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Imaging of the unusual pediatric 'blastomas'.

Papaioannou G, Sebire NJ, McHugh K - Cancer Imaging (2009)

Bottom Line: We present examples of the more unusual blastematous pediatric tumors (lipoblastoma, osteoblastoma, chondroblastoma, hemangioblastoma, gonadoblastoma, sialoblastoma, pleuropulmonary blastoma, pancreatoblastoma, pineoblastoma, and medullomyoblastoma) that were recorded in our institution.Imaging is often non-specific but plays an important role in their identification, management and follow-up.Some characteristic imaging features at diagnosis, encountered in cases diagnosed and treated in our institution, are described and reviewed.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Great Ormond Street Hospital, Great Ormond Street, London, WC1N 3JH, UK. gpapaio@hotmail.com

ABSTRACT
'Blastomas' are tumors virtually unique to childhood. Controversy surrounds their nomenclature and there is no globally accepted classification. They are thought to arise from immature, primitive tissues that present persistent embryonal elements on histology, affect a younger pediatric population and are usually malignant. The 'commoner' blastomas (neuroblastoma, nephroblastoma, hepatoblastoma, medulloblastoma) account for approximately 25% of solid tumors in the pediatric age range. We present examples of the more unusual blastematous pediatric tumors (lipoblastoma, osteoblastoma, chondroblastoma, hemangioblastoma, gonadoblastoma, sialoblastoma, pleuropulmonary blastoma, pancreatoblastoma, pineoblastoma, and medullomyoblastoma) that were recorded in our institution. Although these rare types of blastomas individually account for <1% of pediatric malignancies, collectively they may be responsible for up to 5% of pediatric tumors in a given population of young children. Imaging is often non-specific but plays an important role in their identification, management and follow-up. Some characteristic imaging features at diagnosis, encountered in cases diagnosed and treated in our institution, are described and reviewed.

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Related in: MedlinePlus

An 8-year-old girl presented with ‘cystic’ swelling of the left forearm due to lipoblastoma. Ultrasound (extended longitudinal view (a), sagittal view (b)) revealed a slightly lobulated heterogeneous mass of low vascularity (b), in close proximity to muscles. On MRI images, the lobulated lesion did not exhibit any aggressive features, appeared as high signal on T1W (c) and suppressed signal on STIR (d), suggesting the presence of fat. Prominent symmetrically distributed vessels in the periphery of the lesion became evident on axial STIR images (arrows) (d). (e) Low power photomicrograph demonstrating the lobulated nature of the tumor with fatty lobules separated by fibrous septae (H&E, original magnification ×20).
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Figure 2: An 8-year-old girl presented with ‘cystic’ swelling of the left forearm due to lipoblastoma. Ultrasound (extended longitudinal view (a), sagittal view (b)) revealed a slightly lobulated heterogeneous mass of low vascularity (b), in close proximity to muscles. On MRI images, the lobulated lesion did not exhibit any aggressive features, appeared as high signal on T1W (c) and suppressed signal on STIR (d), suggesting the presence of fat. Prominent symmetrically distributed vessels in the periphery of the lesion became evident on axial STIR images (arrows) (d). (e) Low power photomicrograph demonstrating the lobulated nature of the tumor with fatty lobules separated by fibrous septae (H&E, original magnification ×20).

Mentions: Plain radiographs show a non-specific soft-tissue mass with no calcifications and no erosion of the adjacent bones (Fig. 1). On ultrasound (US), the lesion is lobulated, of mixed echogenicity. With large lesions, adjacent structures are usually displaced but not significantly compressed (Figs. 2 and 3). The lipomatous features of the lesion, which strongly depend on the amount of mature lipocytes[5,6], are usually evident on computed tomography (CT) and magnetic resonance imaging (MRI) where a detailed map of its location and extent is provided (Figs. 2 and 3). The increased cellularity of lipoblastomas makes their appearances on MRI confusing, as they show high signal on both T1-weighted (T1W) and T2-weighted (T2W) images but the signal on T1W is lower and often more heterogeneous than that of the ‘mature’ subcutaneous fat (Figs. 1 and 2). This feature may differentiate lipoblastomas from lipomas.


