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Intracranial extramedullary hematopoiesis in beta-thalassemia.

Karki B, Xu YK, Tamrakar K, Wu YK - Korean J Radiol (2012)

Bottom Line: Intracranial EMH is an extremely rare occurrence.It is essential to distinguish EMH from other extradural central nervous system tumors, because treatment and prognosis are totally different.Herein, we report the imaging findings of beta-thalassemia in a 13-year-old boy complaining of weakness of left side of the body and gait disturbance; CT and MRI revealed an extradural mass in the right temporoparietal region.

View Article: PubMed Central - PubMed

Affiliation: Department of Medical Imaging, Southern Medical University, Guangzhou, Guangdong, China.

ABSTRACT
Extramedullary hematopoiesis (EMH) represents tumor-like proliferation of hemopoietic tissue which complicates chronic hemoglobinopathy. Intracranial EMH is an extremely rare occurrence. Magnetic resonance imaging (MRI) offers a precise diagnosis. It is essential to distinguish EMH from other extradural central nervous system tumors, because treatment and prognosis are totally different. Herein, we report the imaging findings of beta-thalassemia in a 13-year-old boy complaining of weakness of left side of the body and gait disturbance; CT and MRI revealed an extradural mass in the right temporoparietal region.

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Related in: MedlinePlus

Intracranial EMH in 13-year-old boy with beta-thalassemia.A. Axial non-contrast CT showing soft tissue mass with density of 45 Hounsfield units in right temporo-parietal region with smooth margin compressing adjacent brain parenchyma. Mass is in close contact with diploic space of skull and displays periosteal reaction. B. T1-weighted MR image showing homogeneously slightly high signal intensity mass compared to gray matter with clear margin in right temporo-parietal region, causing buckling of white matter, ipsilateral ventricle compression, and midline shift. Uniform thickening of diploic space and skull bone is also seen. C-E. Mass showing homogeneously low signal intensity on T2-weighted image (C), fluid attenuation inversion recovery (D) and diffusion weighted imaging (E). F. Contrast enhanced coronal T1-weighted image showing significant dural enhancement and moderate enhancement of mass. G. Pathological examination of postoperative specimen on Hematoxylin and Eosin staining showing round to oval cells with erythroblasts, megakaryocytes and promyelocytes (magnification, × 400).
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Figure 1: Intracranial EMH in 13-year-old boy with beta-thalassemia.A. Axial non-contrast CT showing soft tissue mass with density of 45 Hounsfield units in right temporo-parietal region with smooth margin compressing adjacent brain parenchyma. Mass is in close contact with diploic space of skull and displays periosteal reaction. B. T1-weighted MR image showing homogeneously slightly high signal intensity mass compared to gray matter with clear margin in right temporo-parietal region, causing buckling of white matter, ipsilateral ventricle compression, and midline shift. Uniform thickening of diploic space and skull bone is also seen. C-E. Mass showing homogeneously low signal intensity on T2-weighted image (C), fluid attenuation inversion recovery (D) and diffusion weighted imaging (E). F. Contrast enhanced coronal T1-weighted image showing significant dural enhancement and moderate enhancement of mass. G. Pathological examination of postoperative specimen on Hematoxylin and Eosin staining showing round to oval cells with erythroblasts, megakaryocytes and promyelocytes (magnification, × 400).

Mentions: The boy underwent a non-contrast computed tomography (CT) of the head using a Somatom Plus 4 (Siemens Healthcare, Forchheim, Germany). The CT showed a high-attenuating mass with a density of 45 Hounsfield units and a clear margin in the right temporo-parietal region compressing the lateral ventricle and brain parenchyma causing midline shift. The lesion was in close contact with the diploic space of the skull bones, which were thickened and associated with abnormal bony proliferation (Fig. 1A).


Intracranial extramedullary hematopoiesis in beta-thalassemia.

Karki B, Xu YK, Tamrakar K, Wu YK - Korean J Radiol (2012)

Intracranial EMH in 13-year-old boy with beta-thalassemia.A. Axial non-contrast CT showing soft tissue mass with density of 45 Hounsfield units in right temporo-parietal region with smooth margin compressing adjacent brain parenchyma. Mass is in close contact with diploic space of skull and displays periosteal reaction. B. T1-weighted MR image showing homogeneously slightly high signal intensity mass compared to gray matter with clear margin in right temporo-parietal region, causing buckling of white matter, ipsilateral ventricle compression, and midline shift. Uniform thickening of diploic space and skull bone is also seen. C-E. Mass showing homogeneously low signal intensity on T2-weighted image (C), fluid attenuation inversion recovery (D) and diffusion weighted imaging (E). F. Contrast enhanced coronal T1-weighted image showing significant dural enhancement and moderate enhancement of mass. G. Pathological examination of postoperative specimen on Hematoxylin and Eosin staining showing round to oval cells with erythroblasts, megakaryocytes and promyelocytes (magnification, × 400).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3303909&req=5

Figure 1: Intracranial EMH in 13-year-old boy with beta-thalassemia.A. Axial non-contrast CT showing soft tissue mass with density of 45 Hounsfield units in right temporo-parietal region with smooth margin compressing adjacent brain parenchyma. Mass is in close contact with diploic space of skull and displays periosteal reaction. B. T1-weighted MR image showing homogeneously slightly high signal intensity mass compared to gray matter with clear margin in right temporo-parietal region, causing buckling of white matter, ipsilateral ventricle compression, and midline shift. Uniform thickening of diploic space and skull bone is also seen. C-E. Mass showing homogeneously low signal intensity on T2-weighted image (C), fluid attenuation inversion recovery (D) and diffusion weighted imaging (E). F. Contrast enhanced coronal T1-weighted image showing significant dural enhancement and moderate enhancement of mass. G. Pathological examination of postoperative specimen on Hematoxylin and Eosin staining showing round to oval cells with erythroblasts, megakaryocytes and promyelocytes (magnification, × 400).
Mentions: The boy underwent a non-contrast computed tomography (CT) of the head using a Somatom Plus 4 (Siemens Healthcare, Forchheim, Germany). The CT showed a high-attenuating mass with a density of 45 Hounsfield units and a clear margin in the right temporo-parietal region compressing the lateral ventricle and brain parenchyma causing midline shift. The lesion was in close contact with the diploic space of the skull bones, which were thickened and associated with abnormal bony proliferation (Fig. 1A).

Bottom Line: Intracranial EMH is an extremely rare occurrence.It is essential to distinguish EMH from other extradural central nervous system tumors, because treatment and prognosis are totally different.Herein, we report the imaging findings of beta-thalassemia in a 13-year-old boy complaining of weakness of left side of the body and gait disturbance; CT and MRI revealed an extradural mass in the right temporoparietal region.

View Article: PubMed Central - PubMed

Affiliation: Department of Medical Imaging, Southern Medical University, Guangzhou, Guangdong, China.

ABSTRACT
Extramedullary hematopoiesis (EMH) represents tumor-like proliferation of hemopoietic tissue which complicates chronic hemoglobinopathy. Intracranial EMH is an extremely rare occurrence. Magnetic resonance imaging (MRI) offers a precise diagnosis. It is essential to distinguish EMH from other extradural central nervous system tumors, because treatment and prognosis are totally different. Herein, we report the imaging findings of beta-thalassemia in a 13-year-old boy complaining of weakness of left side of the body and gait disturbance; CT and MRI revealed an extradural mass in the right temporoparietal region.

Show MeSH
Related in: MedlinePlus