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Objective: To report the prevalence of malignant transformation of vulvar lichen sclerosus (VLS) and possible risk factors. Methods: This is a cohort study with data analysis from medical records of 138 patients with histological diagnosis of VLS registered at the Vulvar Pathology Outpatient Clinic of the University Hospital, between 2007 and 2017. Predominance of risk factors was performed using logistic regression analysis. The variables studied were the length of follow-up, age, regular or irregular follow up; presence of symptoms (dyspareunia, pruritus and/or vulvar burning); histology characteristics, the presence of epithelial hyperplasia; and the presence of autoimmune diseases. Results: There were 138 patients included in the study, and among them five progressed to malignant transformation. The patients had a median age of 59 years and 83% were symptomatic. The most frequent symptom was itching with 72%. Autoimmune diseases were present in 11.6%, the most prevalent being thyroid disease. All five case of malignant transformation (0.6%) had an irregular follow up. The logistic regression analysis was used among the studied variables, and no statistical significance was found among them (p ≥ 0.05). The relationship between hyperplasia and the clinical outcome of malignant transformation, in which non-significant but acceptable p value close to 0.05 was observed. Conclusion: The prevalence of malignant transformation in patients with VLS was 0.6%, and common factors were the lack of adherence to medical treatments and the loss of follow-up.
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Transformación Celular Neoplásica , Liquen Escleroso Vulvar , Humanos , Femenino , Persona de Mediana Edad , Liquen Escleroso Vulvar/epidemiología , Liquen Escleroso Vulvar/complicaciones , Factores de Riesgo , Adulto , Anciano , Estudios de Cohortes , Neoplasias de la Vulva/epidemiología , Neoplasias de la Vulva/patología , Prevalencia , Estudios Retrospectivos , Anciano de 80 o más Años , Adulto JovenRESUMEN
OBJECTIVE: To describe a single-center experience managing women with vulvar squamous cancer and analyze factors influencing their survival. STUDY DESIGN: It is an observational longitudinal retrospective study that reviewed medical records of patients admitted for treatment at the University of Campinas between 2010 and 2019, followed up until June 2022. The final sample was 108 cases. The main outcomes were disease-free survival (DFS) and overall survival (OS). Other variables were age, stage, relapse, and race. Vital status was accessed by medical records, active search, or public online register. Survival analysis was performed by the Kaplan-Meier method and Log-rank Test, and Regression Cox-Model assessed risks. RESULTS: The mean age in stages IA and IB were 65.0 years, and in stages II + III + IVA 71.1 years. Women 70 years or older were more related to diagnosis in stages II + III + IVA (p = 0.019). Progression was observed in 7 (16.7 %) patients in stage IB and 30 (65.2 %) in stage II + III + IVA. Both five-year (5y) DFS and OS were significantly different in stage IB and II + III + IVA (5y-DFS 70.5 % and 39.3 %, p = 0.024; 65.1 % and 24.3 %, p < 0.001). In stages II + III + IVA, most deaths happened before 24 months of follow-up. The primary treatment was surgery in 81.0 % of stage IB and 47.8 % of stage II + III + IVA. A higher OS was observed in patients treated primarily by surgery compared to radiotherapy in stage IB (p = 0.008), and in stages II + III + IVA (p = 0.013). Surgery followed or not by adjuvant radiotherapy was independently associated with a 60 % adjusted death protection compared to radiotherapy alone as primary treatment (0.40, 0.23;0.70). CONCLUSIONS: Half of the patients have been diagnosed in stage I. The progression rate was high in the advanced stages of the disease. Overall survival by stage was improved when surgery was the primary treatment. Surgery was independently associated with death.
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Carcinoma de Células Escamosas , Neoplasias de la Vulva , Anciano , Femenino , Humanos , Carcinoma de Células Escamosas/patología , Supervivencia sin Enfermedad , Recurrencia Local de Neoplasia , Estadificación de Neoplasias , Estudios Retrospectivos , Neoplasias de la Vulva/cirugía , Estudios LongitudinalesRESUMEN
A necropsy was performed on a 43-year-old female zoo chimpanzee, with cancer in the vulvar and perivulvar region. She was diagnosed with squamous cell carcinoma, the presence of this tumor in domestic animals and non-human primates is very rare in the vulvar region and there were no previous reports found on it in chimpanzee, due to which this report contributes to the knowledge on chimpanzee pathologies.
