Anal intraepithelial neoplasia associated with high-grade vulva injury (usual-type vulvar intraepithelial neoplasia): surgical treatment with cutaneous flap advancement

The aim of the present article is to report the case of a young patient with bowenoid papulosis who was a carrier of other sexually-transmitted infections (STIs), such as HIV and high-grade vulva lesion (usual-type vulvar intraepithelial neoplasia, VIN), and to demonstrate the strategy used to manage the case, as well as to discuss important issues regarding the standardization of intraepithelial lesions. (AU)

A case of chronic and relapsing paget disease of the vulva

Abstract Extramammary Paget disease is a rare neoplastic condition that more commonly affects postmenopausal Caucasian women. Although the vulvar area is the most frequently affected location, it corresponds solely to 1 to 2% of all vulvar malignancies. A 72-year-old female patient was observed in our outpatient clinic with a 2-year history of an erythematous and pruritic plaque on the vulva. Histopathology and immunohistochemistry studies were compatible with extramammary Paget disease of the vulva. Associated neoplastic conditions were excluded. Due to multiple relapses, the patient was submitted to three surgical interventions, including a total vulvectomy, and to external radiotherapy. The present case illustrates the chronic and recurrent nature of extramammary Paget disease despite aggressive procedures as well as the challenge in obtaining tumor-free resection margins.

Melanoma vulvar en una paciente de la tercera edad / Vulvar melanoma in a third age patient

Se describe el caso clínico de una paciente de 62 años de edad, negra, atendida en el Hospital Oncológico Docente Provincial Conrado Benítez García de Santiago de Cuba, por presentar aumento de volumen en el labio menor que comenzó a doler. Se realizó biopsia excisional, cuyo resultado reveló un melanoma vulvar. Se remitió a la consulta de Ginecología oncológica donde se le indicó tratamiento con quimioterapia e inmunoterapia por ser un tumor no operable. Se le administraron 4 ciclos de quimioterapia con buena respuesta y estabilidad. Se continuó su atención en consulta de seguimiento.

The case report of a black-skinned 62 years female patient assisted in Conrado Benítez García Teaching Provincial Oncological Hospital in Santiago de Cuba is described due to an increase of volume in the outer lip that began to hurt. A excisional biopsy was carried out which result revealed a vulvar melanoma. She was referred to the Oncological gynecology service where she was indicated treatment with chemotherapy and immunotherapy for being a non-surgical tumor. Four chemotherapy cycles were administered with good response and stability. Her care continued through follow-up.

Reconstrucción vulvar con colgajo fasciocutáneo del surco glúteo / Vulvar reconstruction with fasciocutaneous fl ap of the gluteal fold

Introducción. La reconstrucción inmediata de la región vulvar posterior a vulvectomía radical por patología oncológica representa un desafío para el cirujano plástico dado el tamaño del defecto que genera dicha cirugía, en relación con la complejidad que posee la región anatómica a tratar. Material y métodos. Se presenta el caso de una paciente de sexo femenino de 74 años de edad con antecedentes de resección local de tumor vulvar asociado a radioterapia adyuvante en otra Institución, 5 años previos a la consulta. Actualmente presenta recidiva de su enfermedad. Se aborda de forma multidisciplinaria y se realiza vulvectomía radical más reconstrucción inmediata del defecto con dos colgajos del surco glúteo. Resultado. Una vez realizada la cirugía, la paciente fue dada de alta de la Institución al séptimo día posoperatorio y fue instruida para evitar sentarse durante 7 días más. Al 12 día posoperatorio presentó una dehiscencia parcial de la herida en región subglútea derecha, la cual se resolvió suturándola nuevamente. Al cumplirse 1 mes de la cirugía, la paciente se encontró satisfecha con el resultado estético y en condiciones de volver a su vida normal desde el punto de vista funcional y estético. Conclusión. El presente trabajo confirma que la utilización del colgajo fasciocutáneo del surco glúteo es una buena elección para la reconstrucción de la región vulvar, tanto estética como funciona

