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Journal of Lower Genital Tract Disease Jul 2020The aims of the study were to evaluate clinicopathologic features, management, and outcomes in vulval melanoma and to review the literature. (Review)
Review
OBJECTIVES
The aims of the study were to evaluate clinicopathologic features, management, and outcomes in vulval melanoma and to review the literature.
MATERIALS AND METHODS
Data were collected retrospectively on patients with vulval melanoma from 2001 to 2017 in 5 gynecological oncology cancer centers (Bristol, Taunton, Truro, Plymouth, and Cheltenham). SPSS software was used for univariate and multivariate statistical analysis. Disease-specific median survival was calculated using Kaplan-Meier curves.
RESULTS
Forty-four patients with vulval melanoma were included, with a median age of 71 years. Forty-three of 44 had wide local excision with full inguinal lymphadenectomy if abnormal lymph nodes. Seven patients had sentinel lymph nodes. However, 2 patients with negative sentinel lymph nodes had distant recurrences within 16 months.On univariate analysis, presence of ulceration (p = .012), perineural invasion (p = .03), and area of lesion (p = .016) were associated with risk of recurrence but only presence of microsatellites (p = .01) was associated with risk of death.There were 31 deaths (70%): 29 (94%) of 31 from melanoma and 28 (64%) of 44 recurrences: 17 local (10 groin, 7 vulval) and 9 distant. Overall median survival was 32.5 months (95% CI, 17.8-46.5 months) and median recurrence-free survival 12.6 months (95% CI, 7.7-17.4 months).
CONCLUSIONS
This retrospective multicenter study highlights the high recurrence rate and poor prognosis of vulval melanoma. Lymph node surgery did not make any difference to recurrence-free survival or overall survival. The presence of microsatellites was associated with a statistically increased risk of death.
Topics: Aged; Aged, 80 and over; Female; Humans; Male; Melanoma; Risk Assessment; Skin Neoplasms; Vulvar Neoplasms
PubMed: 32205765
DOI: 10.1097/LGT.0000000000000521 -
Japanese Journal of Clinical Oncology Dec 2020Primary vulvar melanoma was an aggressive and poorly understood gynecological tumor. Unlike cutaneous melanoma, the incidence of vulvar melanoma was low but the survival...
BACKGROUND
Primary vulvar melanoma was an aggressive and poorly understood gynecological tumor. Unlike cutaneous melanoma, the incidence of vulvar melanoma was low but the survival was poor. There were no standard staging system and no census on treatment strategies of vulvar melanoma. Therefore, we aimed to conduct and validate a comprehensive prognostic model for predicting overall survival of vulvar melanoma and provide guidance for clinical management.
METHODS
Patients diagnosed with vulvar melanoma between year 2004 and 2015 from Surveillance, Epidemiology, and End Result (SEER) database were randomized to training cohort and validation cohort. Multivariate survival analysis was performed to screen for independent factors of survival. A nomogram was established to predict overall survival of vulvar melanoma. Receiver operating characteristic curve and calibration plot were performed to verify the discrimination and accuracy of the model. The decision curve analysis was performed to verify the clinical applicability of the model.
RESULTS
Total 737 patients with vulvar melanoma were randomized to the training cohort (n = 517) and the validation cohort (n = 220). Nomogram including age, race, tumor site, depth of tumor invasion, lymph node status, distant metastasis, tumor size, surgery, chemotherapy and radiotherapy was established and validated. The c-indexes for SEER stage, American Joint Committee on Cancer stage and this model were 0.561, 0.635 and 0.826, respectively. The high-risk group scored by this model had worse survival than the low-risk group (P < 0.001). Decision curve analysis revealed this model was superior in predicting survival.
CONCLUSIONS
Our model was deemed to be a useful tool for predicting overall survival of vulvar melanoma with good discrimination and clinical applicability. We hoped this model would assist gynecologists in clinical decision and management of patients diagnosed with vulvar melanoma.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Cohort Studies; Female; Humans; Melanoma; Middle Aged; Multivariate Analysis; Neoplasm Staging; Nomograms; Prognosis; ROC Curve; Reproducibility of Results; Risk Factors; SEER Program; Survival Analysis; Vulvar Neoplasms; Young Adult
PubMed: 32776099
DOI: 10.1093/jjco/hyaa137 -
JAMA Dermatology Nov 2020Vulvar melanosis is a common pigmentary change that accounts for most pigmented vulvar lesions. It presents as single or multiple asymptomatic macules or patches of...
IMPORTANCE
Vulvar melanosis is a common pigmentary change that accounts for most pigmented vulvar lesions. It presents as single or multiple asymptomatic macules or patches of varying size and color that may be asymmetric with poorly defined borders. The differential diagnosis of melanocytic lesions includes melanoma, which creates anxiety for patients and the physicians who diagnose the condition and treat the patients.
