The expression of corticotropin-releasing hormone family peptides in premalignant and malignant vulvar lesions

Objectives To examine the relation of corticotropin-releasing hormone (CRH) family peptides with inflammatory processes and oncogenesis, emphasizing in vulvar inflammatory, premalignant and malignant lesions, as well as to investigate the possibility of lesion cells immunoescaping, utilizing FAS/FAS-L complex. Methods Immunohistochemical expression of CRH, urocortin (UCN), FasL and their receptors CRHR1, CRHR2 and Fas was studied in vulvar tissue sections obtained from patients with histologically confirmed diagnosis of lichen, vulvar intraepithelial neoplasia (VIN) and vulvar squamous cell carcinoma (VSCC). The patient cohort was selected from a tertiary teaching Hospital in Greece, between 2005 and 2015. For each of the disease categories, immunohistochemical staining was evaluated and the results were statistically compared. Results A progressive increase of the cytoplasmic immunohistochemical expression of CRH and UCN, from precancerous lesions to VSCC was observed. A similar increase was detected for Fas and FasL expression. Nuclear localization of UCN was demonstrated in both premalignant and VSCC lesions, with staining being significantly intensified in carcinomas, particularly in the less differentiated tumor areas or in the areas at invasive tumor front. Conclusions Stress response system and CRH family peptides seem to have a role in inflammation maintenance and progression of vulvar premalignant lesions to malignancy. It seems that stress peptides may locally modulate the stroma through Fas/FasL upregulation, possibly contributing to vulvar cancer development (AU)

Proximal epithelioid sarcoma of the vulva. A rare neoplasm. A clinicopathological case / Sarcoma epitelioide proximal de la vulva. Una neoplasia rara. Un caso clínico-patológico

Epithelioid sarcoma is a rare aggressive soft tissue sarcoma, which can be distal or proximal types. The classic form (distal-type) of epithelioid sarcoma mainly occurs in teenagers and young adults. A rarer form, called large-cell (proximal-type) epithelioid sarcoma, tends to be more aggressive and mainly affects adults. The proximal subtype mostly arises from the proximal pelvis, limbs, and genital tract. We report a case of a 59 -year-old female, presented with a progressively growing mass in the left labia majora. Gynecologic examination revealed a 2 cm mobile and painless mass that was not attached to deep planes. The histological study showed a multinodular tumor was seen comprising sheets of oval to polygonal cells with moderate amount of cytoplasm. Interspersed were larger, rhabdoid cells with abundant eosinophilic cytoplasm and prominent nucleoli. On IHC, the tumor cells showed positivity for EMA and CKAE1/AE3 and do not expressed INI-1 in the nucleus. All tumor cells were negative for S-100 protein and CD34. The histopathological diagnosis was soft tissue of the vulvar region with proximal epithelioid sarcoma. The patient received adjuvant external pelvic radiotherapy and brachytherapy in the vulvar bed. Currently, 3 years after diagnosis, the patient does not present signs of tumor recurrence in her controls. Due to its low incidence, there are no evidence-based diagnostic algorithms or published recommendations for treatment. The prognosis is generally poor. A wide excision with clear margins is imperative with options of post-operative CT/RT in individual cases during a close follow-upbehavior, as seen in our case. (AU)

El sarcoma epitelioide es un sarcoma de tejido blando agresivo poco frecuente, que puede ser de tipo distal o proximal. La forma clásica (tipo distal) de sarcoma epitelioide se presenta principalmente en adolescentes y adultos jóvenes. Una forma más rara, llamada sarcoma epitelioide de células grandes (tipo proximal), tiende a ser más agresiva y afecta principalmente a adultos. El subtipo proximal surge principalmente de la pelvis proximal, las extremidades y el tracto genital. Presentamos el caso de una mujer de 59 años, que presentó una masa de crecimiento progresivo en labios mayores izquierdos. El examen ginecológico reveló una masa móvil e indolora de 2 cm que no estaba adherida a planos profundos. El estudio histológico mostró un tumor multinodular compuesto por láminas de células de forma ovalada a poligonal con moderada cantidad de citoplasma. Intercaladas había células rabdoides más grandes con abundante citoplasma eosinófilo y nucléolos prominentes. En IHC, las células tumorales mostraron positividad para EMA y CKAE1/AE3 y no expresaron INI-1 en el núcleo. Todas las células tumorales fueron negativas para la proteína S-100 y CD34. El diagnóstico histopatológico fue tejido blando de la región vulvar con sarcoma epitelioide proximal. La paciente recibió radioterapia pélvica externa adyuvante y braquiterapia en el lecho vulvar. Actualmente, a 3 años del diagnóstico, la paciente no presenta signos de recurrencia tumoral en sus controles. Debido a su baja incidencia, no existen algoritmos de diagnóstico basados en evidencia ni recomendaciones de tratamiento publicadas. El pronóstico es generalmente malo. Es imperativa una escisión amplia con márgenes claros con opciones de CT/RT postoperatoria en casos individuales durante un seguimiento cercano, como se observa en nuestro caso. (AU)

