Spinal fibromatosis: a report of two cases and review of the literature.

ABSTRACT

BACKGROUND CONTEXT Spinal fibromatosis is a unique subset of fibromatosis that is only anecdotally described in the literature in sporadic case reports. According to our review of the literature, only 11 cases of spinal fibromatosis have been previously documented. This paucity of clinical data limits our understanding of its presentation and treatment. PURPOSE: The authors present the first two cases of spinal fibromatosis encountered at their institution, and review the literature of reported cases to elucidate the presentation and outcomes of patients with this rare tumor. STUDY DESIGN: A report of two cases and review of the literature. METHODS: The two patients in our case report were women aged 45 and 38 years. Both of the patients presented to our clinic after previous excisional biopsy of a spinal mass, 17 years and 1 year later, respectively, with pain and paresthesias that recapitulated their former symptoms. Thirteen cases, including the two described in the current article, were culled from the literature. RESULTS: Magnetic resonance imaging revealed an enhancing lesion in the posterior elements of the spinal column in the first case and a paraspinal soft tissue mass in the second case. The tumors were histologically defined by haphazardly arranged, elongated, and slender spindle cells separated by abundant collagen without mitoses or necrosis. Surgical management resulted in intralesional resection in the first case and en bloc resection in the second case. By 40 and 10 months after surgery, both patients remain without neurologic deficits. CONCLUSIONS: Among 13 cases of spinal fibromatosis, pain with or without a mass is the most common symptom at presentation. Tumor etiology is evenly distributed between de novo origin and surgical trauma. Treatment outcomes, although, cannot be determined from the limited data currently available.