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Traumatic bone cyst, aneurysmal bone cyst, and central giant cell granuloma--pathogenetically related lesions?

Three central bone lesions, a traumatic bone cyst, an aneurysmal bone cyst, and a central giant cell granuloma are reported. Comparisons of the clinical, radiographic, and histological features were made. Although these lesions are recognized as separate and distinct pathological entities, a common pathogenesis is suggested by similarities in certain features. Diagnostic aids are suggested to assist in a differential diagnosis.

Adolescent↗

[Echography for the diagnosis of aneurysmal bone cysts].

Aneurysmal bone cysts are benign tumors which are likely to recur. While growing, they cause severe osteolysis, able to balloon the bone and to thin the cortex. The latter is reduced to a thin bone shell, and sometimes completely interrupted. Tumor mass can spread into the surrounding soft tissues as in other bone tumors, especially malignant ones, which are often misdiagnosed. Whether the cortex is interrupted or only thinned, an acoustic window is obtained which allows an easy study of tumor mass with US. Seven patients were examined, who presented lytic lesions of different bones, produced by aneurysmal cysts. US allowed the expansile lesion to be detected, and its size to be estimated. Moreover, US pattern of the lesion could be determined, which appeared both solid and liquid. A few minutes after beginning the examination, fluid levels could be observed to appear, which are typical of this condition, as both CT and MR imaging demonstrate both in vivo and in vitro. Thus US, thanks to its short execution time, low cost and non-invasiveness, can be considered as the second-step exam, after plain film, in the diagnosis of aneurysmal bone cyst.

Adult↗

Juxtaphyseal aneurysmal bone cysts.

Aneurysmal bone cysts are benign primary or secondary lesions that commonly arise in long bones and often before skeletal maturity. Little has been written about aneurysmal bone cysts that abut the physeal plate. The records of 15 patients with juxtaphyseal aneurysmal bone cysts were reviewed. Fourteen of the patients were referred with abnormal radiographs after evaluation for pain in the affected limb. One patient presented with abnormal radiographs after fracture about the aneurysmal bone cyst. None of the patients had evidence of growth plate disruption. The children's ages ranged from 2 to 14 years, with a mean of 9.8 years. There were 10 boys and five girls. Lesion locations included: six in the proximal tibia, three in the distal fibula, two in the distal tibia, two in the proximal femur, one in the distal femur, and one in the distal radius. All of the lesions abutted the physeal plate and fell into one of the types in Campanacci's classification of juxtaphyseal aneurysmal bone cysts. Three lesions were classified as Type 1, eight were Type 2, and four were Type 3. This study included no cases of Type 4 or 5 lesions. Treatment of all lesions consisted of excision, curettage, and bone grafting with care taken to preserve the growth plate. Adjunctive cauterization was performed in two cases. There were no incidences of postoperative physeal plate arrest. Overgrowth of the fibula occurred in one patient. Three patients experienced recurrent lesions. One of the children underwent repeat curettage and bone grafting with no additional recurrence. In the other two children with recurrence, the lesion had grown away from the physeal plate while remaining static in size and asymptomatic. Based on this study, juxtaphyseal aneurysmal bone cysts may be treated satisfactorily with intralesional surgery and bone grafting with expectation of normal physeal growth.

Adolescent↗

Fluid levels in aneurysmal bone cysts.

Aneurysmal bone cysts are benign, expansile, osteolytic lesions of the bone. All the aneurysmal bone cysts studied in this case report were observed during the 2 year period, 1989 to 1991. The patients involved had received radiographic examination: plain and magnetic resonance (MR) image; one of the patients also received a computed tomography (CT). The plain radiograph generally showed a well-defined, expansile, osteolytic lesion associated with a pathologic fracture; the CT and MR images showed a fluid-fluid level within the lesion. This paper will report the findings of fluid-fluid levels, using radiographic images, in aneurysmal bone cysts.

Adult↗

Bone matrix therapy for aneurysmal bone cysts.

Aneurysmal bone cysts are unique pathologic entities that cause pain and local osseous destruction. Many surgical treatment modalities have been described. This article reports on the case of a 16-year-old high school athlete with left heel pain due to an aneurysmal bone cyst in the calcaneus. Curettage of the bone cyst was performed, and the void was filled with a commercially available mixture of cancellous bone and demineralized bone matrix. Early return to athletic activity was achieved, with no recurrence noted at 3-year follow-up.

Adolescent↗

Recurrent t(16;17)(q22;p13) in aneurysmal bone cysts.

Aneurysmal bone cyst (ABC) is a benign bone lesion for which no previous cytogenetic data exist. We describe the finding of clonal chromosome aberrations in three tumors; two had a t(16;17)(q22;p13) as the sole anomaly, and the third had a del(16)(q22) as the only change. These findings show that somatic mutations contribute to the development of ABC and furthermore indicate that bands 16q22 and 17p13 may harbor genes of importance in this process.

Adolescent↗

USP6 (Tre2) fusion oncogenes in aneurysmal bone cyst.

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.

Adolescent↗

Case report: calcification within aneurysmal bone cyst.

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.

Bone Cysts, Aneurysmal↗

USP6 and CDH11 oncogenes identify the neoplastic cell in primary aneurysmal bone cysts and are absent in so-called secondary aneurysmal bone cysts.

