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Cureus Mar 2024Adenoid cystic carcinoma (ACC) of the vulva represents a highly uncommon type of female malignancy. Due to the absence of specific treatment guidelines, such cases are...
Adenoid cystic carcinoma (ACC) of the vulva represents a highly uncommon type of female malignancy. Due to the absence of specific treatment guidelines, such cases are typically managed by the treatment protocols for vulvar cancer. Here, we report the case of a 52-year-old woman who presented with a painful right vulvar mass, leading to a diagnosis of ACC of the vulva after biopsy and immunohistochemical analysis. She underwent vulvectomy, bilateral inguinal lymphadenectomy, and targeted radiotherapy, and no evidence of recurrence has been found for three years, with ongoing monitoring for post-radiation effects. This case adds valuable insights into the management of ACC of the vulva and underscores the need for further research and guideline development to optimize care for future patients.
PubMed: 38606234
DOI: 10.7759/cureus.56048 -
Radiotherapy and Oncology : Journal of... Jun 2024The aim of the study is to examine the present status of reirradiation with high-dose-rate (HDR) brachytherapy for recurrent gynecologic cancer in Japan and to determine...
BACKGROUND AND PURPOSE
The aim of the study is to examine the present status of reirradiation with high-dose-rate (HDR) brachytherapy for recurrent gynecologic cancer in Japan and to determine the role of this therapy in clinical practice.
MATERIALS AND METHODS
A retrospective multicenter chart review was performed for reirradiation for gynecologic cancer using HDR brachytherapy. Each center provided information on patient characteristics, treatment outcomes, and complications.
RESULTS
The study included 165 patients treated at 9 facilities from 2000 to 2018. The analysis of outcomes included 142 patients treated with curative intent. The median follow-up time for survivors was 30 months (range 1-130 months). The 3-year overall survival (OS), progression-free survival (PFS), and local control (LC) rates were 53 % (95 %CI: 42-63 %), 44 % (35-53 %), and 61 % (50-70 %) for cervical cancer; 100 % (NA), 64 % (30-85 %), and 70 % (32-89 %) for endometrial cancer; and 54 % (13-83 %), 38 % (6-72 %), and 43 % (6-78 %) for vulvar and vaginal cancer, respectively. In multivariate analysis, interval to reirradiation (<1 year) was a significant risk factor for OS, PFS and LC; Gross Tumor Volume (≥25 cm) was a significant risk factor for OS. Toxicities were analyzed in all enrolled patients (n = 165). Grade ≥ 3 late toxicities occurred in 49 patients (30 %). A higher cumulative EQD2 (α/β = 3) was significantly associated with severe complications.
CONCLUSION
Reirradiation with HDR brachytherapy for recurrent gynecologic cancer is effective, especially in cases with a long interval before reirradiation.
Topics: Humans; Female; Brachytherapy; Middle Aged; Aged; Neoplasm Recurrence, Local; Japan; Retrospective Studies; Re-Irradiation; Genital Neoplasms, Female; Adult; Aged, 80 and over; Practice Patterns, Physicians'; Radiotherapy Dosage; Treatment Outcome
PubMed: 38583719
DOI: 10.1016/j.radonc.2024.110269 -
International Journal of Cancer Jul 2024Human papillomavirus (HPV) proteins may elicit antibody responses in the process toward HPV-related malignancy. However, HPV seroepidemiology in noncervical HPV-related...
