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Two cases of aneurysmal bone cysts are reported. Each patient presented with a palpable mass in the occipital region and signs of compression of structures in the posterior fossa. One of the cases is unique, in that the aneurysmal bone cyst was associated with an epidural hematoma in the posterior fossa. The pertinent literature is reviewed.
Aneurysmal bone cyst (ABC) usually affects older children or young adults. The radiological appearance is of a purely osteolytic expansile metaphyseal lesion with thinned out cortex and internal septations. We present a case of ABC in a 5-year-old girl which showed internal calcification and was initially diagnosed as enchondroma. However, pathological examination revealed typical appearance of ABC with large area of dystrophic calcification.
Aneurysmal bone cyst (ABC) is a locally aggressive osseous lesion that typically occurs during the first two decades of life. ABC was regarded historically as a nonneoplastic process, but recent cytogenetic data have shown clonal rearrangements of chromosomal bands 16q22 and 17p13, indicating a neoplastic basis in at least some ABCs. Herein we show that a recurring ABC chromosomal translocation t(16;17)(q22;p13) creates a fusion gene in which the osteoblast cadherin 11 gene (CDH11) promoter region on 16q22 is juxtaposed to the entire ubiquitin-specific protease USP6 (Tre2) coding sequence on 17p13. CDH11-USP6 fusion transcripts were demonstrated only in ABC with t(16;17) but other ABCs had CDH11 or USP6 rearrangements resulting from alternate cytogenetic mechanisms. CDH11 is expressed strongly in bone, and our findings implicate a novel oncogenic mechanism in which deregulated USP6 transcription results from juxtaposition to the highly active CDH11 promoter.
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An aneurysmal bone cyst (ABC) is an uncommon benign bone lesion that principally occurs in long bones and in the vertebral column. The appearance of ABC in carpal bones is very rare. We report a case of ABC that occurred in the hamate bone.
OBJECTIVE AND IMPORTANCE: Aneurysmal bone cysts (ABC) are benign bone neoplasms which typically involve the spine and long bones. We present a rare case of an ABC in the temporal bone with significant cerebellar compression. CLINICAL PRESENTATION: The patient was a young boy who presented with several weeks of left posterior auricular pain. Computed tomography, magnetic resonance imaging and angiogram showed an expansile bone neoplasm involving the left petrous temporal bone. INTERVENTION: A retrosigmoid-transmastoid craniectomy was performed, and total removal of the bone lesion was achieved. The pathological examination revealed the diagnosis of ABC. CONCLUSION: The postoperative course was uneventful, and the imaging studies demonstrated total removal of the neoplasm. This case represents the imaging and surgical management of a rare ABC in the temporal bone. This diagnosis should be considered in the differential of bone neoplasms in this region.
Occult malformations of the skull base are very rare disorders which cannot initially be diagnosed clinically. They become first symptomatic, if complications arise. The most frequent clinical manifestation is a recurrent bacterial meningitis. Based on the unique case of a presumably congenital aneurysmal bone cyst of the petrosal bone, which imposed clinically as hearing loss and recurrent bacterial meningitis, we have evaluated the extensive literature on this theme. Considering mostly atypical clinical findings, solely coronal thin-section computed tomographic scans of the skull base allow a reliable diagnosis. The possible risks of this investigation (necessary sedation up to insufflation anesthesia and the radiation exposure of the child's lens) are justifiable and subordinate to the diagnostic importance.
Percutaneous autogeneic bone marrow injection is an effective method for managing simple bone cysts, and it might be considered before the application of more extensive procedures.
Curettage and bone grafting are the accepted methods of treatment of aneurysmal bone cysts. Unfortunately, recurrence is common. We treated 4 patients with atypical aneurysmal bone cysts that lacked aneurysmal dilatation by endoscopic curettage without bone grafting. New bone formation and remodeling were observed in all patients. In the patients in whom the follow-up was longer than 30 months, there was no evidence of recurrence. Endoscopic curettage without bone grafting is a simple and effective treatment for aneurysmal bone cysts.
OBJECTIVE AND IMPORTANCE: A rare case of aneurysmal bone cyst (ABC) of the temporal bone is presented which, following recurrence after surgery, was successfully treated with radiotherapy. The role of radiotherapy in such cases is reviewed. CLINICAL PRESENTATION: A 30-year-old man presented with a recurrent swelling and pain in right temporal region following surgery for ABC at that site. INTERVENTION: Local radiotherapy to a dose of 31.5 Gy in 18 fractions over 3.5 weeks was delivered to the site of recurrence. The patient had a near total regression of the ABC as evident clinically and on radiological images. CONCLUSION: To the best of our knowledge, radiation for the recurrent ABC at the temporal bone has not been described in the literature. However, in view of the response evident in this patient, radiotherapy seems to be effective for recurrent cases of ABC at the temporal bone and a dose of around 30 to 36 Gy could be effectively delivered with satisfactory results.