Aneurysmal bone cyst (ABC) is a locally recurrent bone lesion that has been regarded as a reactive process. Recently, a neoplastic basis in primary ABC was evidenced by demonstration of clonal chromosome band 17p13 translocations that place the USP6 (TRE2 or TRE17) oncogene under the regulatory influence of the highly active CDH11 promoter. Herein, we report CDH11 and/or USP6 rearrangements in 36 of 52 primary ABCs (69%), of which 10 had CDH11-USP6 fusion, 23 had variant USP6 rearrangements without CDH11 rearrangement, and three had variant CDH11 rearrangements without USP6 rearrangement. USP6 and CDH11 rearrangements were restricted to spindle cells in the ABC and were not found in multinucleated giant cells, inflammatory cells, endothelial cells, or osteoblasts. CDH11 and USP6 rearrangements did not correlate with recurrence-free survival, or with other clinicopathological features. CDH11 and USP6 rearrangements were not found in any of 17 secondary ABC associated with giant cell tumor, chondroblastoma, osteoblastoma, and fibrous dysplasia. These findings demonstrate that primary ABC are mesenchymal neoplasms exhibiting USP6 and/or CDH11 oncogenic rearrangements. By contrast, secondary ABC lack CDH11 and USP6 rearrangements, and although morphological mimics of primary ABC, appear to represent a non-specific morphological pattern of a diverse group of non-ABC neoplasms.

Adolescent↗

Case reports: malignant transformation of aneurysmal bone cysts.

An aneurysmal bone cyst is an uncommon benign primary bone tumor. Careful intralesional curettage through a wide cortical window in addition to cauterization with or without adjuvant therapy (phenol or hydrogen peroxide) and bone grafting or cementation is the preferred surgical treatment. Adjuvant or primary radiation of an aneurysmal bone cyst rarely is used because of its association with malignant transformation of the lesion. Several cases of late malignant transformation of primary aneurysmal bone cysts without adjuvant radiation have been reported. We provide additional documentation of two primary aneurysmal bone cysts treated surgically with careful intralesional curettage through a wide cortical window and allograft bone grafting without adjuvant radiation. At 5.5 years and 12 years after treatment, a telangiectatic osteosarcoma and a fibroblastic osteosarcoma, respectively, were identified in the site of the original lesions. Not only should aneurysmal bone cysts be evaluated carefully through histologic examination at presentation, patients also should be counseled regarding possible recurrence and the need for routine followups, especially if symptoms change.

Adolescent↗

[Aneurysmal bone cysts].

An aneurysmal bone cyst is a tumor-simulating bone lesion, the etiology of which is still unclear and probably inhomogeneous. This type of lesion is mainly observed during the second decade of life and is rarely diagnosed beyond the age of 30 years. It is characterized by hollow spaces consisting of several compartments filled with blood and partially divided by septs consisting of spindle-cell tissue with ample multinuclear giant cells and frequent reactive new bone formation. The locations of preference are the metaphyses of the long bones, the spine and flat bones; however, they can appear in every sort of bone. The radiological picture is characterized by osteolytic expansion of the bone with more or less distinct formation of trabeculae. In the CT image we can often find fluid formation; in MRT images alteration of signals can also be seen, indicating former or fresh bleeding. The primary aneurysmal bone cyst should be distinguished from its secondary counterpart; the latter one is occasionally found in giant cell tumors, chondroblastomas, chondromyxoid-fibromas, osteoblastomas, but also in osteosarcomas. When planning biopsy or therapy, this possibility should be also taken into account. The therapy of choice consists of accurate curettage and autologous or allogenous bone transplantation. To avoid recurrences, curettage should be followed by adjuvant therapy with phenol or cryotherapy. When there are extensive recurrences that can no longer be treated by the surgical method described above, embolization of the nutrient vessels may be curative.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Therapy of aneurysmal bone cyst].

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.

Adolescent↗

Fibro-osseous lesions of the face and skull with aneurysmal bone cyst formation.

Aneurysmal bone cyst engraftment on benign fibro-osseous lesions, well known to occur in the extracranial skeleton, has been reported infrequently in the craniofacial bones. When this combined lesion occurs in the skull the clinical and radiographic features often suggest an aggressive neoplasm. The clinical, radiographic, and pathologic features of five cases are presented, and this uncommon lesion of the craniofacial bones is discussed in view of these cases and those reported in the literature.

Adolescent↗

[Frontal bone aneurysmal cyst. Case report].

Aneurysmal bone cysts are very rare in the calvaria. We are reporting a 23 year old white male with a very large right frontal aneurysmal bone cyst and a large follow-up period. The MRI findings--a multiloculated expansive tumor with fluid levels inside--are almost pathognomonic for this lesion. Radical surgery was curative for this patient.

Adult↗

Metachronous and multiple aneurysmal bone cysts: a rare variant of primary aneurysmal bone cysts.

In 1942, Jaffe and Lichtenstein introduced the term aneurysmal bone cyst (ABC). Primary ABC is characterized by the presence of spongy or multi-cameral cystic tissue filled with blood. The process is benign, but it is locally destructive and has a high propensity for recurrence. In this paper, we present the third case of multiple metachronous primary ABCs as a rare variant of ABC. We describe the 10-year history of a 12-year-old boy with metachronous multiple primary ABCs at five different sites (right proximal humerus, right ulna, bilateral distal radius and right lateral clavicle). Furthermore, our patient suffered from vascular malformations, such as aortic isthmus stenosis, hypoplastic thoraco-abdominal aorta and bilateral renal artery stenosis. To date, in contrast to solitary ABC, the multiple lesions have been found more frequently in male individuals. Using interphase cytogenetics, we analyzed three of five of the patient's ABCs and one of these was also analyzed by GTG-banding. No chromosomal abnormalities were found. Significantly, we excluded the missense mutation of codon 201 in guanine nucleotide-binding protein 1 gene consistently found in McCune-Albright syndrome (MAS) and in non-MAS cases of polyostotic fibrous dysplasia of bone with or without secondary ABC.

Blood Vessels↗