Endodermal sinus tumor
Editor-in-Chief: Somal Khan, M.D.
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| Endodermal sinus tumor | |
| ICD-O: | 9071/3 |
|---|---|
| DiseasesDB | 4248 |
| MeSH | D018240 |
Overview
Endodermal sinus tumor (EST), also known as yolk sac tumor (YST), is a member of the germ cell tumor group of cancers. Endodermal sinus tumor frequently develops within other kinds of germ cell tumor, particularly within teratoma. While pure teratoma is usually benign, endodermal sinus tumor is malignant.
Diagnosis
The histology of EST is variable, but usually includes malignant endodermal cells. These cells express alpha-fetoprotein (AFP), which can be detected in tumor tissue or serum. When there is incongruence between biopsy and AFP test results for EST, the result indicating presence of EST should guide treatment.[1] This is because EST often occurs as small "malignant foci" within a larger tumor, usually teratoma; biopsy of the tumor may reveal only teratoma, whereas elevated AFP in serum or cerebrospinal fluid reveals that EST is also present. GATA-4, a transcription factor, also may be useful in the diagnosis of EST.[2]
Treatment
Most treatments involve some combination of surgery and chemotherapy.
Before modern chemotherapy, this type of neoplasm was highly lethal, but the prognosis has significantly improved since.
References
- ↑ Luther N, Edgar MA, Dunkel IJ, Souweidane MM (2006). "Correlation of endoscopic biopsy with tumor marker status in primary intracranial germ cell tumors". J. Neurooncol. 79 (1): 45–50. doi:10.1007/s11060-005-9110-0. PMID 16598424.
- ↑ Siltanen S, Anttonen M, Heikkilä P, Narita N, Laitinen M, Ritvos O, Wilson DB, Heikinheimo M (1999). "Transcription factor GATA-4 is expressed in pediatric yolk sac tumors". Am. J. Pathol. 155 (6): 1823–9. PMID 10595911. (free full text on PubMed Central)