Aneurysmal bone cyst and giant cell tumor of bone of the hand and distal radius

Aneurysmal bone cyst and giant cell tumor of bone of the hand and distal radius

Hand Clin 20 (2004) 269–281 Aneurysmal bone cyst and giant cell tumor of bone of the hand and distal radius Edward A. Athanasian, MD Hospital for Spe...

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Hand Clin 20 (2004) 269–281

Aneurysmal bone cyst and giant cell tumor of bone of the hand and distal radius Edward A. Athanasian, MD Hospital for Special Surgery, 535 East 70th Street, New York, New York 10021, USA

Aneurysmal bone cyst accounts for approximately 5% to 6% of benign bone tumors [1] and may be seen as a primary or secondary lesion in association with another neoplasm, such as a giant cell tumor of bone or chondroblastoma. Fewer than 5% of aneurysmal bone cysts in general case series are localized to the hand (Fig. 1) [2,3]. Most publications about aneurysmal bone cyst in the hand are limited to isolated case reports or a small number of cases collected over a long period of time [2–11]. The behavior of lesions in the hand, therefore, must be largely inferred from these limited reports and general case series [12–17]. Aneurysmal bone cyst most commonly presents in the second decade of life and is uncommon in patients over the age of 40. Most studies suggest an equal frequency in males and females, although some authors have suggested the lesion has a female preponderance [1,15–17]. Metacarpal lesions are more frequent than phalanx or carpal lesions. The clinical presentation may be a slowly enlarging firm mass, which may or may not be painful. When the lesion occurs in the distal phalanx, significant bone destruction and pain may be seen. Occasionally a patient may present with a pathologic fracture. Clinically there may be extensive swelling and warmth. When the lesion is close to a joint there may be limitation in range of motion. Plain radiographs of the hand will often demonstrate a central, expansile metaphyseal or epiphyseal lesion that thins the cortex. A reactive sclerotic margin may or may not be seen (Fig. 2A). Matrix trabeculation may be seen, which may be mixed or purely lytic. Lesions in the hand may be purely lytic, lack a sclerotic margin, E-mail address: [email protected]

and appear more destructive, much like giant cell tumor of bone (Fig. 3A). Differential diagnosis based on plain radiographs alone will often include giant cell tumor of bone, giant cell reparative granuloma, and enchondroma. Magnetic resonance imaging (MRI) may be particularly useful in narrowing the differential diagnosis if fluid–fluid levels are detected (see Figs. 2B and 3B), and occasionally soft tissue extension may be seen. Although aneurysmal bone cyst can be locally aggressive and destructive, it is not known to have metastatic potential. Radiographically and histologically, the lesion may be confused with telangiectatic osteogenic sarcoma [1]. The natural history of aneurysmal bone cyst is one of progressive enlargement and bone destruction. Treatment is directed at controlling the lesion locally. Previous large general case series report rates of recurrence after curettage and bone grafting alone, which approximates 60% [16]. More recent studies suggest the risk of local recurrence can be reduced with the use of an adjuvant such as liquid nitrogen, cementation, or high-speed burring. Marcove et al [16] reduced the risk of local recurrence after curettage to 18% when liquid nitrogen was instilled into the curetted cavity, which was then packed with bone graft or cement. Ultimately, 96% of lesions could be controlled with a second treatment. Schreuder et al [18] noted only one local recurrence after curettage, cryosurgery, and bone grafting in 26 lesions treated. Gibbs et al [15] retrospectively reviewed 40 patients treated for aneurysmal bone cyst. Local recurrence was seen in only 4 of 34 patients (12%) treated with high-speed burring after curettage. They attributed the low risk of recurrence

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Bone graft healing after cryosurgery is impaired, which may increase the risk of fracture or joint collapse. Packing cement in the cavity of the treated lesion may reduce this risk, although it remains to be seen how well cement will be tolerated in the long term in the hand. This technique might be best indicated for recurrent lesions or those in which recurrence would increase the need for wide excision or amputation. Some authors have advocated wide en bloc excision and bone grafting as treatments for hand lesions, particularly those occurring in the metacarpals [4,5]. This technique carries a lower local recurrence risk than curettage and bone grafting but may have greater morbidity. Amputation may be necessary for very large destructive lesions, particularly when they occur in the distal phalanx. Fig. 1. Aneurysmal bone cyst of the proximal phalanx of the hand.

