Periosteal Osteosarcoma: Emerging Clinical Concepts, Evolving Treatment Options: A Narrative Review

Periosteal osteosarcomas are rare bone tumors that comprise less than 2% of all bone malignancies. The tumor was previously thought to affect the diaphysis of long bones solely, but Recent case reports have demonstrated its existence in the clavicle, mandible, and scapula.

Several factors, including stage, grade, and medullary invasion primarily drive survival and recurrence of periosteal osteosarcomas. Related to its rarity, little data currently exists regarding optimum treatment strategies, including limb amputation, surgical resection, and chemotherapy in the neoadjuvant/adjuvant setting. In this regard, bone pain is the most common manifestation. Pain treatment can include medications such as oral or transdermal opioids. Adjuvant drugs can reduce inflammation and neuropathic pain, including corticosteroids, gabapentinoid agents such as gabapentin or pregabalin, lidocaine patches, tricyclic antidepressants such as amitriptyline, nortriptyline, and desipramine, or selective serotonin-norepinephrine reuptake inhibitors such as duloxetine and venlafaxine. More advanced pain treatment interventions can be provided by interventional pain medicine physicians, such as radiofrequency ablation, peripheral nerve stimulation, spinal cord stimulation, or intrathecal drug delivery systems, when conventional opioids and adjuvants do not adequately provide relief. Molecular workups of case reports have shown common mutations, such as the p53 gene, which may provide utility for future molecular targets. The present investigation aims to synthesize currently available data on periosteal osteosarcomas, including common clinical, histologic, and radiographic presentations. In addition, this review aims to further examine the potential for biomarkers and provide recommendations for treatment and future research direction.