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International Journal of Gynecological... Apr 2023Standard treatment of early-stage vulvar cancer is a radical, wide, local excision of the primary tumor and a sentinel lymph node (SLN) procedure for the groins. An...
Phase II activity trial of high-dose radiation and chemosensitization in patients with macrometastatic lymph node spread after sentinel node biopsy in vulvar cancer: GROningen INternational Study on Sentinel nodes in Vulvar cancer III (GROINSS-V III/NRG-GY024).
BACKGROUND
Standard treatment of early-stage vulvar cancer is a radical, wide, local excision of the primary tumor and a sentinel lymph node (SLN) procedure for the groins. An inguinofemoral lymphadenectomy is no longer necessary for patients who have a negative SLN or micrometastasis (2 mm). When there is macrometastasis (>2 mm) in the SLN, an inguinofemoral lymphadenectomy is indicated; however, this procedure is associated with major morbidity, such as wound healing, lymphoceles, and lymphedema.
PRIMARY OBJECTIVE
To investigate the safety of replacing inguinofemoral lymphadenectomy by chemoradiation in patients with early-stage vulvar cancer with a macrometastasis (>2 mm) and/or extracapsular extension in the sentinel node.
STUDY HYPOTHESIS
Combination of 56 Gy of radiation to the inguinal site and concurrent cisplatin chemotherapy without completion inguinofemoral lymphadenectomy will be feasible and safe, with low groin recurrence rates.
TRIAL DESIGN
This is a single-arm, prospective phase II treatment trial with stopping rules for unacceptable groin recurrences. Eligible patients will receive 56 Gy of radiation to the involved inguinal site and chemotherapy with concurrent cisplatin.
MAJOR INCLUSION/EXCLUSION CRITERIA
Eligible patients undergoing sentinel node procedure will have stage I, unifocal, invasive (>1 mm depth of invasion) squamous cell carcinoma of the vulva with tumor size <4 cm, and no suspicious nodes on imaging. Those eligible for the trial are those with a metastasis >2 mm in the sentinel node and/or extracapsular extension, or more than one sentinel node with micrometastasis ≤2 mm.
PRIMARY ENDPOINT
Groin recurrence rate in the first 2 years after primary treatment.
SAMPLE SIZE
157 patients with macrometastases in their SLN.
ESTIMATED DATES FOR COMPLETING ACCRUAL AND PRESENTING RESULTS
January 1, 2029.
TRIAL REGISTRATION NUMBER
NCT05076942.
Topics: Female; Humans; Sentinel Lymph Node; Vulvar Neoplasms; Prospective Studies; Neoplasm Micrometastasis; Extranodal Extension; Cisplatin; Lymphatic Metastasis; Sentinel Lymph Node Biopsy; Lymph Nodes; Lymph Node Excision
PubMed: 36653060
DOI: 10.1136/ijgc-2022-004122 -
Ghana Medical Journal Sep 2011Vulvar lipoma is said to be so rare that only a few cases have been reported. We present two cases of vulvar lipoma that were diagnosed within six months in our centre...
Vulvar lipoma is said to be so rare that only a few cases have been reported. We present two cases of vulvar lipoma that were diagnosed within six months in our centre in a 28 year-old para 2 and 35 year-old para 1 woman both of whom presented with slow-growing masses in the vulva. A detailed discussion of the clinical features and current management options are outlined, with emphasis on the need to subject all excised lesions to histopathological evaluation; to ensure accurate diagnosis and differentiate this benign swelling from cystic swellings and malignant neoplasms in the vulva.
Topics: Adult; Female; Humans; Lipoma; Vulvar Neoplasms
PubMed: 22282580
DOI: No ID Found -
International Journal of Environmental... Oct 2022Granulomatosis with polyangiitis is a rare autoimmune disease with the presence of c-ANCA in most cases. It involves necrotizing inflammation in small and medium-sized...
Granulomatosis with polyangiitis is a rare autoimmune disease with the presence of c-ANCA in most cases. It involves necrotizing inflammation in small and medium-sized vessels with multiple granulomas. The disease can affect many systems, but the typical triad of attacked systems are the upper and lower respiratory tracts and kidneys, with varying degrees of severity. Involvement of the respiratory tract may manifest, among other symptoms, as nasal crusting, nosebleeds, and dyspnea. Among patients with granulomatosis with polyangiitis, only less than 1% develop genitourinary system involvement. We present a case study of a 36-year-old woman with an 8-year-long GPA history and a lesion, which, due to its appearance and accompanying symptoms, aroused the suspicion of a neoplasm but was proven to be a granuloma with a nontypical location. The systemic disease was treated with glucocorticosteroids and cyclophosphamide. The lesion on the labium minus was surgically removed. We concluded that the macroscopic picture of GPA of the vulva and vulvar cancer is similar. The patient's medical history may help differentiate GPA and vulvar cancer. Although vulvar GPA is extremely rare, it should be considered in the differential diagnosis of vulvar lesions, especially those suspected to be oncological.