Author’s preferred treatment to this technique [15], and all recurrences were seen in skeletally immature girls. They conclude that young age and open physes are associated with an increased incidence of local recurrence [15]. The limited published data regarding aneurysmal bone cyst in the hand after intralesional procedures suggests that local recurrence risk is high [2,3]. Most recurrences occur within 2 years of primary treatment [16]. Many lesions can be successfully treated with additional intralesional procedures (see Fig. 2C). Although data suggest a decreased risk of local recurrence when a highspeed burr is used to abrade the endosteal cavity, there may be insufficient bone stock in hand lesions to allow the use of this technique on a routine basis. There is also technical difficulty in using power tools aggressively with accuracy in the small bones of the hand. There may be a greater risk of soft tissue or joint penetration. Creation of a large cortical window or ‘‘exteriorization’’ has been advocated as a means of improving visualization of the lesion and facilitating treatment of the lesion cavity. The use of liquid nitrogen in the hand may reduce the risk of recurrence but is technically demanding and may be associated with increased risks of infection, fracture, and joint collapse (see Fig. 3C) [2,17]. Limited data regarding cryosurgery in the distal radius and hand, however, suggest it may be effective for both aneurysmal bone cyst and giant cell tumor of bone [2,18,19,20]. Premature physeal closure may result from thermal injury during cryosurgery when lesions abut the physis [21].

I prefer curettage, endosteal burring, and bone grafting, provided there is sufficient bone stock. These procedures are performed with the knowledge that there is a significant risk of local recurrence. The high risk of local recurrence and the possible need for multiple procedures are carefully explained to the patient or parent. If local recurrence could result in the loss of a joint or amputation, I will consider the use of curettage, cryosurgery using liquid nitrogen, and bone grafting or cementation. I make every effort to avoid the use of liquid nitrogen adjacent to open physes because of the risk of premature physeal closure and growth disturbance. Small local recurrences can be treated with additional curettage procedures or with the use of liquid nitrogen after curettage if the lesion appears particularly aggressive. I strongly consider the use of cement as packing material if there is a substantial risk of fracture or subchondral collapse after the application of liquid nitrogen to the endosteal cavity. Wide en bloc excision is reserved for very aggressive lesions with inadequate bone stock for an intralesional procedure.

Giant cell tumor of bone Giant cell tumor of bone is most commonly seen in the distal femur or proximal tibia [1]. The distal radius is the third most common site of tumor presentation. Only 2% of tumors arise in the hand. Giant cell tumor of bone occurs most commonly in the fourth decade and is slightly

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Fig. 2. (A) Aneurysmal bone cyst of the distal radius. The lesion expands the radius. A sclerotic margin with a narrow zone of transition is seen. (B) Axial MR image demonstrating fluid–fluid levels characteristic of aneurysmal bone cyst. (C) Remodeling of distal radius after repeated curettage and cancellous allograft placement following one local recurrence.

more common in women [19]. Although giant cell tumor of bone is considered to be benign, based on histology, it does have the potential to metastasize and result in death [20–23]. Patients commonly present with pain or swelling of the affected area and may also present after pathologic fracture. Radiographs typically demonstrate an eccentric epiphyseal lesion with a lytic matrix. The limited volumes of the bones of the hand may result in a more central appearance because of extensive involvement by the time the patient presents (Fig. 4). The matrix typically has no calcification, but pseudotrabeculation may be

seen. Cortical expansion, perforation, and soft tissue extension are commonly seen on plain radiographs and are often better defined on MRI. Campanacci et al [9,21,24] describe a staging system based on plain radiographs that is commonly used to assess giant cell tumor of bone. Lesions which do not distort or perforate the cortex are considered stage I lesions (Fig. 5). Stage II lesions distort or expand the cortex but do not extend into the surrounding soft tissues. Stage III lesions perforate the cortex and extend into the surrounding soft tissues. Patients presenting with giant cell tumor of bone should be staged with

