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  • Imaging Case Report
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Congenital infantile fibrosarcoma

Journal of Perinatology volume 34, pages 329–330 (2014)Cite this article

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Soft tissue tumors of the neonate and infant are uncommon. Most of these are benign, and the most common are congenital hemangiomas and fibrous hamartoma of infancy.1 Malignant lesions of the soft tissues are rhabdomyosarcoma and infantile fibrosarcoma (IFS).1 Congenital hemangiomas are often violaceous massesthat do not stain for endothelial immunoreactivity for the erythrocyte-type glucose transporter protein GLUT-1.2 Fibrous hamartoma of infancy is a mass found overwhelmingly in males in the subcutis or dermis. The lesion is composed of primitive mesenchyme, mature adipose tissue and dense fibrous tissue with overlying hypertrichiosis and eccrine gland hyperplasia.3 Embryonal rhabdomyosarcoma is the most common variant of congenital rhabdomyosarcoma.4 It is composed of spindle-shaped closely packed cells with small round hyperchromatic cells with interspersed rhabdomyoblasts, arranged in fascicles.

IFS is a rare tumor, comprising <1% of cancers in children. In 30% of cases IFS presents in the neonate and is termed congenital IFS.5 IFS is distinct from fibrosarcoma in adults, in that metastases are rare, local recurrence is common and the prognosis is good.6 This low-grade malignant lesion is slightly more common in boys than in girls and has a 90% survival at 5 years. However, lesions that occur in the axial skeleton have a somewhat worse prognosis. In the extremities, 8% of cases of IFS metastasize and there is a 5% overall mortality; in the axial skeleton, there is an incidence of metastases of 26% and an overall mortality of 26%.7 IFS expresses tyrosine kinase receptor 3, NTRK3, and the fusion protein product, ETV6-NTRK3, associated with a chromosome 12 to 15 translocation.7 This chromosomal translocation and fusion protein are characteristic of IFS, but also occur in congenital mesoblastic nephroma and in some leukemias and breast cancers.1

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Authors and Affiliations

  1. Mallinckrodt Institute of Radiology, Washington University School of Medicine, St Louis, MO, USA

    S G Farmakis, T E Herman & M J Siegel

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  1. S G Farmakis
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  2. T E Herman
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  3. M J Siegel
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Correspondence to T E Herman.

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Farmakis, S., Herman, T. & Siegel, M. Congenital infantile fibrosarcoma. J Perinatol 34, 329–330 (2014). https://doi.org/10.1038/jp.2013.164

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