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Carcinoma de Células Escamosas , Neoplasias de la Vulva , Femenino , Animales , Pan troglodytes , Carcinoma de Células Escamosas/diagnóstico , Carcinoma de Células Escamosas/veterinaria , Carcinoma de Células Escamosas/patología , Neoplasias de la Vulva/diagnóstico , Neoplasias de la Vulva/veterinaria , Neoplasias de la Vulva/patología , Animales DomésticosRESUMEN
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.
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Carcinoma de Células Escamosas , Lesiones Precancerosas , Neoplasias de la Vulva , Femenino , Humanos , Hormona Liberadora de Corticotropina/genética , Hormona Liberadora de Corticotropina/metabolismo , Regulación hacia Arriba , Urocortinas/genética , Urocortinas/metabolismoRESUMEN
Introduction: HPV infection is a common risk factor for all anogenital cancers. However, there are important differences in the epidemiology of anogenital cancers and these have not been compared considering diverse epidemiological indicators over a long period of time. To fill this gap, we investigated incidence, mortality, and survival trends of anogenital cancers over a period of three decades. Methods: We conducted an observational registry-based study using data from the population-based cancer registry of Granada in southern Spain. We collected data on all incident cases of anogenital cancer (cervical, anal, penile, vulvar, and vaginal cancer) diagnosed between 1985 and 2017. We calculated crude and age-standardized incidence and mortality rates, and 1, 3, and 5-year overall and net survival. We further conducted time-trend analysis calculating annual percent changes (APC) for each cancer site. Results: The incidence of anogenital cancers decreased slightly during the past 30 years, with the exception of vulvar cancer, where a slight increase was observed. Mortality decreased significantly for cervical cancer over the study period but increased non-significantly for the remaining cancer sites. Survival rates were similar to those reported in comparable countries and increased for cervical and vulvar cancer. Discussion: Cervical cancer was the greatest contributor to the burden of anogenital cancers and showed a marked improvement in all indicators in comparison to the remaining cancer sites.
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Neoplasias del Ano , Infecciones por Papillomavirus , Neoplasias del Cuello Uterino , Neoplasias de la Vulva , Femenino , Humanos , Virus del Papiloma Humano , Neoplasias del Cuello Uterino/epidemiología , Neoplasias de la Vulva/epidemiología , Neoplasias de la Vulva/complicaciones , Neoplasias del Ano/epidemiología , Neoplasias del Ano/diagnóstico , Infecciones por Papillomavirus/complicacionesRESUMEN
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.
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Humanos , Femenino , Adulto , Neoplasias de la Vulva/cirugía , Neoplasias de la Vulva/diagnóstico , Angiomioma/cirugía , Angiomioma/diagnóstico , Angiofibroma/cirugía , Angiofibroma/diagnóstico , Perineo , Neoplasias de la Vulva/patología , Angiomioma/patología , Angiofibroma/patologíaRESUMEN
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.
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Humanos , Femenino , Adulto , Fibroma/cirugía , Fibroma/etiología , Procedimientos Quirúrgicos Ginecológicos , Vulva/patología , Enfermedades de la Vulva/complicaciones , Neoplasias de la Vulva , Heridas y Lesiones/complicaciones , Informes de Casos , Células del Estroma/patología , Neoplasias Fibroepiteliales/rehabilitaciónRESUMEN
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.
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Oncología por Radiación , Neoplasias de la Vulva , Femenino , Humanos , Neoplasias de la Vulva/radioterapia , Radioterapia Adyuvante , Ganglios Linfáticos/patología , ConsensoRESUMEN
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.
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Braquiterapia , Carcinoma , Oncología por Radiación , Neoplasias de la Vulva , Femenino , Humanos , Neoplasias de la Vulva/radioterapia , Recurrencia Local de Neoplasia/radioterapiaRESUMEN
BACKGROUND: Leiomyoma of the vulva is rare, accounting for only 0.03% of all gynecological tumors, and it is seldom seen in teenagers. CASE: We describe a case of vulvar leiomyoma in a 14-year-old girl who presented a 10 × 10 cm solid tumor in the right hemivulva without other complaints. SUMMARY AND CONCLUSION: Differential diagnosis includes Bartholin cysts, abscesses, fibromas, and other solid lesions. Although rare, vaginal leiomyoma must be remembered and included as a differential diagnosis for solid lesions in the vagina.
