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Skin Health and Disease Apr 2023Lichen sclerosus (LSc) is a chronic, inflammatory, destructive skin disease with a predilection for the genitalia (GLSc). An association with vulval (Vu) and penile (Pe)... (Review)
Review
BACKGROUND
Lichen sclerosus (LSc) is a chronic, inflammatory, destructive skin disease with a predilection for the genitalia (GLSc). An association with vulval (Vu) and penile (Pe) squamous carcinoma (SCC) is now well established but melanoma (MM) has only rarely been reported complicating GLSc.
METHODS
We have performed a systematic literature review of GLSc in patients with genital melanoma (GMM). Only articles that mentioned both GMM and LSc affecting either the penis or vulva were included.
RESULTS
Twelve studies with a total of 20 patients were included. Our review shows that an association of GLSc with GMM has been more frequently reported in women and female children than men viz, 17 cases compared with three. It is notable that five of the cases (27.8%) concerned female children aged under twelve.
DISCUSSION
These data suggest a rare association between GLSc and GMM. If proven, there arise intriguing questions about pathogenesis and consequences for counselling of patients and follow-up.
PubMed: 37013116
DOI: 10.1002/ski2.198 -
Histopathology Jul 2023Extramammary Paget disease (EMPD) is an epithelial neoplasm that can occur at many sites, including the vulva and scrotum. EMPD is characterised by the presence of...
AIMS
Extramammary Paget disease (EMPD) is an epithelial neoplasm that can occur at many sites, including the vulva and scrotum. EMPD is characterised by the presence of neoplastic cells, in single cells and clusters, that infiltrate all layers of non-neoplastic squamous epithelium. The differential diagnosis for EMPD includes melanoma in situ and secondary involvement of tumours from other sites, such as urothelial or cervical; pagetoid spread of tumor cells can also been seen at other sites, such as anorectal mucosa. The most frequently utilised biomarkers for confirming the diagnosis of EMPD include CK7 and GATA3; however, these biomarkers lack specificity. The purpose of this study was to evaluate TRPS1, a newly described breast biomarker, in pagetoid neoplasms of the vulva, scrotum and anorectum.
METHODS AND RESULTS
Fifteen cases of primary EMPD of the vulva (two with associated invasive carcinoma) and four primary EMPD of the scrotum showed strong nuclear immunoreactivity for TRPS1. In contrast, five cases of vulvar melanoma in situ, one case of urothelial carcinoma with secondary pagetoid spread into the vulva and two anorectal adenocarcinomas with pagetoid spread into anal skin (one with associated invasive carcinoma) were negative for TRPS1. Additionally, weak nuclear TRPS1 staining was observed in non-neoplastic tissues (e.g. keratinocytes), but always with less intensity when compared to tumour cells.
CONCLUSIONS
These results demonstrate that TRPS1 is a sensitive and specific biomarker for EMPD, and may be especially useful for excluding secondary involvement of the vulva by urothelial and anorectal carcinomas.
Topics: Male; Female; Humans; Carcinoma, Transitional Cell; Paget Disease, Extramammary; Biomarkers, Tumor; Urinary Bladder Neoplasms; Melanoma; Repressor Proteins; Melanoma, Cutaneous Malignant
PubMed: 36971374
DOI: 10.1111/his.14908 -
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 -
Dermatologic Surgery : Official... May 2023Vulvar melanoma is a rare malignancy with frequent recurrence and poor prognosis. National guidelines recommend wide local excision of these tumors with allowances for...
BACKGROUND
Vulvar melanoma is a rare malignancy with frequent recurrence and poor prognosis. National guidelines recommend wide local excision of these tumors with allowances for narrower margins for anatomic and functional limitations, which are common on specialty sites. There is presently a lack of data of margin positivity after standard excision of vulvar melanomas.
OBJECTIVE
We aim to evaluate the rate of positive margins after standard excision of vulvar melanomas.
MATERIALS AND METHODS
Retrospective cohort study of surgically excised vulvar melanomas from the NCDB diagnosed from 2004 to 2019.
RESULTS
We identified a total of 2,226 cases. Across surgical approaches and tumor stages, 17.2% (Standard Error [SE]: 0.8%) of cases had positive surgical margins. Among tumor stages, T4 tumors were most commonly excised with positive margins (22.9%, SE: 1.5%). On multivariable survival analysis, excision with positive margins was associated with significantly poorer survival (Hazard Ratio 1.299, p = .015).
