Angiomiofibroblastoma vulvoperineal, presentación de un caso / Vulvoperineal angiomyofibroblatoma, a case report

An angiomyofibroblastoma is a benign tumor that may present itself in the genital tract of a reproductive age woman. Despite it being a benign neoplasia, unable to be invasive, it can reach large sizes, producing discomfort, pain, and anatomical deformation. A late diagnosis may result in increased surgical difficulty for resection. The following is the clinical case of a woman with a vulvo-perineal tumor of 6 years of evolution, with a final diagnosis of angiomyofibroblastoma, in which complete resection of the lesion was achieved with good cosmetic results. The highlight of this tumor is its slow, silent, but progressive growth, leading to diagnoses that are often confused and late, with a consequent delay in treatment. Among the common differential diagnoses are the Bartholin gland cyst and the aggressive angiomyxoma.

El angiomiofibroblastoma es un tumor benigno que puede presentarse en el tracto genital de la mujer en edad reproductiva. A pesar de ser una neoplasia benigna, sin capacidad invasora, puede alcanzar grandes tamaños, produciendo molestia, dolor y alteración importante de la anatomía. Si el diagnóstico es tardío, aumenta la dificultad quirúrgica en su resección. A continuación, se presenta el caso clínico de una mujer con un tumor vulvoperineal de 6 años de evolución, con diagnóstico final de angiomiofibroblastoma, en el que se logró la resección completa de la lesión con un buen resultado estético. La importancia de este tumor está dada por su crecimiento lento, silencioso, pero progresivo, llevando a que su diagnóstico sea en muchas ocasiones confuso y tardío, con el consecuente retraso en el tratamiento. Dentro de los diagnósticos diferenciales comunes se encuentran el quiste de la glándula de Bartholino y el angiomixoma agresivo.

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

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

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.

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

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

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

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

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

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

Genital angiokeratoma in a woman with Fabry disease: the dermatologist's role

Abstract: Fabry disease is a rare lysosomal storage disorder, inherited in an X-linked manner. It is characterized by the deficiency of the enzyme alpha-galactosidase, leading to a buildup of glycosphingolipids in the cells. Angiokeratoma is one of the cutaneous manifestations of this condition, and it helps making the diagnosis. The typical site involves the genital area in men and lumbosacral, buttocks and trunk region in both sexes. We report a case of genital angiokeratoma in a woman with Fabry disease. The diagnosis is through molecular analysis and, when made early, starting treatment reduces the morbidity and mortality of the disease. Thus, the dermatologist has an important role in the identification of angiokeratoma as a cutaneous marker, and the knowledge of its different presentations is essential for the early diagnosis and management of Fabry disease.

Carcinoma escamoso de vulva / Vulvar squamous carcinoma

El cáncer de vulva es una neoplasia relativamente rara, supone 5 por ciento de todos los cánceres ginecológicos. Su etiología, en general, se desconoce, pero existen pruebas que categorizan el virus del papiloma humano como un factor causal. En Cuba representa menos de 1 por ciento de todos los tumores malignos de la mujer, con su máxima aparición en la pos menopausia. Presentamos un caso de carcinoma escamoso de vulva diagnosticado en la atención primaria de salud. Se trata de una fémina de 65 años de edad, mestiza, fumadora inveterada, primeras relaciones sexuales a los 20 años, múltiples parejas sexuales, la mayoría desprotegidas, citología realizada en el año 2014 negativa, quien acudió a su consultorio, perteneciente al Policlínico Docente Efraín Mayor Amaro, del Cotorro, por presentar aumento de volumen en sus genitales, en ocasiones le producía prurito. Al examen físico se constató lesión verrugosa en el labio mayor derecho de aproximadamente 4 cm de diámetro, de contornos irregulares, no dolorosa a la palpación. Se interconsulta con Ginecología donde se indica exámenes complementarios, que demostraron en la citología actual, una displasia leve, NIC I, HPV. En consulta de Patología de cuello se realiza biopsia por ponche que se informa como: Carcinoma escamoso moderadamente diferenciado verrugoso. Posteriormente fue remitida a consulta de oncología para tratamiento quirúrgico, siguiendo los protocolos marcado por el comité oncológico de la Federación Internacional de Ginecólogos y Obstetras (FIGO). Se reafirma que el diagnóstico temprano es importante, debiéndose realizar mediante evaluación clínico e histopatológica, garantizando su tratamiento oportuno(AU)

