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Aneurysmal bone cyst is an uncommon nonneoplastic lesion that occurs predominantly during the first 2 decades of life. Angiographic- and tissue-phase imaging provided more striking evidence of this unexpected lesion than skeletal-phase imaging in a 13-year-old girl with pain in the right buttock and posterior thigh.
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In 1993 a patient aged 16 years affected with pathologic fracture on bone cyst of the proximal femur came to our observation. The fracture and the cyst healed in 10 months after treatment using an external fixator and autoplastic bone grafts.
Epiphyseal involvement of a unicameral bone cyst (UBC) is rare. This anatomic setting represents a distinct clinical and radiographic entity. This study reports a new case and analyzes the clinical and biological behavior of seven additional UBCs with epiphyseal involvement from the literature. We report the first successful treatment of this variant with methylprednisolone acetate. The average age was 20.1 years with a male to female ratio of 1.3:1. Anatomic location: proximal femur (4), proximal humerus (2), and proximal tibia (2). Both age and location were atypical when compared to the classic metaphyseal location. Radiographically, all lesions presented a characteristic involvement of the epiphysis and metaphysis in various proportions. The epiphyseal plates were judged closed versus open in 50%, respectively. Follow-up ranged from 9 months to 3 years. Six cases healed following a single curettage (three with and three without bone graft). There were no late complications of fracture, deformity, shortening, or avascular necrosis. Recurrence was 0%. No secondary procedures were required. We conclude the age, location, and radiographic appearance is atypical and diagnosis is difficult, but the biological behavior is less aggressive and the prognosis more favorable than the typical, metaphyseal UBC. Curettage with or without bone graft has a high success rate. We recommend aspiration and intralesional methylprednisolone as the initial management. We hypothesize that epiphyseal UBCs have a better prognosis than metaphyseal location alone due to the older age, atypical location, and the potential of the epiphysis to reossify.
DNA cytometric investigations of intraosseous cystic lesions and osteoblastic tumors may be helpful in morphological diagnosis. The detection of a DNA-aneuploid stemline supports the diagnosis of a high-grade malignant bone neoplasm, even in small biopsies. Solitary and aneurysmal bone cysts have DNA-diploid cell populations. Low-grade central osteosarcomas may show single-cell aneuploidies of varying extent as well as a DNA-diploid stemline. Their presence may be a sign of genetic instability within the population of neoplastic cells before a DNA-aneuploid stemline is established and should prompt careful postoperative observation.
We report on a 6-year-old girl with a polypoid mass, filling up the entire right nasal cavity as shown on a magnetic resonance imaging scan. Histologically, the tumor had the characteristics of an aneurysmal bone cyst, which is extremely rare in this location. Cytogenetic analysis disclosed a single (6:17)(p21;p13) translocation, confirming a specific genetic involvement in the development of aneurysmal bone cysts. Fluorescent in situ hybridization analysis mapped the putative gene between the p53 (17p13.1) and the Mieller-Dieker gene (17p13.3) loci.
Aneurysmal bone cysts (ABC) are locally aggressive bone tumors that often feature chromosome 17p13 rearrangements. One of the ABC 17p13 rearrangements--t(16;17)(q22;p13)--was recently shown to create a CDH11-USP6 fusion in which the USP6/TRE17 oncogene is overexpressed through juxtaposition with the CDH11 promoter. Herein, we characterize four different ABC translocations involving 17p13, and we show that each is associated with a novel USP6 fusion oncogene. Specifically, we demonstrate that t(1;17), t(3;17), t(9;17), and t(17;17) result in USP6 fusions with TRAP150 (thyroid receptor-associated protein 150), ZNF9 (ZiNc Finger 9), Osteomodulin, and COL1A1 (Collagen 1A1), respectively. The oncogenic mechanism in these fusion genes is akin to CDH11-USP6, with the USP6 coding sequences juxtaposed to the promoter regions in each of the four novel translocation partners. The novel fusion partners appear well suited to drive USP6 transcription in the bone/mesenchymal context: osteomodulin is expressed strongly in osteoblastic lineages, and the COL1A1 promoter has an oncogenic role in the mesenchymal cancer dermatofibrosarcoma protuberans. In summary, these studies show that USP6 oncogenic activation results from heterogeneous genomic mechanisms involving USP6 transcriptional upregulation by juxtaposition with ectopic promoters.
The clinical features, diagnosis and radiological findings of eight cases of a subchondral bone cyst involving the medial condyle of the femur are described. Surgical treatment, which comprised performing an arthrotomy of the femorotibial joint, curetting and packing the cystic cavity with an autogenous cancellous bone graft, is described.
An 18-year old boy presented with a tumour involving a large part of the petrous bone. A radical resection of a petrous bone 'aneurysmal bone cyst' was achieved. A large defect in the dura adjoining the posterior surface of the petrous bone was made during surgery. Post-operative MR imaging showed complete tumour resection but herniation of cerebellum into the operative defect, a phenomenon probably assisting to seal the site of cerebrospinal fluid fistula.
A case of two secondary aneurysmal bone cysts arising in fibrous dysplasia during pregnancy is reported. Marked radiographic changes were seen in one lesion over a 3-week period. The development of these cysts during pregnancy strongly suggests that the hemodynamic and/or hormonal changes of pregnancy were responsible for their formation.
