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Diagnostics (Basel, Switzerland) Jun 2023Benign tumours comprise the majority of primary vertebral tumours, and these are often found incidentally on imaging. Nonetheless, accurate diagnosis of these benign... (Review)
Review
Benign tumours comprise the majority of primary vertebral tumours, and these are often found incidentally on imaging. Nonetheless, accurate diagnosis of these benign lesions is crucial, in order to avoid misdiagnosis as more ominous malignant lesions or infection. Furthermore, some of these tumours, despite their benign nature, can have localised effects on the spine including neural compromise, or can be locally aggressive, thus necessitating active management. Haemangiomas and osteomas (enostosis) are the commonest benign tumours encountered. Others include osteoid osteoma, osteoblastoma, fibrous dysplasia, osteochondroma, chondroblastoma, haemangioma, simple bone cysts, aneurysmal bone cysts, giant cell tumours, eosinophilic granuloma and notochordal rests. The majority of lesions are asymptomatic; however, locally aggressive lesions (such as aneurysmal bone cysts or giant cell tumours) can present with nonspecific symptoms, such as back pain, neurological deficits and spinal instability, which may be indistinguishable from more commonly encountered mechanical back pain or malignant lesions including metastases. Hence, imaging, including radiography, computed tomography (CT) and magnetic resonance imaging (MRI), plays a critical role in diagnosis. Generally, most incidental or asymptomatic regions are conservatively managed or may not require any follow-up, while symptomatic or locally aggressive lesions warrant active interventions, which include surgical resection or percutaneous treatment techniques. Due to advances in interventional radiology techniques in recent years, percutaneous minimally invasive techniques such as radiofrequency ablation, sclerotherapy and cryoablation have played an increasing role in the management of these tumours with favourable outcomes. The different types of primary benign vertebral tumours will be discussed in this article with an emphasis on pertinent imaging features.
PubMed: 37370901
DOI: 10.3390/diagnostics13122006 -
Orthopedic Research and Reviews 2019Hereditary multiple exostoses (HME), also called hereditary multiple osteochondromas, is a rare genetic disorder characterized by multiple osteochondromas that grow near... (Review)
Review
Hereditary multiple exostoses (HME), also called hereditary multiple osteochondromas, is a rare genetic disorder characterized by multiple osteochondromas that grow near the growth plates of bones such as the ribs, pelvis, vertebrae and especially long bones. The disease presents with various clinical manifestations including chronic pain syndromes, restricted range of motion, limb deformity, short stature, scoliosis and neurovascular alteration. Malignant transformation of exostosis is rarely seen. The disease has no medical treatment and surgery is only recommended in symptomatic exostoses or in cases where a malignant transformation is suspected. HME is mainly caused by mutations and functional loss of the EXT1 and EXT2 genes which encode glycosyltransferases, an enzyme family involved in heparan sulfate (HS) synthesis. However, the peculiar molecular mechanism that leads to the structural changes of the cartilage and to osteochondroma formation is still being studied. Basic science studies have recently shown new insights about altering the molecular and cellular mechanism caused by HS deficiency. Pediatricians, geneticists and orthopedic surgeons play an important role in the study and treatment of this severe pathology. Despite the recent significant advances, we still need novel insights to better specify the role of HS in signal transduction. The purpose of this review was to analyze the most relevant aspects of HME from the literature review, give readers an important tool to understand its clinical features and metabolic-pathogenetic mechanism, and to identify an effective treatment method. We focused on the aspects of the disease related to clinical management and surgical treatment in order to give up-to-date information that could be useful for following best clinical practice.
PubMed: 31853203
DOI: 10.2147/ORR.S183979 -
Best Practice & Research. Clinical... Jun 2020Multiple hereditary exostoses (MHE) and enchondromatosis are rare multifocal benign disorders usually causing skeletal deformities appearing already in childhood. MHE is... (Review)
Review
Multiple hereditary exostoses (MHE) and enchondromatosis are rare multifocal benign disorders usually causing skeletal deformities appearing already in childhood. MHE is a dominant autosomal inherited disorder characterized by multiple osteochondromas (exostoses) growing outward from the metaphyses of long bones as well as from flat bones. They may cause reduced joint motion and pain due to tendon, muscle, and nerve compression. Enchondromatosis (or Ollier's disease) is a noninherited disorder characterized by the presence of multiple intraosseous enchondromas located asymmetrically in the skeleton and with a wide variation regarding location, size, and number ranging from the involvement of a single hand to the involvement of the entire skeleton. It can occur together with soft-tissue hemangiomas in Maffucci's syndrome. Clinical problems caused by the enchondromas are mainly related to skeletal deformities causing malalignment and restricted motion of joint. In both disorders, there is a risk of malignant transformation as well as secondary degenerative joint changes.