OBJECTIVE
To evaluate the clinical and dermoscopic features of vulvar melanosis and their changes over time.
DESIGN, SETTING, AND PARTICIPANTS
In this cohort study, patients with vulvar melanosis were recruited and followed up in the Department of Dermatology, University of Florence, Florence, Italy, between January 1, 1998, and June 30, 2019. Data on patient characteristics and on both the clinical and dermoscopic features of the vulvar lesions were collected. Each lesion was photographed clinically and dermoscopically at initial evaluation and at annual follow-up visits.
MAIN OUTCOMES AND MEASURES
The clinical, dermoscopic, and histopathologic features of vulvar melanosis and their changes over time.
RESULTS
This cohort study included 129 women (mean age at diagnosis, 46 years [range, 19-83 years]) with vulvar melanosis. A total of 87 patients (67%) with vulvar melanotic lesions were premenopausal, and 84 patients (65%) had received some type of hormone therapy. The most frequent location for vulvar melanosis was the labia minora (55 [43%]), followed by the labia majora (33 [26%]). In 39 of 129 cases (30%), the lesions increased in size and changed color after initial evaluation but ultimately stabilized. No malignant evolution was documented in any patient during a median follow-up of 13 years (range, 5-20 years).
CONCLUSIONS AND RELEVANCE
This study suggests that vulvar melanosis was a benign entity, and changes in lesions over time did not signify malignant transformation. An association between hormonal status and vulvar melanosis may be hypothesized.
Topics: Adult; Aged; Aged, 80 and over; Biopsy; Color; Dermoscopy; Diagnosis, Differential; Disease Progression; Female; Follow-Up Studies; Hormone Replacement Therapy; Humans; Italy; Melanoma; Melanosis; Middle Aged; Mucous Membrane; Photography; Retrospective Studies; Vulva; Vulvar Diseases; Vulvar Neoplasms; Young Adult
PubMed: 32785609
DOI: 10.1001/jamadermatol.2020.2528 -
Minerva Ginecologica Dec 2020Vulvar and vaginal melanomas are rare cancers of the female genital tract and account for 1% to 3% of all melanomas diagnosed in women. Due to the rarity of the disease,... (Review)
Review
INTRODUCTION
Vulvar and vaginal melanomas are rare cancers of the female genital tract and account for 1% to 3% of all melanomas diagnosed in women. Due to the rarity of the disease, few data are available on the clinical and pathologic features of these cancers. Furthermore, treatment options are generally based on extrapolations of the information available for the more common cutaneous counterparts. Surgery represents the mainstay of treatment for lower genital tract melanoma. Moreover, the role of sentinel lymph node (SLN) assessment is controversial because no prospective data are available.
EVIDENCE ACQUISITION
Data were collected from Medline, Embase, Web of Sciences and Scopus databases. On July 10, 2020, we used the search comprising the terms "vulvar melanoma," "genital melanoma" and "vulvovaginal melanoma" including only studies in which SLN biopsy was performed.
EVIDENCE SYNTHESIS
Ten retrospective studies have been found. No randomized trials have been reported. The studies included 132 patients while only 63 (47%) undergone SLN. 99mTC with or without blue dye followed by ultrastaging was highly accurate and is currently the gold standard. Mean detection rate was 98.3%. No clear evidence supported the execution of back lymphadenectomy (after SLN mapping), in fact, extrapolating data from cutaneous melanomas of other sites, completion of lymphadenectomy does not confer a melanoma-specific survival advantage.
CONCLUSIONS
Although the small amount of available data, sentinel lymph node procedure is feasible and capable of identifying patients who have occult lymph node metastases. However, the potential role of the sentinel lymph node procedure as an alternative method of lymph node staging in patients with vulvar or vaginal melanoma needs further investigation.
Topics: Female; Humans; Lymph Node Excision; Melanoma; Radiopharmaceuticals; Retrospective Studies; Sentinel Lymph Node; Sentinel Lymph Node Biopsy; Technetium; Vaginal Neoplasms; Vulvar Neoplasms
PubMed: 32744452
DOI: 10.23736/S0026-4784.20.04628-6 -
Gynecologic Oncology Apr 2021To identify clinicopathological characteristics, treatment patterns, clinical outcomes and prognostic factors in patients with vulvar melanoma (VM).
OBJECTIVE
To identify clinicopathological characteristics, treatment patterns, clinical outcomes and prognostic factors in patients with vulvar melanoma (VM).
