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Congenital infantile fibrosarcoma: review of imaging features

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Abstract

Background

Fibrosarcoma is a rare tumor in children with limited information on imaging features of these tumors in the literature.

Objective

To retrospectively review the imaging features of histologically proven congenital infantile fibrosarcoma.

Materials and methods

The list of histologically confirmed congenital infantile fibrosarcomas between November 1999 and June 2013 was obtained from the oncology-pathology database. Imaging features and pathology reports of these tumors were reviewed. Patient charts were reviewed and clinical features, management and outcomes were recorded.

Results

During the study period, 13 children (9 girls and 4 boys; age range: 0 day–16 months, median age: 2.5 months) with congenital infantile fibrosarcomas were available for complete radiological review. The translocation (t[12;15]) was present in 11/13 (84.6%) and absent in 2/13. Eight/thirteen (61.5%) tumors were located in extremities (5 in lower and 3 in upper), 3/13 in thoracolumbar paraspinal regions, and one each in abdomen and sternocleidomastoid muscle. Imaging features included iso- to hyperintensity on T1-W, hyperintensity on T2-W as compared to skeletal muscles and heterogeneous enhancement. Six (37.5%) tumors showed hemorrhagic components and 2 (15.4%) showed low intensity foci. None of the patients had evidence of regional or distant metastases at diagnosis. Management included surgical resection only (1/13) and combined surgery and chemotherapy (10/13). Overall survival was 100% with a median follow-up of 49.3 months.

Conclusion

Congenital infantile fibrosarcoma has nonspecific imaging characteristics but should be high on the differential diagnosis in a soft-tissue tumor presenting in infancy, located in an extremity and showing tumoral hemorrhage. Patients have a favorable outcome.

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References

  1. Sheng WQ, Hisaoka M, Okamoto S et al (2001) Congenital-infantile fibrosarcoma: a clinicopathologic study of 10 cases and molecular detection of the ETV6-NTRK3 fusion transcripts using paraffin-embedded tissues. Am J Clin Pathol 115:348–355

    Article  CAS  PubMed  Google Scholar 

  2. Orbach D, Rey A, Cecchetto G et al (2010) Infantile fibrosarcoma: management based on the European experience. J Clin Oncol 28:318–323

    Article  PubMed  Google Scholar 

  3. Kumar V, Abbas AK, Fasto N (2004) Diseases of infancy and childhood. In: Robbins, Coltran (eds) Pathologic basis of disease, 7th edn. Saunders, Philadelphia, p 499

    Google Scholar 

  4. Canale S, Vanel D, Couanet D et al (2009) Infantile fibrosarcoma: magnetic resonance imaging findings in six cases. Eur J Radiol 72:30–37

    Article  PubMed  Google Scholar 

  5. Coffin CM, Jaszcz W, O’Shea PA et al (1994) So-called congenital-infantile fibrosarcoma: does it exist and what is it? Pediatr Pathol 14:133–150

    Article  CAS  PubMed  Google Scholar 

  6. Brisse HJ, Orbach D, Klijanienko J (2010) Soft tissue tumors: imaging strategy. Pediatr Radiol 40:1019–1028

    Article  PubMed  Google Scholar 

  7. Muzumdar D, Michaud J, Ventureyra ECG (2006) Primary giant congenital infantile fibrosarcoma of the scalp. Case report and review of the literature. Childs Nerv Syst 22:300–304

    Article  PubMed  Google Scholar 

  8. Laor T (2004) MR imaging of soft tissue tumors and tumor-like lesions. Pediatr Radiol 34:24–37

    Article  PubMed  Google Scholar 

  9. Cheng SM, Shih SL, Sheu CY et al (1999) Congenital-infantile fibrosarcoma: a case report. Chin J Radiol 24:131–134

    Google Scholar 

  10. Eich GF, Hoeffel JC, Tschappeler H et al (1998) Fibrous tumors in children: imaging features of a heterogenous group of disorders. Pediatr Radiol 28:500–509

    Article  CAS  PubMed  Google Scholar 

  11. Lee MJ, Cairns RA, Munk PL et al (1996) Congenital-infantile fibrosarcoma: magnetic resonance imaging findings. Can Assoc Radiol J 47:121–125