Background. The immediate reconstruction of the vulvar region after radical vulvectomy due to oncological pathology represents a challenge for the plastic surgeon do to the size of the defect generated by said surgery, in relation to the complexity of the anatomical region to be treated. Material and Methods. We present the patient 74-years-old woman with a history of local resection of vulvar tumor associated with adjuvant radiotherapy in another Institution and currently she has recurrence of the disease. Is addressed by a multidisciplinary team and radical vulvectomy is performed with immediate reconstruction of the defect with two gluteal fold fl aps. Results. Once the surgery was performed, the patient was discharged from the Institution on the seventh postoperative day and was instructed to avoid sitting for 7 days. On the 12th postoperative day, she presented a partial dehiscence of the wound, which was resolved by suturing it. One month after surgery, the patient was satisfi ed with the aesthetic result and able to return to her normal life from a functional point of view. Conclusion. The present work confi rms that the use of the fasciocutaneous fl ap of the gluteal fold is a very good option for the vulvar regionreconstruction, not only functionally as aesthetically

Vulvar melanoma: relevant aspects in therapeutic management

Abstract Cancer of the vulva accounts for at least 1% of malignant neoplasms among women. Although rare, vulvar melanoma is the second most common histological type of vulvar cancer, representing 7-10% of all malignant vulvar neoplasms. Initial symptoms are non-specific and complete excision of the lesion is indicated in cases with suspected diagnosis. Prognosis of patients with these neoplasms is poor and remains unchanged despite the treatment approach. Hemivulvectomy with lymph node dissection is the current procedure of choice, associated or not with adjuvant therapies. We report two cases of patients presenting with late diagnosed vulvar melanoma and the relevant aspects in their therapeutic management.

Mammary and extramammary Paget's disease

Paget's disease, described by Sir James Paget in 1874, is classified as mammary and extramammary. The mammary type is rare and often associated with intraductal cancer (93-100% of cases). It is more prevalent in postmenopausal women and it appears as an eczematoid, erythematous, moist or crusted lesion, with or without fine scaling, infiltration and inversion of the nipple. It must be distinguished from erosive adenomatosis of the nipple, cutaneous extension of breast carcinoma, psoriasis, atopic dermatitis, contact dermatitis, chronic eczema, lactiferous ducts ectasia, Bowen's disease, basal cell carcinoma, melanoma and intraductal papilloma. Diagnosis is histological and prognosis and treatment depend on the type of underlying breast cancer. Extramammary Paget's disease is considered an adenocarcinoma originating from the skin or skin appendages in areas with apocrine glands. The primary location is the vulvar area, followed by the perianal region, scrotum, penis and axillae. It starts as an erythematous plaque of indolent growth, with well-defined edges, fine scaling, excoriations, exulcerations and lichenification. In most cases it is not associated with cancer, although there are publications linking it to tumors of the vulva, vagina, cervix and corpus uteri, bladder, ovary, gallbladder, liver, breast, colon and rectum. Differential diagnoses are candidiasis, psoriasis and chronic lichen simplex. Histopathology confirms the diagnosis. Before treatment begins, associated malignancies should be investigated. Surgical excision and micrographic surgery are the best treatment options, although recurrences are frequent.

Tumores no espinocelulares de vulva. Incidencia en ocho años / Non-squamous-cell tumors of the vulva. Study of eigth-year incidence

Introducción.La vulva puede ser asiento de diversos tumores malignos, de los cuales el más frecuente es el espinocelular; sin embargo, los tumores vulvares no espinocelulares son de gran importancia debido su mal pronóstico.Objetivos. Determinar la forma de presentación clínica, sintomatología, variedad histopatológica y el estadio de los tumores vulvares no espinocelulares al momento de la consulta. Evaluar el rol del dermatólogo en el diagnóstico y seguimiento de estos pacientes. Métodos. Se realizó un estudio retrospectivo, observacional y analítico de pacientes que concurrieron por derivación al consultorio de Patología Vulvar desde agosto de 2002 a agosto de 2010. Se evaluaron las historias clínicas y los archivos anatomopatológicos e iconográficos delos tumores vulvares no espinocelulares. Resultados. De un total de 637 consultas por derivación en el consultorio de patología vulvar, el 9,1% (n=56) correspondió a tumores malignos. De éstos, el 76,78% (n=43) fueron carcinoma espinocelular (incluidos neoplasia intravulvar y carcinoma invasor) y el 23,22% (n=15), carcinoma no espinocelular. Del total de estos últimos, ocuparon en orden de frecuenta decreciente: tumores secundarios de otros órganos, enfermedad de Paget extramamaria, melanoma, adenocarcinoma de la glándula de Bartolino y sarcoma. El motivo de consulta más frecuente fue dolor, y la forma clínica de presentación fue lesión exofítica única. Conclusiones. Destacamos la alta incidencia de tumores vulvares no espinocelulares en nuestro medio: representa el 23,2% de las neoplasias vulvares, en comparación con la literatura, que es del 10%.

