used to compare survival between groups. RESULTS: One hundred and fourteen patients met the study criteria. Median age was 68 years and median follow-up was 80 months. The minimum margin distance that conferred long-term oncological safety was 5 mm. OS, DSS were significantly lower in the < 5 mm group when compared with the ≥ 5 mm group (p = 0.002 and p = 0.033, respectively) although no difference in RR was observed between groups. Analysis at the 8-mm cut-off indicated there is no difference in OS, DSS, or RR between groups. CONCLUSION: FIGO stage IB/II VSCC patients' prognosis is affected by margin distance. Long-term survival is significantly reduced in patients with tumor-free margins < 5 mm, even in the absence of lymph node metastasis. Thus, these patients should be offered further surgical or adjuvant treatment.