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Undifferentiated embryonal sarcoma of the liver

MedGen UID:
902204
•Concept ID:
C2205345
•
Neoplastic Process
Synonyms: Embryonal Sarcoma; Embryonal Sarcoma (Undifferentiated Sarcoma); Embryonal sarcoma of liver; Embryonal Sarcoma of the Liver; Embryonal sarcoma of the liver; embryonal sarcoma of the liver; Liver Embryonal Sarcoma; UES; Undifferentiated (Embryonal) Sarcoma; Undifferentiated (Embryonal) Sarcoma of the Liver; undifferentiated embryonal sarcoma of the liver; Undifferentiated Sarcoma; Undifferentiated sarcoma of the liver; undifferentiated sarcoma of the liver
SNOMED CT: Embryonal sarcoma of liver (716648006)
 
Monarch Initiative: MONDO:0015795
Orphanet: ORPHA178315

Definition

Embryonal sarcoma of the liver is a rare primary malignant hepatic neoplasm of childhood of mesenchymal origin. It can rarely occur in adults. It is characterized by abdominal mass, right upper quadrant or epigastric pain, nausea, anorexia, intermittent fever or headache. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUndifferentiated embryonal sarcoma of the liver

Professional guidelines

PubMed

A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article
Treatment of undifferentiated embryonal sarcoma of the liver in children--single center experience.
Ismail H, Dembowska-Bagińska B, Broniszczak D, Kaliciński P, Maruszewski P, Kluge P, Swięszkowska E, Kościesza A, Lembas A, Perek D
J Pediatr Surg 2013 Nov;48(11):2202-6. doi: 10.1016/j.jpedsurg.2013.05.020. PMID: 24210186
Management of undifferentiated embryonal sarcoma of the liver in children: a case series and management review.
Geel JA, Loveland JA, Pitcher GJ, Beale P, Kotzen J, Poole JE
S Afr Med J 2013 Jun 27;103(10):728-31. doi: 10.7196/samj.6058. PMID: 24079623

Recent clinical studies

Etiology

Adult versus paediatric undifferentiated embryonal sarcoma of the liver: a SEER database analysis.
Pan L, Yin L, Liu XC, Ying RC, Kong WC
ANZ J Surg 2021 Dec;91(12):2690-2694. Epub 2021 Nov 1 doi: 10.1111/ans.17290. PMID: 34724322
Liver Tumors in Pediatric Patients.
Meyers R, Hiyama E, Czauderna P, Tiao GM
Surg Oncol Clin N Am 2021 Apr;30(2):253-274. doi: 10.1016/j.soc.2020.11.006. PMID: 33706899
Long-term survival outcomes of undifferentiated embryonal sarcoma of the liver: a pooled analysis of 308 patients.
Wu Z, Wei Y, Cai Z, Zhou Y
ANZ J Surg 2020 Sep;90(9):1615-1620. Epub 2020 Jan 20 doi: 10.1111/ans.15684. PMID: 31957153
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article
Characteristics and outcomes in children with undifferentiated embryonal sarcoma of the liver: A report from the National Cancer Database.
Shi Y, Rojas Y, Zhang W, Beierle EA, Doski JJ, Goldfarb M, Goldin AB, Gow KW, Langer M, Meyers RL, Nuchtern JG, Vasudevan SA
Pediatr Blood Cancer 2017 Apr;64(4) Epub 2016 Oct 26 doi: 10.1002/pbc.26272. PMID: 27781381Free PMC Article

Diagnosis

Mesenchymal Neoplasms of the Liver.
Papke DJ Jr
Surg Pathol Clin 2023 Sep;16(3):609-634. Epub 2023 Jun 11 doi: 10.1016/j.path.2023.04.013. PMID: 37536892
Liver Tumors in Pediatric Patients.
Meyers R, Hiyama E, Czauderna P, Tiao GM
Surg Oncol Clin N Am 2021 Apr;30(2):253-274. doi: 10.1016/j.soc.2020.11.006. PMID: 33706899
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article
Undifferentiated Embryonal Sarcoma of the Liver (UESL): A Single-Center Experience and Review of the Literature.
Techavichit P, Masand PM, Himes RW, Abbas R, Goss JA, Vasudevan SA, Finegold MJ, Heczey A
J Pediatr Hematol Oncol 2016 May;38(4):261-8. doi: 10.1097/MPH.0000000000000529. PMID: 26925712
Undifferentiated embryonal sarcoma of the liver: a concise review.
Putra J, Ornvold K
Arch Pathol Lab Med 2015 Feb;139(2):269-73. doi: 10.5858/arpa.2013-0463-RS. PMID: 25611111

