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Seminars in Diagnostic Pathology Jan 2021Extramammary Paget disease (EMPD) is a rare neoplasm with uncertain histogenesis, usually presenting in the anogenital area, most commonly in the vulva. The disease is... (Review)
Review
Extramammary Paget disease (EMPD) is a rare neoplasm with uncertain histogenesis, usually presenting in the anogenital area, most commonly in the vulva. The disease is characterized by slow grow and high recurrence rates. This article reviews the epidemiological, clinical, morphological, genetic and treatment features of EMPD of the vulva reported in recent years.
Topics: Diagnosis, Differential; Female; Humans; Paget Disease, Extramammary; Prognosis; Vulvar Neoplasms
PubMed: 32921571
DOI: 10.1053/j.semdp.2020.09.007 -
Journal of Lower Genital Tract Disease Jul 2022The European Society of Gynaecological Oncology (ESGO), the International Society for the Study of Vulvovaginal Disease (ISSVD), the European College for the Study of...
The European Society of Gynaecological Oncology (ESGO), the International Society for the Study of Vulvovaginal Disease (ISSVD), the European College for the Study of Vulval Disease (ECSVD) and the European Federation for Colposcopy (EFC) Consensus Statements on Pre-invasive Vulvar Lesions.
The European Society of Gynaecological Oncology (ESGO), the International Society for the Study of Vulvovaginal Disease (ISSVD), the European College for the Study of Vulval Disease (ECSVD), and the European Federation for Colposcopy (EFC) developed consensus statements on pre-invasive vulvar lesions in order to improve the quality of care for patients with vulvar squamous intraepithelial neoplasia, vulvar Paget disease in situ, and melanoma in situ. For differentiated vulvar intraepithelial neoplasia (dVIN), an excisional procedure must always be adopted. For vulvar high-grade squamous intraepithelial lesion (VHSIL), both excisional procedures and ablative ones can be used. The latter can be considered for anatomy and function preservation and must be preceded by several representative biopsies to exclude malignancy. Medical treatment (imiquimod or cidofovir) can be considered for VHSIL. Recent studies favor an approach of using imiquimod in vulvar Paget's disease. Surgery must take into consideration that the extension of the disease is usually wider than what is evident in the skin. A 2 cm margin is usually considered necessary. A wide local excision with 1 cm free surgical margins is recommended for melanoma in situ. Following treatment of pre-invasive vulvar lesions, women should be seen on a regular basis for careful clinical assessment, including biopsy of any suspicious area. Follow-up should be modulated according to the risk of recurrence (type of lesion, patient age and immunological conditions, other associated lower genital tract lesions).
Topics: Carcinoma in Situ; Colposcopy; Female; Humans; Imiquimod; Melanoma; Paget Disease, Extramammary; Pregnancy; Skin Neoplasms; Squamous Intraepithelial Lesions; Vulvar Neoplasms; Melanoma, Cutaneous Malignant
PubMed: 35763611
DOI: 10.1097/LGT.0000000000000683 -
Expert Review of Anticancer Therapy Jun 2015Squamous cell carcinoma of the vulva is a rare disease, accounting for approximately 5% of cancers of the female genital tract. Standard therapy for early-stage vulvar... (Review)
Review
Squamous cell carcinoma of the vulva is a rare disease, accounting for approximately 5% of cancers of the female genital tract. Standard therapy for early-stage vulvar cancer mainly comprises of surgery of the vulva and groins. In locally advanced or metastatic vulvar cancer, neoadjuvant or definitive chemoradiation is often considered as an alternative treatment option. Given its rarity, the level of evidence for different treatment modalities is poor and few clinical trials have been performed on this disease. Therefore indication criteria for systemic treatment in advanced stage vulvar cancer vary widely among countries and institutions. This review focuses on the different systemic treatment options for patients with locally advanced, recurrent or metastatic vulvar cancer, and highlights the need for an international multicenter approach to identify the most effective therapeutic options.
