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Yolk sac tumor

MedGen UID:
41782
•Concept ID:
C0014145
•
Neoplastic Process
Synonym: Endodermal-sinus tumor
SNOMED CT: Infantile embryonal carcinoma (74409009); Endodermal sinus tumor (74409009); Yolk sac tumor (74409009); Polyvesicular vitelline tumor (74409009); Orchioblastoma (74409009); Embryonal carcinoma, infantile (74409009); Yolk sac tumor (404081005); Hepatoid yolk sac tumor (74409009)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0005744
OMIM®: 273300
Orphanet: ORPHA876

Definition

A rare germ cell tumor characterized by multiple patterns reflecting endodermal extraembryonal differentiation (secondary yolk sac and allantois) or endodermal somatic tissues (intestine, liver, and mesenchyme). The tumors most commonly occur in the second or third decade of life. They are typically located in the gonads, occasionally also in other regions. Patients present with a pelvic mass and/or abdominal pain (females) or an often painless, unilateral testicular mass (males). Elevated serum alpha fetoprotein is a common laboratory finding. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVYolk sac tumor
Follow this link to review classifications for Yolk sac tumor in Orphanet.

Professional guidelines

PubMed

Critical elements in the operative management of pediatric malignant ovarian germ cell tumors.
Weil BR, Rich BS, Madenci AL, Stambough KC, Schmoke N, Peace A, Bruny JL, Rescorla FJ, Dicken BJ, Dietrich JE, Billmire DF
Semin Pediatr Surg 2023 Oct;32(5):151342. Epub 2023 Nov 17 doi: 10.1016/j.sempedsurg.2023.151342. PMID: 38039829
Is fertility sparing surgery a treatment option for young patients with yolk sac tumor?
Iavazzo C, Vorgias G, Iavazzo PE, Minis EE, Gkegkes ID
Minerva Ginecol 2018 Dec;70(6):750-753. doi: 10.23736/S0026-4784.18.04194-1. PMID: 30465413
Current Management for Pediatric Urologic Oncology.
Caldwell BT, Wilcox DT, Cost NG
Adv Pediatr 2017 Aug;64(1):191-223. doi: 10.1016/j.yapd.2017.04.001. PMID: 28688589

Recent clinical studies

Etiology

Ovarian yolk sac tumor in a premenarchal girl.
Miletić AI, Habek D, Medić F, Prka M, Berić Lerotić S, Knežević F
Wien Med Wochenschr 2023 Mar;173(3-4):70-73. Epub 2023 Jan 13 doi: 10.1007/s10354-022-00996-1. PMID: 36637654
Germ Cell Tumors of the Ovary: A Review.
Ramalingam P
Semin Diagn Pathol 2023 Jan;40(1):22-36. Epub 2022 Jul 29 doi: 10.1053/j.semdp.2022.07.004. PMID: 36088223
Testicular Tumors: A Contemporary Update on Morphologic, Immunohistochemical and Molecular Features.
Al-Obaidy KI, Idrees MT
Adv Anat Pathol 2021 Jul 1;28(4):258-275. doi: 10.1097/PAP.0000000000000302. PMID: 33871428
Vaginal Yolk Sac Tumor: A Case Series and Review of the Literature.
Mayhew AC, Rytting H, Olson TA, Smith E, Childress KJ
J Pediatr Adolesc Gynecol 2021 Feb;34(1):54-60.e4. Epub 2020 Jul 3 doi: 10.1016/j.jpag.2020.06.020. PMID: 32628992
Germ Cell Tumors of the Female Genital Tract.
Euscher ED
Surg Pathol Clin 2019 Jun;12(2):621-649. doi: 10.1016/j.path.2019.01.005. PMID: 31097117

