Original Article | Volume 6 | Issue 1 | JBST Jan-April 2020 | Page 17-20| DOI: 10.13107/jbst.2020.v06i01.009
Author: Nitin Shetty, Prateek Hegde, Hemant Singh, Ashish Gulia
Department of Radio-Diagnosis, Tata Memorial Centre, Homi Bhabha National Institute (HBNI), Mumbai, India.
Department of Surgical Oncology, Tata Memorial Centre, HBNI, Mumbai, India.
Address of Correspondence
Dr. Ashish Gulia
Tata Memorial Centre, Homi Bhabha National Institute, Dr. E Borges Road, Parel, Mumbai – 400 012, India.
E- mail: email@example.com
Background: Aneurysmal bone cyst (ABC) is a rare, benign, expansile lesion that produces blood-filled cavities inside the bone. The term, ‘Aneurysmal bone cyst’ was first time used by Jaffe and Lichtenstein in 1942 . The name is a misnomer, as they are neither aneurysmal nor are, they truly cystic, as these lesions do not have an endothelial lined cyst wall. ABC is a disease of childhood or young adulthood with a median age of 13 years, with an incidence of 0.14 per 105 individuals with slight female preponderance .
Pathophysiology: ABC usually present as a solitary lesion either as a primary neoplasm (translocation driven) or a secondary lesion arising adjacent to previous bony lesions like giant cell tumours (GCT), osteoblastomas, chondroblastomas . Few authors have proposed post traumatic hypothesis whereas others feel it could due to an hemodynamic disturbance especially venous impedance.
Primary ABCs: Primary ABC is one where it occurs in a bone without any previously known lesion. But now there has been identification of TRE17 also known as USP6 (ubiquitin-specific protease 6) gene on chromosome 17p13.Pathogenesis of some primary ABC involves transcriptional up-regulation of USP6 when there is the chromosomal translocation t(16;17)(q22;p13) which fuses the promoter region of the osteoblast cadherin 11 gene (CDH11) on chromosome 16q22 to the entire coding sequence of the ubiquitin protease USP6 gene on chromosome 17p13 .
Secondary ABCs: Approximately one third of the ABCs appear secondary to other pre-existing bone tumours, most commonly from GCT, which accounts for 19-39% of these cases . Other common precursor lesions are chondroblastomas, chondromyxoid fibroma, fibrous dysplasia, osteoblastomas, haemangioendothelioma, angioma, fibroxanthoma (nonossifying fibroma), solitary bone cyst, fibrous histiocytoma, eosinophilic granuloma, radiation osteitis, osteosarcoma, trauma (including fracture), fibrosarcoma and even metastatic carcinoma [3, 5].
ABCs have been likened to a “blood-filled sponge”, composed of blood-filled, anastomosing, cavernomatous spaces, separated by a cyst like wall composed of fibroblasts, myofibroblasts, osteoclast like giant cells, osteoid and woven bone.
In approximately one third of cases, a characteristic reticulated lacy chondroid like material, described as a calcified matrix with a chondroid aura, is seen . These are called as “solid aneurysmal bone cyst”. The term “solid aneurysmal bone cyst,” was coined by Sanerkin et al. in 1983 .
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|How to Cite this article: Shetty N, Hegde P, Singh H, Gulia A. Aneurysmal Bone Cyst – Review. Journal of Bone and Soft Tissue Tumors January-April 2020;6(1): 17-20.|