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Congenital fibrosarcoma

MedGen UID:
87246
Concept ID:
C0334459
Neoplastic Process
Synonym: Infantile fibrosarcoma
SNOMED CT: Infantile fibrosarcoma (403996004)
 
Monarch Initiative: MONDO:0004557

Definition

A fibrosarcoma that occurs in infants. It shares identical morphologic features with adult fibrosarcoma but carries the t(12;15)(p13;q25) translocation that results in ETV6-NTRK3 gene fusion. It usually affects the superficial and deep soft tissues of the extremities. The prognosis is generally much more favorable than for adult fibrosarcoma, and it rarely metastasizes. [from NCI]

Professional guidelines

PubMed

AbouZeid AA, Mohammad SA, Aly NH, Elghandour MM, AbdelLatif M, Elsayed SM, El-Naggar O, Attia EAS, Ragab IA
Eur J Pediatr 2021 Aug;180(8):2581-2589. Epub 2021 May 19 doi: 10.1007/s00431-021-04119-7. PMID: 34009469
Waelti SL, Garel L, Soglio DD, Rypens F, Messerli M, Dubois J
Pediatr Radiol 2017 Dec;47(13):1766-1775. Epub 2017 Sep 7 doi: 10.1007/s00247-017-3953-3. PMID: 28884206
Hill DA, O'Sullivan MJ, Zhu X, Vollmer RT, Humphrey PA, Dehner LP, Pfeifer JD
Am J Surg Pathol 2002 Aug;26(8):965-77. doi: 10.1097/00000478-200208000-00001. PMID: 12170083

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

AbouZeid AA, Mohammad SA, Aly NH, Elghandour MM, AbdelLatif M, Elsayed SM, El-Naggar O, Attia EAS, Ragab IA
Eur J Pediatr 2021 Aug;180(8):2581-2589. Epub 2021 May 19 doi: 10.1007/s00431-021-04119-7. PMID: 34009469
Ertürk C, Yarmpapuç R, Altay MA, Büyükbebeci O, Ozsaraç MC
J Pediatr Orthop B 2010 Jul;19(4):348-52. doi: 10.1097/BPB.0b013e32833831fc. PMID: 20442675
Dei Tos AP, Dal Cin P
Virchows Arch 1997 Aug;431(2):83-94. doi: 10.1007/s004280050073. PMID: 9293889
Blocker S, Koenig J, Ternberg J
J Pediatr Surg 1987 Jul;22(7):665-70. doi: 10.1016/s0022-3468(87)80123-9. PMID: 3612464
Balsaver AM, Butler JJ, Martin RG
Cancer 1967 Oct;20(10):1607-16. doi: 10.1002/1097-0142(196710)20:10<1607::aid-cncr2820201007>3.0.co;2-#. PMID: 6058170

Diagnosis

Cagan M, Yildirim S, Turkmen GG, Ozyuncu O, Akcoren Z, Deren O, Gucer S
Fetal Pediatr Pathol 2022 Oct;41(5):843-851. Epub 2021 Oct 8 doi: 10.1080/15513815.2021.1988010. PMID: 34622726
AbouZeid AA, Mohammad SA, Aly NH, Elghandour MM, AbdelLatif M, Elsayed SM, El-Naggar O, Attia EAS, Ragab IA
Eur J Pediatr 2021 Aug;180(8):2581-2589. Epub 2021 May 19 doi: 10.1007/s00431-021-04119-7. PMID: 34009469
Dei Tos AP, Dal Cin P
Virchows Arch 1997 Aug;431(2):83-94. doi: 10.1007/s004280050073. PMID: 9293889
Variend S, Bax NM, van Gorp J
Histopathology 1995 Jan;26(1):57-62. doi: 10.1111/j.1365-2559.1995.tb00621.x. PMID: 7713484
Blocker S, Koenig J, Ternberg J
J Pediatr Surg 1987 Jul;22(7):665-70. doi: 10.1016/s0022-3468(87)80123-9. PMID: 3612464

