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Ultrasound in Obstetrics & Gynecology :... Jun 2021In centers with access to high-end ultrasound machines and expert sonologists, ultrasound is used to detect metastases in regional lymph nodes from melanoma, breast...
In centers with access to high-end ultrasound machines and expert sonologists, ultrasound is used to detect metastases in regional lymph nodes from melanoma, breast cancer and vulvar cancer. There is, as yet, no international consensus on ultrasound assessment of lymph nodes in any disease or medical condition. The lack of standardized ultrasound nomenclature to describe lymph nodes makes it difficult to compare results from different ultrasound studies and to find reliable ultrasound features for distinguishing non-infiltrated lymph nodes from lymph nodes infiltrated by cancer or lymphoma cells. The Vulvar International Tumor Analysis (VITA) collaborative group consists of gynecologists, gynecologic oncologists and radiologists with expertise in gynecologic cancer, particularly in the ultrasound staging and treatment of vulvar cancer. The work herein is a consensus opinion on terms, definitions and measurements which may be used to describe inguinal lymph nodes on grayscale and color/power Doppler ultrasound. The proposed nomenclature need not be limited to the description of inguinal lymph nodes as part of vulvar cancer staging; it can be used to describe peripheral lymph nodes in general, as well as non-peripheral (i.e. parietal or visceral) lymph nodes if these can be visualized clearly. The association between the ultrasound features described here and histopathological diagnosis has not yet been established. VITA terms and definitions lay the foundations for prospective studies aiming to identify ultrasound features typical of metastases and other pathology in lymph nodes and studies to elucidate the role of ultrasound in staging of vulvar and other malignancies. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.
Topics: Female; Gynecology; Humans; Lymphatic Metastasis; Practice Guidelines as Topic; Societies, Medical; Ultrasonography; Vulvar Neoplasms
PubMed: 34077608
DOI: 10.1002/uog.23617 -
Human Pathology Oct 2020Vulvar malignant melanoma (VMM), although uncommon, comprises 5-10% of all vulvar malignancies. Local control is notoriously poor in VMM with recurrence rates of 30-50%... (Comparative Study)
Comparative Study
Vulvar malignant melanoma (VMM), although uncommon, comprises 5-10% of all vulvar malignancies. Local control is notoriously poor in VMM with recurrence rates of 30-50% compared with approximately 3% in cutaneous melanomas. We studied clinicopathologic features of 37 women with VMM, after reviewing three decades of clinical follow-up data in our institutional databases. Most patients were Caucasian (n = 35) with an average age at diagnosis of 60.6 years (range 23-83). The most common subtype was mucosal lentiginous melanoma (n = 25). We compared Kaplan-Meier survival curves of 31 patients defined by clinical and microscopic attributes using exact log-rank tests. Younger patients at diagnosis (23-64 years), those with thin melanomas (≤1 mm), and those with Clark's level II or III tumors had better 5-year survival rates than older patients (65-83 years) and those with thick melanomas (>1 mm) and those with Clark's level IV or V (P ≤ 0.05), respectively, by exact log-rank test. Local recurrence of melanoma occurred in 15 patients. Nine patients (24%) had eventual urethral involvement by malignant melanoma, and this feature was associated with significantly shorter survival (P = 0.036). Patients with urethral involvement had shorter median time to death and worse 5-year survival rates. Given that spread to the urethra is common in VMM and urethral recurrence is also associated with mortality, pathology excision specimens should be carefully reviewed with attention to urethral involvement as a potentially important prognostic factor.
Topics: Adult; Age Factors; Aged; Aged, 80 and over; Databases, Factual; Female; Humans; Melanoma; Middle Aged; Neoplasm Invasiveness; Neoplasm Recurrence, Local; Neoplasm Staging; Philadelphia; Risk Assessment; Risk Factors; Time Factors; Treatment Outcome; Urethra; Urethral Neoplasms; Vulvar Neoplasms; Young Adult
PubMed: 32702401
DOI: 10.1016/j.humpath.2020.07.017 -
Medicine Sep 2022Vulvar melanoma is a rare and aggressive tumor with a high risk of local recurrence and distant metastasis. The prognosis is poor with a 5-year overall survival rate of... (Review)
Review
RATIONALE
Vulvar melanoma is a rare and aggressive tumor with a high risk of local recurrence and distant metastasis. The prognosis is poor with a 5-year overall survival rate of only 46.6%. Management of vulvar melanoma remains a clinical challenge. Recent evidences have shown that immune checkpoint inhibitors are effective in the treatment of vulvar melanoma.
PATIENT CONCERNS AND DIAGNOSES
A 63-year-old woman with vulvar malignant melanoma suffered inguinal lymph node metastasis after vulvectomy and chemotherapy. She underwent inguinal lymph node dissection and inguinal radiotherapy. The tumor progressed again and she received immunotherapy.
INTERVENTIONS
The tumor progressed again, and she was admitted to our hospital and received toripalimab combined with apatinib and abraxane.
