Tumor del saco vitelino primario de vagina en una niña. A propósito de un caso / Primary vaginal yolk sac tumor in a girl. Case report

Las neoplasias ginecológicas en niñas son raras. Representan menos del 5 % de todos los cánceres en pediatría. No existen estadísticas sobre la incidencia de tumores de vagina en esta etapa de la vida. Se presenta el caso de una niña de 9 meses con sangrado por genitales. La ecografía evidencia una masa sólida en vagina, y la vaginoscopia, un tumor friable. Presenta valores de α-fetoproteína elevados, por lo que se diagnostica tumor de saco vitelino, confirmado por biopsia. Se realiza tratamiento quimioterápico. A menos de 1 año del diagnóstico, se encuentra en remisión completa. Este caso resulta de interés no solo por la rareza, sino también porque el diagnóstico rápido de tumor de saco vitelino permite mejorar los resultados y la sobrevida de las pacientes

Gynecological neoplasms in girls are rare and represent only less than 5 % of all childhood tumors. There are no statistics on the incidence of vaginal tumors at this stage in life. We present a 9-month-old girl evaluated for genital bleeding. Ultrasound reveals a vaginal solid mass and vaginoscopy reports a friable tumor. AFP is elevated. A yolk sac tumor is confirmed by biopsy she receives chemotherapy. Within a year after diagnosis, she remains tumor-free. This is a case of interest, not only because of its rarity, but also because a rapid diagnosis of a yolk sac tumor improves outcomes and patient's survival rates.

Epidermoid carcinoma in the neovagina of a patient with Mayer-Rokitansky-Küster-Hauser syndrome. Case report and literature review / Carcinoma epidermoide de neovagina en paciente con síndrome de mayer- rokitansky-küster-hauser. reporte de un caso y revisión de la literatura

RESUMEN Objetivo: presentar el caso de una paciente con síndrome de Mayer-Rokitansky-Küster-Hauser, con diagnóstico de carcinoma escamoso en neovagina, y realizar una revisión de la literatura del tratamiento y pronóstico de las neoplasias o la infección por VPH de tejidos neovaginales en mujeres transgénero o con síndrome MRKH. Materiales y métodos: mujer de 56 años de edad que consulta en un hospital universitario regional de la ciudad de Sevilla (España). Tras la exploración clínica se aprecia una tumoración exofítica en fondo de vagina, con resultado de biopsia de carcinoma escamoso y cultivo positivo para virus del papilloma humano (VPH) tipo 16. Se realizó una búsqueda bibliográfica en la base de datos Medline vía PubMed, con los términos: "congenital abnormalities"; "mullerian aplasia"; "neovagina"; "vaginal neoplasms"; "squamous carcinoma"; "HPV infection", de reportes y series de caso, y estudios observacionales publicados desde el año 2000 hasta octubre de 2019 en inglés y español. Resultados: se incluyeron 14 estudios; de estos, siete correspondieron a carcinoma escamocelular, tres a adenocarcinoma y cuatro informan infección solo por VPH. Todos los carcinomas escamocelulares presentaban estadios avanzados por compromiso local o linfático y recibieron radioterapia con quimioterapia concomitante o cirugía radical. El pronóstico fue malo en tres de los casos. Las pacientes con adenocarcinoma también presentaron lesiones avanzadas con compromiso local, el tratamiento fue quirúrgico, con quimioterapia concomitante en dos de los casos. Solo una paciente tuvo seguimiento mayor a cinco años. La infección por VPH es frecuente en mujeres sometidas a construcción de neovagina. Conclusiones: las pacientes con neovagina son susceptibles de desarrollar carcinomas escamosos si se utilizaron injertos cutáneos, o adenocarcinomas en aquellas en las que se reconstruyó la vagina a partir de injertos tisulares intestinales. Dado que al momento del diagnóstico presentan compromiso local, a menudo requieren tratamientos radicales o combinados. Se debe investigar cuáles son las mejores estrategias de tamizaje de VPH, cáncer escamoso y adenocarcinoma en este tipo de pacientes.

ABSTRACT Objective: To present the case of a squamous carcinoma in the neovagina of a patient with Mayer-Rokitansky-Küster-Hauser syndrome and review of the literature related to treatment and prognosis of vaginal neoplasms or HPV infection in transgender women or with MRKH syndrome. Materials and methods: A 56-year-old woman consulted to the Hospital Universitario of Sevilla (Spain). During the clinical examination, a exophytic tumor at the bottom of the vagina was found and the biopsy reported squamous carcinoma and positive nucleic acid amplification test for human papilloma (HPV) type 16. A literature search of case reports, case series and observational studies published from 2000 to October 2019 in English and Spanish was performed in Medline via PubMed, with the following terms: "congenital abnormalities"; "Mullerian aplasia"; "neovagina"; "Vaginal neoplasms"; "Squamous carcinoma"; "HPV infection" was performed. Results: 14 studies were finally included; seven corresponded to squamous cell carcinoma, three to adenocarcinoma and four reported HPV infection only. All of the squamous cell carcinomas were at advanced stages due to local or lymphatic compromise and received radiotherapy with concomitant chemotherapy or radical surgery. The prognosis was bad in three of the cases. Patients with adenocarcinoma also presented with advanced lesions due to local extensión and received surgical treatment and two cases received concomitant chemotherapy. Only one patient was followed-up for five years or more. HPV infection is common in women who underwent neovagina reconstruction. Conclusions: Patients with neovagina are susceptible to develope squamous carcinomas or adenocarcinomas depending if skin or intestinal tissue grafts are used. According to local compromise at the time of diagnosis, radical or combined treatments are required. Which screening strategies for HPV, squamous cell carcinomas and adenocarcinoma is to be investigated.

