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==Epidemiology and Demographics==
==Epidemiology and Demographics==
*In 2015, the incidence of endodermal sinus tumor is estimated to less than 1/100,000 case per year in U.S.<ref name="pmid1111694">{{cite journal| author=Young JL, Miller RW| title=Incidence of malignant tumors in U. S. children. | journal=J Pediatr | year= 1975 | volume= 86 | issue= 2 | pages= 254-8 | pmid=1111694 | doi=10.1016/s0022-3476(75)80484-7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1111694  }} </ref>
*In 2015, the [[incidence]] of endodermal sinus tumor is estimated to less than 1/100,000 case per year in U.S.<ref name="pmid1111694">{{cite journal| author=Young JL, Miller RW| title=Incidence of malignant tumors in U. S. children. | journal=J Pediatr | year= 1975 | volume= 86 | issue= 2 | pages= 254-8 | pmid=1111694 | doi=10.1016/s0022-3476(75)80484-7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1111694  }} </ref>
===Age===
===Age===
*Endodermal sinus tumor have a bimodal distribution more commonly observed among young and adult population<ref name="pmid18767035">{{cite journal| author=Shah JP, Kumar S, Bryant CS, Ali-Fehmi R, Malone JM, Deppe G et al.| title=A population-based analysis of 788 cases of yolk sac tumors: A comparison of males and females. | journal=Int J Cancer | year= 2008 | volume= 123 | issue= 11 | pages= 2671-5 | pmid=18767035 | doi=10.1002/ijc.23792 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18767035  }} </ref>
*Endodermal sinus tumor have a [[bimodal distribution]] more commonly observed among [[Young adult|young]] and [[Adult|adult population]]<ref name="pmid18767035">{{cite journal| author=Shah JP, Kumar S, Bryant CS, Ali-Fehmi R, Malone JM, Deppe G et al.| title=A population-based analysis of 788 cases of yolk sac tumors: A comparison of males and females. | journal=Int J Cancer | year= 2008 | volume= 123 | issue= 11 | pages= 2671-5 | pmid=18767035 | doi=10.1002/ijc.23792 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18767035  }} </ref>
* Pure yolk sac tumor (endodermal sinus tumor) affects mostly [[children]] of less 2 years old.<ref name="pmid8127257">{{cite journal| author=Coppes MJ, Rackley R, Kay R| title=Primary testicular and paratesticular tumors of childhood. | journal=Med Pediatr Oncol | year= 1994 | volume= 22 | issue= 5 | pages= 329-40 | pmid=8127257 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8127257  }} </ref>
* Pure yolk sac tumor (endodermal sinus tumor) affects mostly [[children]] of less 2 years old.<ref name="pmid8127257">{{cite journal| author=Coppes MJ, Rackley R, Kay R| title=Primary testicular and paratesticular tumors of childhood. | journal=Med Pediatr Oncol | year= 1994 | volume= 22 | issue= 5 | pages= 329-40 | pmid=8127257 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8127257  }} </ref>
===Gender===
===Gender===
*Endodermal sinus tumor affects men and women equally.
*Endodermal sinus tumor affects [[men]] and women equally.
===Race===
===Race===
*There is no racial predilection for endodermal sinus tumor.
*There is no [[racial]] predilection for endodermal sinus tumor.
== Natural History, Complications and Prognosis==
== Natural History, Complications and Prognosis==
*Endodermal sinus tumor has a poor [[prognosis]] in [[adult]].<ref name="pmid12432104">{{cite journal| author=Jung SE, Lee JM, Rha SE, Byun JY, Jung JI, Hahn ST| title=CT and MR imaging of ovarian tumors with emphasis on differential diagnosis. | journal=Radiographics | year= 2002 | volume= 22 | issue= 6 | pages= 1305-25 | pmid=12432104 | doi=10.1148/rg.226025033 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12432104  }} </ref><ref name="pmid18063508">{{cite journal| author=Hung JH, Shen SH, Hung J, Lai CR| title=Ultrasound and magnetic resonance images of endodermal sinus tumor. | journal=J Chin Med Assoc | year= 2007 | volume= 70 | issue= 11 | pages= 514-8 | pmid=18063508 | doi=10.1016/S1726-4901(08)70052-2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18063508  }} </ref>