OUTCOMES
After 6 cycles of immunotherapy, the efficacy achieved partial remission. And with toripalimab as maintenance therapy, the patient achieved durable antitumor efficacy and good safety.
LESSONS
In this rare case, the patient with metastatic vulvar malignant melanoma had durable antitumor efficacy and good safety when receiving toripalimab.
Topics: Antibodies, Monoclonal, Humanized; Female; Humans; Melanoma; Middle Aged; Neoplasms, Second Primary; Skin Neoplasms; Vulvar Neoplasms
PubMed: 36086787
DOI: 10.1097/MD.0000000000030239 -
Modern Pathology : An Official Journal... Jan 2020Female genital melanomas are rare. At diagnosis, most affected patients have advanced disease. Surgery remains the primary treatment, and adjuvant therapy is largely...
Female genital melanomas are rare. At diagnosis, most affected patients have advanced disease. Surgery remains the primary treatment, and adjuvant therapy is largely ineffective. Recently, immune checkpoints and the mitogen-activated protein kinase pathway have been explored as treatment targets. However, evaluation of these biomarkers in genital melanomas is limited. We evaluated the clinicopathological features of 20 vulvar, 32 vaginal, and three cervical melanomas and assessed programmed cell death ligand 1 (PD-L1) expression, CD8 tumor-infiltrating lymphocyte density, mismatch repair proteins, VE1 immunohistochemistry, and KIT and BRAF mutations. The median age of the patients was 66 years, and median tumor sizes were 25, 30, and 20 mm for vulvar, vaginal, and cervical tumors, respectively. Mean mitotic figures were 18, 19, and 30 per mm. Thirty-seven patients (67%) had operable tumors. After a median follow-up of 15 months, only nine patients (16%) were alive. Eight of the nine survivors did not have lymph node metastasis. Using 5% as the threshold, PD-L1 expression was observed in 55%, 50%, and 33% of vulvar, vaginal, and cervical tumors, respectively, when the Roche SP263 antibody was used and 20%, 53%, and 0%, respectively, when the Dako 28-8 antibody was used. The median CD8 tumor-infiltrating lymphocyte density was significantly higher in vulvar/vaginal than cervical melanomas and correlated with PD-L1 expression. No cases exhibited loss of mismatch repair proteins. Five cases harbored KIT mutations, three of which were hotspots. BRAF V600E mutation was not detected. Univariable analysis showed that tumor size greater than or equal to 33 mm, mitotic figures of greater than or equal to 10 per mm, lymph node metastasis, and low CD8+ tumor-infiltrating lymphocyte density were adverse prognostic factors. Thus, patients with genital melanomas have a poor prognosis, and evaluation of multiple biomarkers is necessary to identify patients who may benefit from immunotherapy or targeted therapy.
Topics: Adult; Aged; Aged, 80 and over; Biomarkers, Tumor; Female; Genital Neoplasms, Female; Humans; Lymphocytes, Tumor-Infiltrating; Melanoma; Middle Aged; Tumor Microenvironment
PubMed: 31383965
DOI: 10.1038/s41379-019-0345-2 -
Cureus Jun 2020Introduction Malignant melanoma, which arises from melanocytes or pigment cells, is one of the most common forms of epithelial cancer. Cutaneous and noncutaneous...
Introduction Malignant melanoma, which arises from melanocytes or pigment cells, is one of the most common forms of epithelial cancer. Cutaneous and noncutaneous melanomas differ clinically and genetically. Mucosal melanomas are rare. In the female genital tract, the most frequent location of melanoma is the vulva, whereas the vagina is seldom affected. The occult nature of their anatomical location contributes to the late presentation and late diagnosis of vaginal melanoma, resulting in an exceedingly poor patient prognosis. The present study describes the incidence, symptoms, management, and prognosis of women in Pakistan with malignant melanoma of the vulva, vagina, and cervix. Materials and methods The Hospital Information System of Shaukat Khanam Memorial Cancer Hospital and Research Center was searched electronically to identify patients diagnosed with malignant melanoma from January 1995 to December 2017. Patients with cutaneous malignant melanoma, multiple primary tumors, and metastases to the female genital tract from primary tumors located elsewhere were excluded. All included patients had been diagnosed with primary malignant melanoma of the female genital tract. Results The search of medical records identified 271 patients with malignant melanoma, of whom 13 had primary malignant melanomas of the female genital tract. Of these 13 patients, nine, three, and one had primary vaginal, vulvar, and cervical melanomas, respectively. Median age at presentation was 60 years (range, 30-70 years), with 10 patients being post-menopausal. The most common presentations were per-vaginal bleeding and per-vaginal discharge (five patients each). The mean duration of symptoms was 7.46 months. Seven patients underwent wide local excision. Six patients had nodular type malignant melanoma, two had superficial spreading type, and five were unclassified. Nine patients had pathological T4 disease, and two had pathological T3. Mean Breslow depth was 5.4 millimeters (mm), with 10 patients having tumor depth >4 mm. Eight patients were positive for the microscopic involvement of margins. The mean time to recurrence was 11.8 months (range, 1-24 months), and the mean time to metastasis was 17.6 months (range, 2-44 months). The median survival after surgery was 25 months (range, 2-75 months). Conclusion This study is the first to report the incidence, symptoms, management, and prognosis of patients in Pakistan with malignant melanoma of the female genital tract. Meta-analyses and prospective multicenter studies are needed.