Relato de caso: melanoma primário de vagina / Case Report: Primary Vaginal Melanoma

Introdução: Melanoma maligno de vagina é um tumor raro e agressivo com incidência de 0,46 casos a cada milhão de mulheres por ano, descrito pela primeira vez por Poronas, em 1887. São relatados aproximadamente 500 casos mundialmente.É umavariante mucosa do melanoma, acometendo regiões não expostas à radiação ultravioleta. Representa menos de 3% dos tumores primários malignos da vagina, de prognóstico ruim e taxa sobrevida em 5 anos de 5-25%. O tratamento preconizado é controverso e assunto de discussões. Existem poucos dados na literatura, justificando o relato deste caso, que contribuirá para novos estudos a fim de definir um melhor tratamento. Objetivo: Relatar um caso de melanoma primário de vagina invasor. Relato de caso: Paciente do sexo feminino, 78 anos, relatava sangramento vaginal há 6 meses e secreção serosanguinolenta de odor fétido. Ao exame ginecológico, visualizou-se lesão vegetante em parede vaginal esquerda, com sangramento ativo, friável e odor fétido, ausência de lesões em colo uterino e útero de volume habitual. A biópsia evidenciou à imunohistoquímica neoplasia de células epitelioides com atipias proeminentes com pigmento melânico associado à extensão pagetoide para camadas superficiais do epitélio, mitoses atípicas e positividade forte e difusa para Melan A e proteína S100, favorecendo a hipótese de Melanoma invasor primário de vagina. A ressonância magnética da pelve evidenciou linfonodo inguinal heterogêneo à esquerda. Desfecho: Paciente submetida à colpectomia com excisão de lesão neoplásica em parede anterior da vagina e ressecção de lesões suspeitas em paredes laterais da vagina e parede lateral direita da vulva, sem intercorrências cirúrgicas. Conclusão: O caso relatado fomenta a discussão de doença rara e que mesmo quando o diagnóstico e o tratamento são instituídos, tem seu prognóstico ainda muito imprevisível, no que diz respeito à cura e melhoria da qualidade de vida.

Introduction: Malignant melanoma of the vagina is a rare tumor with 0.46 cases per one million women per year and first described by Poronas in 1887. There are 500 cases reported. It is a mucosal variant of melanoma wich affects regions not exposed to ultraviolet radiation. It represents less than 3% of vagina primary tumors, with a poor prognosis and five-year survival of 5-25%. The gold standard treatment is controversial and subject of discussions. There are few data in the literature, justifying the case report, which contribute to new studies. Objective: case report of primary invasive melanoma of the vagina. Case report: Female patient, 78 years old, reports vaginal bleeding for 6 months and serum blood secretion of foul-smelling. Gynecological examination revealed vegetation in the left vaginal wall, with active bleeding, friable and foul-smelling, no lesions in the cervix and uterus of normal volume. The biopsy followed by immunohistochemical test showed a neoplasia of epithelioid cells with prominent atypia with melanic pigment associated with pagetoid extension to superficial layers of the epithelium, atypical mitoses, and a strong and diffuse positivity for Melan A and protein S100, favoring the hypothesis of primary invasive melanoma of the vagina. The magnetic resonance imaging of the pelvis showed heterogeneous inguinal lymph node on the left. Results: Patient submitted to colpectomy with excision of anterior neoplastic lesion of the vagina and resection of suspicious lesions on side walls of the vagina and right lateral wall of the vulva, without surgical complications. Conclusion: The case reported encourages discussion of this pathology, and demonstrated that even if diagnosis and therapy are performed early, still has a very unpredictable prognosis, in terms of healing and improvement of the quality of life.

Rabdomiosarcoma botrioide del aparato genital femenino en una adolescente virgen / Botryoid Rhabdomyosarcoma of the Female Genital Tract in a Virgin Adolescent

El rabdomiosarcoma es un tumor complejo y de gran malignidad que se origina en las células de la mesénquima embrionaria con capacidad para diferenciarse en células musculares esqueléticas. Este es el tumor maligno de tejido blando más frecuente. Representa aproximadamente 3,5 por ciento de los casos de cáncer en niños de 0 a 14 años de edad. Se presenta una paciente adolescente y virgen en la que se diagnostica histológicamente rabdomiosarcoma botrioide de la vagina. La presentación clínica del rabdomiosarcoma embrionario variedad botrioide es, en general, una masa que protruye por la uretra o el introito vaginal, o por la presencia de flujo fétido o sangrado vaginal en niñas menores de 2 años. En estas lesiones el apoyo diagnóstico con inmunohistoquímica es de vital importancia. El pronóstico de esta enfermedad está determinado por variables como el tamaño tumoral, órgano comprometido, edad del paciente, resultado quirúrgico (R0/R1) y presencia de metástasis. La evaluación médica multidisciplinaria precoz y oportuna permitirá siempre establecer un diagnóstico y tratamiento adecuados(AU)

Rhabdomyosarcoma is a complex and highly malignant tumor that originates in the cells of the embryonic mesenchyme with the ability to differentiate into skeletal muscle cells. This is the most common malignant soft tissue tumor. It represents approximately 3.5 percent of cancer cases in children from zero to 14 years of age. A case of a virgin adolescent patient is presented. A botryoid rhabdomyosarcoma of the vagina was diagnosed histologically. The clinical presentation of embryonic botryoid rhabdomyosarcoma variety is, in general, a mass that protrudes through the urethra or vaginal introitus, or the presence of fetid flow or vaginal bleeding in girls under 2 years. In these lesions, diagnostic support with immunohistochemistry is of vital importance. The prognosis of this disease is determined by variables such as tumor size, compromised organ, age of the patient, surgical result (R0 / R1) and presence of metastasis. Early and timely multidisciplinary medical evaluation will always allow an adequate diagnosis and treatment to be established(AU)

