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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 -
European Journal of Gynaecological... 2011Verrucous carcinoma of the vulva is a rare type of squamous cell neoplasm with distinct morphology, pathogenesis and special therapeutic management. A case of a... (Review)
Review
Verrucous carcinoma of the vulva is a rare type of squamous cell neoplasm with distinct morphology, pathogenesis and special therapeutic management. A case of a 72-year-old patient who developed verrucus carcinoma of the vulva is reported. Data regarding the diagnosis, management and treatment of this neoplasm are presented, and a review of the literature is performed.
Topics: Aged; Carcinoma, Verrucous; Female; Humans; Papillomaviridae; Vulvar Neoplasms
PubMed: 22335036
DOI: No ID Found -
Journal of Lower Genital Tract Disease Apr 2017
Topics: Aged; Antineoplastic Agents; Female; Histocytochemistry; Humans; Lymphoma; Microscopy; Vulvar Neoplasms
PubMed: 28166106
DOI: 10.1097/LGT.0000000000000296 -
Pathology, Research and Practice 2005In this paper, we report a new case of metastatic endometrial carcinoma to the vulva and describe the clinical and pathological features. We reviewed the literature to... (Review)
Review
In this paper, we report a new case of metastatic endometrial carcinoma to the vulva and describe the clinical and pathological features. We reviewed the literature to determine the frequency and to evaluate the prognostic significance of this rare disease. Moreover, we discuss the criteria for the differential diagnosis of endometrial carcinoma metastatic to the vulva, the primary vulvar adenocarcinomas, and other metastatic adenocarcinomas. The patient, previously diagnosed to have endometrial adenocarcinoma with squamous differentiation at III C stage (according to the FIGO system) and T2N1M0 stage (according to the TNM system), presented with a small plaque on the vulvar mucosa 8 months after endometrial primary carcinoma had been diagnosed. The histological evaluation of excisional vulvar biopsy revealed a neoplasm with pathological features of endometrial carcinoma. Thus, the final diagnosis was metastatic endometrial carcinoma to the vulva. A total body computed tomography scan (CT) and an echotomography with contrast medium revealed a second metastatic lesion at the 8th segment of the liver. No other metastatic lesions developed, nor was a reduction in the size of liver metastasis observed after 3 months of hormonic treatment with progesterone. Fourteen months after the diagnosis of primary endometrial carcinoma, the patient died of disseminated metastatic lesions. In conclusion, metastatic endometrial carcinoma to the vulva, although rare, might develop and could appear within a few months after the diagnosis of primary tumor. Moreover, in the presence of metastatic endometrial carcinoma to the vulva, it is necessary to verify if other visceral metastases are present.
Topics: Adenocarcinoma; Aged; Diagnosis, Differential; Endometrial Neoplasms; Female; Humans; Liver Neoplasms; Neoplasm Staging; Prognosis; Vulvar Neoplasms
PubMed: 16325518
DOI: 10.1016/j.prp.2005.08.006 -
Tumori 1996Malignant rhabdoid tumor (MRT) is an uncommon aggressive neoplasm which usually occurs in the kidney of children, but it has also been found in extrarenal sites. MRT... (Review)
Review
Malignant rhabdoid tumor (MRT) is an uncommon aggressive neoplasm which usually occurs in the kidney of children, but it has also been found in extrarenal sites. MRT arising in the vulva is extremely rare. Only four cases of MRT of the vulva have been reported in the English literature. We herein present another case. The diagnosis and management of vulva MRT are reviewed.
Topics: Adult; Female; Humans; Rhabdoid Tumor; Vulvar Neoplasms
PubMed: 8623515
DOI: 10.1177/030089169608200120 -
Journal of Lower Genital Tract Disease Jan 2016The aim of the study was to assess the clinicopathological characteristics of patients with verrucous carcinoma (VC) of the vulva. (Review)
Review
OBJECTIVE
The aim of the study was to assess the clinicopathological characteristics of patients with verrucous carcinoma (VC) of the vulva.
MATERIALS AND METHODS
We reviewed data on the age, disease course, clinical manifestation, pathologic diagnosis, treatment, and follow-up of patients with vulvar VC who were treated at Tianjin Medical University General Hospital and cases that were reported in the Chinese and English literature during the past 20 years.
RESULTS
Six cases were identified in Tianjin Medical University General Hospital, the mean age of patients was 55 years, and their mean disease course was 26 months. Primary symptoms were exophytic neoplasm with pruritus and/or pain. Surgical treatment included wide local excision, simple vulvectomy, and radical vulvectomy with or without lymph node dissection in the groin. Vulvar VC occurred simultaneously with vulvar intraepithelial neoplasia in 2 cases and well-differentiated squamous cell carcinoma in 2 cases. The mean follow-up was 16.8 months with no recurrence in those 6 cases. During the 20-year period, 20 and 41 cases were reported in Chinese and English literature, respectively. Three cases were misdiagnosed with giant condyloma acuminatum in China. One case (1/20) was reported with coexistent squamous carcinoma in Chinese literature and 8 cases (8/41) in the English literature. The recurrence rate was 12.5% (2/19) and 17.5% (7/40) in Chinese and English literature, respectively.
