A tale of two rare entities: extramammary Paget's disease and hepatocellular carcinoma-a singular case report

This case report underscores the intricate challenges in managing extramammary Paget's disease (EMPD) of the vulva, a rare neoplasm with diverse clinical manifestations. The presented case, involving a postmenopausal woman, highlights the complexity of EMPD diagnosis and its association with underlying malignancies, particularly hepatocellular carcinoma (HCC). Despite initial management with 5% Imiquimod cream, the patient's clinical course revealed the aggressive nature of the disease, as evidenced by the development of invasive HCC. The rapid deterioration and subsequent demise of the patient emphasize the need for comprehensive monitoring and early intervention in cases of EMPD, considering its potential association with internal malignancies. The discussion delves into the typical presentation of Paget's disease, its association with adnexal carcinomas, and the challenging link between EMPD and internal malignancy. The retrospective analysis of cases in the literature further underscores the variability in underlying adnexal carcinoma rates, emphasizing the complexity of this association. In this particular case, immunohistochemical markers failed to establish a direct correlation between vulval EMPD and hepatocellular carcinoma, highlighting the need for continued research and understanding of the intricate pathogenesis of EMPD and its relationship with internal malignancies.

Técnica Boomerang para plástica genital estética feminina / Boomerang technique for female genital aesthetic surgery

Introdução: A hipertrofia de pequenos lábios combinada com capuz clitoriano redundante é uma queixa comum entre mulheres que procuram cirurgia plástica genital cosmética por queixas funcionais, psicológicas e estéticas. O objetivo deste estudo foi descrever a técnica boomerang, um tratamento cirúrgico da hipertrofia labial que se estende a todo o capuz clitoriano. Método: Foi conduzido um estudo retrospectivo, de caráter analítico, para avaliar os prontuários médicos de 48 pacientes consecutivas submetidas a cirurgia estética genital feminina entre julho de 2017 e julho de 2021. Todos os procedimentos cirúrgicos foram realizados pela mesma cirurgiã. A técnica utilizada nas pacientes consistiu na ressecção longitudinal dos excessos de pequenos lábios vaginais associado à ressecção de capô clitoriano em forma de boomerang e à clitoropexia. Resultados: A idade média das pacientes submetidas a cirurgia foi de 36,25 anos (intervalo 18-59 anos), entre as quais 94,44% apresentaram queixas estéticas associadas ou não a queixas funcionais, e 5,56% apresentaram somente queixas funcionais. Duas pacientes apresentaram hematomas nos grandes lábios no pós-operatório imediato, e uma paciente teve deiscência de sutura nos pequenos lábios. Conclusão: A técnica boomerang é reprodutível e proporciona benefícios estéticos e/ou funcionais na genitália feminina.

Introduction: Hypertrophy of the labia minora combined with a redundant clitoral hood is a common complaint among women seeking aesthetic genital cosmetic surgery for functional, psychological, and aesthetic complaints. The objective of this study was to describe the boomerang technique, a surgical treatment for labial hypertrophy that extends to the entire clitoral hood. Method: A retrospective, analytical study was conducted to evaluate the medical records of forty-eight consecutive patients who underwent female genital cosmetic surgery between July 2017 and July 2021. The same surgeon performed all surgical procedures. The technique used in the patients consisted of longitudinal resection of excess small vaginal lips associated with resection of the boomerang-shaped clitoral hood associated with clitoroplasty. Results: The average age of patients undergoing surgery was 36.25 years (range 18-59 years), among whom 94.44% had aesthetic complaints associated or not with functional complaints, and 5.56% had only functional complaints. Two patients had bruises on the labia majora in the immediate postoperative period, and one patient had suture dehiscence on the labia minora. Conclusion: The boomerang technique is reproducible and provides aesthetic and/or functional benefits to the female genitalia.