CONCLUSION
We find that positive margin rates after standard excision of vulvar malignancies are higher than for other specialty site melanomas. Our data suggest that use of surgical approaches with complete margin assessment may improve local control and functional outcomes for patients with vulvar melanoma as they have for patients with other specialty site melanomas.
Topics: Female; Humans; Retrospective Studies; Vulvar Neoplasms; Margins of Excision; Skin Neoplasms; Melanoma; Neoplasm Recurrence, Local
PubMed: 36857160
DOI: 10.1097/DSS.0000000000003734 -
International Journal of Gynecological... Jul 2023Under 10% of gynaecological cancers are diagnosed in the vulva and vagina, mostly squamous cell carcinomas. Melanoma, Paget disease, basal cell carcinomas, and other...
Under 10% of gynaecological cancers are diagnosed in the vulva and vagina, mostly squamous cell carcinomas. Melanoma, Paget disease, basal cell carcinomas, and other cancers can present with vulval/vaginal symptoms. The pathology information system of a tertiary referral center for vulvo-vaginal cancers was searched for cancers of the vulva and vagina from 1996 to 2019. Squamous carcinomas were excluded, and the remaining entities were catalogued. A total of 221 nonsquamous cancers were found, including 135 vaginal and 86 vulval cases. One hundred eight cases of metastatic carcinomas from the endometrium, cervix, ovary, bowel, bladder, kidney, and breast formed the largest category. Basal cell carcinomas constituted the second largest category. Others included melanomas, Paget disease, and adenoid cystic carcinomas. Primary adenocarcinomas included porocarcinoma, mammary type carcinoma, enteric type carcinoma, clear cell carcinoma, Bartholin gland adenocarcinoma and malignant transformation of hidradenoma papilliferum. The vulva and vagina can harbor a wide range of nonsquamous malignancies. The most challenging of these are adenocarcinomas which can be metastatic from other sites. The dominance of metastatic carcinomas in this series is likely to reflect consultation practice of specialist pathologists.
Topics: Female; Humans; Vulva; Vagina; Vulvar Neoplasms; Carcinoma, Squamous Cell; Adenocarcinoma; Melanoma; Carcinoma, Basal Cell; Skin Neoplasms
PubMed: 36731045
DOI: 10.1097/PGP.0000000000000924 -
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 -
World Journal of Surgery Apr 2023Inguinal lymph node dissection (ILND) plays a crucial role in the oncological management of patients with melanoma, penile, and vulvar cancer. This study aims to... (Meta-Analysis)
Meta-Analysis Review
Assessment and Reporting of Perioperative Adverse Events and Complications in Patients Undergoing Inguinal Lymphadenectomy for Melanoma, Vulvar Cancer, and Penile Cancer: A Systematic Review and Meta-analysis.
BACKGROUND
Inguinal lymph node dissection (ILND) plays a crucial role in the oncological management of patients with melanoma, penile, and vulvar cancer. This study aims to systematically evaluate perioperative adverse events (AEs) in patients undergoing ILND and its reporting.
METHODS
A systematic review was conducted according to PRISMA. PubMed, MEDLINE, Scopus, and Embase were queried to identify studies discussing perioperative AEs in patients with melanoma, penile, and vulvar cancer following ILND.
RESULTS
Our search generated 3.469 publications, with 296 studies meeting the inclusion criteria. Details of 14.421 patients were analyzed. Of these studies, 58 (19.5%) described intraoperative AEs (iAEs) as an outcome of interest. Overall, 68 (2.9%) patients reported at least one iAE. Postoperative AEs were reported in 278 studies, combining data on 10.898 patients. Overall, 5.748 (52.7%) patients documented ≥1 postoperative AEs. The most reported ILND-related AEs were lymphatic AEs, with a total of 4.055 (38.8%) events. The pooled meta-analysis confirmed that high BMI (RR 1.09; p = 0.006), ≥1 comorbidities (RR 1.79; p = 0.01), and diabetes (RR 1.81; p = < 0.00001) are independent predictors for any AEs after ILND. When assessing the quality of the AEs reporting, we found 25% of studies reported at least 50% of the required criteria.
CONCLUSION
ILND performed in melanoma, penile, and vulvar cancer patients is a morbid procedure. The quality of the AEs reporting is suboptimal. A more standardized AEs reporting system is needed to produce comparable data across studies for furthering the development of strategies to decrease AEs.
