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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 -
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 -
Biomedicines Jun 2021Melanomas of the skin are poorly circumscribed lesions, very frequently asymptomatic but unfortunately with a continuous growing incidence. In this landscape, one can... (Review)
Review
Melanomas of the skin are poorly circumscribed lesions, very frequently asymptomatic but unfortunately with a continuous growing incidence. In this landscape, one can distinguish melanomas originating in the mucous membranes and located in areas not exposed to the sun, namely the vulvo-vaginal melanomas. By contrast with cutaneous melanomas, the incidence of these types of melanomas is constant, being diagnosed in females in their late sixties. While hairy skin and glabrous skin melanomas of the vulva account for 5% of all cancers located in the vulva, melanomas of the vagina and urethra are particularly rare conditions. The location in areas less accessible to periodic inspection determines their diagnosis in advanced stages, often metastatic. Moreover, despite the large number of drugs newly approved in recent decades for the treatment of cutaneous melanoma, especially in the category of biological drugs, the mortality of vulvo-vaginal melanomas has remained almost constant. This, together with the absence of specific treatment guidelines due to the lack of a sufficient number of cases to conduct randomized clinical trials, makes melanomas with this localization a discouraging diagnosis, associated with a very poor prognosis. Our aim is therefore to draw attention to this oftentimes overlooked entity in order to encourage the community to employ various strategies meant to increase research in this area. By highlighting the main risk factors of vulvar and vaginal melanomas, as well as the clinical manifestations and molecular changes underlying these neoplasms, ideally novel therapeutic schemes will, in time, be brought into effect.
PubMed: 34209084
DOI: 10.3390/biomedicines9070758 -
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 -
BMC Cancer Jun 2021Malignant melanoma of the female genital tract is relatively uncommon and accounts for 3-7% of all melanoma localizations. This study aimed to identify driver genes in...
BACKGROUND
Malignant melanoma of the female genital tract is relatively uncommon and accounts for 3-7% of all melanoma localizations. This study aimed to identify driver genes in melanoma of the female genital tract with the purpose of enhancing understanding of disease pathogenesis and identifying potential new therapeutic targets to develop effective therapies.
METHODS
KIT (CD117) and BRAF expression were detected immunohistochemically. Polymerase Chain Reaction (PCR) and Sanger sequencing techniques were performed to identify the mutational status of BRAF, NRAS, KRAS, NF1, KIT, PDGFRA and SF3B1 on 19 melanomas of the female genital tract, paired with 25 cutaneous melanomas, 18 acral melanomas and 11 melanomas of nasal cavity.
RESULTS
Somatic variant analysis identified KRAS (6/19; 32%) as the most commonly mutated gene, followed by KIT (4/19; 21%), SF3B1 (3/19; 16%) and NRAS (1/19; 5%). None of the cases were found to harbor BRAF, NF1 and PDGFRA mutations in melanomas of the female genital tract. However, none of the cases were found to harbor SF3B1 and KIT mutations in cutaneous melanomas, acral melanomas and melanomas of nasal cavity. Recurrent KIT mutations, as well as mutations in the less frequently mutated genes NRAS and SF3B1, were exclusively detected in vulvovaginal melanomas, but not in tumors arising in the cervix. However, recurrent KRAS mutations were detected in similar frequencies in tumors of the vulva, vagina, and cervix. Additionally, recurrent KRAS and KIT mutations occurred predominantly in polygonal and epithelioid cell types of melanoma in the female genital tract. Immunohistochemistry revealed moderate or strong cytoplasmic CD117 expression in 6 of the 19 cases (31.6%).
CONCLUSIONS
We observed that gynecologic melanoma harbored distinct mutation rates in the KIT, BRAF, SF3B1, KRAS, and NRAS genes. Our findings support the notion that gynecologic melanoma is a distinct entity from non-gynecologic melanoma, and these findings offer insights into future therapeutic options for these patients.
Topics: Adult; Aged; Female; Genital Neoplasms, Female; High-Throughput Nucleotide Sequencing; Humans; Melanoma; Middle Aged; Mutation; Phosphoproteins; Proto-Oncogene Proteins c-kit; Proto-Oncogene Proteins p21(ras); RNA Splicing Factors
PubMed: 34102999
DOI: 10.1186/s12885-021-08427-x -
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 -
Cancer Medicine Jun 2021Increased risk of a second primary malignancy (SPM) before or after diagnosis of anal squamous cell carcinoma (ASCC) has been reported in a previous single-institution...
BACKGROUND
Increased risk of a second primary malignancy (SPM) before or after diagnosis of anal squamous cell carcinoma (ASCC) has been reported in a previous single-institution study. We hypothesize that patients diagnosed with ASCC are at increased risk for developing SPMs before or after the diagnosis of ASCC. The primary objective of this study was to identify the diagnoses of cancer most likely to occur as SPMs before or after ASCC.