Vulvar cancer is a relatively rare neoplasm, accounting for 5 percent of all gynecological cancers. The etiology is unknown, but there are tests that categorize by preference the human papillomavirus (HPV) as a causal factor. In Cuba, it represents less than 1 percent of all malignant tumors in women, and the maximum appearance in post menopause. We present the case of a patient over 65 years of age, mestizo, and incorrigible smoker. She had her first sexual intercourses at age 20, most of them unprotected, she had multiple sexual partners. The cytology performed in 2014 was negative. She came to consultation due to an increase in the volume of her genitals, which sometimes caused itching. In pathology consultation, punch biopsy is performed, which is reported as moderately differentiated verrucous squamous carcinoma. She was then taken on an Oncology consult for surgical treatment, following the protocols marked by the oncologic committee of the International Federation of Gynecologists and Obstetrics (FIGO). Early diagnosis is important and should be carried out through clinical and histopathologically evaluation to ensure timely treatment(AU)

Enfermedad de Paget extramamaria de la vulva: a propósito de un caso clínico / Extramammary Paget's disease of the vulva: apropos of a clinical case

La Enfermedad de Paget extramamaria de la vulva es una neoplasia poco frecuente que representa solo el 1% de los cánceres de vulva. Clínicamente, se caracteriza por una placa eritematosa, bien definida, con erosiones y ulceraciones. El síntoma más frecuente es el prurito. Esta clínica tan inespecífica puede retrasar el diagnóstico, además de inducir a tratamientos inadecuados. El diagnóstico se realiza con estudio histopatológico e inmunohistoquímica, la cual orienta si es primario de piel o metástasis de otro cáncer, usualmente genitourinario o gastrointestinal subyacente. Actualmente no hay guías clínicas de tratamiento, pero dentro de las opciones la más aceptada es la cirugía de Mohs. Otros tratamientos incluyen uso de imiquimod, láser CO2, quimioterapia tópica o local, radioterapia o terapia fotodinámica. Cuando se trata de una enfermedad de Paget extramamaria primaria, el pronóstico es excelente y la mayoría de los pacientes fallece por otra condición. En cuanto a la enfermedad invasora, tiene una mortalidad de 13-18% con sobrevida a 5 años de 72% (1)

Tumor de células granulares de la vulva: caso clínico / Granular cell tumor of the vulva: a clinical case

El tumor de células granulares es una neoplasia de la piel y los tejidos blandos muy poco frecuente, benigna y de crecimiento lento, pero con altas tasa de recurrencia. La localización más frecuente en el aparato genital es en la vulva. Se debe realizar diagnóstico diferencial con otras lesiones vulvares, debido al manejo distinto de esta tumoración. El diagnóstico generalmente es histológico, ya que clínicamente es muy difícil diferenciarlo de otras tumoraciones a nivel vulvar. El tratamiento recomendado es la exéresis quirúrgica, con márgenes de seguridad. En caso de bordes afectos, se recomienda realizar una reescisión, por la frecuencia de recurrencia local y porque ésta puede ser el primer indicador de una conducta agresiva. Presentamos un caso clínico a nivel vulvar, con el objetivo de destacar la importancia de realizar un diagnóstico correcto, para un buen manejo clínico y seguimiento. Las formas malignas suponen menos del 3% de estos tumores y son muy agresivas

The granular cell tumor is a neoplasm of the skin and soft tissue very rare, benign and slow growing, but with high recurrence rate. The most frequent location in the genital tract is in the vulva. Differential diagnosis should be made with other vulvar lesions, due to differences in the management of this tumor. The diagnosis is usually histological, since it is clinically difficult to differentiate it from other tumors at the vulvar level. The recommended treatment is surgical excision, with safety margins. In case of affected borders, it is recommended to perform a resection, due to the local recurrence and this may be the first indicator of aggressive behavior. We present a clinical case at the vulvar level, in order to emphasize the importance of making a correct diagnosis, for a good clinical management and follow-up. Malignant forms account for less than 3% of these tumors and are very aggressive.

