Radical Resection of a Recurrent Giant Cell Tumor of the Distal Ulna and Immediate Reconstruction With a Distal Radio-Ulnar Joint Implant Arthroplasty

Abstract

Background: Giant cell tumors (GCTs) of the distal ulna are rare. Despite being benign, they can be locally aggressive and may recur following conventional treatment by curettage and bone grafting. Salvage reconstructive options after failed conventional treatment include Darrach resection, Suave-Kapandji procedure, hemi-arthroplasty, or total joint arthroplasty. Methods: We discuss reconstruction options for the distal radio-ulnar joint following tumor resection, and present the outcomes of a constrained distal radio-ulnar prosthesis in a 29-year-old male following resection of a distal ulna GCT. Results: Reconstruction of the distal radio-ulnar joint by a constrained prothesis yielded excellent functional outcomes following resection of a GCT of the distal ulna. Conclusions: This case demonstrates that successful oncologic and functional outcomes can be achieved by radical resection of a recurrent GCT of the distal ulna and reconstruction with a constrained total joint arthroplasty.

Introduction

Giant cell tumors (GCTs) are benign but locally aggressive tumors, usually occurring at the epiphyses of the distal femur, proximal tibia, and distal radius. GCTs of the upper extremity are relatively rare accounting for approximately 3% to 5% of all primary bone tumors^1,2 and GCTs of the distal ulna are extremely rare.³ The peak incidence is in the third decade, with 70% of the patients diagnosed between the second and fourth decades. The conventional treatment of GCTs of the distal ulna are similar to that of other locations—curettage and bone grafting for quiescent types and wide resection of more aggressive lesions. However, wide excision of the distal ulna has implications for function, since the distal radio-ulnar joint allows forearm rotation and has an important role in load bearing.⁴ Wide local excision, with or without stabilization of the proximal stump of the ulna, is the currently accepted treatment for recurrent GCTs of the distal ulna. While previous studies have reported excellent results with just resection of the distal ulna,³ distal ulna resections for arthritis show mixed results with an average of 2.2 surgeries to correct instability of the ulna stump.⁵ A recent systematic review of distal radio-ulnar joint arthroplasties has demonstrated that the Scheker constrained total distal radio-ulnar joint prosthesis (APTIS Medical, Louisville, Kentucky, USA) has infrequent complications, with 97% implant survival at a mean of 56 months postoperatively.⁶

Methods

A 29-year-old right-handed man employed as an air conditioning installation technician presented with pain and swelling over the ulnar aspect of his left wrist for 4 months. Radiographs revealed an expansile lytic lesion of the distal ulna, suspicious for a GCT, and a magnetic resonance imaging (MRI) scan supported this diagnosis of a mass measuring 5.5 cm x 4.5 cm x 3.5 cm with low intensity on T1-weighted images and a heterogeneous signal on T2 fat-suppression images. A biopsy confirmed the diagnosis of GCT. The patient underwent preoperative adjuvant chemotherapy with 5 doses of denosumab over a 2-month period before the lesion was subsequently curetted and bone grafted with allograft and demineralized bone matrix.

Seven months later, the patient presented again complaining of increasing pain and swelling of the dorsal and ulnar aspect of his left wrist (Figure 1) and radiographs (Figure 2) revealed that the lesion had enlarged, extending 5.5 cm proximal to the ulnar styloid.

Figure 1.

Preoperative radiograph showing a large erosive lesion in the distal ulna.

Figure 2.

Preoperative clinical photograph of the wrist.

Surgical options were discussed with the patient including a Darrach resection, although this would necessitate a very proximal resection of the ulna, hemi-arthroplasty, or a constrained total joint arthroplasty. The patient wanted to continue in his current job, which involved heavy lifting, and was concerned about the functional limitations of a proximal resection or hemi-arthroplasty and therefore elected for a total joint arthroplasty.

A preoperative MRI scan with contrast was used to determine the proximal margin of resection which was determined to be 7 cm proximal to the ulnar corner of the articular surface of the radius and a radiographic template was designed to ensure appropriate placement of the prosthesis. Surgery was performed under tourniquet control but without prior exsanguination. The tumor was approached through an L-shaped incision with a longitudinal component over the distal ulna and a transverse extension at the level of the wrist joint. A wide extra-capsular resection was performed so as to not violate the tumor capsule (Figure 3). The tumor involved the dorsal sensory branch of the ulnar nerve and the extensor carpi ulnaris tendon and both these structures were included in the resection. Two millimeters of the ulnar aspect of the distal radial cortex encompassing the sigmoid notch was also resected both to ensure a negative margin and for better placement of the radial component of the prosthesis. The level of the proximal osteotomy of the ulna was performed 7 cm proximal to the ulnar styloid as prescribed by the preoperative MRI scan.

Figure 3.

Intra-operative photograph of the resected giant cell tumor.

