From Wikipedia, the free encyclopedia
Endodermal sinus tumor (EST), also known as yolk sac tumor (YST), is a member of the germ cell tumor group of cancers. It is the most common testicular tumor in children under 3, and is also known as infantile embryonal carcinoma. This age group has a very good prognosis. In contrast to the pure form typical of infants, adult endodermal sinus tumors are often found in combination with other kinds of germ cell tumor, particularly teratoma and embryonal carcinoma. While pure teratoma is usually benign, endodermal sinus tumor is malignant.
[edit] 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] Schiller-Duval bodies on histology are pathognomonic.
[edit] 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.
90% survival rate when treated effectively.
[edit] 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)
[edit] External links
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Pathology: Cancer, Tumors, Neoplasms, and oncology (C00-D48, 140-239) |
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| Malignant progression |
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| Topography |
lip, oral cavity and pharynx: Oral - Head/Neck - Nasopharyngeal
digestive system: tract (Esophagus, Stomach, Small intestine, Colon/rectum, Appendix, Anus) - glands (Liver, Bile duct, Gallbladder, Pancreas)
respiratory system: Larynx - Lung
bone, articular cartilage, skin, and connective tissue: Bone (Tailbone) - Skin - Blood
urogenital: breast and female genital organs (Breast, Vulva, Vagina, Cervix, Uterus, Endometrium, Ovaries) - male genital organs (Penis, Prostate, Testicles) - urinary organs (Kidney, Bladder)
nervous system: Eye (Uvea) - Brain (Choroid plexus)
endocrine system: Thyroid ( Papillary, Follicular, Medullary, Anaplastic) - Adrenal tumor ( Adrenocortical carcinoma, Pheochromocytoma) - Pituitary
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