Diagnosis

Pure post-pubertal yolk sac tumor of the testis: An extremely rare and aggressive entity.
Patel S, Satturwar S, Khan W, Ashman D, Quiroga-Garza GM
Ann Diagn Pathol 2023 Apr;63:152086. Epub 2022 Dec 23 doi: 10.1016/j.anndiagpath.2022.152086. PMID: 36580878
Germ Cell Tumors of the Ovary: A Review.
Ramalingam P
Semin Diagn Pathol 2023 Jan;40(1):22-36. Epub 2022 Jul 29 doi: 10.1053/j.semdp.2022.07.004. PMID: 36088223
Testicular Tumors: A Contemporary Update on Morphologic, Immunohistochemical and Molecular Features.
Al-Obaidy KI, Idrees MT
Adv Anat Pathol 2021 Jul 1;28(4):258-275. doi: 10.1097/PAP.0000000000000302. PMID: 33871428
Vaginal Yolk Sac Tumor: A Case Series and Review of the Literature.
Mayhew AC, Rytting H, Olson TA, Smith E, Childress KJ
J Pediatr Adolesc Gynecol 2021 Feb;34(1):54-60.e4. Epub 2020 Jul 3 doi: 10.1016/j.jpag.2020.06.020. PMID: 32628992
Germ Cell Tumors of the Female Genital Tract.
Euscher ED
Surg Pathol Clin 2019 Jun;12(2):621-649. doi: 10.1016/j.path.2019.01.005. PMID: 31097117

Therapy

Establishment and characterization of an ovarian yolk sac tumor patient-derived xenograft model.
Luo M, He Y, Xie B, Li S, Gan F, Zhang S, Luo P
Pediatr Surg Int 2021 Aug;37(8):1031-1040. Epub 2021 May 24 doi: 10.1007/s00383-021-04895-1. PMID: 34031745
A yolk sac tumor of the pancreas and derived xenograft model effectively responded to VIP chemotherapy.
Yonemaru J, Takahashi M, Nara S, Ichikawa H, Ishigamori R, Imai T, Hiraoka N
Pancreatology 2020 Apr;20(3):551-557. Epub 2019 Dec 31 doi: 10.1016/j.pan.2019.12.021. PMID: 31917123
Yolk sac tumor of endometrium: A case report and literature review.
Lin SW, Hsieh SW, Huang SH, Liang HS, Huang CY
Taiwan J Obstet Gynecol 2019 Nov;58(6):846-848. doi: 10.1016/j.tjog.2019.09.020. PMID: 31759539
New Anti-Cancer Strategies in Testicular Germ Cell Tumors.
Chieffi P, De Martino M, Esposito F
Recent Pat Anticancer Drug Discov 2019;14(1):53-59. doi: 10.2174/1574892814666190111120023. PMID: 30636617
Primary orbital yolk sac tumor: report of a case and review of literature.
Kamal S, Kaliki S, Sreedhar A, Mishra DK
Int Ophthalmol 2016 Jun;36(3):435-44. Epub 2015 Oct 19 doi: 10.1007/s10792-015-0142-y. PMID: 26481249

Prognosis

Targeting CLDN6 in germ cell tumors by an antibody-drug-conjugate and studying therapy resistance of yolk-sac tumors to identify and screen specific therapeutic options.
Skowron MA, Kotthoff M, Bremmer F, Ruhnke K, Parmaksiz F, Richter A, Küffer S, Reuter-Jessen K, Pauls S, Stefanski A, Ströbel P, Stühler K, Nettersheim D
Mol Med 2023 Mar 29;29(1):40. doi: 10.1186/s10020-023-00636-3. PMID: 36991316Free PMC Article
Ovarian yolk sac tumor in a premenarchal girl.
Miletić AI, Habek D, Medić F, Prka M, Berić Lerotić S, Knežević F
Wien Med Wochenschr 2023 Mar;173(3-4):70-73. Epub 2023 Jan 13 doi: 10.1007/s10354-022-00996-1. PMID: 36637654
Somatic-type malignancies in testicular germ cell tumors.
Guo CC, Czerniak B
Hum Pathol 2022 Sep;127:123-135. Epub 2022 Jul 5 doi: 10.1016/j.humpath.2022.06.024. PMID: 35803413
Unusual Presentations of Gynecologic Tumors: Extragonadal Yolk Sac Tumor of the Vulva.
Euscher ED
Arch Pathol Lab Med 2017 Feb;141(2):293-297. Epub 2016 Dec 13 doi: 10.5858/arpa.2016-0151-SA. PMID: 27959583
Prognosis of hepatoid yolk sac tumor in women: what's up, Doc?
Rittiluechai K, Wilcox R, Lisle J, Everett E, Wallace HJ 3rd, Verschraegen CF
Eur J Obstet Gynecol Reprod Biol 2014 Apr;175:25-9. Epub 2014 Jan 3 doi: 10.1016/j.ejogrb.2013.12.029. PMID: 24462393

