Aneurysmal bone cyst: results of an off label treatment with Denosumab

Abstract

Background: The treatment of aneurysmal bone cysts (ABCs) has evolved and less invasive methods have been tried. Denosumab is a monoclonal antibody which inhibits osteoclasts. It has been shown to be effective in giant cell tumour of bone (GCT) of bone and hence promises some effect also in ABC. We report on 6 patients treated with Denosumab and compare our results to the cases already published. Methods: Data of 6 patients with ABCs and patients whose treatment included Denosumab were retrospectively analyzed. Denosumab was used at a dose of 120 mg on days 1, 8, 15 and 29, and every 4 weeks thereafter. In some of these patients the dose was reduced at the end of the treatment. Clinical and radiological responses were evaluated. Results: In 4 female and 2 male patients with a mean age of 17 years (range: 6-30 years) the lesions were located in the sacrum (2), in distal radius, distal femur, talus and pelvis. One of the sacral lesions healed after 12 months and has stayed stable for 3 years since. The second patient received 2 years of therapy with recalcification, but recurred 1 year later and is under renewed therapy. The pelvic lesion improved but recurred. This patient has a 13-years history of intermittent therapy including surgery, two pregnancies and remains in a stable situation. The lesion of the talus did not improve with Denosumab after surgery and was complicated by destruction of the ankle joint with osteoarthritis. Recurrent lesions of the distal femur and the distal radius, previously treated by curettage and bone grafting healed under Denosumab and have remained stable for 2 and 3 years, respectively. One case of severe hypercalcemia was observed in a 7-year old child 6 months after discontinuation of Denosumab. Conclusion: Denosumab provides a treatment option for ABCs in anatomically critical locations. Adjuvant application might reduce the rate of local recurrence. In young patients, severe rebound hypercalcemia months after discontinuation of Denosumab may occur.

Hypercalcaemia after treatment with denosumab in children: bisphosphonates as an option for therapy and prevention?

“…There are no data on real frequency of these sequelae. So far, there are only two case series reports of children with and without hypercalcemia after treatment [19,24]. An incidence of 22, respectively, 20% could be calculated, but, of course, the sample size is too small to generalize these data.…”

“…Grasemann et al [13] found this side effect in a child with juvenile Paget, and Boyce et al [9] in a boy with severe fibrous dysplasia both treated with denosumab. Recently, Kurucu et al [19] and Dürr et al [24] reported reactive hypercalcemia in several denosumab-treated children and adolescents with aneurysmatic bone cysts, the phenomenon clearly must be related to the treatment.…”

Treatment of Aneurysmal Bone Cysts with Titanium Elastic Nails in Children

“…Table 1 showed the speci c focus distribution. The content includes 16 cases of the study group and 10 cases of control group; the mean age for the patients of the 2 groups were 5.94 (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14) years old and 7.20…”

“…The imaging of ABC shows that ABC is a polycystic and expansile osteolytic disorder, 70% of which are primary, and 30% are secondary. Up to now, the causes and pathogenic mechanisms of the ABC are still unknown, which leads to the diversi ed treatment methods of ABC [3][4]. Currently, the main treatment method of ABC is curettage and bone grafts with high-speed burring.…”

Successful Use of Denosumab for Life-Threatening Hypercalcemia in a Pediatric Patient with Primary Hyperparathyroidism

“…According to the literature, denosumab has been used offlabel in adults with PHPT for the treatment of hypercalcemia prior to a parathyroidectomy [5, 6], long-term management of parathyroid carcinoma-mediated hypercalcemia [7-9], and medical treatment of osteoporosis in PHPT [10]. It has been used off-label in children with several skeletal diseases, for example, osteogenesis imperfecta [11][12][13], fibrous dysplasia [14], aneurysmal bone cysts [15][16][17][18], giant-cell tumors [19], Paget's disease of bone [20], glucocorticoid-induced osteoporosis in Duchenne muscular dystrophy [21], osteoradionecrosis [22], cherubism [18,23], as well as for hypercalcemia reduction after bone-marrow transplantation in osteopetrosis [24] and in squamous cell carcinoma [25]. To our knowledge, only 1 case of denosumab usage in a child with severe PHPT due to a parathyroid carcinoma and ineffectiveness of bisphosphonates has been reported to date [26].…”