Malignant and aggressive forearm tumors are rare and challenging to treat. Malignant tumors comprise only 3% of upper limb tumors; osteosarcoma is rare in the hand and forearm.4,6 Available options for definitive treatment of malignant and aggressive forearm tumors include amputation, resection with reconstruction of bony deficits using autograft or allograft, and creation of a single-bone forearm with or without the use of bone graft. Patients undergoing upper extremity amputation have considerable problems, including negative perception of body image, prosthesis-fitting problems, and decreased functionality compared with individuals having a functioning upper extremity.2,8 Massive bone defects after tumor resection impose limitations on autogenous bone grafting. If substantial portions of the radius and ulna are resected, reestablishing a two-bone forearm with allografts potentially doubles the number of graft-host junctions and the inherent risk of nonunion, delayed union, infection, and fracture associated with structural allografts.11
We performed single-bone forearm reconstruction in four children with malignant and aggressive forearm tumors that required extensive bone resection. We describe the procedures performed, local recurrences and metastases, functional outcomes, and complications.
MATERIALS AND METHODS
From our musculoskeletal tumor database, we identified four patients who had single-bone forearm reconstruction after tumor resection. We reviewed their medical records and imaging studies for details of surgery, complications, presence of local recurrence or metastases, functional outcome, and radiographic union of osteosynthesis sites. The patients' ages ranged from 10 to 15 years at the time of reconstruction and all had malignant or aggressive tumors in the forearm. Patient 1 had a soft tissue Ewing's sarcoma of the distal forearm (Fig 1), Patient 2 had a recurrent desmoplastic fibroma involving the radius and ulna (Fig 2), and Patients 3 and 4 had sarcomas of the radius (Figs 3 and 4) (Table 1). Preoperative imaging included plain orthogonal radiographs and MRI of the entire forearm, computed tomography (CT) of the chest, and whole-body bone scan.
Three patients with sarcomas received neoadjuvant and adjuvant chemotherapy as per their oncologist's protocol. We held discussions with the patients and their families, exploring the options of allograft reconstructions and vascularized free fibular grafting. The patients' decisions were made according to which risks were more acceptable to them, ie, longer surgery and donor-site morbidity with fibular grafting versus infection and risk of disease transmission with allografts. A distal radius allograft was used in two patients (Figs 1C, 2D-E), one patient received a vascularized free fibular graft to his distal radius (Fig 3C-E), and no structural bone graft was used in the last patient (Fig 4B-D). Wide, negative surgical margins were achieved for all patients. One patient received postoperative external beam radiation.
Functional evaluation was conducted using the Musculoskeletal Tumor Society (MSTS) upper extremity scoring system3 at each followup. The MSTS upper extremity scoring system consists of a maximum of 5 points for each category, including pain, function, emotional acceptance, hand position, manual dexterity, and lifting ability. The highest possible score is 30.
The same imaging studies performed preoperatively were repeated for followup surveillance and were performed every 3 months for the first 2 years, then every 6 months for the following 3 years, and annually beyond 5 years. Radiographs were taken at 6-week intervals to assess union of osteosynthesis sites.
In the three patients with sarcomas, there was no local recurrence. One patient died of pulmonary metastasis at 23 months; two are continuously disease-free at 65 and 82 months (Table 2). The patient with desmoplastic fibroma of bone has new evidence of a soft tissue recurrence on MRI at 12 months; additional treatment is being discussed with the patient.
The average overall MSTS score was 26.25 (87.5% of a maximum possible score of 30). The average component scores were: pain, 4.75/5; function, 4.25/5; emotional acceptance, 4.25/5; hand position, 4.75/5; manual dexterity, 4.5/5; and lifting ability, 3.75/5 (Table 2).
There were seven osteosynthesis sites in four patients. The patient who had centralization of the ulna had partial union of the osteosynthesis site at 4 months but had no additional progress during the next 6 months. She was given pulsed electromagnetic field stimulation (EBI, Parsippany, NJ) and full consolidation was achieved at 21 months. The patient who had a vascularized fibular graft had healed graft-host junctions at 2 months proximally and 4 months distally. Of the two patients with allografts, one died with a nonunited proximal junction; the distal junction had healed at 7 months. He had received postoperative external beam radiation to his surgical site because of poor chemotherapy response. In the other patient, the allograft-host junctions healed at 4 months proximally and 8 months distally.
We observed five complications. There was a superficial wound infection that resolved with débridement and antibiotics in one patient; he also had nonunion of an allograft host-junction subjected to radiation. An acute carpal tunnel syndrome developed within 1 week in one patient, which resolved after surgical release; he also had wound necrosis develop 1 month postoperatively and that was treated successfully with débridement, flap closure, and skin grafting. One patient had a delayed union of a native bone osteosynthesis site that consolidated after pulsed electromagnetic field stimulation.
We performed single-bone forearm reconstruction in four children with malignant and aggressive forearm tumors that required extensive bone resection. We described the procedures performed, the presence of local recurrence or metastases, functional outcomes, and complications.