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Glándulas Vestibulares Mayores , Leiomioma , Neoplasias Vaginales , Neoplasias de la Vulva , Adolescente , Glándulas Vestibulares Mayores/patología , Diagnóstico Diferencial , Femenino , Humanos , Leiomioma/diagnóstico por imagen , Leiomioma/cirugía , Neoplasias Vaginales/diagnóstico , Vulva/patología , Neoplasias de la Vulva/diagnóstico , Neoplasias de la Vulva/patología , Neoplasias de la Vulva/cirugíaRESUMEN
OBJECTIVE: To investigate the immunohistochemical (IHC) expression of the ErbB/HER family in primary vulvar squamous cell carcinoma (VSCC). METHODS: We analyzed a series of 125 patients who were surgically treated for VSCC from January 1980 to June 2016. All cases had lymph node (LN) staging and 80 had LN metastasis. A tissue microarray was built for epidermal growth factor receptor (EGFR), HER2, HER3, and HER4 IHC staining. RESULTS: In the primary tumor we found positive expressions for EGFR, HER2, HER3, and HER4 in 5%, 0.9%, 0.9%, and 22.8%, respectively. For the LN metastasis, expressions of EGFR and HER4 were positive in 22.2% and 39.1%, respectively. No cases had positive staining for HER2 and HER3 in the LN metastasis. For HER4, positive expression correlated with smaller tumor sizes (P = 0.02). However, positive HER4 was related to adverse prognostic factors such as: histological grade (P = 0.012), presence of lymphovascular space invasion (40.9% vs 16.2%; P = 0.035), and perineural invasion (57.1% vs 16.7%; P = 0.006). Notably, all cases with LN metastasis had positive HER4 in the primary tumor (P < 0.001). ErbB/HER family expression was not related to worse survival. CONCLUSION: EGFR, HER2, and HER3 were infrequently expressed in VSCC by IHC. HER4 IHC expression was found in 22.8% of cases and was related to adverse prognostic factors.
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Neoplasias de la Vulva , Femenino , Humanos , Inmunohistoquímica , Receptor ErbB-2/metabolismo , Receptor ErbB-3/metabolismo , Receptor ErbB-4/metabolismoRESUMEN
Background: Squamous cell carcinoma (SCC) is a common cutaneous neoplasm in horses, which mainly affects the external genitalia, oral cavity, and periocular region. The development of SCC metastases is rare in these animals, and the most common occurrence is a marked local infiltration. Exposure to ultraviolet rays and skin depigmentation are the main etiological factors of SCC. Definitive diagnosis of the neoplasm is performed through histological examination of lesions. The present report describes the clinical, anatomopathological, and immunohistochemical (IHC) aspects of a case of metastatic SCC, with the vulva as the primary site in a mare. Case: A 17-year-old mare, mixed breed, was referred to the Veterinary Hospital of the Universidade de Passo Fundo (UPF), with history of areas of depigmented skin (pinto coat), and clinical history of anorexia, frequent episodes of colic, and recumbency. During clinical evaluation, heart rate (68 bpm), rectal temperature (38.4ºC), and respiratory rate (48 mpm) were elevated, and the oral mucosa was pale. The mare also had an ulcerated tumor mass involving the vulva, which extended to the inguinal region and involved the mammary gland. A cytological aspirate of the vulvar tumor was performed, in which no neoplastic cells were found. Next, a biopsy of 2 distinct areas of the vulva was performed. The material was sent for anatomopathological examination, which showed markedly pleomorphic malignant squamous cells, with individual keratinization and high mitotic index, organized in trabeculae with rare keratin pearl-like formations. The exam allowed the diagnosis of SCC Grade II. Due to the poor prognosis and high cost of treatment, the owner consented to euthanasia and necropsy examination. During necropsy, the vulvar tumor mass was grayish, firm to hard, infiltrative, and had friable areas. Tumor foci suggestive of metastasis were also observed in inguinal, mesenteric, mediastinal and renal lymph nodes, adrenal glands, lung, pericardium, medullary canal, intercostal muscles, right 15th rib, and tissue surrounding the azygos vein. Samples from all organs were collected for anatomopathological examination. Diagnosis of metastatic vulvar SCC was confirmed through histological and IHC studies, which evaluated the expression of cytokeratins (AE1/ AE3), as well as the proliferative activity of neoplastic cells through the PCNA marker. Discussion: The diagnosis of metastatic SCC was obtained through the observed clinical, necroscopic, histological, and IHC characteristics. This neoplasm usually appears in depigmented regions exposed to ultraviolet light, and older animals are more likely to be affected. The mare in the present case had areas of depigmented skin (pinto coat). The animal was kept in a paddock outdoor and exposed to constant solar radiation. Although rare in horses, manifestation of anorexia, progressive weight loss, and frequent colic episodes and recumbency may be closely related to the multiple sites of metastasis in the present case. The main histological findings of the neoplasm were the dense proliferation of malignant squamous cells with individual keratinization, arranged in a trabecular pattern and with rare formations of keratin pearls, in line with previous studies. During IHC evaluation, the neoplastic cells showed expression of cytokeratins (AE1/AE3), as well as high proliferative activity evidenced by the PCNA marker. Given this background, the present report describes the clinical, anatomopathological, and IHC aspects of a case of metastatic SCC with a primary site in the vulva of a mare.