*Endodermal sinus tumor has a poor [[prognosis]] in [[adult]].<ref name="pmid12432104">{{cite journal| author=Jung SE, Lee JM, Rha SE, Byun JY, Jung JI, Hahn ST| title=CT and MR imaging of ovarian tumors with emphasis on differential diagnosis. | journal=Radiographics | year= 2002 | volume= 22 | issue= 6 | pages= 1305-25 | pmid=12432104 | doi=10.1148/rg.226025033 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12432104  }} </ref><ref name="pmid18063508">{{cite journal| author=Hung JH, Shen SH, Hung J, Lai CR| title=Ultrasound and magnetic resonance images of endodermal sinus tumor. | journal=J Chin Med Assoc | year= 2007 | volume= 70 | issue= 11 | pages= 514-8 | pmid=18063508 | doi=10.1016/S1726-4901(08)70052-2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18063508  }} </ref>
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*If left untreated, endodermal sinus tumor quickly [[Metastasize|metastasizes]] in other parts of the [[body]] such as the [[brain]].<ref name="urlDefinition of endodermal sinus tumor - NCI Dictionary of Cancer Terms - National Cancer Institute">{{cite web |url=https://www.cancer.gov/publications/dictionaries/cancer-terms/def/791307 |title=Definition of endodermal sinus tumor - NCI Dictionary of Cancer Terms - National Cancer Institute |format= |work= |accessdate=}}</ref>
*If left untreated, endodermal sinus tumor quickly [[Metastasize|metastasizes]] in other parts of the [[body]] such as the [[brain]].<ref name="urlDefinition of endodermal sinus tumor - NCI Dictionary of Cancer Terms - National Cancer Institute">{{cite web |url=https://www.cancer.gov/publications/dictionaries/cancer-terms/def/791307 |title=Definition of endodermal sinus tumor - NCI Dictionary of Cancer Terms - National Cancer Institute |format= |work= |accessdate=}}</ref>
*Endodermal sinus tumor can be found in the [[ovaries]] or [[testicles]] including the [[chest]], [[abdomen]], and the [[brain]].<ref name="urlDefinition of endodermal sinus tumor - NCI Dictionary of Cancer Terms - National Cancer Institute">{{cite web |url=https://www.cancer.gov/publications/dictionaries/cancer-terms/def/791307 |title=Definition of endodermal sinus tumor - NCI Dictionary of Cancer Terms - National Cancer Institute |format= |work= |accessdate=}}</ref>
*Endodermal sinus tumor can be found in the [[ovaries]] or [[testicles]] including the [[chest]], [[abdomen]], and the [[brain]].<ref name="urlDefinition of endodermal sinus tumor - NCI Dictionary of Cancer Terms - National Cancer Institute">{{cite web |url=https://www.cancer.gov/publications/dictionaries/cancer-terms/def/791307 |title=Definition of endodermal sinus tumor - NCI Dictionary of Cancer Terms - National Cancer Institute |format= |work= |accessdate=}}</ref>
*Ovarian endodermal sinus tumor metastasizes quiclky into the intraabdominal area compared to testicular endodermal sinus tumor.<ref>{{cite book | last = Saia | first = Philip | title = Clinical gynecologic oncology | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 978-0-323-40067-1 }} </ref>
*Ovarian endodermal sinus tumor [[Metastasize|metastasizes]] quiclky into the intraabdominal area compared to [[testicular]] endodermal sinus tumor.<ref>{{cite book | last = Saia | first = Philip | title = Clinical gynecologic oncology | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 978-0-323-40067-1 }} </ref>
== Diagnosis ==
== Diagnosis ==
=== Diagnostic criteria ===
=== Diagnostic criteria ===
