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Journal of Cancer Research and... 2010Sarcomas of the vulva account for only 1-3% of all vulvar malignancies. Most common vulvar sarcomas are leiomyosarcomas, malignant fibrohistiocytomas, and aggressive...
Sarcomas of the vulva account for only 1-3% of all vulvar malignancies. Most common vulvar sarcomas are leiomyosarcomas, malignant fibrohistiocytomas, and aggressive angiomyxomas. Malignant rhabdoid tumor (MRT) of the kidney is a distinctive clinicopathological entity that is recognized as a highly aggressive renal tumor of childhood. Extrarenal malignant rhabdoid tumors have been proposed to exist at several sites, including soft parts. MRT of the vulva is a rare and very aggressive neoplasm. Median survival reported in other studies is 9 months. Only 10 cases have been reported thus far in the English literature. We are reporting the 11 th case who remains disease free 30 months following surgery and radiotherapy till the time of reporting.
Topics: Female; Gynecologic Surgical Procedures; Humans; Middle Aged; Radiotherapy, Adjuvant; Rhabdoid Tumor; Vulvar Neoplasms
PubMed: 20479553
DOI: 10.4103/0973-1482.63557 -
Revista Brasileira de Ginecologia E... Jun 2019Extramammary Paget disease is a rare neoplastic condition that more commonly affects postmenopausal Caucasian women. Although the vulvar area is the most frequently...
Extramammary Paget disease is a rare neoplastic condition that more commonly affects postmenopausal Caucasian women. Although the vulvar area is the most frequently affected location, it corresponds solely to 1 to 2% of all vulvar malignancies. A 72-year-old female patient was observed in our outpatient clinic with a 2-year history of an erythematous and pruritic plaque on the vulva. Histopathology and immunohistochemistry studies were compatible with extramammary Paget disease of the vulva. Associated neoplastic conditions were excluded. Due to multiple relapses, the patient was submitted to three surgical interventions, including a total vulvectomy, and to external radiotherapy. The present case illustrates the chronic and recurrent nature of extramammary Paget disease despite aggressive procedures as well as the challenge in obtaining tumor-free resection margins.
Topics: Aged; Antineoplastic Agents; Female; Humans; Imiquimod; Neoplasm Recurrence, Local; Paget Disease, Extramammary; Plastic Surgery Procedures; Treatment Outcome; Vulvar Neoplasms; Vulvectomy
PubMed: 31018229
DOI: 10.1055/s-0039-1687861 -
Cancer Oct 1983The authors describe two cases of epithelioid sarcoma of the vulva and summarize the clinical and pathologic experience with this vulvar neoplasm. Findings indicate that... (Comparative Study)
Comparative Study Review
The authors describe two cases of epithelioid sarcoma of the vulva and summarize the clinical and pathologic experience with this vulvar neoplasm. Findings indicate that epithelioid sarcoma of the vulva usually occurs in the labia majora of young women, but may occur in later life. Clinically it may mimic a Bartholin's duct cyst, thus leading to inadequate treatment. Pathologic findings are characterized by sheets and nests of acidophilic, polygonal cells. Follow-up study suggests that vulvar epithelioid sarcoma behaves more aggressively than extragenital epithelioid sarcoma. Four of five patients died of metastatic sarcoma. The courses of three patients were quite rapid, whereas a fourth patient had multiple recurrences over a long period, which is typical of extragenital epithelioid sarcoma. Complete initial excision is important. All patients with local recurrence died with distant metastases. Vascular invasion also indicates a poor outcome.
Topics: Adult; Bartholin's Glands; Cysts; Female; Humans; Microscopy, Electron; Middle Aged; Neoplasm Metastasis; Prognosis; Sarcoma; Vulvar Neoplasms
PubMed: 6352000
DOI: 10.1002/1097-0142(19831015)52:8<1462::aid-cncr2820520821>3.0.co;2-y -
American Journal of Obstetrics and... Jan 1999Our aim was to determine the prevalence of associated vulvar adenocarcinoma, invasive Paget's disease, and recurrence of Paget's disease of the vulva. (Review)
Review
OBJECTIVE
Our aim was to determine the prevalence of associated vulvar adenocarcinoma, invasive Paget's disease, and recurrence of Paget's disease of the vulva.
STUDY DESIGN
A retrospective review of tumor and pathology registries at 8 institutions is presented. Patients with recurrent disease were excluded. Histologic slide review was performed.
RESULTS
The median age of the 100 patients was 70 years. The median duration of pruritus before surgery was 2 years. Thirty-four percent of patients experienced a recurrence at a median of 3 years. There was a 12% prevalence of invasive vulvar Paget's disease and a 4% prevalence of associated vulvar adenocarcinoma. One patient died of Paget's disease with associated vulvar adenocarcinoma.
CONCLUSIONS
Paget's disease of the vulva is rarely associated with an underlying vulvar adenocarcinoma or invasive Paget's disease, but there is a high recurrence rate.