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Animales , Femenino , Vulva/patología , Neoplasias de la Vulva/veterinaria , Carcinoma de Células Escamosas/veterinaria , Caballos , Metástasis de la Neoplasia , Inmunohistoquímica/veterinariaRESUMEN
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.
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Humanos , Femenino , Anciano de 80 o más Años , Neoplasias de los Tejidos Blandos/patología , Neoplasias de la Vulva/patología , Diagnóstico DiferencialRESUMEN
INTRODUCTION: In the present study, we report a case of primary adenoid cystic carcinoma (ACC) of the Bartholin gland with high-grade transformation (HGT). Adenoid cystic carcinoma of the Bartholin gland is a rare tumor and HGT has only been reported in head and neck tumors. CASE REPORT: A 77-year-old woman with a non-ulcerated vulvar lesion on the topography of the right Bartholin gland. The patient was submitted to tumor resection followed by V-Y island flap and adjuvant radiotherapy. The histopathological examination revealed primary ACC of the Bartholin gland, with areas of HGT and extensive perineural invasion. The immunohistochemical study with p53 showed a diffuse and strong positive reaction in areas with HGT. After 24 months of follow-up, the patient presented distant metastases and died, despite having undergone to chemotherapy. CONCLUSION: As far as we know, this case is the first description in the literature of HGT in ACC of the Bartholin gland, and HGT appears to be associated with tumor aggressiveness.
INTRODUçãO: Este estudo relata o caso de um carcinoma adenoide cístico (CAC) de glândula de Bartholin com transformação de alto grau. O CAC de glândula de Bartholin é um tumor raro, e sua transformação de alto grau é relatada somente em tumores de cabeça e pescoço. RELATO DE CASO: Paciente de 77 anos de idade, do sexo feminino, com lesão vulvar não ulcerada na topografia da glândula de Bartholin direita. A paciente foi submetida a ressecção do tumor e realização de retalho em V-Y, seguidas de radioterapia adjuvante. O exame histopatológico revelou CAC primário de glândula de Bartholin, com áreas de transformação de alto grau e invasão perineural. O estudo imunohistoquímico com p53 mostrou reação positiva difusa e intensa em áreas com transformação de alto grau. Após 24 meses de seguimento, a paciente apresentou metástases à distância e faleceu, apesar de ter sido submetida a quimioterapia. CONCLUSãO: Pelo que sabemos, este caso é a primeira descrição na literatura de transformação de alto grau em CAC de glândula de Bartholin, e a transformação de alto grau parece estar associada à agressividade do tumor.