Background. The vulva may be the seat of many malignancies, of which the most common is squamous-cell carcinoma; nevertheless non squamous-cell vulvar neoplasms are significant because of their poor prognosis. Objectives. To determine the symptoms, clinical presentation and stage of the disease at thetime of consultation. To establish associations with various risk factors and evaluate prognosis. Toasess the role of the dermatologist in the appropriate diagnosis and monitoring of these patients.Methods. We performed a retrospective and observational study of patients seen at a vulvar-disease Clinic from August 2002 to August 2010. We reviewed the clinical records, biopsy specimens and iconographic files.Results.From a total of 680 consultations to a vulvar-disease Clinic 7% (n=48) corresponded to malignant tumors. Of these 73% (n=35) were squamous-cell carcinomas and 27% (n=13) were non-squamous-cell carcinomas, which in decreasing order of frequency, were: secondarytumors from other organs, extramamammary Paget’s disease, melanoma, adenocarcinoma andsarcoma. The most frequent cause of consultation was pain, and the clinical presentation was a single exophytic lesion.

Angioceratoma da vulva / Angiokeratoma of the vulva

Os angioceratomas são tumores benignos caracterizados por ectasia dos vasos sanguíneos da derme papilar associada à acantose e hiperceratose da epiderme. Ao exame dermatológico, os angioceratomas de Fordyce caracterizam-se por lesões papulo-ceratóticas de coloração eritemato-violácea. São mais comuns no escroto, sendo raramente relatada a localização vulvar. A histopatologia é particularmente importante para difer enciá-los de outros tumores benignos e malignos. O artigo relata o caso de uma mulher negra de meia idade com história de obstipação intestinal, varizes de membros inferiores e cesárea há 20 anos que apresentava múltiplos angioceratomas vulvares há três meses.

Angiokeratomas are benign tumors characterized by ectasia of blood vessels in the papillary dermis associated with acanthosis and hyperkeratosis of the epidermis. Dermatological examination of angiokeratomas of Fordyce is characterized by papular keratotic lesions of erythematous-violet color. They are more common in the scrotum, and vulvar involvement is rarely reported. Histopathology is particularly important to distinguish them from other benign and malignant tumors. The article reports the case of a middle-aged black woman with a history of chronic constipation, varicose veins of the lower limbs and cesarean section performed 20 years ago who had had multiple vulvar angiokeratomas for three months.

Série de casos de neoplasia intraepitelial vulvar tratados com imiquimode / Case series of vulvar intraepithelial neoplasia treated with imiquimod

O termo neoplasia intraepitelial vulvar (NIV) inclui as lesões precursoras do câncer de vulva, sendo divididas em NIV escamosa e não escamosa. A NIV escamosa compreende a NIV tipo usual, a qual se encontra relacionada à infecção pelo HPV, à multicentricidade de lesões, à atividade sexual e ao fumo,sendo mais frequentemente observada em mulheres jovens. Já a NIV diferenciada está relacionada ao líquen escleroso, não estando associada à infecção induzida por HPV, nem ao comportamento sexual, acometendo pacientes mais idosas. A NIV não escamosa está relacionada à doença de Paget e ao melanoma in situ. O tratamento padrão da NIV é cirúrgico e consiste na excisão ampla da lesão com margem de segurança. Este estudo objetiva avaliar ouso do imiquimode creme a 5% no tratamento da NIV tipo usual de forma isolada e/ou, associado ao procedimento cirúrgico. O imiquimode já é utilizadocom eficácia no tratamento de lesões condilomatosas genitais. Além disso, tem-se mostrado eficaz em lesões de NIV tipo usual.