*The diagnosis of endodermal sinus tumor is made based on the imaging and alpha fetoprotein (AFP) tumor marker greater than 100 ng/ml.<ref name="pmid6155988">{{cite journal| author=Talerman A, Haije WG, Baggerman L| title=Serum alphafetoprotein (AFP) in patients with germ cell tumors of the gonads and extragonadal sites: correlation between endodermal sinus (yolk sac) tumor and raised serum AFP. | journal=Cancer | year= 1980 | volume= 46 | issue= 2 | pages= 380-5 | pmid=6155988 | doi=10.1002/1097-0142(19800715)46:2<380::aid-cncr2820460228>3.0.co;2-u | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6155988  }} </ref><ref>{{cite book | last = Saia | first = Philip | title = Clinical gynecologic oncology | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 978-0-323-40067-1 }} </ref>
*The diagnosis of endodermal sinus tumor is made based on the imaging and alpha fetoprotein (AFP) tumor marker greater than 100 ng/ml.<ref name="pmid6155988">{{cite journal| author=Talerman A, Haije WG, Baggerman L| title=Serum alphafetoprotein (AFP) in patients with germ cell tumors of the gonads and extragonadal sites: correlation between endodermal sinus (yolk sac) tumor and raised serum AFP. | journal=Cancer | year= 1980 | volume= 46 | issue= 2 | pages= 380-5 | pmid=6155988 | doi=10.1002/1097-0142(19800715)46:2<380::aid-cncr2820460228>3.0.co;2-u | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6155988  }} </ref><ref>{{cite book | last = Saia | first = Philip | title = Clinical gynecologic oncology | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 978-0-323-40067-1 }} </ref>
=== Symptoms ===
=== Symptoms ===
*Endodermal sinus tumor is usually asymptomatic.
*Endodermal sinus tumor is usually [[asymptomatic]].
*Most children and young adults with endodermal sinus tumor have stage I.<ref>{{cite book | last = Weidner | first = Noel | title = Modern surgical pathology | publisher = Saunders/Elsevier | location = Philadelphia, PA | year = 2009 | isbn = 978-1-4160-3966-2 }}</ref>
*Most [[children]] and [[young adults]] with endodermal sinus tumor have stage I.<ref>{{cite book | last = Weidner | first = Noel | title = Modern surgical pathology | publisher = Saunders/Elsevier | location = Philadelphia, PA | year = 2009 | isbn = 978-1-4160-3966-2 }}</ref>
*Symptoms of endodermal sinus tumor may include the following:<ref name="www">{{cite book | last = Hoffman | first = Barbara | title = Williams gynecology | publisher = McGraw-Hill Medical | location = New York | year = 2012 | isbn = 9780071716727 }}</ref> <ref name="pmid6185892">{{cite journal| author=Gershenson DM, Del Junco G, Herson J, Rutledge FN| title=Endodermal sinus tumor of the ovary: the M. D. Anderson experience. | journal=Obstet Gynecol | year= 1983 | volume= 61 | issue= 2 | pages= 194-202 | pmid=6185892 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6185892  }} </ref>
*Symptoms of endodermal sinus tumor may include the following:<ref name="www">{{cite book | last = Hoffman | first = Barbara | title = Williams gynecology | publisher = McGraw-Hill Medical | location = New York | year = 2012 | isbn = 9780071716727 }}</ref> <ref name="pmid6185892">{{cite journal| author=Gershenson DM, Del Junco G, Herson J, Rutledge FN| title=Endodermal sinus tumor of the ovary: the M. D. Anderson experience. | journal=Obstet Gynecol | year= 1983 | volume= 61 | issue= 2 | pages= 194-202 | pmid=6185892 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6185892  }} </ref>
:*[[Abdominal distention]]
:*[[Abdominal distention]]
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*Patients with endodermal sinus tumor usually appear normal.
*Patients with endodermal sinus tumor usually appear normal.
*Physical examination may be remarkable for:<ref name="abc">{{cite book | last = Hoffman | first = Barbara | title = Williams gynecology | publisher = McGraw-Hill Medical | location = New York | year = 2012 | isbn = 9780071716727 }}</ref>
*Physical examination may be remarkable for:<ref name="abc">{{cite book | last = Hoffman | first = Barbara | title = Williams gynecology | publisher = McGraw-Hill Medical | location = New York | year = 2012 | isbn = 9780071716727 }}</ref>
:*[[Abdominal distention]]  
 
