Rare vulvar lesions: A case series

@#Mass of the vulva include both benign and malignant lesions. Benign mass lesions of the vulva include tumors, hamartomas, cysts, infectious disorders, and non-neoplastic epithelial disorders. Some are rare mass lesions. Here, we presented three rare mass lesions of the vulva. The first case presented with the complaint of vulvar lesion since childhood, while the two cases presented in reproductive age and perimenopausal age. All three cases presented as mass lesions in the vulva with pain or uneasiness during work. In all these cases, excision was done. On histopathological examination, the lesions have different diagnoses which are common in other parts of the body, but rarely present at the vulva. A definitive diagnosis of a vulvar mass lesion is difficult to make, especially in the case of rare mass lesion. Histopathology is the principal tool of diagnosis.

Aggressive Angiomyxoma of the Vulva: Which Is the Best Management Strategy? Description of a Case Report and Review of Literature of the Last Ten Years.

BACKGROUND: Aggressive angiomyxoma is a rare mesenchymal tumour of the genital tract with a high incidence in women of reproductive age. The aim of our work is to understand which is the best management strategy for this condition, starting from the description of a rare case report up to the performance of a narrative review of the literature. METHODS: We report a case of a 46-year-old woman who came to our attention because of the growth of a 10-centimetre pedunculated, non-soft, non-tender mass of the left labium majus. She underwent surgical excision, and the histologic diagnosis was aggressive angiomyxoma. Due to a lack of tumour-free margins, radicalization surgery was carried out after three months. A review of the literature of the last ten years was performed following the PRISMA statement on MEDLINE (PubMed). We obtained data from twenty-five studies describing thirty-three cases. RESULTS: Aggressive angiomyxoma is characterized by a high recurrence rate of between 36 and 72% after surgery. There is no universal consensus about hormonal therapy, and most studies (85%) describe surgical excision followed by only clinical and radiological follow-up. CONCLUSION: Wide surgical excision is the gold-standard treatment for aggressive angiomyxoma, succeeded by either clinical or radiological (ultrasound or MRI) follow-up.

Extramammary Paget's Disease of the Vulva and Concomitant Premalignant/Malignant Vulvar Lesions: A Potential Challenge in Diagnosis and Treatment.

The aim of the present study was to evaluate the incidence of concomitant vulvar cancers or premalignant lesions in women surgically treated for extramammary Paget's disease of the vulva (EMPDV) through a multicenter case series. The medical records of all women diagnosed with and treated for EMPDV from January 2010 to December 2020 were retrospectively analyzed. Women with EMPDV and synchronous vulvar cancer, vulvar intraepithelial neoplasia (VIN) and/or lichen sclerosus (LS) at the histology report were included in the study. A total of 69 women eligible for the present study were considered. Concomitant vulvar lesions occurred in 22 cases (31.9%). A total of 11 cases of synchronous VIN (50%) and 14 cases (63.6%) of concomitant LS were observed. One patient (4.5%) had synchronous vulvar SCC (FIGO stage 1B). Women with EMPDV and concomitant premalignant/malignant vulvar lesions had a significantly higher rate of invasive EMPDV and wider lesions with an extravulvar involvement. The specific meaning of the association between EMPDV, VIN, SCC and LS remains unclear. The potential overlapping features between different vulvar lesions highlight the importance of dedicated gynecologists and pathologists in referral centers.

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.

Depigmentation of the vulvar area: Is it an alarming sign?

A 34-year-old female patient presented with recurrent bilateral hypopigmented macules on the labia majora. The lesions were treated with topical steroids, which led to mild improvement, but erosive plaques developed after discontinuing the treatment. Histopathological findings were compatible with extramammary Paget disease (EMPD), which was treated with radical vulvectomy with no recurrence in the next months of follow-up.

DEVIL, VAAD and vLSC constitute a spectrum of HPV-independent, p53-independent intra-epithelial neoplasia of the vulva.

AIMS: We aimed to characterise a large cohort of non-invasive, human papillomavirus (HPV) and p53-independent verruciform lesions, such as 'vulvar acanthosis with altered differentiation' (VAAD), 'differentiated exophytic vulvar intra-epithelial lesion' (DEVIL) and 'verruciform lichen simplex chronicus' (vLSC). METHODS AND RESULTS: From January 2008 to December 2020 we retrospectively identified 36 eligible patients with verruciform non-invasive lesions (n = 36) and collected clinical, histological and follow-up parameters. Verruciform non-invasive lesions occurred at a median age of 71 years, with a median follow-up of 33.5 months. Clinically, pruritus was only reported in patients with VAAD (n = 3, 21%). Lesion colour was significantly different across categories (P = 0.028). Apart from the histopathological criteria already known to distinguish these entities (hypogranulosis, epithelial pallor and low-magnification architecture), no other significant criteria were discovered and significant overlap was observed, particularly between VAAD and DEVIL. Patients with vLSC trended towards longer survival without recurrence compared to VAAD and DEVIL (P = 0.082), but showed comparable invasion-free survival interval (P = 0.782). Squamous cell carcinomas (SCC) associated with either VAAD, DEVIL or vLSC displayed similar clinical, histopathological and biological parameters. In non-invasive precursor lesions, stromal oedema was associated with invasion (P = 0.015) and remained so upon Cox regression analysis (P = 0.009). CONCLUSION: Our study of HPV and p53 independent non-invasive verruciform lesions of the vulva highlights significant clinical, histopathological and biological overlap between VAAD, DEVIL and vLSC, suggesting that these pre-invasive lesions should be viewed as a spectrum. We also show that stromal features such as oedema might play an import role in progression to invasion.

