Giant cell tumour of bone with late presentation - Semantic Scholar

Journal of Orthopaedic Surgery 2002: 10(2): 120–128

Giant cell tumour of bone with late presentation: review of treatment and outcome ES Ng, A Saw, S Sengupta Department of Orthopedic Surgery, University Malaya Medical Center, University of Malaya, Kuala Lumpur, Malaysia

AR Nazarina, M Path Department of Pathology, University Malaya Medical Center, University of Malaya, Kuala Lumpur, Malaysia

ABSTRACT Purpose. To review cases of giant cell tumour of bone or osteoclastoma managed at the University Malaya Medical Center, University of Malaya, Kuala Lumpur, from January 1990 to December 1999. Methods. Medical records of all patients with musculoskeletal tumours were reviewed. Demographic data, clinical presentation, surgical management, and clinical outcomes were reviewed retrospectively. Results. Most of the 31 patients who were treated for giant cell tumour of bone presented late on the basis of the duration of their symptoms and radiological features. Five of the patients had been referred for local recurrences. 26 patients were treated for primary tumours: 18 needed wide excision, 7 curettage, and one amputation. The joint could not be preserved and arthrodesis was performed for 11 patients. Three (12%) of the 26 patients had local recurrence during a mean follow-up of 60 months, including one (6%) who had recurrence after wide excision and 2 (29%) after curettage. Pulmonary metastasis was noted in 4 cases, 2 of which were confirmed histologically.

Conclusion. Even in an advanced stage of disease, good clinical outcomes can be achieved with adequate excision and appropriate reconstruction. For lesions around the knee, autologous rotational grafting is a good alternative method of reconstruction. Key words: bone tumor; giant cell tumor of bone; osteoclastoma

INTRODUCTION Giant cell tumour (GCT) of bone, or osteoclastoma, is classically described as a locally invasive tumour that occurs close to the joint of a mature bone. It is generally considered to be a benign tumour. The incidence of bone GCT also varies depending on the reporting centre. In an earlier study conducted in our institution, Peh et al.1 found that 28 (21%) out of 135 biopsies performed for bone tumours were GCT. In our experience, a substantial proportion of patients seek traditional means of treatment before medical consultation. Infrequently, they present late with extensive involvement of soft tissue and articular surface, making joint preservation difficult or

Address correspondence and reprint requests to: Dr A Saw, Department of Orthopedic Surgery, Faculty of Medicine, University of Malaya, 50603 Kuala Lumpur, Malaysia. E-mail: [email protected]

Vol. 10 No. 2, December 2002

Giant cell tumour of bone with late presentation: review of treatment and outcome 121

impossible. This study aimed to investigate the epidemiological data of bone GCT in the local Malaysian population, and to review the outcomes of treatment among these patients.

MATERIALS AND METHODS We reviewed medical records of all patients with musculoskeletal tumours who were treated at the University Malaya Medical Center, University of Malaya, Kuala Lumpur, from January 1990 to December 1999. Of a total of 224 cases, 141 were primary bone tumours; 34 of these cases were GCT of bone. Three of the cases of bone GCT were excluded from the study: one case was eventually managed in another hospital, while in 2 cases, patients refused surgery and were lost to follow-up. 31 patients underwent surgery and were followed up for a mean duration of 60 months (range, 20–121 months). 26 of these patients were treated for primary tumours, whereas the remaining 5 had been referred to us for local recurrences. Six patients with primary tumours had a biopsy performed before referral. All the tumour specimens from definitive surgeries were retrieved and reviewed again by one of the authors to confirm the diagnosis. We classified the surgical margin as one of 4 types according to Enneking et al. 2 : intralesional (curettage), marginal, wide, and radical (compartmental). Demographic data, clinical presentation, surgical management, and clinical outcome were reviewed retrospectively.

RESULTS Patient characteristics There were 20 (59%) female and 14 (41%) male patients. The mean age of all the patients was 30.2 years (range, 16–73 years), while 71% of them were aged between 16 and 35 years (Fig. 1). Approximately half (52%) of the patients were Chinese, 27% were Malay, and 21% were Indian. Site of lesion Eight (24%) tumours involved the upper limb, whereas 25 (74%) were in the lower limb (Fig. 2). 19 (56%) lesions occurred around the knee joint. There was only one (3%) axial lesion at the T10 vertebra. Three patients had multicentric lesions that involved 2 sides of a joint: one patient had lesions at the distal tibia and talus (Fig. 3), one had lesions at the femoral head and acetabulum, and one had lesions at the proximal tibia and fibula. Clinical presentation Most patients in our series presented late and at an advanced stage of disease, when preservation of articular cartilage was not possible. Their mean duration of symptoms was 8 months, while 54% of them presented at more than 6 months after the onset of symptoms. The chief symptoms at presentation among the 26 patients who were treated

Number of patients 9 8 7 6 5 4 3 2 1 0 50

Age (years)

Figure 1 Distribution of patients according to age and sex.