The term vulvar intraepithelial neoplasia (VIN) includes the precursor lesions of vulvar cancer, being divided into squamous VIN and non-squamous VIN. The squamous VIN refers to the usual type VIN, which is related to HPV infection, multicentricity of lesions, sexual activity and smoking beingmore frequently observed in young woman. The VIN differentiated type, which is related to vulvar dermatoses, such as lichen sclerosus, is not associated with HPV-induced infection or sexual behavior and affects older patients. Thenon-squamous VIN is related to Paget's disease and melanoma in "situ".The standard VIN's treatment is a surgical wide excision of the lesion with a safety margin. This study aims to test the use of imiquimod 5% cream in thetreatment of usual type VIN isolated, and/or associated with surgical procedure. The imiquimod is already successfully used in the treatment of genital condylomatous lesions. Moreover, it has been shown to be effective in usual type VIN lesions.

Enfermedad de Paget vulvar / Vulvar Paget`s disease

Se describe el caso de una paciente con diagnóstico de enfermedad de Paget vulvar, la cual fue tratada con cistostático tópico local con criterio coadyuvante al tratamiento quirúrgico. Hubo mejoría clínica evidente, pero la paciente falleció 10 meses después por una afección cardiovascular secundaria a hipertensión arterial. Teniendo en cuenta la escasa frecuencia de esta entidad y de su tratamiento no quirúrgico, con buenos resultados inmediatos, se muestra a la comunidad científica como una opción paliativa o previa a la cirugía definitiva.

The case of a patient with diagnosis of vulvar Paget`s disease, who was treated with local topical cytostatic with coadjutant approach for surgical treatment is described. There was evident clinical improvement, but the patient died 10 months later due to a cardiovascular disorder secondary to hypertension. Keeping in mind the scarce frequency of this disease and of its non surgical treatment, with good immediate results, it is shown to the scientific community as a palliative option or an option previous to the definitive surgery.

Neoplasia intraepitelial vulvar: um problema atual / Vulvar intraepithelial neoplasia: a current problem

A neoplasia intraepitelial da vulva (VIN) é uma denominação que foi introduzida incialmente pela International Society for Study of Vulvo-vaginal Diseases (ISSVD) e reconhecida posteriormente pela International Society of Gynaecological Pathology (ISGYP) e Organização Mundial da Saúde. É uma entidade patológica a que correspondem as VIN de tipo usual (verrucoso, basalióide e misto) e as VIN de tipo diferenciado. A incidência das lesões de VIN tem aumentado progressivamente, principalmente em mulheres jovens. A infecção pelo papilomavírus humano (HPV) de alto risco, pelo vírus da imunodeficiência humana (HIV), o tabagismo e a neoplasia intraepitelial do colo do útero, da vagina e região anal são factores de risco estabelecidos para as VIN. Não existem sintomas e sinais característicos das VIN, mas a doença se traduz sempre por lesões clinicamente identificáveis. A biópsia com o auxílio do colposcópio permite o diagnóstico. O tratamento da doença está sempre justificado pelo elevado risco de progressão para cancro invasivo. A excisão alargada das lesões ou a sua destruição com laser CO2 têm sido os métodos mais populares de tratamento. Independentemente do método terapêutico utilizado, as taxas de recidiva são elevadas, pelo que está aconselhada a vigilância apertada das doentes após tratamento. A terapêutica tópica com imiquimod se afigura promissora no tratamento das VIN. As vacinas profiláticas contra os tipos de HPV de alto risco prometem se tornar armas poderosas na prevenção primária da doença.

Vulvar intraepithelial neoplasia (VIN) is a pathological denomination coined by the International Society for Study of Vulvo-vaginal Diseases (ISSVD) and adopted by the International Society of Gynaecological Pathology (ISGYP) and by the World Health Organization. VIN is a heterogeneous pathological entity with a usual type (warty, basaloid and mixed) and a differentiated type. The incidence of the disease is increasing, especially in young women. The high-risk human papilomavirus (HR-HPV) infection, human immunodeficiency virus (HIV) infection, smoking, cervical, vaginal and rectal intraepithelial neoplasia are considered to be high risk factors for development of VIN. There are no specific symptoms or vulvar macroscopic aspects of VIN. However, a clinical lesion is always present. Liberal vulvar biopsies under colposcopy guidance should be done. Patients with diagnosis of VIN harbor an increased risk for vulvar invasive cancer. Surgical excision and laser CO2 vaporization are the most popular therapeutic modalities for VIN treatment, both with high rates of recurrence. A close follow-up of the patients is advised. Topical imiquimod seems to be a promising treatment option. Probably, prophylactic vaccination against HR-HPV will be an important tool for VIN prevention.