CONCLUSIONS
Vulvar VC is a distinct type of slow-growing, nonmetastatic tumor with unclear etiology. These tumors should be distinguished from giant condyloma acuminatum and well-differentiated squamous cell carcinoma. Surgery is the most effective treatment.
Topics: Adult; Aged; Aged, 80 and over; Carcinoma, Verrucous; China; Female; Hospitals, University; Humans; Middle Aged; Retrospective Studies; Vulvar Neoplasms
PubMed: 26704335
DOI: 10.1097/LGT.0000000000000164 -
Seminars in Oncology Nursing Aug 1990Vulvar and vaginal cancer are rare malignancies and require aggressive treatment for survival. The prospect for cure in early stage disease is excellent. The major... (Review)
Review
Vulvar and vaginal cancer are rare malignancies and require aggressive treatment for survival. The prospect for cure in early stage disease is excellent. The major treatment modalities for these diseases, surgery and radiation, will affect structural alteration of the genitalia, requiring physical and psychological rehabilitation over an extended period of time. Quality of life is an important focus in cancer nursing. Efforts to control symptoms or adverse effects and to enhance the psychological adjustment are important aspects of patient care. Vulvar and vaginal cancer leave obvious residual effects, and disfigurement and dysfunction will be a part of these women's lives. Husbands and partners are also affected, and adjustments of both the patient and her partner are expected to continue for years. An active position assumed by the nurse will aid the patients' adaptive responses to these devastating diseases.
Topics: Combined Modality Therapy; Female; Humans; Neoplasm Staging; Postoperative Care; Radiotherapy; Vaginal Neoplasms; Vulvar Neoplasms
PubMed: 2204974
DOI: 10.1016/0749-2081(90)90004-o -
Clinical Obstetrics and Gynecology Dec 2005
Review
Topics: Carcinoma, Squamous Cell; Female; Humans; Neoplasm Invasiveness; Neoplasm Recurrence, Local; Vulvar Neoplasms
PubMed: 16286832
DOI: 10.1097/01.grf.0000179668.14187.3a -
Journal of Pediatric and Adolescent... Jun 2018Granular cell tumors (GCTs) are rare soft tissue lesions that can involve the female genital tract, including the vulva. Although malignant tumors are aggressive and... (Review)
Review
BACKGROUND
Granular cell tumors (GCTs) are rare soft tissue lesions that can involve the female genital tract, including the vulva. Although malignant tumors are aggressive and uncommon, benign and atypical lesions still have associated risks, including recurrence and multisite development.
CASES
Four cases of pediatric vulvar GCT are presented, including 1 atypical and 3 benign tumors. The atypical lesion occurred in a 3-year-old girl, to our knowledge, the youngest reported patient with GCT of the vulva. Follow-up information is available for 3 patients, ranging 0-12 years.
SUMMARY AND CONCLUSION
GCTs are an important differential diagnosis in patients with vulvar lesions. In the pediatric population, the tumors' risk of recurrence and multisite development require gynecologists to advocate for complete resection, careful follow-up, and thorough examination for additional tumor sites.
Topics: Child; Child, Preschool; Diagnosis, Differential; Female; Granular Cell Tumor; Humans; Neoplasm Recurrence, Local; Vulva; Vulvar Neoplasms
PubMed: 29305965
DOI: 10.1016/j.jpag.2017.12.010 -
Obstetrics and Gynecology Mar 2023Vulvar Paget disease often requires extensive and, in some cases, multiple resections to treat. A fluorescein-mapping procedure followed by a staged vulvectomy may be an...
BACKGROUND
Vulvar Paget disease often requires extensive and, in some cases, multiple resections to treat. A fluorescein-mapping procedure followed by a staged vulvectomy may be an effective technique to tailor resection and identify clinically occult lesions.
TECHNIQUE
We describe a two-step procedure; first, intravenous fluorescein sodium is injected, and the vulva is illuminated with a Wood's lamp. Representative biopsies are obtained and correlated on final pathology with the extent of disease to develop a final plan for excision. Second, using fluorescein to identify the confirmed areas of disease, the appropriate excisional procedure is performed once mapping biopsy pathology is known.
EXPERIENCE
We describe our experience with eight patients with vulvar Paget disease undergoing fluorescein mapping biopsies and staged vulvectomy. Using intravenous fluorescein sodium, all patients were found to have Paget disease beyond the visible margins of their gross lesions. No patients experienced a recurrence of Paget disease within a median follow-up time of 32 months, comparable with other directed methods of surgical resection.
CONCLUSION
We report a technique for the injection of fluorescein sodium for the visualization of vulvar Paget disease capable of providing accurate surgical margins and identification of occult satellite lesions with a high degree of safety and a favorable cost profile. This staged approach to vulvectomy could offer improved accuracy of resection for vulvar Paget disease with few drawbacks.
Topics: Female; Humans; Fluorescein; Vulvar Neoplasms; Vulva; Paget Disease, Extramammary; Biopsy; Neoplasm Recurrence, Local
PubMed: 36735397
DOI: 10.1097/AOG.0000000000005084