Enfermedad de Paget extramamario primario de vulva concurrente con Lesión intraepitelial escamosa vulvar: reporte de caso / Concurrence of primary extramammary Paget's disease of the vulva and vulval intra-epithelial neoplasia: A case report

La enfermedad de Paget extramamario y la neoplasia intraepitelial vulvar son lesiones comunes de la vulva. No obstante, su ocurrencia sincrónica es una manifestación sumamente rara en la misma localización. Presentamos el caso de una paciente de 77años, quien hace 16meses aqueja de prurito y sarpullido en la región vulvar con sangrado escaso que empezó a incrementarse en frecuencia y en cantidad. Es sometida a hemivulvectomía derecha y vulvectomía simple izquierda. El informe de patología reportó la presencia concurrente de enfermedad de Paget vulvar y neoplasia intraepitelial vulvar de alto grado.(AU)

Extramammary Paget's disease and intraepithelial vulvar neoplasia are common lesions in the vulva. However, their simultaneous occurrence is extremely rare. We present the case of a 77year-old woman who presented with a 16month history of pruritus and a rash in the vulvar region with gradually increasing bleeding. She underwent a right hemivulvectomy and a left simple vulvectomy. The histopathology revealed a coexistence of both Paget's disease and high grade intraepithelial vulvar neoplasia.(AU)

A rare case of metachronous ectopic breast cancer of the vulva / Un caso raro de cáncer de mama ectópico metacrónico de la vulva

Ectopic breast tissue can arise at any point along the primitive milk line, which runs from the axilla to the groin. The incidence of ectopic breast cancer ranges from 0.2% to 0.6%. We report the case of a 60-year-old woman with a history of left mastectomy for breast cancer one year prior to presentation, who presented with bilateral vulvar erosion found to be a moderately differentiated adenocarcinoma on biopsy. The extension workup being negative, a radical vulvectomy was performed. The postoperative course was uneventful, and the specimen's pathologic study revealed a vulvar localization of non-specific invasive breast carcinoma. It was positive for estrogen receptor and human epidermal growth factor receptor 2/neu, and negative for progesterone receptor. Adjuvant chemoradiotherapy was given to the patient. There is no dedicated guideline for both diagnosis and management of this particular tumor; the treatment is similar to that of orthotopic breast cancer. (AU)

El tejido de mama ectópico puede presentarse en cualquier punto de la línea de la leche primitiva, que discurre desde la axila a la ingle. La incidencia de cáncer de mama ectópico oscila entre el 0,2 y el 0,6%. Reportamos el caso de una mujer de 60 años con historia de mastectomía izquierda por cáncer de mama un año antes de la presentación, que acudió con erosión bilateral en la vulva, que fue considerada un adenocarcinoma moderadamente diferenciado en la biopsia. Siendo negativas las pruebas complementarias, se realizó una vulvectomía radical. El curso postoperatorio transcurrió sin incidentes, y el estudio de la muestra patológica reveló la localización en la vulva de un cáncer de mama invasivo no específico, que fue positivo para receptor de estrógenos y receptor del factor de crecimiento epidérmico humano 2/neu, y negativo para el receptor de progesterona. Se administró quimioterapia adyuvante a la paciente. No existen directrices especializadas para el diagnóstico y manejo de este tumor particular, siendo su tratamiento similar al del cáncer de mama ortotópico. (AU)

