1. [Lipoblastoma and lipoblastomatosis: especially in children].
- Author
-
Besouw MT, Verlinde PF, Uyttebroeck AM, and Renard MM
- Subjects
- Child, Preschool, Cytogenetic Analysis, DNA-Binding Proteins genetics, Diagnosis, Differential, Humans, Infant, Lipoma surgery, Lipomatosis surgery, Chromosomes, Human, Pair 8 genetics, Lipoma diagnosis, Lipoma genetics, Lipomatosis diagnosis, Lipomatosis genetics
- 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.
- Published
- 2011