Endodermal sinus tumor: Difference between revisions

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==Pathophysiology==
==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.<ref name="pmid12875960">{{cite journal| author=Kato N, Tamura G, Fukase M, Shibuya H, Motoyama T| title=Hypermethylation of the RUNX3 gene promoter in testicular yolk sac tumor of infants. | journal=Am J Pathol | year= 2003 | volume= 163 | issue= 2 | pages= 387-91 | pmid=12875960 | doi=10.1016/S0002-9440(10)63668-1 | pmc=1868235 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12875960  }} </ref>
*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.<ref name="pmid12875960">{{cite journal| author=Kato N, Tamura G, Fukase M, Shibuya H, Motoyama T| title=Hypermethylation of the RUNX3 gene promoter in testicular yolk sac tumor of infants. | journal=Am J Pathol | year= 2003 | volume= 163 | issue= 2 | pages= 387-91 | pmid=12875960 | doi=10.1016/S0002-9440(10)63668-1 | pmc=1868235 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12875960  }} </ref>
*Ovarian endodermal sinus tumor may be result of differentiation of primitive [[malignant]] [[germ cell]] components of vitelline structures.<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>
*[[Ovarian]] endodermal sinus tumor may be result of differentiation of primitive [[malignant]] [[germ cell]] components of vitelline structures.<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>
*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.<ref>{{cite book | last = Carmen | first = Marcela | title = Uncommon gynecologic cancers | publisher = Wiley Blackwell | location = Chichester, England | year = 2015 | isbn = 978-1-118-65535-1 }}</ref>
*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.<ref>{{cite book | last = Carmen | first = Marcela | title = Uncommon gynecologic cancers | publisher = Wiley Blackwell | location = Chichester, England | year = 2015 | isbn = 978-1-118-65535-1 }}</ref>
*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<ref>{{cite book | last = Carmen | first = Marcela | title = Uncommon gynecologic cancers | publisher = Wiley Blackwell | location = Chichester, England | year = 2015 | isbn = 978-1-118-65535-1 }}</ref>.<ref name="pmid63318">{{cite journal| author=Kurman RJ, Norris HJ| title=Endodermal sinus tumor of the ovary: a clinical and pathologic analysis of 71 cases. | journal=Cancer | year= 1976 | volume= 38 | issue= 6 | pages= 2404-19 | pmid=63318 | doi=10.1002/1097-0142(197612)38:6<2404::aid-cncr2820380629>3.0.co;2-3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=63318  }} </ref>
*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<ref>{{cite book | last = Carmen | first = Marcela | title = Uncommon gynecologic cancers | publisher = Wiley Blackwell | location = Chichester, England | year = 2015 | isbn = 978-1-118-65535-1 }}</ref>.<ref name="pmid63318">{{cite journal| author=Kurman RJ, Norris HJ| title=Endodermal sinus tumor of the ovary: a clinical and pathologic analysis of 71 cases. | journal=Cancer | year= 1976 | volume= 38 | issue= 6 | pages= 2404-19 | pmid=63318 | doi=10.1002/1097-0142(197612)38:6<2404::aid-cncr2820380629>3.0.co;2-3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=63318  }} </ref>
==Causes==
==Causes==
* There are no established [[causes]] for endodermal sinus tumor.<ref name="Makris2017">{{cite journal|last1=Makris|first1=Georgios-Marios|title=Giving Birth After Fertility Sparing Treatment for a Yolk Sac Tumour: Case Report|journal=JOURNAL OF CLINICAL AND DIAGNOSTIC RESEARCH|year=2017|issn=2249782X|doi=10.7860/JCDR/2017/25820.10524}}</ref>
* There are no established [[causes]] for endodermal sinus tumor.<ref name="Makris2017">{{cite journal|last1=Makris|first1=Georgios-Marios|title=Giving Birth After Fertility Sparing Treatment for a Yolk Sac Tumour: Case Report|journal=JOURNAL OF CLINICAL AND DIAGNOSTIC RESEARCH|year=2017|issn=2249782X|doi=10.7860/JCDR/2017/25820.10524}}</ref>
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* 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.<ref name="urlEndodermal Sinus Tumor - St. Jude Children’s Research Hospital">{{cite web |url=https://www.stjude.org/disease/endodermal-sinus-tumor.html |title=Endodermal Sinus Tumor - St. Jude Children’s Research Hospital |format= |work= |accessdate=}}</ref>
 
