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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

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
Imaging features of undifferentiated embryonal sarcoma of the liver: a series of 15 children.
Gabor F, Franchi-Abella S, Merli L, Adamsbaum C, Pariente D
Pediatr Radiol 2016 Nov;46(12):1694-1704. Epub 2016 Aug 26 doi: 10.1007/s00247-016-3670-3. PMID: 27562247
Undifferentiated embryonal sarcoma of liver: a multi-institutional experience with 9 cases.
Cao Q, Ye Z, Chen S, Liu N, Li S, Liu F
Int J Clin Exp Pathol 2014;7(12):8647-56. Epub 2014 Dec 1 PMID: 25674229Free 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

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 sarcoma of the liver: a single institution experience using a uniform treatment approach.
May LT, Wang M, Albano E, Garrington T, Dishop M, Macy ME
J Pediatr Hematol Oncol 2012 Apr;34(3):e114-6. doi: 10.1097/MPH.0b013e3182331fbe. PMID: 22217489Free PMC Article
Undifferentiated embryonal sarcoma of the liver in a child complicated by haemorrhage.
Küpeli S, Yalçin B, Cil BE, Akçören Z, Büyükpamukçu M
Pediatr Radiol 2008 Nov;38(11):1259-61. Epub 2008 Aug 8 doi: 10.1007/s00247-008-0972-0. PMID: 18688607
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
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
Undifferentiated embryonal sarcoma of liver: a multi-institutional experience with 9 cases.
Cao Q, Ye Z, Chen S, Liu N, Li S, Liu F
Int J Clin Exp Pathol 2014;7(12):8647-56. Epub 2014 Dec 1 PMID: 25674229Free PMC Article

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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