Epidemiological dynamic modeling of human papillomavirus-related diseases to assess vaccination strategies in Argentina

Our objective was to develop and test a dynamic simulation model of human papillomavirus (HPV)- related diseases to assess rational vaccination strategies in Argentina. A dynamic stochastic transmission model for hetero- and homosexual transmission of HPV oncogenic and low-risk oncogenic types among females and males was developed. The model included HPV transmission and vaccination, the natural history of HPV-related diseases, disease outcomes, and cervical cancer screening. Considering all cervical cancers, covered or not by the current quadrivalent vaccine, the existing coverage rate would lead to 60% reduction in the global incidence of cervical cancer at 25 years, and to 79% at 50 years. Isolated current female vaccination without a screening program would need around 100 years to eliminate cervical cancer from the local population. Current coverage rate would lead to 59% reduction of vulvar cancer, 76% of vaginal cancer, 85% of anal cancer, and 87% of oropharyngeal cancer, estimated over a 25-year time prospect. Female HPV vaccination within the context of current cervical cancer screening should reach a minimum long-term mean coverage of 60% of girls, receiving at least a two-dose vaccine schedule, to significantly reduce or virtually eliminate cervical cancer at 50 years. Including vaccination to boys to improve herd immunity did not influence the incidence of cervical cancer over time, as long as female coverage did not fall below 50%. Regarding vulvar, vaginal, anal, penile, and some oropharyngeal cancers, current girls-only based vaccination could virtually eliminate these cancer types after 35-40 years, both in women and men.

Se desarrolló un modelo de simulación dinámica de enfermedades relacionadas con papilomavirus humano (VPH) para evaluar estrategias de vacunación. Se desarrolló un modelo dinámico estocástico para transmisión hetero/homosexual de VPH oncogénicos y de bajo riesgo oncogénico, entre mujeres y hombres. El modelo incluyó transmisión y vacunación contra VPH, historia natural de enfermedades relacionadas con VPH, mortalidad y programas de detección de cualquier cáncer de cuello uterino (CCU); teniendo en cuenta todos estos, con o sin vacunación cuadrivalente con la cobertura actual, la reducción sería 60% en la incidencia global de CCU en 25 años, y de 79% en 50 años. Vacunando solo mujeres, sin programa de detección precoz, necesitaría unos 100 años para eliminar el CCU localmente. La tasa de vacunación actual determinaría 59% de reducción del cáncer de vulva, 76% del cáncer vaginal, 85% del cáncer anal y 87% del cáncer orofaríngeo, a 25 años. La vacunación de mujeres, con el cribado actual del CCU, deberá alcanzar una cobertura media mínima a largo plazo del 60% de las niñas, con al menos dos dosis de vacunas, para reducir significativamente o eliminar el CCU en 50 años. La vacunación en niños para mejorar la inmunidad de grupo no influiría en la incidencia del CCU de n o caer la cobertura femenina por debajo de 50%. Con respecto a cánceres de vulva, vagina, ano, pene y algunos orofaríngeos, la vacunación actual solo en niñas podría eliminar virtualmente estos tipos de cáncer después de 35-40 años, tanto en mujeres como en hombres.

Vaginal cuff recurrence after radical cystectomy: an under - studied site of bladder cancer relapse

ABSTRACT Introduction: Vaginal cuff recurrence of tumor following radical cystectomy is a rare site of disease recurrence, however it has never been specifically studied. The aim of the study is to evaluate incidence, risk factors, and long-term oncologic outcomes of vaginal cuff recur- rence in a cohort of female patients treated with radical cystectomy for invasive urothelial carcinoma of the bladder. Materials and Methods: From 1985 to 2012, a prospectively maintained institutional blad- der cancer registry was queried for vaginal cuff recurrence post radical cystectomy. Over- all mortality and cancer-specific mortality were reported using the Kaplan-Meier method for patients with vaginal cuff recurrence, recurrence at another local or distant site, and those without evidence of recurrence. Comparisons were performed using the log-rank test. Cox proportional hazards regression model was performed to assess predictors of vaginal cuff recurrence. Results: From 469 women treated with radical cystectomy for bladder cancer, 34 patients (7.3%) developed vaginal cuff recurrence, 130 patients (27.7%) had recurrence involving ei- ther a local or distant site, and 305 patients (65%) had no evidence of recurrence. The 5-year overall mortality-free survival rate was 32.4% for vaginal cuff recurrence, but 25.0% for other sites of recurrence. Cancer-specific mortality-free survival rate was 32.4% for vaginal cuff recurrence, and 30.3% for the other sites of recurrence. Multivariate Cox proportional hazards regression analysis demonstrated that the presence of tumor in posterior location at radical cystectomy (Hazard Ratio [HR], 0.353 [95% CI, 0.159-0.783]) and anterior vaginec- tomy, compared to no vaginectomy (HR, 2.595 [95% CI, 1.077-6.249]) were independently associated with vaginal cuff recurrence. Conclusion: Anterior vaginectomy, despite our best attempts, is perhaps not sufficient to prevent vaginal cuff recurrence. Therefore, follow-up evaluation is essential, and further studies are necessary to address the optimal approach for initial management. Patient Summary: Although vaginal cuff recurrence is an unusual site of recurrence, careful evaluation is needed before cystectomy and during follow-up to identify patients at risk.