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Glándulas Vestibulares Mayores , Carcinoma Adenoide Quístico , Neoplasias de la Vulva , Anciano , Carcinoma Adenoide Quístico/terapia , Femenino , Humanos , Radioterapia AdyuvanteRESUMEN
Introducción: Los tumores malignos de vulva son tumores raros del tracto genital femenino, que representan menos del 1 % de los tumores malignos del sexo femenino, cerca del 95% de estos son carcinoma escamo celulares, los adenocarcinomas son el tipo histológico menos frecuente, con 36 casos reportados hasta el año 2020. Caso Clínico: Presentamos el caso de una mujer de 36 años con antecedente de histerectomía total por miomatosis, con cuadro clínico de 1 año de evolución caracterizado por una masa indolora en labio mayor izquierdo la cual se somete a biopsia que reporta adenocarcinoma motivo por el cual se somete a vulvectomia radical, en resultado patológico definitivo se confirma el diagnostico de adenocarcinoma de vulva tipo glándula mamaria. Evolución: Se realiza vulvectomía radical + vaciamiento ganglionar inguino-iliaco bilateral. El estu-dio histo-patológico definitivo reveló adenocarcinoma de vulva tipo glándula mamaria pobremente diferenciado G3, Unifocal, con metástasis en 22/25 ganglios derechos y 30/30 izquierdos. Un estu-dio reportó recidiva local extensa, metástasis ganglionares pélvicas, retroperitoneales, mediastinales, axilares, cervicales y pulmonar. Se inició Paclitaxel 137 mg semanal. Se completa quimioterapia hasta 11ra semana posterior a lo cual presentó infección de lecho tumoral y fallece. Conclusión: En el presente caso el curso clínico de adenocarcinoma de vulva tipo glándula mamaria fue agresivo con una supervivencia inferior a 5 meses
Introduction: Malignant tumors of the vulva are rare tumors of the female genital tract, which rep-resent less than 1% of malignant tumors of the female sex, about 95% of these are squamous cell carcinoma, adenocarcinomas are the least frequent histological type, with 36 cases reported until 2020. Clinical Case: We present the case of a 36-year-old woman with a history of total hysterectomy due to myomatosis, with a clinical picture of 1 year of evolution characterized by a painless mass in the left labia majora which undergoes a biopsy that reports adenocarcinoma, which is why undergoes radical vulvectomy, the definitive pathological result confirms the diagnosis of adenocarcinoma of the vulva type mammary gland. Evolution: Radical vulvectomy + bilateral inguino-iliac lymph node dissection was performed. The definitive histopathological study revealed poorly differentiated G3, Unifocal mammary gland adeno-carcinoma of the vulva, with metastases in 22/25 right and 30/30 left lymph nodes. One study reported extensive local recurrence, pelvic, retroperitoneal, mediastinal, axillary, cervical and pulmonary lymph node metastases. Paclitaxel 137 mg weekly was started. Chemotherapy was completed up to the 11th week after which he presented a tumor bed infection and died. Conclusion: In the present case, the clinical course of adenocarcinoma of the vulva type of the mammary gland was aggressive with a survival of less than 5 months.
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Humanos , Femenino , Adulto , Neoplasias de la Vulva , Informes de Casos , Oncología Médica , Vulva , VulvectomíaRESUMEN
Abstract Introduction In the present study, we report a case of primary adenoid cystic carcinoma (ACC) of the Bartholin gland with high-grade transformation (HGT). Adenoid cystic carcinoma of the Bartholin gland is a rare tumor and HGT has only been reported in head and neck tumors. Case Report A 77-year-old woman with a non-ulcerated vulvar lesion on the topography of the right Bartholin gland. The patient was submitted to tumor resection followed by V-Y island flap and adjuvant radiotherapy. The histopathological examination revealed primary ACC of the Bartholin gland, with areas of HGT and extensive perineural invasion. The immunohistochemical study with p53 showed a diffuse and strong positive reaction in areas with HGT. After 24 months of follow-up, the patient presented distant metastases and died, despite having undergone to chemotherapy. Conclusion As far as we know, this case is the first description in the literature of HGT in ACC of the Bartholin gland, and HGT appears to be associated with tumor aggressiveness.
Resumo Introdução Este estudo relata o caso de um carcinoma adenoide cístico (CAC) de glândula de Bartholin com transformação de alto grau. O CAC de glândula de Bartholin é um tumor raro, e sua transformação de alto grau é relatada somente em tumores de cabeça e pescoço. Relato de caso Paciente de 77 anos de idade, do sexo feminino, com lesão vulvar não ulcerada na topografia da glândula de Bartholin direita. A paciente foi submetida a ressecção do tumor e realização de retalho em V-Y, seguidas de radioterapia adjuvante. O exame histopatológico revelou CAC primário de glândula de Bartholin, com áreas de transformação de alto grau e invasão perineural. O estudo imunohistoquímico com p53 mostrou reação positiva difusa e intensa em áreas com transformação de alto grau. Após 24 meses de seguimento, a paciente apresentou metástases à distância e faleceu, apesar de ter sido submetida a quimioterapia. Conclusão Pelo que sabemos, este caso é a primeira descrição na literatura de transformação de alto grau em CAC de glândula de Bartholin, e a transformação de alto grau parece estar associada à agressividade do tumor.