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Fig. 3. (A) Anteroposterior radiograph of a distal metacarpal lesion initially interpreted as giant cell tumor of bone. (B) Axial MR image demonstrating fluid–fluid levels seen with local recurrence after curettage and bone grafting. (C) Longterm follow-up anteroposterior radiograph demonstrating articular collapse after curettage, cryosurgery, and bone grafting. The patient had severe restriction of metacarpal phalangeal range of motion.

computed tomography of the chest and total body scintigraphy because of the potential for multifocal lesions and metastasis [25]. Current treatment in general case series is intralesional, provided there is sufficient remaining bone stock and limited cortical perforation [26–29]. A primary goal in the treatment of giant cell tumors of the distal radius is preservation of the articular surface. Wide excision has been reserved for very extensive tumors or those with pathologic fracture. Most stage I and II lesions in long bones can be treated with curettage and adjuvant therapy. O’Donnell et al [30] reviewed 225 patients treated at multiple centers and found a general recurrence rate of 25% after curettage, application of phenol, and cementation. More recently, Blackley et al [31] found a local

recurrence rate of 12% in 59 patients after curettage, burring of the endosteal cavity with a high-speed burr, and packing with bone graft. Nearly 50% of patients treated had stage III disease. The authors suggest that adequacy of mechanical removal of the tumor rather than the use of adjuvants likely determines the risk of local recurrence after curettage procedures [31]. A multicenter retrospective study by Malawer et al [32] assessed 102 patients treated with curettage, burring, and cryosurgery using liquid nitrogen with a direct pour technique. Local recurrence was seen in 2.3% of patients treated for primary tumors. Complications include pathologic fracture, partial skin necrosis, and joint degeneration. Excellent function was seen in 92.2% of patients treated with this technique.

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Fig. 4. (A) Radiograph of a thumb metacarpal showing giant cell tumor of bone. (B) Wide en bloc resection of the metacarpal is reconstructed with a large iliac crest bone graft, metacarpal phalangeal arthrodesis, and ligament reconstruction tendon interposition at the carpal metacarpal joint.

Several authors have reported lung metastases from benign giant cell tumor of bone in the distal radius and hand [32–38]. Maloney et al [22] noted a mean time to metastasis of 3.2 years, with metastasis occurring as late as 10 years after initial treatment. The radius accounted for more than one quarter of sites with metastases, even though only 10% of giant cell tumor cases occur in the distal radius. The reason for this disproportionate metastatic rate is not clear. Tumors in

this location have been postulated to be more aggressive. The risk of metastasis may be related to repeated attempts at local control after initial treatment failure. Bertoni et al [20] noted only one of seven patients with metastasis who presented at initial diagnosis. Patients treated for giant cell tumor of the distal radius and hand must be followed for both local and systemic recurrence. Surveillance chest radiographs at regular intervals are essential, although the ideal interval and

Fig. 5. Campanacci staging system for giant cell tumor of bone. In stage I there is no distention or perforation of the cortex. In stage II there is distention of the cortex but no soft tissue perforation. In stage III there is cortical destruction with extension into the surrounding soft tissues. Most commonly with giant cell tumor of bone in the distal radius, the destruction and extension are seen from a palmar view, deep to the pronator quadratus.