Imaging of the unusual pediatric 'blastomas'.

Papaioannou G, Sebire NJ, McHugh K - Cancer Imaging (2009)

An 8-year-old girl presented with ‘cystic’ swelling of the left forearm due to lipoblastoma. Ultrasound (extended longitudinal view (a), sagittal view (b)) revealed a slightly lobulated heterogeneous mass of low vascularity (b), in close proximity to muscles. On MRI images, the lobulated lesion did not exhibit any aggressive features, appeared as high signal on T1W (c) and suppressed signal on STIR (d), suggesting the presence of fat. Prominent symmetrically distributed vessels in the periphery of the lesion became evident on axial STIR images (arrows) (d). (e) Low power photomicrograph demonstrating the lobulated nature of the tumor with fatty lobules separated by fibrous septae (H&E, original magnification ×20).
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2651735&req=5

Figure 2: An 8-year-old girl presented with ‘cystic’ swelling of the left forearm due to lipoblastoma. Ultrasound (extended longitudinal view (a), sagittal view (b)) revealed a slightly lobulated heterogeneous mass of low vascularity (b), in close proximity to muscles. On MRI images, the lobulated lesion did not exhibit any aggressive features, appeared as high signal on T1W (c) and suppressed signal on STIR (d), suggesting the presence of fat. Prominent symmetrically distributed vessels in the periphery of the lesion became evident on axial STIR images (arrows) (d). (e) Low power photomicrograph demonstrating the lobulated nature of the tumor with fatty lobules separated by fibrous septae (H&E, original magnification ×20).
Mentions: Plain radiographs show a non-specific soft-tissue mass with no calcifications and no erosion of the adjacent bones (Fig. 1). On ultrasound (US), the lesion is lobulated, of mixed echogenicity. With large lesions, adjacent structures are usually displaced but not significantly compressed (Figs. 2 and 3). The lipomatous features of the lesion, which strongly depend on the amount of mature lipocytes[5,6], are usually evident on computed tomography (CT) and magnetic resonance imaging (MRI) where a detailed map of its location and extent is provided (Figs. 2 and 3). The increased cellularity of lipoblastomas makes their appearances on MRI confusing, as they show high signal on both T1-weighted (T1W) and T2-weighted (T2W) images but the signal on T1W is lower and often more heterogeneous than that of the ‘mature’ subcutaneous fat (Figs. 1 and 2). This feature may differentiate lipoblastomas from lipomas.

Bottom Line: We present examples of the more unusual blastematous pediatric tumors (lipoblastoma, osteoblastoma, chondroblastoma, hemangioblastoma, gonadoblastoma, sialoblastoma, pleuropulmonary blastoma, pancreatoblastoma, pineoblastoma, and medullomyoblastoma) that were recorded in our institution.Imaging is often non-specific but plays an important role in their identification, management and follow-up.Some characteristic imaging features at diagnosis, encountered in cases diagnosed and treated in our institution, are described and reviewed.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Great Ormond Street Hospital, Great Ormond Street, London, WC1N 3JH, UK. gpapaio@hotmail.com

ABSTRACT
'Blastomas' are tumors virtually unique to childhood. Controversy surrounds their nomenclature and there is no globally accepted classification. They are thought to arise from immature, primitive tissues that present persistent embryonal elements on histology, affect a younger pediatric population and are usually malignant. The 'commoner' blastomas (neuroblastoma, nephroblastoma, hepatoblastoma, medulloblastoma) account for approximately 25% of solid tumors in the pediatric age range. We present examples of the more unusual blastematous pediatric tumors (lipoblastoma, osteoblastoma, chondroblastoma, hemangioblastoma, gonadoblastoma, sialoblastoma, pleuropulmonary blastoma, pancreatoblastoma, pineoblastoma, and medullomyoblastoma) that were recorded in our institution. Although these rare types of blastomas individually account for <1% of pediatric malignancies, collectively they may be responsible for up to 5% of pediatric tumors in a given population of young children. Imaging is often non-specific but plays an important role in their identification, management and follow-up. Some characteristic imaging features at diagnosis, encountered in cases diagnosed and treated in our institution, are described and reviewed.

Show MeSH
Related in: MedlinePlus