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Melanoma Research Aug 2023Malignant vulvar melanoma (VuM) and vaginal melanoma (VaM) represent a unique subgroup of rare malignant melanomas with critical biological properties that differ from... (Observational Study)
Observational Study
Malignant vulvar melanoma (VuM) and vaginal melanoma (VaM) represent a unique subgroup of rare malignant melanomas with critical biological properties that differ from other cancers. In Japan, adequate surveys have yet to be conducted. This study aimed to elucidate the clinicopathological demographics and outcomes of VuM and VaM in Japan. This retrospective observational study included women with invasive VuM or VaM identified from older medical records in Japan. We collected clinical data and used the Kaplan-Meier method to analyze progression-free survival (PFS) and overall survival (OS). Univariate and multivariate regression models were used to identify factors significantly related to survival. We identified 217 patients, 109 (50.2%) with VuM and 108 (49.8%) with VaM. The median PFS was 16.8 months in patients with VuM [95% confidence interval (CI), 23.1-87.7] and 15.6 months in those with VaM (95% CI, 8.4-12.6). The median OS was 43.9 months (95% CI, 60-138) and 31.1 months (95% CI, 24.8-45.3) in patients with VuM and VaM, respectively. Multivariate analysis showed that a disease stage higher than stage III, based on the American Joint Committee on Cancer (AJCC) guidelines, was associated with poorer PFS [hazard ratio (HR), 2.063; 95% CI, 0.995-4.278] and an unknown surgical margin was the only independent factor influencing OS (HR, 2.188; 95% CI, 1.203-3.977). The overall outcomes of invasive VuM and VaM in Japan remain poor. AJCC staging and surgical margins were significant predictors of survival.
Topics: Humans; Female; Melanoma; Skin Neoplasms; Japan; Vaginal Neoplasms; Vulvar Neoplasms; Vagina; Vulva; Demography; Retrospective Studies; Neoplasm Staging; Prognosis; Melanoma, Cutaneous Malignant
PubMed: 37162526
DOI: 10.1097/CMR.0000000000000894 -
Cancers Mar 2023Vulvar Paget's disease (VPD) is a rare form of cutaneous adenocarcinoma of the vulva, which accounts for about 1-2% of all vulvar neoplasms and mainly affects... (Review)
Review
Vulvar Paget's disease (VPD) is a rare form of cutaneous adenocarcinoma of the vulva, which accounts for about 1-2% of all vulvar neoplasms and mainly affects post-menopausal women. The clinical presentation is usually non-specific and mimics chronic erythematous skin lesions; therefore, the diagnosis is often difficult and delayed. Although VPD is typically diagnosed at a locally advanced stage and has a high recurrence rate, the prognosis is overall favorable with a 5-year survival of nearly 90%. Due to the limited and poor-quality evidence, there is no global consensus on optimal management. Therefore, we performed a systematic review of the literature through the main electronic databases to deepen the current knowledge of this rare disease and discuss the available treatment strategies. Wide surgical excision is recommended as the standard-of-care treatment and should be tailored to the tumor position/extension and the patient's performance status. The goal is to completely remove the tumor and achieve clear margins, thus reducing the rate of local recurrences. Non-surgical treatments, such as radiotherapy, chemotherapy, and topical approaches, can be considered, especially in the case of unresectable and recurrent disease. In the absence of clear recommendations, the decision-making process should be individualized, also considering the new emerging molecular targets, such as HER2 and PD-L1, which might pave the way for future targeted therapies. The current review aims to raise awareness of this rare disease and encourage international collaboration to collect larger-scale, high-quality evidence and standardize treatment.
PubMed: 36980691
DOI: 10.3390/cancers15061803 -
Cureus Oct 2021Vulvar leiomyosarcoma is a rare smooth muscle malignant neoplasm but it is the commonest type of vulvar sarcomas. It may mimic benign tumors and misdiagnosis could...
