[Aggressive fibromatosis in childhood: retrospective analysis of four cases].

OBJECTIVE

Aggressive fibromatosis is a rare illness in children. We analyzed the records of four patients, taken from a total of 505, all of which had different types of tumours, over a period of ten years. In the four cases, three were male and one female. Their ages ranged from 0-7 years old. We also did a review of this pathology.

RESULTS

The illness may present itself a a painless mass found particularly in the pelvic area, knee, buttock and anterior mediastinum, respectively. Two of these cases presented lysis of osseous tissue shown in radiologic assays. The diagnosis was histological in every case. The only treatment was surgical in all of them. Two of the cases required only one intervention to achieve remission and up to now there has been no record of further illness. At the time of writing this paper the patients are alive after a follow-up period of thirty months.

CONCLUSIONS

Up to now, no other alternative forms of treatment have been convincing. Adjuvant radiation and chemotherapy are probably beneficial, but the precise indication for its use is not well defined.