:*[[Abdominal distention]]
:*Abdominal [[tenderness]]
:*Abdominal [[tenderness]]
:*Adnexal mass
:*[[Adnexal]] [[mass]]
:*Testicular mass
:*[[Testicular mass]]
 
=== Laboratory Findings ===
=== Laboratory Findings ===
*An elevated concentration of serum alpha feto-protein is diagnostic of endodermal sinus tumor.<ref name="pmid6155988">{{cite journal| author=Talerman A, Haije WG, Baggerman L| title=Serum alphafetoprotein (AFP) in patients with germ cell tumors of the gonads and extragonadal sites: correlation between endodermal sinus (yolk sac) tumor and raised serum AFP. | journal=Cancer | year= 1980 | volume= 46 | issue= 2 | pages= 380-5 | pmid=6155988 | doi=10.1002/1097-0142(19800715)46:2<380::aid-cncr2820460228>3.0.co;2-u | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6155988  }} </ref>
*An elevated concentration of [[serum]] [[alpha fetoprotein]] is a [[diagnostic]] of endodermal sinus tumor.<ref name="pmid6155988">{{cite journal| author=Talerman A, Haije WG, Baggerman L| title=Serum alphafetoprotein (AFP) in patients with germ cell tumors of the gonads and extragonadal sites: correlation between endodermal sinus (yolk sac) tumor and raised serum AFP. | journal=Cancer | year= 1980 | volume= 46 | issue= 2 | pages= 380-5 | pmid=6155988 | doi=10.1002/1097-0142(19800715)46:2<380::aid-cncr2820460228>3.0.co;2-u | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6155988  }} </ref>
*SALL4 (sal-like protein 4) is positive in endodermal sinus tumor.<ref>{{cite book | last = Crum | first = Christopher | title = Diagnostic gynecologic and obstetric pathology | publisher = Elsevier, Inc | location = Philadelphia, PA | year = 2018 | isbn = 978-0-323-44732-4 }} </ref>
*SALL4 (sal-like protein 4) is [[positive]] in endodermal sinus tumor.<ref>{{cite book | last = Crum | first = Christopher | title = Diagnostic gynecologic and obstetric pathology | publisher = Elsevier, Inc | location = Philadelphia, PA | year = 2018 | isbn = 978-0-323-44732-4 }} </ref>
* AFP is very important for diagnosis, disease monitoring and early metastasis.<ref name="pmid6155988">{{cite journal| author=Talerman A, Haije WG, Baggerman L| title=Serum alphafetoprotein (AFP) in patients with germ cell tumors of the gonads and extragonadal sites: correlation between endodermal sinus (yolk sac) tumor and raised serum AFP. | journal=Cancer | year= 1980 | volume= 46 | issue= 2 | pages= 380-5 | pmid=6155988 | doi=10.1002/1097-0142(19800715)46:2<380::aid-cncr2820460228>3.0.co;2-u | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6155988  }} </ref>
*[[Alpha-fetoprotein|AFP]] is very important for [[diagnosis]], [[disease]] monitoring and early [[metastasis]].<ref name="pmid6155988">{{cite journal| author=Talerman A, Haije WG, Baggerman L| title=Serum alphafetoprotein (AFP) in patients with germ cell tumors of the gonads and extragonadal sites: correlation between endodermal sinus (yolk sac) tumor and raised serum AFP. | journal=Cancer | year= 1980 | volume= 46 | issue= 2 | pages= 380-5 | pmid=6155988 | doi=10.1002/1097-0142(19800715)46:2<380::aid-cncr2820460228>3.0.co;2-u | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6155988  }} </ref>
===Imaging Findings===
===Imaging Findings===
*On MRI, endodermal sinus tumor is characterized by  areas of [[haemorrhage]] and necrosis.<ref name="pmid18063508">{{cite journal| author=Hung JH, Shen SH, Hung J, Lai CR| title=Ultrasound and magnetic resonance images of endodermal sinus tumor. | journal=J Chin Med Assoc | year= 2007 | volume= 70 | issue= 11 | pages= 514-8 | pmid=18063508 | doi=10.1016/S1726-4901(08)70052-2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18063508  }} </ref>
*On MRI, endodermal sinus tumor is characterized by  areas of [[haemorrhage]] and [[necrosis]].<ref name="pmid18063508">{{cite journal| author=Hung JH, Shen SH, Hung J, Lai CR| title=Ultrasound and magnetic resonance images of endodermal sinus tumor. | journal=J Chin Med Assoc | year= 2007 | volume= 70 | issue= 11 | pages= 514-8 | pmid=18063508 | doi=10.1016/S1726-4901(08)70052-2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18063508  }} </ref>
* Ultrasound shows solid and cystic mass.<ref name="pmid18063508">{{cite journal| author=Hung JH, Shen SH, Hung J, Lai CR| title=Ultrasound and magnetic resonance images of endodermal sinus tumor. | journal=J Chin Med Assoc | year= 2007 | volume= 70 | issue= 11 | pages= 514-8 | pmid=18063508 | doi=10.1016/S1726-4901(08)70052-2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18063508  }} </ref>
* Ultrasound shows [[solid]] and [[cystic]] [[mass]].<ref name="pmid18063508">{{cite journal| author=Hung JH, Shen SH, Hung J, Lai CR| title=Ultrasound and magnetic resonance images of endodermal sinus tumor. | journal=J Chin Med Assoc | year= 2007 | volume= 70 | issue= 11 | pages= 514-8 | pmid=18063508 | doi=10.1016/S1726-4901(08)70052-2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18063508  }} </ref>