Leiomiosarcoma primario vaginal en el embarazo. A propósito de un caso / Primary vaginal leiomyosarcoma in pregnancy. About A case

RESUMEN Los sarcomas primarios de vagina son extremadamente raros especialmente durante la gestación. Se presenta el caso de una gestante de 25+2 semanas que acude a urgencias con clínica de sangrado vaginal objetivándose a la exploración una formación nodular dependiente de pared vaginal derecha. Dada la clínica se reseca en quirófano con el hallazgo histopatológico de leiomiosarcoma. A la gestante se le realiza sin incidencias una cesárea en la semana 34 de gestación, previa maduración pulmonar fetal, completándose en el puerperio inmediato el estudio de extensión sin encontrarse ningún hallazgo relevante. Finalmente, se somete a la paciente a una histerectomía y salpinguectomía bilateral con preservación ovárica. El seguimiento y los controles sucesivos de la gestante en los meses posteriores muestran ausencia de enfermedad.

ABSTRACT Primary sarcomas of the vagina are extremely rare, especially during pregnancy. We report a case of a 25-week-pregnant who came to emergency room complaining of vaginal bleeding. Pelvic examination revealed a globular mass located on the right vaginal wall. Given the persistent bleeding, resection of the mass was performed. The specimen was sent to pathology and confirmed to be a leiomyosarcoma. At 34-weeks of pregnancy after fetal lung maturation a cesarean section is done. It was completed the extension study during immediate postpartum without any relevant finding. Finally, she had a totally hysterectomy and bilateral salpingectomy with ovarian preservation. Clinical follow-up over the following months shows no evidence of disease.

Carcinoma neuroendocrino primario de vagina. Revisión de literatura a propósito de un caso / Primary neuroendocrine carcinoma of the vagina. Literature review on the subject of a case

RESUMEN El carcinoma primario de vagina representa 1 a 2% de los cánceres ginecológicos, siendo la diferenciación histológica neuroendocrina de células pequeñas extremadamente infrecuente, sólo se han reportado 28 casos en la literatura, describiéndose en orden de frecuencia en cérvix, endometrio, ovario, vagina y vulva. Se observa con más frecuencia en mujeres postmenopáusicas. Se presenta el caso de paciente femenino, de 39 años de edad, quien acude a la consulta por presentar secreción genital serohemática, fétida, de 1 mes de evolución, evidenciándose al examen físico lesión exofítica, friable, renitente, de aproximadamente 4 cm de diámetro, en tercio superior y cara posterolateral izquierda de vagina, por lo que se realiza biopsia excisional de dicha lesión, siendo el diagnóstico anatomopatológico carcinoma neuroendocrino de células pequeñas, grado histológico: 3. A propósito de este caso, se realiza una revisión del tema, haciendo hincapié en la importancia de la exploración ginecológica exhaustiva que incluya tomas de citologías y colposcopias periódicas, tomando en cuenta la vagina y así, realizar diagnóstico precoz en vista que el riesgo de carcinoma en dicha zona es infrecuente, mejorando el pronóstico y sobrevida de las pacientes.

ABSTRACT The primary carcinoma of the vagina represents 1 to 2 % of gynecologic cancers, being the histological differentiation neuroendocrine of small cells extremely infrequent, only 28 cases have been reported in the literature, describing in order of frequency: in the cervix, endometrium, ovary, vagina and vulva. It is most commonly seen in postmenopausal women. We present the case of a female patient, 39 years old, who comes to medical consult due to serohematic, fetid, genital discharge of 1 month of evolution, evidencing at physical examination an exophytic lesion, friable, renitent, with a diameter of approximately 4 cm, in the upper third and posterolateral left side of the vagina, for which an excisional biopsy of said lesion is performed, being the anatomopathological diagnosis neuroendocrine carcinoma of small cells, histological grade: 3. A bibliography reviewed was made, emphasizing the importance of exhaustive gynecological exploration with periodic cytology and colposcopy examinations, including the vagina for early diagnosis in view of carcinoma risk in this area is uncommon, improving the prognosis and survival of patients.

When an unexpected diagnosis occurs: a vaginal premenopausal sarcoma / Quando um diagnóstico inesperado ocorre: um sarcoma vaginal na pré-menopausa

Abstract Vaginal cancer is a rare entity. The evidence on its management resides mostly in clinical cases or small case series. Of the histological types, the most frequent is the squamous cell carcinoma, followed by adenocarcinoma. But what to do when identifying an even more infrequent sarcoma in a premenopausal woman? In this study, we describe the case of a 53-year-old woman presenting with metrorrhagia for two months, who was evaluated after an intense episode. A necrotic and ulcerative vaginal swelling was documented and then submitted to biopsy, which revealed a vaginal sarcoma. The patient was referred to radiation therapy with 50 Gy (aiming to control the symptoms and to cause tumor reduction for posterior pelvic exenteration with intraoperative radiotherapy) and developed an extra-pelvic metastization at the end of the treatment, which caused a fast negative outcome. Despite the initial poor prognosis, a chemo-irradiation or primary surgery regimen might have achieved (although with greater side effects) a better survival. This case-report entails a discussion about the strategies to manage vaginal sarcoma in advanced stage and in premenopausal women.

