Pediatric recurrent aggressive spinal fibromatosis with progressive kyphosis and neurological deficits

Abstract

Aggressive fibromatosis is a benign, locally invasive fibroblastic proliferation that can cause compressive effects on adjacent structures. The primary cure of this disease rests in wide excision of the tumor. Unfortunately even when surgical margins are clear of tumor, recurrence rates are high. Postoperative radiotherapy is indicated following surgical excision. We present a 13-year-old girl who had been operated for the intraspinal mass in upper thoracic spine and paraparesis with thoracic limited laminectomy and excision of the tumor mass elsewhere. The histopathological examination was reported to be aggressive fibromatosis. After 2 years, she presented again with 1-year duration of progressive deformity in the upper thoracic spine and weakness of both lower limbs. Focal kyphosis at T4–T5 was measuring 68°. Magnetic resonance imaging (MRI) showed recurrent tumor involvement of posterior elements of T2–T5 and paravertebral soft tissues with signal changes in the cord at T2–T5 vertebral levels with focal kyphosis and internal gibbus. She underwent posterior spinal revision decompression with internal gibbectomy and instrumented fusion. The histopathology showed features suggestive of aggressive fibromatosis. After wound healing at 2 weeks, she underwent 3-D conformal radiotherapy, based on the preoperative tumor extent on MRI (dose of 45 Gy in 25 fractions over 5 weeks). She had normal neurology at 2-year follow-up and was tumor free on MRI. Hence, aggressive fibromatosis can recur following successful surgical wide excision. Multilevel thoracic laminectomy in growing children can cause progressive spinal deformity and neurological deficits. Operative treatment of recurrent tumor involves en bloc excision with instrumented fusion followed by local radiotherapy. This is the first pediatric recurrent spinal fibromatosis reported with successful treatment as per author’s knowledge.