=== Other Diagnostic Studies ===
=== Other Diagnostic Studies ===
*Endodermal sinus tumor may also be diagnosed using biopsy and measurement of  GATA-4, a [[transcription factor]].<ref name="pmid10595911">{{cite journal| author=Siltanen S, Anttonen M, Heikkilä P, Narita N, Laitinen M, Ritvos O et al.| title=Transcription factor GATA-4 is expressed in pediatric yolk sac tumors. | journal=Am J Pathol | year= 1999 | volume= 155 | issue= 6 | pages= 1823-9 | pmid=10595911 | doi=10.1016/S0002-9440(10)65500-9 | pmc=1866939 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10595911  }} </ref>
*Endodermal sinus tumor may also be [[Diagnosis|diagnosed]] using [[biopsy]] and measurement of  GATA-4, a [[transcription factor]].<ref name="pmid10595911">{{cite journal| author=Siltanen S, Anttonen M, Heikkilä P, Narita N, Laitinen M, Ritvos O et al.| title=Transcription factor GATA-4 is expressed in pediatric yolk sac tumors. | journal=Am J Pathol | year= 1999 | volume= 155 | issue= 6 | pages= 1823-9 | pmid=10595911 | doi=10.1016/S0002-9440(10)65500-9 | pmc=1866939 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10595911  }} </ref>
== Treatment ==
== Treatment ==
* Endodermal sinus tumor is treated with [[surgery]] depending on the [[tumor]] [[marker]] and [[imaging]] as well as [[fertility]] desired.<ref>"NCCN Clinical Practice Guidelines in Oncology: Endodermal sinus tumor. National comprehensive cancer network, 2017; https://www.nccn.org/patients/guidelines/content/PDF/ovarian.pdf."</ref>
* Endodermal sinus tumor is treated with [[surgery]] depending on the [[tumor]] [[marker]] and [[imaging]] as well as [[fertility]] desired.<ref>"NCCN Clinical Practice Guidelines in Oncology: Endodermal sinus tumor. National comprehensive cancer network, 2017; https://www.nccn.org/patients/guidelines/content/PDF/ovarian.pdf."</ref>
* For positive [[tumor]] [[Marker|markers]] and [[imaging]], [[fertility]] desired
* For positive [[tumor]] [[Marker|markers]] and [[imaging]], [[fertility]] desired
**Treat with [[fertility]]-sapring [[surgery]] and comprehensive [[Staging (pathology)|staging]].
**Treat with [[fertility]]-sapring [[surgery]] and comprehensive [[Staging (pathology)|staging]].
* For positive tumor markers and imaging, fertility not desired
* For [[positive]] [[tumor markers]] and [[imaging]], [[fertility]] not desired
**Treat with completion staging surgery with possible tumor reductive surgery or chemotherapy
**Treat with completion staging surgery with possible [[tumor]] reductive [[surgery]] or [[chemotherapy]]
* For negative imaging and positive or negative tumor markers
* For negative imaging and positive or negative tumor markers
**Treat with chemotherapy
**Treat with chemotherapy

