Skeletal sarcomas are commonly seen in the proximal upper extremity and much less commonly in the distal upper extremity. All suspected skeletal sarcomas should be appropriately staged and biopsied (preferably by or after discussion with the treating orthopaedic oncologist), and then the patient should be referred for appropriate chemotherapy following discussion and recommendations from a multidisciplinary tumor board. At the time of surgery, an oncologically sound resection is the first and foremost concern; only after resection has been successfully accomplished can reconstruction begin.

With advances in chemotherapy and imaging, limb salvage surgery is now possible in 90% to 95% of patients with primary bone sarcomas, with limb salvage being comparable to amputation in terms of disease-free interval and long-term survival with only a slight increase in local recurrence rates.¹ With patients experiencing longer survival times, it is important that a durable reconstruction be attained.

Reconstruction can generally be thought of in the broad categories of arthrodesis, allograft, autograft, or arthroplasty. Historically, three primary means of reconstruction have been used as alternatives to amputation or arthrodesis after periarticular tumor resection: osteoarticular allograft, allograft-prosthetic composite (APC), and endoprosthesis. Table 1 shows the advantages and disadvantages of each method of reconstruction. Diaphyseal sarcomas of both the humerus and the forearm are often amenable to intercalary resection. Options for reconstruction following intercalary resection include intercalary allograft, vascularized fibular graft, extracorporally irradiated autograft, segmental prosthesis, and bone transport. Options for reconstruction following scapulectomy, resection of the proximal humerus, diaphyseal humerus, distal humerus and elbow, and forearm are discussed.

The scapula is a less common location for skeletal sarcoma; however, Ewing sarcoma, osteosarcoma, and chondrosarcoma, among others, are all found in this location. The first total scapulectomy for neoplastic disease was performed in 1856 and has since become an accepted technique for the treatment of primary malignant bone tumors. Scapulectomy as limb salvage surgery is contraindicated if the brachial plexus is involved and resections of portions of the brachial plexus would lead to a nonfunctional hand. Involvement of the brachial plexus may be suspected clinically if the patient has motor deficits or intractable pain at presentation.

The Malawer classification of shoulder girdle resections was proposed in 1991.² According to this classification system, a scapulectomy can be partial (type II), complete intra-articular (type III), complete extra-articular (type IV), or complete extra-articular in a more traditional Tikhoff-Linberg-type procedure (type VI). This classification scheme was modified by the Musculoskeletal Tumor Society (MSTS)³ (Figure 1).

Challenges following scapulectomy include attempts to maintain the mobility and stability of the shoulder joint as well as an acceptable cosmetic result because scapulectomy leads to loss of the normal shoulder contour. The functional goal following reconstruction after scapulectomy is to provide a stable base against which the patient can position the arm in space because elbow, wrist, and hand functions are typically preserved.

The patient is typically positioned in the lateral decubitus position with the entire forequarter draped free. The incision can vary, but can commonly begin at the inferior angle of the scapula, continue obliquely along the scapular spine toward the acromion process where it then crosses in a strap manner to the anterior shoulder, and can then be continued distally along the deltopectoral interval if needed.⁴ The biopsy site is excised en bloc with the tumor specimen. All muscular attachments are divided approximately 1 cm away from bone through normal tissue, beginning from the inferior angle and elevating the scapula away from the chest wall.

Partial scapulectomy typically only requires soft-tissue closure. If the glenoid is retained, the patient can expect near-normal function, but if the glenoid must be resected, functional outcomes and patient acceptance decrease dramatically.^4,5 Options after total scapulectomy include humeral suspension to the remaining clavicle or reconstruction with allograft or endoprosthesis. Humeral suspension involves suspending the native humerus or proximal humerus endoprosthesis to the remaining clavicle. Following this method of reconstruction, shoulder abduction ranges from 30° to 45°. Complications include deep infection (5.0% to 12.5%), periprosthetic fracture (10.5%), subluxation of endoprosthesis, and traction neuralgia or paresthesias if the humerus is not adequately suspended.^6,7 Reconstruction with scapular allograft involves using an osteoarticular allograft with soft-tissue reattachments preserved and careful repair of remaining host muscle back to the donor tissue. With allograft reconstruction, shoulder range of motion has been reported with ranges of flexion to 40° to 80°, abduction to 30° to 75°, and functional MSTS scores ranging from 66.7% to 82%. Complications include infection, dislocation, fixation hardware failure, and allograft fracture and resorption.^8,9 Reconstruction with scapular endoprosthesis involves laying the prosthesis in place; careful tenodesis of the latissimus dorsi, rhomboids, trapezius, and deltoid over the prosthesis; and articulation with a proximal humerus prosthesis (most recently in a constrained implant design). Shoulder abduction and flexion have been reported in the range from 25° to 45°; functional MSTS scores ranged from 66% to 90%. Complications include dislocation, infection, aseptic loosening, and periprosthetic fracture.^10,11 As in other orthopaedic subspecialties, surgeons are evaluating the use of custom three-dimensionally printed implants to aid in reconstruction after scapulectomy; however, the results are currently limited to small series and case reports.¹²

Based on the current literature, reconstruction with allograft or endoprosthesis could provide moderately improved range of motion (depending largely on the extent of soft-tissue [deltoid and rotator cuff] resection required), and improved cosmesis; however, it is associated with additional complications such as aseptic loosening, dislocation, periprosthetic fracture, and allograft fracture and resorption. The treating surgeon must carefully consider the risks and benefits when deciding whether to proceed with reconstruction.