122 ES Ng et al.

Journal of Orthopaedic Surgery

Figure 2 Distribution of GCT of bone according to the site of tumour, indicating the number of cases of GCT in parts of the body.

(a)

(b)

primarily in our institution were pain (n⫽26), swelling (n⫽20), and pathological fracture (n⫽5). 13 (50%) patients had history of antecedent trauma. The mean duration of symptoms was 8 months (range, 7 days– 2 years). Management Tissue diagnosis was established for all patients before definitive surgery. Surgical procedures were selected according to tumour location, tumour size, and the

Figure 3 (a) and (b) X-ray of a 20-yearold Indian woman with right ankle pain and swelling, showing lytic lesions involving both the distal tibia and talus (case 9).

extent of the surrounding structures involved. Eight patients (7 with primary tumours) underwent intralesional curettage, 19 (18 primary) underwent wide excision with limb preservation, and 4 (one primary) required amputation of the affected limb (Table 1). Intralesional curettage was done by thorough curettage of the tumour through a wide cortical window. For most patients, an autologous bone graft was used to fill the cavity. Bone cement was used in one patient (case 1). Wide excision was performed



Table 1 Surgical procedure and recurrence in patients with primary GCT Treatment Curettage

Wide excision

Amputation

Total

No. of patients

7

18

1

26

Recurrence

2

1

0

3

6%

0%

Percentage recurrence

29%

12%

Table 2 Summary of management and outcome of patients with GCT of bone Case

Location

Margin of excision Curettage

Reconstruction

Local recurrence (months)

Pulmonary metastasis (months)

Duration of follow-up (months)

Current outcome

Bone cement

N

N

30

Disease-free

1

Femur proximal

2

Radius distal

Wide

Fibula graft

N

N

60

Disease-free

3

Radius distal

Wide

Fibula graft

N

N

96

Disease-free

4

Radius distal

Wide

Fibula graft

N

N

94

Disease-free

5

Humerus proximal

Wide

Fibula graft

N

N

60

Disease-free

6

Femur distal

Wide

Rotational tibial graft, knee arthrodesis

N

N

55

Disease-free

7

Femur distal

Wide

Allograft, knee arthrodesis

N

N

24

Disease-free

8

Femur, acetabulum

Wide

Hip arthrodesis

N

N

120

Disease free

9

Tibia distal, talus

Wide

Ankle arthrodesis

N

N

21

Disease free

10

Tarsal

Amputation

Below-knee amputation

N

Y, 5

—

Died

11

Humerus

Amputation

Forequarter amputation

N

N

20

Disease-free

12

Radius distal

Amputation

Below-elbow amputation

N

Y, 5

33

Alive with disease

13

Femur distal

Amputation

High transfemoral amputation

N

N

39

Disease-free

14

T10

Y, 6

N

86

Disease-free

Curettage

Fusion with rib graft

124 ES Ng et al.

Journal of Orthopaedic Surgery Table 2

Case

Location

Margin of excision

15

Femur distal

Curettage

16

Tibia proximal

17

Reconstruction

Continued Local recurrence (months)

Pulmonary metastasis (months)

Duration of follow-up (months)

Current outcome

Bone graft

Y, 4.5

Y, 0

91

Disease-free

Wide

Allograft

Y, 42

N

42

Disease-free

Tibia proximal

Wide

Rotational femoral graft, knee arthrodesis

N

N

84

Disease-free

18

Femur distal

Wide

Bone graft, K wire

N

N

61

Disease-free

19

Femur distal

Wide

Bone graft, DCS*

N

Y, 22

39

Disease-free

20

Ulna distal

Wide

None

N

N

120

Disease-free

21

Fibula proximal

Wide

None

N

N

58

Disease-free

22

Tibia proximal

Wide


N

N

42

Disease-free

23

Tibia proximal

Wide


N

N

121

Disease-free

24

Tibia proximal

Wide


N

N

26

Disease-free

25

Tibia proximal

Wide

Bone graft, knee arthrodesis

N

N

89

Disease-free

26

Tibia proximal

Wide


N

N

44

Disease-free

27

Talus

Curettage

None

N

N

24

Disease-free

28

Femur distal

Curettage

Bone graft

N

N

85

Disease-free

29

Femur distal

Curettage

Bone graft

N

N

24

Disease-free

30

Tibia proximal

Curettage

Bone graft

N

N

20

Disease free

31

Humerus proximal

Curettage

Bone graft

N

N

82

Disease-free

* DCS: dynamic condylar screw fixation

on 19 patients. The method of reconstruction depended on the size and site of the lesion (Table 2). Autologous non-vascularised fibula graft was used for the reconstruction of 3 distal radial tumours (cases 2, 3, and 4) and one proximal humeral tumour (case 5).

