Endodermal sinus tumor

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

For patient information, click here Synonyms and Keywords: Endodermal sinus tumor(EST)

Overview

Endodermal sinus tumor was first discovered by Dr. Gunner Telium, a Danish pathologist, in 1971 following publication of a distinctive variety of germ cell tumor. 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, 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[1][2] 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:[3]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 1971 following publication of a distinctive variety of germ cell tumor.

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, 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.
800px-Mixed germ cell tumour - high mag.jpg
800px-Mixed germ cell tumour - intermed mag.jpg

Causes

  • There are no established causes for endodermal sinus tumor.

Differentiating From Endodermal sinus tumor Other Diseases

Endodermal sinus tumor must be differentiated from other diseases that cause ovarian mass, such as:

  • Stein-Leventhal syndrome[1][2]
  • Ovary adenocarcinoma

Epidemiology and Demographics

Age

  • Endodermal sinus tumor have a bimodal distribution more commonly observed among young and adult population.

Gender

  • Endodermal sinus tumor affects men and women equally.

Natural History, Complications and Prognosis

  • 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:[4]
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:[3]

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.

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

  • Unilateral salpingo-oophorectomy with adjuvant chemotherapy

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:[9][5][10][11]

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. 1.0 1.1 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
  2. 2.0 2.1 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
  3. 3.0 3.1 3.2 Hoffman, Barbara (2012). Williams gynecology. New York: McGraw-Hill Medical. ISBN 9780071716727. 
  4. 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
  5. 5.0 5.1 Hoffman, Barbara (2012). Williams gynecology. New York: McGraw-Hill Medical. ISBN 9780071716727. 
  6. 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
  7. 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
  8. 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
  9. 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
  10. 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
  11. 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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