MATERIALS & METHODS
This retrospective multicentre cohort study included 198 women with VM treated in eight cancer centres in the Netherlands and UK between 1990 and 2017. Clinicopathological features, treatment, recurrence, and survival data were collected. Overall and recurrence-free survival was estimated with the Kaplan-Meier method. Prognostic parameters were identified with multivariable Cox regression analysis.
RESULTS
The majority of patients (75.8%) had localized disease at diagnosis. VM was significantly associated with high-risk clinicopathological features, including age, tumour thickness, ulceration, positive resection margins and involved lymph nodes. Overall survival was 48% (95% CI 40-56%) and 31% (95% CI 23-39%) after 2 and 5 years respectively and did not improve in patients diagnosed after 2010 compared to patients diagnosed between 1990 and 2009. Recurrence occurred in 66.7% of patients, of which two-third was non-local. In multivariable analysis, age and tumour size were independent prognostic factors for worse survival. Prognostic factors for recurrence were tumour size and tumour type. Only the minority of patients were treated with immuno- or targeted therapy.
CONCLUSION
Our results show that even clinically early-stage VM is an aggressive disease associated with poor clinical outcome due to distant metastases. Further investigation into the genomic landscape and the immune microenvironment in VM may pave the way to novel therapies to improve clinical outcomes in these aggressive tumours. Clinical trials with immunotherapy or targeted therapy in patients with high-risk, advanced or metastatic disease are highly needed.
Topics: Aged; Cohort Studies; Disease-Free Survival; Female; Humans; Immune Checkpoint Inhibitors; Melanoma; Middle Aged; Molecular Targeted Therapy; Neoplasm Staging; Netherlands; Prognosis; Retrospective Studies; Survival Rate; United Kingdom; Vulvar Neoplasms
PubMed: 33514483
DOI: 10.1016/j.ygyno.2021.01.018 -
Archives of Gynecology and Obstetrics Oct 2021
Topics: Diagnosis, Differential; Female; Genitalia; Humans; Melanosis; Vitiligo; Vulvar Diseases
PubMed: 33025087
DOI: 10.1007/s00404-020-05818-6 -
Pediatric Dermatology 2023Herein we describe the case of a Black adolescent who was found to have widely metastatic melanoma originating from a primary vulvar lesion. The lesion presented as a...
Herein we describe the case of a Black adolescent who was found to have widely metastatic melanoma originating from a primary vulvar lesion. The lesion presented as a pink, vegetative nodule of the clitoral hood which grew in size over several years and was confirmed to be melanoma on shave biopsy. This patient's amelanotic presentation in conjunction with the rare incidence of vulvar melanoma contributed to the delay in diagnosis. This case exemplifies the challenge of early recognition of potentially malignant vulvar lesions for primary care providers in adolescents.
Topics: Female; Adolescent; Humans; Melanoma, Amelanotic; Vulvar Neoplasms; Skin Neoplasms; Vulva; Vulvar Diseases
PubMed: 36949654
DOI: 10.1111/pde.15296 -
Journal of Gynecology Obstetrics and... May 2021Mucosal melanomas (MM) of the female genital tract are rare a. We aimed to study the prognostic factors of vulvar and vaginal locations of MM.
INTRODUCTION
Mucosal melanomas (MM) of the female genital tract are rare a. We aimed to study the prognostic factors of vulvar and vaginal locations of MM.
MATERIAL AND METHOD
A multicenter, retrospective cohort study conducted between 01/01/2000 and 01/06/2019.
RESULT
Of the 33 patients included 25 (75.8 %) had vulvar (VuM) and eight (24.2 %) vaginal melanomas (VaM). VaMs were deeper: median Breslow index: 17.5mm [3.5-22] versus 4.3mm [0.35-18] (p=0.013). Average follow-up was 24.0±59.8 months. Twenty-six patients (78.8 %) experienced recurrence. Disease-free survival was 52.9 % at 1year (64.7 % for VuM and 14.3 % for VaM) and 8.4 % at 3 years (11 % for VuM and 0% for VaM) (p=0.002). Median time to the first recurrence was 9.01 months [CI95 %: 2.07-56.71]. VaM recurred earlier than VuM (3.12 months [CI95 %: 2.07-12.49] versus 17.72 [CI95 %: 3.58-56.71], p=0.011). VaM had a higher risk of recurrence (HR=5.64 [CI95 %: 2.01-15.82], p=0.001) in multivariate analysis. Overall survival was 88.5 % at 1year (100 % for VuM and 50 % for VaM), and 59.4 % at 3 years (69.3 % for VuM and 25 % for VaM). Women with VaM died earlier: median specific death occurrence of 8.76 months [CI95 %: 6.54-24.72] versus 39.61 [CI95 %: 21.89-209.21], p=0.013 (HR=5.08 [CI95 %: 1.39-18.60], p=0.014). A lesion size ≥3cm was associated with an increased risk of mortality (HR=8.45 [CI95 %: 1.60-44.52], p=0.012). In multivariate analysis, vaginal location remained an independent and predictive variable of a higher risk of specific death (HR=8.56 [CI95 %: 1.95-37.64], p=0.005).