Multiple vulvar dilated pores in a postmenopausal woman

Abstract: Winer's dilated pore is an infrequent appendageal tumor characterized by a giant comedone on the face, neck, and upper trunk in adults. We report a 57-year-old woman who developed multiple asymptomatic black papules on both labia majora. Histopathology showed grouped dilated follicles lined by keratinizing squamous epithelium in the superficial dermis. The superficial lining epithelium and interfollicular epidermis were atrophic, while the deep epithelium showed mild proliferation and melanin pigmentation with a few short projections extending into the surrounding dermis. We diagnosed multiple Winer's dilated pores based on late-onset lesions and pathological features. This patient may represent the first case of multiple vulvar Winer's dilated pores. We suggest that electrocautery may be effective for treating this type of superficial entity.

Differential Diagnosis between Bartholin Cyst and Vulvar Leiomyoma: Case Report / Diagnóstico diferencial entre cisto de Bartholin e leiomioma vulvar: relato de caso

Abstract Genital leiomyomas are rare tumors that can often be misdiagnosed as Bartholin cyst. We report a case of a 32-year-old patient who had a cystic nodulation in the left labium majus that was suggestive of Bartholin cyst. A resection surgery was performed, and the definitive histopathology diagnosis was vulvar leiomyoma. The macroscopic features of cystic lesions difficult the differential diagnosis between leiomyoma and Bartholin cyst; therefore, a histopathologic examination is often recommended.

Resumo Os leiomiomas genitais são tumores raros, e frequentemente são diagnosticados como cisto de Bartholin. Relatamos o caso de uma paciente de 32 anos em que ocorreu nodulação cística no grande lábio esquerdo sugestiva de cisto de Bartholin. Uma cirurgia de ressecção foi realizada, e o diagnóstico histopatológico definitivo foi leiomioma vulvar. As características macroscópicas das lesões císticas dificultam o diagnóstico diferencial entre o leiomioma e o cisto de Bartholin, demodo que o exame histopatológico é frequentemente recomendado.

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.

Metastasis pulmonares tardías de carcinoma adenoideo quístico de la glandula de bartolino / Late lung metastases of cystic adenoid carcinoma of the bartolino gland

El carcinoma adenoideo quístico de la glándula de Bartolino (GB) es una entidad infrecuente, de crecimiento lento y comportamiento agresivo con alta recurrencia local y metástasis. Presentamos el caso de una paciente de 48 años, con antecedente de tratamiento quirúrgico de un absceso Bartolino derecho. La biopsia demostró un carcinoma adenoideo quístico (CAQ) de la GB, por lo que se realizó hemivulvectomía radical derecha y linfadenectomía inguinofemoral. El resultado de la biopsia mostró 4 ganglios positivos de 12 y margen lateral positivo a menos de 2 mm. Por lo que se realizó adyuvancia con radioterapia. La paciente no se presenta a su control y acude luego de tres años por dolor urente desde cadera izquierda hacia rodilla y cara lateral de la pierna y pie. Se realiza cintigrama óseo que muestra lesión osteoblástica sacroilíaca izquierda de 2 cm, por lo que se completó estudio de diseminación con tomografía computada de tórax, abdomen y pelvis con contraste que evidenció múltiples lesiones nodulares pulmonares bilaterales de distribución generalizada en parénquima central, periférico y subpleurales. Se deriva al Instituto Nacional del Tórax, completando estudio preoperatorio y se realizó una biopsia quirúrgica por video toracoscopía resecando un nódulo subpleural, el informe histológico señaló una metástasis de CAQ concordante con primario en GB. La paciente fue presentada en el comité oncológico y se indicó radioterapia paliativa a lesión sacroilíaca y control clínico ambulatorio. Ha evolucionado estable desde el punto de vista funcional pulmonar.

