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Skeletal Radiology Oct 2021Solitary osteochondromas are common, benign hyaline cartilage-capped exostoses that primarily arise from the metaphyses of long and flat bones. Diaphyseal aclasis is an... (Review)
Review
Solitary osteochondromas are common, benign hyaline cartilage-capped exostoses that primarily arise from the metaphyses of long and flat bones. Diaphyseal aclasis is an autosomal dominant condition resulting from EXT1 or EXT2 gene mutations and is characterized by multifocal osteochondromas. These can result in a wide spectrum of complications, such as skeletal deformity, neurological and vascular complications, adventitial bursa formation, fracture, and rarely malignant transformation to peripheral chondrosarcoma. In this review, we outline in detail the multimodality imaging features of DA and its associated complications.
Topics: Bone Neoplasms; Chondrosarcoma; Diagnostic Imaging; Exostoses, Multiple Hereditary; Humans; Mutation; Osteochondroma
PubMed: 33791832
DOI: 10.1007/s00256-021-03770-3 -
Matrix Biology : Journal of the... Oct 2018Heparan sulfate (HS) is an essential component of cell surface and matrix proteoglycans (HS-PGs) that include syndecans and perlecan. Because of their unique structural... (Review)
Review
Heparan sulfate (HS) is an essential component of cell surface and matrix proteoglycans (HS-PGs) that include syndecans and perlecan. Because of their unique structural features, the HS chains are able to specifically interact with signaling proteins -including bone morphogenetic proteins (BMPs)- via their HS-binding domain, regulating protein availability, distribution and action on target cells. Hereditary Multiple Exostoses (HME) is a rare pediatric disorder linked to germline heterozygous loss-of-function mutations in EXT1 or EXT2 that encode Golgi-resident glycosyltransferases responsible for HS synthesis, resulting in a systemic HS deficiency. HME is characterized by cartilaginous/bony tumors -called osteochondromas or exostoses- that form within perichondrium in long bones, ribs and other elements. This review examines most recent studies in HME, framing them in the context of classic studies. New findings show that the spectrum of EXT mutations is larger than previously realized and the clinical complications of HME extend beyond the skeleton. Osteochondroma development requires a somatic "second hit" that would complement the germline EXT mutation to further decrease HS production and/levels at perichondrial sites of osteochondroma induction. Cellular studies have shown that the steep decreases in local HS levels: derange the normal homeostatic signaling pathways keeping perichondrium mesenchymal; cause excessive BMP signaling; and provoke ectopic chondrogenesis and osteochondroma formation. Data from HME mouse models have revealed that systemic treatment with a BMP signaling antagonist markedly reduces osteochondroma formation. In sum, recent studies have provided major new insights into the molecular and cellular pathogenesis of HME and the roles played by HS deficiency. These new insights have led to the first ever proof-of-principle demonstration that osteochondroma formation is a druggable process, paving the way toward the creation of a clinically-relevant treatment.
Topics: Animals; Bone Morphogenetic Proteins; Disease Models, Animal; Exostoses, Multiple Hereditary; Heparitin Sulfate; Humans; Mice; Mutation; N-Acetylglucosaminyltransferases; Signal Transduction
PubMed: 29277722
DOI: 10.1016/j.matbio.2017.12.011 -
RoFo : Fortschritte Auf Dem Gebiete Der... Mar 2021Chondrogenic tumors are the most frequent primary bone tumors. Malignant chondrogenic tumors represent about one quarter of malignant bone tumors. Benign chondrogenic... (Review)
Review
BACKGROUND
Chondrogenic tumors are the most frequent primary bone tumors. Malignant chondrogenic tumors represent about one quarter of malignant bone tumors. Benign chondrogenic bone tumors are frequent incidental findings at imaging. Radiological parameters may be helpful for identification, characterization, and differential diagnosis.
METHODS
Systematic PubMed literature research. Identification and review of studies analyzing and describing imaging characteristics of chondrogenic bone tumors.