Carcinoma basocelular de vulva: relato de caso / Basal cell carcinoma of the vulva: case report

Introdução: O carcinoma basocelular (CBC) de vulva é uma condição rara que corresponde a menos de 0,4% dos casos de CBC e de 2% a 4% das neoplasias de vulva. O CBC de vulva é mais comum entre mulheres brancas, multíparas e na pósmenopausa, especialmente na sétima década de vida. O objetivo é relatar um caso de CBC de vulva no qual discutiram-se os aspectos do diagnóstico e tratamento. Relato de Caso: Mulher de 63 anos de idade, G1P1A0, chega ao consultório em janeiro de 2022 para tratamento de lesão persistente em vulva. Realizou-se biópsia incisional que mostrou tratar-se de provável carcinoma basocelular nodular com invasão da derme. A paciente submeteu-se a uma ressecção do tumor com margens macroscópicas livres e sutura primária. A cirurgia não teve complicações no pré-operatório e no pós-operatório. O histopatológico da peça cirúrgica mostrou tratar-se de carcinoma basocelular nodular com área irregular, plana, branco, medindo 0,7x0,4cm, com as margens laterais distando 7,0 e 5,0mm e profundas, 5,9mm; todas livres. Conclusão: O caso relatado é raro, tendo sido o tratamento de ressecção cirúrgica do CBC de vulva com margens bem-sucedido. Catorze meses após a cirurgia, a paciente encontra-se sem evidências de recidiva local ou regional.

Introduction: Basal cell carcinoma (BCC) of the vulva is a rare condition that accounts for less than 0.4% of BCC cases and 2% to 4% of vulvar neoplasms. BCC of the vulva is more common among white, multiparous and postmenopausal women, especially in the seventh decade of life. The aim is to report a case of BCC of the vulva in which aspects of diagnosis and treatment were discussed. Case report: A 63-year-old woman, G1P1A0, arrives at the office in January 2022 for treatment of a persistent lesion on her vulva. An incisional biopsy was performed and showed that it was likely nodular basal cell carcinoma with invasion of the dermis. The patient underwent tumor resection with free macroscopic margins and primary suture. The surgery had no complications preoperatively or postoperatively. The histopathology of the surgical specimen showed that it was a nodular basal cell carcinoma with an irregular, flat, white area, measuring 0.7x0.4cm, with the lateral margins 7.0 and 5.0mm apart and 5.9mm deep; all free. Conclusion: The reported case is rare, with surgical resection of BCC of the vulva with margins being successful. Fourteen months after surgery, the patient has no evidence of local or regional recurrence.

Tumor filoide borderline gigante e recidivante: ressecção e reconstrução da mama / Recurrent giant borderline phyllodes tumor: resection and breast reconstruction

O tumor filoide é uma neoplasia fibroepitelial rara que representa 0,3 a 1% de todas as neoplasias mamárias. De acordo com a classificação histopatológica, 12 a 26% são do tipo borderline e aproximadamente 15% desses tumores recorrem após excisão cirúrgica. O tratamento recomendado para todos os tipos de tumor filoide é a excisão cirúrgica, e no caso de tumores gigantes o tratamento deve ser multidisciplinar. Apresentamos o caso de uma mulher de 46 anos com tumor filoide na mama esquerda que recorreu 4 anos após a excisão cirúrgica. O estudo anatomopatológico qualificou-o como tumor gigante e o estudo histopatológico relatou tumor filoide borderline. Foi submetida a excisão cirúrgica com mastectomia esquerda e reconstrução mamária com retalho de grande dorsal mais enxerto de gordura. A paciente apresentou evolução favorável sem recidiva. Concluindo, o tumor filoide gigante borderline recorrente é raro e seu manejo cirúrgico representa um desafio tanto na excisão quanto na reconstrução mamária.

Phyllodes tumor is a rare fibroepithelial neoplasm that represents 0.3 to 1% of all breast neoplasms. According to histopathologic classification, 12 to 26% are borderline type and approximately 15% of these tumors recur after surgical excision. The recommended treatment for all types of phyllodes tumor is surgical excision, and in the case of giant tumors the treatment should be multidisciplinary. We present the case of a 46-yearold woman with a phyllodes tumor in the left breast that recurred 4 years after surgical excision. The anatomopathological study qualified it as a giant tumor and the histopathological study reported a borderline phyllodes tumor. She underwent surgical excision with left mastectomy and breast reconstruction by means of a latissimus dorsi flap plus fat graft. The patient presented a favorable evolution without recurrence. In conclusion, the recurrent giant borderline phyllodes tumor is rare and its surgical management represents a challenge both in breast excision and reconstruction.