Revision as of 10:22, 23 September 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Gertrude Djouka, M.D.[2]; Monalisa Dmello, M.B,B.S., M.D. [3]; Shivali Marketkar, M.B.B.S. [4]; Ammu Susheela, M.D. [5]

For patient information, click here Synonyms and keywords: Endodermal sinus tumor(EST), yolk sac tumor.

Overview

Endodermal sinus tumor was first discovered by Dr. Gunner Telium, a Danish pathologist, in 1959. The hypermethylation of the RUNX3 gene promoter and overexpression of GATA-4, a transcription factor has been associated with the development of endodermal sinus tumor. Endodermal sinus tumor is a rare type of malignant ovarian tumor that occurs in the second decade of life. On gross pathology, a solid gray-white with gelatinous, myxoid, or mucoid appearance, necrosis, cystic changes, and hemorrhage are characteristic findings of endodermal sinus tumor. On microscopic histopathological analysis, Schiller-Duval bodies is a characteristic finding of endodermal sinus tumor. Endodermal sinus tumor must be differentiated from other diseases that cause pelvic mass, such as Stein-Leventhal syndrome, and seminoma. In 2015, the incidence of endodermal sinus tumor is estimated to less than 1/100,000 case per year in U.S. Endodermal sinus tumor may be classified according to histology groups.The diagnosis of endodermal sinus tumor is made based on the imaging (U/S and MRI) and alpha fetoprotein (AFP) tumor marker greater than 100 ng/ml. Endodermal sinus tumor have a bimodal distribution more commonly observed among young and adult population. The symptoms of endodermal sinus tumor include the followings: abdominal distention, acute/sub acute abdominal pain. Endodermal sinus tumor is treated with surgery depending on the tumor marker and imaging as well as fertility desired.The medical therapy for endodermal sinus tumor is chemotherapy such as bleomycin, etoposide, and cisplatin for 3 to 4 cycles for 21 days.

Historical Perspective

  • Endodermal sinus tumor was first discovered by Dr. Gunner Telium, a Danish pathologist in 1959.[1][2]

Classification

  • Endodermal sinus tumor may be classified according to histology into 10 groups:[3]

Pathophysiology

  • The hypermethylation of the RUNX3 gene promoter and overexpression of GATA-4, a transcription factor has been associated with the development of endodermal sinus tumor.[4]
  • Ovarian endodermal sinus tumor may be result of differentiation of primitive malignant germ cell components of vitelline structures.[5]
  • On gross pathology, encaptulated, firm, smooth, round, globular, solid gray-white with a gelatinous, myxoid, or mucoid appearance, necrosis, cystic changes, and hemorrhage are characteristic findings of endodermal sinus tumor.[6]
  • On microscopic histopathological analysis, Schiller-Duval bodies (invaginated papillary structures with central vessel) is a characteristic finding of endodermal sinus tumor. The tumors are composed of irregular space lined by flattened to cuboidal cells and recticular stroma[7].[8]

Causes

  • There are no established causes for endodermal sinus tumor.

Differentiating Endodermal sinus tumor from Other Diseases

Endodermal sinus tumor must be differentiated from other diseases that cause pelvic mass, abdominal pain and abdominal distention such as:

Epidemiology and Demographics

  • In 2015, the incidence of endodermal sinus tumor is estimated to less than 1/100,000 case per year in U.S.[11]

Age

Gender

  • Endodermal sinus tumor affects men and women equally.

Race

  • There is no racial predilection for endodermal sinus tumor.