RESULTS AND CONCLUSIONS
The 2020 World Health Organization (WHO) classification system differentiates between benign, intermediate (locally aggressive or rarely metastasizing), and malignant chondrogenic tumors. On imaging, typical findings of differentiated chondrogenic tumors are lobulated patterns with a high signal on T2-weighted magnetic resonance imaging (MRI) and ring- and arc-like calcifications on conventional radiography and computed tomography (CT). Depending on the entity, the prevalence of this chondrogenic pattern differs. While high grade tumors may be identified due to aggressive imaging patterns, the differentiation between benign and intermediate grade chondrogenic tumors is challenging, even in an interdisciplinary approach.
KEY POINTS
· The WHO defines benign, intermediate, and malignant chondrogenic bone tumors. · Frequent benign tumors: osteochondroma and enchondroma; Frequent malignant tumor: conventional chondrosarcoma. · Differentiation between enchondroma versus low-grade chondrosarcoma is challenging for radiologists and pathologists. · Pain, deep scalloping, cortical destruction, bone expansion, soft tissue component: favor chondrosarcoma. · Potential malignant transformation of osteochondroma: progression after skeletal maturity, cartilage cap thickness (> 2 cm adult; > 3 cm child). · Potentially helpful advanced imaging methods: Dynamic MRI, texture analysis, FDG-PET/CT.
CITATION FORMAT
· Engel H, Herget GW, Füllgraf H et al. Chondrogenic Bone Tumors: The Importance of Imaging Characteristics. Fortschr Röntgenstr 2021; 193: 262 - 274.
Topics: Adult; Bone Neoplasms; Child; Chondroma; Chondrosarcoma; Humans; Osteochondroma; Positron Emission Tomography Computed Tomography
PubMed: 33152784
DOI: 10.1055/a-1288-1209 -
The Journal of Pediatrics Aug 2014
Topics: Bone Neoplasms; Child; Exostoses; Female; Humans; Nail Diseases; Osteochondroma; Toes
PubMed: 24787537
DOI: 10.1016/j.jpeds.2014.03.046 -
Journal of the American Veterinary... Feb 2018
Topics: Animals; Bone Neoplasms; Cervical Vertebrae; Diagnosis, Differential; Dog Diseases; Dogs; Fatal Outcome; Female; Magnetic Resonance Imaging; Osteochondroma; Pain; Spinal Cord Compression
PubMed: 29393733
DOI: 10.2460/javma.252.4.423 -
Joint Bone Spine Mar 2022
Topics: Bone Neoplasms; Cervical Vertebrae; Humans; Osteochondroma
PubMed: 34715354
DOI: 10.1016/j.jbspin.2021.105305 -
Radiologic Clinics of North America Jan 2015The perivertebral space extends from the skull base to the mediastinum and is delineated by the deep layer of the deep cervical fascia. The different tissue types,... (Review)
Review
The perivertebral space extends from the skull base to the mediastinum and is delineated by the deep layer of the deep cervical fascia. The different tissue types, including muscles, bones, nerves, and vascular structures, give rise to the various disorders that can be seen in this space. This article defines the anatomy of the perivertebral space, guides lesion localization, discusses different disease processes arising within this space, and reviews the best imaging approaches.
Topics: Bone Cysts, Aneurysmal; Diagnostic Imaging; Fascia; Humans; Lemierre Syndrome; Magnetic Resonance Imaging; Neck; Neoplasms, Nerve Tissue; Osteochondroma; Spine; Tendinopathy; Tomography, X-Ray Computed; Vascular Diseases
PubMed: 25476179
DOI: 10.1016/j.rcl.2014.09.008 -
Clinical Radiology Dec 2014Osteochondromas are common developmental benign bone lesions, which rarely involve the spine. In a narrow space, such as the spinal canal, they may potentially lead to... (Review)
Review
Osteochondromas are common developmental benign bone lesions, which rarely involve the spine. In a narrow space, such as the spinal canal, they may potentially lead to serious complications. Moreover, because this condition is fairly rare in the spine, it may be confused with other entities. The objective of this review is to describe the typical imaging findings of this rare, but occasionally significant condition of the spine.
