Gerrit Blignaut Diagnostic Radiology UFS17/08/2012

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Case Presentation

Gerrit BlignautDiagnostic RadiologyUFS17/08/2012

3Y6M girl from Lesotho.

3D history of right hemiparesis, preceded by 3D history of vomiting.

No previous hospital admission or any other illnesses.

Father passed due to TB 3 years ago.

2 Siblings, both healthy

History

Extensive Tinea Capitis

CNS: Right hemiparesis with power 4/5. GCS 15/15

CVS, Resp, Abd, ENT: NAD

Bloods: FBC, U&E, HIV normal

CXR: Normal

Examination

CT

MRI

Differential diagnosis

AstrocytomaPNET

Ependymoma

30% supratentorial tumors in children. 3 Patterns: Cystic with enhancing mural nodule solid with necrotic centre Solid with variable enhancement.

Solid part iso- hypodense on CT and hyperintense on T2

Astrocytoma

Mean age 5 years. Arise from precursor cells of germinal matrix. Poor prognosis if supratentorial

CT: dense ca++, large heterogenous mass. Solid and cystic components. Can be peri/ intraventricular with hydrocephalous. Necrosis and hemorrhage.

MRI: Solid- Low signal T1 and intermediate to low signal T2 compared to gray matter.

Little peritumoral oedema

PNET

From ependymal cells that line ventricular canal. 70% Post fossa Can originate supratentorially 1/3, intraaxially

CT: heterogenous, solid, cystic, Ca++, hemorrhage

MRI; Solid: Isointense T1 and hyperintense on T2 compared to gray matter. More oedema

Ependymoma

Rare glial neoplasms, 0.45-2.8% of primary glial tumors.

Present as a large well demarcated, supratentorial hemispheric mass.

Prefer frontoparietal region.

Children, adolescents and young adults.

Median age 11 years.

Female predominance

Astroblastoma

• NECT Solid and cystic lobular mass, solid portion may be mildly hyperattenuated Occasional punctate Ca++

• CECT: Heterogeneous enhancement: Rim of cystic

Heterogenous solid

MRI : Mixed solid cystic mass. Solid part has heterogeneous bubbly appearance on T2. Relatively hypointense to gray matter on T1 and isointense to gray matter on T2 Little peritumoral edema on T2.Mixed signal after contrast administration.

Imaging

Cell of origin has features of both astrocytoma and ependymoma.Tancytes, variety of ependymal cells.Present in the floor of 4th ventricle.

Perivascular pseudorosettes: Astrocytic cell processesradiate toward a central, often hyalinized blood vessel • Oval to elongated hyperchromatic nuclei; +/- Ca++ • Immunohistochemistry: GFAP +, vimentin+, S-100

+

Pathology

GFAP +

Low grade astroblastomas may have long term survival.

Anaplastic histology is associated with recurrence and progression.

Surgical excision mainstay of treatment with postoperative adjuvant radiation and chemotherapy.

Treatment

1: Astroblastoma: A Radio-histological diagnosis J Pediatrc Neurosci Vol 3 2008.2: Astroblastoma: A Rare Glial Tumor International Journal of Pathology; 2004; 2(2):100-102 3: Astroblastoma: Radiologic-Pathologic Correlation and Distinction from Ependymoma AJNR Am J Neuroradiol 23:243–247, February 20024:

References

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