Natural History, Complications and Prognosis

Diagnosis

Diagnostic criteria

  • The diagnosis of endodermal sinus tumor is made based on the imaging and alpha fetoprotein (AFP) tumor marker greater than 100 ng/ml.[17][18]

Symptoms

Physical Examination

  • Patients with endodermal sinus tumor usually appear normal.
  • Physical examination may be remarkable for:[22]

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Stage I endodermal sinus tumor

  • Unilateral salpingo-oophorectomy with adjuvant chemotherapy
  • Unilateral salpingo-oophorectomy followed by observation

Stage II endodermal sinus tumor

Stage III endodermal sinus tumor

  • Total abdominal hysterectomy and bilateral salpingo-oophorectomy with adjuvant chemotherapy, with or without neoadjuvant chemotherapy
  • Unilateral salpingo-oophorectomy with adjuvant chemotherapy, with or without neoadjuvant chemotherapy
  • Second-look laparotomy

Stage IV endodermal sinus tumor

  • Total abdominal hysterectomy and bilateral salpingo-oophorectomy with adjuvant chemotherapy with or without neoadjuvant chemotherapy
  • Unilateral salpingo-oophorectomy with adjuvant chemotherapy with or without neoadjuvant chemotherapy

Prevention

  • There are no primary preventive measures available for endodermal sinus tumor.