Therapy

See WSQ, Cheuk DKL, To KF, Ip PP, Chiang AKS, Ha SY, Chan GCF
Pediatr Int 2017 Jun;59(6):733-736. Epub 2017 Apr 24 doi: 10.1111/ped.13252. PMID: 28436622
Chi HT, Ly BT, Kano Y, Tojo A, Watanabe T, Sato Y
Biochem Biophys Res Commun 2012 Dec 7;429(1-2):87-92. Epub 2012 Nov 3 doi: 10.1016/j.bbrc.2012.10.087. PMID: 23131561
Ortega-García JA, Soldin OP, López-Hernández FA, Trasande L, Ferrís-Tortajada J
Pediatrics 2012 Oct;130(4):e1019-25. Epub 2012 Sep 3 doi: 10.1542/peds.2011-1307. PMID: 22945410Free PMC Article
Lohi O, Vornanen M, Kähkönen M, Vettenranta K, Parto K, Arola M
J Pediatr Hematol Oncol 2012 Jul;34(5):e202-5. doi: 10.1097/MPH.0b013e31823320e9. PMID: 22217490
Pousti TJ, Upton J, Loh M, Grier H
Plast Reconstr Surg 1998 Sep;102(4):1158-62. doi: 10.1097/00006534-199809040-00037. PMID: 9734437

Prognosis

AbouZeid AA, Mohammad SA, Aly NH, Elghandour MM, AbdelLatif M, Elsayed SM, El-Naggar O, Attia EAS, Ragab IA
Eur J Pediatr 2021 Aug;180(8):2581-2589. Epub 2021 May 19 doi: 10.1007/s00431-021-04119-7. PMID: 34009469
Marguet F, Bergogne L, Laurent N, Rousseau T, Laquerrière A
Clin Neuropathol 2015 Mar-Apr;34(2):70-5. doi: 10.5414/NP300811. PMID: 25492889
Huang SY, Wang CW, Wang CJ, Chao A, Chao AS
Fetal Diagn Ther 2005 Jul-Aug;20(4):266-71. doi: 10.1159/000085083. PMID: 15980638
Blocker S, Koenig J, Ternberg J
J Pediatr Surg 1987 Jul;22(7):665-70. doi: 10.1016/s0022-3468(87)80123-9. PMID: 3612464
Balsaver AM, Butler JJ, Martin RG
Cancer 1967 Oct;20(10):1607-16. doi: 10.1002/1097-0142(196710)20:10<1607::aid-cncr2820201007>3.0.co;2-#. PMID: 6058170

Clinical prediction guides

Hechtman JF, Benayed R, Hyman DM, Drilon A, Zehir A, Frosina D, Arcila ME, Dogan S, Klimstra DS, Ladanyi M, Jungbluth AA
Am J Surg Pathol 2017 Nov;41(11):1547-1551. doi: 10.1097/PAS.0000000000000911. PMID: 28719467Free PMC Article
Henno S, Loeuillet L, Henry C, D'Hervé D, Azzis O, Ferrer J, Poulain P, Babut JM, Merlio JP, Jouan H, Dubus P
Pathol Res Pract 2003;199(1):35-40. doi: 10.1078/0344-0338-00350. PMID: 12650516
Dubus P, Coindre JM, Groppi A, Jouan H, Ferrer J, Cohen C, Rivel J, Copin MC, Leroy JP, de Muret A, Merlio JP
J Pathol 2001 Jan;193(1):88-94. doi: 10.1002/1096-9896(2000)9999:9999<::AID-PATH724>3.0.CO;2-S. PMID: 11169520
Dei Tos AP, Dal Cin P
Virchows Arch 1997 Aug;431(2):83-94. doi: 10.1007/s004280050073. PMID: 9293889
Argyle JC, Tomlinson GE, Stewart D, Schneider NR
Arch Pathol Lab Med 1992 Sep;116(9):972-5. PMID: 1524466

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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

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