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Journal of Lower Genital Tract Disease Jan 2023Neoplasms arising from the vulva are uncommon and comprise various subtypes. Given the recent advancements in the molecular aspects of oncologic pathology and how they...
OBJECTIVES
Neoplasms arising from the vulva are uncommon and comprise various subtypes. Given the recent advancements in the molecular aspects of oncologic pathology and how they have impacted cancer treatment, an understanding of recent innovations in the molecular features of vulvar lesions is important.
MATERIALS AND METHODS
Systematic literature search was performed on PubMed, Google Scholar, and Scopus databases for molecular and genetic characteristics of vulvar neoplasms. Peer-reviewed literature published in English is included.
RESULTS
Squamous cell carcinoma (SCC) and its precursors are the predominant neoplasm at this site. Human papillomavirus (HPV) plays a crucial role in the pathogenesis of some of these lesions. Human papillomavirus-associated SCC follows the carcinogenic pathway driven by viral proteins E6 and E7 while HPV-independent SCC shows a high incidence of mutation of TP53 and CDKN2A genes. Mutations in the genes involving the PI3K-Akt pathway play an important role in the pathogenesis of both types of SCC. Among other vulvar malignancies, melanoma, and vulvar Paget disease (VPD) pose a significant clinical challenge and have unique molecular characteristics. Compared with dermal cutaneous melanoma, vulvar melanoma shows a higher rate of mutation of cKIT and NRAS genes and a lower rate of mutations in BRAF . Less than 20% of VPD shows amplification of ERBB2 and seldom shows mutation in genes involving the PI3K-Akt pathway.
CONCLUSIONS
Several potentially targetable molecular pathways have emerged as they have been shown to be involved in the tumorigenesis of SCC, melanoma, and VPD.
Topics: Female; Humans; Carcinoma, Squamous Cell; Human Papillomavirus Viruses; Melanoma; Papillomavirus Infections; Phosphatidylinositol 3-Kinases; Proto-Oncogene Proteins c-akt; Skin Neoplasms; Vulvar Neoplasms
PubMed: 36083687
DOI: 10.1097/LGT.0000000000000701 -
Journal of Cancer Research and Clinical... Jul 2022Secondary extramammary Paget's disease (EMPD) related to urothelial carcinoma is rare, with some cases presenting synchronously with either a primary neoplasm or... (Review)
Review
PURPOSE
Secondary extramammary Paget's disease (EMPD) related to urothelial carcinoma is rare, with some cases presenting synchronously with either a primary neoplasm or recurrence of a neoplasm and other cases presenting up to 13 years prior to the detection of urothelial carcinoma. In this report, we will review the presentation, diagnosis, pathophysiology, management, and literature review of cases of secondary EPMD associated with urothelial carcinoma.
METHODS
We reviewed the English literature for all cases of secondary EMPD presenting synchronously with or in patients with a history of urothelial carcinoma, as well as treatment data for secondary vulvar Paget's.
RESULTS
We identified 16 case reports and case series with a total of 20 cases of vulvar EMPD associated with urothelial carcinoma. Twelve cases presented asynchronously and 8 had EMPD preceding the diagnosis of the underlying neoplasm. There is a paucity in the literature regarding management and surgical resection is a common treatment strategy; however, nonsurgical interventions may also be effective.
CONCLUSION
There is a paucity in the literature regarding management of secondary EPMD of urothelial origin, but consideration of radiation and systemic chemotherapy may be a reasonable treatment approach.
Topics: Carcinoma, Transitional Cell; Female; Humans; Paget Disease, Extramammary; Urinary Bladder Neoplasms; Vulva; Vulvar Neoplasms
PubMed: 35430687
DOI: 10.1007/s00432-022-04007-z -
Journal of the National Comprehensive... Mar 2024Vulvar cancer is annually diagnosed in an estimated 6,470 individuals and the vast majority are histologically squamous cell carcinomas. Vulvar cancer accounts for 5% to...
Vulvar cancer is annually diagnosed in an estimated 6,470 individuals and the vast majority are histologically squamous cell carcinomas. Vulvar cancer accounts for 5% to 8% of gynecologic malignancies. Known risk factors for vulvar cancer include increasing age, infection with human papillomavirus, cigarette smoking, inflammatory conditions affecting the vulva, and immunodeficiency. Most vulvar neoplasias are diagnosed at early stages. Rarer histologies exist and include melanoma, extramammary Paget's disease, Bartholin gland adenocarcinoma, verrucous carcinoma, basal cell carcinoma, and sarcoma. This manuscript discusses recommendations outlined in the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for treatments, surveillance, systemic therapy options, and gynecologic survivorship.