Radiation therapy for vulvar cancer: consensus guidelines of the GINECOR working group of the Spanish Society of Radiation Oncology. Part 1: clinical recommendations

Purpose The present consensus statement was developed by the GINECOR working group on behalf of the Spanish Society of Radiation Oncology (SEOR). Given the lack of prospective data on the management of vulvar carcinoma, this document provides an up-to-date review of radiotherapy treatment in vulvar cancer and a series of consensus-based recommendations from a group of experts. Methods A two-round, online modified Delphi study was conducted to reach consensus treatment recommendations in three clinical settings: 1) adjuvant treatment, 2) locally-advanced vulvar cancer (LAVC), and 3) recurrent disease. After the first round, we comprehensively reviewed the available medical literature from peer-reviewed journals to assess and define the evidence-based treatment options. In the second round, participants were asked to indicate their level of agreement with the preliminary recommendations according to the GRADE (Grade of Recommendation, Assessment, Development, and Evaluation) criteria, as follows: strongly agree; agree; neither agree nor disagree; disagree; strongly disagree. Results The main recommendations were as follows: 1) following surgical resection, adjuvant radiotherapy is recommended with the presence of adverse risk factors (primarily positive margins and lymph node involvement); 2) radiotherapy (with or without chemotherapy) should be considered in LAVC; and 3) in recurrent disease, radiotherapy should be individualised on a case-by-case basis. A high level of agreement over 80% was reached. Conclusions In the absence of robust clinical data, these final recommendations may help to select the optimal radiotherapy approach for this relatively rare cancer (AU)

Radiation therapy for vulvar cancer: consensus technical guidelines of the GINECOR working group of the Spanish Society of Radiation Oncology. Part 2: radiotherapy recommendations

Purpose The present consensus statement was developed by the GINECOR working group on behalf of the Spanish Society of Radiation Oncology (SEOR). This document provides an up-to-date review of the technical aspects in radiation treatment of vulvar cancer. Methods A two-round modified Delphi study was conducted to reach consensus on the appropriateness of technical aspects of external beam radiotherapy and brachytherapy. Three clinical scenarios were proposed: adjuvant treatment of vulvar cancer, radiation treatment of locally advanced vulvar carcinoma and locoregional recurrences. After the first round, an extensive analysis of current medical literature from peer-reviewed journal was performed to define evidence-based treatment options. In the second round, participants were asked to indicate their level of agreement with the preliminary recommendations according to the GRADE (Grade of Recommendation, Assessment, Development, and Evaluation) criteria, as follows: strongly agree; agree; neither agree nor disagree; disagree and strongly disagree. Results The main recommendations on external beam radiotherapy and brachytherapy, both in adjuvant setting and local advanced disease are summarized. Recommendations include treatment technique, treatment volume, and doses in target and organs at-risk. Taking into consideration the different clinical scenarios of recurrent disease, the radiation treatment should be individualized. Conclusions In the absence of robust clinical data, these recommendations may help to select the optimal radiotherapy approach for this relatively rare cancer (AU)

Tumor vulvar similar a lipoblastoma: descripción de un nuevo caso y revisión de la literatura / Lipoblastoma-like tumor of the vulva: A case report and review of the literature

El tumor vulvar similar a lipoblastoma (LBLTV) fue descrito inicialmente como una neoplasia mesenquimal benigna. Desde entonces, se han reportado únicamente 19 casos. Además, esta entidad no ha sido reconocida aún como diagnóstico separado en la clasificación de la OMS (2013) de los tumores de tejido blando. El diagnóstico diferencial de LBLTV incluye otros tumores de la región vulvoperineal, así como tumores con diferenciación adipocítica, la mayoría de ellos benignos. Por tanto, un diagnóstico erróneo aporta pocas consecuencias clínicas. Sin embargo, LBLTV puede imitar también algunas neoplasias lipomatosas agresivas. Describimos aquí un nuevo caso de LBLTV en una mujer de 28 años, así como una revisión de la literatura.(AU)

