Aggressive fibromatosis involving the cranial base in children

Aggressive fibromatoses are proliferations of fibrous tissue that infiltrate surrounding tissues and tend to recur after surgical resection. We report a series of five children with aggressive fibromatosis involving the cranial base.

A search of the database maintained by the Medical Informatics Department at The University of Texas M.D. Anderson Cancer Center identified 78 patients with aggressive fibromatosis of the head and neck. Of these patients, five demonstrated involvement of the cranial base. All five were children. A retrospective review of the charts, diagnostic images, and pathological findings for these five patients was performed.

Surgery, radiotherapy, chemotherapy, and hormonal therapy were the treatment methods used. Four patients underwent gross total resections, but tumor cells were present at the margins in all cases. Two of these four patients experienced recurrences; one patient was cured of disease by a second surgical resection and radiotherapy, and the other underwent a second gross total resection. The fifth patient underwent a subtotal resection. He has experienced four recurrences and continues to live with residual disease. One transformation to a Grade 2 fibrosarcoma was observed.

Fibromatoses of the cranial base are rare and seem to be most common in the pediatric age group. Gross total resection, preferably in the first attempt, should be the goal. Radiotherapy and hormonal therapy are useful adjunctive methods for the treatment of aggressive fibromatosis. Fibromatoses that involve the cranial base are most likely to require a multidisciplinary treatment approach.