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Journal of Cancer Research and Clinical... Jul 2020To evaluate the difference in oncologic outcome between vulvar cancer patients with uni- and bilateral inguino-femoral lymph nodal involvement and to identify factors...
PURPOSE
To evaluate the difference in oncologic outcome between vulvar cancer patients with uni- and bilateral inguino-femoral lymph nodal involvement and to identify factors affecting their oncologic outcome MATERIALS AND METHODS: Patients who underwent inguino-femoral lymphadenectomy for vulvar cancer were classified into three groups according to their lymph nodal status at the histology analysis (negative, positive one side, positive bilaterally). PFS and OS survival were calculated using the Kaplan-Meier method. Univariate and multivariate analyses were performed to analyze factors predicting overall survival and progression-free survival. Multivariable models were used for variables reporting a p value ≤ 0.1 at the univariate analysis. p values ≤ 0.05 were considered statistically significant.
RESULTS
One hundred and forty-six patients were considered for the analysis. Patients with bilaterally negative lymph nodes had significantly longer PFS and OS as compared to patients with unilateral and bilateral involvement. Patients with unilateral lymph nodal involvement had better PFS than patients with bilateral lymph nodal involvement. Among these patients, the difference in the OS approached but did not reach statistical significance. At the multivariate analysis, the tumor size affected PFS and lymph nodal involvement affected OS.
CONCLUSION
Vulvar cancer patients with bilateral positive lymph nodes have worse oncologic outcome as compared to patients with unilateral lymph nodal involvement; similarly, patients with unilateral lymph nodal involvement have worse oncological outcome as compared to patients with bilateral negative lymph nodes. Furthermore, tumor size and lymph nodal status are independent factors predicting recurrence rate and overall survival, respectively.
Topics: Adult; Aged; Aged, 80 and over; Female; Humans; Kaplan-Meier Estimate; Lymph Nodes; Lymphatic Metastasis; Middle Aged; Neoplasm Grading; Neoplasm Staging; Prognosis; Proportional Hazards Models; Vulvar Neoplasms; Young Adult
PubMed: 32266536
DOI: 10.1007/s00432-020-03196-9 -
Acta Obstetricia Et Gynecologica... Jun 2010No standard screening programs exist to detect vulvar carcinoma or its precursor lesions, and therefore gynecologists, dermatologists and other healthcare providers in... (Review)
Review
No standard screening programs exist to detect vulvar carcinoma or its precursor lesions, and therefore gynecologists, dermatologists and other healthcare providers in this field should be aware of the clinical features, behavior and management of the different existing premalignant vulvar lesions, squamous vulvar intraepithelial neoplasia (VIN), vulvar Paget's disease and melanoma in situ. In 2004, a new classification for squamous VIN was introduced by the International Society for the Study of Vulvar Disease, subdividing squamous VIN into the HPV-related usual type, and into differentiated type, which is associated with lichen sclerosus. This review describes the relevant aspects of squamous VIN, vulvar Paget's disease and melanoma in situ, its epidemiological characteristics, diagnosis, management and malignant potential.
Topics: Carcinoma in Situ; Epithelium; Female; Humans; Melanoma; Paget Disease, Extramammary; Precancerous Conditions; Skin Neoplasms; Vulvar Neoplasms
PubMed: 20504079
DOI: 10.3109/00016341003739575 -
Gynecologic Oncology Jun 1991Dermatofibrosarcoma protuberans of the vulva is an uncommon low-grade sarcoma of dermal origin. Although wide local excision is the treatment of choice, microscopic... (Review)
Review
Dermatofibrosarcoma protuberans of the vulva is an uncommon low-grade sarcoma of dermal origin. Although wide local excision is the treatment of choice, microscopic tumor projections beyond the central tumor nodule explain the tumors' propensity for local recurrence. Frozen sections of margins may be useful to ensure complete resection. The following report contributes two additional patients with this uncommon neoplasm. Notably, one of these two had a fibrosarcomatous area within the dermatofibrosarcoma protuberans. This is the second reported case of a fibrosarcoma arising in a dermatofibrosarcoma protuberans of the vulva.
Topics: Adult; Female; Fibrosarcoma; Humans; Incidence; Middle Aged; Vulvar Neoplasms
PubMed: 1869103
DOI: 10.1016/0090-8258(91)90317-x -
BMC Cancer Jul 2016Primary invasive Extramammary Paget's vulvar disease is a rare tumor that is challenging to control. Wide surgical excision represents the standard treatment approach... (Review)
Review
BACKGROUND
Primary invasive Extramammary Paget's vulvar disease is a rare tumor that is challenging to control. Wide surgical excision represents the standard treatment approach for Primary invasive Extramammary Paget's vulvar disease. The goal of the current study was to analyze the appropriate indications of radiotherapy in Primary invasive Extramammary Paget's vulvar disease because they are still controversial.
