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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 -
Pathology Oncology Research : POR Jan 2020Rapid advance in oncology leads to increasing survival of oncologic patients. More and more of them live long enough to reach either the natural age of menopause or, as... (Review)
Review
Rapid advance in oncology leads to increasing survival of oncologic patients. More and more of them live long enough to reach either the natural age of menopause or, as a side effect of their oncotherapy, experience the cessation of gonadal function, leading to premature ovarian insufficiency, with disturbing vasomotor symtoms and long-term negative cardiovascular and skeletal effects. Thus, an ever increasing number of cancer survivors search endocrinologic help in the form of hormone replacement therapy (HRT). The misinterpretation of the WHI (Women's Health Initiative) Study has lead to an irrational fear of female hormone replacement, both by the general population and medical professionals. It has seemed the logical and safe conclusion to many physicians to avoid HRT, supposing that this attitude definitely causes no harm, whereas the decision of prescribing estrogen alone or with progestins might bear oncologic and thromboembolic risks and may even lead to litigation in case of a potentially related complication. However, it was known even before the WHI results that premature menopause and hypogonadism decreases the life expectancy of women by years through its skeletal and cardiovascular effects, and this negative effect correlates with the length of the hypoestrogenaemic period. Therefore, the denial of HRT also needs to be supported by evidence and should be weighed againts the risks of HRT. Yet, the oncologic risk of HRT is extremely difficult to assess. In this work we review the latest evidence from in vitro experiments to clinical studies, regarding HRT in survivors of gynecologic and non-gynecologic cancers. Based on our literature research, we group tumours regarding the oncologic risk of properly chosen female hormone replacement therapy in cancer survivors as follows: 'HRT is advanageous' (e.g. endometrial cancer type I, cervical adenocarcinoma, haematologic malignancies, local cutaneous malignant melanoma, colorectal cancer, hepatocellular cancer); 'HRT is neutral' (e.g. BRCA 1/2 mutation carriers without cancer, endometrial cancer type II, uterinal carcinosarcoma and adenosarcoma, certain types of ovarian cancer, cervical, vaginal and vulvar squamous cell carcinoma, prolactinoma, kidney cancer, pancreatic cancer, thyroid cancer); 'HRT is relatively contraindicated' for various reasons (e.g. leiomyosarcoma, certain types of ovarian tumours, brain tumours, advanced metastatic malignant melanoma, lung cancer, gastric cancer, bladder cancer); 'HRT is diasadvantageous and thus contraindicated' (e.g. breast cancer, endometrial stroma sarcoma, meningioma, glioma, hormone receptor positive gastric and bladder cancer).
Topics: Breast Neoplasms; Cancer Survivors; Estrogens; Female; Genital Neoplasms, Female; Hormone Replacement Therapy; Humans; Neoplasms; Neoplasms, Second Primary; Progesterone
PubMed: 30617760
DOI: 10.1007/s12253-018-00569-x -
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 -
JAMA Dermatology Jan 2023
Topics: Female; Humans; Skin Neoplasms; Melanoma; Vulvar Neoplasms; Hyperpigmentation
PubMed: 36449308
DOI: 10.1001/jamadermatol.2022.4550 -
Seminars in Nuclear Medicine Jan 2020Well executed multicenter clinical trials often provide significant evidence and support for, or against, foundational aspects of clinical procedures perceived to... (Review)
Review
Well executed multicenter clinical trials often provide significant evidence and support for, or against, foundational aspects of clinical procedures perceived to improve clinical management of a medical condition. In this review, discussed are reports of multicenter clinical trials designed to investigate sentinel lymph node biopsy procedures in seven types of cancer: breast, melanoma, head and neck, gastric, colon, uterine, and vulvar-with focus on the most recent reports of the hypotheses, objectives, parameters, data, results, implications, and impacts of the included trials. Such trials generally enroll more subjects, in shorter time periods, than do single-center studies. Such studies generally also have greater diversities among investigator practitioners and investigative environments than do single-center studies. The greater number of subjects provides more power to statistical analyses performed in such studies. The more rapid accrual usually results in data being more consistently acquired. The diversities of practitioners and environments may produce results that are more conservative than might be obtained from more "focused" studies; however, diversities in a study often identify implicitly results that are more robust-that is results applicable by more practitioners and applicable in more environments.
Topics: Clinical Trials as Topic; Humans; Multicenter Studies as Topic; Neoplasms; Sentinel Lymph Node Biopsy
PubMed: 31843062
DOI: 10.1053/j.semnuclmed.2019.10.001 -
Cancers Oct 2022Malignant melanoma is a fatal disease that affects all skin sites. Among these, vulvar melanoma (VM) is a rare gynecological condition that accounts for 5% of all vulvar... (Review)
Review
Malignant melanoma is a fatal disease that affects all skin sites. Among these, vulvar melanoma (VM) is a rare gynecological condition that accounts for 5% of all vulvar neoplasms. VM primarily affects older Caucasian women and its relationship to sun exposure is undefined. Diagnosis is defined by biopsy but many clinical, dermatoscopic, and confocal microscopic features can guide doctors. The molecular profile is characterized by the KIT mutation, revealed by all of the technologies that are used (classical sequencing, next-generation sequencing, and immunohistochemical staining). BRAF and NRAS mutations are also common in VM. All of these mutations are possible therapeutic targets. Today, surgery remains the first treatment choice for primary VM. The role of neoadjuvant and adjuvant therapy is scarce and the treatment of relapses is widely debated.
PubMed: 36358637
DOI: 10.3390/cancers14215217 -
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 -
Journal Der Deutschen Dermatologischen... Dec 2019Vulvar cancer represents the fourth most common gynecologic malignancy and is often encountered by the general Dermatologist or Gynecologist. Dermatooncologists and... (Review)
Review
Vulvar cancer represents the fourth most common gynecologic malignancy and is often encountered by the general Dermatologist or Gynecologist. Dermatooncologists and Gynecologic Oncologists share expertise in this field and the diagnosis and treatment should ideally be interdisciplinary. All subtypes are typically seen in the later decades of life, although all histologic subtypes have been described in women younger than 30 years. The diagnosis is often delayed. Exact mapping of biopsies is of high importance, as the location and distance from the midline guides the surgical approach depending on the underlying histology. Squamous cell carcinoma accounts for more than 76 % of vulvar cancer with vulvar intraepithelial neoplasia being an important precursor. Basal cell carcinoma is the second most common vulvar malignancy. Melanoma accounts for 5.7 % of vulvar cancer and has a worse prognosis compared to cutaneous melanoma. Most of the trials on checkpoint inhibitors and targeted therapy have not excluded patients with vulvar melanoma and the preliminary evidence is reviewed in the manuscript. Surgery remains the primary treatment modality of locally resectable vulvar cancer. In view of the rarity, the procedure should be performed in dedicated cancer centers to achieve optimal disease control and maintain continence and sexual function whenever possible.
Topics: Adenocarcinoma; Biopsy; Carcinoma in Situ; Carcinoma, Basal Cell; Carcinoma, Squamous Cell; Carcinoma, Verrucous; Female; Humans; Melanoma; Vulva; Vulvar Neoplasms
PubMed: 31829526
DOI: 10.1111/ddg.13995