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infantile fibrosarcoma
Case Detail
| Anatomy: Chest |
 Joseph Junewick, MD FACR |
| Diagnostic Category: Neoplasia Malignant |
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| Created: over 3 years ago |
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| Updated: over 2 years ago |
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| Tags:
PEDS
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| Modality/Study Types:
US
CR
CT
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Activities:  PDF  ImageJA |
History
Newborn with rapidly increasing chest wall mass on prenatal imaging.
Case Images
Diagnosis
Infantile Fibrosarcoma of the chest wall..
Findings
CXR – Huge left chest wall mass with intrathoracic and extrathoracic components; no overt fat or calcium density seen. The ribs appear osteopenic and deformed.
US – Cystic mass with thick septations.
CT – Solid and cystic left chest wall mass. The left subclavian artery is enlarged.
Discussion
Infantile fibrosarcoma (IF), also known as congenital myofibroblastic sarcoma, is a rare low-grade malignancy seen in children. About 1/3 of cases are congenital and most occur in pre-school aged children. The limbs are the most common site; the trunk is unusual. IF grows rapidly. Metastatic disease is present in <10% but is more prevalent if IF is diagnosed after 10 years of age. Histologically IF is similar to fibrosarcoma but cytogenetically distinct. It is interesting that IF is most similar to congenital mesoblastic nephroma, both displaying the same chromosome translocation t(12;15)(p13;q25).
Reference
Aggouris K, et al. Infantile fibrosarcoma and infantile fibromatosis diagnosis and treatment: Clinical presentation of three cases. Acta Orthop 2008; 59(3):170-175.
Murphy MD, et al. Musculoskeletal fibromatoses: Radiologic-pathologic correlation. Radiographics (2009); 29(7):2143-2176.