Topics: Child; Enchondromatosis; Exostoses, Multiple Hereditary; Humans
PubMed: 32253147
DOI: 10.1016/j.berh.2020.101505 -
The Pan African Medical Journal 2022
Topics: Bone Neoplasms; Humans; Osteochondroma; Ribs
PubMed: 35949455
DOI: 10.11604/pamj.2022.42.59.35217 -
Insights Into Imaging Jun 2023Bizarre parosteal osteochondromatous proliferation (BPOP) is a surface-based bone lesion belonging to the group of benign chondrogenic tumors. The aim of this review is... (Review)
Review
Bizarre parosteal osteochondromatous proliferation (BPOP) is a surface-based bone lesion belonging to the group of benign chondrogenic tumors. The aim of this review is to familiarize the readers with imaging features and differential diagnosis of BPOP, also addressing pathological presentation and treatment options. The peak of incidence of BPOP is in the third and fourth decades of life, although it can occur at any age. Hands are the most common location of BPOP (55%), followed by feet (15%) and long bones (25%). On imaging, BPOP appears as a well-marginated mass of heterotopic mineralization arising from the periosteal aspect of the bone. Typical features of BPOP are contiguity with the underlying bone and lack of cortico-medullary continuity, although cortical interruption and medullary involvement have been rarely reported. Histologically, BPOP is a benign bone surface lesion characterized by osteocartilaginous proliferation with disorganized admixture of cartilage with bizarre features, bone and spindle cells. Differential diagnosis includes both benign-such as florid reactive periostitis, osteochondroma, subungual exostosis, periosteal chondroma and myositis ossificans-and malignant lesions-such as periosteal chondrosarcoma and surface-based osteosarcoma. Treatment consists of surgical resection. Local recurrences are common and treated with re-excision.Critical relevance statement Bizarre parosteal osteochondromatous proliferation is a benign mineralized mass arising from the periosteal aspect of bone cortex. Multi-modality imaging characteristics, pathology features and differential diagnosis are here highlighted to familiarize the readers with this entity and offer optimal patient care.
PubMed: 37336832
DOI: 10.1186/s13244-023-01455-0 -
Orthopedic Nursing 2020
Topics: Aged; Humans; Knee; Magnetic Resonance Imaging; Male; Osteochondroma
PubMed: 32218012
DOI: 10.1097/NOR.0000000000000644 -
Indian Pediatrics Feb 2021
Topics: Bone Neoplasms; Exostoses; Humans; Nail Diseases; Osteochondroma; Toes
PubMed: 33632969
DOI: No ID Found -
Acta Otorrinolaringologica Espanola 2022
Topics: Humans; Osteochondroma; Hyoid Bone; Bone Neoplasms
PubMed: 36228988
DOI: 10.1016/j.otoeng.2021.10.002 -
Orthopedic Nursing
Topics: Humans; Bone Neoplasms; Osteochondroma; Tibia; Male; Adult; Incidental Findings; Knee Injuries
PubMed: 37989161
DOI: 10.1097/NOR.0000000000000990 -
The American Journal of Pathology Dec 2021Osteochondromas are cartilage-capped tumors that arise near growing physes and are the most common benign bone tumor in children. Osteochondromas can lead to skeletal... (Review)
Review
Osteochondromas are cartilage-capped tumors that arise near growing physes and are the most common benign bone tumor in children. Osteochondromas can lead to skeletal deformity, pain, loss of motion, and neurovascular compression. Currently, surgery is the only available treatment for symptomatic osteochondromas. Osteochondroma mouse models have been developed to understand the pathology and the origin of osteochondromas and develop therapeutic drugs. Several cartilage regulatory pathways have been implicated in the development of osteochondromas, such as bone morphogenetic protein, hedgehog, and WNT/β-catenin signaling. Retinoic acid receptor-γ is an important regulator of endochondral bone formation. Selective agonists for retinoic acid receptor-γ, such as palovarotene, have been investigated as drugs for inhibition of ectopic endochondral ossification, including osteochondromas. This review discusses the signaling pathways involved in osteochondroma pathogenesis and their possible interactions with the retinoid pathway.
Topics: Animals; Bone Neoplasms; Disease Models, Animal; Humans; Mice; Osteochondroma; Retinoids; Signal Transduction
PubMed: 34809786
DOI: 10.1016/j.ajpath.2021.08.003