RESUMEN
PURPOSE: To assess the prevalence of intraosseous cartilaginous lesions in patients with multiple osteochondromas based on total-body (TB) MRI examinations, used for screening purposes. SUBJECTS AND METHODS: Between 2013 and 2020, TB-MRI examinations were performed in 366 patients with proven multiple osteochondromas syndrome, to rule out malignant progression. For this study, presence, or absence of intraosseous central or eccentrical chondroid lesions, defined as lobulated lesions with low signal intensity on T1-weighted images, replacing bone marrow and high signal intensity equal to fluid on T2-weighted images in the bone marrow of the meta-diaphysis of (one of) the long bones, were recorded in the long bones as part of a TB-MRI protocol. RESULTS: In 62 patients out of the 366 MO patients (17%), one or more intraosseous chondroid lesions (either enchondroma or atypical cartilaginous tumor) were detected. The age of the patients at time of diagnosis ranged from 17 to 61 years (mean, 36). Size of the lesions varied from 4 to 69 mm (mean, 16.3 mm). The most common location was the proximal femur (n = 29), followed by the distal femur and proximal humerus (n = 18 and n = 10, respectively). In nine of the patients with an intraosseous chondroid lesion, a second and/or third TB-MRI were available during the period of evaluation (mean interval, 2.7 years between the exams). In none of these patients increase of these intraosseous lesions was noticed. CONCLUSION: Intraosseous chondroid lesions (enchondroma and ACT) appear to occur more frequently in MO patients than in the general population. TB-MRI allows to detect these, besides the identification of OC with suspicious features.
Asunto(s)
Neoplasias Óseas , Condroma , Exostosis Múltiple Hereditaria , Humanos , Adolescente , Adulto Joven , Adulto , Persona de Mediana Edad , Exostosis Múltiple Hereditaria/patología , Neoplasias Óseas/diagnóstico por imagen , Condroma/diagnóstico por imagen , Condroma/patología , Epífisis/patología , Imagen por Resonancia Magnética/métodosRESUMEN
Multiple hereditary exostoses (MHE) are a rare disorder characterized by the growth of bony protrusions. Elbow involvement is found in a considerable number of patients and varies from the presence of a simple osteochondroma to severe forearm deformities and radial head dislocation. Patients encounter a variety of symptoms, for example, pain, functional impairment, and cosmetic concerns. Several types of surgical procedures, therefore, can be offered, ranging from excision of symptomatic osteochondromas to challenging reconstructions. In this paper, we will discuss the essential basics of visualizing, planning, and treatment options of forearm deformities in MHE. In more detail, we will describe our current surgical technique as a salvage procedure for Masada type II forearm deformities in patients with MHE.
Asunto(s)
Artroplastia/métodos , Exostosis Múltiple Hereditaria/cirugía , Radio (Anatomía)/cirugía , Adolescente , Adulto , Niño , Articulación del Codo/cirugía , Femenino , Humanos , Masculino , Estudios Retrospectivos , Adulto JovenRESUMEN
Multiple osteochondroma, also known as hereditary multiple exostoses, is a relatively rare genetic disorder characterized by the presence of multiple osteochondromas. The disease is frequently painful, with restriction of the activities of daily living, problems with carrying out an occupation and performance at school. In addition, characteristic skeletal deformities and postural abnormalities of the joints very frequently occur in patients with this disorder. Malignant transformation of osteochondroma to chondrosarcoma occurs in 1-5% of the patients with multiple osteochondroma. Treatment of patients with multiple osteochondromas must be tuned to the problems experienced by the patient. Symptomatic osteochondromas are often an indication for excision; knowledge of the natural progression of the abnormality is important in this. Periodical screening is essential: in children to prevent or correct deformity and postural abnormalities and in adults to detect and treat malignant transformation of osteochondroma at an early stage.