Osteochondroma: Ignore Or Investigate?
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Bone and Soft Tissue Tumors Have Been Treated Separately
EPIDEMIOLOGY z Sarcomas are rare tumors compared to other BONE AND SOFT malignancies: 8,700 new sarcomas in 2001, with TISSUE TUMORS 4,400 deaths. z The incidence of sarcomas is around 3-4/100,000. z Slight male predominance (with some subtypes more common in women). z Majority of soft tissue tumors affect older adults, but important sub-groups occur predominantly or exclusively in children. z Incidence of benign soft tissue tumors not known, but Fabrizio Remotti MD probably outnumber malignant tumors 100:1. BONE AND SOFT TISSUE SOFT TISSUE TUMORS TUMORS z Traditionally bone and soft tissue tumors have been treated separately. z This separation will be maintained in the following presentation. z Soft tissue sarcomas will be treated first and the sarcomas of bone will follow. Nowhere in the picture….. DEFINITION Histological z Soft tissue pathology deals with tumors of the classification connective tissues. of soft tissue z The concept of soft tissue is understood broadly to tumors include non-osseous tumors of extremities, trunk wall, retroperitoneum and mediastinum, and head & neck. z Excluded (with a few exceptions) are organ specific tumors. 1 Histological ETIOLOGY classification of soft tissue tumors tumors z Oncogenic viruses introduce new genomic material in the cell, which encode for oncogenic proteins that disrupt the regulation of cellular proliferation. z Two DNA viruses have been linked to soft tissue sarcomas: – Human herpes virus 8 (HHV8) linked to Kaposi’s sarcoma – Epstein-Barr virus (EBV) linked to subtypes of leiomyosarcoma z In both instances the connection between viral infection and sarcoma is more common in immunosuppressed hosts. -
Applications of Chondrocyte-Based Cartilage Engineering: an Overview
Hindawi Publishing Corporation BioMed Research International Volume 2016, Article ID 1879837, 17 pages http://dx.doi.org/10.1155/2016/1879837 Review Article Applications of Chondrocyte-Based Cartilage Engineering: An Overview Abdul-Rehman Phull,1 Seong-Hui Eo,1 Qamar Abbas,1 Madiha Ahmed,2 and Song Ja Kim1 1 Department of Biological Sciences, College of Natural Sciences, Kongju National University, Gongjudaehakro 56, Gongju 32588, Republic of Korea 2Department of Pharmacy, Quaid-i-Azam University, Islamabad 45320, Pakistan Correspondence should be addressed to Song Ja Kim; [email protected] Received 14 May 2016; Revised 24 June 2016; Accepted 26 June 2016 Academic Editor: Magali Cucchiarini Copyright © 2016 Abdul-Rehman Phull et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Chondrocytes are the exclusive cells residing in cartilage and maintain the functionality of cartilage tissue. Series of biocomponents such as different growth factors, cytokines, and transcriptional factors regulate the mesenchymal stem cells (MSCs) differentiation to chondrocytes. The number of chondrocytes and dedifferentiation are the key limitations in subsequent clinical application of the chondrocytes. Different culture methods are being developed to overcome such issues. Using tissue engineering and cell based approaches, chondrocytes offer prominent therapeutic option specifically in orthopedics for cartilage repair and to treat ailments such as tracheal defects, facial reconstruction, and urinary incontinence. Matrix-assisted autologous chondrocyte transplantation/implantation is an improved version of traditional autologous chondrocyte transplantation (ACT) method. An increasing number of studies show the clinical significance of this technique for the chondral lesions treatment. -
Advances in the Pathogenesis and Possible Treatments for Multiple Hereditary Exostoses from the 2016 International MHE Conference
Connective Tissue Research ISSN: 0300-8207 (Print) 1607-8438 (Online) Journal homepage: https://www.tandfonline.com/loi/icts20 Advances in the pathogenesis and possible treatments for multiple hereditary exostoses from the 2016 international MHE conference Anne Q. Phan, Maurizio Pacifici & Jeffrey D. Esko To cite this article: Anne Q. Phan, Maurizio Pacifici & Jeffrey D. Esko (2018) Advances in the pathogenesis and possible treatments for multiple hereditary exostoses from the 2016 international MHE conference, Connective Tissue Research, 59:1, 85-98, DOI: 10.1080/03008207.2017.1394295 To link to this article: https://doi.org/10.1080/03008207.2017.1394295 Published online: 03 Nov 2017. Submit your article to this