In order to allow for the more proximal resection of the ulna, the planned position of the radial component had to be translated more proximally by 1.5 cm. The lug of the trial radial implant was drilled and the cortical screws inserted to fix the trial implant along the ulnar border of the radius. Satisfactory position of the trial implant was confirmed by intraoperative radiographs. The proximal ulna was serially reamed and a 6 mm diameter stem with a 4 cm extension was inserted into the proximal ulna and its position confirmed by radiographs. A 20 mm radial component was then fixed to the radius and the prosthesis assembled. Full pronation and supination of the forearm and stability of the distal radio-ulnar prosthesis was confirmed intraoperatively. The extensor retinaculum was repaired with 4-0 nylon mattress sutures and the skin closed with interrupted 4-0 nylon mattress sutures. The forearm was immobilized in neutral position in a sugar-tong splint and the patient was admitted for 24 hours for elevation and intravenous antibiotics. The sugar-tong splint was removed 10 days postoperatively to reveal uneventful healing of the incision and sutures were removed. The patient began pronation and supination exercises under the direction of a hand therapist. Permanent histology confirmed negative margins both for bone and soft tissue. The patient also received postoperative adjuvant chemotherapy with 3 doses of denosumab.

Results

Despite having been advised to seek alternative employment, the patient returned to full duties at his original job as an air conditioning installation technician and continued to do heavy lifting. Two years postoperatively, he continues to have no pain in his left wrist. He has 90 degrees of forearm pronation and 90 degrees of supination, and 70 degrees of wrist flexion and 70 degrees of wrist extension, all similar to his normal right forearm and wrist. Postoperative radiographs confirm continued satisfactory alignment of the implant with no signs of loosening of the implant or recurrence of the GCT (Figures 4 and 5).

Figure 4.

Postoperative radiographs at 2-year follow-up.

Figure 5.

Range of motion at the 2-year follow-up.

Discussion

GCTs of the distal ulna are very rare with a reported incidence of up to 6% of GCTs⁷ and most commonly affect young adults aged 20 to 40 years with a female predominance.^8,9 Several surgical options have been proposed for treatment of GCTs of the distal ulna including curettage alone, curettage with bone graft, cryotherapy, polymethylmethacrylate or phenol, radiation, resection alone, and resection with an allograft or autograft.

Despite being a benign tumor, GCT is locally aggressive and has a significant risk of recurrence, particularly when the lesion is only curetted or even when combined with adjuvant therapy. Haskell et al¹⁰ reported a 25% to 35% recurrence rate and Rock et al¹¹ reported a 10% to 47% recurrence rate following curettage. Park et al¹² reported that most recurrences occurred early within 2 to 5 years, similar to this patient who was young and developed a recurrence within 1 year of treatment with both surgery and chemotherapy.

If a GCT is aggressive or recurs, curettage either alone or with adjuvant therapy is no longer appropriate. Salvage options include Darrach resection of the distal ulna alone or in combination with reconstruction. Some authors believe that reconstruction following excision of the distal ulna for tumors is not necessary, but this is not universally accepted.^3,13 Other authors believe that weakness, stump impingement, and ulnocarpal translation may occur after simple excision alone and therefore advocate reconstruction.^5,14,15 Since these tumors occur in younger higher demand patients, Darrach resection of the distal ulna alone may not provide acceptable strength and function.

Tendon stabilization of the proximal stump of the ulna after resection of the distal ulna was first described by Goldner and Hayes,¹⁶ and this technique was utilized by Kayias et al¹⁷ after excision of a GCT of the distal ulna. Wurapa and Whipple¹⁸ used a 2-stage tendon allograft reconstruction following resection of a GCT of the distal ulna. The patient achieved 50 degrees of pronation and 45 degrees of supination of the forearm, and 30 degrees of flexion and 40 degrees of extension of the wrist. Hashizume et al¹⁹ described using allograft bone graft as an ulnar buttress arthroplasty for reconstruction after resection of the distal ulna for a GCT and reported full range of motion and grasp similar to the contralateral wrist.

Nonconstrained hemi-arthroplasty of the distal ulna has been described for reconstruction of the distal ulna after resection of GCTs. Burke et al²⁰ reported a hemi-arthroplasty stabilized with a brachioradialis wrap-around transfer and Roidis²¹ reported a patient who underwent a hemi-arthroplasty after excision of a GCT who was pain free and had returned to work, but with restrictions of lifting no more than 3 kg.

There are 2 previous case reports describing the use of the Scheker constrained distal radio-ulnar joint arthroplasty following excision of a GCT of the distal ulna. Pirela-Cruz et al²² noted some asymptomatic loosening of the ulnar component in a 21-year-old man, but the patient had returned to work with range of motion and strength only marginally less than the contralateral side. Gracia et al²³ reported a 56-year-old man who achieved a Mayo wrist score of 92 and an excellent Ferracini score of 18/18 with similar range of motion and strength to the contralateral side.

Conclusion

Our patient demonstrates excellent oncologic and functional outcomes so far, at 2 years postoperatively. There is no evidence of further recurrence of the tumor. He has returned to his previous employment, which involves heavy lifting, and has range of motion of his forearm and wrist exactly similar to his opposite side. However, longer follow-up studies will be required to assess the survival rates of this implant especially in younger patients.