Five proximal tibia lesions were reconstructed with rotational grafting (Fig. 4), while 4 distal femur lesions were reconstructed with iliac bone grafting in 2, rotational grafting in one (case 6), and structural allograft in one (case 7). In the case managed by rotational grafting, the uninvolved distal femur or



(a)

(b)

(c)

Figure 4 Case 17 (a) lytic lesion over subchondral region of proximal tibia; (b) after wide excision and rotational femoral autograft, the knee was fused using long plate; (c) the knee was strongly fused and the plate was removed one year later.

proximal tibia was split and rotated with attached condylar soft tissue, and collateral ligaments to bridge the defects after wide excision; the knee was then fused with a long plate or intramedullary nail. Additional cancellous iliac bone graft was deposited around the bone ends. The 2 patients with adjacent bone involvement across the hip and ankle joint (cases 8 and 9) were treated by wide excision followed by hip and ankle fusion. Four patients required limb amputation for extensive local disease (cases 10–13). One of them had a lesion at distal femur with extensive soft tissue involvement, one had local recurrence at the tarsal bones after intralesional curettage had been performed in another hospital, and one had a tumour in the proximal humerus that was treated with wide excision and autologous non-vascularised fibula graft at another hospital. In case 11, local recurrence after 5 years involved the graft and extended into the adjacent scapula (Fig. 5); in this case, forequarter amputation was performed. In case 12, a distal radial tumour had been treated by wide excision followed by bone transport using an external fixator; the patient was referred to us 5 months later with lung metastasis and local recurrence ulcerating through the skin, for which a below-elbow amputation was performed.

Figure 5 X-ray of a 40-year-old Chinese man with a tumour in the proximal humerus managed by wide excision and autologous non-vascularised fibula graft but resulting in local recurrence: destructive lesions involved the graft extending into the adjacent scapula (case 11).

126 ES Ng et al.


Outcome Three (12%) of the 26 patients with primary tumours developed local recurrence during a mean follow-up of 60 months (Table 2). One patient (case 14) had GCT in the T10 vertebral body, which was managed by marginal excision and rib grafting; local recurrence developed 6 months later, which was treated by further excision, bone grafting, and posterior instrumentation. Five months after the second surgery, the tumour recurred; another excision and bone graft was performed, and the patient remained disease-free 4.5 years after the last surgery. Another patient (case 15) had been referred to us with GCT at the distal femur with single lung metastasis; the primary lesion was managed by curettage and the lung nodule was excised by thoracotomy. Both lesions were confirmed to be GCT, but 4.5 months later, the patient again developed knee swelling that was confirmed by biopsy to be GCT; chest radiography showed multiple lung lesions. The patient was treated with a course of local radiotherapy and systemic chemotherapy, after which both distal femur and lung lesions resolved; the patient was clinically and radiologically free of disease 7 years after treatment. The third patient (case 16) had wide excision of GCT at the proximal tibia followed by allograft reconstruction, but the tumour recurred at the proximal fibula 42 months after surgery. Three months before this study, wide excision of fibula with preservation of allograft was performed. Pulmonary metastasis Pulmonary metastasis developed in 4 patients (Table 2): one presented with a lesion in the lung, whereas 3 developed lung nodules after surgical treatment. Two of these patients underwent excision of pulmonary nodules and had histological confirmation of GCT; both remained disease-free after surgery. Diagnosis in the other 2 patients were based on clinical and radiological evidence: one subsequently died of respiratory disease while the other developed recurrent distal radial tumour and haemoptysis (Fig. 6), for whom below-elbow amputation was performed. On last review, 33 months after presentation, this patient was well and had only occasional bouts of dry cough, although X-ray findings of the lungs remained unchanged. Complications There were 19 complications in 12 patients. Two patients had postoperative wound breakdown that healed after secondary suturing. Four patients

