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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]

Professional guidelines

PubMed

Multimodal Treatment of Children With Sacrococcygeal Yolk Sac Tumor: Retrospective Analysis of Clinicopathology Characteristics and Relapse-free Survival.
Zhu J, Chen H, Chen T, Zhen Z, Wang J, Sun F, Lu S, Huang J, Zhang Y, Sun X
J Pediatr Hematol Oncol 2021 Aug 1;43(6):e848-e853. doi: 10.1097/MPH.0000000000002068. PMID: 33512871Free PMC Article
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

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
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
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

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
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
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
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
Gonadoblastoma and hepatoid and endometrioid-like yolk sac tumor: an update.
Ulbright TM
Int J Gynecol Pathol 2014 Jul;33(4):365-73. doi: 10.1097/PGP.0000000000000134. PMID: 24901396

Therapy

Pregnancy and Delivery After Ovarian Yolk Sac Tumor: A Case Report.
Alrjoub MM, Sindiani A, Alshriedeh O, Amarin Z
Am J Case Rep 2021 Aug 19;22:e932091. doi: 10.12659/AJCR.932091. PMID: 34408122Free PMC Article
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
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

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
Fine needle aspiration of mediastinal germ cell tumors.
Reynolds JP, Liu S
Semin Diagn Pathol 2020 Jul;37(4):174-178. Epub 2020 May 13 doi: 10.1053/j.semdp.2020.04.008. PMID: 32444243
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
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

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
Clinical Applications of Immunohistochemistry in Germ Cell Tumors in Men.
Francis F, Zhou M
Methods Mol Biol 2021;2195:13-29. doi: 10.1007/978-1-0716-0860-9_2. PMID: 32852754
Juvenile Granulosa Cell Tumors of the Ovary.
Ye Y, Lv C, Xu S, Chen Y, Qian R, Wang P, Wang S
Am J Clin Pathol 2020 Oct 13;154(5):635-644. doi: 10.1093/ajcp/aqaa081. PMID: 32561911
FDG PET/CT Evaluation of Pediatric Patients With Yolk Sac Tumor.
Tang W, Liu Z, Fu H, Li C, Wang H
AJR Am J Roentgenol 2019 Sep;213(3):676-682. Epub 2019 May 23 doi: 10.2214/AJR.18.20968. PMID: 31120780

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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