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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]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Congenital fibrosarcoma

Professional guidelines

PubMed

Differential diagnostic features between congenital haemangioma, KHE, and congenital fibrosarcoma: a single-centre experience.
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
Neonatal congenital lung tumors - the importance of mid-second-trimester ultrasound as a diagnostic clue.
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
Practical application of molecular genetic testing as an aid to the surgical pathologic diagnosis of sarcomas: a prospective study.
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

Congenital fibrosarcoma and history of prenatal exposure to petroleum derivatives.
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
Signal transduction pathways in sarcoma as targets for therapeutic intervention.
Tuveson DA, Fletcher JA
Curr Opin Oncol 2001 Jul;13(4):249-55. doi: 10.1097/00001622-200107000-00007. PMID: 11429482
The role of cytogenetics in the classification of soft tissue tumours.
Dei Tos AP, Dal Cin P
Virchows Arch 1997 Aug;431(2):83-94. doi: 10.1007/s004280050073. PMID: 9293889
Congenital fibrosarcoma.
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
Congenital fibrosarcoma.
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

Fetal Mediastinal Fibrosarcoma. Report of Two Cases.
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
Differential diagnostic features between congenital haemangioma, KHE, and congenital fibrosarcoma: a single-centre experience.
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
The role of cytogenetics in the classification of soft tissue tumours.
Dei Tos AP, Dal Cin P
Virchows Arch 1997 Aug;431(2):83-94. doi: 10.1007/s004280050073. PMID: 9293889
Cytogenetic analysis of a congenital fibrosarcoma.
Adam LR, Davison EV, Malcolm AJ, Pearson AD, Craft AW
Cancer Genet Cytogenet 1991 Mar;52(1):37-41. doi: 10.1016/0165-4608(91)90051-u. PMID: 2009510
Congenital fibrosarcoma.
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

Congenital fibrosarcoma and history of prenatal exposure to petroleum derivatives.
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
Use of sarcoma-based chemotherapy in a case of congenital mesoblastic nephroma with liver metastases.
Patel Y, Mitchell CD, Hitchcock RJ
Urology 2003 Jun;61(6):1260. doi: 10.1016/s0090-4295(03)00143-2. PMID: 12809924
Signal transduction pathways in sarcoma as targets for therapeutic intervention.
Tuveson DA, Fletcher JA
Curr Opin Oncol 2001 Jul;13(4):249-55. doi: 10.1097/00001622-200107000-00007. PMID: 11429482
Congenital fibrosarcoma of the upper extremity.
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
Congenital fibrosarcoma. Preoperative chemotherapy and conservative surgery.
Ninane J, Gosseye S, Panteon E, Claus D, Rombouts JJ, Cornu G
Cancer 1986 Oct 1;58(7):1400-6. doi: 10.1002/1097-0142(19861001)58:7<1400::aid-cncr2820580703>3.0.co;2-q. PMID: 3755646

Prognosis

Differential diagnostic features between congenital haemangioma, KHE, and congenital fibrosarcoma: a single-centre experience.
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
Congenital fibrosarcoma and history of prenatal exposure to petroleum derivatives.
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
Congenital fibrosarcoma of the upper extremity.
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
Congenital fibrosarcoma.
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
Congenital fibrosarcoma.
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

Pan-Trk Immunohistochemistry Is an Efficient and Reliable Screen for the Detection of NTRK Fusions.
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
In utero ultrasound detection of a large fetal sarcoma of the back.
Meizner I, Potlog-Nahari C, Mashiach R, Shalev J, Vardimon D, Ben-Sira L
Ultrasound Obstet Gynecol 2001 Nov;18(5):540-2. doi: 10.1046/j.0960-7692.2001.00514.x. PMID: 11844181
A multiplex real-time pcr assay for the detection of gene fusions observed in solid tumors.
Peter M, Gilbert E, Delattre O
Lab Invest 2001 Jun;81(6):905-12. doi: 10.1038/labinvest.3780299. PMID: 11406651
Prenatal sonographic appearance of congenital fibrosarcoma.
Tadmor OP, Ariel I, Rabinowitz R, Ne'eman Z, Stark M, Newman M, Yagel S, Diamant YZ
J Clin Ultrasound 1998 Jun;26(5):276-9. doi: 10.1002/(sici)1097-0096(199806)26:5<276::aid-jcu10>3.0.co;2-g. PMID: 9608374
The role of cytogenetics in the classification of soft tissue tumours.
Dei Tos AP, Dal Cin P
Virchows Arch 1997 Aug;431(2):83-94. doi: 10.1007/s004280050073. PMID: 9293889

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    Curated

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

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    Reviews

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