Lipoblastoma-like tumor of the vulva (LBLTV) was first described as a benign mesenchymal neoplasia; it was not recognized as a separate diagnosis in the 2013 WHO classification of soft-tissue tumors. To date, only 19 cases have been reported. LBLTV differential diagnosis includes other tumors of the vulvoperineal region and tumors with adipocytic differentiation, most of which are benign and thus a misdiagnosis has few clinical consequences. However, LBLTV may also mimic some aggressive lipomatous neoplasms. We describe a case of LBLTV in a 28 year-old woman and review the literature.(AU)

The role of brachytherapy (interventional radiotherapy) for primary and/or recurrent vulvar cancer: a Gemelli Vul. Can multidisciplinary team systematic review

Objective The aim of our systematic review was to assess the role of interventional radiotherapy (IRT, brachytherapy) in the management of primary and/or recurrent vulvar carcinoma. Evidence acquisition A systematic research using PubMed, Scopus and Cochrane library was performed. ClinicalTrials.gov was searched for ongoing or recently completed trials, and PROSPERO was searched for ongoing or recently completed systematic reviews. Only full-text English-language articles related to IRT for treatment of primary or recurrent VC were identified and reviewed. Conference paper, survey, letter, editorial, book chapter and review were excluded. Time restriction (1990–2018) as concerns the years of the publication was considered. Evidence synthesis Primary disease: the median 5-year LC was 43.5% (range 19–68%); the median 5-year DFS was 44.5% (range 44–81%); the median 5-year OS was 50.5% (range 27–85%). Recurrent disease: the median 5-year DFS was 64% (range 56–72%) and the median 5-year OS was 45% (range 33%-57%). Acute ≥ grade 2 toxicity was reported in three patients (1.6%). The severe late toxicity rates (grade 3–4) ranged from 0% to 14.3% (median 7.7%). Conclusion IRT as part of primary treatment for primary and/or recurrent vulvar cancer is associated with promising clinical outcomes (AU)

Biopsia selectiva de ganglio centinela en el carcinoma escamoso de vulva. Análisis de seguimiento a 10 años / Sentinel lymph node biopsy procedure in squamous vulvar cancer. 10 years follow-up analysis

OBJETIVO: Aplicación de la biopsia selectiva de ganglio centinela (BSGC) en el carcinoma de vulva en estadios iniciales y análisis de los resultados, recurrencias y complicaciones. MATERIAL Y MÉTODOS: Se revisaron retrospectivamente 40 pacientes con cáncer de vulva y BSGC entre 2008 y 2018. El día de la intervención se rastrearon las cadenas ganglionares inguinales mediante sonda gammadetectora para identificar los ganglios centinela que se extirparon y remitieron para estudio anatomopatológico intraoperatorio. Posteriormente, se realizó seguimiento a largo plazo con análisis de complicaciones, recaída y mortalidad. RESULTADOS: De las 40 pacientes (edad media: 72 años [47-86]), la tasa de detección global por paciente fue del 95%, con un total de 129 ganglios centinela (GC) (3,22 GC/paciente). En tres de 25 pacientes con lesiones tumorales laterales el drenaje fue bilateral y en dos de 15 con lesiones de línea media fue unilateral. De las 40 linfogammagrafías 16 presentaban drenaje bilateral y 24 unilateral. Se obtuvieron un total de 119 GC- y 10 GC+, realizándose vaciamiento en ocho. En el grupo de GC- se incluyeron un caso de bloqueo linfático y un falso negativo. En 12 de 40 pacientes hubo complicaciones posquirúrgicas, cuatro de ellas linfedemas. Durante una mediana de seguimiento de 40 meses, recayeron seis de 10 con GC+ (40% mortalidad) y siete de 30 GC- (16% mortalidad). CONCLUSIONES: La BSGC en cáncer de vulva es la técnica de elección para una correcta estadificación y tratamiento locorregional. Es importante una adecuada estadificación ganglionar previa a la cirugía para evitar posibles bloqueos linfáticos que puedan inducir falsos negativos

