Endodermal sinus tumor

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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:
  • Reticular
  • Endodermal sinus-like
  • Microcystic
  • Papillary
  • Solid
  • Glandular
  • Alveolar
  • Polyvesicular vitelline
  • Enteric
  • Hepatoid

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.
  • 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.[3]
  • 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[4].[5]

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:

  • Stein-Leventhal syndrome[6][7]
  • Ovary adenocarcinoma

Epidemiology and Demographics

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.[8]

Gender

  • Endodermal sinus tumor affects men and women equally.

Natural History, Complications and Prognosis

  • Endodermal sinus tumor has a poor prognosis.[9]
  • Endodermal sinus tumor is malignant tumor.[9]
  • If left untreated, endodermal sinus tumor may progress to develop death.
  • Prognosis is generally dependant on the response to treatment.
  • Ovarian germ cell tumor (endodermal sinus tumor) is surgically staged using the FIGO cancer staging system:[10]
Stage Finding
I Growth limited to the ovaries
Ia Growth limited to one ovary; no ascites present containing malignant cells. No tumor on the external surface; capsule intact
Ib Growth limited to both ovaries; no ascites present containing malignant cells. No tumor on the external surfaces; capsules intact
Icb Tumor either stage Ia or Ib, but with tumor on surface of one or both ovaries, or with capsule ruptured, or with ascites present containing malignant cells, or with positive peritoneal washings
II Growth involving one or both ovaries with pelvic extension
IIa Extension and/or metastases to the uterus and/or tubes
IIb Extension to other pelvic tissues
IIcb Tumor either stage IIa or IIb, but with tumor on surface of one or both ovaries, or with capsule(s) ruptured, or with ascites present containing malignant cells, or with positive peritoneal washings
III Tumor involving one or both ovaries with histologically confirmed peritoneal implants outside the pelvis and/or positive regional lymph nodes. Superficial liver metastases equals stage III. Tumor is limited to the true pelvis, but with histologically proven malignant extension to small bowel or omentum
IIIa Tumor grossly limited to the true pelvis, with negative nodes, but with histologically confirmed microscopic seeding of abdominal peritoneal surfaces, or histologic proven extension to small bowel or mesentery
IIIb Tumor of one or both ovaries with histologically confirmed implants, peritoneal metastasis of abdominal peritoneal surfaces, none exceeding 2 cm in diameter; nodes are negative
IIIc Peritoneal metastasis beyond the pelvis >2 cm in diameter and/or positive regional lymph nodes
IV Growth involving one or both ovaries with distant metastases. If pleural effusion is present, there must be positive cytology to allot a case to stage IV. Parenchymal liver metastasis equals stage IV

Diagnosis

Symptoms

The symptoms of endodermal sinus tumor include the following:[11] [12]

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.[14]
  • AFP is very important for diagnosis, disease monitoring and early metastasis.[14]

Imaging Findings

  • On MRI, endodermal sinus tumor is characterized by areas of haemorrhage.

Other Diagnostic Studies

  • Endodermal sinus tumor may also be diagnosed using biopsy and measurement of GATA-4, a transcription factor.

Treatment

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:[18][13][19][20]

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

Video

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. Carmen, Marcela (2015). Uncommon gynecologic cancers. Chichester, England: Wiley Blackwell. ISBN 978-1-118-65535-1.
  4. Carmen, Marcela (2015). Uncommon gynecologic cancers. Chichester, England: Wiley Blackwell. ISBN 978-1-118-65535-1.
  5. 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.
  6. 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
  7. 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
  8. Coppes MJ, Rackley R, Kay R (1994). "Primary testicular and paratesticular tumors of childhood". Med Pediatr Oncol. 22 (5): 329–40. PMID 8127257.
  9. 9.0 9.1 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.
  10. Stage Information for Ovarian Germ Cell Tumors. http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_8. URL Accessed on November 5, 2015
  11. 11.0 11.1 Hoffman, Barbara (2012). Williams gynecology. New York: McGraw-Hill Medical. ISBN 9780071716727.
  12. 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.
  13. 13.0 13.1 Hoffman, Barbara (2012). Williams gynecology. New York: McGraw-Hill Medical. ISBN 9780071716727.
  14. 14.0 14.1 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.
  15. 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
  16. 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
  17. 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
  18. 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
  19. 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
  20. 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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