Ninfoplastia o Labioplastia: Técnica quirúrgica y tratamiento de sus complicaciones / Nymphoplasty or Labioplasty: Surgical Technique and Treatment of its Complications

Se describe la técnica quirúrgica denominada ninfoplastia o labioplastia. Es la reducción del tamaño de los labios menores de la vulva hipertróficos, requerida además por razones estéticas. Se realiza una reseña de sus técnicas quirúrgicas y el detalle que nosotros realizamos en la resección, con el fin de respetar la zona clitoriana. Se señalan además las complicaciones presentadas y cómo resolverlas

The surgical technique called nymphoplasty or labiaplasty is described. It is the reduction in the size of the hypertrophic labia minora of the vulva, furthermore, required for aesthetic reasons. A review is made of their surgical techniques and the detail that we carry out in the resection, in order to respect the clitoral area. The complications presented and how to resolve them are also pointed out

A vulval leiomyoma: a rare camouflaging tumor

Uterine leiomyomas are a benign tumor of human uterus and common problem in gynecology. Despite this, leiomyomas of the vulva are rare, masquerading, and usually misdiagnosed as Bartholin cyst preoperatively. These benign smooth muscle tumors are typically painless, solitary, and well circumscribed and can affect female of any age group. We hereby present a case of a 39-year-old female that presented in OPD at ESIC model hospital, Bapunagar, Ahmedabad with left labial mass and was misdiagnosed as Bartholin cyst initially and later on histopathology final diagnosis of vulvar leiomyoma was made.

Application and prospects of ctDNA detection in HPV associated cervical cancer / 实用医学杂志

Circulating tumor DNA(ctDNA)is a kind of cell-free DNA derived from tumors,which carries comprehensive tumor genetic information;Recent studies have found that ctDNA detection can play a role in the early diagnosis,targeted therapy,and prediction of recurrence in tumors.Human papillomavirus(HPV)-associated gynecological malignancies include most cervical cancer,some vulvar cancer,and vaginal cancer.High-risk HPV long-term infection and integration with cell genome are important causes of these cancers.Studies found that the use of ctDNA detection technology to dynamically monitor changes in HPV-ctDNA can provide valuable information for the clinical management and prognosis of these cancers.Thus,HPV-ctDNA is expected to become an biomarker for HPV-associated tumors.

O pH da região vulvar no ciclo vital: revisão de escopo / The pH of the vulvar region in the life cycle: scoping review / El pH de la región vulvar en el ciclo vital: revisión del alcance

Introdução: mapear os estudos que mensuraram o potencial Hidrogeniônico (pH) da região vulvar no ciclo vital da mulher. Métodos: revisão de escopo conforme recomendações do Manual for Evidence Synthesis do Joanna Briggs Institute (JBI), nas bases SCOPUS, Web of Science, Academic Search Premier, PubMed, Bielefeld Academic Search Engine e Google Acadêmico em janeiro de 2022. Foram incluídos estudos publicados em português, espanhol, francês e inglês, sem limite temporal. Resultados: dos 954 documentos recuperados, 13 foram selecionados. Apurou- se que os estudos utilizaram diferentes procedimentos para as medições quanto aos equipamentos, ambiente e preparo das participantes. A média de idade apresentada pela população feminina estudada variou entre 31 e 43 anos. Os locais de medição vulvar foram os grandes e pequenos lábios, dobra interlabial e períneo. O menor valor do pH aferido foi 4,6 e o maior 6,3. Conclusões: constatou-se baixa produção de estudos acerca do pH vulvar e ênfase das pesquisas na população de mulheres adultas. A diversidade de procedimentos e locais de aferição encontrados não permite afirmações seguras sobre uma faixa de valor de pH da superfície da pele vulvar.

Introduction: to map the studies that measured the Potential of Hydrogen (pH) of the vulvar region in women's life cycle. Methods: scoping review according to recommendations from the Joanna Briggs Institute (JBI) Manual for Evidence Synthesis in the SCOPUS, Web of Science, Academic Search Premier, PubMed, Bielefeld Academic Search Engine and Google Scholar databases in January 2022. Studies published in Portuguese, Spanish, French, and English, without time limit, were included. Results: of the 954 documents retrieved, 13 were selected. Different measurement procedures in relation to equipment, environment and preparation of participants were used in the studies. The average age of the female population ranged between 31 and 43 years. The vulvar measurement sites were the labia majora and minora, interlabial sulci and perineum. The lowest pH value measured was 4.6 and the highest was 6.3. Conclusions: there was a low production of studies on vulvar pH and an emphasis of studies on the population of adult women. The diversity of procedures and measurement sites found does not allow for safe statements about a range of pH values on the surface of the vulvar skin.