AIM: Application of sentinel lymph node biopsy (SLNB) procedure in early-stage vulvar cancer and analysis of results, recurrences and complications. MATERIAL AND METHODS: 40 patients with vulvar cancer and SLNB between 2008 and 2018 were retrospectively reviewed. During the surgical procedure the inguinofemoral lymph nodes were checked with a gamma probe to identify the sentinel nodes that were removed and referred for intraoperative pathological assessment. Subsequently, long-term patient follow-up was performed with analysis of complications, relapse and mortality. RESULTS: 40 patients (mean age: 72 years [47-86], the overall detection rate per patient was 95% and a total of 129 Sentinel Lymph Nodes (SLNs) were removed (3.22 SLN/patient). In 3 out of 25 patients with lateral tumour lesions drainage was bilateral and in 2 out of 15 with midline lesions drainage was unilateral. On lymphoscintigraphy, 16 out of 40 had bilateral drainage and 24 unilateral. A total of 119 SLN- and 10 SLN+ were obtained, in 8 out of 10 an inguinofemoral lymphadenectomy was performed. In the SLN- group, one case of lymphatic blockage and one false negative were included. In 12 out of 40 patients there were post-surgical complications, 4 of them lymphoedemas. In the median follow-up (40 months), 6 out of 10 with SLN+ (40% mortality) and 7 out of 30 SLN- (16% mortality) had recurrences. CONCLUSIONS: SLNB in vulvar cancer is the technique of choice for correct staging and locoregional therapy. Correct clinical lymph node staging is important before surgery in order to avoid potential blockage drainages which could induce a false negative SLN

Enfermedad de Paget extramamaria / Extramammary Paget Disease

ANTECEDENTES Y OBJETIVOS: Existen pocos estudios sobre la enfermedad de Paget extramamaria (EPEM) en la población mediterránea. Nuestro objetivo fue revisar las características de nuestros pacientes con EPEM, su asociación con neoplasia en continuidad y su evolución a largo plazo. PACIENTES Y MÉTODOS: Realizamos un estudio observacional retrospectivo sobre 27 pacientes diagnosticados de EPEM entre 1990-2015. Las historias clínicas fueron revisadas retrospectivamente para obtener los datos clínico-patológicos y de seguimiento. RESULTADOS: Se trata de 20 mujeres y 7 varones de entre 42 y 88 años de edad (mediana de 76 años). Las lesiones se localizaron en la vulva (16 casos), en el pubis-región inguinal (5), en la región perianal (4) y en la axila (2). El tiempo de evolución al diagnóstico osciló entre 1 y 60 meses (mediana de 12 meses) y el diámetro máximo entre 20 y 140 mm (mediana de 55 mm). En 3 casos (11,1%) la EPEM fue secundaria. Ningún caso se desarrolló sobre adenocarcinoma anexial cutáneo previo. Diez de 24 EPEM primarias (41,7%) presentaban invasión de la dermis. Ocho de los 27 pacientes (29,6%) presentaron recidiva local tras el tratamiento quirúrgico inicial. Tres pacientes (11,1%) fallecieron a consecuencia de metástasis de la EPEM. CONCLUSIONES: La presencia de un adenocarcinoma anexial cutáneo subyacente es poco frecuente pero no es rara la existencia de un adenocarcinoma extracutáneo en continuidad. A pesar de que la EPEM suele evolucionar lentamente, es frecuente la invasión de la dermis y no son excepcionales las metástasis. Las recidivas locales son frecuentes a pesar de la extirpación con márgenes amplios y pueden ser tardías, por lo que es preciso un seguimiento a largo plazo

BACKGROUND AND OBJECTIVE: Extramammary Paget disease (EMPD) has seldom been studied in Mediterranean populations. We aimed to review the characteristics of our patients with EMPD, the presence of a neoplasm in continuity, and the long-term course of the disease. PATIENTS AND METHODS: Retrospective observational study of 27 patients diagnosed with EMPD between 1990 and 2015. All clinical and pathology findings related to clinical course and outcomes were retrieved for analysis. RESULTS: Twenty patients were women and 7 were men. Ages ranged from 42 to 88 years (median, 76 years). Lesions were in the following locations: vulva (16 cases), pubis-groin (5), perianal region (4), and axilla (2). Time from onset to diagnosis ranged from 1 to 60 months (median, 12 months) and maximum lesion diameter from 20 to 140 mm (median, 55 mm). In 3 cases (11.1%) EMPD was a secondary condition. None of the lesions developed on a previous cutaneous adnexal adenocarcinoma. Ten of the 24 primary EMPDs (41.7%) invaded the dermis. Eight of the 27 patients (29.6%) experienced local recurrence after the initial surgical treatment.Three patients (11.1%) died as a consequence of metastasis from the EMPD. CONCLUSIONS: The presence of an underlying cutaneous adnexal adenocarcinoma is uncommon, but it is not unusual to find an extracutaneous adenocarcinoma in continuity. Although EMPD is a slow-growing tumor, dermal invasion is frequent and metastasis is not uncommon. Local recurrence is common even after excision with wide margins and may be delated, so long term follow-up is essential

