Sacroiliac joint involvement in osteochondromatosis: its prevalence and characteristics on cross-sectional imaging (original) (raw)
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In Vivo, 2021
Osteochondroma, the most common benign bone tumor, is a projection on the external surface of the bone, which can be sessile or pedunculated. 85% of osteochondromas present as solitary lesions, while 15% occur in the context of hereditary multiple exostoses (HME), a genetic disorder that is inherited in an autosomal dominant manner. Although often asymptomatic, symptoms may eventuate from compression of adjacent vessels or nerves, fractures, osseous deformities, bursa formation, or malignant transformation. Cartilage cap thickness >2 cm in adults or >3 cm in children as well as new onset of pain or growth, or rapid growth of the lesion, especially after the closure of the growth plate, might reflect cancerous transformation. Surgical resection is indicated for symptomatic lesions, complications, cosmetic reasons or malignant transformation. Excision of the tumor with free margin is the treatment of choice. Local recurrence is less than 2% if complete resection is achieved. Osteochondroma is the most common benign bone tumor accounting for 20-50% of benign bone tumors and 9% of all bone tumors (1-3). It is defined as a cartilage-capped bony projection on the external surface of a bone (2, 4). The lesion may grow during childhood, but no osteochondroma develops or enlarges after puberty (5, 6). Two pathognomonic features that ascertain the diagnosis are the presence of cortical and medullary continuity with the parent bone and a cartilage cap (7, 8). Most lesions are solitary, but approximately 15% of the patients have multiple tumors (6). Multiple osteochondromas are developed in the context of multiple hereditary exostoses (HME), a rare genetic disorder that is inherited in an autosomal dominant manner (2, 9). In the literature, HME has also been referred to as multiple hereditary osteochondromas, hereditary deforming dyschondroplasia, diaphyseal aclasis, and multiple cartilaginous exostoses (10). The majority of patients have a positive family history, while mutations in one of the EXT genes can be detected in about 90% of the HME cases (11, 12). Traditionally, most osteochondromas are incidentally diagnosed as they are symptomless (2, 4). Complications associated with osteochondromas are common and encompass compression of adjacent structures, fractures, osseous deformities, bursa formation with or without bursitis, and malignant transformation (2, 7, 13). Malignant transformation is more frequent in HME than solitary osteochondromas (6, 14). Plain radiographs are usually sufficient to diagnose osteochondromas, whereas cross-sectional imaging such as multidetector computed tomography (MDCT) and magnetic resonance imaging (MRI) are used to evaluate the cartilage cap, marrow continuity with the parent bone in complex areas, and complications (3, 4, 15). Asymptomatic lesions are treated solely with observation, while symptomatic lesions or lesions with suspicious imaging findings require surgical resection.
Hereditary cancer in clinical practice, 2004
Multiple Osteochondromas is an autosomal dominant disorder characterised by the presence of multiple osteochondromas and a variety of orthopaedic deformities. Two genes causative of Multiple Osteochondromas, Exostosin-1 (EXT1) and Exostosin-2 (EXT2), have been identified, which act as tumour suppressor genes. Osteochondroma can progress towards its malignant counterpart, secondary peripheral chondrosarcoma and therefore adequate follow-up of Multiple Osteochondroma patients is important in order to detect malignant transformation early.This review summarizes the considerable recent basic scientific and clinical understanding resulting in a multi-step genetic model for peripheral cartilaginous tumorigenesis. This enabled us to suggest guidelines for clinical management of Multiple Osteochondroma patients. When a patient is suspected to have Multiple Osteochondroma, the radiologic documentation, histology and patient history have to be carefully reviewed, preferably by experts and if ...
Imaging of Osteochondroma: Variants and Complications with Radiologic-Pathologic Correlation1
RadioGraphics, 2000
Osteochondroma represents the most common bone tumor and is a developmental lesion rather than a true neoplasm. It constitutes 20%-50% of all benign bone tumors and 10%-15% of all bone tumors. Its radiologic features are often pathognomonic and identically reflect its pathologic appearance. Osteochondromas are composed of cortical and medullary bone with an overlying hyaline cartilage cap and must demonstrate continuity with the underlying parent bone cortex and medullary canal. Osteochondromas may be solitary or multiple, the latter being associated with the autosomal dominant syndrome, hereditary multiple exostoses (HME). Complications associated with osteochondromas are more frequent with HME and include deformity (cosmetic and osseous), fracture, vascular compromise, neurologic sequelae, overlying bursa formation, and malignant transformation. Malignant transformation is seen in 1% of solitary osteochondromas and in 3%-5% of patients with HME. Continued lesion growth and a hyaline cartilage cap greater than 1.5 cm in thickness, after skeletal maturity, suggest malignant transformation. Variants of osteochondroma include subungual exostosis, dysplasia epiphysealis hemimelica, turret and traction exostoses, bizarre parosteal osteochondromatous proliferation, and florid reactive periostitis. Recognition of the radiologic spectrum of appearances of osteochondroma and its variants usually allows prospective diagnosis and differentiation of the numerous potential complications, thus helping guide therapy and improving patient management.
Manifestations and management of osteochondromas : A retrospective analysis of 382 patients
2006
Osteochondromas represent the most common primary bone tumours ; they reportedly represent 20-50% of all benign bone tumours and 10-15% of all bone tumours. Malignant transformation is their most severe complication. However, deformities and interference with major joint function are the most frequent complaints in patients with hereditary multiple osteochondroma. Treatment should therefore aim not only at surgical resection of the masses but also at prevention of deformities. This article reports observations made on 69 patients with hereditary multiple osteochondroma and 313 patients with solitary osteochondroma, with a mean follow-up of 13.4 years.
