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BMJ Case Reports Aug 2021Aggressive angiomyxoma is a rare and locally aggressive mesenchymal tumour, predominantly occurring in women of reproductive age group. The term aggressive is attributed...
Aggressive angiomyxoma is a rare and locally aggressive mesenchymal tumour, predominantly occurring in women of reproductive age group. The term aggressive is attributed to the infiltrative nature and frequent local recurrences. They arise commonly from the vulvovaginal region, perineum or pelvis and are usually misdiagnosed as other common entities in these regions. Radiological investigations aid in the diagnosis and planning of surgery. However, the final diagnosis in most of the cases is established by histopathological examination. We herein report a case of a middle-aged woman presenting with recurrent large right vulvar mass highlighting the surgical challenges posed by its intrapelvic extension.
Topics: Female; Humans; Middle Aged; Myxoma; Neoplasm Recurrence, Local; Pelvis; Perineum; Vulvar Neoplasms
PubMed: 34404643
DOI: 10.1136/bcr-2020-240687 -
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. Their incidence has increased dramatically during recent years, probably due to the different sexual habits and changes in the prevalence of HIV/ AIDS and HPV virus carriers, among other factors. 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. The overall incidence of tumors with vulvar location is between two and seven cases per 100,000 women, and it increases with age, while the death rate is estimated at 0.7 per 100,000 women. Sarcomas of the vulva comprise approximately 1-3% of all vulvar cancers, with leiomyosarcomas, epithelioid sarcomas, and rhabdomyosarcomas being the most common among them. They are characterized by rapid growth, high metastatic potential, frequent recurrences, aggressive behavior, and high mortality rate. In this paper, we present the most common forms of sarcomas of the vulva (leiomyosarcoma, epithelioid sarcoma, malignant rhabdoid tumor, rhabdomyosarcoma) in order to emphasize the broad differential diagnosis, rare appearance, non-specific clinical picture, aggressive course, and high mortality.
Topics: Diagnosis, Differential; Female; Humans; Neoplasm Recurrence, Local; Sarcoma; Vulvar Neoplasms
PubMed: 25816394
DOI: 10.1177/0394632015576029 -
Obstetrical & Gynecological Survey Jan 2021Vulvar intraepithelial neoplasia (VIN) represents an increasingly common, yet challenging diagnosis that shares many common risk factors with cervical intraepithelial... (Review)
Review
IMPORTANCE
Vulvar intraepithelial neoplasia (VIN) represents an increasingly common, yet challenging diagnosis that shares many common risk factors with cervical intraepithelial neoplasia. However, unlike cervical intraepithelial neoplasia, effective screening and treatment strategies are much less defined for patients with VIN.
OBJECTIVE
The objective of this article is to review the underlying risk factors leading to the development of VIN, identify special populations at risk for VIN, and outline acceptable treatment strategies.
EVIDENCE ACQUISITION
This literature review was performed primarily using PubMed.
RESULTS
Vulvar intraepithelial neoplasia can be separated into usual VIN (uVIN) and differentiated VIN (dVIN). The more common uVIN is related to underlying human papillomavirus infection, whereas dVIN occurs in the setting of other vulvar inflammatory conditions such as lichen sclerosis. Differentiated VIN carries a higher risk of progression to invasive malignancy. Extramammary Paget disease is a rare intraepithelial adenocarcinoma unrelated to uVIN and dVIN, although management is similar.
CONCLUSIONS AND RELEVANCE
Vulvar intraepithelial neoplasia is a preinvasive neoplasia of the vulva with few robust strategies for surveillance or management. Careful examination with targeted biopsy is warranted for suspicious lesions, and a combination of surgical and medical management can be tailored for individual patient needs.
Topics: Carcinoma in Situ; Disease Management; Female; Humans; Papillomaviridae; Papillomavirus Infections; Risk Factors; Vulva; Vulvar Lichen Sclerosus; Vulvar Neoplasms
PubMed: 33506879
DOI: 10.1097/OGX.0000000000000857 -
Dermatologic Surgery : Official... Jan 2017Hidradenitis suppurativa (HS) is a chronic inflammatory disease that frequently involves the perineal and perianal regions. The association between HS and malignant... (Review)
Review
BACKGROUND
Hidradenitis suppurativa (HS) is a chronic inflammatory disease that frequently involves the perineal and perianal regions. The association between HS and malignant transformation is a rare but under recognized phenomenon.
OBJECTIVE
This systematic review aims to summarize all available cases of vulvar and perianal/perineal cancer emerging in patients with HS, describing clinical and therapeutic particularities of these coexisting conditions in female patients.
MATERIALS AND METHODS
This systematic review and pooled analysis was performed in accordance with the PRISMA guidelines; end-of-search date was June 15, 2015.
RESULTS
A total of 13 eligible articles were identified; 7 cases of vulvar cancer and 6 cases of perineal/perianal carcinomas in patients with HS were noted. A majority of published cases pertained to rather advanced carcinomas; only occasionally early stage carcinomas were identified. The optimal modifications in the treatment scheme of vulvar, perianal, and perineal cancer in patients with HS have not been established; detailed reporting of recurrence- and survival-related aspects is advised.