Topics: Adenocarcinoma; Adult; Aged; Female; Humans; Middle Aged; Neoplasm Invasiveness; Neoplasm Recurrence, Local; Paget Disease, Extramammary; Registries; Retrospective Studies; Vulvar Neoplasms
PubMed: 9914572
DOI: 10.1016/s0002-9378(99)70143-2 -
Gynakologisch-geburtshilfliche Rundschau 2009Paget disease is a rare disorder of the skin of the vulva, comprising less than 1% of vulvar neoplasms. Surgical excision is considered the standard of care. While the... (Review)
Review
Paget disease is a rare disorder of the skin of the vulva, comprising less than 1% of vulvar neoplasms. Surgical excision is considered the standard of care. While the invasive variant of Paget disease is rare, frequent local recurrences from non-invasive disease, which are inoperable, constitute a major clinical problem. In this article we report on a 71-year-old patient with recurrent disease treated successfully with imiquimod cream. Symptoms such as local itching subsided shortly after initiation of treatment. Four weeks later, first signs of resolution could be demonstrated colposcopically. Within 8 weeks, complete clinical remission was achieved. Thus, topical imiquimod is not only effective as treatment of vulvar intraepithelial neoplasia, but may also be used for selected patients with recurrent Paget disease as an alternative to repeat surgical interventions.
Topics: Administration, Topical; Aged; Aminoquinolines; Antineoplastic Agents; Chemotherapy, Adjuvant; Colposcopy; Combined Modality Therapy; Female; Follow-Up Studies; Humans; Imiquimod; Laser Therapy; Neoplasm Recurrence, Local; Paget Disease, Extramammary; Vulva; Vulvar Neoplasms
PubMed: 20530949
DOI: 10.1159/000301110 -
Gynecologic Oncology May 1984A retrospective analysis of 24 patients with early invasive squamous carcinoma was performed. No nodal metastases were noted in these patients. Based on a review of the... (Comparative Study)
Comparative Study Review
A retrospective analysis of 24 patients with early invasive squamous carcinoma was performed. No nodal metastases were noted in these patients. Based on a review of the literature, no absolute definition of microinvasive carcinoma could be formulated, but a treatment outline has been formulated based on depth of invasion for Stage I lesions.
Topics: Adult; Aged; Carcinoma, Squamous Cell; Female; Humans; Lymph Node Excision; Middle Aged; Neoplasm Invasiveness; Neoplasm Metastasis; Neoplasm Recurrence, Local; Prognosis; Retrospective Studies; Vulvar Neoplasms
PubMed: 6370797
DOI: 10.1016/0090-8258(84)90008-8 -
European Journal of Surgical Oncology :... Oct 2018The recommended pathological resection margin (8 mm) for vulvar squamous cell carcinoma (SCC) is broader than for SCC located elsewhere, and does not depend on tumor... (Observational Study)
Observational Study
INTRODUCTION
The recommended pathological resection margin (8 mm) for vulvar squamous cell carcinoma (SCC) is broader than for SCC located elsewhere, and does not depend on tumor grade or lesion size. Our aim is to evaluate the resection margin in vulvar SCC in relation to local recurrence, and to determine the impact of other prognostic factors.
MATERIALS AND METHODS
Data of all surgically treated patients at the Gynecological Oncology Center South with vulvar SCC, FIGO IB-IIIC, between 2005 and 2015 were analysed retrospectively. The relation between the pathological resection margin and other clinicopathological factors with the risk of local recurrence was analysed.
RESULTS
In this cohort of 167 patients, the tumor was radically removed in 87% of the patients. Yet, in 57% the pathological resection margin was <8 mm. Including re-excisions, the median closest margin was 7.0 mm. There was no significant difference in the risk of local recurrence for a resection margin <8 mm or ≥8 mm (25.0% (n = 20) and 22.2% (n = 16)), nor in the median resection margin of patients with or without local recurrence (6.5 mm and 7.0 mm). Lichen sclerosus was the only significant risk factor for local recurrence.
CONCLUSION
A pathological resection margin <8 mm was not related to an increased risk of local recurrence. The most important predictor of local recurrence was the presence of lichen sclerosus. A resection margin <8 mm in vulvar SCC can therefore be accepted, especially in tumors located close to clitoris, urethra or anus.
Topics: Adult; Aged; Aged, 80 and over; Carcinoma, Squamous Cell; Female; Humans; Margins of Excision; Middle Aged; Neoplasm Grading; Neoplasm Recurrence, Local; Neoplasm Staging; Retrospective Studies; Tumor Burden; Vulvar Lichen Sclerosus; Vulvar Neoplasms
PubMed: 29934053
DOI: 10.1016/j.ejso.2018.05.031 -
Annals of Oncology : Official Journal... Sep 2016The incidence of vulvar squamous cell carcinoma (VSCC) has been on the rise since the 1990s. There has been no new treatment for advanced and recurrent disease in... (Review)
Review
BACKGROUND
The incidence of vulvar squamous cell carcinoma (VSCC) has been on the rise since the 1990s. There has been no new treatment for advanced and recurrent disease in decades, with most women succumbing to the disease. Despite two distinct etiologies of VSCC, human papillomavirus (HPV)-associated and HPV-independent disease, there is no difference in therapeutic options.
