Aneurysmal bone cyst is a rare, rapidly growing, and destructive benign bone tumor that even more rarely involves the bones of the hand. Various treatment options for aneurysmal bone cyst have been reported in the literature, but controversy exists regarding optimal treatment. Due to its rarity in the hand, no evidence-based treatment regimen has been established.
A 21-year-old man presented with a history of pain and local swelling over his fifth metacarpal of 5 months’ duration. Physical and radiographic examination of the hand was consistent with aneurysmal bone cyst. After biopsy, pathologic examination confirmed the diagnosis of aneurysmal bone cyst. En-block resection of the tumor and autologous bicortical strut graft fixation with Kirschner wires was performed. The hand was immobilized in a short arm cast for 3 weeks after the patient received 3 weeks of physiotherapy. Kirschner wires were removed 6 weeks postoperatively. Excellent clinical and functional results were obtained with no recurrence after 3 years of follow-up with en-block resection and reconstruction with iliac crest graft. Radiographic examination demonstrated the osseous integration of the graft with no signs of recurrence.
Although treatment should be planned individually according to lesion site and size and to patient age, we suggest en-block resection to prevent recurrence and secondary surgical interventions particularly in cases with no articular involvement.
Aneurysmal bone cyst is a rare, rapidly growing, and destructive benign bone tumor. Jaffe and Lichtenstein1 described aneurysmal bone cyst in 1942 as a distinct pathological entity by clearly separating it from hemangiomas of the bone and from other tumors in which giant cells were also a prominent feature. It is a rare tumor and accounts for 1% to 2% of all primary bone tumors.2 Aneurysmal bone cysts usually occur in the first 2 decades of life and exhibit a slight female preponderance.2,3 Aneurysmal bone cyst shows an evident predilection for long bones and the vertebral column, particularly the femur, humerus, tibia, and fibula. However, aneurysmal bone cysts arising from long bones of the hand occur rarely. Less than 5% of all aneurysmal bone cysts involve long bones of the hand.4
The pathogenesis of aneurysmal bone cyst is obscure. Lichtenstein5 suggested that persistent local disturbance in hemodynamics (venous thromboses or arteriovenous aneurysm) causes marked increase in venous pressure and leads to development of a dilated engorged vascular bed. Some authors proposed that aneurysmal bone cysts arise on a preexisting bone lesion as a secondary reaction.6,7 Trauma has been implicated as an initiative factor due to the fact that aneurysmal bone cyst is preceded by trauma with fracture or subperiosteal hematoma in some cases.8 However, most authors agree that trauma draws attention to a preexisting lesion.9,10 Recently, the genetic basis of aneurysmal bone cyst has been investigated, and specific chromosomal translocations have been reported.2,11,12 Furthermore, overexpression of insulin-like growth factor 1 is postulated to play a role in the pathogenesis.2,13
The natural history of aneurysmal bone cyst has been described as evolving through 4 radiologic stages: initial, active, stabilization, and healing.14 In the initial phase, the lesion is characterized by a well-defined area of osteolysis with discrete elevation of the periosteum. This is followed by a growth phase, in which the lesion grows rapidly with progressive destruction of bone and development of the characteristic blown-out radiologic appearance. The growth phase is succeeded by a period of stabilization, in which the characteristic soap bubble appearance develops as a result of maturation of the bony shell. Diagnosis generally occurs during the active or stabilization phase. Final healing results in progressive calcification and ossification, with the lesion transformed into a dense bony mass.
Histologically, aneurysmal bone cyst is composed of cavernous or slit-like hemorrhagic spaces surrounded and traversed by fibrous septa containing spindled cells, inflammatory cells, and a lesser number of osteoclast-like multinucleated giant cells that are often distributed around the hemorrhagic, cystic spaces. Typically, osteoid formation with or without osteoblastic rimming is observed.2,15
Various options for the treatment of aneurysmal bone cyst have been reported in the literature,16 but controversy exists regarding optimal treatment. Due to its rarity in the hand, there is no established evidence-based treatment regimen. This article presents a case of aneurysmal bone cyst affecting the fifth metacarpal that was treated by en-block resection and reconstruction with bicortical iliac crest graft.
