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International Journal of Gynaecology... Oct 2021Vulvar cancer is an uncommon gynecological malignancy primarily affecting postmenopausal women. There is no specific screening and the most effective strategy to reduce...
Vulvar cancer is an uncommon gynecological malignancy primarily affecting postmenopausal women. There is no specific screening and the most effective strategy to reduce vulvar cancer incidence is the opportune treatment of predisposing and preneoplastic lesions associated with its development. While vulvar cancer may be asymptomatic, most women present with vulvar pruritus or pain, or have noticed a lump or ulcer. Therefore, any suspicious vulvar lesion should be biopsied to exclude invasion. Once established, the most common subtype is squamous cell carcinoma. Treatment of vulvar cancer depends primarily on histology and surgical staging. Treatment is predominantly surgical, particularly for squamous cell carcinoma, although concurrent chemoradiation is an effective alternative, particularly for advanced tumors. Management should be individualized and carried out by a multidisciplinary team in a cancer center experienced in the treatment of these tumors.
Topics: Carcinoma, Squamous Cell; Chemoradiotherapy; Female; Humans; Neoplasm Staging; Vulva; Vulvar Neoplasms
PubMed: 34669204
DOI: 10.1002/ijgo.13881 -
Seminars in Diagnostic Pathology Jan 2021Extramammary Paget disease (EMPD) is a rare neoplasm with uncertain histogenesis, usually presenting in the anogenital area, most commonly in the vulva. The disease is... (Review)
Review
Extramammary Paget disease (EMPD) is a rare neoplasm with uncertain histogenesis, usually presenting in the anogenital area, most commonly in the vulva. The disease is characterized by slow grow and high recurrence rates. This article reviews the epidemiological, clinical, morphological, genetic and treatment features of EMPD of the vulva reported in recent years.
Topics: Diagnosis, Differential; Female; Humans; Paget Disease, Extramammary; Prognosis; Vulvar Neoplasms
PubMed: 32921571
DOI: 10.1053/j.semdp.2020.09.007 -
Critical Reviews in Oncology/hematology May 2016In this review, we provide an overview of the clinical aspects, histopathology, molecular genetics, and treatment options for Vulvar Paget's Disease (VPD), a rare skin... (Review)
Review
In this review, we provide an overview of the clinical aspects, histopathology, molecular genetics, and treatment options for Vulvar Paget's Disease (VPD), a rare skin disease, most commonly found in postmenopausal Caucasian women. The underlying cause of VPD remains not well understood. VPD is rarely associated with an underlying urogenital, gastrointestinal or vulvar carcinoma. In approximately 25% of the cases, VPD is invasive; in these cases, the prognosis is worse than in non-invasive cases. Recurrence rates in invasive VPD are high: 33% in cases with clear margins, and even higher when surgical margins are not clear, regardless of invasion. Historically, surgical excision has been the treatment of choice. Recent studies show that imiquimod cream may be an effective and safe alternative.
Topics: Diagnosis, Differential; Female; Humans; Paget Disease, Extramammary; Prognosis; Tumor Microenvironment; Vulvar Neoplasms
PubMed: 26971063
DOI: 10.1016/j.critrevonc.2016.03.008 -
International Journal of Gynaecology... Oct 2015
Topics: Female; Humans; Mass Screening; Neoplasm Staging; Practice Guidelines as Topic; Vulvar Neoplasms
PubMed: 26433678
DOI: 10.1016/j.ijgo.2015.06.002 -
Ugeskrift For Laeger Aug 2019This review summarises the current guidelines for vulva cancer in Denmark. Vulva cancer is a rare disease. The diagnosis is often delayed, which results in large tumours... (Review)
Review
This review summarises the current guidelines for vulva cancer in Denmark. Vulva cancer is a rare disease. The diagnosis is often delayed, which results in large tumours and regional spread. The most important prognostic factor is inguinal lymph node metastases. Staging and treatment is centralised to two hospitals. Primary treatment is wide local excision combined with removal of either inguinal sentinel nodes or lymphadenectomy. Treatment is associated with considerable morbidity, and supportive care is often necessary. Local curable recurrences are common. Relapses in the groin are associated with a poor prognosis. Thus, long term follow-up is essential. *) On behalf of Dansk Gynækologisk Cancer Gruppe for vulvacancer.
