Endodermal sinus tumor
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Endodermal sinus tumor (EST) is part of the germ cell tumor group of cancers. It is the most common testicular tumor in children under three and is also referred to as infantile embryonal carcinoma and yolk sac tumor. This age group has a very good prognosis. In contrast to the pure form seen in infants, adult endodermal sinus tumors are often found in combination with other types of germ cell tumors, particularly teratoma and embryonal carcinoma. While pure teratoma is usually benign, endodermal sinus tumor is malignant.
Cause
The causes of this cancer are poorly understood.
Diagnosis
The histology of EST is variable, but it typically includes malignant endodermal cells. These cells secrete alpha-fetoprotein (AFP), which can be detected in tumor tissue, serum, cerebrospinal fluid, urine, and in the rare case of fetal EST, in amniotic fluid. When there is incongruence between biopsy and AFP test results for EST, the result indicating the presence of EST dictates treatment. This is because EST often occurs as small "malignant foci" within a larger tumor, usually teratoma, and biopsy is a sampling method; thus, a biopsy of the tumor may reveal only teratoma, while elevated AFP indicates that EST is also present. GATA-4, a transcription factor, may also be useful in diagnosing EST.
The diagnosis of EST in pregnant women and infants is complicated by the extremely high levels of AFP in these two groups. Tumor surveillance through monitoring AFP requires precise adjustments for gestational age in pregnant women and age in infants. For pregnant women, this can be accomplished by testing maternal serum AFP instead of tumor marker AFP. In infants, the tumor marker test is employed but must be interpreted using a reference table or graph of normal AFP levels in infants.
Treatment
Most treatments involve a combination of surgery and chemotherapy. Treatment using cisplatin, etoposide, and bleomycin has been documented. Before the advent of modern chemotherapy, this type of neoplasm was highly lethal, but the prognosis has significantly improved since then. When endodermal sinus tumors are treated promptly with surgery and chemotherapy, fatal outcomes are exceedingly rare.
- More information is available at [ Wikipedia:Endodermal_sinus_tumor ]
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