Endodermal sinus tumor
(Redirected from Endodermal cancer)
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
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
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]
- Stein-Leventhal syndrome
- Tubal pregnancy
- Tubo-ovarian abscess
- Seminoma
- For more information on endodermal sinus tumor differential diagnosis, click here.
- Also, you can find more information by clicking here.
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
- 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.[13]
Gender
Race
- There is no racial predilection for endodermal sinus tumor.
Natural History, Complications and Prognosis
- Endodermal sinus tumor has a poor prognosis in adult.[15][16]
- Endodermal sinus tumor has a favorable prognosis in children.[4]
- Endodermal sinus tumor is the most common malignant germ cell tumor in children.[15][17]
- If left untreated, endodermal sinus tumor quickly metastasizes in other parts of the body such as the brain.[17]
- Endodermal sinus tumor can be found in the ovaries or testicles including the chest, abdomen, and the brain.[17]
- Ovarian endodermal sinus tumor metastasizes quiclky into the intraabdominal area compared to testicular endodermal sinus tumor.[18]
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.[19][20]
Symptoms
- Endodermal sinus tumor is usually asymptomatic.[21]
- Most children and young adults with endodermal sinus tumor have stage I.[22]
- Symptoms of endodermal sinus tumor may include the following:[23] [24]
- Abdominal distention
- Acute/subacute abdominal pain
Physical Examination
- Patients with endodermal sinus tumor usually appear normal.
- Physical examination may be remarkable for:[25]
Laboratory Findings
- An elevated concentration of serum alpha fetoprotein is a diagnostic of endodermal sinus tumor.[19]
- SALL4 (sal-like protein 4) is positive in endodermal sinus tumor.[26]
- AFP is very important for diagnosis, disease monitoring and early metastasis.[19]
Imaging Findings
- On MRI, endodermal sinus tumor is characterized by areas of haemorrhage and necrosis.[16]
- Ultrasound shows solid and cystic mass.[16]
Other Diagnostic Studies
- Endodermal sinus tumor may also be diagnosed using biopsy and measurement of GATA-4, a transcription factor.[27]
Treatment
- Endodermal sinus tumor is treated with surgery depending on the tumor marker and imaging as well as fertility desired.[28]
- 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
- The medical therapy for endodermal sinus tumor is chemotherapy such as bleomycin, etoposide, and cisplatin for 3 to 4 cycles for 21 days depending on the stage.[29][30].
Surgery
- Surgery is the mode of treatment for endodermal sinus tumor with adjuvant chemotherapy:[31][25][32][33]
Stage I endodermal sinus tumor
- Unilateral salpingo-oophorectomy with adjuvant chemotherapy[34]
- Unilateral salpingo-oophorectomy followed by observation
Stage II endodermal sinus tumor
- Unilateral salpingo-oophorectomy with adjuvant chemotherapy
- Second-look laparotomy
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
- ↑ Carmen, Marcela (2015). Uncommon gynecologic cancers. Chichester, England: Wiley Blackwell. ISBN 978-1-118-65535-1.
- ↑ 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.
- ↑ Weidner, Noel (2009). Modern surgical pathology. Philadelphia, PA: Saunders/Elsevier. ISBN 978-1-4160-3966-2.
- ↑ 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.
- ↑ Crum, Christopher (2018). Diagnostic gynecologic and obstetric pathology. Philadelphia, PA: Elsevier, Inc. ISBN 978-0-323-44732-4.
- ↑ Carmen, Marcela (2015). Uncommon gynecologic cancers. Chichester, England: Wiley Blackwell. ISBN 978-1-118-65535-1.
- ↑ Carmen, Marcela (2015). Uncommon gynecologic cancers. Chichester, England: Wiley Blackwell. ISBN 978-1-118-65535-1.
- ↑ 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.
- ↑ Makris, Georgios-Marios (2017). "Giving Birth After Fertility Sparing Treatment for a Yolk Sac Tumour: Case Report". JOURNAL OF CLINICAL AND DIAGNOSTIC RESEARCH. doi:10.7860/JCDR/2017/25820.10524. ISSN 2249-782X.
- ↑ 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
- ↑ 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
- ↑ 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.
- ↑ Coppes MJ, Rackley R, Kay R (1994). "Primary testicular and paratesticular tumors of childhood". Med Pediatr Oncol. 22 (5): 329–40. PMID 8127257.
- ↑ "Endodermal Sinus Tumor - St. Jude Children's Research Hospital".
- ↑ 15.0 15.1 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.
- ↑ 16.0 16.1 16.2 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.
- ↑ 17.0 17.1 17.2 "Definition of endodermal sinus tumor - NCI Dictionary of Cancer Terms - National Cancer Institute".
- ↑ Saia, Philip (2018). Clinical gynecologic oncology. Philadelphia, PA: Elsevier. ISBN 978-0-323-40067-1.
- ↑ 19.0 19.1 19.2 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.
- ↑ Saia, Philip (2018). Clinical gynecologic oncology. Philadelphia, PA: Elsevier. ISBN 978-0-323-40067-1.
- ↑ Chen, Li Hsun; Yip, Kui-Chuen; Wu, Hsing-Ju; Yong, Su-Boon (2019). "Yolk Sac Tumor in an Eight-Year-Old Girl: A Case Report and Literature Review". Frontiers in Pediatrics. 7. doi:10.3389/fped.2019.00169. ISSN 2296-2360.
- ↑ Weidner, Noel (2009). Modern surgical pathology. Philadelphia, PA: Saunders/Elsevier. ISBN 978-1-4160-3966-2.
- ↑ Hoffman, Barbara (2012). Williams gynecology. New York: McGraw-Hill Medical. ISBN 9780071716727.
- ↑ 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.
- ↑ 25.0 25.1 Hoffman, Barbara (2012). Williams gynecology. New York: McGraw-Hill Medical. ISBN 9780071716727.
- ↑ Crum, Christopher (2018). Diagnostic gynecologic and obstetric pathology. Philadelphia, PA: Elsevier, Inc. ISBN 978-0-323-44732-4.
- ↑ 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.
- ↑ "NCCN Clinical Practice Guidelines in Oncology: Endodermal sinus tumor. National comprehensive cancer network, 2017; https://www.nccn.org/patients/guidelines/content/PDF/ovarian.pdf."
- ↑ 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.
- ↑ "NCCN Clinical Practice Guidelines in Oncology: Endodermal sinus tumor. National comprehensive cancer network, 2017; https://www.nccn.org/patients/guidelines/content/PDF/ovarian.pdf."
- ↑ 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
- ↑ 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
- ↑ 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
- ↑ "Ovarian Germ Cell Tumors Treatment (PDQ®) - PDQ Cancer Information Summaries - NCBI Bookshelf".