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duration of surveillance are not known. Computerized chest tomography should be considered in those patients who develop local recurrence. Distal radius tumors Giant cell tumor of bone occurring in the distal radius may yield insight into the biologic behavior of this disease. Tumors at this site are known to have particularly high rates of local recurrence whether treatment is performed with curettage or wide en bloc excision [36,37,39–41]. Reconstruction of the defect produced by excision of the distal radius is difficult and is frequently associated with complications. The numerous methods of reconstruction that have been described and reported reflect this difficulty [36,38,39,42–62]. Tumors occurring in the distal radius are more likely to metastasize than those occurring in other long bones [22,23]. From a functional standpoint, the ideal treatment for giant cell tumors of the distal radius would allow preservation of pain-free wrist motion and a durable result while exposing the patient to low morbidity. For these reasons, curettage and bone grafting are appealing; unfortunately curettage or curettage and bone grafting has resulted in local recurrence rates of 50% or more in some studies [37,40]. Although the use of adjuvant therapies such as power burring, pulsatile irrigation, phenolization, and packing with methyl methacrylate have decreased rates of local recurrence and improved functional results in periarticular tumors occurring in large long bones, similar improvements in the distal radius have not been reliably attained [30]. O’Donnell et al [30] reviewed the results of treatment of giant cell tumor of bone after curettage, phenol instillation, and packing with methyl methacrylate in 225 patients and note a combined local recurrence rate of 25%. When results in the distal radius were specifically assessed, the rate of local recurrence was 50%, suggesting either inadequate mechanical removal of tumor tissue or a lack of benefit from adjuvant therapy [30]. Vander Griend and Funderburk [38], however, note greater success when these techniques were used in five patients without soft tissue extension (Campanacci stage I and II tumors), finding no local recurrences. Sheth et al [33] report a local recurrence rate of 25% in a review of 18 patients who were treated with curettage, cryosurgery, and bone grafting or packing with methyl methacrylate. The technique involved a direct pour of

liquid nitrogen through a funnel into the curetted tumor cavity (Fig. 6). Their results are noteworthy because of the advanced stage of presentation and the presence of soft tissue extension in several patients [33]. Although they achieved superior local control with these techniques in radiographically advanced lesions, the authors note difficulty with the reconstruction of these lesions because of poor native bone quality [33]. Some authors have suggested that the heat produced by the packing of the bone cavity with methyl methacrylate may extend the zone of curettage, increase tumor kill, and thereby reduce the risk of local recurrence [30,38]. Capanna et al [63], however, note similar rates of local recurrence whether the bone cavity was packed with cement or bone graft. Perhaps the most significant benefit of the use of methyl methacrylate is that it allows excellent contrast with surrounding bone, thereby facilitating detection of local recurrence; it also provides immediate structural stability in periarticular regions [30,64]. Wide en bloc excision of giant cell tumors of the distal radius is appealing because complete removal of a tumor with wide margins should result in reduced rates of local recurrence. Although rates of local recurrence after en bloc excision are generally lower than intralesional procedures, the chance of local recurrence remains [33,36–38,41,65]. Cortical penetration, soft tissue seeding, or intra-articular contamination may predispose excisional procedures to failure unless these areas are completely excised with the major tumor mass. This could require excision of the entire proximal carpal row for lesions penetrating the radiocarpal joint. Excision of surrounding soft tissues en bloc with the tumor mass can result in additional functional impairment of the hand (Fig. 7). Compromising the excision margin to spare functional soft tissues also can result in local recurrence. Accurate assessment of the extent of medullary involvement is essential when planning the site of proximal osteotomy. MRI of the radius is critical in delineating the extent of bone and soft tissue involvement. Metachronous lesions (or skip lesions) can also be identified with MRI. When local recurrence occurs after en bloc excision, these lesions may be seen either in the soft tissues or in the bone graft used for reconstruction [36,41,65]. Planning for a biopsy of distal radius tumors must incorporate a thorough understanding of the distal radius surface anatomy and consideration

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Fig. 6. (A) Radiograph of giant cell tumor, Campanacci stage III, of the distal radius. (B) Axial MRI demonstrating palmar soft tissue extension bound by the pronator quadratus. (C) After curettage using a palmar radial approach, liquid nitrogen is poured into the tumor cavity through a funnel. Three separate freeze-thaw cycles may be performed. (D) The dried cavity is then packed with methylmethacrylate. (E) Postoperative wrist extension and (F) flexion.