CONCLUSION
A vaginal location of MM is associated with a poorer prognosis than a vulvar location.
Topics: Adult; Aged; Aged, 80 and over; Disease-Free Survival; Female; Follow-Up Studies; Humans; Lymph Node Excision; Melanoma; Middle Aged; Mucous Membrane; Neoplasm Recurrence, Local; Prognosis; Retrospective Studies; Tertiary Care Centers; Time Factors; Vaginal Neoplasms; Vulvar Neoplasms
PubMed: 33592345
DOI: 10.1016/j.jogoh.2021.102091 -
Archives of Gynecology and Obstetrics Mar 2021To analyze the prognostic factors of recurrence and overall survival in rare histotypes of vulvar cancer.
PURPOSE
To analyze the prognostic factors of recurrence and overall survival in rare histotypes of vulvar cancer.
METHODS
An international multicenter retrospective study including patients diagnosed with vulvar cancer was performed. One hundred centers participated in the study and 2453 vulvar cancer cases were enrolled from January 2001 until December 2005. After exclusion of squamous vulvar cancer, Paget´s disease and vulvar melanoma 112 tumors were analyzed for the present study.
RESULTS
The mean age at diagnosis was 64.9 ± 17.2 years. 99 (88.4%) patients had a single lesion, in 25 (22.3%) cases the vulvar tumor involved the midline, and only 13 (11.5%) patients had clinically positive inguinal lymph nodes. The mean size of the lesion was 33.8 ± 33.9 mm. Regarding the surgical treatment, 2 (1.8%) patients underwent skinning vulvectomy, 63 (56.3%) local excision, 41 (36.6%) vulvectomy, 3 (2.7%) exenteration and 3 (2.7%) did not receive any surgical treatment. The mean free surgical margin was 8.2 ± 9 mm and 7 (6.2%) patients presented positive inguinal nodes. Radiotherapy was administered in 22 (19.6%) patients and it was performed postoperatively in all cases; 14 (12.5%) patients received adjuvant chemotherapy. The mean overall follow-up time was 44.1 ± 35.7 months. The risk factors associated with overall survival were chemotherapy and radiotherapy, tumor size and stromal invasion (p < 0.05). The only independent factor significantly associated with global recurrence and absence of metastasis was radiotherapy (p = 0.02 and p = 0.002, respectively).
CONCLUSION
Postoperative radiotherapy seems to be the only independent factor associated with recurrence and overall survival in uncommon types of vulvar cancer.
Topics: Adult; Aged; Aged, 80 and over; Antineoplastic Agents; Chemotherapy, Adjuvant; Female; Humans; Lymph Node Excision; Lymph Nodes; Middle Aged; Neoplasm Recurrence, Local; Prognosis; Retrospective Studies; Risk Factors; Treatment Outcome; Vulvar Neoplasms; Vulvectomy
PubMed: 33067709
DOI: 10.1007/s00404-020-05813-x -
JID Innovations : Skin Science From... Sep 2022Cases of vulvar melanocytic lesions in juveniles are rarely reported. We analyze the evidence regarding vulvar melanocytic lesions in juveniles with or without vulvar...
Cases of vulvar melanocytic lesions in juveniles are rarely reported. We analyze the evidence regarding vulvar melanocytic lesions in juveniles with or without vulvar lichen sclerosus to help decision making by clinicians and pathologists. A scoping review on vulvar melanocytic lesions with or without vulvar lichen sclerosus, including malignant vulvar melanomas, in females up to age 18 years was performed. In addition, the histopathology records of the cohort of all such lesions in The Netherlands from 1991 through 2020 were investigated, and a structured analysis of tissue samples of the subset of cases with lichen sclerosus was performed. The literature study performed confirms that vulvar melanomas in juveniles are extremely rare and that published case reports are often disputed. In The Netherlands, there are no cases of malignant vulvar melanomas up to age 18 years recorded from 1991 through 2020. Atypical histopathological features are often found in biopsies of vulvar nevi in juveniles, especially with concomitant lichen sclerosus, confirming earlier case studies in the literature. We conclude that even with atypical findings, vulvar melanocytic lesions in juveniles have a benign course. To avoid unnecessary and possibly mutilating procedures, we advise referral to an expert center and adaption of existing guidelines for vulvar melanocytic lesions in juveniles.
PubMed: 36105669
DOI: 10.1016/j.xjidi.2022.100140