    CAS  PubMed  Google Scholar 

  12. Fink AM, Stringer DA, Cairns RA et al (1995) Pediatric case of the day. Radiographics 15:243–246

    Article  CAS  PubMed  Google Scholar 

  13. Vinnicombe SJ, Hall CM (1994) Infantile fibrosarcoma: radiological and clinical features. Skelet Radiol 23:337–341

    Article  CAS  Google Scholar 

  14. Wong CH, Dietrich RB, Pais MJ et al (1994) Pediatric case of the day. Radiographics 14:1428–1430

    Article  CAS  PubMed  Google Scholar 

  15. Dahlin DC (1982) Case report 189. Infantile fibrosarcoma (congential fibrosarcoma-like fibromatosis). Skelet Radiol 8:77–78

    Article  CAS  Google Scholar 

  16. Cecchetto G, Carli M, Alaggio R et al (2001) Fibrosarcoma in pediatric patients: results of the Italian Cooperative Group Studies (1979–1995). J Surg Oncol 78:225–231

    Article  CAS  PubMed  Google Scholar 

  17. Al-Salem AH (2011) Congential-infantile fibrosarcoma masquerading as sacrococcygeal teratoma. J Pediatr Surg 46:2177–2180

    Article  PubMed  Google Scholar 

  18. Demir HA, Akyüz C, Varan A et al (2010) Right foot congenital infantile fibrosarcoma treated only with chemotherapy. Pediatr Blood Cancer 54:618–620

    PubMed  Google Scholar 

  19. Stein-Wexler R (2009) MR imaging of soft tissue masses in children. Magn Reson Imaging Clin N Am 17:489–507

    Article  PubMed  Google Scholar 

  20. Stein-Wexler R (2011) Pediatric soft tissue sarcomas. Semin Ultrasound CT MR 32:470–488

    Article  PubMed  Google Scholar 

  21. Huang SY, Wang CW, Wang CJ et al (2005) Combined prenatal ultrasound and magnetic resonance imaging in an extensive congenital fibrosarcoma: a case report and review of the literature. Fetal Diagn Ther 20:266–271

    Article  PubMed  Google Scholar 

  22. Durin L, Jeanne-Pasquier C, Bailleul P et al (2006) Prenatal diagnosis of a fibrosarcoma of the thigh: a case report. Fetal Diagn Ther 21:481–484

    Article  PubMed  Google Scholar 

  23. Morón FE, Morriss MC, Jones JJ et al (2004) Lumps and bumps on the head in children: use of CT and MR imaging in solving the clinical diagnostic dilemma. Radiographics 24:1655–1674

    Article  PubMed  Google Scholar 

  24. Patrick LE, O'Shea P, Simoneaux SF et al (1996) Fibromatoses of childhood: the spectrum of radiographic findings. AJR Am J Roentgenol 166:163–169

    Article  CAS  PubMed  Google Scholar 

  25. Fromowitz MB, Hurst LC, Nathan J et al (1987) Infantile (desmoids type) fibromatosis with extensive ossification. Am J Surg Pathol 11:66–75

    Article  CAS  PubMed  Google Scholar 

  26. McCarville MB, Kaste SC, Pappo AS (1999) Soft-tissue malignancies in infancy. AJR Am J Roentgenol 173:973–977

    Article  CAS  PubMed  Google Scholar 

  27. Yan AC, Chamlin SL, Liang MG et al (2006) Congenital infantile fibrosarcoma: a masquerader of ulcerated hemangioma. Pediatr Dermatol 23:330–334

    Article  PubMed  Google Scholar 

  28. Hu Z, Jennings JJ, Arva NC (2013) Infantile fibrosarcoma—a clinical and histologic mimicker of vascular malformations: case report and review of the literature. Pediatr Dev Pathol 16:357–363

    Article  PubMed  Google Scholar 

  29. Jain D, Kohli K (2012) Congenital infantile fibrosarcoma: a clinical mimicker of hemangioma. Cutis 89:61–64

    CAS  PubMed  Google Scholar 

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Correspondence to Govind B. Chavhan.

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Ainsworth, K.E., Chavhan, G.B., Gupta, A.A. et al. Congenital infantile fibrosarcoma: review of imaging features. Pediatr Radiol 44, 1124–1129 (2014). https://doi.org/10.1007/s00247-014-2957-5

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  • DOI: https://doi.org/10.1007/s00247-014-2957-5

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