Human papillomavirus (HPV) proteins may elicit antibody responses in the process toward HPV-related malignancy. However, HPV seroepidemiology in noncervical HPV-related cancers remains poorly understood, particularly in populations with a high prevalence of human immunodeficiency virus (HIV). Using a glutathione S-transferase-based multiplex serology assay, antibodies against E6, E7 and L1 proteins of HPV16 and HPV18 were measured in sera of 535 cases of noncervical HPV-related cancers (anal (n = 104), vulval (n = 211), vaginal (n = 49), penile (n = 37) and oropharyngeal (n = 134)) and 6651 non-infection-related cancer controls, from the Johannesburg Cancer Study that recruited Black South African with newly diagnosed cancer between 1995 and 2016. Logistic and Poisson regression models were used to calculate adjusted odds ratios (aOR) and prevalence ratios (aPR) and 95% confidence intervals (CI) in cases versus controls. HPV16 E6 was more strongly associated with noncervical HPV-related cancers than HPV16 L1 or E7, or HPV18 proteins: anal (females (HPV16 E6 aOR = 11.50;95%CI:6.0-22.2), males (aOR = 10.12;95%CI:4.9-20.8), vulval (aOR = 11.69;95%CI:7.9-17.2), vaginal (aOR = 10.26;95%CI:5.0-21), penile (aOR = 18.95;95%CI:8.9-40), and oropharyngeal (females (aOR = 8.95;95%CI:2.9-27.5), males (aOR = 3.49;95%CI:1.8-7.0)) cancers. HPV16-E6 seropositivity ranged from 24.0% to 35.1% in anal, vulval, vaginal and penile cancer but was significantly lower (11.2%) in oropharyngeal cancer. After adjustment for HIV, prevalence of which increased from 22.2% in 1995-2005 to 54.1% in 2010-2016, HPV16 E6 seropositivity increased by period of diagnosis (aPR for 2010-2016 vs. 1995-2006 = 1.84;95%CI:1.1-3.0). Assuming HPV16 E6 seroprevalence reflects HPV attributable fraction, the proportion of certain noncervical-HPV-related cancers caused by HPV is increasing over time in South Africa. This is expected to be driven by the increasing influence of HIV.
Topics: Humans; Male; Female; South Africa; Papillomavirus Infections; Middle Aged; Adult; Antibodies, Viral; Oncogene Proteins, Viral; HIV Infections; Human papillomavirus 16; Aged; Oropharyngeal Neoplasms; Seroepidemiologic Studies; Case-Control Studies; Human papillomavirus 18; Vulvar Neoplasms; Penile Neoplasms; Anus Neoplasms; Vaginal Neoplasms; Black People; Repressor Proteins; Neoplasms; Human Papillomavirus Viruses
PubMed: 38577820
DOI: 10.1002/ijc.34919 -
Journal of Clinical Oncology : Official... Jun 2024To assess efficacy and toxicity of cisplatin (C) and gemcitabine (G) with intensity-modulated radiation therapy (IMRT) in patients with locally advanced vulvar cancer...
PURPOSE
To assess efficacy and toxicity of cisplatin (C) and gemcitabine (G) with intensity-modulated radiation therapy (IMRT) in patients with locally advanced vulvar cancer not amenable to surgery.
METHODS
Patients enrolled in a single-arm phase II study. Pretreatment inguinal-femoral nodal assessment was performed. Sixty-four Gy IMRT was prescribed to the vulva, with 50-64 Gy delivered to the groins/low pelvis. Radiation therapy (RT) plans were quality-reviewed pretreatment. C 40 mg/m and G 50 mg/m were administered once per week throughout IMRT. Complete pathologic response (CPR) was the primary end point. Progression-free survival (PFS) and overall survival (OS) were estimated using the Kaplan-Meier method, and adverse events were assessed with Common Terminology Criteria for Adverse Events v 4.0.
RESULTS
Fifty-seven patients enrolled, of which 52 were evaluable. The median age was 58 years (range, 25-58), and 94% were White. Forty (77%) had stage II or III disease, and all had squamous histology. A median of six chemotherapy cycles (range, 1-8) were received. Eighty-five percent of RT plans were quality-reviewed with 100% compliance to protocol. Seven patients came off trial because of toxicity or patient withdrawal. Of 52 patients available for pathologic assessment, 38 (73% [90% CI, 61 to 83]) achieved CPR. No pelvic exenterations were performed. With a median follow-up of 51 months, the 12-month PFS was 74% (90% CI, 62.2 to 82.7) and the 24-month OS was 70% (90% CI, 57 to 79). The most common grade 3 or 4 adverse events were hematologic toxicity and radiation dermatitis. There was one grade 5 event unlikely related to treatment.
CONCLUSION
Weekly C and G concurrent with IMRT sufficiently improved CPR in women with locally advanced vulvar squamous cell carcinoma not amenable to surgical resection.
Topics: Humans; Female; Middle Aged; Vulvar Neoplasms; Radiotherapy, Intensity-Modulated; Cisplatin; Gemcitabine; Adult; Carcinoma, Squamous Cell; Deoxycytidine; Antineoplastic Combined Chemotherapy Protocols; Chemoradiotherapy; Progression-Free Survival
PubMed: 38574312
DOI: 10.1200/JCO.23.02235 -
Pathology Oncology Research : POR 2024Mammary-like vulvar adenocarcinoma (MLVA) is an exceedingly rare subtype of vulvar adenocarcinoma that shares features with mammary gland tissue. Due to its rarity and...