Vulvar leiomyosarcoma is a rare smooth muscle malignant neoplasm but it is the commonest type of vulvar sarcomas. It may mimic benign tumors and misdiagnosis could delay proper management. We report a case of a 38-year-old pregnant woman with leiomyosarcoma of the vulva. The patient presented to her primary general practitioner with a small vulvar mass that she had first noticed one year prior. The tumor was suspected to be benign Bartholin's cyst and treated with antibiotics. The patient declined improvement and had many consultations to different clinics where she had been diagnosed and treated the same. The tumor size started to grow rapidly after she got pregnant, and the patient was referred to our hospital where she underwent tumor resection. Histopathology revealed leiomyosarcoma. The patient had further assessment and close follow-up and has had no recurrence for 12 months. There is little literature available on vulvar leiomyosarcoma, most of which are case reports, and most gynecologic oncologists will go through their whole careers without seeing a single case.
PubMed: 34796063
DOI: 10.7759/cureus.18772 -
International Journal of Molecular... May 2023Phytotherapy has long represented a widely accepted treatment alternative to conventional therapy. Bitter melon is a vine with potent antitumor effects against numerous... (Review)
Review
Phytotherapy has long represented a widely accepted treatment alternative to conventional therapy. Bitter melon is a vine with potent antitumor effects against numerous cancer entities. To date, no review article has, however, been published on the role of bitter melon in breast and gynecological cancer prevention and therapy. The current work constitutes the most comprehensive, up-to-date review of the literature, which highlights the promising anticancer effects of bitter melon on breast, ovarian, and cervical cancer cells and discusses future research recommendations.
Topics: Female; Humans; Momordica charantia; Phytotherapy; Uterine Cervical Neoplasms; Plant Extracts
PubMed: 37240264
DOI: 10.3390/ijms24108918 -
Current Oncology Reports Oct 2023Extramammary Paget disease (EMPD) is a rare entity which is more frequently localized at the vulva, though it only accounts for 1-2% of vulvar neoplasms. It is a primary... (Review)
Review
PURPOSE OF REVIEW
Extramammary Paget disease (EMPD) is a rare entity which is more frequently localized at the vulva, though it only accounts for 1-2% of vulvar neoplasms. It is a primary cutaneous adenocarcinoma whose cell of origin is still a matter of controversy: it can either arise from apocrine/eccrine glands or from stem cells. The diagnosis demands a biopsy and entails a histopathological analysis by which cells show similar characteristics as breast Paget disease.
RECENT FINDINGS
Treatment approach can entail surgery, radiotherapy, photodynamic therapy, systemic chemotherapy, and topical chemotherapy. For metastatic disease, many different chemotherapy regimens have been explored and even targeted therapy can play an important role in this disease. Since almost 30-40% of patients overexpress HER-2, trastuzumab and anti-HER-2 therapies can be employed in this setting. Due to its low incidence, there is almost no specific evidence on therapeutic interventions for this disease. Thus, there is a neat unmet need for molecular characterization of EMPD and diagnostic tools that allow clinicians to guide treatment both in the early and in the advanced disease settings. In this review, we aim to summarize available evidence about diagnosis and treatment of EMPD, both localized and metastatic, and to provide a comprehensive analysis that may help clinicians for therapeutic decisions.
PubMed: 37421583
DOI: 10.1007/s11912-023-01434-0 -
Current Oncology (Toronto, Ont.) Jan 2022Enhanced Recovery After Surgery (ERAS) is a global surgical quality improvement program that started in colorectal surgery and has now expanded to numerous specialties,... (Review)
Review
Enhanced Recovery After Surgery (ERAS) is a global surgical quality improvement program that started in colorectal surgery and has now expanded to numerous specialties, including gynecologic oncology. ERAS guidelines comprise multidisciplinary, evidence-based recommendations in the preoperative, intraoperative, and postoperative period; these interventions broadly encompass patient education, anesthetic choice, multimodal pain control, avoidance of unnecessary drains, maintenance of nutrition, and prevention of emesis. Implementation of ERAS has been shown to be associated with improved clinical outcomes (length of hospital stay, complications, readmissions) and cost. Marx and colleagues first demonstrated the feasibility of ERAS in gynecologic oncology in 2003; since then, over 30 comparative studies and 4 guidelines have been published encompassing major gynecologic surgery, cytoreductive surgery, and vulvar/vaginal surgery. Implementation of ERAS in gynecologic oncology has been demonstrated to provide improvements in length of stay, complications, cost, opioid use, and patient satisfaction. Increased compliance with ERAS guidelines has been associated with greater improvement in outcomes.
