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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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A 14-year-old girl presented with pain and limited range of motion of the right hip joint due to chondroblastoma of the femoral head. (a) Plain film of the pelvis demonstrated an expansile, lobulated, lytic lesion (arrows) in proximal epiphysis of the right femur with matrix punctate calcification. Note is made of a prior biopsy needle tract (arrowheads). (b) On MRI, the lesion presented a heterogeneous high signal on T2W axial images (I) with small, widely scattered foci of higher signal intensity. In addition, extensive inflammatory reaction in the proximal part of the femur and adjacent soft-tissues is noted on coronal T2W images with fat suppression (arrows) (II). (c) Photomicrograph demonstrating numerous ovoid chondroblasts with bland nuclei within a hemorrhagic background admixed with numerous osteoclast-like giant cells indicating chondroblastoma (H&E, original magnification ×100).
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Figure 5: A 14-year-old girl presented with pain and limited range of motion of the right hip joint due to chondroblastoma of the femoral head. (a) Plain film of the pelvis demonstrated an expansile, lobulated, lytic lesion (arrows) in proximal epiphysis of the right femur with matrix punctate calcification. Note is made of a prior biopsy needle tract (arrowheads). (b) On MRI, the lesion presented a heterogeneous high signal on T2W axial images (I) with small, widely scattered foci of higher signal intensity. In addition, extensive inflammatory reaction in the proximal part of the femur and adjacent soft-tissues is noted on coronal T2W images with fat suppression (arrows) (II). (c) Photomicrograph demonstrating numerous ovoid chondroblasts with bland nuclei within a hemorrhagic background admixed with numerous osteoclast-like giant cells indicating chondroblastoma (H&E, original magnification ×100).

Mentions: On plain radiographs, chondroblastoma appears as an expansile, lobulated, lytic lesion in the epiphysis (Fig. 5a)[20]; it often shows matrix punctate calcification which is better demonstrated on CT[13,21]. In aggressive cases, destruction of the cortex may be seen but this is generally unusual[16]. On MRI, chondroblastoma shows low or intermediate signal intensity on T1W images. T2W images demonstrate internal heterogeneity with often small, widely scattered foci of higher signal intensity on gradient-recalled imaging, which correspond to foci of hyaline cartilage or aneurysmal bone cyst (Fig. 5b)[13,16]. Prominent soft tissue and regional bone marrow edema and periosteal reaction may coexist (Fig. 5bII)[13]. Post gadolinium administration, there is mild enhancement of the tumor and more marked enhancement of the adjacent inflammatory regions in the bone marrow, soft tissues and periosteum[13].Figure 5


Imaging of the unusual pediatric 'blastomas'.

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

A 14-year-old girl presented with pain and limited range of motion of the right hip joint due to chondroblastoma of the femoral head. (a) Plain film of the pelvis demonstrated an expansile, lobulated, lytic lesion (arrows) in proximal epiphysis of the right femur with matrix punctate calcification. Note is made of a prior biopsy needle tract (arrowheads). (b) On MRI, the lesion presented a heterogeneous high signal on T2W axial images (I) with small, widely scattered foci of higher signal intensity. In addition, extensive inflammatory reaction in the proximal part of the femur and adjacent soft-tissues is noted on coronal T2W images with fat suppression (arrows) (II). (c) Photomicrograph demonstrating numerous ovoid chondroblasts with bland nuclei within a hemorrhagic background admixed with numerous osteoclast-like giant cells indicating chondroblastoma (H&E, original magnification ×100).
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Related In: Results  -  Collection

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Figure 5: A 14-year-old girl presented with pain and limited range of motion of the right hip joint due to chondroblastoma of the femoral head. (a) Plain film of the pelvis demonstrated an expansile, lobulated, lytic lesion (arrows) in proximal epiphysis of the right femur with matrix punctate calcification. Note is made of a prior biopsy needle tract (arrowheads). (b) On MRI, the lesion presented a heterogeneous high signal on T2W axial images (I) with small, widely scattered foci of higher signal intensity. In addition, extensive inflammatory reaction in the proximal part of the femur and adjacent soft-tissues is noted on coronal T2W images with fat suppression (arrows) (II). (c) Photomicrograph demonstrating numerous ovoid chondroblasts with bland nuclei within a hemorrhagic background admixed with numerous osteoclast-like giant cells indicating chondroblastoma (H&E, original magnification ×100).
Mentions: On plain radiographs, chondroblastoma appears as an expansile, lobulated, lytic lesion in the epiphysis (Fig. 5a)[20]; it often shows matrix punctate calcification which is better demonstrated on CT[13,21]. In aggressive cases, destruction of the cortex may be seen but this is generally unusual[16]. On MRI, chondroblastoma shows low or intermediate signal intensity on T1W images. T2W images demonstrate internal heterogeneity with often small, widely scattered foci of higher signal intensity on gradient-recalled imaging, which correspond to foci of hyaline cartilage or aneurysmal bone cyst (Fig. 5b)[13,16]. Prominent soft tissue and regional bone marrow edema and periosteal reaction may coexist (Fig. 5bII)[13]. Post gadolinium administration, there is mild enhancement of the tumor and more marked enhancement of the adjacent inflammatory regions in the bone marrow, soft tissues and periosteum[13].Figure 5

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