Introducción: mapear los estudios que midieron el Potencial de Hidrógeno (pH) de la región vulvar en el ciclo vital de las mujeres. Métodos: revisión del alcance según las recomendaciones del Manual for Evidence Synthesis del Joanna Briggs Institute (JBI), en las bases de datos SCOPUS, Web of Science, Academic Search Premier, PubMed, Bielefeld Academic Search Engine y Google Scholar en enero de 2022. Se incluyeron estudios publicados. en portugués, español, francés e inglés, sin límite de tiempo. Resultados: de los 954 documentos recuperados, se seleccionaron 13. En los estudios se utilizaron diferentes procedimientos de medición en relación con el equipo, el entorno y la preparación de los participantes. La edad media de la población femenina osciló entre 31 y 43 años. Los sitios de medición vulvar fueron los labios mayores y menores, el pliegue interlabial y el perineo. El valor de pH más bajo medido fue 4,6 y el más alto fue 6,3. Conclusiones: hubo una baja producción de estudios sobre pH vulvar y un énfasis de estudios en población de mujeres adultas. La diversidad de procedimientos y sitios de medición encontrados no permite realizar afirmaciones seguras sobre un rango de valores de pH en la superficie de la piel vulvar.

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

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

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

Pólipo fibroepitelial vulvar: una serie de casos

Los pólipos fibroepiteliales son tumores cutáneos benignos frecuentes en la población general. Sin embargo, la afectación del tracto genital es inhabitual. Su etiología no es clara, pero se han descrito asociaciones con trastornos metabólicos y fluctuaciones hormonales, lo cual explica su mayor prevalencia en mujeres. Debido a la variedad de diagnósticos diferenciales, es necesaria la evaluación histopatológica. Su manejo es habitualmente conservador; sin embargo, pueden requerir intervención quirúrgica en algunos casos. Exponemos cuatro casos de tumores fibroepiteliales vulvares de diferentes tamaños, uno de ellos clasificado como gigante, así como la aproximación de manejo. Con esta presentación, esperamos mejorar el conocimiento, la precisión del diagnóstico y contribuir al tratamiento eficaz de las pacientes con esta patología vulvar tan poco frecuente.

Fibroepithelial polyps are common benign skin tumors in the general population. However, genital tract involvement is unusual. Their etiology is unclear, but associations with metabolic disorders and hormonal fluctuations have been described, which explains their higher prevalence in women. Due to the variety of differential diagnoses, histopathological evaluation is necessary. Their management is usually conservative. However, they may require surgical intervention in some cases. We present four cases of vulvar fibroepithelial tumors of different sizes, one of them classified as giant, as well as the management approach. With this presentation, we hope to improve knowledge, diagnostic accuracy and contribute to the effective treatment of patients with this rare vulvar pathology.

Metastatic Crohn's disease: a difficult diagnosis from different perspectives

Metastatic Crohn's disease (MCD) is a rare cutaneous manifestation of Crohn's disease (CD). The simultaneous involvement of the vulva and oral region is uncommon in clinical presentations of MCD. We present the case of a middle-aged woman with a family history of autoimmunity who initially presented with oral and vulvoperineal involvement. Initially, Behçet's disease was ruled out, but histopathological studies of the vulva revealed findings compatible with MCD. The patient had no gastrointestinal symptoms, and fecal calprotectin levels were normal. Upper and lower endoscopic examinations and capsule endoscopy of the small intestine (SI) did not reveal any significant findings. Treatment with anti-tumor necrosis factor (anti-TNF) agents was initiated but resulted in paradoxical psoriasis with adalimumab and infliximab. Cyclosporine was also used, but the patient experienced intolerable tachycardia. After 18 months, the patient developed episcleritis and experienced diarrhea accompanied by cramp-like abdominal pain. Repeat upper and lower endoscopic examinations showed normal results, while capsule endoscopy of the SI revealed CD enteritis. The patient was diagnosed with CD of the small intestine, along with extraintestinal manifestations of vulvoperineal MCD, oral involvement, and episcleritis. Management with azathioprine and ustekinumab was initiated, resulting in significant clinical improvement. MCD poses a diagnostic challenge due to its unusual manifestations. It may present without gastrointestinal tract involvement, mimicking other conditions. Therefore, timely diagnosis and the selection of the most appropriate therapeutic strategy are crucial.

