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Annals of Medicine and Surgery (2012) Sep 2022Vulvar melanoma is a rare malignant tumor of the female genital sphere, representing postmenopausal women's prerogative, the diagnosis is based on immunohistochemicals...
Vulvar melanoma is a rare malignant tumor of the female genital sphere, representing postmenopausal women's prerogative, the diagnosis is based on immunohistochemicals analysis, and treatment requires a multidisciplinary approach. On account of its high metastatic potential as well as the late diagnosis given that it has non-specific clinical signs, the prognosis remains poor. In this study, we report the case of a woman of childbearing who presented a vaginal mass associated to chronic pelvic pain. Paraclinical investigations revealed a right vulvar tumoral process with pathological-looking inguinal adenomegalies on the right side with a necrotic center measuring 16.7 mm on the short axis, micronodules and secondary pulmonary nodules. The patient has been put under palliative chemotherapy, then passed out 8 months later. By this work, we attempt to review the diagnostic circumstances to better understand this delay, also to encourage self-examination and self-screening of abnormal lesions, as well as leveling the awareness of health professionals on this rare disease.
PubMed: 36147087
DOI: 10.1016/j.amsu.2022.104473 -
Archives of Medical Science : AMS Mar 2015
PubMed: 25861315
DOI: 10.5114/aoms.2013.36184 -
The Indian Journal of Surgery Feb 2016Primary malignant melanoma of vagina is a rare variant of melanoma and usually associated with a grave prognosis. Radical surgery is the only treatment option with...
Primary malignant melanoma of vagina is a rare variant of melanoma and usually associated with a grave prognosis. Radical surgery is the only treatment option with reasonable loco regional control. A case of primary malignant melanoma involving whole of vagina infiltrating urethra and reaching up to vulva was treated by surgery and postoperative radiotherapy. The tumor was infiltrating bladder and rectum reaching the anal sphincter. Total pelvic exenteration was done to achieve tumor-free surgical margins. One year after treatment, patient is disease free.
PubMed: 27186045
DOI: 10.1007/s12262-015-1349-x -
Revista Brasileira de Ginecologia E... Mar 2018Melanomas of the female genital tract may occur in the vulva, the vagina, the ovary or the cervix. Pregnancy has been considered an aggravating factor in the evolution...
Melanomas of the female genital tract may occur in the vulva, the vagina, the ovary or the cervix. Pregnancy has been considered an aggravating factor in the evolution and prognosis of melanoma. A 35-year-old female presented with vaginal bleeding 2 months after a term cesarean delivery. An endovaginal ultrasound revealed a lesion in the uterine cervix. The pathological report revealed a small round-cell neoplasm, and the immunohistochemistry confirmed the diagnosis of malignant melanoma. A positron emission tomography revealed an expansive hypermetabolic lesion centered on the cervix, and hypermetabolic lesions in the liver and right kidney. Non-surgical treatment was provided, with biochemotherapy followed by ipilimumab and nivolumab. The patient died one year later. Postpartum vaginal bleeding, even if late-onset, should be investigated, as it may be a pregnancy-associated malignant melanoma, which has a poor prognosis.
Topics: Adult; Female; Humans; Melanoma; Postpartum Period; Uterine Cervical Neoplasms
PubMed: 29475202
DOI: 10.1055/s-0038-1624578 -
Journal of Lower Genital Tract Disease Apr 2021The aims of the study were to assess the clinical and histopathological characteristics of a comprehensive cohort of women with vulvovaginal melanoma (VVM) treated at...
OBJECTIVES
The aims of the study were to assess the clinical and histopathological characteristics of a comprehensive cohort of women with vulvovaginal melanoma (VVM) treated at our institution and to study the treatment response of checkpoint inhibitors in this patient cohort.
MATERIALS AND METHODS
This is a retrospective study of women with invasive VVM treated at the Princess Margaret Cancer Centre in Toronto, Ontario, Canada, over a period of 15 years. Clinical and histopathological characteristics, treatment, as well as treatment-related outcome were analyzed in 32 women. Treatment response was evaluated retrospectively using the "response criteria for use in trials testing immunotherapeutics" (iRECIST). The objective response rate was defined as the proportion of patients with complete or partial response based on the best overall response.
RESULTS
At a median follow-up of 37.8 months (5.8-110.4), 26 women (81.3%) had disease progression and 16 (50%) died. Thirteen patients with locally unresectable or metastatic melanoma were treated with immune checkpoint inhibitors. Ten additional cases were identified from previously published reports. The best objective response rate for immune checkpoint inhibitors was 30.4% (95% CI = 11.6%-49.2%) and the clinical benefit rate was 52.2% (95% CI = 31.8%-72.6%). The clinical benefit rate was significantly better for programmed cell death protein 1 inhibitors (or a combination) compared with ipilimumab alone (Fisher exact, p = .023). Grade 3/4 adverse events were observed in 3 (13.0%) of the 23 patients.
