Sporadic desmoid fibromatosis of the neck after dorsal spondylodesis of the cervical spine

Summary

Background: Rare soft-tissue tumors, termed desmoid fibromatosis (DF), are comprised proliferated spindle cell fibroblasts and myofibroblasts embedded in a prominent collagenous stroma. They can occur either sporadically, due to prior trauma or surgery, or may have a genetic component. Clinically, DF has a high infiltrative growth/ local recurrence rate, but does not metastasize. Case description: A 58-year-old male underwent a C5-C7 laminectomy/instrumented fusion. Two years later, he presented with a large gross swelling on the right side of the neck. The lesion was removed and proved to histologically consist of DF. Within the first postoperative 12 months, tumor did not recur. Conclusion: Sporadic DF may follow trauma or prior surgery. Symptomatic tumors are treated by surgical en bloc resection (preferably R0). If lesions are inoperable, partially resected, or recur, different hormonal/ chemotherapeutic systematic treatment options are available (e.g., tamoxifen or tyrosine kinase inhibitors). In the future, better molecular understanding of DF likely offers additional therapeutic approaches (e.g., immune checkpoint inhibitors).