Osteochondroma of acromioclavicular joint
BMJ Case Reports, 2019
Osteochondroma is the most common type of benign bone tumour. It is a benign chondrogenic lesion derived from aberrant cartilage from the perichondral ring, and it commonly presents in the proximal humerus, proximal femur and knee. Osteochondroma is usually solitary but can be multiple with patients with hereditary multiple exostoses. Malignant changes happen in approximately 1% of cases. Osteochondroma usually causes local pain or swelling. We discuss a unique case of an osteochondroma that highlights the fact that osteochondroma can occur in the most unlikely places, and they should be properly visualised via radiography to evaluate any extensions and compromised surrounding structures before surgical intervention.
European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society, 2015
To report a case of solitary sacral osteochondroma without neurological symptoms and describe the en bloc excision of the tumour, as well as review the literature on osteochondroma involving the sacrum. Summary of the background data: although osteochondromas are among the most common benign tumours of the bone, they uncommonly involve the spine. Its occurence in the sacrum is rare, accounting for only 0.5 % of the osteochondromas involving spine. All previous cases of sacral osteochondroma have reported neurological symptoms on presentation. A 21-year-old male presented with a palpable, painless mass in the left side of the sacral region of 1 year duration, without neurological symptoms. Radiological studies showed a well-circumscribed lesion with bony osteoid within arising from the sacrum at S3-S4 level left to midline, with features suggestive of osteochondroma. The tumour was excised en bloc through posterior approach. A literature review of sacral osteochondroma was conducted ...
An Overview and Insights into Osteochondroma – A Rare Tumor of Bone and Cartilage
2016
Osteochondromas are the rare benign and malignant tumour of the growing bone, usually affecting the young adults. Solitary osteocartilaginous exostosis is more common than the hereditary multiple exostosis (HME). The first 3 decades of life has maximum chances of getting affected with osteochondroma and hardly occurs in craniofacial bones because of the fact that these bones are not formed by endochondral ossification. Most of the symptoms occur at the periphery of the bone tissues, with causes of osteochondroma being unknown. It involves in genetic condition and is associated with mutations of EXT1 or EXT2 genes. Diagnosis is difficult at symptomless stage, incidentally it is diagnosed when X-ray is carried out. Detection of tumor by ultra sound is accurate than other diagnosis process. No treatment is required other than regular monitoring of tumor. Standard allele specific PCR based techniques on osteochondroma showing both recombine and intact alleles were reported. The observat...
Osteochondroma at Unusual Sites: A Case Series Report
Journal of Orthopedics and Joint Surgery
No scars, sinuses, or distended veins. X-ray of the left ankle showed exostosis arising from the body of the talus (Fig. 1). Under tourniquet control, through posteromedial approach to ankle, skin incision made longitudinally midway between medial malleolus and Achilles tendon, deep fascia incised, and Achilles tendon retracted exposing deeper flexor compartment, fibro-osseous tunnel over flexor halluces longus tendon incised and posterior tibial artery with tibial nerve retracted medially: exostosis found arising from the body of talus removed using a bone chisel and sent for histopathology (Figs 2 and 3). Histopathological examination (HPE) confirmed the diagnosis as osteochondroma. In this case, surgical excision was performed in view of prevention of further progression of pes valgus deformity and to prevent neurovascular compromise. The patient was immobilized with below knee slab until suture removal on 12th postoperative day followed by below knee cast for another 2 weeks and then allowed weight-bearing. However the parents were informed regarding the chances of recurrence and malignant transformation in future. Case 2 A 55-year-old female presented with right elbow deformity and restricted range of movements of the right elbow for past 2 years. No history of trauma, previous surgeries, immobilization, or native treatment in the past. No scars, sinuses, or distended veins.
Osteochondroma. An Osteological Study
Background: Osteochondroma is the most common tumor of bone, accounting for approximately one third of benign lesions in the skeleton, is a broad (sessile) or narrow (pedunculated) skeletal protrusion comprised of marrow and cortical bone. Although it is benign, its biological behavior still has a malignant potential. Chondrosarcoma arising in osteochondroma has been recognized for many years. Osteochondromas might occur on any bone preformed in cartilage, they were especially seen on the long bones of the extremities, predominantly around the knee. In fact, the lower limb seemed to be at a higher risk of the tumor than the upper limb on the top two locations: distal part of the femur and proximal part of the tibia, however, the proximal humerus ranked the third place for solitary osteochondroma while the proximal fibula for multiples osteochondromas. Methods and Findings: A total of 20 skeletons and 18 cadavers of both sex, 16 males and 2 females, with different age groups were used, this study was carried out by routine dissection classes for undergraduate medical student´s in the Morphology Laboratory of the University of Pamplona during the years 2013-2016. In only one sample was found the presence of osteochondroma. The measurement of the osseous mass was of 7x3.5x1.5 cm, attached to the posterolateral aspect of the middle third of the femur. Conclusions: Osteochondroma is generally asymptomatic or discovered accidentally, but it can cause different symptoms depending on site of occurrence local swelling, bony deformities, fracture, bursa formation, arthritis and impingement on adjacent tendons, vascular or neurologic compromise, and dislocation The importance of correct treatment and follow-up of large osteochondromas to avoid complications such as further damage to neighboring neurovascular structures, and potential malignant degeneration. The present study describe a rare location from an osteochondroma in the femur diaphysis.