La enfermedad de Crohn metastásica (ECM) es una entidad rara, es una manifestación cutánea en enfermedad Crohn (EC), y en su presentación clínica es inusual el compromiso vulvar y oral de modo concomitante. Se describe el caso de una mujer de edad media que tiene historia familiar de autoinmunidad, presenta inicialmente compromiso oral y vulvoperineal. En su abordaje inicial se descartó enfermedad de Behçet, pero los estudios histopatológicos a nivel vulvar tuvieron hallazgos compatibles para ECM. Hubo ausencia de síntomas gastrointestinales y sus niveles de calprotectina fecal eran normales. Los estudios endoscópicos altos y bajos, y la cápsula endoscópica del intestino delgado (ID) no tuvieron hallazgos relevantes. Se inició el tratamiento con antifactor de necrosis tumoral (anti-TNF) y presentó una psoriasis paradójica con adalimumab e infliximab. También se utilizó ciclosporina y presentó una taquicardia intolerable. 18 meses después de estos síntomas tuvo epiescleritis e inició con diarrea asociada a dolor abdominal tipo cólico, por lo que se repitieron los estudios endoscópicos altos y bajos, que resultaron normales, y cápsula endoscópica de ID mostró una enteritis por EC. Se consideró EC de ID con manifestaciones extraintestinales y con ECM vulvoperineal, compromiso oral y epiescleritis. Se requirió manejo con azatioprina y ustekinumab, con una mejoría clínica significativa. La ECM es un reto diagnóstico, pues es una manifestación inusual; en su debut puede haber ausencia de compromiso en el tracto gastrointestinal y también simular otras entidades. Debe buscarse lograr el diagnóstico oportuno y la estrategia terapéutica más segura.

Abordaje de la enfermedad de Behçet vulvar: comunicación de caso

La enfermedad de Behçet (EB) es un trastorno inflamatorio, multisistémico, recidivante y remitente de etiología desconocida. Una característica clave de la enfermedad es la ulceración orogenital que provoca una morbilidad considerable con gran impacto en la calidad de vida de los pacientes. Su manejo médico consiste en un esquema con colchicina, esteroides o inmunomoduladores. Comunicamos el caso de una paciente con lesiones genitales quien consultó en múltiples ocasiones, recibiendo manejo antibiótico e incluso quirúrgico, con respuesta refractaria a estas intervenciones. Posteriormente, por las características de las lesiones y el comportamiento clínico se sospechó enfermedad de Behçet y se instauró manejo médico con esteroides orales y curaciones con fitoestimulina. Hubo resolución de las úlceras genitales y posteriormente la paciente fue llevada a cirugía para corrección de las sinequias vulvares.

Behçet's disease (BD) is an inflammatory, multisystemic, relapsing-remitting disorder of unknown etiology. A key feature of the disease is orogenital ulceration that causes considerable morbidity with great impact on patient's quality of life. Its medical management consists of a scheme with colchicine, steroids or immunomodulators. We report the case of a patient with genital lesions who consulted on multiple occasions, receiving antibiotics and even surgical management, with refractory response to these interventions. Subsequently, due to the characteristics of the lesion and clinical behavior, Behçet's disease was suspected and medical management with oral steroids and cures with phytostimulin was initiated. The genital ulcers were resolved, and the patient was subsequently taken to surgery for correction of the vulvar synechiae.

Carcinoma neuroendocrino de vulva, una entidad poco frecuente. Reporte del primer caso en Venezuela / Neuroendocrine carcinoma of the vulva, a rare entity. Report of the first case in Venezuela

