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Mediastinal Teratoma
Joseph Junewick, MD FACR
over 7 years ago
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ARSt Case Repository

Lipoblastoma

Case Detail

Anatomy: Musculoskeletal
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Benign
Created: over 7 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: MR
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History

9 month old female with rapidly enlarging right axillary mass.


Case Images


Diagnosis

Lipoblastoma

Clinical Notes

Pathology – Encapsulated mass with clear margins. Multiple lobules of adipose tissue showing mature adipocytes centrally with occasional lipoblasts peripherally imbedded in a fibrovascular myxoid matrix.

Findings

MR – Coronal and axial T1, axial fat-suppressed T2 and coronal fat-suppressed post-gadolinium T1 images of the chest show a macrolobulated, encapsulated mass composed largely of fat with enhancement of the margins and septae.

Discussion

Lipoblastoma is a rare benign mesenchymal tumor of embryonal white fat. It manifests in two forms, focal and diffuse. The circumscribed lipoblastoma is more common and tends to be located in the superficial soft tissues. The diffuse type lipoblastomatosis is less common and infiltrates the soft tissues.

Most patients are infants with a male predominance; this tumor is rare in children and adolescents. Lipoblastomas are associated with rearrangements in the PLAG1 gene on chormosome 8q11-13.

Lipoblastomas most commonly present as asymptomatic, painless, progressively growing masses in the superficial soft tissue of the extremities and less commonly the trunk, neck, retroperitoneum, mediastinum, and perineum . Symptoms are directly related to the location and size of the mass.

Reference

Murphy MD, et al. From the Archives of the AFIP: Benign Musculoskeletal Lipomatous Lesions. Radiographics (2004); 24:1433-1466.



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