Clinical prediction guides

Associations between birth defects and childhood and adolescent germ cell tumors according to sex, histologic subtype, and site.
Schraw JM, Sok P, Desrosiers TA, Janitz AE, Langlois PH, Canfield MA, Frazier AL, Plon SE, Lupo PJ, Poynter JN
Cancer 2023 Oct 15;129(20):3300-3308. Epub 2023 Jun 27 doi: 10.1002/cncr.34906. PMID: 37366624Free PMC Article
Targeting CLDN6 in germ cell tumors by an antibody-drug-conjugate and studying therapy resistance of yolk-sac tumors to identify and screen specific therapeutic options.
Skowron MA, Kotthoff M, Bremmer F, Ruhnke K, Parmaksiz F, Richter A, Küffer S, Reuter-Jessen K, Pauls S, Stefanski A, Ströbel P, Stühler K, Nettersheim D
Mol Med 2023 Mar 29;29(1):40. doi: 10.1186/s10020-023-00636-3. PMID: 36991316Free PMC Article
How and where to expect endosalpingiosis intraoperatively.
Burla L, Kalaitzopoulos DR, Mrozek A, Eberhard M, Samartzis N
Fertil Steril 2022 Feb;117(2):461-462. Epub 2022 Jan 2 doi: 10.1016/j.fertnstert.2021.11.016. PMID: 34986982
Yolk Sac Tumor of the Ovary: A Report of 150 Cases and Review of the Literature.
Young RH, Wong A, Stall JN
Am J Surg Pathol 2022 Mar 1;46(3):309-325. doi: 10.1097/PAS.0000000000001793. PMID: 34469331
Yolk Sac Tumor in Extragonadal Pelvic Sites: Still a Diagnostic Challenge.
Ravishankar S, Malpica A, Ramalingam P, Euscher ED
Am J Surg Pathol 2017 Jan;41(1):1-11. doi: 10.1097/PAS.0000000000000722. PMID: 27631522

Recent systematic reviews

Prognostic factors in patients with clinical stage I nonseminoma-beyond lymphovascular invasion: a systematic review.
Zengerling F, Beyersdorff D, Busch J, Heinzelbecker J, Pfister D, Ruf C, Winter C, Albers P, Kliesch S, Schmidt S
World J Urol 2022 Dec;40(12):2879-2887. Epub 2022 Jul 29 doi: 10.1007/s00345-022-04063-7. PMID: 35906286Free PMC Article
Primary intracranial pure endodermal sinus tumor: A retrospective series of 6 cases in a single center and a systematic review of overall survival.
Raynald, Yang H, Zhang X, Ma'ruf L, Li C, Jiang T
Neurochirurgie 2021 Nov;67(6):587-598. Epub 2021 Mar 19 doi: 10.1016/j.neuchi.2021.02.014. PMID: 33753128
Primary yolk sac tumor of the endometrium: a case report and review of the literatures.
Lu T, Qi L, Ma Y, Lu G, Zhang X, Liu P
Arch Gynecol Obstet 2019 Nov;300(5):1177-1187. Epub 2019 Sep 23 doi: 10.1007/s00404-019-05309-3. PMID: 31549219
Feto-maternal outcomes of pregnancy complicated by ovarian malignant germ cell tumor: a systematic review of literature.
Kodama M, Grubbs BH, Blake EA, Cahoon SS, Murakami R, Kimura T, Matsuo K
Eur J Obstet Gynecol Reprod Biol 2014 Oct;181:145-56. Epub 2014 Aug 7 doi: 10.1016/j.ejogrb.2014.07.047. PMID: 25150953

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