Adenoid cystic carcinoma of Bartholin glands (BG) is a rare, slow-growing but a highly aggressive tumor with remarkable capacity for local recurrence and distant metastasis. We present the case of a 48 year-old female patient with a history of surgical treatment for right Bartholin abscess. The biopsy showed a cystic adenoid carcinoma of the BG, which led to radical hemivulvectomy and inguinofemoral lymphadenectomy. The biopsy show inguinal lymph node metastasis on 4 of 12 lymph nodes and positive surgical resection margin. Adjuvant radiotherapy was applied. The patient doesnt present to her clinical follow up, and consult three years later with radiological evidence of sacroiliac metástasis on bone scintigraphy. Study was completed with thorax, abdomen and pelvis tomography scan, and showed multiple bilateral lung nodules. The patient was transfered to National Thoracic Institute, and preoperative study was performed. We decide to obtain histologycal confirmation by a video thoracoscopy, it was performed without incidents. Histology show metastasis of adenoid cystic carcinoma pattern, concordant with primary tumor. The patient was submitted to the oncology committee and palliative radiotherapy was indicated for sacroiliac lesion. The patient evolve stable and asintomatic at ambulatory clinical control.

Verrucous carcinoma of the vulva: diagnosis and treatment

Abstract: Vulvar cancer accounts for less than 1% of malignancies in women. Verrucous carcinoma of the vulva is a rare histological variation, comprising less than 1% of vulvar cancer cases. Although it is characterized as being locally invasive, the condition is not associated with metastatic spreading. Lesions present in the form of a verrucous, ulcerated, and bleeding tumor that can reach large dimensions. This type of tumor can be mistaken for condylomata, both macroscopically and microscopically. We report the case of an 81-year-old patient with a large vulvar tumor presented for eight years, initially considered as a Buschke-Löwenstein tumor. The patient underwent radical vulvectomy with a V-Y advancement flap technique. This type of tumor should be considered by clinicians dealing with condylomatous ulcerative lesions that do not respond to the usual treatment.

Extramammary Paget Disease of the Vulva - Case Report / Relato de Caso: Doença de Paget Extramamária da Vulva

Abstract Extramammary Paget disease (EPD) is a rare malign neoplasm that may affect the vulva and has manifestations common to benign diseases such as itching, pain and eczema. This leads to delay in diagnosis and consequent worse prognosis. The definitive diagnosis is obtained by biopsy of the vulva, which shows Paget cells. The treatment of choice is wide excision with margins, which leads to sequelae, functional and aesthetic. Recurrence is common. This article reports the case of a 48-year-old female patient with history of vulvar itching for the past 2 years, who had been submitted to various treatments for benign pathologies. The patient was biopsied and was diagnosed with extensive EPD, being submitted to vulvectomy. This article aims to draw attention to the need for biopsy of pruritic vulvar lesions that do not respond to usual treatment.

Resumo A doença de Paget extramamária (DPEM) é uma neoplasia maligna incomum que pode acometer a vulva, e que tem manifestações comuns a doenças benignas como prurido, dor e eczema. Isto leva a atraso no diagnóstico e, consequente, piora do prognóstico. O diagnóstico de certeza se dá pela biópsia de vulva que evidencia células de Paget. O tratamento de escolha é a excisão com margens amplas, que leva a sequela funcional e estética. A recidiva é frequente. Este artigo relata o caso de uma paciente do sexo feminino, de 48 anos, com história de prurido vulvar há 2 anos com diversos tratamentos para patologias benignas. A biopsia da paciente confirmou diagnóstico de DPEM extensa sendo submetida a vulvectomia. Este artigo visa chamar a atenção para a necessidade de biópsia das lesões vulvares pruriginosas que não respondem ao tratamento habitual.

Giant Condyloma (Buschke-Loewenstein Tumor) in a 16-year-old Patient: Case Report / Condiloma gigante (tumor de Buschke-Loewenstein) em uma paciente de 16 anos: relato de caso

Abstract The Buschke-Loewenstein tumor is characterized by excessive growth of verrucous lesions on the genitals and/or perianal region. It is considered benign despite the high rate of recurrence and the possibility of malignant transformation. It is commonly associated with subtypes 6 and 11 of the human papillomavirus (HPV), and host 's immunity plays an important role in the development of the disease. Surgical excision is the recommended treatment in most cases. We present the case of a 16 years old female patient with extensive vulvar lesions successfully treated surgically.