Topics: Female; Humans; Adult; Granulomatosis with Polyangiitis; Vulvar Neoplasms; Cyclophosphamide; Inflammation; Vulva
PubMed: 36360741
DOI: 10.3390/ijerph192113862 -
International Journal of Clinical and... 2013Paraganglioma is a neuroendocrine neoplasm, which is extremely rare in the vulva and only one case has been reported. Here we present a case of vulvar paraganglioma in a... (Review)
Review
Paraganglioma is a neuroendocrine neoplasm, which is extremely rare in the vulva and only one case has been reported. Here we present a case of vulvar paraganglioma in a 48-year-old woman and a literature review. The patient found a lump located in the genitals below the symphysis pubis 3 months before presentation when she complained that the lump was increasing in size. A 3.2 cm x 2.3 cm x 1.5 cm nodule was excised from subcutaneous soft tissue in the vulva. Microscopy showed a diversity of cell morphologies and structures in the rich vascular network of the tumor separated the chief cells into round cell nests (Zellballen pattern). Some areas of the tumor presented epithelioid and spindle-shaped cells with increased cell density and indistinct structural characteristics. Hyaline degeneration of collagen fibers or mucoid degeneration was found in tumor interstitium. Immunohistochemical staining showed diffused expression of synaptophysin in the chief cells, focal expression of S-100 protein in the sustentacular cells and high expression of CD34 in the vascular components. Based on morphological and immunohistochemical results, a rare paraganglioma of the vulva was diagnosed.
Topics: Antigens, CD34; Biomarkers, Tumor; Female; Humans; Middle Aged; Paraganglioma; S100 Proteins; Synaptophysin; Vulva; Vulvar Neoplasms
PubMed: 24133606
DOI: No ID Found -
Experimental & Molecular Medicine Feb 2018Vulvar squamous cell carcinoma (SCC) consists of two different etiologic categories: human papilloma virus (HPV)-associated (HPV (+)) and HPV-non-associated (HPV (-))....
Vulvar squamous cell carcinoma (SCC) consists of two different etiologic categories: human papilloma virus (HPV)-associated (HPV (+)) and HPV-non-associated (HPV (-)). There have been no genome-wide studies on the genetic alterations of vulvar SCCs or on the differences between HPV (+) and HPV (-) vulvar SCCs. In this study, we performed whole-exome sequencing and copy number profiling of 6 HPV (+) and 9 HPV (-) vulvar SCCs and found known mutations (TP53, CDKN2A and HRAS) and copy number alterations (CNAs) (7p and 8q gains and 2q loss) in HPV (-) SCCs. In HPV (+), we found novel mutations in PIK3CA, BRCA2 and FBXW7 that had not been reported in vulvar SCCs. HPV (-) SCCs exhibited more mutational loads (numbers of nonsilent mutations and driver mutations) than HPV (+) SCCs, but the CNA loads and mutation signatures between HPV (+) and HPV (-) SCCs did not differ. Of note, 40% and 40% of the 15 vulvar SCCs harbored PIK3CA and FAT1 alterations, respectively. In addition, we found that the SCCs harbored kataegis (a localized hypermutation) in 2 HPV (+) SCCs and copy-neutral losses of heterozygosity in 4 (one HPV (+) and 3 HPV (-)) SCCs. Our data indicate that HPV (+) and HPV (-) vulvar SCCs may have different mutation and CNA profiles but that there are genomic features common to SCCs. Our data provide useful information for both HPV (+) and HPV (-) vulvar SCCs and may aid in the development of clinical treatment strategies.
Topics: Adult; Aged; Aged, 80 and over; Alleles; Biomarkers, Tumor; Carcinoma, Squamous Cell; Chromosome Aberrations; Chromosome Mapping; DNA Copy Number Variations; DNA Mutational Analysis; Female; Gene Expression Profiling; Genetic Predisposition to Disease; Humans; Loss of Heterozygosity; Middle Aged; Mutation; Neoplasm Staging; Tumor Burden; Vulvar Neoplasms; Exome Sequencing
PubMed: 29422544
DOI: 10.1038/emm.2017.265 -
Critical Reviews in Oncology/hematology Jan 2024Gynecological neuroendocrine neoplasms are rare entities and can be divided into two groups: carcinoids and neuroendocrine carcinomas. Due to their rarity their... (Review)
Review
Gynecological neuroendocrine neoplasms are rare entities and can be divided into two groups: carcinoids and neuroendocrine carcinomas. Due to their rarity their management is not standardized. The aim of this work is to summarize and discuss the current literature evidence on this pathology. A scoping literature review was performed in multiple databases. Thirty-one studies were included: 30 case reports and one case series. Patients' age ranged between 28 and 92 years. Surgery was the most used treatment and the surgical approach included local excision (N = 16/31; 51.6%) with (N = 5/16; 31.25%) or without (N = 11/16; 68.75%) inguinal lymphadenectomy. Adjuvant radiotherapy was delivered in 12 (38.7%) cases; instead, platinum-based therapies were frequently used when chemotherapy was chosen for adjuvant treatment. The overall survival ranged between 20 days to 4 years. However, further research is needed; currently, multimodal approach including surgery, chemotherapy and radiotherapy appeared safe and feasible for the treatment of these rare and aggressive diseases.