Topics: Male; Female; Humans; Penile Neoplasms; Vulvar Neoplasms; Lymph Node Excision; Melanoma; Lymphatic Vessels
PubMed: 36709215
DOI: 10.1007/s00268-022-06882-6 -
Gynecologic Oncology Mar 2023This multicenter study aimed to investigate the role of preoperative lymphatic mapping and sentinel node biopsy (SNB) as well as the impact of negative SNB on...
OBJECTIVE
This multicenter study aimed to investigate the role of preoperative lymphatic mapping and sentinel node biopsy (SNB) as well as the impact of negative SNB on loco-regional control and survival in vulvar melanoma patients with clinically negative nodes (cN0).
METHODS
Patients who had a proven vulvar melanoma with a Breslow thickness of 1-4 mm, cN0 and underwent a preoperative lymphatic mapping followed by SNB between July 2013 and March 2021 were retrospectively included. Groin recurrence and mortality rate were calculated as absolute and relative frequency. Disease-free survival (DFS) and overall survival (OS) were assessed by the Kaplan-Meier method. We provided a systematic review, searching among PubMed/Medline and Embase libraries. A total of 6 studies were identified (48 patients).
RESULTS
A total of 18 women were included. Preoperative planar images showed 51 SNs in 28 groins. Additional SPECT/CT images were acquired in 5/18 cases; SNs were identified pre- and intra-operatively in all cases. A total of 65 SNs were excised from 28 groins. A total of 13/18 (72.2%) patients (21/28 groins, 75%) had negative SNs with no groin recurrences and 12/13 (92.3%) were still alive at last follow-up. Five out of the 18 (27.8%) patients (7/28 groins, 25%) had positive SNs, 2/5 (40%) patients died of cancer after 26.2 and 33.8 months, respectively. The median DFS and OS for the entire cohort were 17.9 months (95% CI, 10.3-19.9) and 65.0 months (95% CI, 26.2-infinite), respectively. The probability of DFS and OS at 3 years were 15.5% (95% CI, 2.6-38.7) and 64.3% (95% CI, 15.5-90.2), respectively.
CONCLUSIONS
The use of preoperative lymphatic mapping followed by SNB permits a precise and minimally invasive surgical approach in cN0 vulvar melanoma patients. Negative SNB is associated with low risk of groin relapse and good survival.
Topics: Humans; Female; Lymphatic Metastasis; Retrospective Studies; Neoplasm Recurrence, Local; Sentinel Lymph Node Biopsy; Skin Neoplasms; Melanoma; Vulvar Neoplasms; Lymph Node Excision; Lymph Nodes; Multicenter Studies as Topic
PubMed: 36696819
DOI: 10.1016/j.ygyno.2023.01.011 -
Cureus Dec 2022Mucosal melanomas (MM) are a rare type of melanomas commonly found in the vulvovaginal, anorectal, and respiratory tract. In this case report, a 71-year-old female...
Mucosal melanomas (MM) are a rare type of melanomas commonly found in the vulvovaginal, anorectal, and respiratory tract. In this case report, a 71-year-old female presented to her OB/GYN clinic with dark raised mass on her right labial region adjacent to the perineum. Past medical and surgical history of note included third-degree uterine prolapse, senile vaginitis, fibrocystic changes of the breasts bilaterally, hypothyroidism, hypertension, as well as a past hysterectomy and anterior colporrhaphy. Upon further workup, the 2.7 x 1.8 x 2 cm polyploid mass was biopsied and was found to be consistent with malignant melanoma. The patient then underwent a wide local excision confirming that the lesion was a nodular vulvar melanoma with superficial ulcerations and lymphovascular invasion of the vulvar region. Post-wide local incisions were found to be healed well after the procedure and the patient was referred to a gynecological oncologist for continuous monitoring. The purpose of this case report is to bring awareness of melanomas arising in atypical regions. While MMs are rare in comparison to cutaneous melanomas (CM), the prognosis can be poor if not caught early.
PubMed: 36686100
DOI: 10.7759/cureus.32698 -
The American Journal of Dermatopathology Feb 2023
Review
Topics: Female; Humans; Paget Disease, Extramammary; Vulvar Neoplasms; Melanoma; Skin Neoplasms
PubMed: 36669079
DOI: 10.1097/DAD.0000000000002371