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Dermatologic Surgery : Official... Feb 2021Vulvar cancers, although rare, are becoming an increasingly serious threat to women's health. Cancer of the vulva accounted for 0.3% of all new cancers in the United...
BACKGROUND
Vulvar cancers, although rare, are becoming an increasingly serious threat to women's health. Cancer of the vulva accounted for 0.3% of all new cancers in the United States in 2019, with 6,070 newly diagnosed cases. This review details the epidemiology, pathogenesis, diagnosis, staging, and treatment of vulvar malignancies.
OBJECTIVE
To review cancer entities of the vulva, including vulvar intraepithelial neoplasms, squamous cell carcinoma (SCC), malignant melanoma, basal cell carcinoma, neuroendocrine tumors, and adenocarcinomas.
MATERIALS AND METHODS
Literature review using PubMed search for articles related to cancer of the vulva.
RESULTS
Vulvar intraepithelial neoplasms represent premalignant precursors to SCC of the vulva. There are several different histopathologic subtypes of SCC, and treatment is dependent on characteristics of primary tumor and lymph node involvement. Melanoma is the second most common cancer to affect the vulva, and staging is based on tumor, node, and metastatic spread.
CONCLUSION
Vulvar malignancies are rare, and diagnosis is dependent on biopsy and pathologic evaluation. Treatment for vulvar malignancies depends on histopathologic diagnosis but ranges from wide local excision with or without lymph node biopsy or dissection to radiation therapy with chemo- or immunotherapy. Overall survival varies by diagnosis.
Topics: Adenocarcinoma; Antineoplastic Combined Chemotherapy Protocols; Biopsy; Carcinoma in Situ; Carcinoma, Basal Cell; Carcinoma, Squamous Cell; Chemotherapy, Adjuvant; Disease-Free Survival; Female; Humans; Lymph Nodes; Melanoma; Neoplasm Recurrence, Local; Neoplasm Staging; Radiotherapy, Adjuvant; Treatment Outcome; Vulva; Vulvar Neoplasms
PubMed: 32947298
DOI: 10.1097/DSS.0000000000002584 -
International Journal of Gynaecology... Oct 2021Vulvar cancer is an uncommon gynecological malignancy primarily affecting postmenopausal women. There is no specific screening and the most effective strategy to reduce...
Vulvar cancer is an uncommon gynecological malignancy primarily affecting postmenopausal women. There is no specific screening and the most effective strategy to reduce vulvar cancer incidence is the opportune treatment of predisposing and preneoplastic lesions associated with its development. While vulvar cancer may be asymptomatic, most women present with vulvar pruritus or pain, or have noticed a lump or ulcer. Therefore, any suspicious vulvar lesion should be biopsied to exclude invasion. Once established, the most common subtype is squamous cell carcinoma. Treatment of vulvar cancer depends primarily on histology and surgical staging. Treatment is predominantly surgical, particularly for squamous cell carcinoma, although concurrent chemoradiation is an effective alternative, particularly for advanced tumors. Management should be individualized and carried out by a multidisciplinary team in a cancer center experienced in the treatment of these tumors.
Topics: Carcinoma, Squamous Cell; Chemoradiotherapy; Female; Humans; Neoplasm Staging; Vulva; Vulvar Neoplasms
PubMed: 34669204
DOI: 10.1002/ijgo.13881 -
Journal of Gynecology Obstetrics and... Nov 2020Vulvar intraepithelial neoplasia (VIN) is classified into two entities: differentiated (dVIN) and vulvar high-grade squamous intraepithelial lesions (vH-SIL). dVIN is a... (Review)
Review
Vulvar intraepithelial neoplasia (VIN) is classified into two entities: differentiated (dVIN) and vulvar high-grade squamous intraepithelial lesions (vH-SIL). dVIN is a premalignant lesion that develops on an existing vulvar lesion such as lichen sclerosus, while vH-SIL is associated with HPV infection. The two entities differ in terms of pathophysiology, background, prognosis, and management. The incidence of VIN in young women is rising and recurrence is common, even after radical surgery, which can cause significant disfigurement. Alternative strategies include topical treatments, ablation, and a watch-and-wait approach. There is currently no consensus on how these lesions should be managed. We review the literature in this field.