===Race===
===Race===
*There is no [[racial]] predilection for endodermal sinus tumor.
*There is no [[racial]] predilection for endodermal sinus tumor.
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*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]].<ref name="ChenYip2019">{{cite journal|last1=Chen|first1=Li Hsun|last2=Yip|first2=Kui-Chuen|last3=Wu|first3=Hsing-Ju|last4=Yong|first4=Su-Boon|title=Yolk Sac Tumor in an Eight-Year-Old Girl: A Case Report and Literature Review|journal=Frontiers in Pediatrics|volume=7|year=2019|issn=2296-2360|doi=10.3389/fped.2019.00169}}</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>
*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]]
:*Acute/sub acute [[abdominal pain]]
:*[[Acute]]/subacute [[abdominal pain]]
=== Physical Examination ===
=== Physical Examination ===
*Patients with endodermal sinus tumor usually appear normal.
*[[Patient|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>


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=== Laboratory Findings ===
=== Laboratory Findings ===
*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>
*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>
*[[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>
*[[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>


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===Stage I endodermal sinus tumor===
===Stage I endodermal sinus tumor===


:* Unilateral [[Salpingo-oophorectomy|salpingo-oophorectom]]<nowiki/>y with [[Adjuvant|adjuvan]]<nowiki/>t [[chemotherapy]]
:* Unilateral [[Salpingo-oophorectomy|salpingo-oophorectom]]<nowiki/>y with [[Adjuvant|adjuvan]]<nowiki/>t [[chemotherapy]]<ref name="urlOvarian Germ Cell Tumors Treatment (PDQ®) - PDQ Cancer Information Summaries - NCBI Bookshelf">{{cite web |url=https://www.ncbi.nlm.nih.gov/books/NBK66014/ |title=Ovarian Germ Cell Tumors Treatment (PDQ®) - PDQ Cancer Information Summaries - NCBI Bookshelf |format= |work= |accessdate=}}</ref>
:* Unilateral [[salpingo-oophorectomy]] followed by [[observation]]
:* Unilateral [[salpingo-oophorectomy]] followed by [[observation]]


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===Stage III endodermal sinus tumor===
===Stage III endodermal sinus tumor===


:* Total abdominal [[hysterectomy]] and bilateral [[salpingo-oophorectomy]] with [[adjuvant]] [[chemotherapy]], with or without neoadjuvant [[chemotherapy]]
:* 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]]
:* Unilateral [[salpingo-oophorectomy]] with [[adjuvant chemotherapy]], with or without [[neoadjuvant chemotherapy]]
:* Second-look [[laparotomy]]
:* Second-look [[laparotomy]]
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===Stage IV endodermal sinus tumor===
===Stage IV endodermal sinus tumor===


:* Total abdominal hysterectomy and [[Salpingo-oophorectomy|bilateral salpingo-oophorectomy]] with [[adjuvant chemotherapy]] with or without [[neoadjuvant chemotherapy]]
:* Total [[abdominal]] [[hysterectomy]] and [[Salpingo-oophorectomy|bilateral salpingo-oophorectomy]] with [[adjuvant chemotherapy]] with or without [[neoadjuvant chemotherapy]]
:* Unilateral [[salpingo-oophorectomy]] with [[adjuvant chemotherapy]] with or without [[neoadjuvant chemotherapy]]
:* Unilateral [[salpingo-oophorectomy]] with [[adjuvant chemotherapy]] with or without [[neoadjuvant chemotherapy]]



Latest revision as of 15:55, 14 November 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.[9]

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:[10][11]

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.[12]

Age

Gender

  • Endodermal sinus tumor affects men and women equally.[14]

Race

  • There is no racial predilection for endodermal sinus tumor.

Natural History, Complications and Prognosis

Diagnosis

Diagnostic criteria

Symptoms

Physical Examination

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Stage I endodermal sinus tumor

Stage II endodermal sinus tumor

Stage III endodermal sinus tumor

Stage IV endodermal sinus tumor

Prevention

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

Video

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References

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