Aneursymal Bone Cysts (ABCs) involving the hand are a rare occurrence. We report a case of an ABC of the proximal phalanx of the thumb in a boy which was treated successfully with curettage and autologous bone grafting. When the diagnosis of ABC of the small bones of the hand is entertained, prompt therapeutic intervention is indicated because of the potential for aggressive local behaviour. In the paediatric patient, simple surgery to preserve the growth plate is recommended.
The authors have operated on 2 children aged 7 and 8 years who had developed a large unicameral bone cyst in the trochanteric and upper femoral diaphysial region. Ender's nails were inserted into the bone above the lower femoral epiphysial plate. Healing occurred rapidly.
Aneurysmal bone cyst (ABC) is a relatively rare, non-neoplastic expansile lesion of bone. Bleeding may occur during an operation or biopsy for ABC, as this cyst is an aneurys with numerous pools of blood. Therefore, it is necessary to diagnose ABC before treatment or biopsy. In the present report, we describe the characteristic computed tomography (CT) and magnetic resonance imaging (MRI) features of ABC in the mandible. Based on the literature and on our own experiences, we compare with the features of ABC with the corresponding features of other lesions showing similar conventional radiographic appearances. In the present case, bone-targeting CT showed the characteristic feature, which reflected the histopathological appearance of a partially cystic meshwork divided by coarse septa. MRI showed almost homogeneous intermediate signal intensity, including a partial slight low-signal-intensity area on the T1-weighted image, and homogeneous high signal intensity, which showed a 'bubbly' appearance, on T2-weighted image. On the enhanced T1-weighted image, the intermediate signal intensity areas apart from the areas that showed slight low-signal intensity on the non-enhanced T1-weighted image, were well enhanced. This creates a 'honeycomb' appearance. The 'meshwork' appearance on bone-targeting CT, the 'bubbly' appearance on the T2WI and the 'honeycomb' appearance on Gd-T1WI may be the characteristic features of ABC.
We report on a 19-year-old man with a 6-month history of pain and stiffness in the upper cervical region who proved to have a lytic lesion affecting the anterior and posterior elements of C2-4, causing a severe gibbous deformity at C3. The tumour was excised through a posterior approach, and spinal fusion with instrumentation was performed. Two weeks later, through a right-sided, anteromedial approach, the remaining tumour was excised, and the spine was reconstructed with tricortical iliac bone as a strut graft. The patient was placed in a Minerva jacket for 3 months, during which time a solid fusion was obtained. The histological diagnosis was aneurysmal bone cyst. The patient has remained pain free and suffered no recurrence 2 and a half years after surgery. Involvement of several adjacent cervical vertebrae by an aneurysmal bone cyst is rare, and conventional treatment with curettage and bone grafting is most likely to carry a high rate of recurrence and spinal instability. We recommend complete excision of the tumour in a combined staged procedure and instrumentation to prevent recurrence and avoid instability.
The clinical and radiologic patterns of amyloid osteoarthropathy in long-term hemodialysis patients are well known. However, few studies about the incidence of dialysis-associated amyloidosis in continuous ambulatory peritoneal dialysis (CAPD) patients have been published to date. In a series of 27 CAPD patients, the authors found bone cysts in 63.6% of cases, at the beginning of their dialysis treatment. After a mean follow-up period of 20.9 +/- 13.8 months, carpal bone cysts were seen in 70.4% of cases and clinical patterns worsened in 26.3% of patients. Therefore, CAPD does not prevent the development of dialysis-associated amyloidosis; prolonged uremic state and decreased diuresis seem to be the main pathogenetic factors of osteoarthropathy. The incidence and the evolution of bone lesions were related to the clinical data of this series and then compared with those of a group of hemodialysis patients. This study confirms that plain wrist films are a simple and useful method in the early detection and in the follow-up of amyloidosis-related disease.
Aneurysmal bone cyst (ABC) is a rare benign lesion of the head and neck. A case of an ABC in a 12-year-old female is presented, the twentieth reported such case in the maxilla. Literature review shows ABC are common in the long bones; only 2% are in the head and neck. Of the 77 lesions in the head and neck found in the literature, 31 (40%) are in the mandible, 20 (26%) are in the maxilla (including this case) and the remainder mainly in the skull. Current recommended treatment is curettage with enucleation first if technically possible.
Experience collected by us from treating 15 patients suffering from giant cell tumours, aneurysmatic bone cysts or an osteoid osteoma prompted us to investigate which progress has been made to date in the diagnosis and treatment of these lesions that contain giant cells, basing on recent diagnostic methods such as computed tomography and magnetic resonance, as well as neurosurgical methods. It was found that the course of these rare changes cannot be influenced either by extensive diagnostic or special neurosurgical measures and that the prognosis finally depends on the growth trends inherent in the lesion concerned. Now that both diagnostic and surgical techniques are more refined than previously, there is a tendency to an improved individual approach that is better suited to the needs of the patient. Radiotherapy of benign changes involving giant cells in the region of the vertebral column is no longer of any importance.