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*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>
== Diagnosis ==
== 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.<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>
=== Symptoms ===
=== Symptoms ===
The symptoms of endodermal sinus tumor 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>
The symptoms of endodermal sinus tumor 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>

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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, solid gray-white with a 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 ovarian mass, such as Stein-Leventhal syndrome. Ovarian malignant germ cell tumors: cellular classification and clinical and imaging features. CT and MR imaging of ovarian tumors with emphasis on differential diagnosis. ovary teratoma, tubal pregnancy, ovary adenocarcinoma, and tubo-ovarian abscess. Endodermal sinus tumor have a bimodal distribution more commonly observed among young and adult population. The symptoms of endodermal sinus tumor include the following:abdominal distention, acute/sub acute abdominal pain. The mainstay of therapy for endodermal sinus tumor is chemotherapy.

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 ovarian mass, such as:

Epidemiology and Demographics

  • The prevalence of endodermal sinus tumor is very low since it is a rare disease
  • In 2015, the incidence of endodermal sinus tumor is estimated to less than 1/100,000 case per year in U.S.[11]

Age

  • Endodermal sinus tumor have a bimodal distribution more commonly observed among young and adult population[2]
  • Pure yolk sac tumor (endodermal sinus tumor) affects mostly children of less 2 years old.[12]

Gender

  • Endodermal sinus tumor affects men and women equally.

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.[16]

Symptoms

The symptoms of endodermal sinus tumor include the following:[17] [18]

Physical Examination

Ovarian Germ Cell Tumor Physical Examination

Abdomen
Pelvic exam
  • Adnexal mass

Laboratory Findings

  • An elevated concentration of serum alpha feto-protein is diagnostic of endodermal sinus tumor.[16]
  • AFP is very important for diagnosis, disease monitoring and early metastasis.[16]

Imaging Findings

  • On MRI, endodermal sinus tumor is characterized by areas of haemorrhage and necrosis.[14]
  • Ultrasound shows solid and cystic mass.[14]

Other Diagnostic Studies

Treatment

  • Endodermal sinus tumor is treated with surgery depending on the tumor marker and imaging as well as fertility desired.[21]
  • For positive tumor markers and imaging, fertility desired
  • For positive tumor markers and imaging, fertility not desired
    • Treat with completion staging surgery with possible tumor reductive surgery or chemotherapy
  • For negative imaging and positive or negative tumor markers
    • Treat with chemotherapy

Medical Therapy

Stage I endodermal sinus tumor

Stage II endodermal sinus tumor

  • Unilateral salpingo-oophorectomy with adjuvant chemotherapy

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

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

Surgery

Surgery is the mode of treatment for endodermal sinus tumor when chemotherapy is not effective:[26][19][27][28]

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

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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. 16.0 16.1 16.2 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.
  17. 17.0 17.1 Hoffman, Barbara (2012). Williams gynecology. New York: McGraw-Hill Medical. ISBN 9780071716727.
  18. 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.
  19. 19.0 19.1 Hoffman, Barbara (2012). Williams gynecology. New York: McGraw-Hill Medical. ISBN 9780071716727.
  20. 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.
  21. "NCCN Clinical Practice Guidelines in Oncology: Endodermal sinus tumor. National comprehensive cancer network, 2017; https://www.nccn.org/patients/guidelines/content/PDF/ovarian.pdf."
  22. "NCCN Clinical Practice Guidelines in Oncology: Endodermal sinus tumor. National comprehensive cancer network, 2017; https://www.nccn.org/patients/guidelines/content/PDF/ovarian.pdf."
  23. 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
  24. Stage II Ovarian Germ Cell Tumors . http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_43. URL Accessed on Nov 5, 2015
  25. 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
  26. 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
  27. 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
  28. 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