Topics: Denmark; Female; Follow-Up Studies; Humans; Lymph Node Excision; Lymph Nodes; Lymphatic Metastasis; Neoplasm Recurrence, Local; Neoplasm Staging; Prognosis; Sentinel Lymph Node Biopsy; Vulvar Neoplasms
PubMed: 31495360
DOI: No ID Found -
International Journal of Gynecological... Mar 2022The primary treatment for resectable vulvar cancer includes wide local excision of the primary tumor and surgical lymph node assessment. Following surgery, up to 40-50%... (Review)
Review
The primary treatment for resectable vulvar cancer includes wide local excision of the primary tumor and surgical lymph node assessment. Following surgery, up to 40-50% of patients develop a local recurrence. Historically, the strongest predictor of local recurrence is a positive or close margin (defined as <8 mm), although recent studies question the importance of margin status. Post-operative radiotherapy to the vulva is recommended for all women with a positive margin where re-excision is not possible. Radiotherapy may also be considered in the setting of risk factors for local recurrence: close margin, lymphovascular invasion, large tumor size, and/or depth of invasion >5 mm. Nodal assessment is an important component of vulvar cancer management. A negative sentinel node is associated with a low false-negative predictive value (2% in patients with vulvar tumor <4 cm in GOG 173), 2-year groin recurrence rate of 2.3%, and 3-year disease-specific survival rate of 97% in patients with unifocal vulvar tumor <4 cm in the GROningen INternational Study on Sentinel nodes in Vulvar Cancer (GROINSS-V I) study. Thus, patients with tumor size <4 cm (without additional local risk factors) and negative sentinel node can be observed. Patients with sentinel node metastasis ≤2 mm can be treated with post-operative radiotherapy (2-year isolated groin recurrence rate of 1.6% in GROINSS-V II), as a safe alternative to lymphadenectomy. Patients with sentinel node metastasis >2 mm following sentinel node biopsy should undergo inguinofemoral lymphadenectomy followed by post-operative radiotherapy-based on the GROINSS-V II study, the 2-year isolated groin recurrence rate remains unacceptably high (22%) with radiotherapy alone. Retrospective studies suggest that the addition of concurrent chemotherapy to radiotherapy may improve survival. The ongoing GROINSS-V III study is investigating concurrent chemotherapy and radiotherapy dose escalation. The main goal of these post-operative treatments is to reduce the risk of local, and especially groin, recurrences, which are almost universally fatal.
Topics: Female; Humans; Lymph Node Excision; Lymph Nodes; Lymphadenopathy; Lymphatic Metastasis; Neoplasm Recurrence, Local; Neoplasm Staging; Retrospective Studies; Sentinel Lymph Node Biopsy; Vulvar Neoplasms
PubMed: 35256421
DOI: 10.1136/ijgc-2021-002463 -
JAAPA : Official Journal of the... Mar 2019Vulvar Paget disease (VPD) is an unusual and rare adenocarcinoma that can be challenging to diagnose. Although the condition has a high treatment success rate, it can be...
Vulvar Paget disease (VPD) is an unusual and rare adenocarcinoma that can be challenging to diagnose. Although the condition has a high treatment success rate, it can be associated with underlying malignancy, recurrence rates can be high, and patients often need regular and frequent follow-up. The article describes a woman whose nonresolving, pruritic vulvar lesion was found to be VPD on biopsy.
Topics: Adenocarcinoma; Female; Follow-Up Studies; Humans; Middle Aged; Neoplasm Recurrence, Local; Paget Disease, Extramammary; Treatment Outcome; Vulvar Neoplasms
PubMed: 30817477
DOI: 10.1097/01.JAA.0000550285.65463.a9 -
Journal of Gynecology Obstetrics and... Jan 2021Paget's disease of the vulva is a rare form of extramammary Paget's disease mainly affecting postmenopausal women. Its pathophysiology remains largely unknown. Up to...
Paget's disease of the vulva is a rare form of extramammary Paget's disease mainly affecting postmenopausal women. Its pathophysiology remains largely unknown. Up to fairly recently, the only treatment for this disease was surgery, often mutilating the vulva, with significant psychosexual repercussions without the assurance of complete therapeutic efficacy. New therapeutic approaches -topical treatments, radiotherapy or chemotherapy- have emerged in recent years but lack consensual guidelines. We present a literature review of the recent results published in this field.
Topics: Administration, Topical; Antineoplastic Agents; Diagnosis, Differential; Female; Humans; Imiquimod; Lasers, Gas; Neoplasm Invasiveness; Neoplasm Recurrence, Local; Paget Disease, Extramammary; Photochemotherapy; Prognosis; Radiotherapy Dosage; Vulvar Neoplasms
PubMed: 32828871
DOI: 10.1016/j.jogoh.2020.101896 -
Dermatologic Surgery : Official... Jun 2017Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade cutaneous neoplasm with a low propensity for metastasis and a high rate of local recurrence. It... (Review)
Review
BACKGROUND
Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade cutaneous neoplasm with a low propensity for metastasis and a high rate of local recurrence. It typically presents as a dermal plaque or nodule on the trunk, limbs, or head and neck region. Vulvar DFSP has also been described, although it is less common.
OBJECTIVE
To review the available literature and discuss the clinical course of DFSP affecting the vulva.
MATERIALS AND METHODS
We reviewed the existing English-language literature on DFSP of the vulva with respect to clinical presentation, diagnosis, treatment, and outcome.
RESULTS
Thirty three case reports and series were included (n = 54 patients). Vulvar DFSP most commonly presents as a slowly enlarging tender or asymptomatic mass on the labia majora, with histological findings of classic DFSP. Most patients were treated with wide local excision. Three patients were treated with Mohs micrographic surgery, which may decrease local recurrence and seems well suited for use in vulvar DFSP.
CONCLUSION
This literature review comprehensively reviews and describes the clinical presentation of vulvar DFSP and the treatment options for this rare vulvar neoplasm.
Topics: Dermatofibrosarcoma; Female; Humans; Skin Neoplasms; Vulvar Neoplasms
PubMed: 28323651
DOI: 10.1097/DSS.0000000000001113 -
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