Resumo O cancro vaginal é uma doença rara. A evidência para a sua abordagem reside fundamentalmente em casos clínicos ou pequenas séries de casos. Dentre os tipos de cancro histológicos, o mais frequente é o carcinoma espinocelular, seguido do adenocarcinoma. Mas o que fazer em presença de um sarcoma ainda mais raro numa mulher pré-menopáusica? No presente estudo, descrevemos o caso de uma mulher de 53 anos apresentando metrorragia por dois meses, avaliada após um episódio intenso. Foi então documentada uma tumefacção vaginal necrótica e ulcerativa, submetida a biópsia, que revelou um sarcoma vaginal. A paciente foi encaminhada para radioterapia com50 Gy (comos objetivos de controlo da sintomatologia e de redução tumoral para posterior exenteração pélvica com radioterapia intraoperatória) e desenvolveu, ao final do tratamento, umquadro demestastização extra pélvica, que ocasionou um desfecho negativo rápido. Apesar do mau prognóstico inicial, um esquema de quimiorradiação ou cirurgia primária poderiam ter alcançado (ainda que com maiores efeitos laterais) uma maior sobrevivência. Este estudo de caso aborda uma discussão sobre as estratégias de abordagem do sarcoma vaginal em estádios avançados e na mulher pré-menopáusica.

Rabdomiosarcoma botrioide del aparato genital femenino en paciente adolescente virgen / Botryoid rhabdomyosarcoma of the female genital apparatus in a virgin adolescent

El rabdomiosarcoma es un tumor complejo y de gran malignidad que se origina en las células del mesénquima embrionario con capacidad para diferenciarse en células musculares esqueléticas. Este es el tumor maligno de tejido blando más frecuente. Representa aproximadamente 3,5 por ciento de los casos de cáncer en niños de 0 a 14 años de edad. La presentación clínica del rabdomiosarcoma embrionario variedad botrioides es, en general, una masa que protruye por la uretra o el introito vaginal, o por la presencia de flujo fétido o sangrado vaginal en niñas menores de 2 años. Se presenta el caso de un rabdomiosarcoma botrioides de la vagina diagnosticado es una paciente de 16 años y virgen. El apoyo diagnóstico con inmunohistoquímica es de vital importancia y la evaluación médica multidisciplinaria precoz y oportuna permitirá siempre establecer un diagnóstico y tratamiento adecuados que mejoren el pronóstico de quienes padecen esta enfermedad(AU)

Rhabdomyosarcoma is a complex tumor of great malignity that originates in the embryonary mesenchymal cells with the capacity of differentiating into skeletal muscle cells. This is the most frequent malignant tumor in the soft tissue. It roughly represents 3.5 percent of cancer in children aged 0 to 14 years. Generally, the clinical presentation of botryoid-type embryonary rhabdomyosarcoma is a mass that protrudes from the urethra or the vaginal introit or the presence of fetid fluid or vaginal bleeding in girls under 2 years-old. This is the case of a 16 years-old virgin female patient diagnosed with botryoid rhabdomyosarcoma of the vagina. The diagnostic support with immunohistochemistry is of vital importance in addition to the early and timely multidisciplinary medical assessment for setting adequate diagnosis and treatment that improve the prognosis of persons suffering this disease(AU)

Aspectos clínicos, cirúrgicos, histológicos e pós-operatórios de oito cadelas com leiomioma vaginal / Clinical, surgical, histological and postoperative aspects in the case of eight bitches with vaginal leiomyoma

O presente trabalho reporta as características clínicas e cirúrgicas de oito casos de cadelas portadoras de leiomioma vaginal, com histórico de aumento de volume na região perineal, aparecimento de massa pela vulva, disquezia, tenesmo e disúria. As pacientes foram submetidas à cirurgia de episiotomia para ressecção da massa neoplásica, e em quatro casos foi necessária a ovariossalpingo-histerectomia. Porém, uma delas apresentou recidiva, e, no segundo procedimento cirúrgico de exérese de neoformação, o histopatológico diagnosticou leiomiossarcoma. A paciente ainda apresentou mais duas recidivas, até o proprietário optar pela eutanásia. Decorridos quatro meses, as sete cadelas diagnosticadas com leiomioma não apresentaram recidiva, e os proprietários relataram ausência de sinais relacionados com a doença descrita. Conclui-se que a episiotomia para ressecção da massa vaginal e a ovariossalpingo-histerectomia nas cadelas inteiras nos casos de tumores vaginais benignos são tratamentos satisfatórios, com bom prognóstico, ao contrário do leiomiossarcoma.

This study reports the clinical and surgical procedures of eight cases of bitches with vaginal leiomyoma. All animals presented increased volume in the perineal region, and exteriorization of the vulva, dyschezia, tenesmus and dysuria. The patients underwent episiotomy surgery for resection of the neoplastic mass, and ovariosalpingohisterectomy in four cases. However, one had recurrence on the second surgical procedure for removal of neoformation, histopathological diagnosed leiomyosarcoma, the patient also had two more relapses, until the owner chose euthanasia. After four months, the seven dogs diagnosed with leiomyoma showed no recurrence and the owners reported no signs of the disease described. We concluded that episiotomy for vaginal mass resection combined with ovariohysterectomy in cases of benign vaginal tumors have shown satisfactory treatment with good prognostic.

Cervical cancer screening in young and elderly women of the Xingu Indigenous Park: evaluation of the recommended screening age group in Brazil / Rastreamento do câncer de colo uterino em jovens e idosas do Parque Indígena do Xingu: avaliação quanto à faixa etária preconizada no Brasil

Objective To analyze the occurrence of atypia in the cytology/histology examinations of young women under the age of 25 years and of elderly women aged over 64 years, in the Xingu Indigenous Park and to evaluate, in a subjective manner, if the age range for screening established by the Ministry of Health and the Instituto Nacional de Câncer is appropriate for this population. Methods The Xingu/UNIFESP Project, in partnership with the Center for Gynecological Disease Prevention, develops programs to prevent cervical cancer. The exploratory, retrospective and descriptive study of cytological and histopathological examinations of young (12-24 years) and elderly (aged 64 and over) women of the Xingu Indigenous Park, between 2005 and 2011. Results There was low occurrence of cytological atypia in the elderly female population, but there were occasional high-grade lesions in the indigenous youth. Conclusion Interrupting screening at the limit age of 64 years, as established by the Ministry of Health and the Instituto Nacional de Câncer is justified. However, screening of young women should begin at an earlier age. .

