A Unique Collaboration Between Otolaryngology, Cardiothoracic Surgery, and Spine Surgery for Paraspinal Desmoid-type Fibromatosis: A Case Report and Systematic Review

Introduction: Desmoid-type fibromatosis is a rare aggressive tumor of mesenchymal origin that mimics malignancy. Though being non-metastasizing, desmoid tumors exhibit local invasion and tissue destruction with high rates of recurrence, leading to severe morbidity. However, there is no standard approach to managing these tumors. Hence, we present a 69-year-old male with cervicothoracic desmoid-type fibromatosis and conduct a systematic review to analyze risk factors, treatment modalities, and recurrence.

Methods: Extensive chart review of the patient was conducted, with special attention to contributing risk factors and follow-up examinations. A systematic search was conducted in PubMed, SCOPUS, and Embase with the following term: ((vertebral) OR (spine)) AND (desmoid fibromatosis). Publications with desmoid-type fibromatosis at the cervical or cervicothoracic spine were included. A manual reference search was also conducted to include all relevant publications.

Results: Our patient’s tumor extended from the right anterolateral C3 to T1 level, with C6 and C7 vertebral infiltration. The patient underwent a radical neck dissection, hemithyroidectomy, and hemisternotomy for tumor resection. No spinal intervention or adjuvant medical therapy was utilized. His postoperative course was complicated by right vertebral artery occlusion and transient right-sided Horner syndrome. He was discharged on postoperative day 5 with no subsequent tumor recurrence or complications. Our systematic review resulted in 21 studies with 24 patients (8 males, 16 females) at an average age of 39.98±17.23 years. The most common treatment modality was resection (n=16, 66.67%). Five patients (20.83%) had concomitant spinal decompression with or without fusion. Recurrence occurred in three patients (12.5%), at an average of 7±5.57 months after initial intervention.

Conclusion : In our case report and systematic review, we found that the most common treatment modality of paraspinal desmoid-type fibromatosis confined to the cervical or cervicothoracic spine is resection, with minimal rates of recurrence. Concomitant spinal intervention may not be necessary depending on tumor localization and invasion.