Mammary-like vulvar adenocarcinoma (MLVA) is an exceedingly rare subtype of vulvar adenocarcinoma that shares features with mammary gland tissue. Due to its rarity and lack of consensus, MLVA presents diagnostic challenges to pathologists. We present the case of a 59-year-old female with an ulcerated mass on the right side of the external genitalia, diagnosed as MLVA. Comprehensive immunohistochemistry (IHC) and gene sequencing studies were performed to characterize the tumor. IHC analysis revealed triple expression of hormonal receptors (estrogen receptor, progesterone receptor, and HER2), supporting the mammary gland origin of the tumor. Gene sequencing identified unique genetic mutations associated with the expression of hormonal markers. One fusion gene (ERBB2-NAGLU) has not been reported in any tumors, and other mutations with unique mutation types have not been previously reported in MLVA. Our findings shed light on the molecular characteristics of MLV and may help improve the diagnosis and treatment of this rare type of vulvar adenocarcinoma.
Topics: Female; Humans; Middle Aged; Mammary Glands, Human; Adenocarcinoma; Vulvar Neoplasms; Breast; High-Throughput Nucleotide Sequencing
PubMed: 38572338
DOI: 10.3389/pore.2024.1611376 -
Japanese Journal of Radiology Jul 2024This study aimed to show the results of radical radiation therapy (RT) and concurrent chemoradiotherapy (CCRT) for vulvar cancer (VC) based on data from a Japanese...
OBJECTIVE
This study aimed to show the results of radical radiation therapy (RT) and concurrent chemoradiotherapy (CCRT) for vulvar cancer (VC) based on data from a Japanese nationwide survey.
MATERIALS AND METHODS
We collected data from 108 institutions on cases of VC diagnosed between January 2001 and December 2010. Patients with histologically proven squamous cell carcinoma and adenocarcinoma with curative intent were selected, and 172 patients with VC were included in this study. The collected data were analyzed for overall survival (OS) using the Kaplan-Meier method. Univariate and multivariate analyses were performed to examine the prognostic factors for patients with VC.
RESULTS
The median follow-up period was 16.8 (range; 3.2-154.8) months. Fifty-five patients received CCRT, and 117 patients received RT alone. The 2-year OS rates (95% confidence interval [CI]) for stages I, II, III, and IV were 77.9% (55.8-100.0), 71.9% (53.8-89.9), 55.4% (42.5-68.3), and 41.5% (27.3-55.7) respectively. Univariate analyses showed that the FIGO stage (p = 0.001), tumor diameter (p = 0.005), and lymph node (LN) status (p = 0.001) were associated with OS. The concurrent use of chemotherapy resulted in a significantly longer OS in Stage III (p = 0.013). Multivariate analysis showed that the hazard ratios (95% CI) for tumor diameter, positivity for LN metastasis, and RT alone (no concurrent chemotherapy) were 1.502 (1.116-2.021), 1.801 (1.287-2.521), and 1.936 (1.187-3.159), respectively.
CONCLUSIONS
Our analysis revealed that CCRT should be recommended, especially for Stage III VC patients. Further studies are warranted to determine who benefits from CCRT, considering primary tumor size and LN status. The study was registered at the University Hospital Medical Information Network (protocol number: UMIN000017080) on April 8th, 2015.
Topics: Humans; Female; Vulvar Neoplasms; Middle Aged; Aged; Chemoradiotherapy; Japan; Aged, 80 and over; Adult; Carcinoma, Squamous Cell; Survival Rate; Treatment Outcome; Neoplasm Staging; Prognosis; Adenocarcinoma; Retrospective Studies; Follow-Up Studies; East Asian People
PubMed: 38568430
DOI: 10.1007/s11604-024-01557-9 -
Archives of Gynecology and Obstetrics Jul 2024
Topics: Humans; Female; Vulvar Neoplasms; Lymph Node Excision; Laparoscopy; Vulvectomy; Middle Aged; Inguinal Canal; Aged; Carcinoma, Squamous Cell
PubMed: 38522043
DOI: 10.1007/s00404-024-07459-5 -
Journal of Lower Genital Tract Disease Apr 2024Human papillomavirus (HPV)-independent vulvar intraepithelial neoplasia (VIN) is a rare yet aggressive precursor lesion of vulvar cancer. Our objectives were to estimate...