of the subsequent plan for definitive treatment. There are no well-defined guidelines for the biopsy of distal radius tumors. Dorsal biopsy runs a significant risk of tendon contamination and spread of hematoma. Biopsy at Lister’s tubercle facilitates a subsequent dorsal approach, but the window of entry is often less than 5 mm between

the extensor pollicis longus and extensor carpi radialis brevis (see Fig. 7C). Visualization of the palmer structures is not possible unless the radius and ulna are transected and maximally flexed or a second palmer incision is made to better visualize palmer structures (see Fig. 7D). The dorsal approach may not allow adequate

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Fig. 7. (A) Radiograph of giant cell tumor, Campanacci stage III, of the distal radius. (B) Axial MRI demonstrates extension to the radiocarpal articulation. (C) Extra-articular wide excision of the distal radius and ulna, including the proximal carpal row, performed from a dorsal approach. The radius and ulna are transected proximally after completion of dorsal dissection. (D) The distal radius, ulna, and carpus are flexed to facilitate palmer dissection. (E) The carpus is transected, and the intervening defect is filled with a vascularized fibular osteoseptocutaneous bone graft. Internal fixation spans the length of the graft, with segmental arthrodesis from the radius to the third metacarpal. The patient experienced a postoperative metacarpal fracture.

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visualization and excision of palmer perforation if an intralesional (curettage) procedure is being considered. Biopsy at the volar radial aspect of the distal radius at the radial-most origin of the pronator quadratus facilitates exposure of the volar radius and any soft tissue extension into the pronator quadratus that may be present (see Fig. 6D). The surface interval is between the first dorsal compartment and the radial artery. A branch of the superficial radial nerve will usually be seen during the approach. This approach allows excellent exposure of the volar distal radius without potential contamination of the finger flexors and the median nerve. This approach is best suited to curettage procedures. The approach is not easily extended distally and is not ideal for extra-articular wide excision, should this approach be needed. Intercalary wrist arthrodesis after tumor resection can be performed with grafts from the tibia, fibula, or iliac crest. The precise indications for the use of vascularized bone grafts in this setting are not clear but may be related to the length of the defect spanned. Rigid fixation, particularly at the proximal osteosynthesis site, appears to decrease the possibility of nonunion, although the presence of a dorsal plate may interfere with tendon function and result in attritional tendon rupture [38]. Articular fibula autograft reconstruction of the distal radius has been attempted to reconstruct the wrist after wide en bloc excision of the distal radius, including the articular surface. Long-term follow-up suggests that this procedure can retain a flexion–extension arc of approximately 60( [47,49,52]. Complications include donor morbidity, instability, and articular degeneration, and are frequent, and patients may experience pain with functional activities. It is likely that the remaining motion is occurring largely at the midcarpal articulation. Osteoarticular distal radius allograft reconstruction has been suggested as a means of maximizing functional outcome after distal tumor radius resection. The theoretic advantages are anatomic reconstruction and maintenance of wrist motion. Long-term follow-up of this method of reconstruction has been shown to have significant risks of complication and failure [66]. Kocher et al [66] report on the Massachusetts General Hospital experience with 24 distal radius reconstructions using a size-matched osteoarticular radius allograft. Eight patients (33%) failed and required conversion to an arthrodesis or amputation.