A 21-year-old man presented with a history of pain and local swelling over his fifth metacarpal of 5 months’ duration. On physical examination, the lesion was firm and immobile and there was slight tenderness with palpation. Active range of motion of his fifth metacarpophalangeal joint was slightly restricted, and pain was aggravated with movement. There was no history of trauma. His past medical history revealed no abnormality.
Radiographic examination of the hand showed a marked increase in diameter along the fifth metacarpal and widening of the medullary canal. The cortex was uniformly thin and the metacarpal head was spared (Figure 1A). The characteristics of the lesion were consistent with aneurysmal bone cyst. After biopsy, pathologic examination confirmed the diagnosis of aneurysmal bone cyst (Figure 1B).
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Figure 1: Radiograph of the hand at admission (A). Pathologic appearence of the tumor (B; hematoxylin-eosin, magnification ×40).
En-block resection of the tumor was performed through a dorsal longitudinal incision over the fifth metacarpal. The metacarpal head, together with its carpometacarpal joint capsule, was left intact. Autologous bicortical strut graft was harvested from left iliac crest. The graft was molded into its definitive shape and inserted into the created bony defect. Multiple K-wires were used for graft fixation (Figure 2). The hand was immobilized in a short arm cast for 3 weeks after the patient received 3 weeks of physiotherapy consisting of progressive active range of motion exercises. Kirschner wires were removed 6 weeks postoperatively.
At final follow-up 3 years postoperatively, the patient had gained full range of hand motion with no pain. The patient was satisfied with the functional and cosmetic results. Radiographic examination demonstrated osseous integration of the graft with no signs of recurrence (Figure 3).
The main goals in the treatment of aneurysmal bone cyst of the hand are eradication of the lesion, prevention of recurrence, and preservation of hand function. The literature contains conflicting knowledge about the optimal treatment method.
Currently, curettage and bone grafting is the most common operative procedure used. However, recurrence rate is high after this procedure. Basarir et al17 reported that 2 of 3 cases that were initially treated with curettage and grafting recurred. Similarly, in a case series by Frassica et al,18 curettage and bone grafting in 7 cases was associated with 4 recurrences.
However, contrary reports are also found in the literature. Ropars et al19 suggested that curettage and grafting is sufficient for treatment, and aggressive methods such as cryotherapy or resection with reconstruction should only be used in case of recurrences and articular involvement. Other authors have reported parallel successful outcomes with no relapse after simple curettage and grafting.20,21 A problem with this method is that osteoclastic activity can reabsorb the graft material, depending on the aggressiveness of the lesion. Another limitation of this method is that if graft incorporation occurs, the original size of the lesion is present and can take years to remodel. Possible recurrence after insufficient primary treatment will increase the size of the defect; the tumor may reach joint structures, and consequent bone grafting must include the epiphysis and even complete joints.
Due to the high risk of recurrence after curettage and grafting alone, various forms of adjunctive therapy have been used to decrease the rate of local recurrence.16 There are 2 cases in which cryosurgery and sclerotherapy were used as an adjuvant intralesional treatment for aneurysmal bone cyst arising in the hand.22,23 These treatments are difficult to use in the small bones of the hand and may damage surrounding intact tissue and cause serious complications such as neurapraxia, postoperative fracture, burn, infection, and wound necrosis, which may happen more easily in distal lesions. Although a 3.7% local recurrence rate was reported with cryosurgery, there is a potential risk of amputation of small bones.22,24
En-block resection and reconstruction with strut grafting is another operative treatment option. Given the aggressive nature of aneurysmal bone cysts with the tendency to develop local recurrence, en-block resection seems to be the therapy of choice. No recurrences have been reported after en-block resection in the relevant literature.10,17,18,25-29 Despite it being a curative method of treatment, its use is limited, particularly in cases where the lesion is close to articular surfaces. Articular surface reconstruction and preservation of hand function need further advanced operative techniques such as nonvascularized or vascularized toe phalanx transplantation.23,30,31 Long operation time, the need for microsurgical skills, and donor site complications are major problems associated with these techniques. Otherwise, reconstruction can only be achieved with arthrodesis, which may impair hand function.
