Objective: Vulvar cancer is rare in Eastern females, especially in pre- and peripubertal girls. The prognosis of vulvar cancer is poor and treatment is variable.
Case Report: A 14-year-old girl suffered from a left vulvar tenderness mass and underwent excision of the mass. The diagnosis of the pathology was vulvar yolk tumor with an embryonal carcinoma. After the vulvectomy, inguinal area lymph node dissection, chemotherapy (bleomycin, etoposide and cisplatin) treatment and radiotherapy, metastasis to lung was also noted after eight months. Resection of lung tumor was performed. She received chemotherapy with a combination of paclitaxel, ifosphamide and cisplatin (TIP) and received peripheral blood stem cell transplantation (PBSCT) twice and chemotherapy treatment of gemitabine and oxaliplatin (GEMOX). Up until now, the patient has been free of disease.
Conclusion: High-dose TIP and GEMOX chemotherapy plus PBSCT for bone marrow rescues could be considered to treat patients with metastatic malignant vulvar germ cell tumor after failed first-line chemotherapy and radiation.
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Underreporting alteration by clinical sequencing: Integrative patho-genomic analysis captured /INI-1 deficiency in a vulvar yolk sac tumor.
Gynecol Oncol Rep
December 2023
Department of Pediatrics, City of Hope Medical Center, Duarte, CA, USA.
•/INI1-deficient gynecologic tumors are rare and clinically aggressive. A subset shows primitive yolk sac tumor features.•Due to technical limitation of next generation sequencing (NGS) and interlaboratory variability in sequencing methodologies and analytical pipelines, deficiency caused by somatic copy number variations (SCNV) may be underreported by NGS.
View Article and Find Full Text PDFA Subset of SMARCB1 (INI-1)-deficient vulvar neoplasms express germ cell markers.
Histopathology
September 2022
Department of Pathology, Stanford University School of Medicine, Stanford, CA, USA.
Aims: SMARCB1 (INI-1)-deficient vulvar neoplasms comprise a group of rare tumours that include epithelioid sarcoma (ES), myoepithelial carcinoma (MEC), the recently described myoepithelioma-like tumour of the vulvar region (MELTVR), and sarcomas that are difficult to classify. It has been suggested that so-called vulvar yolk sac tumours (YST) may represent morphologic variants of SMARCB1-deficient tumours; thus, we investigated the immunoreactivity of germ cell markers in SMARCB1-deficient vulvar neoplasms.
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Vulvar Yolk Sac Tumors Are Somatically Derived SMARCB1 (INI-1)-Deficient Neoplasms.
Am J Surg Pathol
February 2022
Department of Pathology, Stanford University Medical Center, Stanford, CA.
So-called primary yolk sac tumors of the vulva are very rare and often have an aggressive disease course. Their molecular features have not been previously characterized. There is also a well-documented group of SMARCB1 (INI-1)-deficient vulvar neoplasms, which includes proximal-type epithelioid sarcoma and myoepithelial carcinoma.
View Article and Find Full Text PDFPediatric vulvar malignancies: rare but important to know.
Semin Diagn Pathol
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Department of Pathology, University of California San Diego, San Diego, CA, USA. Electronic address:
Malignancies of the vulva in the pediatric population are exceptionally rare, which makes it difficult to gain any insight into their clinicopathologic profile. In this review, we summarize all published cases of a vulva malignancy in pediatric patients (≤21 years) reported in the English language literature for the 50-year period between 1970 and 2020. We estimate that less than 100 malignancies have been reported in total, approximately 50% of which were rhabdomyosarcomas.
View Article and Find Full Text PDFPrepubertal Vaginal Bleeding: An Inpatient Series from a Single Center in Fujian China.
J Pediatr Adolesc Gynecol
April 2020
Department of Gynecology, Fujian Provincial Maternity and Children's Hospital, Fuzhou, China. Electronic address:
Study Objective: This study characterizes the etiology, clinical features and outcomes of prepubescent vaginal bleeding and summarizes our experience.
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Setting: A provincial Grade III Level A Maternity and Children's Hospital.
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