Asunto(s)
Humanos , Femenino , Anciano , Glándulas Vestibulares Mayores , Neoplasias de la Vulva , Carcinoma Adenoide Quístico/terapia , Radioterapia AdyuvanteRESUMEN
ANTECEDENTES: En cumplimiento del inciso e, sobre nuestras funciones como UFETS, que dice: "Evaluar las tecnologías sanitarias ya existentes en la entidad, y proponer estrategias para su uso eficiente y/o reposición", realizamos esta revisión rápida sobre la utilidad del nanocoloide de albumina combinado con la tinción de "patent blue" en la detección de ganglio centinela en pacientes con tumoración maligna en la región mamaria, vulvar o melanoma cutáneo. ESTRATEGIA DE BÚSQUEDA DE INFORMACIÓN: Pregunta Clínica: En pacientes con enfermedad oncológica ¿Cuál es la utilidad del nanocoloide de albumina Tc-99m combinado con la tinción de "patent blue" para la detección del ganglio centinela? Recolecciòn de los manuscritos a revisar: Tipos de estudios: La estrategia de búsqueda sistemática de información científica para el desarrollo del presente informe se realizó siguiendo las recomendaciones de la Pirámide jerárquica de la evidencia propuesta por Haynes y se consideró los siguientes estudios: umarios y guías de práctica clínica. Revisiones sistemáticas y/o meta-análisis. Ensayos Controlados Aleatorizados (ECA). Estudios Observacionales (cohortes, caso y control, descriptivos). No hubo limitaciones acerca de la fecha de publicación o el idioma para ningún estudio. Fuentes de información: De acceso libre . ases de datos: Pubmed y Cochrane. DISCUSIÓN: En base a los estudios expuesto se ha evidenciado que se avala que el uso de la inclusión de la estrategia combinada (Uso del nanocoloide de albumina Tc99m como parte de la linfogammagrafía y posteriormente el uso de "patent blue" durante la biopsia) para la detección y extirpación del ganglio centinela sobre todo en los tipos de cáncer de vulva, mama y melanoma cutáneo. La estrategia combinada permite tener valores de sensibilidad superiores a 95% y razones de verosimilitud positiva por encima de 10. Además, lo más resaltante es que permiten disminuir los falsos negativos hasta en menos del 5% de los casos. CONCLUSIONES: En el Instituto Nacional de Enfermedades Neoplásicas se realizan anualmente aproximadamente entre 800 a 900 intervenciones que requieren la detección del ganglio centinela por Tc-900m con albumina sérica humana. Se realizó una búsqueda sistemática y una búsqueda dirigida de la evidencia para evaluar la utilidad del Tc-900m con albumina sérica humana en pacientes con tumoraciones malignas de vulva, mama y melanoma cutáneo en quienes se va a identificar la presencia de ganglio centinela. Se encontraron 06 guías de las principales sociedades científicas que abordan el tema; una revisión sistemática, una revisión no sistemática y 03 estudios prospectivos (Dentro de ellos un Ensayo Clínico Aleatorizado). Con respecto a las guías, se ha encontrado que todas avalan el uso de coloides unidos a Tc-99m e incluso las guías de la sociedad europea de medicina nuclear menciona que el coloide de albumina sérica humana es el preferido en Europa. Con respecto a la revisión sistemática y los estudios primarios se encontró valores aceptables de sensibilidad y una baja cantidad de falsos negativos lo cual permite mejorar la exactitud diagnóstica del ganglio centinela en los cánceres de vulva, mama y melanoma cutáneo. Con respecto a la adquisición, está disponible a nivel nacional y cuenta con más de 10 años en el mercado. Además, cada unidad puede ser usado hasta en 05 pacientes. Finalmente, en base a la evidencia encontrada tanto a nivel internacional como a nivel nacional el panel establece que la intervención de coloide de albumina sérica humana más Tc-99m y combinada con "patent blue" es útil en la detección del ganglio centinela de neoplasias de mama, vulva y melanoma; además, disminuye la cantidad de falsos negativos. Por lo cual, se aprueba su uso a nivel institucional.