Resumo O tumor de Buschke-Loewenstein se caracteriza pelo crescimento excessivo de lesões verrucosas na região genital e/ou perianal. É considerado benigno apesar da elevada taxa de recorrência e da possibilidade de transformação maligna. Está comumente associado aos sorotipos 6 e 11 do papiloma vírus humano (HPV) e a imunidade do hospedeiro tem importante papel no desenvolvimento da doença. A excisão cirúrgica é o tratamento recomendado na maioria dos casos. Apresentamos o caso de uma paciente do sexo feminino, de 16 anos, com lesão vulvar de grande extensão tratada cirurgicamente com sucesso.

Groin lymph node detection and sentinel lymph node biopsy in vulvar cancer / 부인종양

OBJECTIVE: To identify suitable diagnostic tools and evaluate the efficacy of sentinel lymph node (SLN) biopsy for inguinal lymph node metastasis in vulvar cancer. METHODS: Data from 41 patients with vulvar cancer were evaluated retrospectively, including magnetic resonance imaging (MRI) measurements, SLN biopsy status, groin lymph node metastasis, and prognosis. RESULTS: SLN biopsy was conducted in 12 patients who had stage I to III disease. Groin lymphadenectomy was omitted in five of the nine patients with negative SLNs. All SLN-negative patients who did not undergo groin lymphadenectomy showed no evidence of disease after treatment. On MRI, the long and short diameters of the inguinal node were significantly longer in metastasis-positive cases, compared with negative cases, in 25 patients whose nodes were evaluated pathologically (long diameter, 12.8 mm vs. 8.8 mm, p=0.025; short diameter, 9.2 mm vs. 6.7 mm, p=0.041). The threshold of >10.0 mm for the long axis gave a sensitivity, specificity, positive predictive value, and negative predictive value of 87.5%, 70.6%, 58.3%, and 92.3%, respectively, using a binary classification test. Decision tree analysis revealed a sensitivity, specificity, and accuracy of 87.5%, 70.6%, and 76.0%, respectively, with the threshold of >10.0 mm for the long axis on MRI. The criteria of >10.0 mm for the long axis on MRI predicted an advanced stage and poorer prognosis using a validation set of 15 cases (p=0.028). CONCLUSION: Minimally invasive surgery after preoperative evaluation on MRI and SLN biopsy is a feasible strategy for patients with vulvar cancer.

Syringocystadenoma papilliferum in an unusual location

Abstract: Syringocystadenoma papilliferum is a rare benign hamartomatous adnexal tumor of the apocrine or eccrine sweat glands. Most patients present a solitary lesion in the head and neck region. Presentation outside the head and neck region is even more uncommon. We present a case of Syringocystadenoma papilliferum with papulonodular lesion located on the vulva of an infant girl. This case illustrates the atypical location of this rare disease and adds to the differential diagnosis of lesions on the vulva.

Aggressive angiomyxoma of the vulva: case report / Angiomixoma agressivo da vulva: relato de caso

Female patient, 42-years-old, complaining of difficulty in urinating and swelling in the vulvar area for one year. Her gynecological examination showed extensive injury in the vulvar region and the biopsy done was inconclusive. The removal of the lesion was conducted. After the procedure, the patient remains free of recurrence for 15 months. This case highlights the need to consider angiomyxoma in the differential diagnosis for tumors of unknown cause in the vulvar region.

Paciente do gênero feminino, 42 anos, com queixas de dificuldade ao urinar e aumento de volume na região vulvar há 1 ano. Ao exame ginecológico, apresentava extensa lesão na região vulvar. Biópsia da lesão foi inconclusiva. Realizou-se a exérese da lesão. A paciente permanece livre de recorrências há 15 meses. Este caso destaca a necessidade de considerar o angiomixoma no diagnóstico diferencial de massas de causa desconhecida na região vulvar.