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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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CT (a,b) and MRI (c–e) images through the brain in a 2.5-year-old girl with pineoblastoma. An infiltrative mass in the pineal region is demonstrated (black arrows), with hyperdense indistinct borders on unenhanced CT (a), suggestive of increased cellularity. Post injection of contrast (b), there is peripheral enhancement of the mass evident on CT (b) and MRI (T1W axial (c) and sagittal (d) post gadolinium images). On T2W images, there are areas of low signal intensity suggestive of hypercellularity (arrowheads) (e).
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Figure 12: CT (a,b) and MRI (c–e) images through the brain in a 2.5-year-old girl with pineoblastoma. An infiltrative mass in the pineal region is demonstrated (black arrows), with hyperdense indistinct borders on unenhanced CT (a), suggestive of increased cellularity. Post injection of contrast (b), there is peripheral enhancement of the mass evident on CT (b) and MRI (T1W axial (c) and sagittal (d) post gadolinium images). On T2W images, there are areas of low signal intensity suggestive of hypercellularity (arrowheads) (e).

Mentions: On unenhanced CT, pineoblastoma shows areas of increased cellularity which are demonstrated as hyperdense regions, and features of aggressive infiltration (Fig. 12a). MRI depicts a lobulated, invasive, solid lesion which enhances avidly with contrast. Signal intensity varies but pineoblastomas are essentially isointense to grey matter on T2W images, possibly related to the known paucity of cytoplasm and their dense cellularity (Fig. 12)[49]. Reports in adults with pineoblastoma outline the restricted water diffusion the lesions show due to their high cellularity in diffusion-weighted MRI images[50]. Pineoblastomas are given grade IV WHO classification, implying the highest degree of malignancy, usually show rapid recurrence and cerebrospinal dissemination, and are associated with a poor prognosis[2,51].Figure 12


Imaging of the unusual pediatric 'blastomas'.

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

CT (a,b) and MRI (c–e) images through the brain in a 2.5-year-old girl with pineoblastoma. An infiltrative mass in the pineal region is demonstrated (black arrows), with hyperdense indistinct borders on unenhanced CT (a), suggestive of increased cellularity. Post injection of contrast (b), there is peripheral enhancement of the mass evident on CT (b) and MRI (T1W axial (c) and sagittal (d) post gadolinium images). On T2W images, there are areas of low signal intensity suggestive of hypercellularity (arrowheads) (e).
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Related In: Results  -  Collection

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

Figure 12: CT (a,b) and MRI (c–e) images through the brain in a 2.5-year-old girl with pineoblastoma. An infiltrative mass in the pineal region is demonstrated (black arrows), with hyperdense indistinct borders on unenhanced CT (a), suggestive of increased cellularity. Post injection of contrast (b), there is peripheral enhancement of the mass evident on CT (b) and MRI (T1W axial (c) and sagittal (d) post gadolinium images). On T2W images, there are areas of low signal intensity suggestive of hypercellularity (arrowheads) (e).
Mentions: On unenhanced CT, pineoblastoma shows areas of increased cellularity which are demonstrated as hyperdense regions, and features of aggressive infiltration (Fig. 12a). MRI depicts a lobulated, invasive, solid lesion which enhances avidly with contrast. Signal intensity varies but pineoblastomas are essentially isointense to grey matter on T2W images, possibly related to the known paucity of cytoplasm and their dense cellularity (Fig. 12)[49]. Reports in adults with pineoblastoma outline the restricted water diffusion the lesions show due to their high cellularity in diffusion-weighted MRI images[50]. Pineoblastomas are given grade IV WHO classification, implying the highest degree of malignancy, usually show rapid recurrence and cerebrospinal dissemination, and are associated with a poor prognosis[2,51].Figure 12

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