Hosting 718 files, 2916 images, and 4 contributors.

 
Random Case

Pulmonary Hemosiderosis
Joseph Junewick, MD FACR
over 4 years ago
Enter A Workflow
Standard2   Academic2

Please choose a workflow. A standard workflow allows you to browse the repository with full case detail; the academic workflow allows you to browse the repository with limited case detail revealed. Double click on the images to launch image viewer.

ARSt Case Repository

Desmoplastic Infantile Astrocytoma

Case Detail

Anatomy: Brain-Spine
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Malignant
Created: over 7 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: US MR
Activities:
Adobe32 PDF Imagej32 ImageJA

History

Newborn with bulging anterior fontanelle.


Case Images


Diagnosis

Desmoplastic Infantile Astrocytoma

Findings

US – Large hyperechoic and hypervascular right cerebral mass.

MR – Axial and coronal T2 and 3 plane post-gadolinium images of the brain demonstrate an enhancing T2 heterogeneous right cerebral mass with occasional areas of cystic degeneration and several flow voids resulting in significant mass effect.

Discussion

Desmoplastic infantile astrocytoma (DIT) tends to be large slow growing supratrentorial mass. The frontal and parietal lobes are most often affected but multillobar involvement is common. The lesions are heterogenous with cysts and calcification usually present. Typically, DIT appears as a hypointense cystic mass with an isointense peripheral solid component on T1-weighted MR images. On T2-weighted MR images, the cystic component is hyperintense and the solid portion isointense or heterogeneous. Edema is usually absent or moderate The peripheral solid component enhances with gadolinium administration. Leptomeningeal enhancement and remodeling of the inner table are common. Desmoplastic infantile astrocytomas are frequently adherent to the dura mater.

DIT is rarely symptomatic but is usually found within the first 18 months of life as a result of increased head circumference, paresis or seizure. The male-female ratio is 1.7:1. This tumor is not exclusively seen in pediatric patients; it may be diagnosed in older patients. DIT represents a formidable surgical challenge. Complete surgical excision usually results in a cure with no further treatment being necessary. In case of incomplete resection, favorable development is noted in most and adjuvant treatment is determined individually. Recurrence and death have been noted in some atypical cases.

The radiologic differential diagnoses of DIT include primitive neuroectodermal tumor, ependymoma, ganglioglioma, and dysembryoplastic neuroepithelial tumor, and choroid plexus carcinoma.

Reference

Guillaume T, Brugea H, Vinchonb M, Khalila C, et al. MR Imaging in the Diagnosis of Desmoplastic Infantile Tumor: Retrospective Study of Six Cases. Am J Neurorad (2004); 25:1028-1033.



Corporate Office: 616.363.7272, 3264 North Evergreen Drive, Grand Rapids, MI 49525

Spectrum Health Helen Devos Childrens Hospital GE HealthCare