Extremely rare musculoskeletal tumors of the upper extremity: An institutional experience with four cases from a dedicated orthopedic oncology team

The musculoskeletal structure of the upper extremity, due to its crucial functional role in daily life, makes it an exceptionally challenging area to operate on. The complex network of vessels, nerves, and muscular structures further complicates surgical procedures, particularly in the context of tumor surgery. While the upper extremity is not a common site for tumors, it can occasionally be affected. Achieving negative surgical margins in this difficult area is particularly challenging. In this study, we discuss evidence-based strategies employed in the treatment of several rare upper extremity tumors and present the first reported case of an upper extremity-originated chordoma. This retrospective case series adheres to the PROCESS guidelines. Patients with rare bone or soft tissue tumors treated in our orthopedic oncology unit between January 2022 and December 2023 were included. Data were collected from electronic medical records, including demographics, clinical presentation, imaging studies, surgical interventions, and postoperative outcomes. Diagnostic workup involved physical examination, radiographic imaging, and histopathological analysis. Surgical interventions were based on tumor type and location. Primary outcomes included symptom resolution, postoperative complications, and recurrence rates. Secondary outcomes focused on functional recovery. Case 1:A 21-year-old male with wide spread synovial chondromatosis of shoulder underwent sequential arthroscopic and open excision of lesions. Case 2: A 27-year-old female with intraosseous schwannoma and pathological fracture received curettage and intramedullary nailing after confirmation with pre operative biopsy. Case 3: A 37-year-old male with a paraganglioma had the mass excised completely after pre opeative diagnosis with biopsy. Case 4: A 60-year-old female with extra-axial chordoma of the proximal humerus underwent wide resection and prosthetic reconstruction. All cases achieved successful oncological and functional outcomes with no complications. Evidence-based surgical strategies, multidisciplinary collaboration, and advanced techniques can achieve successful oncological and functional outcomes in the upper extremity. Awareness of rare tumors is crucial for accurate diagnosis and effective treatment.