Objetivo Analisar a ocorrência de atipias nos exames citológicos e histológicos de jovens e idosas indígenas, e também avaliar se a faixa etária preconizada pelo Ministério da Saúde e pelo Instituto Nacional de Câncer em rastreamento do câncer de colo uterino é adequada para essa população. Métodos O Projeto Xingu/UNIFESP, em parceria com o Núcleo de Prevenção de Doenças Ginecológicas, desenvolve programas de prevenção do câncer do colo de útero. Foi realizado estudo exploratório, retrospectivo e descritivo de exames cito/histopatológicos em jovens do Parque Indígena do Xingu de 12 a 24 anos e em idosas a partir dos 64 anos, no período de 2005 a 2011. Resultados Em idosas, houve baixa ocorrência de atipias citológicas, mas nas jovens indígenas, ocorreram casos eventuais de lesão de alto grau. Conclusão Justifica respeitar o limite de 64 anos para a interrupção do rastreamento, como o estabelecido pelo Ministério da Saúde/Instituto Nacional de Câncer, mas, nas jovens, seria importante iniciar o rastreamento mais precocemente. .

Opções terapêuticas na neoplasia intraepitelial vaginal: revisão de literatura / Therapeutic modalities for vaginal intraepitelial neoplasia: literature review

A neoplasia intraepitelial de vagina (NIVA) é uma condição pré-maligna rara do epitélio vaginal, sua incidência corresponde a 0,2 casos por 100.000 mulheres, classifica-se em NIVA I, II, III esta última tem um potencial de invasão de 9 a 12%. O diagnóstico é feito pela citologia, colposcopia, histopatologia. Todos os métodos terapêuticos tem uma taxa razoável de sucesso e a taxa de recorrência varia entre 10 e 42%. O tratamento pode ser: cirúrgico, ablativo, radioterápico, clínico e expectante. O excisional tem preferência por fornecer peça para análise histopatológica excluindo a doença invasiva apresentando as melhores taxas de sucesso entre 66% a 83% . A ablação é indicada quando a suspeita de invasão foi afastada e é a modalidade de eleição nas mulheres jovens, a eficácia varia entre 69 a 87%. A radiação tem uma taxa de sucesso entre 69 a 100%; porém, as complicações giram em torno de 36%. Pela sua praticidade o tratamento feito com os agentes tópicos tem ganhado espaço terapêutico na abordagem da NIVA. Não existe evidência científica quanto a melhor forma e o tempo de seguimento das mulheres com NIVA. Qualquer que seja a opção de tratamento utilizada um seguimento longo deve ser preconizado.(AU)

Vaginal intraepithelial neoplasia (VAIN) is an uncommon premalignant condition of the vaginal epithelium, its incidence is found to be 0.2 per 100.000 women, this condition is classificated in VAIN I, II and III, the lifetime risk of transformation to invasive carcinoma has been reported as 9 to 12%. The diagnosis was done by cytology, colposcopy and histology. All the therapeutics modalities have a successful cure rate and the recurrence ranges are between 10 to 42%. The treatment modalities are: excisional, ablative, radiotherapic, clinical and expectante. Excisional has preference as it could exclude invasion disease and has the best success rate, 66 to 83%. Ablation has preference in young women with no suspect of invasion, with efficacy ranging between 69 to 87%. The cure rate of radiotherapy is high as 69 to 100% but this modality has 36% morbidity rate. Treatment with topical agents constitutes a promising option in VAIN management. There is no scientific evidence of what kind follow-up is better. Whatever treatment modality is used long-term follow-up is essential.(AU)

Aggressive angiomyxoma of the vagina: a case report / Angiomiosarcoma agressivo da vagina: relato de caso

Aggressive angiomyxoma is a rare, slow-growing soft tissue tumor that usually arises in the pelvis and perineal regions of women in reproductive age, with a marked tendency to local recurrence. Because of its rarity, it is often initially misdiagnosed. Surgical resection is the main treatment modality of aggressive angiomyxoma. We describe a case of a vaginal aggressive angiomyxoma in a 47-year-old woman in which the diagnosis was only made after histological examination. The etiology, presentation, diagnosis and management of this rare tumor are outlined. Angiomyxoma of vulva and vagina refers to a rare disease. Pre-operative diagnosis is difficult due to rarity and absence of diagnostic features, but it should be considered in every mass in genital, perianal and pelvic region in a woman in the reproductive age. Thus, these cases should have complete radiological workup before excision, as pre-diagnosis can change the treatment modality and patient prognosis'.

O angiomixoma agressivo é tumor raro de tecidos moles, de lento crescimento e que geralmente ocorre na pelve e nas regiões perineais de mulheres em idade reprodutiva, com forte tendência à recorrência local. Devido à sua raridade, ele é frequentemente mal diagnosticado. A exerese cirúrgica é a modalidade principal para o tratamento do angiomixoma agressivo. Descrevemos aqui um caso de angiomixoma vaginal agressivo em mulher de 47 anos de idade, que foi diagnosticado apenas após exame histológico. Descrevemos também a etiologia e apresentação, bem como o diagnóstico e tratamento desse raro tumor. O angiomixoma da vulva e vagina constitui doença rara. Devido à raridade desse tumor e à ausência de elementos diagnósticos, o diagnóstico pré-operatório é difícil, mas deveria ser considerado na presença de qualquer massa na região genital, perianal e pélvica de mulheres em idade reprodutiva. Portanto, esses casos deveriam ser submetidos a propedêutica radiológica completa antes da exerese, uma vez que um pré-diagnóstico pode modificar a modalidade de tratamento e o prognóstico do paciente.