Video

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References

  1. ↑ Carmen, Marcela (2015). Uncommon gynecologic cancers. Chichester, England: Wiley Blackwell. ISBN 978-1-118-65535-1.
  2. ↑ 2.0 2.1 Shah JP, Kumar S, Bryant CS, Ali-Fehmi R, Malone JM, Deppe G; et al. (2008). "A population-based analysis of 788 cases of yolk sac tumors: A comparison of males and females". Int J Cancer. 123 (11): 2671–5. doi:10.1002/ijc.23792. PMID 18767035.
  3. ↑ Weidner, Noel (2009). Modern surgical pathology. Philadelphia, PA: Saunders/Elsevier. ISBN 978-1-4160-3966-2.
  4. ↑ 4.0 4.1 Kato N, Tamura G, Fukase M, Shibuya H, Motoyama T (2003). "Hypermethylation of the RUNX3 gene promoter in testicular yolk sac tumor of infants". Am J Pathol. 163 (2): 387–91. doi:10.1016/S0002-9440(10)63668-1. PMC 1868235. PMID 12875960.
  5. ↑ Crum, Christopher (2018). Diagnostic gynecologic and obstetric pathology. Philadelphia, PA: Elsevier, Inc. ISBN 978-0-323-44732-4.
  6. ↑ Carmen, Marcela (2015). Uncommon gynecologic cancers. Chichester, England: Wiley Blackwell. ISBN 978-1-118-65535-1.
  7. ↑ Carmen, Marcela (2015). Uncommon gynecologic cancers. Chichester, England: Wiley Blackwell. ISBN 978-1-118-65535-1.
  8. ↑ Kurman RJ, Norris HJ (1976). "Endodermal sinus tumor of the ovary: a clinical and pathologic analysis of 71 cases". Cancer. 38 (6): 2404–19. doi:10.1002/1097-0142(197612)38:6<2404::aid-cncr2820380629>3.0.co;2-3. PMID 63318.
  9. ↑ Shaaban AM, Rezvani M, Elsayes KM, et al. Ovarian malignant germ cell tumors: cellular classification and clinical and imaging features. Radiographics. 2014;34(3):777-801.http://pubs.rsna.org/doi/pdf/10.1148/rg.343130067
  10. ↑ Jung SE, Lee JM, Rha SE, Byun JY, Jung JI, Hahn ST. CT and MR imaging of ovarian tumors with emphasis on differential diagnosis. Radiographics. 2002;22(6):1305-25.http://www.ncbi.nlm.nih.gov/pubmed/12432104
  11. ↑ Young JL, Miller RW (1975). "Incidence of malignant tumors in U. S. children". J Pediatr. 86 (2): 254–8. doi:10.1016/s0022-3476(75)80484-7. PMID 1111694.
  12. ↑ Coppes MJ, Rackley R, Kay R (1994). "Primary testicular and paratesticular tumors of childhood". Med Pediatr Oncol. 22 (5): 329–40. PMID 8127257.
  13. ↑ 13.0 13.1 Jung SE, Lee JM, Rha SE, Byun JY, Jung JI, Hahn ST (2002). "CT and MR imaging of ovarian tumors with emphasis on differential diagnosis". Radiographics. 22 (6): 1305–25. doi:10.1148/rg.226025033. PMID 12432104.
  14. ↑ 14.0 14.1 14.2 Hung JH, Shen SH, Hung J, Lai CR (2007). "Ultrasound and magnetic resonance images of endodermal sinus tumor". J Chin Med Assoc. 70 (11): 514–8. doi:10.1016/S1726-4901(08)70052-2. PMID 18063508.
  15. ↑ 15.0 15.1 15.2 "Definition of endodermal sinus tumor - NCI Dictionary of Cancer Terms - National Cancer Institute".
  16. ↑ Saia, Philip (2018). Clinical gynecologic oncology. Philadelphia, PA: Elsevier. ISBN 978-0-323-40067-1.
  17. ↑ 17.0 17.1 17.2 Talerman A, Haije WG, Baggerman L (1980). "Serum alphafetoprotein (AFP) in patients with germ cell tumors of the gonads and extragonadal sites: correlation between endodermal sinus (yolk sac) tumor and raised serum AFP". Cancer. 46 (2): 380–5. doi:10.1002/1097-0142(19800715)46:2<380::aid-cncr2820460228>3.0.co;2-u. PMID 6155988.
  18. ↑ Saia, Philip (2018). Clinical gynecologic oncology. Philadelphia, PA: Elsevier. ISBN 978-0-323-40067-1.
  19. ↑ Weidner, Noel (2009). Modern surgical pathology. Philadelphia, PA: Saunders/Elsevier. ISBN 978-1-4160-3966-2.
  20. ↑ Hoffman, Barbara (2012). Williams gynecology. New York: McGraw-Hill Medical. ISBN 9780071716727.
  21. ↑ Gershenson DM, Del Junco G, Herson J, Rutledge FN (1983). "Endodermal sinus tumor of the ovary: the M. D. Anderson experience". Obstet Gynecol. 61 (2): 194–202. PMID 6185892.
  22. ↑ 22.0 22.1 Hoffman, Barbara (2012). Williams gynecology. New York: McGraw-Hill Medical. ISBN 9780071716727.
  23. ↑ Crum, Christopher (2018). Diagnostic gynecologic and obstetric pathology. Philadelphia, PA: Elsevier, Inc. ISBN 978-0-323-44732-4.
  24. ↑ Siltanen S, Anttonen M, Heikkilä P, Narita N, Laitinen M, Ritvos O; et al. (1999). "Transcription factor GATA-4 is expressed in pediatric yolk sac tumors". Am J Pathol. 155 (6): 1823–9. doi:10.1016/S0002-9440(10)65500-9. PMC 1866939. PMID 10595911.
  25. ↑ "NCCN Clinical Practice Guidelines in Oncology: Endodermal sinus tumor. National comprehensive cancer network, 2017; https://www.nccn.org/patients/guidelines/content/PDF/ovarian.pdf."
  26. ↑ Williams S, Blessing JA, Liao SY, Ball H, Hanjani P (1994). "Adjuvant therapy of ovarian germ cell tumors with cisplatin, etoposide, and bleomycin: a trial of the Gynecologic Oncology Group". J Clin Oncol. 12 (4): 701–6. doi:10.1200/JCO.1994.12.4.701. PMID 7512129.
  27. ↑ "NCCN Clinical Practice Guidelines in Oncology: Endodermal sinus tumor. National comprehensive cancer network, 2017; https://www.nccn.org/patients/guidelines/content/PDF/ovarian.pdf."
  28. ↑ Stage I Ovarian Germ Cell Tumors . http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_33. URL Accessed on Nov 5, 2015
  29. ↑ Stage III Ovarian Germ Cell Tumors . http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_54. URL Accessed on Nov 5, 2015
  30. ↑ Stage IV Ovarian Germ Cell Tumors . http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_65. URL Accessed on Nov 5, 2015
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