Figure 6 Computed tomogram of a 23-year-old Chinese man who presented with recurrent GCT at the distal radius, showing multiple bilateral lung nodules (case 12).

developed deep infection: one had a tumour of the distal radius, which was reconstructed with a fibula graft, and 3 had tumours of proximal tibia, and were treated with knee fusion and plate fixation. Three of the 4 patients responded to debridement and intravenous antibiotic, but the fourth developed chronic osteomyelitis of the upper tibia with persistent sinus discharge. Common peroneal nerve palsy occurred postoperatively in 4 patients: 3 underwent wide excision for tumours around the knee, and one developed footdrop after harvesting of fibula graft. Three of these 4 patients recovered spontaneously. Non-union with implant failure occurred in 6 patients (one with a broken K nail, 2 with broken plates, 2 with implant loosening for knee fusion, and one with broken screws for hip fusion). Another patient had a fracture proximal to the fused knee following a fall. All these complications occurred in patients with wide excision.

DISCUSSION Bone GCT is not a common tumour. It makes up 5% to 7% of all primary bone tumours in several major western series.3–5 34 (24%) of 141 primary bone tumours treated in our institution were GCT. Similar proportions of bone tumours were identified to be GCT in 2 separate studies in our country (21% and 28%).1,6 Likewise, another study conducted in Hong Kong and China showed that GCT of bone contributed 15% to 20% of all primary bone tumours. 7 The higher percentage of GCT in bone tumours in Asian



populations is clear. There were more females (59%) than males (41%), which is in agreement with western series4,8 although Campanacci et al.3 and Larsson et al.9 could not demonstrate any sex predilection in their series. The majority of cases described occurred between second to fourth decades,3–5,7–9 and 59% of the patients in our series were aged between 20 and 40 years. As in most series,3–5,7–9 the most common sites were proximal tibia and distal femur, followed by distal radius. More than half (56%) of the patients in our series had lesions around the knee. Lesions at the spine are not common, and multicentric GCT is also rare (occurrence, ⬍1%).3,8–10 Cummins et al.10 reviewed 29 cases of multicentric GCT in the literature and observed that they were more common in atypical sites, and among younger patients. About 80% of patients published were younger than 25 years. Two of the 3 patients with multicentric lesions were younger than 25 years. Most patients in our series presented late, and nearly two-thirds (64%) had a grade III tumour according to radiological grading by Campanacci.3 This proportion is higher than that found in other series of 27% to 50%.3,5 Moreover, 4 cases either presented with or eventually developed lung metastasis, which may reflect delay in presentation. One in 5 admitted that they had visited a traditional healer before medical consultation, which may have contributed to the delay. The local recurrence rate after surgery reported in the literature ranges from 15% to 26%.3,5,7 Many authors have tried to determine the factors that predict the risk of local recurrence. Histological grading by Jaffe et al.11 and radiological grading by Campanacci3 have been shown to be unreliable.4,8,9 The most important factor that predicts prognosis is the adequacy of tumour resection. It is generally accepted that recurrence rate

after intralesional curettage is higher (27%–41%) than that after wide excision (0%–7%).3,5,7 Most of our cases had soft tissue extension and articular surface involvement that rendered joint preservation impossible. This accounted for more wide excision procedures (n⫽19; 61%) being performed compared with intralesional curettage (n⫽8; 26%). Nearly half (n⫽9; 47%) of the wide excisions (n⫽19) were followed by arthrodesis. Three (12%) of 26 patients developed local recurrence after a mean follow-up of 17.5 months. There was only one (6%) local recurrence among 18 patients who underwent wide excision, whereas 2 (29%) of the 7 patients who had intralesional curettage developed local recurrence. These results showed that in spite of their more advanced stage of disease, the overall clinical outcomes of our series are comparable to those of other series. Pulmonary metastasis of benign GCT is very rare. So far, 69 cases have been reported in the literature. Most cases were single case reports and only a few larger series have been reported recently.4,12–19 Four (13%) of the 31 patients in our series developed lung lesions, including 2 whose diagnosis were confirmed histologically. There has been no standard treatment for GCT metastasis described in the literature and the results of those described are variable. One of the patients in our series who had multiple pulmonary metastasis was still alive 2.5 years after the diagnosis was made. Nodule excision has been reported to yield good results,4,12,14,15,18 and the role of chemotherapy is still debatable.20 In conclusion, even in an advanced stage of disease, good clinical outcomes can be achieved with adequate excision and appropriate reconstruction. For lesions around the knee, autologous rotational grafting is a good alternative method of reconstruction.

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