Topics: Female; Humans; Adenocarcinoma; Genital Neoplasms, Female; Paget Disease, Extramammary; Skin Neoplasms; Vulvar Neoplasms
PubMed: 38503056
DOI: 10.6004/jnccn.2024.0013 -
Best Practice & Research. Clinical... Oct 2014Vulvar carcinoma is an uncommon tumor that is seen most often in older women. Subtle symptoms such as pruritus should prompt examination and targeted biopsy in all women... (Review)
Review
Vulvar carcinoma is an uncommon tumor that is seen most often in older women. Subtle symptoms such as pruritus should prompt examination and targeted biopsy in all women as this disease can be successfully treated even in elderly, frail individuals. Vulvar cancer has a bimodal age distribution and is seen in both young and older women with risk factors including human papillomavirus (HPV) infection, smoking, and vulvar skin diseases (i.e., lichen sclerosus). This cancer is staged surgically, with an update in 2009 incorporating prognostic factors. The treatment of vulvar carcinoma has evolved to include more conservative surgical techniques that provide improved cure rates with emphasis on minimizing morbidity. Advanced and metastatic lesions are now treated with chemoradiation which produces substantial cure rates with decreased morbidity. Promising areas of research in vulvar cancer include refinement of sentinel lymph node biopsy, prevention of lymphedema, and preservation of sexual function following treatment.
Topics: Female; Humans; Papillomaviridae; Papillomavirus Infections; Risk Factors; Vulvar Neoplasms
PubMed: 25151473
DOI: 10.1016/j.bpobgyn.2014.07.001 -
Archives of Pathology & Laboratory... Jun 2019Differentiated-type vulvar intraepithelial neoplasia (dVIN) is a non-human papilloma virus (HPV)-related precursor lesion to vulvar squamous carcinoma. The terminology... (Review)
Review
Differentiated-type vulvar intraepithelial neoplasia (dVIN) is a non-human papilloma virus (HPV)-related precursor lesion to vulvar squamous carcinoma. The terminology has only become recognized clinically and histopathologically in recent years despite being described more than 50 years ago. As opposed to the HPV-related VIN (uVIN), dVIN has different features of histomorphology, risk of progression, and molecular pathogenesis. Notably, dVIN commonly develops in a background of chronic inflammatory dermatoses such as lichen sclerosis and lichen simplex chronicus. The recognition of dVIN remains a challenge owing to lack of accurate and reproducible diagnostic criteria. Morphologically, basal layer atypia, dyskeratosis, and elongation and anastomosis of the rete ridges are regarded as very useful diagnostic features. Ancillary tests can be very helpful to establish a definitive diagnosis in some ambiguous cases. In contrast to uVIN, dVIN is more likely to progress to vulvar squamous carcinoma in a shorter period. The goal of this review is to elaborate on the clinicopathologic characteristics and underline the key histologic features that best facilitate the diagnosis of dVIN.
Topics: Carcinoma in Situ; Carcinoma, Squamous Cell; Female; Humans; Vulvar Neoplasms
PubMed: 30640512
DOI: 10.5858/arpa.2018-0019-RS -
Current Opinion in Oncology Sep 2022Studies on treatment options for patients with locally advanced vulvar cancer (LAVC) are scarce, and high-level evidence for a primary treatment choice is lacking.... (Review)
Review
PURPOSE OF REVIEW
Studies on treatment options for patients with locally advanced vulvar cancer (LAVC) are scarce, and high-level evidence for a primary treatment choice is lacking. Furthermore, current treatment options are associated with extensive morbidity and high complication rates. More effective treatment options are urgently needed. This review describes current treatment possibilities, focusing on literature regarding neoadjuvant chemotherapy (NACT) followed by surgery.
RECENT FINDINGS
Although data are heterogeneous and limited, NACT followed by surgery might be an effective and well tolerated treatment alternative associated with lower morbidity compared with current treatment options, such as excenterative surgery or definitive chemoradiation.
SUMMARY
Up until now, several studies describe an overall response rate of 40-86%. Surgery turned out to be possible in 40-90% of the LAVC patients who received NACT. Prospective studies on the efficacy and safety of NACT followed by surgery with a homogeneous chemotherapy regimen are urgently awaited. NACT should, at this point, still be considered investigational.
