title: [Lipoblastoma and lipoblastomatosis: especially in children].
abstract: Lipoblastoma and lipoblastomatosis are rare benign fatty tumours that mainly occur in children under the age of 3 years. Several body sites can be affected. The term 'lipoblastoma' is reserved for an encapsulated neoplasm; 'lipoblastomatosis' for tumours demonstrating infiltrative growth. Most of the clinical symptoms arise from a mass effect of the tumour on surrounding tissues. The tumours should be differentiated from lipoma, myxoid liposarcoma and hibernoma. A clear distinction can be made by cytogenetic analysis, since each of these tumours is known for its own typical genetic abnormalities. In lipoblastoma and lipoblastomatosis, these include a breakpoint in the 8q11-13 region or polysomy of chromosome 8, both leading to the activation of the oncogenic pleomorphic adenoma gene 1 (PLAG1) on 8q12. Treatment consists of surgical resection; there is no need for radiotherapy or chemotherapy. Clinical outcome depends on the completeness of the resection and damage that is done to the surrounding tissues during surgery.
authors.affiliation: Universitair Ziekenhuis Gasthuisberg, afd. Kindergeneeskunde, Kinderhematologie en Kinderoncologie, Leuven, België. martine.besouw@uzleuven.be
authors.forename: Martine T P
authors.initials: MT
authors.lastname: Besouw
authors.forename: Piet F L
authors.initials: PF
authors.lastname: Verlinde
authors.forename: Anne M
authors.initials: AM
authors.lastname: Uyttebroeck
authors.forename: Marleen M
authors.initials: MM
authors.lastname: Renard
mesh_terms: D002675:Child, Preschool; D002898:Chromosomes, Human, Pair 8 / Q000235:genetics*; D020732:Cytogenetic Analysis; D004268:DNA-Binding Proteins / Q000235:genetics; D003937:Diagnosis, Differential; D006801:Humans; D007223:Infant; D008067:Lipoma / Q000175:diagnosis* / Q000235:genetics* / Q000601:surgery; D008068:Lipomatosis / Q000175:diagnosis* / Q000235:genetics* / Q000601:surgery
chemical_list: D004268:DNA-Binding Proteins; C104382:PLAG1 protein, human