Topics: Adult; Aged; Carcinoma in Situ; Female; Humans; Lichen Planus; Lichen Sclerosus et Atrophicus; Middle Aged; Papillomavirus Infections; Risk Factors; Vulvar Diseases; Vulvar Neoplasms
PubMed: 32417455
DOI: 10.1016/j.jogoh.2020.101801 -
Radiology and Oncology Sep 2020Background Vulvar cancer accounts for 3-5% of malignant diseases of the female genital tract. The Slovenian incidence rate is 5.5/100,000, which means 57 new cases per... (Review)
Review
Background Vulvar cancer accounts for 3-5% of malignant diseases of the female genital tract. The Slovenian incidence rate is 5.5/100,000, which means 57 new cases per year. The most common histological type (90%) is squamous cell carcinoma. Based on etiology, it can be classified into the first type which correlates with human papillomavirus (HPV) infection and the second type which is not associated with HPV. The most common and long-lasting symptom of vulvar cancer is pruritus. The preferred diagnostic procedure to confirm the diagnosis is a punch or incision biopsy. Surgery in combination with radiotherapy is the standard treatment for vulvar cancer. Sentinel lymph node biopsy with lymphoscintigraphy is now a standard part of surgical treatment. Chemotherapy is a palliative treatment option. Conclusions Vulvar cancer is a rare disease. Because of the pathogenesis, surgery and radiotherapy are the main treatment modalities. The sentinel node biopsy (SNB) represents a contemporary approach to the vulvar cancer treatment and significantly reduces morbidity. Improvements in treatment of vulvar cancer contributed to the decrease of mortality among Slovenian women.
Topics: Combined Modality Therapy; Female; Humans; Incidence; Lymphoscintigraphy; Sentinel Lymph Node Biopsy; Vulvar Neoplasms
PubMed: 32960779
DOI: 10.2478/raon-2020-0053 -
American Journal of Clinical Dermatology Sep 2021Ten percent of all women have pigmented vulvar lesions. Fortunately, most of these are benign but 1% of all melanomas in women affect the vulva. While the mortality rate... (Review)
Review
Ten percent of all women have pigmented vulvar lesions. Fortunately, most of these are benign but 1% of all melanomas in women affect the vulva. While the mortality rate of cutaneous melanoma has dropped by 7% annually during the last 5 years, the prognosis of vulvar melanoma remains dismal: the 5-year overall survival rate is 47% compared with 92% for cutaneous melanoma. The current evidence suggests that this likely results from a combination of delayed diagnosis and different tumor biology, treatment strategies, and treatment response. Although many landmark trials on checkpoint inhibitors included mucosal and vulvar melanomas, the results were often not reported separately. Post-hoc analyses indicate overall response rates between 19 and 37% for checkpoint inhibitors. A recently published retrospective study on vulvar melanomas suggests an objective response in 33.3% with a similar safety profile to cutaneous melanoma. Tyrosine kinase inhibitors may be considered in recurrent disease if a c-KIT mutation is present.
Topics: Diagnosis, Differential; Female; Humans; Melanoma; Skin Neoplasms; Vulva; Vulvar Neoplasms; Melanoma, Cutaneous Malignant
PubMed: 34125416
DOI: 10.1007/s40257-021-00614-7 -
International Journal of Gynecological... Jul 2023As part of its mission to improve the quality of care for women with gynecological cancers across Europe, the European Society of Gynaecological Oncology (ESGO) first...
BACKGROUND
As part of its mission to improve the quality of care for women with gynecological cancers across Europe, the European Society of Gynaecological Oncology (ESGO) first published in 2017 evidence-based guidelines for the management of patients with vulvar cancer.
OBJECTIVE
To update the ESGO guidelines based on the new evidence addressing the management of vulvar cancer and to cover new topics in order to provide comprehensive guidelines on all relevant issues of diagnosis and treatment of vulvar cancer.
METHODS
The ESGO Council nominated an international development group comprised of practicing clinicians who provide care to vulvar cancer patients and have demonstrated leadership through their expertize in clinical care and research, national and international engagement and profile as well as dedication to the topics addressed to serve on the expert panel (18 experts across Europe). To ensure that the statements were evidence-based, new data identified from a systematic search were reviewed and critically appraised. In the absence of any clear scientific evidence, judgment was based on the professional experience and consensus of the international development group. Prior to publication, the guidelines were reviewed by 206 international practitioners in cancer care delivery and patient representatives.