Resultados de la implantación de un protocolo para reconstrucción vulvar inmediata tras vulvectomía / Outcomes of the implementation of a protocol for immediate vulvar reconstruction after vulvectomy

Introducción y objetivo. La cirugía de vulvectomía se asocia a una gran incidencia de complicaciones de la herida quirúrgica que pueden evitarse mediante la reconstrucción inmediata de la vulva. En este trabajo buscamos presentar los resultados obtenidos en nuestro centro hospitalario mediante la introducción de un protocolo de reconstrucción inmediata tras vulvectomía. Material y método. En enero de 2017 se implantó en nuestro hospital un protocolo de reconstrucción inmediata tras vulvectomía con el fin de establecer una serie de criterios que permitan al ginecólogo detectar aquellas pacientes con alto riesgo de dehiscencia de la herida tras vulvectomía y contactar con el Servicio de Cirugía Plástica para coordinar la reconstrucción inmediata de la vulva. Durante un periodo de 18 meses registramos los datos de las pacientes sometidas a dicha intervención: edad, tipo de neoplasia, criterio reconstructivo, técnica reconstructiva, uni o bilateralidad, estancia hospitalaria y complicaciones en los 30 primeros días de postoperatorio. Resultados. Registramos un total de 9 pacientes sometidas a reconstrucción inmediata: 8 mediante colgajo en flor de loto y 1 mediante colgajo miocutáneo de gracilis. La estancia media hospitalaria fue de 31.4 días y 3 pacientes presentaron complicaciones postoperatorias en forma de dehiscencia de la herida quirúrgica. Conclusiones. Consideramos que nuestro protocolo es una herramienta adecuada para desarrollar una colaboración interdepartamental entre Ginecología y Cirugía Plástica y para implantar la reconstrucción vulvar inmediata

Background and objective. Vulvectomy surgery is associated with a high incidence of surgical wound complications that may be avoided with immediate vulvar reconstruction. In this paper we present the results obtained in our hospital with the implementation of an immediate vulvar reconstruction protocol. Methods. In January 2017 we established an immediate vulvar reconstruction protocol in our center with a list of criteria that would allow the gynecologist to detect those patients with a high risk of wound dehiscence after vulvectomy in order to coordinate the immediate reconstruction of the vulva with our Plastic Surgery Service. For the next 18 months, we recorded the information of those patients intervened: age, pathology, reconstructive criteria, reconstructive technique, laterality, hospital stay and complications during the first 30 postoperative days. Results. We performed immediate vulvar reconstruction in 9 patients: 8 with a lotus flap and 1 with a gracilis myocutaneous flap. Mean stay was 31.4 days and 3 patients developed complications in the form of surgical wound dehiscence. Conclusions. We consider that our protocol is a useful tool for the implantation of immediate vulvar reconstruction and to establish a collaboration between gynecologists and plastic surgeons

Angioleiomiosarcoma de clítoris. Presentación de caso / Clitoral angio-leiomyosarcoma: Case report