Linfoma no Hodgkin primario de vagina / Primary non Hodgkin's lymphoma of the vagina

La afectación primaria del tracto genital femenino de los linfomas no Hodgkin es poco frecuente (2 por ciento de los linfomas primarios extraganglionares). Los órganos más afectados son los ovarios seguidos del cérvix, siendo la localización endometrial y vaginal extremadamente rara. Presentamos el caso de una paciente de 44 años diagnosticada de linfoma primario de vagina, estadio IE A, con remisión completa tras tratamiento combinado quimioterápico y quirúrgico. Es importante tener presente estas raras aunque posibles localizaciones de linfomas para evitar que se puedan confundir con lesiones inflamatorias u otros tipos de tumores que nos lleven a un fracaso terapéutico.

Primary non-Hodgkin lymphomas rarely involve the female genital tract (2 percent of primary extranodal lym-phomas). The ovaries followed by the cervix are the most common affected sites while the involvement of the endometrium and vagina is extremely rare. We report the case of a 44 year old woman with a primary lymphoma of the vagina, stage IE A, with complete remission after combined chemotherapy and surgical treatment. These rare but possible locations must be kept in mind to avoid misdiagnosis with inflammatory lesions or other types of tumors which may lead us to a therapeutic failure.

Neoplasia vaginal intraepitelial / Vaginal intraepithelial neoplasia

Objetivo: actualizar los conocimientos disponibles sobre la neoplasia vaginal intraepitelial (VAIN) especialmente en el diagnóstico y tratamiento. Métodos: revisión de la literatura en Pubmed de los últimos 20 años, especialmente de los publicados desde 2005 hasta la actualidad y considerando sobre todo los ensayos clínicos aleatorizados. Resultados: su prevalencia real es desconocida, aunque es una patología rara generalmente en mujeres posmenopaúsicas. Su fisiopatología es similar a la neoplasia cervical intraepitelial, con el HPV como principal factor de riesgo, sobre todo el serotipo 16, pero su progresión a cáncer es menor. Un grupo importante es el de VAIN tras histerectomía, que supone el 48-70 por ciento del total de las VAIN. La colposcopia para el diagnóstico no es sencilla y se aconseja preparación con estrógenos en las pacientes menopáusicas y utilización de ácido acético y lugol para identificar las zonas sospechosas. Las tres opciones terapéuticas son la cirugía (excisional, láser, ultrasonidos), braquiterapia y tratamiento médico (imiquimod, 5-fluorouracilo, ácido tricloroacético). Se expone la técnica y las ventajas e inconvenientes de cada uno de ellos. Conclusión: aunque la VAIN es una entidad rara, es preciso tenerla en cuenta ante un diagnóstico de lesión citológica. Su diagnóstico a veces no es sencillo y el tratamiento dependerá del grado, la localización y el tamaño de la lesión y las circunstancias personales de la paciente. Se necesitan más ensayos aleatorizados que comparen la eficacia entre las distintas opciones terapéuticas y su repercusión en la calidad de vida de las pacientes.

Aims: update the knowledge about vaginal intraepithelial neoplasia (VAIN) with special emphasis on diagnosis and therapeutic management. Method: electronic search of Pubmed of all kinds of articles about the VAIN, for the last 20 years with special attention to those published from 2005 to the present and considering especially randomized clinical trials. Results: its prevalence is unknown, although it is a rare condition that usually occurs in postmenopausal women. The physiopathology is similar to cervical intraepithelial neoplasia, being HPV the main risk factor, particularly serotype 16, but its progression to cancer is lower. An important group is VAIN after hysterectomy, 48-70 percent of total VaIN. Colposcopy for the diagnosis is not easy and in patients with postmenopausal the preparation with local estrogen is necessary and use both of acetic acid and lugol to identify suspicious areas. The three treatment options are surgery (excisional, laser, ultrasound), brachytherapy and medical management (imiquimod, 5-fluorouracil, tricholoroacetic acid). The technique and the advantages and disadvantages of each are explained. Conclusion: although VAIN is rare, it must take it into account before a cytological diagnosis of dysplasia. Its diagnosis is sometimes not easy and the treatment depends on the extent, location and size of the lesion and the individual preferences of the patient. We need more randomized trials comparing different treatment options and also their impact on quality of life of patients.

Comparative study of the results from conventional cervico-vaginal oncotic cytology and liquid-based cytology / Estudo comparativo dos resultados obtidos pela citologia oncótica cérvico-vaginal convencional e pela citologia em meio líquido

OBJECTIVE: To compare two oncotic cervical cytology techniques, the conventional and the liquid-based cytology, in low risk patients for uterine cervical cancer. METHODS: Comparative prospective study with 100 patients who came to their annual gynecological exam, and were submitted simultaneously to both techniques. We used the McNemar test, with a significance level of p<0.05 to compare the results obtained related to adequacy of the smear quality, descriptive diagnosis prevalence, guided biopsy confirmation and histology. RESULTS: Adequacy of the smear was similar for both methods. The quality with squamocolumnar junction in 93% of conventional cytology and in 84% of the liquid-based cytology had statistical significance. As for the diagnosis of atypical cells they were detected in 3% of conventional cytology and in 10% of liquidbased cytology (p=0.06). Atypical squamous cells of undetermined significance were the most prevalent abnormality. The liquid-based cytology performance was better when compared with colposcopy (guided biopsy), presenting sensitivity of 66.7% and specificity of 100%. There was no cytological and histological concordance for the conventional cytology. CONCLUSIONS: Liquid-based cytology had a better performance to diagnose atypical cells and the cytohistological concordance was higher than in the conventional cytology.