El cáncer de vulva representa 1 % de los cánceres en mujeres; el carcinoma neuroendocrino de vulva es extremadamente raro, con menos de 20 casos publicados. Su comportamiento es muy agresivo produciendo recurrencias locales tempranas, metástasis a ganglios linfáticos y a distancia. Se describe el caso de una paciente de 60 años de edad, quien consultó por lesión vulvar de 6 meses de evolución, con extensión a tercio inferior de uretra y vagina y plastrón ganglionar inguinal izquierda. La biopsia de vulva reportó neoplasia maligna de células redondas y el resultado de la inmunohistoquímica fue compatible con carcinoma neuroendocrino. Recibió quimioterapia y radioterapia concurrente, evidenciando respuesta completa en vulva y parcial en región inguinal, posteriormente se realizó linfadenectomía inguinofemoral bilateral. El carcinoma neuroendocrino de vulva debe ser considerado al momento de manejar patología vulvar maligna, el diagnóstico oportuno requiere histología e inmunohistoquímica para establecer un pronóstico y tratamiento adecuados(AU)

Vulvar cancer represents 1% of cancers in women; vulvar neuroendocrine carcinoma is extremely rare, with less than 20 published cases. Its behavior is very aggressive, producing early local recurrences, lymph node and distant metastases. The case of a 60-year-old patient who consulted for a vulvar lesion of 6 months of evolution, with extension to the lower third of the urethra and vagina and left inguinal nodal plastron is described. The vulvar biopsy reported malignant round cell neoplasm and the immunohistochemistry result was compatible with neuroendocrine carcinoma. She received concurrent chemotherapy and radiotherapy, evidencing a complete response in the vulva and a partial response in the inguinal region. Later, bilateral inguinofemoral lymphadenectomy was performed. Neuroendocrine carcinoma of the vulva should be considered when managing malignant vulvar pathology, timely diagnosis requires histology and immunohistochemistry to establish an adequate prognosis and treatment(AU)

Carcinosarcoma of the vulva: a rare case report

Carcinosarcomas are rare and clinically aggressive neoplasms with poor outcome. A very few cases has been reported in the literature. We present a case of a 54 yrs woman with bleeding per vagina for 1.5 months. On per vaginal examination, a lump of size 3×2 cm was found over the left anterior region of vulva. CECT abdomen revealed a well-defined heterogeneously enhancing lesion with calcific foci involving both labia minora. MRI pelvis reveals carcinoma left vulva with involvement of lower 3rd of vaginal wall. Histopathological examination showed features suggestive of carcinosarcoma of the vulva. This was further confirmed by immunohistochemistry which showed positivity for CK, SMA, caldesmon and negative for S100, CD34, desmin, CD31, ERG and MyoD1. Because it is a very rare tumor with severe prognosis, we presented this case as this case report can be a useful addition to the literature.

A Solitary Trichilemmoma in Uncommon Location Of Vulva: A Rare Case Report

Trichilemmoma is a benign neoplasm with differentiation towards pilosebaceous follicular epithelium or outer root sheath. It often presents with solitary lesions but when multiple can be associated with Cowden syndrome with various risks of neoplasms. It is commonly found in the region of the head and neck. It is often asymptomatic and long-standing. The treatment is optional but malignancy has to be excluded in the rapidly growing lesion. We are hereby presenting the occurrence of trichilemmoma in a rare location over the genital area. Initial biopsy was suggestive of features of trichilemmoma. She underwent total excision due to fear of malignancy and cosmetic reasons. The histopathology findings corroborated with trichilemmoma. The patient is asymptomatic without any local complaints and recurrence on follow-up for 6 months. Trichilemmoma should be kept as a differential diagnosis in the verrucous lesions over the genital skin.

Fios de PDO, hidroxiapatita de cálcio e ácido l-polilático para flacidez vulvar - indicações, técnica e resultados / PDO threads, calcium hydroxyapatite and l-polylactic acid for vulvar flaccidity - indications, technique and results

Os grandes lábios tornam-se flácidos com o envelhecimento. Apesar de o preenchimento com ácido hialurônico proporcionar um rejuvenescimento significativo, ele também pode levar à formação de verdadeiros testículos vulvares se mal indicado, principalmente em vulvas excessivamente flácidas, que não suportam qualquer volumização adicional. Nesses casos de flacidez excessiva, acreditamos que os bioestimuladores de colágeno seriam o melhor tratamento inicial, pois sua injeção levaria à reposição de colágeno dérmico, com melhora da frouxidão, sem acréscimo expressivo de volume. Temos utilizado a hidroxiapatita de cálcio, o ácido l-polilático e os fios não espiculados de PDO, desde 2019, com bons resultados.

