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Pathologic Fracture Does Not Influence Prognosis in Stage IIB Osteosarcoma: a Case–Control Study Dongqing Zuo†, Longpo Zheng†, Wei Sun, Yingqi Hua* and Zhengdong Cai*
Zuo et al. World Journal of Surgical Oncology 2013, 11:148 http://www.wjso.com/content/11/1/148 WORLD JOURNAL OF SURGICAL ONCOLOGY RESEARCH Open Access Pathologic fracture does not influence prognosis in stage IIB osteosarcoma: a case–control study Dongqing Zuo†, Longpo Zheng†, Wei Sun, Yingqi Hua* and Zhengdong Cai* Abstract Objective: This study tested the implication of pathologic fractures on the prognosis in stage IIb osteosarcoma. Methods: A single center retrospective evaluation of clinical management and oncologic outcome was conducted with 15 pathological fracture patients (M:F = 10:5; age: mean 23.2, range 12–42) and 50 non-fracture patients between April 2002 and December 2010. These stage IIB osteosarcoma patients were matched for age, tumor site (femur, tibia, and humerus), and osteosarcoma subtype (i.e., control patients with osteosarcoma in the same sites as the fracture patients). All osteosarcoma patients with pathological fractures underwent brace or cast immobilization, adjuvant chemotherapy, and limb salvage surgery or amputation. Musculoskeletal Tumor Society (MSTS) functional scores were assessed. The mean follow-up time was 34.7 months (range, 8–47 months). Results: Following limb salvage surgery, no statistical differences were observed in major complications (fracture = 20.0%, control = 12.0%, P = 0.43) or local recurrence complications (fracture = 26.7%, control = 14.0%, P = 0.25). Overall 3-year survival rates of the fracture and control groups (66.7% and 75.3%, respectively) were not statistically different (P = 0.5190). Three-year disease-free survival rates of the fracture and control groups were 53.3% and 66.5%, respectively (P = 0.25). -
Evaluating and Treating the Reproductive System
18_Reproductive.qxd 8/23/2005 11:44 AM Page 519 CHAPTER 18 Evaluating and Treating the Reproductive System HEATHER L. BOWLES, DVM, D ipl ABVP-A vian , Certified in Veterinary Acupuncture (C hi Institute ) Reproductive Embryology, Anatomy and Physiology FORMATION OF THE AVIAN GONADS AND REPRODUCTIVE ANATOMY The avian gonads arise from more than one embryonic source. The medulla or core arises from the meso- nephric ducts. The outer cortex arises from a thickening of peritoneum along the root of the dorsal mesentery within the primitive gonadal ridge. Mesodermal germ cells that arise from yolk-sac endoderm migrate into this gonadal ridge, forming the ovary. The cells are initially distributed equally to both sides. In the hen, these germ cells are then preferentially distributed to the left side, and migrate from the right to the left side as well.58 Some avian species do in fact have 2 ovaries, including the brown kiwi and several raptor species. Sexual differ- entiation begins by day 5 in passerines and domestic fowl and by day 11 in raptor species. Differentiation of the ovary is characterized by development of the cortex, while the medulla develops into the testis.30,58 As the embryo develops, the germ cells undergo three phases of oogenesis. During the first phase, the oogonia actively divide for a defined time period and then stop at the first prophase of the first maturation division. During the second phase, the germ cells grow in size to become primary oocytes. This occurs approximately at the time of hatch in domestic fowl. During the third phase, oocytes complete the first maturation division to 18_Reproductive.qxd 8/23/2005 11:44 AM Page 520 520 Clinical Avian Medicine - Volume II become secondary oocytes. -
Metastatic Osseous Pain Control: Bone Ablation and Cementoplasty
328 Metastatic Osseous Pain Control: Bone Ablation and Cementoplasty Alexis Kelekis, MD, PhD, EBIR, FSIR1 Francois H. Cornelis, MD, PhD2 Sean Tutton, MD, FSIR3 Dimitrios Filippiadis, PhD, MSc, EBIR1 1 Division of Diagnostic and Interventional Radiology, 2nd Department of Address for correspondence Alexis Kelekis, MD, PhD, EBIR, FSIR, Radiology, University General Hospital “ATTIKON,” Athens, Greece Division of Diagnostic and Interventional Radiology, 2nd Department 2 Department of Radiology, Université Pierre et Marie Curie, Sorbonne of Radiology, University General Hospital “ATTIKON,” 1 Rimini street, Université, Tenon Hospital, Paris, France 12462 Athens, Greece (e-mail: [email protected]). 3 Division of Vascular and Interventional Radiology, Department of Radiology and Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin Semin Intervent Radiol 2017;34:328–336 Abstract Nociceptive and/or neuropathic pain can be present in all phases of cancer (early and metastatic) and are not adequately treated in 56 to 82.3% of patients. In these patients, radiotherapy achieves overall pain responses (complete and partial responses com- bined) up to 60 and 61%. On the other hand, nowadays, ablation is included in clinical guidelines for bone metastases and the technique is governed by level I evidence. Depending on the location of the lesion in the peripheral skeleton, either the Mirels Keywords scoring or the Harrington (alternatively the Levy) grading system can be used for ► ablation prophylactic fixation recommendation. As minimally invasive treatment options may ► cementoplasty be considered in patients with poor clinical status or limited life expectancy, the aim of ► pain this review is to detail the techniques proposed so far in the literature and to report the ► bone metastasis results in terms of safety and efficacy of ablation and cementoplasty (with or without ► interventional fixation) for bone metastases. -