Topics: Female; Humans; Adult; Middle Aged; Aged; Aged, 80 and over; Vulvar Neoplasms; Neoplasm Staging; Vulva; Neuroendocrine Tumors; Lymph Node Excision
PubMed: 37977312
DOI: 10.1016/j.critrevonc.2023.104201 -
The Medical Journal of Malaysia Mar 1978
Topics: Adenofibroma; Adult; Female; Humans; Vulvar Neoplasms
PubMed: 683045
DOI: No ID Found -
BMJ Case Reports Jul 2019
Topics: Biopsy, Needle; Emergency Service, Hospital; Female; Gynecologic Surgical Procedures; Humans; Immunohistochemistry; Neoplasms, Fibroepithelial; Polyps; Prognosis; Rare Diseases; Severity of Illness Index; Surgery, Plastic; Treatment Outcome; Vulvar Neoplasms; Young Adult
PubMed: 31326911
DOI: 10.1136/bcr-2019-230449 -
Medicine Oct 2019Paget's disease can arise in the breast (mammary Paget disease) or in other locations (extramammary Paget disease) such as anogenital skin in both males and females...
Paget's disease can arise in the breast (mammary Paget disease) or in other locations (extramammary Paget disease) such as anogenital skin in both males and females (Paget disease of the vulva [PDV]). Underlying adenocarcinoma can be found in some cases. This study aims to report clinical aspects, surgical procedures, outcomes, and recurrences of patients with PDV.A retrospective chart review was conducted on patients with pathologically confirmed diagnosis of PDV managed at the Department of Obstetrics and Gynecology, University of Bari, and the "Giovanni Paolo II" National Cancer Institute in Bari, between 1998 and 2018.Records of 24 cases of PDV were examined. Median age of the patients at diagnosis was 69.3 (range 38-84), diagnosis of synchronous cancer was made in 2 cases and in 2 other cases of metachronous disease. Three patients had previously been diagnosed with other oncological diseases. All patients underwent surgery including wide local excision (6), simple vulvectomy (8), and extended vulvectomy (10). Lymphadenectomy was performed in 2 cases and reconstructions with advancement flaps in 7 cases. Four patients were found to have invasive disease and 1 had inguinal node involvement. Positive margins were found in 11 patients. Wound dehiscence and urethral stenosis were found in 4 and 1 case each. Eight recurrences (33.33%) were observed, regardless of positive surgical margins.PDV has a low rate of malignancy but a high rate of recurrence. It should be diagnosed early to avoid repeated surgery and to reduce symptoms and morbidity.
Topics: Adenocarcinoma; Adult; Aged; Aged, 80 and over; Female; Humans; Middle Aged; Neoplasm Recurrence, Local; Paget Disease, Extramammary; Retrospective Studies; Vulva; Vulvar Neoplasms
PubMed: 31593076
DOI: 10.1097/MD.0000000000017018 -
The Journal of International Medical... Jul 2020Aggressive angiomyxoma is an interstitial tumour that is often misdiagnosed and is likely to recur. There have been few reported cases of angiomyxoma in pregnant women.... (Review)
Review
Aggressive angiomyxoma is an interstitial tumour that is often misdiagnosed and is likely to recur. There have been few reported cases of angiomyxoma in pregnant women. We report a case of a woman who was previously diagnosed with a tumour in her vulva that increased in size during both of her pregnancies and spontaneously decreased postpartum. Local excision was performed and a gonadotropin-releasing hormone agonist was administered. According to a literature review, aggressive angiomyxoma is associated with good maternal and child outcomes. Caesarean section is not the delivery method of choice, but it is indicated if the tumour is preventing vaginal birth. Treatment for angiomyxoma is mainly postpartum local resection supplemented by hormone therapy. This tumour frequently recurs and patients should undergo long-term follow-up.
Topics: Adult; Cesarean Section; China; Diagnostic Errors; Female; Humans; Myxoma; Neoplasm Recurrence, Local; Pregnancy; Vulva; Vulvar Neoplasms
PubMed: 32644864
DOI: 10.1177/0300060520936414