RESULTS
The updated guidelines cover comprehensively diagnosis and referral, staging, pathology, pre-operative investigations, surgical management (local treatment, groin treatment, sentinel lymph node procedure, reconstructive surgery), (chemo)radiotherapy, systemic treatment, treatment of recurrent disease (vulvar, inguinal, pelvic, and distant recurrences), and follow-up. Management algorithms are also defined.
Topics: Female; Humans; Europe; Gynecology; Plastic Surgery Procedures; Vulvar Neoplasms
PubMed: 37369376
DOI: 10.1136/ijgc-2023-004486 -
Journal of Lower Genital Tract Disease Apr 2022Metastatic neoplasms to the vulva are rare and can pose a diagnostic dilemma. As identification of the primary site can influence patient treatment and prognosis,... (Review)
Review
INTRODUCTION
Metastatic neoplasms to the vulva are rare and can pose a diagnostic dilemma. As identification of the primary site can influence patient treatment and prognosis, correct diagnosis is important.
METHODS
PubMed was searched for applicable publications using the terms vulva, vulvar neoplasms, metastasis, and vulvar metastasis.
RESULTS
Most neoplasms metastatic to the vulva originate from other genital sources; however, extragenital primary neoplasms can also metastasize to the vulva. Vulvar metastases often occur in the setting of widespread disease.
CONCLUSIONS
It is important to consider biopsy for appropriate histologic and immunohistochemical studies, as well as consider patient history to establish the primary site of metastatic lesions to the vulva, allowing optimal therapy.
Topics: Biopsy; Female; Humans; Prognosis; Vulva; Vulvar Neoplasms
PubMed: 35256567
DOI: 10.1097/LGT.0000000000000660 -
Urology Jul 2022Vulvar disease is common, and urologists are often the first providers to see patients with a vulvar skin condition. Primary vulvar dermatoses can be localized to the... (Review)
Review
Vulvar disease is common, and urologists are often the first providers to see patients with a vulvar skin condition. Primary vulvar dermatoses can be localized to the anogenital area or a manifestation of more diffuse cutaneous disease. Additionally, secondary dermatoses can develop from exogenous agents and inflammatory vaginitis. Vulvar conditions are challenging to diagnose due to location and different types of skin and mucosal epithelium involved. Herein, we provide an overview of noninfectious inflammatory vulvar dermatoses (Part I) and benign and malignant vulvar neoplasms (Part II), grouped by morphologic findings. We include diagnostic evaluation, workup, and management of these conditions.
Topics: Female; Humans; Skin; Skin Neoplasms; Vulva; Vulvar Diseases; Vulvar Neoplasms
PubMed: 35218865
DOI: 10.1016/j.urology.2022.02.007 -
Urology Jul 2022Vulvar disease is common, and urologists are often the first providers to see patients with a vulvar skin condition. Primary vulvar dermatoses can be localized to the... (Review)
Review
Vulvar disease is common, and urologists are often the first providers to see patients with a vulvar skin condition. Primary vulvar dermatoses can be localized to the anogenital area or a manifestation of more diffuse cutaneous disease. Additionally, secondary dermatoses can develop from exogenous agents and inflammatory vaginitis. Vulvar conditions are challenging to diagnose due to location and different types of skin and mucosal epithelium involved. Herein, we provide an overview of noninfectious inflammatory vulvar dermatoses (part I) and benign and malignant vulvar neoplasms (part II), grouped by morphologic findings. We include diagnostic evaluation, workup, and management of these conditions.
Topics: Female; Humans; Skin Diseases; Vulvar Diseases; Vulvar Neoplasms
PubMed: 35413374
DOI: 10.1016/j.urology.2022.03.025 -
Radiologic Clinics of North America Jul 2023Vaginal and vulvar malignancies are rare gynecologic malignancies but can be associated with high morbidity and mortality if undiagnosed and untreated. Advanced imaging... (Review)
Review
Vaginal and vulvar malignancies are rare gynecologic malignancies but can be associated with high morbidity and mortality if undiagnosed and untreated. Advanced imaging modalities such as MRI enable assessment of the local extent of disease and evaluation for regional or distant spread. Accurate identification and description of the primary lesion and sites of involvement as well as detection and localization of suspicious lymph nodes are critical in guiding appropriate management. Additionally, radiologists should be aware of potential mimickers on imaging and the differential diagnoses for vaginal and vulvar lesions.
Topics: Female; Humans; Vulvar Neoplasms; Lymph Nodes; Magnetic Resonance Imaging
PubMed: 37169430
DOI: 10.1016/j.rcl.2023.02.010