journal Article views: 323 View related articles View Crossmark data Citing articles: 1 View citing articles Full Terms & Conditions of access and use can be found at https://www.tandfonline.com/action/journalInformation?journalCode=icts20 CONNECTIVE TISSUE RESEARCH 2018, VOL. 59, NO. 1, 85–98 https://doi.org/10.1080/03008207.2017.1394295 PROCEEDINGS Advances in the pathogenesis and possible treatments for multiple hereditary exostoses from the 2016 international MHE conference Anne Q. Phana, Maurizio Pacificib, and Jeffrey D. Eskoa aDepartment of Cellular and Molecular Medicine, Glycobiology Research and Training Center, University of California, San Diego, La Jolla, CA, USA; bTranslational Research Program in Pediatric Orthopaedics, Division of Orthopaedic Surgery, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA ABSTRACT KEYWORDS Multiple hereditary exostoses (MHE) is an autosomal dominant disorder that affects about 1 in 50,000 Multiple hereditary children worldwide. MHE, also known as hereditary multiple exostoses (HME) or multiple osteochon- exostoses; multiple dromas (MO), is characterized by cartilage-capped outgrowths called osteochondromas that develop osteochondromas; EXT1; adjacent to the growth plates of skeletal elements in young patients. -
Exostoses, Enchondromatosis and Metachondromatosis; Diagnosis and Management
Acta Orthop. Belg., 2016, 82, 102-105 ORIGINAL STUDY Exostoses, enchondromatosis and metachondromatosis; diagnosis and management John MCFARLANE, Tim KNIGHT, Anubha SINHA, Trevor COLE, Nigel KIELY, Rob FREEMAN From the Department of Orthopaedics, Robert Jones Agnes Hunt Hospital, Oswestry, UK We describe a 5 years old girl who presented to the region of long bones and are composed of a carti- multidisciplinary skeletal dysplasia clinic following lage lump outside the bone which may be peduncu- excision of two bony lumps from her fingers. Based on lated or sessile, the knee is the most common clinical examination, radiolographs and histological site (1,10). An isolated exostosis is a common inci- results an initial diagnosis of hereditary multiple dental finding rarely requiring treatment. Disorders exostosis (HME) was made. Four years later she developed further lumps which had the radiological associated with exostoses include HME, Langer- appearance of enchondromas. The appearance of Giedion syndrome, Gardner syndrome and meta- both exostoses and enchondromas suggested a possi- chondromatosis. ble diagnosis of metachondromatosis. Genetic testing Enchondroma are the second most common be- revealed a splice site mutation at the end of exon 11 on nign bone tumour characterised by the formation of the PTPN11 gene, confirming the diagnosis of meta- hyaline cartilage in the medulla of a bone. It occurs chondromatosis. While both single or multiple exosto- most frequently in the hand (60%) and then the feet. ses and enchondromas occur relatively commonly on The typical radiological features are of a well- their own, the appearance of multiple exostoses and defined lucent defect with endosteal scalloping and enchondromas together is rare and should raise the differential diagnosis of metachondromatosis. -
Studies on Brain and Spinal Cord Tumors
Studies on Brain and Spinal Cord Tumors Chapter 1 Osteochondroma of the Spine Iraj Lotfinia Professor of Neurosurgery, Tabriz Universsity of medical science, Tabriz, Iran. Fax: 00984113340830; Email: [email protected] Abstract Osteochondroma (OC) is the most common benign tumor of the bones, and it remains the most common precursor for secondary chondrosarcoma, which often occurs in the long bones’ metaphyseal areas. Rarely, it is also found in the spine. This tumor comprises a cartilage capped bone projection and is observed in both solitary and multiple forms. In many cases, the lesion can be definitively diagnosed according to radiological characteristics, but the rarity of these lesions in the spine, gradual onset of symptoms, and the frequent lack of observation of lesions in plain radiography may delay the diagnosis or cause misdiagnosis. These lesions are be- nign and do not risk the patient’s life; however, they rarely may be found to be a malignant degeneration that transformed into chondrosarcoma. When the lesion has led to clinical symptoms or has faced the patient with cosmetic challenges, or when definitive diagnosis is unknown, treatment is required. The primary treatment is the surgical removal of the lesion. Timely diagnosis and complete resection of the le- sion using surgery lead to complete recovery and prevent recurrence. 1. Introduction According to the World Health Organization’s (WHO’s) definition in 2002, osteocar- tilaginous exostosis are benign bone neoplasms covered by a cartilaginous cap created at the outer surface of the bone by endochondral ossification [1]. Osteochondroma (OC) is the most common benign primary tumor of the bone. -