OBJETIVO: Comparar duas técnicas de colpocitologia oncótica, a convencional e a em meio líquido, em pacientes de baixo risco para carcinoma de colo uterino. MÉTODOS: Trata-se de estudo prospectivo e comparativo, em que foram avaliadas cem mulheres que compareceram à consulta médica de rotina e foram submetidas simultaneamente à coleta pelas duas técnicas de citologia. Os resultados obtidos em relação à adequabilidade, à qualidade dos esfregaços, à prevalência nos diagnósticos descritivos e à confirmação com biópsia dirigida e histologia foram comparados pelo teste de McNemar, com nível de significância de p<0,05. RESULTADOS: A adequabilidade dos esfregaços mostrou-se semelhante. A qualidade, com presença de elementos da junção escamo-colunar em 93% das citologias convencionais e 84% das citologias em meio líquido, teve significância estatística. Nos diagnósticos de atipias, elas foram detectadas em 3% das citologias convencionais e em 10% das citologias em meio líquido (p=0,06), sendo as atipias em células escamosas de significado indeterminado a alteração mais prevalente. Quando comparadas à colposcopia com biópsia dirigida, o desempenho da citologia em meio líquido foi superior, com sensibilidade de 66,7% e especificidade de 100%, enquanto que, para a citologia convencional, não houve concordância cito-histológica. CONCLUSÃO: O desempenho em diagnosticar atipias e a concordância cito-histológica da citologia em meio líquido foram superiores ao da citologia convencional.

Reconstrucción de un defecto vulvar crónico secundario al tratamiento de un carcinoma primario de la vagina: reporte de un caso y consideraciones de manejo / Reconstruction of a chronic secondary vulvar defect upon treatment of primary vaginal carcinoma: a case report and management considerations

El cáncer de vagina es una neoplasia rara, que constituye un 1% del cáncer genital femenino, aunque ha aumentado su incidencia en los últimos años. El manejo quirúrgico, adyuvante y reconstructivo resulta importante en el resultado oncológico y funcional de la enfermedad, pero puede comprometer la anatomía y función vaginal, y la de los órganos adyacentes. El avance en las terapias quirúrgicas y adyuvantes en pacientes más jóvenes ha conducido a una mayor supervivencia, implicando la necesidad de un mejor manejo del defecto anatómico de la lesión o de las secuelas de su tratamiento. Debe intentarse siempre que sea necesario un manejo multidisciplinario, desde la primera intervención, incluyendo la técnica reconstructiva correspondiente al defecto. Se presenta un caso de manejo conjunto oncológico y reconstructivo, mediante colgajo unilateral en flor de loto de un defecto vulvar crónico, originado tras el manejo de un cáncer vaginal primario previo.

Vaginal cancer is a rare neoplasm which makes up 1% of female genital cancer; however, its incidence has risen in the past few years. Adjuvant and reconstructive surgical management prove to be important in the oncologic and functional outcome of the disease, but may involve anatomy and function in the vagina, as well as in adjacent organs. Breakthroughs in surgical and adjuvant therapy have led to greater survival rates among younger patients, thus implying a greater need for improved management of the anatomical defect in the lesion or of treatment side effects. An effort should always be made towards interdisciplinary management, from the initial interven tion, including the corresponding reconstructive technique used on the defect. A case is presented on the combined oncologic and reconstructive management achieved by means of a lotus flower unilateral flap for a chronic vulvar defect brought about by previous primary vaginal cancer.

Carcinoma de ano, pene, vulva y vagina: ¿son enfermedades de transmisión sexual? / Anogenital neoplasms: are sexually transmitted diseases?

Introducción: La infección por virus papiloma humano (VPH) sería factor causal de cánceres de ano, pene, vulva y vagina. Objetivo: Analizar la evidencia actual en cuanto a infección por VPH y su rol carcinogénico en estas neoplasias. Metodología: Búsqueda de la literatura para identificar artículos sobre la transmisión sexual como factor de riesgo en cánceres anogenitales. Resultados: En lesiones premalignas y malignas anogenitales se encuentra en gran frecuencia el DNA de VPH, especialmente tipo 16. Se ha demostrado que la vacunación contra VPH previene el desarrollo de lesiones preinvasoras anales; en cambio, ni la vacuna ni la circuncisión parecen ser factores protectores contra cáncer de pene. Discusión: No hay estudios prospectivos que permitan establecer una relación causal entre VPH y cánceres anogenitales, lo que impide la elaboración de estrategias de prevención. El manejo de ciertos factores de riesgo sugeridos previamente en la literatura no reduce el riesgo de cáncer anogenital.

Introduction: Human papillomavirus (HPV) infection has been suggested as a causal factor of anal, penile, vulvar and vaginal cancers. Objective: To analyze current evidence about HPV infection and its carcinogenic role in these neoplasms. Methodology: Literature search to identify articles about sexual transmission as a risk factor in anogenital cancers. Results: In premalignant and malignant anogenital lesions, an important presence of HPV DNA is often found, specially type16. It has been demonstrated that HPV vaccine prevents premalignant anal lesions; however, this vaccine and circumcision do not seem to be protective against penile cancer. Discussion: There are no prospective studies that had established a causal relationship between HPV and anogenital cancers. This keeps off the development of adequate prevention strategies. Management of certain previously suggested risk factors do not reduce the risk of anogenital cancer.