DISCUSSION
We searched the Cochrane Gynecological Cancer Group Trials Register, Cochrane Register of Controlled Trials (CENTRAL), MEDLINE and EMBASE database up to September 2015. Radiotherapy was delivered as a treatment in various settings: i) Radical in 28 cases (range: 60-63 Gy), ii) Adjuvant in 25 cases (range: 39-60 Gy), iii) Salvage in recurrence of 3 patients (63 Gy) and iv) Neoadjuvant in one patient (43.3 Gy). A radiotherapy field that covered the gross tumor site with a 2-5 cm margin for the microscopic disease has been used. Radiotherapy of the inguinal, pelvic or para-aortic lymph node should be considered only for the cases with lymph node metastases within these areas. Radiotherapy alone is an alternative therapeutic approach for patients with extensive inoperable disease or medical contraindications. Definitive radiotherapy can be used in elderly patients and/or with medical contraindications. Adjuvant radiotherapy may be considered in presence of risk factors associated with local recurrence as dermal invasion, lymph node metastasis, close or positive surgical margins, perineal, large tumor diameter, multifocal lesions, extensive disease, coexisting histology of adenocarcinoma or vulvar carcinoma, high Ki-67 expression, adnexal involvement and probably in overexpression of HER-2/neu. Salvage radiotherapy can be given in inoperable loco-regional recurrence and to those who refused additional surgery.
Topics: Female; Humans; Neoplasm Recurrence, Local; Paget Disease, Extramammary; Radiotherapy; Radiotherapy Dosage; Radiotherapy, Adjuvant; Salvage Therapy; Treatment Outcome; Vulvar Neoplasms
PubMed: 27473174
DOI: 10.1186/s12885-016-2622-5 -
Journal of Gynecologic Oncology Nov 2023To elucidate clinical characteristics and build a prognostic nomogram for patients with vulvar cancer.
Epidemiologic characteristics and a prognostic nomogram for patients with vulvar cancer: results from the Surveillance, Epidemiology, and End Results (SEER) program in the United States, 1975 to 2016.
OBJECTIVE
To elucidate clinical characteristics and build a prognostic nomogram for patients with vulvar cancer.
METHODS
The study population was drawn from the Surveillance, Epidemiology, and End Results (SEER) database. Patients were randomly assigned to training and validation sets. Cox proportional hazards model and competing risk model were used to identify the prognostic parameters of overall survival (OS) and cancer-specific survival (CSS) to construct a nomogram. The nomogram was assessed by concordance index (C-index), area under the curve (AUC), calibration plot, and decision curve analysis (DCA).
RESULTS
A total of 20,716 patients were included in epidemiological analysis, of whom 7,025 patients were selected in survival analysis, including 4,215 and 2,810 in training and validation sets, respectively. The multivariate Cox model showed that the predictors for OS were age, marital status, histopathology, differentiation and tumor node metastasis (TNM) stages, whether to undergo surgery and chemotherapy. However, the predictors for CSS were age, race, differentiation and TNM stages, whether to undergo surgery and radiation. The C-index for OS and CSS in the training set were 0.76 and 0.80. The AUC in the training set for 1-, 3- and 5-year OS and CSS were 0.84, 0.81, 0.80 and 0.88, 0.85, 0.83, respectively, which was similar in the validation set. The calibration curves showed good agreement between prediction and actual observations. DCA revealed that the nomogram had a better discrimination than TNM stages.
CONCLUSIONS
The nomogram showed accurate prognostic prediction in OS and CSS for vulvar cancer, which could provide guidance to clinical practice.
Topics: Female; Humans; Area Under Curve; Databases, Factual; Nomograms; Prognosis; SEER Program; United States; Vulvar Neoplasms
PubMed: 37477104
DOI: 10.3802/jgo.2023.34.e81 -
Archives of Gynecology and Obstetrics Aug 2021Despite the less frequent use of surgery in patients with vulvar cancer, the high rates of postoperative complications are still a matter of concern. The aim of the...
INTRODUCTION
Despite the less frequent use of surgery in patients with vulvar cancer, the high rates of postoperative complications are still a matter of concern. The aim of the present study was to identify risk factors that influence postoperative complications rates in vulvar cancer and identify specific clinical parameters that may influence their incidence.