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The aneurysmal bone cyst represents a tumor-like lesion, which does not occur frequently. Every site of the skeleton may be involved. Although it can be observed at any age, it distinctly predominates from 10 to 20 years of age. Distinction of aneurysmatic bone cysts from certain benign or malignant bone tumours requires subtle imaging techniques and an experienced bone tumour pathologist. Since the lesion shows a heterogeneous biological behaviour and typically occurs in the growing skeleton, a wide range of surgical procedures must be provided concerning tumour resection and defect reconstruction. In the current retrospective study results of 41 surgically treated aneurysmal bone cysts, thereof 5 latent, 31 active, and 5 aggressive lesions, were investigated. While morbidity due to the therapeutic measures was comparatively low even in extended lesions and demanding reconstructions, a recurrence rate of 19.5% had to be observed in our series. Taking into consideration the biological activity of the lesion, analysis of the recurrences revealed inappropriate surgical margins had been chosen at the primary operation in all of these patients. Since the risk of a local relapse is linked both to aggressiveness of the primary lesion and to surgical radicality, therapy of aneurysmal bone cyst requires preoperative staging and stage-dependent surgical procedures.
Aneurysmal bone cyst (ABC) of the temporal bone is rare. The nature of the underlying disorder that converted into the ABC might, however, be difficult to ascertain on imaging as well as on histopathology. The unusual CT and MRI findings in a case of ABC of the temporal bone are presented. This had transdural intracerebral spread with a large component of solid enhancing matrix but no peripheral calcific rim. The patient was an adult of 45 years with a history of headache for more than 1 year.
The aneurysmal bone cyst is according to the description of Jaffe and Lichtenstein (1942) a histomorphological entity. It is a benign centric or eccentrically localized tumor like lesion with preference to the metaphysis. 75% of all aneurysmal bone cysts occur within the first two decades of life. On x-ray the diagnosis is not always clear. In differential diagnosis juvenile bone cyst, giant cell tumor, chondroblastoma, chondromyxoid fibroma, non ossifying fibroma, fibrous dysplasia and telangiectasic osteogenic sarcoma have to be considered. Final diagnosis requires histological evaluation. Curettage or resection with bone grafting is the predominant therapeutic procedure. Radiotherapy might cause malignant degradation and should only be used in elected cases. For propaedeutic evaluations of aneurysmal bone cysts 851 cases in world literature are reported.
BACKGROUND: Aneurysmal bone cysts (ABCs) are uncommon lesions of the temporal bone and their occurrence in the calvarium is rare. CASE DESCRIPTION: A case of a right temporal ABC is reported in a 14-year-old boy who presented swelling of the right temporal region. Magnetic resonance imaging showed a destructive and expansile bone lesion on the right anterior temporal and orbital bone. The lesion was removed in total by the right temporal craniotomy and orbitozygomatic osteotomy. ABC was diagnosed in the pathologic examination. The patient had good recovery during the postoperative course. CONCLUSIONS: This report presents the diagnosis and imaging of an ABC in the temporal bone. This localization is very rare for ABC. Total excision, if feasible, is the ideal treatment.
Aneurysmal bone cysts is a benign, slow growing expansile lesion usually found in long bones or vertebrae. Only 5 cases of ABC of the ethmoid bone have been reported in the literature. Plain radiographs are not specific. CT and MRI are very useful to depict the extent of the lesion and demonstrate the presence of blood-fluid levels, which is a characteristic finding of ABC. We present one atypical case of ABC of the ethmoid bone in a 15-year-old female characterised by its extensive involvement and its rare location.
Aneurysmal bone cysts (ABCs) are benign, slow growing expansile lesions usually found in long bones or vertebrae. Plain radiography reveals expansion of bone and cortical thinning. MRI may assist in diagnosis by virtue of its ability to demonstrate blood-fluid levels, which is a characteristic finding in these lesions. Very few cases of ABC of the paranasal sinuses have been reported in the literature. We present MRI findings of ABC of the ethmoid sinus in a 19-year-old male.
Aneurysmal bone cyst is a benign pseudotumoral dystrophy of bone, presenting expansive, destructive, nonresolutive features and often recurrence. A "stand-by" therapeutically attitude, in a survey manner, based on some spontaneous regression of certain areas of the aneurysmal cyst, due to thrombosis and fibrosis, is rarely advocated. When tumoral lesions are located in long bones and the bone length must be preserved, the bioptic curettage, followed or not by auto-grafting or cortical/cancellous allografting to bridge defect, represents the treatment of choice. The inconveniences encountered in using auto- or allografts have raised a growing interest towards synthetic bone substitutes. The most used of them are phosphocalcic ceramics due to their basic properties regarding interaction between bone and the substitution material, especially macroporosity Lately, we started to use (as clinical application) a bone substitute based on synthetic biphasic macroporous ceramic (CERAFORM), covering a wide range of procedures: benign tumors and dystrophies, spinal or joint arthrodesis, periprosthetic fractures, revisions following failures of primary total hip replacements. We obtained promising results, suggesting that in a situation with limited bone defects, as in clinical case presentation, if there exist a good contact and strong mechanical fixation, CERAFORM represents a reliable option comparing to allograft.