CONCLUSIONS
Women with VVM constitute a high-risk group with poor overall prognosis. Immune checkpoint inhibitors are effective in the treatment of metastatic melanoma in this patient cohort.
Topics: Adult; Aged; Cohort Studies; Disease Progression; Female; Humans; Immune Checkpoint Inhibitors; Melanoma; Middle Aged; Ontario; Retrospective Studies; Treatment Outcome; Vaginal Neoplasms; Vulvar Neoplasms
PubMed: 33252450
DOI: 10.1097/LGT.0000000000000583 -
International Journal of Molecular... Apr 2021Gynecological cancers pose an important public health issue, with a high incidence among women of all ages. Gynecological cancers such as malignant germ-cell tumors,... (Review)
Review
Gynecological cancers pose an important public health issue, with a high incidence among women of all ages. Gynecological cancers such as malignant germ-cell tumors, sex-cord-stromal tumors, uterine sarcomas and carcinosarcomas, gestational trophoblastic neoplasia, vulvar carcinoma and melanoma of the female genital tract, are defined as rare with an annual incidence of <6 per 100,000 women. Rare gynecological cancers (RGCs) are associated with poor prognosis, and given the low incidence of each entity, there is the risk of delayed diagnosis due to clinical inexperience and limited therapeutic options. There has been a growing interest in the field of microRNAs (miRNAs), a class of small non-coding RNAs of ∼22 nucleotides in length, because of their potential to regulate diverse biological processes. miRNAs usually induce mRNA degradation and translational repression by interacting with the 3' untranslated region (3'-UTR) of target mRNAs, as well as other regions and gene promoters, as well as activating translation or regulating transcription under certain conditions. Recent research has revealed the enormous promise of miRNAs for improving the diagnosis, therapy and prognosis of all major gynecological cancers. However, to date, only a few studies have been performed on RGCs. In this review, we summarize the data currently available regarding RGCs.
Topics: Biomarkers, Tumor; Circulating MicroRNA; Clinical Decision-Making; Disease Management; Female; Gene Expression Regulation, Neoplastic; Genital Neoplasms, Female; Humans; MicroRNAs; Molecular Diagnostic Techniques; Pregnancy; Prognosis; RNA Interference; RNA, Messenger; Treatment Outcome
PubMed: 33917022
DOI: 10.3390/ijms22083822 -
Romanian Journal of Morphology and... 2016Vulvar malignant melanomas are extremely rare neoplasms, representing less than 3% of all cancers in women, 9% of all external genital tract malignancies and 9% of all...
Vulvar malignant melanomas are extremely rare neoplasms, representing less than 3% of all cancers in women, 9% of all external genital tract malignancies and 9% of all primary vulvar malignancies. We present the case of a 60-year-old Caucasian patient, who has been admitted in the Clinic of Obstetrics and Gynecology with polymorphic, vulvar local, pelvic-abdominal, genitourinary and general symptoms, being diagnosed with nodular and superficial spreading vulvar melanoma and multiple voluminous uterine leiomyoma with various degenerations. Our study presents the approach of this case in terms of surgical pathology, management, prognosis and outcome. Surgical treatment is the central element of therapeutic management. Vulva melanomas are in general a relatively unpredictable unfavorable prognosis. The sizes of the tumor, the thickness and micro-staging are essential factors for prognosis.
Topics: Abdomen; Female; Humans; Melanoma; Middle Aged; Skin Neoplasms; Vulvar Neoplasms
PubMed: 28174807
DOI: No ID Found -
BMC Women's Health Aug 2021Polypoid nodule growing without apparent pigmentation on the vulvar skin usually reminds us of the diagnostic pitfall, which is commonly and mistakenly diagnosed as...
BACKGROUND
Polypoid nodule growing without apparent pigmentation on the vulvar skin usually reminds us of the diagnostic pitfall, which is commonly and mistakenly diagnosed as other types of tumors. Although there are several manifestations of amelanotic melanoma are known, these malignancies are usually pigmented because they are derived from melanocytes containing melanin. However, amelanotic melanomas are easily misdiagnosed or their diagnoses were commonly delayed due to lack of pigmentation. Therefore, a solitary polypoid nodule is worth noting and further reporting. Particularly, the clinical characteristics and outcomes of the solitary polypoid nodule are rare in Asian patients.