MATERIALS
Patients who underwent curative-intent surgery for squamous cell carcinoma of the vulva from 2003 to 2018 were selected. All patient characteristics were analyzed as risk factors for the development of postoperative lymphocele, lymphedema, and wound dehiscence. The patients were followed up for 2 years postoperatively.
RESULTS
The investigation comprised 121 patients, of whom 18.1% developed wound dehiscence, 17.7% a lymphocele, and 20.4% lymphedema. We found no significant evidence of an association between patient's characteristics and postoperative complications. The depth of tumor invasion and the appearance of lymph-node metastasis were significantly associated with postoperative complications. Free resection margins of 5 mm or more were associated with a reduced risk of postoperative complications compared to resection margins less than 5 mm. No complications were encountered after sentinel node biopsy (SNB). Complication rates were associated with inguinofemoral lymphadenectomy, but not with the extent of lymphadenectomy. The development of a lymphocele or wound dehiscence may be correlated with the development of long-term lymphedema.
CONCLUSION
FIGO stage at diagnosis influences the risk of postoperative complications. The use of SNB minimized postoperative complications. Correlations between the free microscopic resection margin distance and the risk of postoperative wound dehiscence must be investigated further.
Topics: Adult; Aged; Aged, 80 and over; Factor Analysis, Statistical; Female; Humans; Lymph Node Excision; Lymphocele; Middle Aged; Neoplasm Staging; Postoperative Complications; Risk Factors; Vulvar Neoplasms
PubMed: 33420814
DOI: 10.1007/s00404-020-05949-w -
Current Oncology (Toronto, Ont.) Jan 2023The aim of the present study was to evaluate the incidence of concomitant vulvar cancers or premalignant lesions in women surgically treated for extramammary Paget's...
The aim of the present study was to evaluate the incidence of concomitant vulvar cancers or premalignant lesions in women surgically treated for extramammary Paget's disease of the vulva (EMPDV) through a multicenter case series. The medical records of all women diagnosed with and treated for EMPDV from January 2010 to December 2020 were retrospectively analyzed. Women with EMPDV and synchronous vulvar cancer, vulvar intraepithelial neoplasia (VIN) and/or lichen sclerosus (LS) at the histology report were included in the study. A total of 69 women eligible for the present study were considered. Concomitant vulvar lesions occurred in 22 cases (31.9%). A total of 11 cases of synchronous VIN (50%) and 14 cases (63.6%) of concomitant LS were observed. One patient (4.5%) had synchronous vulvar SCC (FIGO stage 1B). Women with EMPDV and concomitant premalignant/malignant vulvar lesions had a significantly higher rate of invasive EMPDV and wider lesions with an extravulvar involvement. The specific meaning of the association between EMPDV, VIN, SCC and LS remains unclear. The potential overlapping features between different vulvar lesions highlight the importance of dedicated gynecologists and pathologists in referral centers.
Topics: Female; Humans; Paget Disease, Extramammary; Retrospective Studies; Vulva; Precancerous Conditions; Vulvar Neoplasms; Carcinoma in Situ; Carcinoma, Squamous Cell
PubMed: 36661722
DOI: 10.3390/curroncol30010073 -
Journal of Clinical Pathology Jun 2007Ewing's sarcoma and peripheral primitive neuroectodermal tumour (pPNET) are now regarded as two morphological ends of a spectrum of neoplasms, characterised by a... (Review)
Review
BACKGROUND
Ewing's sarcoma and peripheral primitive neuroectodermal tumour (pPNET) are now regarded as two morphological ends of a spectrum of neoplasms, characterised by a t(11;22) or other related chromosomal translocation involving the EWS gene on chromosome 22 and referred to as Ewing family of tumours (EFTs). EFTs are extremely rare in the vulva and vagina, a review of the literature revealing only 13 previously reported possible cases, most of which have not had molecular confirmation. In this study, four new cases of EFTs involving the vulva (three cases) or vagina (one case) are reported.