CONCLUSION
Vulvar, perianal, and perineal cancer represent a rare but serious complication of HS.
Topics: Buttocks; Carcinoma, Squamous Cell; Female; Hidradenitis Suppurativa; Humans; Perineum; Skin Neoplasms; Vulvar Neoplasms
PubMed: 27755170
DOI: 10.1097/DSS.0000000000000944 -
Systematic Reviews Apr 2021Globally, cancer is generally recognized as a developmental threat yet most countries in Africa lack capacity to diagnose cancer especially gynecological cancers...
BACKGROUND
Globally, cancer is generally recognized as a developmental threat yet most countries in Africa lack capacity to diagnose cancer especially gynecological cancers resulting in late detection and poor outcomes. However, most studies on gynecological cancers in Africa tend to focus on cervical cancer compared to the other gynecological cancers. Therefore, this scoping review will aim to describe the existing literature on the epidemiological burden of ovarian, endometrial, vaginal, and vulva cancers, their risk factors, and potential screening methods/techniques in Africa to identify priority research gaps for further research to inform health policy decisions.
METHODS
The framework promulgated by Arksey and O'Malley and improved by Levac et al. will be used as a guide for this scoping review. A comprehensive search for relevant published studies in PubMed, CINAHL, SCOPUS, Google Scholar, and ScienceDirect with no date limitation to the last search date. The database search strategy will include keywords, Boolean operators, and medical subject heading terms. We will additionally consult the WHO/IARC website, IHME/Global Burden of Disease Study. A snowball approach will also be used to search the reference list of all included studies to obtain relevant papers for possible inclusion in this review. We will include articles that involve African countries, focused on ovarian, endometrial, vaginal, and vulva cancers, their risk factors, and potential screening methods/techniques in any language. We will exclude studies on cervical cancer and other cancers as well as review articles. The abstracts and full-text selection will be conducted by two independent reviewers using this review's eligibility criteria as a guide. All the review selection tools, and the data extraction form will be pilot tested for accuracy and consistency. The data will be organized into thematic areas, summarized and the results communicated narratively.
DISCUSSION
It is anticipated that this review will reveal important literature gaps to guide future research to inform health policy decisions about ovarian, endometrial, and rare gynecological neoplasms in Africa. This review's findings will be disseminated via peer review journals, conferences, and other social media such Twitter and LinkedIn.
Topics: Africa; Female; Humans; Research; Review Literature as Topic; Vulvar Neoplasms
PubMed: 33849664
DOI: 10.1186/s13643-021-01654-0 -
Oncology 2020Background and summary: Among all vulvar cancers, primary adenoid cystic carcinoma (ACC) of Bartholin's gland is a very rare tumor characterized by a slow growth, a high... (Review)
Review
Background and summary: Among all vulvar cancers, primary adenoid cystic carcinoma (ACC) of Bartholin's gland is a very rare tumor characterized by a slow growth, a high local aggressiveness, and a remarkable recurrence rate. Due to its rarity, treatment remains a challenge for oncologists and gynecological surgeons. Key message: The present paper reports clinical, radiological, and histological features of ACC of Bartholin's gland and reviews the literature data on the treatment options with a particular focus on the potential role of particle radiation therapy.
Topics: Bartholin's Glands; Carcinoma, Adenoid Cystic; Female; Heavy Ion Radiotherapy; Humans; Neoplasm Recurrence, Local; Prognosis; Rare Diseases; Risk Factors; Vulvar Neoplasms
PubMed: 32408297
DOI: 10.1159/000506485 -
Annals of Surgical Oncology 2015Primary melanomas of the vulva and vagina are rare. As a result, it has been difficult to develop evidence-based guidelines for their management. By analyzing a large... (Comparative Study)
Comparative Study
BACKGROUND
Primary melanomas of the vulva and vagina are rare. As a result, it has been difficult to develop evidence-based guidelines for their management. By analyzing a large series of patients with vulval and vaginal melanomas, this study sought to document the most common presenting features, identify clinical and pathologic predictors of outcome, and provide management guidelines.
METHODS
A clinicopathologic analysis of 85 patients with primary melanomas of the vulva or vagina diagnosed and treated at Melanoma Institute Australia and associated units in Sydney, Australia, between 1960 and 2011 was performed. Predictors of disease-free and melanoma-specific survival (MSS) were determined.
RESULTS
Patients with American Joint Committee on Cancer (AJCC) stage 0-II had a significantly better MSS (5Y MSS = 63.6 %, n = 59) compared with those with stage III disease (5Y MSS = 0 %, n = 12, p < 0.001). Tumor thickness, ulceration status, and pathologic clearance margin were significant predictors of MSS. Disease-free survival was predicted by these factors and additionally by tumor mitotic rate.
CONCLUSIONS
The results of this study provide evidence to support the appropriateness of utilizing the AJCC (7th edition) cutaneous melanoma staging system for vulval and vaginal melanomas. Detection and removal of these melanomas at an early stage with pathologically confirmed clear margins confers the best chance of cure.