Topics: Carcinoma, Squamous Cell; Female; Humans; Neoplasm Metastasis; Neoplasm Recurrence, Local; Neoplasm Staging; Vulvar Neoplasms
PubMed: 25997120
DOI: 10.1586/14737140.2015.1037837 -
Obstetrics and Gynecology Clinics of... Sep 2017Cutaneous vulvar neoplasms are commonly encountered at gynecology visits, with 2% of women having a benign vulvar melanocytic nevus and 10% to 12% of nevi being vulvar.... (Review)
Review
Cutaneous vulvar neoplasms are commonly encountered at gynecology visits, with 2% of women having a benign vulvar melanocytic nevus and 10% to 12% of nevi being vulvar. High-grade squamous intraepithelial lesions (vulvar intraepithelial neoplasia 2 or 3) occurs in 5 per 100,000 women, with increasing incidence in the past 30 years. The recognition of these lesions and differentiation between benign, premalignant, and malignant stages are crucial for adequate diagnosis, clinical monitoring, and treatment. The presentation, diagnosis, and management of benign and malignant vulvar proliferations are discussed with focus on practical aspects of clinical care.
Topics: Female; Humans; Precancerous Conditions; Vulva; Vulvar Neoplasms
PubMed: 28778635
DOI: 10.1016/j.ogc.2017.04.002 -
Obstetrics and Gynecology Clinics of... Sep 2017Red patches and plaques of the vulva may be manifestations of neoplasms, infections, or inflammatory skin diseases. These diseases can mimic one another clinically;... (Review)
Review
Red patches and plaques of the vulva may be manifestations of neoplasms, infections, or inflammatory skin diseases. These diseases can mimic one another clinically; features that generally allow the diseases to be identified on most cutaneous surfaces can be altered in the moist, occluded vulvar environment, making clinical diagnosis difficult. A detailed history and thorough physical examination can point to the likely diagnosis, but biopsy and culture may be needed for diagnosis especially in refractory disease. It is not uncommon for several of these processes to be present concomitantly or complicating other vulvar diseases.
Topics: Dermatitis; Exanthema; Female; Humans; Vulva; Vulvar Diseases; Vulvar Neoplasms
PubMed: 28778636
DOI: 10.1016/j.ogc.2017.05.002 -
International Journal of Gynecological... Mar 2022The primary treatment for resectable vulvar cancer includes wide local excision of the primary tumor and surgical lymph node assessment. Following surgery, up to 40-50%... (Review)
Review
The primary treatment for resectable vulvar cancer includes wide local excision of the primary tumor and surgical lymph node assessment. Following surgery, up to 40-50% of patients develop a local recurrence. Historically, the strongest predictor of local recurrence is a positive or close margin (defined as <8 mm), although recent studies question the importance of margin status. Post-operative radiotherapy to the vulva is recommended for all women with a positive margin where re-excision is not possible. Radiotherapy may also be considered in the setting of risk factors for local recurrence: close margin, lymphovascular invasion, large tumor size, and/or depth of invasion >5 mm. Nodal assessment is an important component of vulvar cancer management. A negative sentinel node is associated with a low false-negative predictive value (2% in patients with vulvar tumor <4 cm in GOG 173), 2-year groin recurrence rate of 2.3%, and 3-year disease-specific survival rate of 97% in patients with unifocal vulvar tumor <4 cm in the GROningen INternational Study on Sentinel nodes in Vulvar Cancer (GROINSS-V I) study. Thus, patients with tumor size <4 cm (without additional local risk factors) and negative sentinel node can be observed. Patients with sentinel node metastasis ≤2 mm can be treated with post-operative radiotherapy (2-year isolated groin recurrence rate of 1.6% in GROINSS-V II), as a safe alternative to lymphadenectomy. Patients with sentinel node metastasis >2 mm following sentinel node biopsy should undergo inguinofemoral lymphadenectomy followed by post-operative radiotherapy-based on the GROINSS-V II study, the 2-year isolated groin recurrence rate remains unacceptably high (22%) with radiotherapy alone. Retrospective studies suggest that the addition of concurrent chemotherapy to radiotherapy may improve survival. The ongoing GROINSS-V III study is investigating concurrent chemotherapy and radiotherapy dose escalation. The main goal of these post-operative treatments is to reduce the risk of local, and especially groin, recurrences, which are almost universally fatal.