RESULTS
The tumours occurred in women aged 19, 20, 30 and 40 years and ranged in size from 3 to 8 cm. Morphologically, all neoplasms had a lobulated architecture and were composed of solid aggregates of cells. In one case, occasional rosettes were formed. In all the tumours, there was diffuse membranous staining with CD99; nuclear positivity with FLI-1 was present in two cases. Three cases were focally positive with the broad-spectrum cytokeratin AE1/3, all were diffusely positive with vimentin and all were desmin negative. In two cases, a t(11;22) (q24;q12) (EWSR1-FLI-1) chromosomal translocation was demonstrated by reverse transcriptase-PCR (one case) and fluorescence in situ hybridisation (FISH) (one case), and in another case a rearrangement of the EWSR1 gene on chromosome 22 was demonstrated by FISH. In the other case, a variety of molecular studies did not reveal a translocation involving the EWS gene but this tumour, on the balance of probability, is still considered to represent a neoplasm in the EFTs. Follow-up in two cases revealed that one patient developed pulmonary metastasis and died and another is alive without disease at 12 months.
CONCLUSIONS
This report expands the published literature regarding EFTs involving the vulva and vagina and stresses the importance of molecular techniques in firmly establishing the diagnosis, especially when these neoplasms arise at unusual sites.
Topics: 12E7 Antigen; Adolescent; Adult; Antigens, CD; Biomarkers, Tumor; Cell Adhesion Molecules; Child; Chromosomes, Human, Pair 11; Chromosomes, Human, Pair 22; Female; Humans; Immunoenzyme Techniques; Microfilament Proteins; Middle Aged; Neoplasm Proteins; Receptors, Cytoplasmic and Nuclear; Sarcoma, Ewing; Trans-Activators; Translocation, Genetic; Vaginal Neoplasms; Vulvar Neoplasms
PubMed: 17557870
DOI: 10.1136/jcp.2006.040931 -
BMJ Case Reports Jun 2021Extrarenal rhabdoid tumour (ERT) of vulva is a rare gynaecological neoplasm with an aggressive course and no clear management guidelines. We present the case of a...
Extrarenal rhabdoid tumour (ERT) of vulva is a rare gynaecological neoplasm with an aggressive course and no clear management guidelines. We present the case of a 25-year-old woman with a rapidly increasing mass in right vulva suggestive of sarcoma. Wide local excision was done. Histopathology examination revealed ERT of vulva. Six weeks later she manifested lung metastases. Despite adjuvant chemotherapy, the disease progressed and she died 8 months later. We review the literature and briefly discuss the epidemiology, treatment approaches, prognostic factors and expected outcomes of this rare disease.
Topics: Adult; Female; Humans; Rhabdoid Tumor; Sarcoma; Soft Tissue Neoplasms; Vulva; Vulvar Neoplasms
PubMed: 34083195
DOI: 10.1136/bcr-2021-242271 -
Diagnostic Pathology Nov 2019Myeloid sarcoma (MS), which represents a rare malignancy that comprises of myeloid blasts occurring at extra-medullary sites, closely correlates with the onset and... (Review)
Review
BACKGROUND
Myeloid sarcoma (MS), which represents a rare malignancy that comprises of myeloid blasts occurring at extra-medullary sites, closely correlates with the onset and relapse of acute myeloid leukemia (AML) and other hemopoietic neoplasm. Female genital system is an uncommon location of MS, with the vulvar MS being even rarer that only eight cases have been reported in English-written literature.
CASE PRESENTATION
A 47-year-old woman presented with chronic ulceration on her vulva for one and a half month. Microscopic examination of incisional biopsy revealed dermal infiltration of myeloid precursor cells, which were positive for MPO, lysozyme, CD43, CD68, CD38 and CD117. Bone marrow flowcytometric analysis showed myeloblast count of 74%, which expressed CD13, CD33, CD117 and HLA-DR. A diagnosis of AML (M2 type) was made and vulvar MS was the earliest symptom. The patient achieved complete remission after chemotherapy with no evidence of recurrence in a 27-month follow-up. We reviewed the literature and identified 54 cases of Chinese patients with gynecological MS between 1999 and 2018, and discovered that in Chinese population, MS most frequently involved uterine cervix followed by the ovary and vulva, and ovarian MS onset much earlier than other sites. Remarkably, vulvar MS exhibited a high rate of concurrent AML and secondary myeloid leukemia within a short time of its occurrence. Despite its limited distribution, MS should be tackled aggressively with chemotherapy followed by allogeneic hematopoietic stem cell transplantation if the appropriate donor is available.
CONCLUSIONS
Female genital MS, especially vulvar MS, should be included in the differential diagnosis of gynecological neoplasm, which will facilitate its early diagnosis and prompt management.
Topics: Female; Hematopoietic Stem Cell Transplantation; Humans; Leukemia, Myeloid, Acute; Middle Aged; Sarcoma, Myeloid; Vulvar Neoplasms
PubMed: 31699112
DOI: 10.1186/s13000-019-0892-3