Topics: Antineoplastic Combined Chemotherapy Protocols; Chemoradiotherapy; Chemotherapy, Adjuvant; Female; Humans; Neoadjuvant Therapy; Prospective Studies; Vulvar Neoplasms
PubMed: 35943437
DOI: 10.1097/CCO.0000000000000861 -
International Journal of Gynecological... Mar 2022Vulvar cancer is rare, and unresectable disease provides a therapeutic conundrum. Although definitive surgery remains the mainstay for curative treatment of vulvar... (Review)
Review
Vulvar cancer is rare, and unresectable disease provides a therapeutic conundrum. Although definitive surgery remains the mainstay for curative treatment of vulvar cancer, a minority of patients present with advanced disease for which surgical resection would be extraordinarily morbid. Pre-operative and definitive radiation with radiosensitizing systemic therapy allows such patients an opportunity for cure. In this review, we explore the origins of pre-operative radiation, current treatment standards for pre-operative and definitive chemoradiation, and future directions.
Topics: Chemoradiotherapy; Female; Humans; Vulvar Neoplasms
PubMed: 35256420
DOI: 10.1136/ijgc-2021-002530 -
Critical Reviews in Oncology/hematology Oct 2016Recurrent disease occurs in 12-37% of patients with vulvar squamous cell carcinoma (VSCC). Decisions about treatment of recurrent VSCC mainly depend on the location of... (Review)
Review
Recurrent disease occurs in 12-37% of patients with vulvar squamous cell carcinoma (VSCC). Decisions about treatment of recurrent VSCC mainly depend on the location of the recurrence and previous treatment, resulting in individualized and consensus-based approaches. Most recurrences (40-80%) occur within 2 years after initial treatment. Currently, wide local excision is the treatment of choice for local recurrences. Isolated local recurrence of VSCC has a good prognosis, with reported 5-year survival rates of up to 60%. Groin recurrences and distant recurrences are less common and have an extremely poor prognosis. For groin recurrences, surgery with or without (chemo) radiotherapy is a treatment option, depending on prior treatment. For distant recurrences, there are only palliative treatment options. In this review, we give an overview of the available literature and discuss epidemiology, risk factors, and prognostic factors for the different types of recurrent VSCC and we describe treatment options and clinical outcome.
Topics: Carcinoma, Squamous Cell; Female; Humans; Neoplasm Recurrence, Local; Retrospective Studies; Risk Factors; Survival Rate; Vulvar Neoplasms
PubMed: 27637349
DOI: 10.1016/j.critrevonc.2016.07.007 -
Current Oncology Reports Oct 2016The treatment of patients with vulvar cancer remains challenging for gynecologic oncologists. Up to 30 % of the cases are diagnosed in a clinical condition of... (Review)
Review
The treatment of patients with vulvar cancer remains challenging for gynecologic oncologists. Up to 30 % of the cases are diagnosed in a clinical condition of irresectability, and some kind of strategy has to be taken into account beyond surgery. In this regard, a common and standard definition is critical to maximize oncological results and minimize complications after treatments. Each patient treatment must be tailored individually according to their clinical and biological features and to the setting in which they are dealing with.
Topics: Antineoplastic Combined Chemotherapy Protocols; Carcinoma, Squamous Cell; Combined Modality Therapy; Female; Gynecologic Surgical Procedures; Humans; Lymph Node Excision; Lymphatic Metastasis; Neoadjuvant Therapy; Neoplasm Invasiveness; Pelvic Exenteration; Vulva; Vulvar Neoplasms
PubMed: 27586378
DOI: 10.1007/s11912-016-0545-6 -
International Journal of... Jun 2015Malignant tumors of the female reproductive system are a serious health and social problem, as they are the second most common cause of death among women, after breast... (Review)
Review
Malignant tumors of the female reproductive system are a serious health and social problem, as they are the second most common cause of death among women, after breast cancer. Vulvar tumors represent only 4% of all gynecological neoplasms, and they are fourth in frequency after tumors of the cervix, uterus, and ovary. Ninety-eight percent of all vulvar tumors are benign and only 2% are malignant. Sarcomas of the vulva comprise approximately 1-3% of all vulvar cancers. They are characterized by rapid growth, high metastatic potential, frequent recurrences, aggressive behavior, and high mortality rate. In Part 1 of this paper, we presented the most common forms of sarcoma of the vulva: leiomyosarcoma, epithelioid sarcoma, malignant rhabdoid tumor, and rhabdomyosarcoma. The second part of this review will focus mainly on the rarest variants of vulvar sarcoma: low-grade fibromyxoid sarcoma, synovial sarcoma, monophasic synovial sarcoma, carcinosarcoma, Ewing sarcoma, myeloid sarcoma, dermatofibrosarcoma protuberans, malignant fibrous histiocytoma, angiomatoid fibrous histiocytoma, liposarcoma, malignant peripheral nerve sheath tumor, and malignant mesothelioma.
Topics: Female; Humans; Neoplasm Recurrence, Local; Sarcoma; Vulvar Neoplasms
PubMed: 25816393
DOI: 10.1177/0394632015575977