Topics: Adult; Aged; Aged, 80 and over; Disease Management; Female; Follow-Up Studies; Humans; Melanoma; Middle Aged; Neoplasm Recurrence, Local; Neoplasm Staging; Prognosis; Skin Neoplasms; Survival Rate; Vaginal Neoplasms; Vulvar Neoplasms; Young Adult; Melanoma, Cutaneous Malignant
PubMed: 25384702
DOI: 10.1245/s10434-014-4215-3 -
Seminars in Diagnostic Pathology Jan 2021
Review
Topics: Female; Humans; Vulva; Vulvar Neoplasms
PubMed: 33032904
DOI: 10.1053/j.semdp.2020.09.008 -
Akusherstvo I Ginekologiia 2015The term vulvar intraepithelial neoplasia (VIN) was introduced for first time in 1986 year from the International Society for the Study of Vulvar Disease (ISSVD). With... (Review)
Review
The term vulvar intraepithelial neoplasia (VIN) was introduced for first time in 1986 year from the International Society for the Study of Vulvar Disease (ISSVD). With this term are denoted precancer vulvar conditions. According to a classification dated 1986 depending on the degree of affection of multilayered squamous epithelial vulvar precancerous lesion are subdivided into three groups: VINI, VIN2 and VIN3. Subsequently VIN1 is determinate as a lesion which there isn't oncogenic potential. These types of changes often are result from irritation or viral infection which leads to benign condylomata acuminate. Compare to lesions with VIN require histological signs for high grade intraepithelial neoplasia as nuclear pleomorphism, increased mitotic activity, atypical mitotic activity and disordered architecture of squamous cells epithelium. These fundamental morphologic characterizations lead to revision and subsequent change of the current classification. In 2004 ISSVD changed the classification. It included lesions like VIN2 and VIN3, but they are subdivided in two groups: the usual type VIN and the differentiated VIN. They have different etiology, morphology, oncogenic potential and prognosis. The usual type VIN is associated with infection of high risk types of human papilloma virus and is a more frequently met form of VIN. A very good prognosis is characteristic for it. The differentiated VIN is met in postmenopausal women of about 70 with frequency of about 2-5%. It originated from vulvar dermatosis like lichen scierosus and there is a high oncogenic potential and worse prognosis. The treatment of VIN may be surgical and by medicines. The frequency of recurrences after treatment is 30-50% which required frequently follows up. The aim of this literature review is to introduce present terminological classification of VIN, as well as basic clinical, diagnostically and curative methods in treat of the both types of this precancerous.
Topics: Carcinoma in Situ; Female; Humans; Prognosis; Vulva; Vulvar Neoplasms
PubMed: 27025107
DOI: No ID Found -
International Journal of Gynecological... Sep 2020This study aimed to analyze the prognostic factors for overall and progression-free survival in patients with vulvar cancer.
OBJECTIVE
This study aimed to analyze the prognostic factors for overall and progression-free survival in patients with vulvar cancer.
METHODS
This international, multicenter, retrospective study included 2453 patients diagnosed with vulvar cancer at 100 different institutions. Inclusion criteria were institutional review board approval from each collaborating center, pathologic diagnosis of invasive carcinoma of the vulva, and primary treatment performed at the participating center. Patients with intraepithelial neoplasia or primary treatment at non-participating centers were excluded. Global survival analysis and squamous cell histology subanalysis was performed.
RESULTS
After excluding patients due to incomplete data entry, 1727 patients treated for vulvar cancer between January 2001 and December 2005 were registered for analysis (1535 squamous, 42 melanomas, 38 Paget's disease and 112 other histologic types). Melanomas had the worse prognosis (p=0.02). In squamous vulvar tumors, independent factors for increase in local recurrence of vulvar cancer were: no prior radiotherapy (p<0.001) or chemotherapy (p=0.006), and for distant recurrence were the number of positive inguinal nodes (p=0.025), and not having undergone lymphadenectomy (p=0.03) or radiotherapy (p<0.001), with a HR of 1.1 (95% CI 1.2 to 1.21), 2.9 (95% CI 1.4 to 6.1), and 3.1 (95% CI 1.7 to 5.7), respectively. Number of positive nodes (p=0.008), FIGO stage (p<0.001), adjuvant chemotherapy (p=0.001), tumor resection margins (p=0.045), and stromal invasion >5 mm (p=0.001) were correlated with poor overall survival, and large case volume (≥9 vs <9 cases per year) correlated with more favorable overall survival (p=0.05).
CONCLUSIONS
Advanced patient age, number of positive inguinal lymph nodes, and lack of adjuvant treatment are significantly associated with a higher risk of relapse in patients with squamous cell vulvar cancer. Case volume per treating institution, FIGO stage, and stromal invasion appear to impact overall survival significantly. Future prospective trials are warranted to establish these prognostic factors for vulvar cancer.
Topics: Aged; Female; Humans; Prognosis; Retrospective Studies; Survival Analysis; Vulvar Neoplasms
PubMed: 32571891
DOI: 10.1136/ijgc-2019-000526