A rare germ-cell tumor site: vaginal endodermal sinus tumor. 2002

M Arora, and R K Shrivastav, and M P Jaiprakash
Department of Pediatric Surgery, Command Hospital, Kolkata, India. dr_manu_arora@hotmail.com

Malignant germ-cell tumors (MGCT) are rare tumors of childhood accounting for less than 3% of pediatric malignancies. Endodermal sinus tumor (EST) forms the most common histologic subtype of MGCT. The vagina is an extremely rare site for GCTs. A 9-month-old female was admitted with a short history of vaginal bleeding, a mass protruding from the vagina, and difficulty in passing urine. She was pale, the bladder was full, and a mass was palpable anteriorly on rectal examination. Ultrasound showed an ovoid, hyperechoic mass posterior to the bladder. A biopsy revealed a vaginal EST. The serum alpha-fetoprotein (AFP) was elevated partial vaginectomy was done and the tumor was excised in toto. The patient was subsequently given six courses of cis-platinum, etoposide, and bleomycin and on follow-up continues to be free from disease. EST is the most common GCT in children. In females, it is usually encountered in the ovary. EST of the vagina is a rare, highly malignant GCT that exclusively involves children less than 3 years of age. The diagnosis is based on histology and raised AFP. Vaginal EST is both locally aggressive and capable of metastasis. Untreated patients have died within 2 to 4 months of presentation. Radical surgery leads to a loss of sexual and reproductive function. Long-term irradiation has secondary effects of sterility, aseptic necrosis of the femoral head, and abnormal growth of the pelvic bones. Partial vaginectomy with combination chemotherapy is the most recommended line of treatment. The surgery eradicates local tumor cells and makes subsequent chemotherapy more effective. Simple tumor excision is not sufficient, as residual tumor cells induce early recurrence and make chemotherapy ineffective. The serum AFP level is a useful marker for diagnosis and monitoring the recurrence of vaginal EST in infants.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014057 Tomography, X-Ray Computed Tomography using x-ray transmission and a computer algorithm to reconstruct the image. CAT Scan, X-Ray,CT Scan, X-Ray,Cine-CT,Computerized Tomography, X-Ray,Electron Beam Computed Tomography,Tomodensitometry,Tomography, Transmission Computed,X-Ray Tomography, Computed,CAT Scan, X Ray,CT X Ray,Computed Tomography, X-Ray,Computed X Ray Tomography,Computerized Tomography, X Ray,Electron Beam Tomography,Tomography, X Ray Computed,Tomography, X-Ray Computer Assisted,Tomography, X-Ray Computerized,Tomography, X-Ray Computerized Axial,Tomography, Xray Computed,X Ray Computerized Tomography,X Ray Tomography, Computed,X-Ray Computer Assisted Tomography,X-Ray Computerized Axial Tomography,Beam Tomography, Electron,CAT Scans, X-Ray,CT Scan, X Ray,CT Scans, X-Ray,CT X Rays,Cine CT,Computed Tomography, Transmission,Computed Tomography, X Ray,Computed Tomography, Xray,Computed X-Ray Tomography,Scan, X-Ray CAT,Scan, X-Ray CT,Scans, X-Ray CAT,Scans, X-Ray CT,Tomographies, Computed X-Ray,Tomography, Computed X-Ray,Tomography, Electron Beam,Tomography, X Ray Computer Assisted,Tomography, X Ray Computerized,Tomography, X Ray Computerized Axial,Transmission Computed Tomography,X Ray Computer Assisted Tomography,X Ray Computerized Axial Tomography,X Ray, CT,X Rays, CT,X-Ray CAT Scan,X-Ray CAT Scans,X-Ray CT Scan,X-Ray CT Scans,X-Ray Computed Tomography,X-Ray Computerized Tomography,Xray Computed Tomography
D014625 Vaginal Neoplasms Tumors or cancer of the VAGINA. Cancer of Vagina,Vaginal Cancer,Cancer of the Vagina,Neoplasms, Vaginal,Vagina Cancer,Vagina Neoplasms,Cancer, Vagina,Cancer, Vaginal,Cancers, Vagina,Cancers, Vaginal,Neoplasm, Vagina,Neoplasm, Vaginal,Neoplasms, Vagina,Vagina Cancers,Vagina Neoplasm,Vaginal Cancers,Vaginal Neoplasm
D018240 Endodermal Sinus Tumor An unusual and aggressive tumor of germ-cell origin that reproduces the extraembryonic structures of the early embryo. It is the most common malignant germ cell tumor found in children. It is characterized by a labyrinthine glandular pattern of flat epithelial cells and rounded papillary processes with a central capillary (Schiller-Duval body). The tumor is rarely bilateral. Before the use of combination chemotherapy, the tumor was almost invariably fatal. (From DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1189) Yolk Sac Tumor,Endodermal Sinus Tumors,Tumor, Endodermal Sinus,Tumor, Yolk Sac,Tumors, Endodermal Sinus,Tumors, Yolk Sac,Yolk Sac Tumors

Related Publications

M Arora, and R K Shrivastav, and M P Jaiprakash
September 2005, Indian journal of pediatrics,
M Arora, and R K Shrivastav, and M P Jaiprakash
July 1979, Journal of cancer research and clinical oncology,
M Arora, and R K Shrivastav, and M P Jaiprakash
September 1985, The Ceylon medical journal,
M Arora, and R K Shrivastav, and M P Jaiprakash
January 1998, Acta cytologica,
M Arora, and R K Shrivastav, and M P Jaiprakash
September 2023, Cureus,
M Arora, and R K Shrivastav, and M P Jaiprakash
February 1977, American journal of obstetrics and gynecology,
M Arora, and R K Shrivastav, and M P Jaiprakash
January 2000, Clinical and experimental obstetrics & gynecology,
M Arora, and R K Shrivastav, and M P Jaiprakash
March 1976, Annales de pediatrie,
M Arora, and R K Shrivastav, and M P Jaiprakash
June 2009, Cancer imaging : the official publication of the International Cancer Imaging Society,
Copied contents to your clipboard!