SKELETAL DYSPLASIA Dr Vasu Pai
SKELETAL DYSPLASIA Dr Vasu Pai Skeletal dysplasia are the result of a defective growth and development of the skeleton. Dysplastic conditions are suspected on the basis of abnormal stature, disproportion, dysmorphism, or deformity. Diagnosis requires Simple measurement of height and calculation of proportionality [<60 inches: consideration of dysplasia is appropriate] Dysmorphic features of the face, hands, feet or deformity A complete physical examination Radiographs: Extremities and spine, skull, Pelvis, Hand Genetics: the risk of the recurrence of the condition in the family; Family evaluation. Dwarf: Proportional: constitutional or endocrine or malnutrition Disproportion [Trunk: Extremity] a. Height < 42” Diastrophic Dwarfism < 48” Achondroplasia 52” Hypochondroplasia b. Trunk-extremity ratio May have a normal trunk and short limbs (achondroplasia), Short trunk and limbs of normal length (e.g., spondylo-epiphyseal dysplasia tarda) Long trunk and long limbs (e.g., Marfan’s syndrome). c. Limb-segment ratio Normal: Radius-Humerus ratio 75% Tibia-Femur 82% Rhizomelia [short proximal segments as in Achondroplastics] Mesomelia: Dynschondrosteosis] Acromelia [short hands and feet] RUBIN CLASSIFICATION 1. Hypoplastic epiphysis ACHONDROPLASTIC Autosomal Dominant: 80%; 0.5-1.5/10000 births Most common disproportionate dwarfism. Prenatal diagnosis: 18 weeks by measuring femoral and humeral lengths. Abnormal endochondral bone formation: zone of hypertrophy. Gene defect FGFR fibroblast growth factor receptor 3 . chromosome 4 Rhizomelic pattern, with the humerus and femur affected more than the distal extremities; Facies: Frontal bossing; Macrocephaly; Saddle nose Maxillary hypoplasia, Mandibular prognathism Spine: Lumbar lordosis and Thoracolumbar kyphosis Progressive genu varum and coxa valga Wedge shaped gaps between 3rd and 4th fingers (trident hands) Trident hand 50%, joint laxity Pathology Lack of columnation Bony plate from lack of growth Disorganized metaphysis Orthopaedics 1. -
Imaging in Osteogenesis Imperfecta
Paediatr Croat. 2017;61:122-8 PREGLED / REVIEW www.paedcro.com http://dx.doi.org/10.13112/PC.2017.17 Imaging in osteogenesis imperfecta Igor Borić, Renata Prpić Vučković* Osteogenesis imperfecta (OI) is a congenital genetic disorder with skeletal or extra-skeletal manifestations. Phenotypic features and mode of inheritance, clinical features, and radiographic fi ndings make the basis for the currently accepted classifi cation system of OI. The antenatal and postnatal diagnosis of the disease using diff erent radiographic methods (plain radiography, ultrasonography, computed tomography and magnetic resonance imaging) is described and characteristic appearances of bone and other defor- mities are analyzed. Distinctive bone manifestations of OI are illustrated using typical examples. Finally, we give a comment on diff erential diagnosis. Key words: osteogenesis imperfecta, radiography, imaging Osteogenesis imperfecta (OI) is a congenital, genetic disor- od: radiography, ultrasonography (US), computed tomogra- der of collagen type I synthesis that involves connective tis- phy (CT) and magnetic resonance imaging (MRI) (3, 7, 8). sues and bones, and is characterized by increased bone The preferred radiographic examination for initial investiga- fragility and decreased bone density. There is extreme varia- tion of OI is plain radiography because most of the imaging tion in clinical symptoms based on genetics and subtypes characteristics of this disease are apparent on plain radio- including blue sclera, dental fragility, and hearing loss. De- graphs. pending on the disease severity, bone fragility may lead to Prenatal ultrasonography plays a role in the diagnosis of OI; perinatal death or can cause severe deformities that persist typical fi ndings include fractures, decreased calvarial ossifi ca- into adulthood (1, 2). -
The Epiphyseal Plate: Physiology, Anatomy, and Trauma*