In our case, excellent clinical and functional results were obtained with en-block resection and reconstruction with iliac crest graft with no recurrence after 3-year follow-up. The metacarpal head was spared; therefore, articular surface was left intact.
- Jaffe HL, Lichtenstein L. Solitary unicameral cyst with emphasis on the roentgen picture, the pathologic appearance and the pathogenesis. Arch Surg. 1942; (44):1004-1025.
- Mendenhall WM, Zlotecki RA, Gibbs CP, Reith JD, Scarborough MT, Mendenhall NP. Aneurysmal bone cyst. Am J Clin Oncol. 2006; 29(3):311-315.
- Leithner A, Windhager R, Lang S, Haas OA, Kainberger F, Kotz R. Aneurysmal bone cyst. A population based epidemiologic study and literature review. Clin Orthop Relat Res. 1999; (363):176-179.
- Campanacci M. Aneurysmal bone cyst. In: Campanacci M, ed. Bone and Soft Tissue Tumors. Bologna, Italy: Aulo Gaggi Editore; 1990:725-751.
- Lichtenstein L. Aneurysmal bone cyst; further observations. Cancer. 1953; 6(6):1228-1237.
- Biesecker JL, Marcove RC, Huvos AG, Miké V. Aneurysmal bone cysts. A clinicopathologic study of 66 cases. Cancer. 1970; 26(3):615-625.
- Buraczewski J, Dabska M. Pathogenesis of aneurysmal bone cyst. Relationship between the aneurysmal bone cyst and fibrous dysplasia of bone. Cancer. 1971; 28(3):597-604.
- Dabezies EJ, D’Ambrosia RD, Chuinard RG, Ferguson AB Jr. Aneurysmal bone cyst after fracture. A report of three cases. J Bone Joint Surg Am. 1982; 64(4):617-621.
- Fuhs SE, Herndon JH. Aneurysmal bone cyst involving the hand: a review and report of two cases. J Hand Surg Am. 1979; 4(2):152-159.
- Burkhalter WE, Schroeder FC, Eversmann WW Jr. Aneurysmal bone cysts occurring in the metacarpals: a report of three cases. J Hand Surg Am. 1978; 3(6):579-584.
- Panoutsakopoulos G, Pandis N, Kyriazoglou I, Gustafson P, Mertens F, Mandahl N. Recurrent t(16;17)(q22;p13) in aneurysmal bone cysts. Genes Chromosomes Cancer. 1999; 26(3):265-266.
- Oliveira AM, Hsi BL, Weremowicz S, et al. USP6 (Tre2) fusion oncogenes in aneurysmal bone cyst. Cancer Res. 2004; 64(6):1920-1923.
- Leithner A, Lang S, Windhager R, et al. Expression of insulin-like growth factor-I (IGF-I) in aneurysmal bone cyst. Mod Pathol. 2001; 14(11):1100-1104.
- Dabska M, Buraczewski J. Aneurysmal bone cyst. Pathology, clinical course and radiologic appearances. Cancer. 1969; 23(2):371-389.
- Martinez V, Sissons HA. Aneurysmal bone cyst. A review of 123 cases including primary lesions and those secondary to other bone pathology. Cancer. 1988; 61(11):2291-2304.
- Cottalorda J, Bourelle S. Modern concepts of primary aneurysmal bone cyst. Arch Orthop Trauma Surg. 2007; 127(2):105-114.