OBJECTIVES
Human papillomavirus (HPV)-independent vulvar intraepithelial neoplasia (VIN) is a rare yet aggressive precursor lesion of vulvar cancer. Our objectives were to estimate its long-term incidence, the risk of recurrent disease and progression to vulvar cancer, and risk factors thereof.
MATERIALS AND METHODS
Patients with HPV-independent VIN between 1991 and 2019 in a selected region were identified from the Dutch Nationwide Pathology Databank (Palga). Data were collected from the pathology reports. Crude and European age-standardized incidence rates were calculated for 10-year periods. Kaplan-Meier analyses were performed to determine the cumulative recurrence and cancer incidence, followed by Cox regression analyses to identify associated risk factors.
RESULTS
A total of 114 patients were diagnosed with solitary HPV-independent VIN without prior or concurrent vulvar cancer. The European age-standardized incidence rate increased from 0.09 to 0.69 per 100,000 women-years between 1991-2010 and 2011-2019. A cumulative recurrence and cancer incidence of 29% and 46% were found after 8 and 13 years of follow-up, respectively. Nonradical surgery was identified as the only independent risk factor for recurrent HPV-independent VIN. Risk factors associated with progression to cancer were increasing age and a mutant p53 immunohistochemical staining pattern.
CONCLUSIONS
The incidence of detected HPV-independent VIN has substantially increased the last decade and the subsequent recurrence and vulvar cancer risks are high. Although HPV-independent VIN may present as a wide morphologic spectrum, surgical treatment should aim for negative resection margins followed by close surveillance, especially for p53 mutant lesions.
Topics: Humans; Female; Infant; Vulvar Neoplasms; Incidence; Human Papillomavirus Viruses; Papillomavirus Infections; Tumor Suppressor Protein p53; Carcinoma in Situ; Risk Factors; Carcinoma, Squamous Cell; Papillomaviridae
PubMed: 38518213
DOI: 10.1097/LGT.0000000000000794 -
International Journal of Cancer Aug 2024Population-based data on the epidemiology of vulvar lichen sclerosus (LS) are sparse and only few prospective studies have investigated the malignant potential of the...
Population-based data on the epidemiology of vulvar lichen sclerosus (LS) are sparse and only few prospective studies have investigated the malignant potential of the disease. We used the nationwide Danish Pathology Registry to first assess the incidence of biopsy-verified vulvar LS in the period 1997-2022 and second to examine the incidence of vulvar high-grade squamous precancer and squamous cell carcinoma (SCC) in women with biopsy-verified vulvar LS (1978-2019) compared with that expected in the general female population. For the latter aim, we computed standardized incidence ratios (SIRs) with 95% confidence intervals (CIs). During our study period, the age-standardized incidence rate of vulvar LS increased from 5.0 (1997-1998) to 35.7 (2021-2022) per 100,000 person-years. Compared with the general female population, women with biopsy-verified vulvar LS had significantly increased rates of vulvar high-grade squamous precancer (SIR = 8.5; 95% CI: 7.2-10.0) and SCC (SIR = 16.2; 95% CI: 14.2-18.4). The SIRs of vulvar high-grade squamous precancer and SCC did not vary substantially according to length of follow-up. This nationwide and population-based study shows a 7-fold increase in the incidence of biopsy-verified vulvar LS since 1997. Data also show that women with biopsy-verified vulvar LS have 8.5 and 16 times higher than expected incidence of vulvar high-grade squamous precancer and SCC, respectively. The substantially increased incidence of vulvar high-grade squamous precancer and SCC following LS is important in relation to the clinical management and follow-up of LS patients.
Topics: Humans; Female; Carcinoma, Squamous Cell; Vulvar Neoplasms; Incidence; Vulvar Lichen Sclerosus; Middle Aged; Precancerous Conditions; Adult; Denmark; Aged; Biopsy; Registries; Aged, 80 and over; Young Adult; Risk Factors
PubMed: 38517074
DOI: 10.1002/ijc.34927 -
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