3 CE CREDITS CE Article The Epiphyseal Plate: Physiology, Anatomy, and Trauma* ❯❯ Dirsko J. F. von Pfeil, Abstract: This article reviews the development of long bones, the microanatomy and physiology Dr.med.vet, DVM, DACVS, of the growth plate, the closure times and contribution of different growth plates to overall growth, DECVS and the effect of, and prognosis for, traumatic injuries to the growth plate. Details on surgical Veterinary Specialists of Alaska Anchorage, Alaska treatment of growth plate fractures are beyond the scope of this article. ❯❯ Charles E. DeCamp, DVM, MS, DACVS athologic conditions affecting epi foramen. Growth factors and multipotent Michigan State University physeal (growth) plates in imma stem cells support the formation of neo ture animals may result in severe natal bone consisting of a central marrow P 2 orthopedic problems such as limb short cavity surrounded by a thin periosteum. ening, angular limb deformity, or joint The epiphysis is a secondary ossifica incongruity. Understanding growth plate tion center in the hyaline cartilage forming anatomy and physiology enables practic the joint surfaces at the proximal and distal At a Glance ing veterinarians to provide a prognosis ends of the bones. Secondary ossification Bone Formation and assess indications for surgery. Injured centers can appear in the fetus as early Page E1 animals should be closely observed dur as 28 days after conception1 (TABLE 1). Anatomy of the Growth ing the period of rapid growth. Growth of the epiphysis arises from two Plate areas: (1) the vascular reserve zone car Page E2 Bone Formation tilage, which is responsible for growth of Physiology of the Growth Bone is formed by transformation of con the epiphysis toward the joint, and (2) the Plate nective tissue (intramembranous ossifica epiphyseal plate, which is responsible for Page E4 tion) and replacement of a cartilaginous growth in bone length.3 The epiphyseal 1 Growth Plate Closure model (endochondral ossification). -
Multiple Hereditary Exostoses
Multiple Hereditary Exostoses Dror Paley MD, FRCSC Medical Director, Paley Orthopedic and spine Institute, St. Mary’s Medical Center, West palm Beach, FL David Feldman, MD Director, Spinal deformity center, Paley Orthopedic and spine Institute, St. Mary’s Medical Center, West palm Beach, FL Multiple hereditary exostoses (MHE), also known as multiple osteochondromas (MO), is an autosomal dominant skeletal disorder. Approximately 10–20% of individuals are a result of a spontaneous mutation while the rest are familial. The prevalence of MHE/MO is 1/50 000. There are two known genes found to cause MHE/MO, EXT1 located on chromosome 8q23-q24 and EXT2 located on chromosome 11p11-p12. In 10–15% of the patients, no mutation can be located by current methods of genetic testing.1–7 These mutations are scattered across both genes. EXT1/EXT2 is essential for the biosynthesis of heparan sulfate (HS). HS production in patients’ cells is reduced by 50% or more.8–19 MHE/MO is associated with characteristic progressive skeletal deformities of the extremities and shortening of one or both the sides, leading to limb length discrepancy (LLD) and short stature.20–28 Two bone segments, such as the lower leg or forearm, are at greater risk of problems due to either osteochondromas (OCs) from one or both bones impinging on or deforming the other bone or a primary issue of altered growth causing one bone to grow at a faster or slower rate. OCs can also affect joint motion due to impingement of an OC with the opposite side of the joint or subluxation/dislocation related to deformity, impingement, and incongruity.20,24,26,29,30 OCs can also cause nerve or vessel entrapment and/or compression, including the spinal cord and nerve roots. -
New Therapeutic Targets in Rare Genetic Skeletal Diseases
Briggs MD, Bell PA, Wright MJ, Pirog KA. New therapeutic targets in rare genetic skeletal diseases. Expert Opinion on Orphan Drugs 2015, 3(10), 1137- 1154. Copyright: ©2015 The Author(s). Published by Taylor & Francis. DOI link to article: http://dx.doi.org/10.1517/21678707.2015.1083853 Date deposited: 16/10/2015 This work is licensed under a Creative Commons Attribution 4.0 International License Newcastle University ePrints - eprint.ncl.ac.uk Expert Opinion on Orphan Drugs ISSN: (Print) 2167-8707 (Online) Journal homepage: http://www.tandfonline.com/loi/ieod20 New therapeutic targets in rare genetic skeletal diseases Michael D Briggs PhD , Peter A Bell PhD, Michael J Wright MB ChB MSc FRCP & Katarzyna A Pirog PhD To cite this article: Michael D Briggs PhD , Peter A Bell PhD, Michael J Wright MB ChB MSc FRCP & Katarzyna A Pirog PhD (2015) New therapeutic targets in rare genetic skeletal diseases, Expert