Topics: Enhanced Recovery After Surgery; Female; Genital Neoplasms, Female; Gynecologic Surgical Procedures; Humans; Length of Stay; Postoperative Period
PubMed: 35200556
DOI: 10.3390/curroncol29020056 -
Seminars in Diagnostic Pathology Jan 2021Vulvar squamous cell carcinomas (VSCC), which constitute over 90% of vulvar malignancies in adults, are classifiable into 2 subgroups that are mostly... (Review)
Review
Vulvar squamous cell carcinomas (VSCC), which constitute over 90% of vulvar malignancies in adults, are classifiable into 2 subgroups that are mostly clinicopathologically distinct, a classification that is fundamentally based whether or not the tumors are HPV-mediated. In this review, we aim to summarize the recent advances in the understanding of molecular events in the pathogenesis of VSCC, including common and targetable mutations, copy number alterations, epigenetics, noncoding RNAs, and tumor immune microenvironment, which may provide insight into the future management of the disease. These events show substantial differences between the 2 subgroups, although significant areas of overlap exist. Recurrent, driver mutations appear to be substantially more prevalent in HPV(-) VSCC. TP53 mutations are the most common somatic mutations in VSCC overall, and are notably predominant in the HPV(-) VSCC, where 30-88% show a mutation. TP53 mutations are associated with worse patient outcomes, and co-mutations between TP53 and either HRAS, PIK3CA or CDKN2A appear to define subsets with even worse outcomes. A wide variety of other somatic mutations have been identified, including a subset with different mutational frequencies between HPV(+) and HPV(-) VSCC. CDKN2A mutations are common, and have been identified in 21 to 55% of HPV(-) VSCC, and in 2 to 25% of HPV(+) VSCC. Hypermethylation of CDKN2A is the most frequently reported epigenetic alteration in VSCC and the expression of some microRNAs may be associated with patient outcomes. The PTEN/PI3K/AKT/mTOR pathway is commonly altered in HPV(+) VSCC, and is accordingly potentially targetable. HPV-positivity/p16 block expression by immunohistochemistry has been found to be an independent prognostic marker for improved survival in VSCC, and may have some predictive value in VSCC patients treated with definitive radiotherapy. 22-39.3% and 68% of VSCC show EGFR amplification and protein overexpression respectively, although the prognostic and predictive value of an EGFR alteration requires additional study. Recurrent chromosomal gains in VSCCs have been found at 1q, 2q, 3q, 4p, 5p, 7p, 8p, 8q, and 12q, and there may be differential patterns of alterations depending on HPV-status. At least one-third of VSCC patients may potentially benefit from immune checkpoint inhibition therapy, based on a high frequency of PD-L1 expression or amplification, or a high tumor mutational burden. Additional studies are ultimately required to better understand the global landscape of genetic and epigenetic alterations in VSCC, and to identify and test potential targets for clinical application.
Topics: Carcinoma in Situ; Carcinoma, Squamous Cell; Class I Phosphatidylinositol 3-Kinases; Cyclin-Dependent Kinase Inhibitor p16; DNA Copy Number Variations; Epigenomics; Female; Humans; Immunohistochemistry; Mutation; PTEN Phosphohydrolase; Papillomavirus Infections; RNA, Untranslated; Signal Transduction; TOR Serine-Threonine Kinases; Tumor Microenvironment; Tumor Suppressor Protein p53; Vulvar Neoplasms
PubMed: 33032902
DOI: 10.1053/j.semdp.2020.09.010 -
Cancers Oct 2022Cutaneous melanoma is a relatively common neoplasm, with fairly well understood pathogenesis, risk factors, prognosis and therapeutic protocols. The incidence of this... (Review)
Review
Cutaneous melanoma is a relatively common neoplasm, with fairly well understood pathogenesis, risk factors, prognosis and therapeutic protocols. The incidence of this disease is increasing every year. The situation is different for rare malignancies such as vulvar melanomas and for the even rarer vaginal melanomas. The risk factors for vulvovaginal tumors are not fully understood. The basis of treatment in both cases is surgical resection; however, other types of treatments such as immunotherapy are available. This paper focuses on comparing the pathogenesis and risk factors associated with these neoplasms as well as the efficacy of two groups of drugs-anti-PD-L1 and anti-CTLA4 inhibitors-against both cutaneous melanoma and melanoma of the lower genital tract (vulva and vagina). In the case of cutaneous melanoma, the situation looks more optimistic than for vulvovaginal melanoma, which has a much worse prognosis and, as it turns out, shows a poorer response to immune therapy.