Isolated Non-Resolving Vulvar Lesion as a Presentation of Disseminated Histoplasmosis in a Woman with HIV.

Disseminated histoplasmosis is most commonly caused by Histoplasma capsulatum and is a known opportunistic infection in immunocompromised patients. The clinical presentation of histoplasmosis varies from asymptomatic to a progressive disseminated form. Pulmonary and CNS involvement is common in disseminated histoplasmosis. Rarely, disseminated disease can present as vulvar lesion in the absence of pulmonary symptoms, causing a delay in diagnosis. As per our PubMed literature search, there have only been two reported cases where vulvar lesion was the only presenting symptom of disseminated disease. In our patient, a histopathological diagnosis was made with staining showing budding yeast forms of histoplasma. LEARNING POINTS: Histoplasmosis can be a cause of isolated non-resolving vulvar lesion in immunocompromised patients.In patients at risk, we recommend work-up for disseminated histoplasmosis with urine histoplasma antigen and further imaging to rule out disseminated infection when histoplasma is identified on local biopsy samples.In patients from endemic regions and in non-resolving vulvar lesions, fungal infection should be considered.

Childhood-Onset Eosinophilic Granulomatosis with Polyangiitis with a Vulvar Granuloma: A Case Report and Review of the Literature.

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem necrotizing vasculitis associated with eosinophilia and extravascular granuloma and classically involving the upper and lower airways. There have only been a few reported cases of gynecologic involvement in EGPA. CASE: We present an 8-year-old girl diagnosed with EGPA with a vulvar granuloma in what is, to our knowledge, the first reported pediatric gynecologic manifestation of EGPA. Interestingly, the vulvar granuloma did not respond to initial immunosuppressant treatment with prednisone and methotrexate and required treatment regimen modification with mycophenolate mofetil resulting in granuloma resolution. SUMMARY AND CONCLUSION: EGPA in the pediatric population has a relatively high mortality rate compared with in the adult population thus it is important that vulvar granulomas associated with EGPA should be included in the differential diagnosis of a vulvar mass allowing for the prompt diagnosis and treatment of this potentially fatal disease in children.

Recurrence of endometrial carcinoma presenting as vulvar lesions.

Vulvar metastasis is an unusual presentation of recurrent endometrial cancer and should be part of the differential diagnosis of vulvar lesion.

Vulvar Lesions in an 8-Year-Old Girl: Cutaneous Manifestations of Multisystem Langerhans Cell Histiocytosis.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare localized or systemic disease characterized by proliferation of myeloid-derived dendritic cells. Vulvar lesions might be the herald symptom of LCH and might mimic other cutaneous lesions. Prognosis varies widely on the basis of the extent and spread of disease. CASE: An 8-year-old girl with a 4-month history of vulvar lesions resistant to topical steroids was referred by her pediatrician. Vulvar biopsy was diagnostic for LCH. Imaging studies revealed a left hip lesion consistent with LCH. The patient was subsequently treated for multisystem LCH with vinblastine and prednisone. SUMMARY AND CONCLUSION: Although rare, LCH might be diagnosed by gynecologic providers and should be included in the differential diagnosis of genital lesions. We recommend having a low threshold for performing biopsy of vulvar lesions.

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.

Benign Phyllodes Tumor of the Vulva: A Case Report and Literature Review.

Phyllodes tumor is an uncommon breast lesion with characteristic histologic appearance when examined by hematoxylin and eosin staining: leaf-like fronds projecting into cystic spaces on low-power microscopy, and biphasic (epithelial and stromal) components on high-power microscopy. We report a rare primary case of this tumor arising within the vulva. A 34-year old African American female presented with a 3 cm slow-growing vulvar mass initially thought to be an inclusion cyst. The lesion was excised and histologic examination demonstrated this lesion to be a rare case of benign phyllodes tumor with morphologic features similar to those arising from breast tissue. Patient received no further treatment and did not exhibit any recurrence or metastasis. Nearly two years after excision, the patient died due to an unrelated medical cause. This rare tumor should be considered in the differential diagnosis for women presenting with a slow-growing vulvar mass.

Granulomatose linfomatóide-relato de caso / Lymphomatoid granulomatosis -case report

Objetivo: descrever um caso com manifestaçõs clínicas e achados histopatológicos de Granulomatose Linromatóide (GL), uma rara doença linfoproliferativa das células B, angiocêntrica e angiodestrutiva associada ao vírus Epstein-Barr (EBV). A apresentação clínica mais comum é no pulmão, podendo acometer qualquer órgão, sendo os achados extra-pulmonares as principais manifestações nestes casos. Relato do caso: mulher de 65, com lesão cutânea vulvar e que evoluiu com quadro neurológico associado a lesão expansiva envolvendo parênquima cerebelar, sem alterações pulmonares. Considerações Finais: pelo seu prognóstico e capacidade de ser confundida com outras vasculites a GL impõe-se como um importante diagnóstico diferencial dessas doenças.

lntroduction: this report describe a case of Lymphomatoid Granulomatosis (LG,s a rare linfoproliferative disorder of B cell showing angiocentric growth and destructive behaviour caused by Epstain-Barr virus (EBV). The predominant clinical presentations are confined to the pumonary system, however ; extra-pumonary manifestations can sometimes be the main feature of the dieseses. Method: here we report a 65 year old female, with a skin vulvar lesion, and another in cerebelus, without pulmonary disease. Conclusion: the LG can mimics systemic vasculits and is a diagnostic challenge.