Opinion on Orphan Drugs, 3:10, 1137-1154, DOI: 10.1517/21678707.2015.1083853 To link to this article: http://dx.doi.org/10.1517/21678707.2015.1083853 © 2015 The Author(s). Published by Taylor & Francis. Published online: 24 Sep 2015. Submit your article to this journal Article views: 102 View related articles View Crossmark data Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ieod20 Download by: [Newcastle University] Date: 16 October 2015, At: 07:31 Review New therapeutic targets in rare genetic skeletal diseases † Michael D Briggs , Peter A Bell, Michael J Wright & Katarzyna A Pirog † 1. Introduction Newcastle University, Institute of Genetic Medicine, International Centre for Life, Newcastle-upon-Tyne, UK 2. -
Inability of Low Oxygen Tension to Induce Chondrogenesis in Human Infrapatellar Fat Pad Mesenchymal Stem Cells
fcell-09-703038 July 20, 2021 Time: 15:26 # 1 ORIGINAL RESEARCH published: 26 July 2021 doi: 10.3389/fcell.2021.703038 Inability of Low Oxygen Tension to Induce Chondrogenesis in Human Infrapatellar Fat Pad Mesenchymal Stem Cells Samia Rahman1, Alexander R. A. Szojka1, Yan Liang1, Melanie Kunze1, Victoria Goncalves1, Aillette Mulet-Sierra1, Nadr M. Jomha1 and Adetola B. Adesida1,2* 1 Laboratory of Stem Cell Biology and Orthopedic Tissue Engineering, Division of Orthopedic Surgery and Surgical Research, Department of Surgery, University of Alberta, Edmonton, AB, Canada, 2 Division of Otolaryngology-Head and Neck Surgery, Department of Surgery, University of Alberta Hospital, Edmonton, AB, Canada Objective: Articular cartilage of the knee joint is avascular, exists under a low oxygen tension microenvironment, and does not self-heal when injured. Human infrapatellar fat pad-sourced mesenchymal stem cells (IFP-MSC) are an arthroscopically accessible source of mesenchymal stem cells (MSC) for the repair of articular cartilage defects. Human IFP-MSC exists physiologically under a low oxygen tension (i.e., 1–5%) Edited by: microenvironment. Human bone marrow mesenchymal stem cells (BM-MSC) exist Yi Zhang, physiologically within a similar range of oxygen tension. A low oxygen tension of Central South University, China 2% spontaneously induced chondrogenesis in micromass pellets of human BM-MSC. Reviewed by: Dimitrios Kouroupis, However, this is yet to be demonstrated in human IFP-MSC or other adipose tissue- University of Miami, United States sourced MSC. In this study, we explored the potential of low oxygen tension at 2% to Dhirendra Katti, Indian Institute of Technology Kanpur, drive the in vitro chondrogenesis of IFP-MSC. -
Bone Cartilage Dense Fibrous CT (Tendons & Nonelastic Ligaments) Dense Elastic CT (Elastic Ligaments)
Chapter 6 Content Review Questions 1-8 1. The skeletal system consists of what connective tissues? Bone Cartilage Dense fibrous CT (tendons & nonelastic ligaments) Dense elastic CT (elastic ligaments) List the functions of these tissues. Bone: supports the body, protects internal organs, provides levers on which muscles act, store minerals, and produce blood cells. Cartilage provides a model for bone formation and growth, provides a smooth cushion between adjacent bones, and provides firm, flexible support. Tendons attach muscles to bones and ligaments attach bone to bone. 2. Name the major types of fibers and molecules found in the extracellular matrix of the skeletal system. Collagen Proteoglycans Hydroxyapatite Water Minerals How do they contribute to the functions of tendons, ligaments, cartilage and bones? The collagen fibers of tendons and ligaments make these structures very tough, like ropes or cables. Collagen makes cartilage tough, whereas the water-filled proteoglycans make it smooth and resistant. As a result, cartilage is relatively rigid, but springs back to its original shape if it is bent or slightly compressed, and it is an excellent shock absorber. The extracellular matrix of bone contains collagen and minerals, including calcium and phosphate. Collagen is a tough, ropelike protein, which lends flexible strength to the bone. The mineral component gives the bone compression (weight-bearing) strength. Most of the mineral in the bone is in the form of hydroxyapatite. 3. Define the terms diaphysis, epiphysis, epiphyseal plate, medullary cavity, articular cartilage, periosteum, and endosteum. Diaphysis – the central shaft of a long bone. Epiphysis – the ends of a long bone. Epiphyseal plate – the site of growth in bone length, found between each epiphysis and diaphysis of a long bone and composed of cartilage.