Topics: Humans; Magnetic Resonance Imaging; Osteochondroma; Spinal Neoplasms; Spine; Tomography, X-Ray Computed
PubMed: 25282617
DOI: 10.1016/j.crad.2014.08.017 -
Der Radiologe Jun 2016Primary cartilage-forming tumors of the bone are frequent entities in the daily work of skeletal radiologists. This article describes the correlation of pathology and... (Review)
Review
Primary cartilage-forming tumors of the bone are frequent entities in the daily work of skeletal radiologists. This article describes the correlation of pathology and radiology in cartilage-forming skeletal tumors, in particular, enchondroma, osteochondroma, periosteal chondromas, chondroblastoma and various forms of chondrosarcoma. After reading, the radiologist should be able to deduce the different patterns of cartilage tumors on radiographs, CT, and MRI from the pathological aspects. Differentiation of enchondroma and chondrosarcoma is a frequent diagnostic challenge. Some imaging parameters, e. g., deep cortical scalloping (more than two thirds of the cortical thickness), cortical destruction, or a soft-tissue mass, are features of a sarcoma. Osteochondromas are bony protrusions with a continuous extension of bone marrow from the parent bone, the host cortical bone runs continuously from the osseous surface of the tumor into the shaft of the osteochondroma and the osteochondroma has a cartilage cap. Chondromyxoid fibromas are well-defined lytic and eccentric lesions of the metaphysis of the long bones, with nonspecific MRI findings. Chondroblastomas have a strong predilection for the epiphysis of long tubular bones and develop an intense perifocal bone marrow edema. Dedifferentiated chondrosarcomas are bimorphic lesions with a low-grade chondrogenic component and a high-grade noncartilaginous component. Most chondrogenic tumors have a predilection with regard to site and age at manifestation.
Topics: Bone Neoplasms; Chondroma; Chondrosarcoma; Diagnosis, Differential; Evidence-Based Medicine; Humans; Image Enhancement; Magnetic Resonance Imaging; Osteochondroma; Tomography, X-Ray Computed
PubMed: 27233920
DOI: 10.1007/s00117-016-0112-z -
Japanese Journal of Radiology Jul 2022The purpose of this study was to determine imaging features of reactive bursitis secondary to osteochondroma.
PURPOSE
The purpose of this study was to determine imaging features of reactive bursitis secondary to osteochondroma.
MATERIALS AND METHODS
Fourteen patients with reactive bursitis secondary to osteochondroma were retrospectively reviewed.
RESULTS
The 14 patients were 11 males and 3 females ranging in age from 18 to 67 years (mean, 33.6 years). The locations were as follows: scapula (n = 2), lesser trochanter (n = 6), greater trochanter (n = 1), distal femur (n = 2), distal fibula (n = 1), iliac bone (n = 2). On CT, six bursae were hypodense and 1 was isodense compared to muscle. All bursae had a thickened wall, 6 contained multiple villous projections and septa. Five bursae contained a few calcifications. On enhanced CT, the bursa displayed mild enhancement of the wall, villous projections, and septa. On MRI, one bursa was hypointense, three were hyperintense, and 5 were isointense relative to muscles on T1-weighted images. All bursae were hyperintense relative to muscles on T2-weighted images. All bursae had a thickened wall, eight contained multiple villous projections and septa. In five bursae, a few fatty villous projections can be seen within the bursa. On enhanced MRI, all bursae displayed avid enhancement of the bursal wall, villous projections, and septa.
CONCLUSIONS
Reactive bursitis secondary to osteochondroma most often occurred in young male adults. The most common involved site is lesser trochanter. The imaging features is that of an irregular, thick-walled fluid-filled bursa overlying an osteochondroma that containing multiple villous projections and septa. Calcification and/or lipoma arborescens may be developed within the bursa. On contrast-enhanced images, the bursa shows enhancement of the bursal wall, villous projections and septa.
Topics: Adolescent; Adult; Aged; Bone Neoplasms; Bursa, Synovial; Bursitis; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Osteochondroma; Retrospective Studies; Young Adult
PubMed: 35224687
DOI: 10.1007/s11604-022-01255-4