METHODS
This work employs the Surveillance, Epidemiology, and End Results (SEER) Program registry data to conduct a US-population-based study of patients diagnosed with ASCC between 1975 and 2016. In patients diagnosed with ASCC, we evaluated the risk of SPMs and the risk of developing ASCC as an SPM after another cancer using standardized incidence ratios (SIR) for all SPMs by calculating the ratio of observed events in the ASCC cohort compared to expected (O/E) events in a matched reference cohort of the general population.
RESULTS
A total of 7,594 patients with primary ASCC were included. Patients with ASCC were at increased risk of the diagnosis of an SPM (SIR = 1.45), particularly cancers of the lung, vulva, oropharynx, or colon. Patients with ASCC had an increased rate of previous malignancy (SIR = 1.23), especially Kaposi sarcoma or vulvar cancer. Overall elevated incidence of SPMs was unrelated to prior radiation treatment. Radiation treatment was associated with increased risk for SPMs in the female genital system but appeared protective against prostate cancer as SPMs.
CONCLUSIONS
Our findings support increased surveillance and screening for second malignancies in patients with these diagnoses, as patients with ASCC are often either survivors of a prior cancer diagnosis or are at increased risk of developing later malignancies.
Topics: Anus Neoplasms; Carcinoma, Squamous Cell; Colonic Neoplasms; Female; Gastrointestinal Neoplasms; Humans; Incidence; Lung Neoplasms; Lymphoma; Lymphoma, Non-Hodgkin; Male; Melanoma; Neoplasms, Second Primary; Oropharyngeal Neoplasms; Prostatic Neoplasms; Risk; SEER Program; Sarcoma, Kaposi; Skin Neoplasms; United States; Vulvar Neoplasms
PubMed: 33960690
DOI: 10.1002/cam4.3909 -
Chinese Clinical Oncology Apr 2021Tracers and corresponding detection devices for the mapping of sentinel lymph nodes have been evolving since the first use of lymphangiogram methods in 1977 in penile... (Review)
Review
Tracers and corresponding detection devices for the mapping of sentinel lymph nodes have been evolving since the first use of lymphangiogram methods in 1977 in penile carcinoma. Nowadays a variety of dyes and radiotracers have been validated for use in breast, vulvar and cervical cancer as well as melanoma. Each tumor site with its anatomical conditions requires different mapping protocol. While the combination of radiotracer and blue dye or radiotracer alone is an established method for breast surgery, vulvar cancer and melanoma, in pelvic sentinel lymph node mapping indocyanine green is currently gaining popularity. Near infrared fluorescence imaging is an emerging technique that enables a real-time image-guided procedure and is currently approved by the Food and Drug Administration as a sentinel lymph node mapping substance with standard of care. New tracers and devices are constantly under investigation to better understand the pathway of lymphatic drainage and increase the sensibility and sensitivity of the method. In the present review the evolution of available tracers and detection devices is discussed. An exhaustive review of current clinical indications of each method, its particularities and adverse effects is made. Finally, an update on ongoing clinical studies in sentinel lymph node mapping methods is presented.
Topics: Colloids; Coloring Agents; Female; Humans; Lymph Nodes; Lymphatic Metastasis; Radiopharmaceuticals; Sentinel Lymph Node Biopsy; Technetium
PubMed: 33951916
DOI: 10.21037/cco-20-252 -
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 -
The Pan African Medical Journal 2021Malignant melanoma of the vulva is a rare and aggressive tumour with dismal prognosis. It tends to recur and metastasize early. Surgical excision with or without...
Malignant melanoma of the vulva is a rare and aggressive tumour with dismal prognosis. It tends to recur and metastasize early. Surgical excision with or without regional lymph node dissection is still the treatment of choice with adjuvant therapy decided on a case by case. Furthermore, HIV infection has been associated with more aggressive disease. Herein we present a 45-year-old HIV-infected female patient on antiretroviral therapy who presented with vulval ulcer for one year. On examination, she had ulcerated nodule on the labia majora. Radiology showed vulvovaginal tumour without involvement of the adjacent organs. Malignant melanoma was confirmed on both the incisional biopsy and vulvectomy. She responded poorly to radiotherapy. Furthermore, she presented with recurrence and metastatic disease a month after surgery. She was lost to follow-up clinic.
Topics: Anti-HIV Agents; Biopsy; Combined Modality Therapy; Female; HIV Infections; Humans; Melanoma; Middle Aged; Vulvar Neoplasms
PubMed: 33912285
DOI: 10.11604/pamj.2021.38.115.25864