PubMed: 36291906
DOI: 10.3390/cancers14205123 -
International Journal of Gynecological... Mar 2020Many tracers have been introduced into current medical practice with the purpose of improving lymphatic mapping techniques, anatomic visualization, and organ/tissue... (Review)
Review
Many tracers have been introduced into current medical practice with the purpose of improving lymphatic mapping techniques, anatomic visualization, and organ/tissue perfusion assessment. Among them, three tracers have dominated the field: indocyanine green, technetium-99m radiocolloid (Tc99), and blue dye. Tc99 and blue dye are used individually or in combination; however, given particular challenges with these tracers, such as the need for a preoperative procedure by nuclear medicine and cost, other options have been sought. Indocyanine green has proven to be a promising alternative for certain procedures, as it is easy to use and has quick uptake. Its use in the management of gynecologic cancers was first described for sentinel lymph node mapping in cervical cancer, and later for endometrial and vulvar cancers. This review provides an in-depth look at these mapping substances, their uses, and the potential for new discoveries.
Topics: Female; Genital Neoplasms, Female; Humans; Lymph Nodes; Lymphatic Metastasis; Neoplasm Staging; Prognosis; Randomized Controlled Trials as Topic; Sentinel Lymph Node; Sentinel Lymph Node Biopsy
PubMed: 31953349
DOI: 10.1136/ijgc-2019-001078 -
Folia Morphologica 2022Sentinel node biopsy in vulvar cancer is associated with much less morbidity than inguinofemoral node dissection. Our study focused on describing the morphology of...
BACKGROUND
Sentinel node biopsy in vulvar cancer is associated with much less morbidity than inguinofemoral node dissection. Our study focused on describing the morphology of superficial lymphatic drainage of the vulva and its relationship to regional nodes, which may facilitate orientation during surgery.
MATERIALS AND METHODS
In 24 female cadavers, injections of patent blue (at various localisations medially, unilaterally and bilaterally) were used to visualise the lymphatic drainage of the vulva. After dissection of lymphatic vessels and nodes, their course was documented by photograph and then analysed. Subsequently, a map of vulvar superficial lymphatics was created.
RESULTS
The cutaneous and subcutaneous tissue of the vulva primarily drained to superficial inguinal nodes. There was no evidence of a solitary lymph node that drained the unilateral vulva. Each area of the vulva drained to its own lymph node, which was variably localised in the subcutaneous groin around the great saphenous vein. Anastomoses between individual inguinal superficial lymph nodes are likely. Right-left symmetry in the course of lymphatic collectors was not detected. Natural drainage of the medial and paramedial areas to contralateral inguinal nodes was also not detected. The drainage pattern to ipsilateral inguinal nodes was consistent in cadavers without evidence of vulvar disease and may be applicable in the early stages of vulvar cancer.
CONCLUSIONS
There was no evidence of a solitary node that drained the unilateral vulva. Each part of the vulva may drain to a corresponding lymph node in a different localisation of the groin. The surgeon should take this variability into account.
Topics: Female; Humans; Vulvar Neoplasms; Sentinel Lymph Node Biopsy; Lymph Nodes; Lymphatic Vessels; Groin
PubMed: 34590298
DOI: 10.5603/FM.a2021.0096