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Case Report
An female infant patient, aged 8 months old, suffered from irregular colporrhagia for a period of 1 month after which she was taken to our hospital on 30th April, 2003. A pelvic CT examination displayed a 6.5 cm×3.0 cm shadow of a soft-tissue tumor growing longitudinally in her supravaginal uterine area (Fig.1). The density of the shadow was uneven, in which there were irregular low-density foci, an indication of a compression of the colon and bladder and a diffuse boundary between the posterior wall of the urinary bladder and tumor. No abnormalities were found in either kidney or ovary, the liver or gall bladder. Also no obvious lesions were seen on the chest X-ray film, and routine blood and urine laboratory examinations were normal. Her bleeding and clotting time were normal, with a blood level of 13,890 μg/L of serous α-fetoprotein (α-FP or AFP), and hCG of less than 10 IU/L. On the 10th of May, 2003, she received a vaginal smear examination under general anesthesia. The result of a cytopathology examination suggested a botryoid sarcoma cell.
Fig.1A (Sagittal plane) Fig.1B (Transection) Pelvic CT image of Fig. 1 shows that shadow of the soft-tissue neoplasm is seen in the supravaginal portion and uterine area, with a uneven density. Irregular foci of low density can be found, with presure on the rectum and bladder and an ill-defined boundary between the posterior wall of the urinary bladder and neoplasm.
On 20th May, 2003, a fragile sphacelus of 3 cm×2 cm prolapsed from the vagina. It was pathologically diagnosed as an endodermal sinus tumor. The results of immunohistochemical studies were as follows: focal AFP (+), HCG (-), S-100 (-) and PAS (+) (Fig.2). The chemotherapeutic PEB regimen was employed, i.e., administration of cisplatin, etoposide and bleomycin (cisplastin 20 mg/m2 at day 1 to day 3; etoposide 100 mg/m2 at day 1 to day 3; bleomycin 20 mg/m2 at day 2, with 21 days per course of treatment). The patient responded to the PEB regimen. After the first course of chemotherapy, the α-FP showed a logarithmic decrease, and after the second course, the α-FP level had returned to normal and remained at this low level. A pelvic CT scan was conducted after the third course of treatment showing that the nodi below the cervix had completely vanished. A vaginal biopsy was performed under general anesthesia, and 4 sphaceli, each with a size of a grain of rice were taken out, at 4 to 5 rough surfaces on the area of the fornix. No other abnormalities were found.
Arrangement of the tumor cells presents a loop-like structure of the glomerular blood vessel.
A pathological examination of the sphaceli indicated a squamous epithelium and fibrous tissue and, below the epithelium, there were a few inflamed cells and cumulated foam cells. Then one more course of PEB regimen was used for an follow-up treatment. Chemotherapy ceased on the 15th August, 2003 and now the patient is being followed-up. The tumor-free survival of the patient has lasted for 3 years plus 3 months.
Discussion
Endodermal sinus tumors are common in juveniles and children with the most frequent sites being the genital organs and sacroiliac region, but primary endodermal sinus tumors of the vagina are rare. The extragonadal endodermal sinus tumor of our patient was brought about by an aberration or residual tumor of the embryonic migration of archeocyte from the genital ridge endoderm of the yolk sac. The condition of the patient developed rapidly because of its high malignancy. Therefore, early diagnosis is the key for improving the therapeutic efficacy[1].
Diagnosis of an endodermal sinus vaginal tumor mainly relies on a pathological examination, however, comprehensive recognition of the disease has been lacking in the past, for instance, it usually has been misdiagnosed as a clear-cell carcinoma, mesonephric carcinoma, carcinoma of the Woffian duct, undifferentiated sarcoma or botryoid sarcoma, etc[2-5].
An endodermal sinus tumor has the biological feature of synthesizing α-FP, and therefore estimation of the α-FP level is of important clinical significance for early tumor diagnosis. The intermittent watery secretions of the vagina are the most commonly-encountered clinical manifestations of the disease. So, serous α-FP screening and anal examinations are needed for the infants with bloody vaginal secretions to make a definitive diagnosis followed by therapy.
- Received December 29, 2006.
- Accepted January 23, 2007.
- Copyright © 2007 by Tianjin Medical University Cancer Institute & Hospital and Springer
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