The limitations of our study include the small series and absence of control patients using other reconstructive techniques. These four patients were selected and might not represent all patients in whom the procedure might be considered. We used limited objective measures of hand function, such as grip strength, pinch, and two-point discrimination.
The treatment of malignant and aggressive forearm tumors is challenging. The primary goal is to achieve complete resection of the tumor without increasing the risk of local recurrence. Historically, amputation was the preferred treatment, but with the advent of chemotherapy, advances in musculoskeletal imaging, and improvements in surgical techniques, limb salvage is being performed more frequently. The ability to preserve hand function is a key determinant in deciding whether a limb-sparing procedure would be better than an amputation, provided surgical margins are not compromised. It is difficult for a patient with aggressive but benign tumors (such as our Patient 2) to accept an amputation as a primary option and we believe it should be reserved for failed reconstructions.
After resections that leave massive bone defects, reconstructive options include osteoarticular allografts,7,14 free vascularized bone transfer,9 nonvascularized autogenous bone grafts, centralization of the carpus over the remaining ulna,16 and a single-bone forearm reconstruction.1,5,10,12,15 Limb salvage using structural grafts to maintain a two-bone forearm is preferred for smaller forearm tumor resections, and preservation of the radiocarpal joint is ideal if it can be done. In our patients, soft tissue extension of the tumors into the interosseous space necessitated resections of large segments of the ulna in addition to resection of the radius. Performing a two-bone forearm reconstruction in such massive forearm resections increases the risk of delayed union and nonunion because of increased number of graft-host interfaces. There is also an ensuing lack of soft tissue/muscle attachments (such as the interosseous membrane) stabilizing the bony anatomy to permit stable active movements. Reconstruction of the distal radius with an osteoarticular allograft has been used with satisfactory outcomes.7,14 However, because of the very distal location in the radius and soft tissue extent of the tumors in our series, the radiocarpal joint could not be preserved. Therefore, we believed a single-bone forearm with a radiocarpal arthrodesis was the best limb-preserving option.
Single-bone forearm reconstruction was first described for treatment of severe forearm defects resulting from traumatic and infective etiologies. Watson-Jones15 in 1934 described reconstruction of the forearm after loss of the radius with a single-bone forearm approach. Similarly, Murray10 reported two patients undergoing the creation of a single-bone forearm for severe trauma to the upper extremity. They reported cross-union of the proximal part of the ulna and the distal end of the radius as an acceptable form of treatment for individuals with considerable instability and bone loss difficult to reconstruct with bone grafting. Another study by Haddad and Drez5 reported two single-bone forearm reconstructions from a case series of 10 patients with radioulnar fusions. One patient had the procedure because of considerable bone loss from trauma and the other patient because of a congenital absence of the distal ulna. They concluded creation of a single-bone forearm could produce a cosmetically and functionally acceptable upper extremity. Castle1 described a case series in which six patients had single-bone forearm reconstruction with the forearm positioned in neutral supination/pronation for non-united fractures and deficits of the ulna with soft tissue defects (traumatic etiology in five patients and infective etiology in one patient). A stable, functional extremity resulted in all patients. The creation of a single-bone forearm also was described for treating congenital forearm deformities. Starr13 in 1945 reported a single-bone forearm approach consisting of centralization of the hand and wrist on the ulna for treating congenital absence of the radius. The patient had a satisfactory clinical out come.
Seradge12 described the creation of a single-bone forearm in two patients with a giant cell tumor of the distal end of the radius treated by en bloc resection. Resection was followed by reconstruction of the forearm with the ulnar segment internally fixed to the proximal segment of the radius and to the carpus with an intramedullary Steinmann pin. The distal articular surface of the ulna was resected and the ulna was fused to the lunate and the scaphoid. Both patients obtained a useful, pain-free extremity, with greater than 85% forearm rotation and 10° to 15° flexion-extension at the wrist.
We performed single-bone forearm reconstructions on our four patients to preserve a functional and cosmetically acceptable upper extremity. The average MSTS upper extremity score was 26.25/30 (87.5%). A stable, pain-free upper extremity was achieved and was well accepted by the patients. Because of preserved elbow and shoulder motion, hand positioning was not a problem. No major nerves were sacrificed and the majority of the muscle-tendon units were intact, leading to preservation of hand dexterity. We attempted to preserve as much of the forearm musculature as possible, although most resections involved not only the distal but also the proximal parts of the bone and soft tissues. Soft tissues were reflected off sub-periosteally in areas where the tumor appeared intraosseous on MRI. However, maintenance of strength was modest, as reflected by the relatively lower scores for lifting ability. We achieved wide surgical resections with negative margins, but nonetheless one patient with desmoplastic fibroma of bone had a local recurrence. No additional surgical procedures were performed to achieve union of the osteosynthesis sites, although one patient died with a nonunited allograft-host junction. His did receive radiation to the forearm, which is known to adversely affect allograft healing.
The creation of a single-bone forearm produces reasonable functional results and we believe it is a sensible form of treatment for malignant and aggressive forearm tumors that require massive bone resections.
We thank Dr. Shyam Kishan, MD, for help with acquisition of data during our study.
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