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Holoprosencephaly
Joseph Junewick, MD FACR
over 7 years ago
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infantile fibrosarcoma

Case Detail

Anatomy: Chest
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Malignant
Created: over 8 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: US CR CT
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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.



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