El carcinoma vulvar es una entidad poco frecuente; representa el 3-5% de los tumores genitales malignos en la mujer. El 90% de ellos corresponden a carcinomas epidermoides, seguidos de los melanomas, enfermedad de Paget, enfermedad de Bowen, carcinomas basales y sarcomas. El leiomiosarcoma es la forma de presentación más frecuente de este último grupo. Se presenta un caso de una mujer adulta joven, 29 años, mestiza, que acude con una tumoración a nivel del clítoris, con el antecedente de exéresis de una tumoración benigna a nivel del labio mayor 3años antes. La lesión se mostraba móvil, ulcerada, muy dolorosa y sin adenopatías locales ni regionales. Se procede a la escisión total de la lesión y el estudio histológico informó un angioleiomiosarcoma de clítoris. Se envía el caso a Instituto de Oncología para confirmación diagnóstica y terapéutica, confirmándose el diagnóstico y la terapéutica. En 2años la paciente no ha tenido recidiva tumoral

Vulvar carcinoma is rare, and represents 3-5% of the all gynaecological cancers. Epidermoid carcinoma accounts for 90% of them, followed by melanoma, Paget́s disease, Boweńs disease illness, basal carcinoma and sarcomas. The leiomyosarcoma is the most frequent type of sarcoma. The case is presented of a 29 year-old woman that suffered from clitoral tumour. She was treated for a benign lesion in the labia majora 3years ago. In the current referral, she presented with pain and an ulcerated lesion, without local or regional adenopathies. The total excision was referred for a histological study, and the results showed an angio-leiomyosarcoma of the clitoris. Two years later, the patient showed no signs of residual lesion

Tumoración genital interlabial en recién nacida / Genital interlabial mass in newborn girl

El himen imperforado es una rara malformación congénita que ocurre en el 0,1% de los recién nacidos; sin embargo, es la anomalía obstructiva más frecuente del aparato genital femenino. Suele presentarse de forma esporádica y aislada, pero en ocasiones se puede presentar asociado a otras malformaciones, principalmente genitourinarias. Hay dos picos de máxima incidencia: en el periodo neonatal y en la adolescencia. Debido a las múltiples complicaciones que puede presentar, es importante un diagnóstico precoz, preferentemente en la etapa neonatal. El tratamiento es siempre quirúrgico. Presentamos el caso de una recién nacida que en la exploración en las primeras horas de vida presenta una tumoración genital interlabial

The imperforate hymen is a rare congenital malformation that occurs in 0.1% of newborns; however, is the most frequent obstructive anomaly of the female genital tract. It usually occurs sporadically and isolated, but sometimes it can occur associated with other malformations, mainly genitourinary. There are two peaks of maximum incidence: neonatal and in adolescence. Due to the multiple complications that can present, an early diagnosis is important, preferably in the neonatal stage. The treatment is always surgical. We present the case of a newborn girl with a genital interlabial mass detected in the first hours of life

Adenocarcinoma primario de vulva tipo glándula mamaria. Forma histológica muy infrecuente de neoplasia vulvar / Primary adenocarcinoma of mammary-like glands of the vulva. Very rare histological form of vulvar neoplasia

El cáncer de vulva es una entidad relativamente rara que representa menos del 1% de todos los tumores malignos de la mujer y cuya incidencia aumenta con la edad. Dentro de los adenocarcinomas primarios de vulva, que por definición son tumores poco comunes, existe una forma aún más infrecuente que parece tener su origen en las glándulas mamarias, denominadas «mammary-like», las cuales presentan características de glándulas ecrinas y glándulas mamarias. El síntoma de presentación más frecuente es el prurito vulvar, seguido de la aparición de tumoraciones y ulceraciones en los genitales externos. En cuanto al diagnóstico de esta dolencia, basado principalmente en el examen e inspección vulvar, se recomienda como método definitivo de diagnóstico la biopsia escisional de la lesión. Entre las pruebas de imagen más útiles para el manejo del cáncer de vulva se encuentra la tomografía por emisión de positrones/tomografía computada (PET/TC), especialmente para identificar metástasis ganglionares, lo que permite establecer la técnica quirúrgica más idónea, el manejo clínico y la asociación con otras terapias. El tratamiento del carcinoma invasivo de vulva es la vulvectomía radical asociada a linfadenectomía inguinofemoral bilateral. La extensión por continuidad de la dolencia se suele producir por vía linfática: es una rareza por vía hematógena. Presentamos el caso clínico de una paciente con una lesión vulvar anómala, en la que, tras biopsia y estudio anatomopatológico, se confirmó el diagnóstico de un tipo histológico infrecuente de adenocarcinoma de vulva, del tipo glándula mamaria