CASE PRESENTATION
We presented an interesting case of a 33-year-old female with a solitary polypoid nodule without apparent pigmentation on her vulvar skin. Her medical history was unclear, no ulcer was seen in the lesion area, and dermatoscopy was indicated a possible tumorous change, which has caught the attention of clinicians, and then further examined by the pathologist. The final diagnosis was nodular malignant melanoma (NM) (Breslow thickness 9.5mm, Clark level 4).
CONCLUSIONS
Hence, though reviewing this case record, the relevant literature and NM-related materials, we suggest that the combination of skin imaging technology and histopathological examination could provide us a better understanding and reduce the possibility of misdiagnosis in clinic practice.
Topics: Adult; Female; Humans; Melanoma, Amelanotic; Pigmentation; Polyps; Skin Neoplasms
PubMed: 34362374
DOI: 10.1186/s12905-021-01422-1 -
British Medical Journal Feb 1972A series of 127 patients presenting at two London hospitals with carcinoma of the vulva during the period 1948-70 is reviewed. Patients with intraepithelial carcinoma or...
A series of 127 patients presenting at two London hospitals with carcinoma of the vulva during the period 1948-70 is reviewed. Patients with intraepithelial carcinoma or rodent ulcer are excluded, but the series includes two cases of malignant melanoma, one of adenocarcinoma of Bartholin's gland, and two of basal cell carcinoma. In the remainder the pathological diagnosis was squamous cell carcinoma.Leucoplakia was present in only 30 cases (24%) and it is therefore concluded that this condition is probably not so dangerous as a precursor of cancer as has been stated in the past, though an association with cancer of the vulva is undeniable.In only three cases was no form of treatment practicable. Of the remaining 124, two were treated by radiotherapy, 37 by simple vulvectomy, 12 by vulvectomy followed by separate inguinal lymphadenectomy, and 73 by radical vulvectomy combined with bilateral lymphadenectomy en bloc, pelvic lymphadenectomy being included whenever the superficial lymph nodes appeared to be involved or when Cloquet's node was shown by frozen-section examination to be invaded. Excellent results, with an absolute five-year survival rate of 66%, were obtained with this last procedure, especially when primary skin cover was provided by the delayed technique (five-year survival 73%). The results of simple vulvectomy were also surprisingly good, with an absolute five-year survival rate of 65%, and this operation may be a wise choice for the very old or infirm patient.
Topics: Adenocarcinoma; Aged; Bartholin's Glands; Carcinoma, Squamous Cell; Female; Humans; Leukoplakia; Lymph Node Excision; Melanoma; Middle Aged; Skin Transplantation; Vulva; Vulvar Neoplasms
PubMed: 4550741
DOI: 10.1136/bmj.1.5797.397 -
Therapeutic Advances in Medical Oncology 2020Metastatic vulvar melanoma is a rare and aggressive disease and survival is usually poor. Vulvar melanomas harbor BRAF V600 mutations only infrequently; consequently,...
Metastatic vulvar melanoma is a rare and aggressive disease and survival is usually poor. Vulvar melanomas harbor BRAF V600 mutations only infrequently; consequently, target therapy is a rare therapeutic option and immunotherapy usually has only a weak effect. On the other hand, KIT mutations are rare in cutaneous melanomas, but relatively frequent in mucosal melanomas, particularly in vulvar-vaginal melanomas, and can be a therapeutic target. Herein, we report a clinical case of a patient with metastatic vulvar melanoma, harboring an exon 17 c-KIT mutation, treated with avapritinib (BLU-285) - a highly potent and selective oral kinase inhibitor designed to treat imatinib-resistant gastro-intestinal stromal tumors (GIST) by targeting KIT/PDGFRα activation loop mutants (exons 17/18). After failure of the combination of ipilimumab + nivolumab first and then nivolumab alone, the patient received avapritinib 300 mg/daily for central nervous system (CNS), lymph-nodal, right adrenal gland, lung, and subcutaneous metastases. Best response was partial remission, according to RECIST 1.1 criteria. Time to treatment progression was 11 months. Main toxicities were grade 2 cutaneous vasculitis that required avapritinib discontinuation, and grade 2 uveitis of unknown origin, treated by vitrectomy and empiric antibiotic and antiviral therapy due to negative cultural tests. Uveitis was detected at the time of progression and therapy was definitively discontinued. In conclusion, avapritinib proved to be effective even in the presence of a pretreated disease, a high tumor burden, and brain metastases. In our experience, treatment was feasible and toxicity manageable. Considering the lack of effective therapies and the poor outcome of the disease, determination of c-KIT mutations should be performed routinely in cases of metastatic mucosal melanoma.
PubMed: 32821296
DOI: 10.1177/1758835920946158