The labia majora become saggy with aging. Although filling with hyaluronic acid provides significant rejuvenation, it can also lead to the formation of true vulvar testicles if poorly indicated, especially in excessively flaccid vulvas, which do not support any further volumization. In these cases of excessive flaccidity, we believe that collagen biostimulators would be the best initial treatment, as their injection would lead to the replacement of dermal collagen and improvement in laxity, without a significant increase in volume. We have been using calcium hydroxyapatite, l-polylactic acid, and PDO threads since 2019, with good results.

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

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

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

Quemadura genital por producto químico: reporte de caso / Genital burn by chemical product: case report

Resumen ANTECEDENTES: Las quemaduras son la forma más severa de estrés que el cuerpo puede sufrir; pueden generarse por diferentes agentes térmicos y químicos. CASO CLÍNICO: Paciente de 25 años, con dolor intenso en la región genital de 12 horas de evolución, secundario a la introducción en la vagina de una piedra de alumbre. Se le hicieron múltiples irrigaciones con solución salina al 0.9% sin obtener el resto de la piedra de alumbre. Se le aplicó sulfadiazina de plata en la cavidad vaginal cada 12 horas, óvulos vaginales de ketanserina, miconazol y metronidazol cada 8 horas, ketorolaco por vía oral 10 mg cada 8 horas. Durante su estancia hospitalaria tuvo buena evolución, con disminución de la inflamación en la zona genital, epitelización adecuada. Al tercer día se dio de alta del hospital con cita para valoración a los siete días. CONCLUSIÓN: El tratamiento de las quemaduras en el área genital, por agentes químicos, tiene como piedra angular la identificación del agente causante de la lesión que permita actuar de forma inmediata y evitar las secuelas físicas, sexuales y psicológicas mediante el lavado exhaustivo con solución o agua estéril para remover el agente causal y disminuir que continúe actuando en el sitio afectado.

Abstract BACKGROUND: Burns are the most severe form of stress that the body can suffer; they can be caused by various thermal and chemical agents. CLINICAL CASE: A 25-year-old female patient presented with severe genital pain of 12 hours' duration, secondary to the introduction of an alum stone into the vagina. She underwent several irrigations with 0.9% saline without obtaining the rest of the alum stone. She was given vaginal silver sulfadiazine every 12 hours, vaginal ketanserin, miconazole and metronidazole every 8 hours and oral ketorolac 10 mg every 8 hours. During her stay in hospital, she progressed well, with a decrease in genital inflammation and adequate epithelialisation. She was discharged on the third day with an appointment for a seven-day follow-up. CONCLUSION: The management of genital burns caused by chemical agents is based on the identification of the agent causing the lesion, which allows immediate action and prevents physical, sexual and psychological sequelae by thorough washing with sterile solution or water to remove the causative agent and reduce its continued action in the affected area.

Langerhans Cell Histiocytosis in an Adult Female with Multisystem Involvement

Langerhans cell histiocytosis (LCH) also known as histiocytosis X, is a rare systemic disorder arising from the clonal proliferation of myeloid dendritic cells (histiocytes) with a tendency to involve single or multiple organ systems with variable clinical course and prognosis. Clinical presentation usually depends on the site of involvement. The organs commonly affected in adults by order of decreasing frequency include lungs, bone, skin, pituitary glands, lymph nodes, and the liver. Vulval and perianal involvement is extremely rare in adults. We describe the case of a 31-year-old non-smoker adult female with multisystemic LCH involving the vulva, perianal region, and lung. Probable involvement of other sites with LCH included mandibular bone, pituitary gland, skin, lymph nodes, liver, thyroid, and colon. She is undergoing systemic chemotherapy and has completed two cycles of cytarabine and steroids without any complications. Treatment is not standardized due to the very less incidence of the disease and inadequate knowledge regarding its pathophysiology. Langerhans cell histiocytosis remains a major concern for treating physicians because of its rarity with many faces and requires careful consideration for management.

True Hermaphroditism in a Goat – A case report

A four-month-old prepubertal female goat was presented to RVP IVRI Bareilly, with the history of an abnormal growth in the vulva. On clinical examination the growth was found as prominent clitoris, and two peanut sized soft structures were palpable at the subcutaneous in inguinal region. Transabdominal B mode real time ultrasound of inguinal region revealed testis like morphology and presence of uterus and ovaries structures subcutaneously. The case was subsequently diagnosed as hermaphrodism.