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Complications included fracture, volar dislocation, ulnocarpal impaction, painful hardware, and extensor tendon rupture. Of the 16 surviving allografts, 13 patients experienced pain with either moderate or strenuous activities. The flexion– extension arc was 57( at final follow-up. The notable risk of complication and failure with distal radius osteoarticular allografts suggest that this form of reconstruction be reserved for those patients with relatively low functional demand who prioritize or require retention of wrist motion. I treat Campanacci stage I and II lesions with curettage, burring, cryosurgery, pulsatile irrigation, and cementation, as advocated by Sheth et al [33]. Stage III lesions with adequate bone stock in which there is a single site of cortical perforation are also treated with curettage if the site of penetration can be excised en bloc when creating a cortical window to expose the lesion (see Fig. 6). This type of limited soft tissue extension may be seen in a palmar view where the pronator quadratus acts as a barrier to further spread. Stage III lesions with extensive soft tissue contamination, radiocarpal joint contamination, or inadequate bone stock are treated with wide en bloc excision and reconstruction, usually arthrodesis (see Fig. 7). Patients presenting with intra-articular pathologic fracture are most commonly treated with wide en bloc excision. Hand lesions Giant cell tumor of bone is particularly difficult to treat when it occurs in the hand [2,7,34,35,67–81]. Local recurrence rates after intralesional procedures in the wrist and hand are known to be particularly high compared with lesions elsewhere in the body [34,35,78]. Lesions in the hand may have a risk of metastasis as high as 10% [34,35]. Although patients may present with metastasis at initial presentation, more commonly metastasis is seen after local recurrence. The combination of relatively high rates of local recurrence and the possibility of metastasis after local recurrence have lead many physicians to recommend more frequent use of amputation or wide excision for lesions occurring in the hand. Data regarding giant cell tumor of bone in the hand are limited and likely subject to referral bias [34,35]. Averill et al [35] published the Massachusetts General Hospital experience based on only three patients seen before treatment. The remaining cases were seen after local recurrence (12

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Fig. 8. (A) Anteroposterior radiograph and axial MRI (B) of a giant cell tumor of the proximal phalanx.

patients) or taken from consultation files (7 patients). These authors reported local recurrence in 13 of 15 patients after curettage and bone grafting, and they recommend wide excision or amputation. It is likely that the recurrence risk is overstated because of referral bias. Multicentric tumors were seen in 18% of patients. Athanasian et al [2] published the Mayo Clinic experience with giant cell tumor of bone in the hand in patients seen over a 50-year period. All patients were seen at the clinic, but several patients were referred after local recurrence. Interestingly, the local recurrence risk after intralesional procedures (curettage) was approximately 80% [34]. Two patients developed metastasis concurrent with local recurrence. No multicentric tumors were seen. The findings lead to the recommendation of wide excision or amputation for patients with giant cell tumor of the bones of the hand (see Fig. 6A–C). A recent report [68] of three patients treated with curettage, cryosurgery, and cementation had no recurrence. There are additional case reports in the hand suggesting this may be an effective technique, however data are limited [67]. There are data to support the effectiveness of cryosurgery in treating distal radius lesions; however, no randomized studies have been performed [33]. This technique is likely best applied to those patients with limited or no cortical perforation of the bones of the hand (Campanacci stage I or II lesions) by surgeons experienced with cryosurgery. The risks of stiffness, infection, neurapraxia, joint collapse, and the uncertain risk of local recurrence

must be balanced against the morbidity of wide excision or amputation when considering use of this technique (Fig. 8).

Summary Giant cell tumor of bone and aneurysmal bone cyst can have very similar clinical, radiographic, and histologic presentations. Patients with giant cell tumor of bone should be systemically staged and followed long term. It is imperative to appreciate that giant cell tumor of bone can behave in a low-grade malignant fashion, with local recurrence and metastasis. The recognition that systemic metastasis usually occurs concurrent with local recurrence has resulted in more aggressive local treatment of primary lesions. The high risk of local recurrence for lesions in the hand has resulted in the recommendation for wide en bloc excision or amputation as the best form of definitive local treatment. Recent experience with curettage, cryosurgery, and cementation suggest that this technique may have a role for hand lesions without soft tissue extension. Aneurysmal bone cyst, although locally aggressive, does not have potential for malignant degeneration or metastasis. Although local recurrence after curettage is frequent, repeated intralesional treatments may be successful and leave a superior functional result compared with wide excision or more aggressive procedures. Cryosurgery also may have a role in the treatment of recurrent aneurysmal bone cyst.

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