- Basarir K, Saglik Y, Yildiz Y, Tezen E. Aneurysmal bone cyst of the hand: a report of four cases. Hand Surg. 2006; 11(1-2):35-41.
- Frassica FJ, Amadio PC, Wold LE, Beabout JW. Aneurysmal bone cyst: clinicopathologic features and treatment of ten cases involving the hand. J Hand Surg Am. 1988; 13(5):676-683.
- Ropars M, Kaila R, Briggs T, Cannon S. Aneurysmal bone cysts of the metacarpals and phalanges of the hand. A 6 case series and literature review [in French]. Chir Main. 2007; 26(4-5):214-217.
- Sakka SA, Lock M. Aneurysmal bone cyst of the terminal phalanx of the thumb in a child. Arch Orthop Trauma Surg. 1997; 116(1-2):119-120.
- Sproule JA, Salmo E, Mortimer G, O’Sullivan M. Aneursymal bone cyst of the proximal phalanx of the thumb in a child. Hand Surg. 2002; 7(1):147-150.
- Athanasian EA, McCormack RR. Recurrent aneurysmal bone cyst of the proximal phalanx treated with cryosurgery: a case report. J Hand Surg Am. 1999; 24(2):405-412.
- Salon A, Rémi J, Brunelle F, Drapé JL, Glorion Ch. Total replacement of a middle phalanx by free non-vascularized chondral graft, after failure of sclerotherapy for treatment of an aneurysmal bone cyst [in French]. Chir Main. 2005; 24(3-4):187-192.
- Schreuder HW, Veth RP, Pruszczynski M, Lemmens JA, Koops HS, Molenaar WM. Aneurysmal bone cysts treated by curettage, cryotherapy and bone grafting. J Bone Joint Surg Br. 1997; 79(1):20-25.
- Gundes H, Tosun B, Muezzinoglu B, Tosun A. Total destruction of the fourth metacarpal bone by aneurysmal bone cyst: reconstruction with strut fibular graft—a case report. Hand Surg. 2005; 10(2-3):265-269.
- Ertem K, Karadag N, Altinok T, Karakas H. Aneurysmatic bone cyst of the second metacarpal: en-block resection and bicortical iliac crest graft replacement. Eur J Orthop Surg Traumatol. 2007; 17(1):89-91.
- Braatz F, Popken F, Bertram Ch, Rütt J, Hackenbroch MH. Aneurysmal bone cyst of the fourth metacarpal bone—a case report [in German]. Handchir Mikrochir Plast Chir. 2002; 34(2):128-132.
- Mortensen NH, Kuur E. Aneurysmal bone cyst of the proximal phalanx. J Hand Surg Br. 1990; 15(4):482-483.
- Kotwal PP, Jayaswal A, Singh MK, Dave PK. Aneurysmal bone cyst in the metacarpal of a child: a case report. J Hand Surg Br. 1988; 13(4):479-480.
- Gudemez E, Eksioglu F. Aneurysmal bone cyst of the thumb metacarpal: en-block resection and free toe phalanx transplantation. Orthopedics. 2003; 26(12):1229-1230.
- Rao GS, Keogh P, Webster H, Lunn PG, Burke FD. Aneurysmal bone cysts in the hand treated by free non-vascular transfer of metatarsal or proximal phalanx from the foot. J Hand Surg Br. 1993; 18(6):736-741.
Dr Ozyurek is from the Department of Orthopedics, Izmir Military Hospital, Izmir, Drs Rodop, Cilli, and Mahirogullari are from GATA Haydarpasa Training Hospital, Istanbul, and Dr Kose is from Diyarbakir Education and Research Hospital, Diyarbakir, Turkey.
Drs Ozyurek, Rodop, Kose, Cilli, and Mahirogullari have no relevant financial relationships to disclose.
Correspondence should be addressed to: Selahattin Ozyurek, MD, Department of Orthopedics, Izmir Military Hospital, Inonu Caddesi, Hatay, Izmir, Turkey.