Topics: Female; Humans; Lymph Node Excision; Lymph Nodes; Lymphadenopathy; Lymphatic Metastasis; Neoplasm Recurrence, Local; Neoplasm Staging; Retrospective Studies; Sentinel Lymph Node Biopsy; Vulvar Neoplasms
PubMed: 35256421
DOI: 10.1136/ijgc-2021-002463 -
Ugeskrift For Laeger May 2018Vulvar intraepithelial neoplasia (VIN) is a rare but premalig-nant condition. VIN has two aetiological pathways: a human papillomavirus (HPV)-dependent pathway, which is... (Review)
Review
Vulvar intraepithelial neoplasia (VIN) is a rare but premalig-nant condition. VIN has two aetiological pathways: a human papillomavirus (HPV)-dependent pathway, which is a vulvar high-grade squamous intraepithelial lesion (HSIL), and an HPV-independent pathway, called differentiated VIN (d-VIN), associated with lichen sclerosus. d-VIN is more aggressive than vulvar HSIL. In case of symptoms, a biopsy should be performed. The recurrence risk is high: 25-50% regardless of treatment type. We recommend treatment with imiquimod as first choice to avoid mutilating surgery. Particular attention must be payed to immunosuppressed patients with VIN. HPV-vaccine can be discussed with patients with vulvar HSIL.
Topics: Antineoplastic Agents; Carcinoma in Situ; Female; Humans; Imiquimod; Immunocompromised Host; Papillomaviridae; Papillomavirus Infections; Papillomavirus Vaccines; Precancerous Conditions; Vulvar Neoplasms
PubMed: 29761782
DOI: No ID Found -
Archives of Gynecology and Obstetrics Apr 2024
Topics: Female; Humans; Angiofibroma; Vulva; Vulvar Neoplasms
PubMed: 37819477
DOI: 10.1007/s00404-023-07245-9 -
Current Oncology Reports Aug 2019The aim of this article is to provide clinicians and pathologists with an understanding of the aetiopathology, pathogenesis and classification of vulval neoplasia and... (Review)
Review
PURPOSE OF REVIEW
The aim of this article is to provide clinicians and pathologists with an understanding of the aetiopathology, pathogenesis and classification of vulval neoplasia and their molecular correlates.
RECENT FINDINGS
There is an increased understanding of subcellular changes in vulvar malignancies. These provide the direction for further research and aid personalised treatment for patients. The article explores concepts of the aetiology of vulvar cancer and updates the reader with the equivalence of terminology of preneoplastic vulval disease. The differential diagnosis of squamous neoplasia and their clinicopathological correlation is detailed. The salient findings from recent literature into the understanding of the disease of squamous cell neoplasia and rare vulvar malignancies are summarised.
Topics: Carcinoma, Squamous Cell; Female; Humans; Precancerous Conditions; Prognosis; Vulvar Neoplasms
PubMed: 31418120
DOI: 10.1007/s11912-019-0833-z -
Journal of the National Comprehensive... Jan 2017Vulvar cancer is a rare malignancy with high curability in early-stage disease, yet poor outcomes for advanced-stage and recurrent disease. Surgical management is at the... (Review)
Review
Vulvar cancer is a rare malignancy with high curability in early-stage disease, yet poor outcomes for advanced-stage and recurrent disease. Surgical management is at the cornerstone of treatment for most vulvar cancers, and includes conservative and radical resection of the primary vulvar tumor and excision of local lymph nodes, which are major prognostic factors and drive adjuvant treatment. This review summarizes the surgical management of primary squamous cell carcinoma of the vulva, specifically initial treatment guidelines by stage, based on the 2017 NCCN Clinical Practice Guidelines in Oncology for Vulvar Cancer.
Topics: Carcinoma, Squamous Cell; Female; Humans; Inguinal Canal; Lymph Node Excision; Neoplasm Recurrence, Local; Neoplasm Staging; Pelvic Exenteration; Practice Guidelines as Topic; Radiotherapy, Adjuvant; Risk Factors; Sentinel Lymph Node Biopsy; Vulva; Vulvar Neoplasms
PubMed: 28040722
DOI: 10.6004/jnccn.2017.0009