Neoplasia intra-epitelial vulvar - diagnóstico diferencial e conduta / Vulvar intra-epithelial neoplasia - differential diagnose and management

O estudo de patologias na vulva envolve a necessidade de conhecimentos não só em Ginecologia, como também em patologia e dermatologia, o que pode trazer dificuldades para o ginecologista geral na condução do diagnóstico e, conseqüentemente, do tratamento. Assim, a vulva acaba por ser uma região esquecida. Há necessidade de diretrizes que facilitem a avaliação da área vulvar de forma acessível, porém não esquecendo da necessidade de uma abordagem multidisciplinar. Os autores fazem uma revisão da literatura e propõem um fluxograma com o qual o examinador possa direcionar sua conduta no estudo das patologias da vulva, englobando as de origem ginecológica e não ginecológica, com especial ênfase nas neoplasias intra-epiteliais vulvares.

Cáncer de vulva: reporte de un caso clínico y revisión bibliográfica / Vulva cancer: clinic case report and bibliographic revision

sSe reporta un caso de cáncer de vulva, en una paciente postmenopáusica de 50 años, multigesta, que procede de la zona rural, de escasos recursos económicos, se realiza biopsia para su diagnóstico; el tratamiento que se realizó fue vulvectomía radical más linfadenectomía.

Carcinoma de vulva: experiencia en el Hospital Oncológico "Padre Machado" / Carcinoma of the vulva: experience in the Hospital Oncologico \"Padre Machado\"

En el presente estudio retrospectivo se evalúan los resultados del tratamiento de 65 pacientes con diagnóstico de Carcinoma de Vulva, vistas en el Servicio de Ginecología del Hospital Oncológico "Padre Machado", entre 1987 y 1992. El Cáncer de Vulva representa el 2,15 por ciento de la patología ginecológica maligna atendida en nuestro servicio. El promedio de edad para las pacientes con carcinoma in situ fue de 47,44 años y, para las pacientes con carcinoma epidermoide invasor fue de 52,05 años. La distribución por estadios fue: Estadio 0:17 pacientes (26,15 por ciento), Estadio I: 3 pacientes (1,54 por ciento, Estadio II: 14 pacientes (21,54 por ciento), Estadio III: 16 pacientes (24,62 por ciento), Estadio IV 15 pacientes (23,08 por ciento), para el carcinoma in situ el tratamiento fue quirúrgico conservador y laserterapia, no se evidenciaron recurrencias locorregionales. En el carcinoma invasor se realizó tratamiento quirúrgico inicial en 24 pacientes (50,00 por ciento), tratamiento con radioterapia y quimioterapia según el Esquema de Nigro en 8 pacientes (16,67 por ciento), radioterapia externa en 7 pacientes (14,58 por ciento, y no recibieron tratamiento 10 pacientes (20,83 por ciento). El tratamiento quirúrgico inicial de acuerdo a la localización y el tamaño de la lesión primaria. Se realizó vulvectomía radical en 15 pacientes (31,25 por ciento). Las complicaciones postoperatorias ocurrieron en el 31,43 por ciento y en la mayor parte de los casos correspondió a la dehiscencia de la herida. La tasa de recurrencia locorregionales fue del 10,64 por ciento

Melanoma de la vulva: informe de 12 casos / Melanoma of the vulva: report of 12 cases

El melanoma de la vulva es un padecimiento poco frecuente, se acompaña de un diagnóstico tardío con lesiones de espesor mayor a dos mm, frecuentemente con metástasis ganglionares y distantes. Se hace el análisis de 12 casos atendidos en el Servicio de Oncología del Hospital Central Militar, dos del servicio privado de uno de los autores. Se describen las características clínica, histopatlógicas y de espesor. Existen cuatro sobrevivientes a más de dos años, uno de ellos tiene seis años de sobrevida. Se establecen los factores a considerar para el tratamiento quirúrgico del tumor primario y se considera la indicación de la disección inguinal terapéutica y electiva, se plantean nuevos métodos de detección de matástasis ganglionares con isótopos o colorantes y se refieren los resultado del manejo médico del melanoma