Endodermal sinus tumor

Infobox_Disease
Name = Endodermal sinus tumor


Caption =
DiseasesDB = 4248
ICD10 =
ICD9 =
ICDO = 9071/3
OMIM =
MedlinePlus =
eMedicineSubj =
eMedicineTopic =
MeshID = D018240

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.

Diagnosis

The histology of EST is variable, but usually 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 presence of EST dictates treatment.cite journal
author = Luther N, Edgar MA, Dunkel IJ, Souweidane MM
title = Correlation of endoscopic biopsy with tumor marker status in primary intracranial germ cell tumors.
journal = J. Neurooncol.
volume = 79
issue = 1
pages = 45–50
year = 2006
pmid = 16598424
doi = 10.1007/s11060-005-9110-0
issn = ] This is because EST often occurs as small "malignant foci" within a larger tumor, usually teratoma, and biopsy is a sampling method; biopsy of the tumor may reveal only teratoma, whereas elevated AFP reveals that EST is also present. GATA-4, a transcription factor, also may be useful in the diagnosis of EST.cite journal
author = Siltanen S, Anttonen M, Heikkilä P, Narita N, Laitinen M, Ritvos O, Wilson DB, Heikinheimo M
title = Transcription factor GATA-4 is expressed in pediatric yolk sac tumors.
journal = Am. J. Pathol.
volume = 155
issue = 6
pages = 1823–9
year = 1999
pmid = 10595911
doi =
issn = (free full text on PubMed Central)]
Schiller-Duval bodies on histology are pathognomonic.

Diagnosis of EST in pregnant women and in infants is complicated by the normal extremely high levels of AFP in those two groups. Tumor surveillance by monitoring AFP requires accurate correction for gestational age in pregnant women, and age in infants.Fact|date=August 2008 In pregnant women, this can be achieved simply by testing maternal serum AFP rather than tumor marker AFP. In infants, the tumor marker test is used, but must be interpreted using a reference table or graph of normal AFP in infants.

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.

References