Therapy

Outcome of patients with undifferentiated embryonal sarcoma of the liver treated according to European soft tissue sarcoma protocols.
Guérin F, Martelli H, Rogers T, Zanetti I, van Scheltinga ST, De Corti F, Burrieza GG, Minard-Colin V, Orbach D, van Noesel MM, Karanian M, Fajardo RD, Merks JHM, Ferrari A, Bisogno G
Pediatr Blood Cancer 2023 Jul;70(7):e30374. Epub 2023 Apr 21 doi: 10.1002/pbc.30374. PMID: 37083216
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article
Treatment of undifferentiated embryonal sarcoma of the liver in children--single center experience.
Ismail H, Dembowska-Bagińska B, Broniszczak D, Kaliciński P, Maruszewski P, Kluge P, Swięszkowska E, Kościesza A, Lembas A, Perek D
J Pediatr Surg 2013 Nov;48(11):2202-6. doi: 10.1016/j.jpedsurg.2013.05.020. PMID: 24210186
Management of undifferentiated embryonal sarcoma of the liver in children: a case series and management review.
Geel JA, Loveland JA, Pitcher GJ, Beale P, Kotzen J, Poole JE
S Afr Med J 2013 Jun 27;103(10):728-31. doi: 10.7196/samj.6058. PMID: 24079623
Undifferentiated embryonal sarcoma of the liver successfully treated with chemotherapy and liver resection.
Baron PW, Majlessipour F, Bedros AA, Zuppan CW, Ben-Youssef R, Yanni G, Ojogho ON, Concepcion W
J Gastrointest Surg 2007 Jan;11(1):73-5. doi: 10.1007/s11605-006-0044-4. PMID: 17390190

Prognosis

Adult versus paediatric undifferentiated embryonal sarcoma of the liver: a SEER database analysis.
Pan L, Yin L, Liu XC, Ying RC, Kong WC
ANZ J Surg 2021 Dec;91(12):2690-2694. Epub 2021 Nov 1 doi: 10.1111/ans.17290. PMID: 34724322
Long-term survival outcomes of undifferentiated embryonal sarcoma of the liver: a pooled analysis of 308 patients.
Wu Z, Wei Y, Cai Z, Zhou Y
ANZ J Surg 2020 Sep;90(9):1615-1620. Epub 2020 Jan 20 doi: 10.1111/ans.15684. PMID: 31957153
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article
Characteristics and outcomes in children with undifferentiated embryonal sarcoma of the liver: A report from the National Cancer Database.
Shi Y, Rojas Y, Zhang W, Beierle EA, Doski JJ, Goldfarb M, Goldin AB, Gow KW, Langer M, Meyers RL, Nuchtern JG, Vasudevan SA
Pediatr Blood Cancer 2017 Apr;64(4) Epub 2016 Oct 26 doi: 10.1002/pbc.26272. PMID: 27781381Free PMC Article
Undifferentiated embryonal sarcoma of the liver: a concise review.
Putra J, Ornvold K
Arch Pathol Lab Med 2015 Feb;139(2):269-73. doi: 10.5858/arpa.2013-0463-RS. PMID: 25611111

Clinical prediction guides

The genomic landscape of undifferentiated embryonal sarcoma of the liver is typified by C19MC structural rearrangement and overexpression combined with TP53 mutation or loss.
Setty BA, Jinesh GG, Arnold M, Pettersson F, Cheng CH, Cen L, Yoder SJ, Teer JK, Flores ER, Reed DR, Brohl AS
PLoS Genet 2020 Apr;16(4):e1008642. Epub 2020 Apr 20 doi: 10.1371/journal.pgen.1008642. PMID: 32310940Free PMC Article
Long-term survival outcomes of undifferentiated embryonal sarcoma of the liver: a pooled analysis of 308 patients.
Wu Z, Wei Y, Cai Z, Zhou Y
ANZ J Surg 2020 Sep;90(9):1615-1620. Epub 2020 Jan 20 doi: 10.1111/ans.15684. PMID: 31957153
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article
Treatment of undifferentiated embryonal sarcoma of the liver in children--single center experience.
Ismail H, Dembowska-Bagińska B, Broniszczak D, Kaliciński P, Maruszewski P, Kluge P, Swięszkowska E, Kościesza A, Lembas A, Perek D
J Pediatr Surg 2013 Nov;48(11):2202-6. doi: 10.1016/j.jpedsurg.2013.05.020. PMID: 24210186
Undifferentiated embryonal sarcoma of the liver: case report and literature survey.
Pachera S, Nishio H, Takahashi Y, Yokoyama Y, Oda K, Ebata T, Igami T, Nagino M
J Hepatobiliary Pancreat Surg 2008;15(5):536-44. Epub 2008 Oct 4 doi: 10.1007/s00534-007-1265-y. PMID: 18836810

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