The proximal humerus is a common location for skeletal sarcoma, representing the third most common location for osteosarcoma and a common location for chondrosarcoma. The challenge following the resection of proximal humeral lesions is replication of normal shoulder anatomy. The glenohumeral joint is a ball-and-socket joint that allows numerous degrees of freedom, lending itself to tremendous mobility and thus requiring significant stability. Commonly, the rotator cuff is resected with the tumor, and occasionally the deltoid or axillary nerve must be sacrificed, having a significant effect on shoulder function.

At the time of biopsy of a proximal humerus sarcoma, the extended deltopectoral incision should be marked out, with the actual biopsy performed slightly lateral to the interval to position the biopsy tract through the anterior portion of the deltoid muscle and not through the true deltopectoral interval. This technique helps contain any postbiopsy hematoma to the deltoid and avoid contamination of the cephalic vein or pectoralis major or spread onto the chest wall.

In the preoperative workup, it is important to determine whether the tumor extends into the glenohumeral joint and thus whether intra-articular or extra-articular resection is required. Routes of intra-articular tumor spread include direct extension through articular cartilage or capsule, extension of tumor along the long head of the biceps tendon, and hematoma from pathologic fracture. Contraindications to limb salvage are tumor invasion of the brachial plexus and axillary artery and extensive invasion of the chest wall.

The extent of resection can be classified using either the Malawer classification² or the MSTS classification³ (Figure 1). In both classifications, A denotes that the abductor mechanism is intact, and B, that the abductor mechanism is disrupted. The status of the abductor mechanism is key in decision making because allograft or endoprosthesis may be chosen if it is intact or arthrodesis if it is disrupted. A suggested reconstruction algorithm based on the MSTS classification was proposed in 1996⁶ (Table 2).

Limb salvage surgery of the shoulder girdle began with the description of the interscapulothoracic resection, known as the Tikhoff-Linberg procedure, in 1928.¹³ The Tikhoff-Linberg procedure is an en bloc extra-articular resection including the proximal humerus, entire scapula, lateral two-thirds of the clavicle, rotator cuff, deltoid, coracobrachialis, and proximal biceps. This procedure resulted in a fully functional hand and forearm, but a flail shoulder. Modifications include removal of only the glenoid portion of the scapula when possible; use of alternative incisions; and addition of a spacer or prosthetic reconstruction of the humerus, which is then suspended from the remaining scapula and clavicle. Following this method of reconstruction, shoulder abduction ranges from 30° to 45°. The overall complication rate is high (up to 47%). Complications include deep infection (5.0% to 12.5%), periprosthetic fracture (10.5%), subluxation of endoprosthesis, and traction neuralgia or paresthesias if the humerus is not adequately suspended. Functional MSTS scores have ranged from means of 40% to 83%.^13,14 Because of the extensive soft-tissue resection required for extra-articular resections, it is sometimes necessary to add a rotational latissimus dorsi or pectoralis major flap for coverage of the spacer or endoprosthesis.

Arthrodesis can also be performed following extra-articular proximal humerus resection. This procedure may be favorable in a young patient who wants to return to strenuous activity. The goal is to provide a stable, painless base against which the hand can be positioned in space. Techniques include allograft arthrodesis, vascularized fibular grafting, and a combination of the two. The arm is positioned where the hand can easily reach the mouth without excessive scapular winging with the arm at the side, approximately 15° to 30° abduction, 15° to 30° forward flexion, and 20° to 50° internal rotation (the exact position is much debated). Several methods of fixation are described, but the most common probably is the use of a long plate contoured along the scapular spine, over the acromion, and extending down the humerus in addition to screws placed through the humeral head into the glenoid. Patients are immobilized in either a sling or shoulder spica cast for several weeks. Some motion of the arm is maintained through the thoracoscapular articulation, approximately 45° to 60° of forward flexion and abduction, although internal and external rotation are severely limited. The overall complication rate is high, with reports of up to 43% of patients requiring a second major surgical procedure. Complications include nonunion (zero to 40%), fracture (4% to 40%), and infection (zero to 37.5%).¹⁵

Another option is the clavicula pro humero procedure. This procedure was originally described for the treatment of phocomelia in 1963 but was reported to be performed after shoulder girdle resections of malignant tumors in 1992.¹⁶ The clavicle is cut at the sternoclavicular joint, the sternocleidomastoid and trapezius muscles are released, and the entire clavicle is rotated down to meet the residual humerus, pivoting on the acromioclavicular joint. The mean length of the clavicle available is 13.5 cm, and thus the planned resection length must be considered. The arm is immobilized in a sling for 3 weeks, after which passive and active motion are initiated. Using the clavicle as a vascularized autograft improves union rates and avoids the risk of disease transmission with allograft. Stability is provided by the intact acromioclavicular joint. The overall complication rate is high, with a revision rate as high as 67%. Complications include hardware failure, nonunion (14% to 27%), fracture (zero to 27%), and deep infection (zero to 27%).^16,17