Imaging of the unusual pediatric 'blastomas'

Abstract

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.

A Rare case of Pleuro-Pulmonary Blastoma in A 2-Year-old Boy: the value of multi Modality Imaging in Diagnosis And Management.

“…It may be located either peripherally or centrally and there is no definite lobar predominance. It may rarely present as a spontaneouspneumothorax 1 . In the index patient, the radiographic appearance was that of a huge soft tissue dense mass that occupied almost the entire right hemithorax (fig 1) .Radiographic manifestations of 49 patients with pulmonary blastoma reviewed by Koss et al 9 .…”

“…In the index case, the histologic type was III. Because of the rare occurrence of PPB in childhood, they are usually diagnosed with significant delay, being confused with other neoplasms or pleural empyema 1 . Delay in the diagnosis due to unspecific symptoms similar to respiratory infections contribute to the poor clinical outcome in general 5 as in this patient.…”

“…This has raised the possibility that PPB might originate from the splanchno-pleural or somato-pleural mesoderm. Although the exact etiology of PPB remains unknown, pathogenetic molecular abnormalities have been reported 1 Initial symptoms and signs imitate a respiratory tract infection with or without fever, accompanied by mild to moderate respiratory distress 4 . Imaging plays an important role in the identification, management and follow-up of patients with this rare childhood malignancy 5 .…”

“…They are thought to arise from immature, primitive tissues that present persistent embryonal elements on histology. These tumors affect younger pediatric population and are usually malignant 1 . Pleuropulmonary blastoma (PPB) is a rare entity with incidence between 0.25 to 0.5% of all primary lung malignancies that occur exclusively in children younger than 6 years and have a slight male preponderance (1.5/1) 2 .…”

An unusual presentation of pediatric osteoblastoma in a patient with Klippel-Trenaunay-Weber syndrome: case report

“…3,8 Ninety percent of cases occur during the first 2 decades of life, with a peak incidence between the ages of 10 and 20 years. 1,9,17 There is a male predominance (male to female ratio of 1.43-4.7:1). 1,4,5,9,18 Osteoblastoma most commonly presents in the long bones and spine, with sporadic involvement of the mandible, maxilla, sternum, ribs, calvaria, and small bones of the hand.…”