METHODS
A literature review was carried out by searching EMBASE and Medline databases between January 1990 and March 2016 by pairing the keywords of vulvar carcinoma, vulva cancer, vulvar and vulva with molecular markers involved in the cell cycle, apoptosis and angiogenesis. Molecular targets of prognostic significance were identified and targeted agents of therapeutic relevance to both HPV-independent and HPV-associated VSCC were then reviewed.
RESULTS
Recent advances in our understanding of the molecular biology of VSCC provide insight into the future management of VSCC with molecular targeted therapies. Aberrant cell cycle activity is common in both HPV-associated and HPV-independent VSCC and is characterized by overexpression of p53, Rb and cyclin D1, supporting targeting of these protein products and their downstream pathways. Extracellular regulators of cellular activity, such as EGFR, as well as inhibitors of angiogenesis are being clinically evaluated in VSCC. HPV-independent VSCC is characterized by actionable mutations, including PI3K, CDKN2A and PTEN. In HPV-associated disease, therapeutic vaccines targeting the E6 and E7 HPV oncogenes and immune-based therapies are under investigation.
CONCLUSION
There has been a paucity of clinical trials in recent years in this neglected women's cancer. Directed therapy against cell cycle regulatory molecules and extracellular proteins and the inhibition of angiogenesis are of broad therapeutic relevance in VSCC. Therapeutic strategies that target actionable mutations should be explored. In HPV-associated VSCC, novel treatments that exploit the virology of HPV and/or enhance the host immune response merit further study.
Topics: Biomarkers, Tumor; Carcinoma, Squamous Cell; Female; Gene Expression Regulation, Neoplastic; Humans; Molecular Targeted Therapy; Mutation; Neoplasm Proteins; Papillomaviridae; Vulvar Neoplasms
PubMed: 27329249
DOI: 10.1093/annonc/mdw242 -
Journal of Clinical Pathology Nov 1984The pathological, cytological, and clinical features of vulvar intraepithelial neoplasia (VIN) are described. The rate of progression of VIN III to an invasive carcinoma... (Review)
Review
The pathological, cytological, and clinical features of vulvar intraepithelial neoplasia (VIN) are described. The rate of progression of VIN III to an invasive carcinoma is very low and spontaneous regression can occur. These features prevent the drawing of a direct analogy between vulvar and cervical intraepithelial neoplasia. The concept of microinvasive carcinoma of the vulva is discussed, and it is concluded that no satisfactory definition of this entity has been achieved.
Topics: Adult; Age Factors; Carcinoma in Situ; Carcinoma, Squamous Cell; Colposcopy; Epithelium; Female; Humans; Neoplasm Invasiveness; Sexually Transmitted Diseases; Vaginal Smears; Vulvar Neoplasms
PubMed: 6389601
DOI: 10.1136/jcp.37.11.1201 -
International Journal of Gynecological... Jan 2022Basal cell carcinoma (BCC) is the most frequent skin cancer but <1% of the cases develop in the vulva. Histoprognostic features of vulvar BCCs are not recognized and,...
Basal cell carcinoma (BCC) is the most frequent skin cancer but <1% of the cases develop in the vulva. Histoprognostic features of vulvar BCCs are not recognized and, consequently, the treatment of the disease is not well codified. To overcome this lack of knowledge, we have performed a retrospective analysis of vulvar BBCs to assess the value of various histological features regarding the disease outcome. In all, 19 patients surgically treated for a vulvar BCC in the Centre Hospitalier Intercommunal de Créteil from March 1, 2000 to September 26, 2019 were retrieved. Clinical and histologic features were reviewed in all cases and analyzed in comparison with disease recurrence and patient's survival. The median age of the patients was 74 (range 54-99) yr. Tumor location on the labium majus was the most frequent (68%). None presented with a medical condition related to BCC. All the patients were treated by surgery alone, except one who benefited from additional radiotherapy. We found a significant association between tumor size and recurrences (P=0.031). Other features associated with disease outcome were tumor thickness, treatment type, and surgical margins. Recurrence was observed for tumors larger than 20 mm with a surgical margin of less than 3 mm. A combination of tumor size, thickness, and surgical margin are histoprognostic factors more significant than tumor subtype.
Topics: Carcinoma, Basal Cell; Female; Humans; Neoplasm Recurrence, Local; Retrospective Studies; Skin Neoplasms; Vulvar Neoplasms
PubMed: 33770062
DOI: 10.1097/PGP.0000000000000771