PubMed: 32642388
DOI: 10.7759/cureus.8484 -
International Journal of Women's... Mar 2024The incidence of vulvar carcinoma increases with age, though elderly women receive less aggressive cancer therapies and fewer strategies aimed at cancer prevention....
The incidence of vulvar carcinoma increases with age, though elderly women receive less aggressive cancer therapies and fewer strategies aimed at cancer prevention. Furthermore, elderly women dual enrolled in Medicaid-Medicare experience poor survival rates for vulvar carcinoma. Herein, we provide recommendations for the prevention of and guidelines for the multidisciplinary care of vulvar carcinoma. Prevention of vulvar carcinoma can be categorized into primary, secondary, and tertiary prevention. Primary prevention consists of vaccination, secondary prevention consists of screening, and tertiary prevention is aimed at the management of premalignant and early-stage lesions.
PubMed: 38179153
DOI: 10.1097/JW9.0000000000000127 -
Gynecologic Oncology Reports Nov 2021The primary treatment of both in situ and invasive vulvar melanoma is wide local excision of the primary neoplasm. However, this can be a surgical challenge for size,...
The primary treatment of both in situ and invasive vulvar melanoma is wide local excision of the primary neoplasm. However, this can be a surgical challenge for size, multifocal presentation with proximity to urethra or anus and tendency for local recurrence. The data on adjuvant therapy for vulvar MIS is very limited. A 69-year-old patient with melanoma of the vulva underwent a simple vulvectomy with positive margins in peri-clitoral area, followed by modified radical vulvectomy and bilateral inguinofemoral sentinel lymph node dissection with negative margins. She was later diagnosed with MIS of the vulva on different locations and had multiple wide local excisions over several years. One lesion was close to the urethra and a complete excision was difficult. Topical imiquimod × 16 weeks (5% cream) was given. The regimen was augmented from 3 to 5 times weekly. Complete resolution was found at 16 weeks and patient was disease free for 4 years. Recently however, a vaginal melanoma was detected. Imiquimod appeared to be beneficial in the treatment of melanoma in situ of the vulva/ vagina when surgical options were not feasible producing local control of disease with the remaining risk for local and distant metastasis. Metastasis can appear years later, therefore long-term follow-up of patients treated with topical imiquimod is needed.
PubMed: 34926757
DOI: 10.1016/j.gore.2021.100875 -
Case Reports in Oncology 2021Vulvar malignant melanoma is the second most common subtype of vulvar cancer, accounting for 5-10% of all vulvar cancers. The prognosis is still very poor, although some...
Vulvar malignant melanoma is the second most common subtype of vulvar cancer, accounting for 5-10% of all vulvar cancers. The prognosis is still very poor, although some advances have been achieved in the last years. One of the most significant changes in its management has been the development of less invasive surgical techniques that diminish the risk of postoperative morbidity and long-lasting sequelae. In this article, we review the surgical management of the pathology, based on the comment of 3 cases with vulvar melanoma treated at our institution.
PubMed: 34413746
DOI: 10.1159/000517820 -
International Journal of Gynecological... 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; Cidofovir; Colposcopy; Female; Genital Neoplasms, Female; Humans; Imiquimod; Melanoma; Paget Disease, Extramammary; Pregnancy; Skin Neoplasms; Vulvar Neoplasms; Melanoma, Cutaneous Malignant
PubMed: 35728950
DOI: 10.1136/ijgc-2021-003262 -
Taiwanese Journal of Obstetrics &... Jan 2023To demonstrate a successful surgical treatment and reconstruction in a case of malignant mucosal vulvar melanoma.
OBJECTIVE
To demonstrate a successful surgical treatment and reconstruction in a case of malignant mucosal vulvar melanoma.
CASE REPORT
A 52-year-old woman had stage II bulky malignant mucosal vulvar melanoma and received wide surgical excision with partial vulvectomy. She underwent 2-steps reconstructive vulvoplasty and vaginoplasty with skin grafting 1 year after initial surgical treatment. There was no evidence of recurrence after 3 years of follow-up.
CONCLUSION
Vulvar melanoma is a rare malignant neoplasm. Wide local excision with reconstruction can relieve pelvic discomfort and restore local function after the surgery.
Topics: Female; Humans; Middle Aged; Vulvar Neoplasms; Vulva; Melanoma; Skin Neoplasms; Neoplasm Recurrence, Local; Melanoma, Cutaneous Malignant
PubMed: 36720534
DOI: 10.1016/j.tjog.2022.05.019