Malawer limb salvage surgery for the treatment of scapular chondrosarcoma

Chondrosarcoma is a malignant bone tumor and it is the second highest cause of morbidity among adult bone tumors. It accounts for 20% of all malignant bone tumors. Chondrosarcomas constitute a heterogeneous group of neoplasms that share the common feature of producing the cartilage matrix [1]. Generally, chondrosarcoma is intramedullary, and the fundamental tumor tissue is the new cartilage tissue, sometimes with ossification, calcification and mucoid degeneration. The tumor always has a primary origin, but a few secondary cases have also been reported [2]. The main symptoms are pain and swelling. As radiology and chemotherapy are usually ineffective, the treatment most often used is surgical resection. If a complete surgical resection is not performed, the tumors reappear in the lesions and, rarely, lead to metastasis to distant sites. The prognosis depends largely on the location of the original lesion, obtaining negative sections and the histological degree of the tumor [3‐6].

Chondrosarcoma does not usually develop in the osteoid tissue. It usually occurs in cartilage of the pelvis, femur, tibia and humerus. Scapular chondrosarcoma is rare, accounting for about 5 to 7% of all reported cases of chondrosarcoma [7, 8]. Until the 1970s, amputation was the preferred treatment for severe cases of malignant tumors around the scapula. Since then, an increasing number of patients have adopted the limb salvage treatment as the first choice, and in most cases, the outcomes have been good [9]. Although there have been many reports of the use of limb salvage treatment, the tumor was not large and there was no damage to the soft tissue, nerves and blood vessels around the tumor in most studies. To our knowledge, there are few reports on the use of limb salvage treatment for patients with extremely large tumors. Also, the relevant risks and the efficacy of such therapy for the treatment of large tumors are unknown.

In this study, we present a case of a large chondrosarcoma in the scapular area. Based on the preoperative evaluation of the surrounding soft tissue and the reconstruction design, the patient was treated with Malawer limb salvage surgery. No complications were reported postoperatively and the patient retained limb function.

The patient was in compliance with the Declaration of Helsinki. A 59-year-old farmer with a personal history of a lump on his right scapula for the past five years was admitted to hospital. In two years prior to the first hospital visit, the lump had grown significantly and had restricted the activity of the right shoulder. The patient did not have a family history of tumors.

Physical examination indicated no apparent spread to the superficial lymph nodes. There was a huge, irregular lump in the region of the right shoulder, with the superior border reaching the acromion, the left border reaching to 1 cm from the spinal column, the inferior border reaching the seventh rib, and the lateral border reaching the midaxillary line. The lump was nodular, hard and caused a pressing pain. There was an obscure boundary between the tumor and the surrounding tissues, the skin around the tumor was red but there was no venous engorgement or vascular pulsation (Figure 1). Fifty degree abduction of the right shoulder joint, 0° adduction, 30° flexion, 40° extension and 10° external and internal rotation were observed. The pulsation of the right ulnar artery and the right radial artery were good. Myodynamia of the main right upper extremities were as follows: supraspinatus at level III, deltoid at level IV, muscles of biceps brachii at level IV, extensor carpi at level IV, flexor digitorum profundus at level IV and abductor digiti quinti at level IV.

The results of the vascular ultrasound of the right clavicle and the axilla were as follows: the axillary artery was visible in front of the tumor with smoothly flowing blood; there was an obvious boundary between the subclavian artery and the tumor with smoothly flowing blood. The results of the X-ray (Figure 2) and computer tomography (CT) scan (Figure 3) were as follows: a large high-density mass was seen in the right scapula and the surrounding tissue with multifocal calcification. As can be seen in the X-ray and the CT scan, the entire scapula was involved. In addition, the scapula showed osteolytic destruction without normal structure and the remaining scapula was embedded in the tumor tissue. There were no abnormalities adjacent to the ribs, humerus and thoracic vertebra. The isotope bone scan showed the concentration of radioactivity in the right scapular area with abnormal mineral metabolism in the bones. The biopsy indicated grade II (out of III) chondrosarcoma. The diagnosis was as follows: chondrosarcoma in right scapular, Malawer S1 S2 district, Enniking II B district, grade II chondrosarcoma.

Two weeks after hospitalization, the patient was treated with the Malawer III limb-reserving surgery under general anesthesia. The levator scapulae, rhomboid, supraspinatus, infraspinatus and teres muscles along the medial walls of the scapula were stripped and resected in an order based on the principles of wide resection. Simultaneously, the nerves and blood vessels that enter the tumor tissue were excised. The tumor was widely adherent to the surrounding soft tissue. Also, its relationship with the axillary artery and the subclavian artery was obscure. It was difficult to directly turn the rib. Under these circumstances, the incision was extended, the clavicle was resected and then the teres was moved downward. There was quite a close relationship between the subclavian artery, the axillary artery and the tumor, but there was some separation with soft tissue envelope. The serratus anterior was partly cut down to completely expose the tumor (Figure 4 and Figure 5). The scapular was entirely embedded in the tumor tissue, hence it was removed while the tumor was resected. The size of the tumor was 33 × 28 × 25 cm. There was no involvement of the proximal humerus and the thoracic vertebrae. Then, the lesions and the surrounding tissue were obtained for pathological analysis. As the pathology results of the surrounding tissue were negative, the humerus was attached to the second rib with a steel wire, then the supraspinatus, infraspinatus, teres minor and the rhomboid muscles were sutured. The trapezius, ectopectoralis and deltoid were sutured together. The brachialis, musculus biceps brachii and musculus triceps brachii were sutured together and fixed to the first rib. The brachycephaly of musculus biceps brachii was fixed to the second rib to ensure the balance of the shoulder joint (Figure 6). The patient was required to maintain his elbow in a flexed position with a bandage for three weeks. The postoperative X-ray showed that no tumor tissue remained (Figure 7). On the first postoperative day, the myodynamia and sensation of the major muscles under the right shoulder were the same as prior to the operation. On the third postoperative day, the patient exercised his hand and wrist functions with a patient controlled epidural analgesia. At postoperative week three, partly loading functional training was performed and at the sixth week, fully loading functional training was performed. The stitches were removed two weeks postoperatively without any complications. The pathology report was almost the same as that prior to the operation (Figure 8). The MSTS (Musculoskeletal Tumor Society Score) score was 22 one year after the surgery. Postoperative radiography at 3, 6 and 18 months detected no tumor recurrence. There was no evidence of metastasis at the follow-up end point.

In the present case, the tumor was large and many nearby tissues had been invaded, especially anteriorly. The tumor was very near to the axillary nerves and blood vessels. Therefore, it was very difficult to perform the limb salvage surgery and considerable risks were associated with the surgical procedure. Moreover, wide resection of tumor can lead to loss of viability of the skin and the soft tissue. All these issues need to be considered and planned carefully preoperatively. This case is a unique one and cannot provide a therapeutic guide for chondrosarcoma. However, the operation was a great success and the patient showed good recovery. This case provides an example for the treatment of giant chondrosarcomas.

Written informed consent was obtained from the patient for the publication of this report and any accompanying images.