Vulvar cancer is a relatively rare disease that accounts for less than 1% of all malignant tumours in women, whose incidence increases with age. Among the primary adenocarcinomas of the vulva, which by definition are rare tumours, there is an even rarer form that appears to originate in the mammary glands, known as «mammary-like», which present features of both eccrine glands and mammary glands. The most common presenting symptom is vulvar pruritus, followed by the appearance of lumps and ulcerations in the external genitals. This disease is principally diagnosed by vulvar examination and inspection, and an excisional biopsy of the lesion is recommended as the definitive method of diagnosis. Positron emission tomography (PET/CT) is one of the most useful imaging tests for the management of vulvar cancer, especially for the identification of lymph node metastases. This enables the most appropriate surgical technique to be selected and facilitates clinical management and association with other therapies. Invasive vulvar carcinoma is treated by radical vulvectomy associated with bilateral inguinofemoral lymphadenectomy. Lymphatic spread is typical for this disease, while haematogenous spread is very rare. We present the clinical case of a patient with an abnormal vulvar lesion, in which, after biopsy and anatomical and pathological study, the diagnosis of a rare histological type of adenocarcinoma of mammary-like glands of the vulva was confirmed

Lesiones seudoverrugosas vulvares de reciente aparición / Pseudoverrucous Lesions of Recent Appearance on the Vulva

No disponible

Evidencia empírica de la miel como tratamiento eficaz. En la dehiscecia de sutura tras vulvectomía radical / Empirical evidence of honey as an effective treatment for wound dehiscence after a radical vulvectomy

El cáncer de vulva es uno de los tumores ginecológicos menos frecuentes. La mutilación producida por cirugías radicales, como la vulvectomía, pueden ocasionar importantes secuelas físicas, psíquicas y sexuales en la mujer. El caso clínico que presentamos es el de una mujer de 83 años diagnosticada de cáncer de vulva, a la que se intervino en nuestro centro por recidiva local y que presentó dehiscencia de la cicatriz pocos días después de la cirugía. Nuestro objetivo es mostrar que la cura con miel es una alternativa eficaz, por sus propiedades curativas, en la cura de dehiscencias en las vulvectomías, además de ser una terapia barata, accesible y fácil de aplicar (AU)

Vulvar cancer is one of the less common gynecological tumors. Nevertheless, the mutilation produced by a radical surgery such as the vulvectomy can cause important sequels in women, both physical and psychological. We present a clinical case of an 83-year-old woman diagnosed with vulvar cancer, who underwent a radical vulvectomy after local recurrence at our hospital. She presented a wound dehiscence a few days after the surgery. Our aim is to discuss that honey is an effective alternative cure of wound dehiscence in vulvectomy due to its healing properties; it is also a cheaper treatment, accessible, and easy to apply (AU)

Tumor del seno endodérmico extragonadal de localización vulvar: descripción de un caso y revisión de la literatura / Extragonadal yolk sac tumour of the vulva. A case report and a review of the literature

La localización extragonadal del tumor del seno endodérmico es excepcional y puede presentar niveles normales de alfafetoproteína sérica (AFP). Describimos el caso de una mujer de 34 años con una tumoración vulvar con diagnóstico inicial en otro centro de carcinoma indiferenciado de vulva. Una nueva valoración histológica e inmunohistoquímica y una posterior determinación de AFP en suero permitieron realizar el diagnóstico. El tumor del seno endodérmico en la región vulvar es excepcional. Solamente se han descritos 16 casos en la literatura. No obstante, es posible realizar un diagnóstico específico y, por lo tanto, un tratamiento acorde con este tipo de neoplasia (AU)

An extragonadal location of a yolk sac tumour is rare and may display normal levels of serum alpha-fetoprotein. We report a case of a 34-year-old woman with a vulvovaginal tumour initially thought to be an undifferentiated carcinoma of the vulva. However, a new histological and immunohistochemical assessment and subsequent determination of serum AFP confirmed the diagnosis of yolk sac tumour. Vulvar yolk sac tumor is rare with only 16 cases described in the literature. However, an accurate differential diagnosis should be made in order to indicate the correct treatment (AU)