Benign Fibro-Osseous Lesions Plus…
“Vision is the art of seeing things invisible.” Jonathan Swift 1667 - 1745 Benign Fibro-osseous Lesions Plus… Steven R. Singer, DDS [email protected] 212.305.5674 Benign Fibro-osseous Lesions Fibrous Dysplasia A group of lesions in which normal bone is Localized change in bone metabolism replaced initially by fibrous connective tissue Normal cancellous bone is replaced by Over time, the lesion is infiltrated by osteoid fibrous connective tissue and cementoid tissue The connective tissue contains varying amounts of abnormal bone with irregular This is a benign and idiopathic process trabeculae Trabeculae are randomly oriented. (Remember that normal trabeculae are aligned to respond to stress) Fibrous Dysplasia Fibrous Dysplasia Lesions may be solitary (monostotic) or Fibrous dysplasia is non-hereditary involve more than one bone (polyostotic) Caused by a mutation in a somatic cell. Monostotic form accounts for 70% of all Extent of lesions depends on the timing of cases the mutation. Polyostotic form is more common in the first If the mutation occurs earlier, the disease decade will be more widespread throughout the M=F except in McCune-Albright syndrome, body. An example is McCune-Albright which is almost exclusively found in females Syndrome 1 Fibrous Dysplasia Fibrous Dysplasia McCune-Albright Syndrome • Monostotic and polyostotic forms usually -Almost exclusively begins in the second decade of life females -Polyostotic fibrous • Slow, painless expansion of the jaws dysplasia • Patients may complain of swelling or have -
Works Neuroembryology
Swarthmore College Works Biology Faculty Works Biology 1-1-2017 Neuroembryology D. Darnell Scott F. Gilbert Swarthmore College, [email protected] Follow this and additional works at: https://works.swarthmore.edu/fac-biology Part of the Biology Commons Let us know how access to these works benefits ouy Recommended Citation D. Darnell and Scott F. Gilbert. (2017). "Neuroembryology". Wiley Interdisciplinary Reviews: Developmental Biology. Volume 6, Issue 1. DOI: 10.1002/wdev.215 https://works.swarthmore.edu/fac-biology/493 This work is brought to you for free by Swarthmore College Libraries' Works. It has been accepted for inclusion in Biology Faculty Works by an authorized administrator of Works. For more information, please contact [email protected]. HHS Public Access Author manuscript Author ManuscriptAuthor Manuscript Author Wiley Interdiscip Manuscript Author Rev Dev Manuscript Author Biol. Author manuscript; available in PMC 2018 January 01. Published in final edited form as: Wiley Interdiscip Rev Dev Biol. 2017 January ; 6(1): . doi:10.1002/wdev.215. Neuroembryology Diana Darnell1 and Scott F. Gilbert2 1University of Arizona College of Medicine 2Swarthmore College and University of Helsinki Abstract How is it that some cells become neurons? And how is it that neurons become organized in the spinal cord and brain to allow us to walk and talk, to see, recall events in our lives, feel pain, keep our balance, and think? The cells that are specified to form the brain and spinal cord are originally located on the outside surface of the embryo. They loop inward to form the neural tube in a process called neurulation. -
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. -
The Genetic Basis of Mammalian Neurulation
REVIEWS THE GENETIC BASIS OF MAMMALIAN NEURULATION Andrew J. Copp*, Nicholas D. E. Greene* and Jennifer N. Murdoch‡ More than 80 mutant mouse genes disrupt neurulation and allow an in-depth analysis of the underlying developmental mechanisms. Although many of the genetic mutants have been studied in only rudimentary detail, several molecular pathways can already be identified as crucial for normal neurulation. These include the planar cell-polarity pathway, which is required for the initiation of neural tube closure, and the sonic hedgehog signalling pathway that regulates neural plate bending. Mutant mice also offer an opportunity to unravel the mechanisms by which folic acid prevents neural tube defects, and to develop new therapies for folate-resistant defects. 6 ECTODERM Neurulation is a fundamental event of embryogenesis distinct locations in the brain and spinal cord .By The outer of the three that culminates in the formation of the neural tube, contrast, the mechanisms that underlie the forma- embryonic (germ) layers that which is the precursor of the brain and spinal cord. A tion, elevation and fusion of the neural folds have gives rise to the entire central region of specialized dorsal ECTODERM, the neural plate, remained elusive. nervous system, plus other organs and embryonic develops bilateral neural folds at its junction with sur- An opportunity has now arisen for an incisive analy- structures. face (non-neural) ectoderm. These folds elevate, come sis of neurulation mechanisms using the growing battery into contact (appose) in the midline and fuse to create of genetically targeted and other mutant mouse strains NEURAL CREST the neural tube, which, thereafter, becomes covered by in which NTDs form part of the mutant phenotype7.At A migratory cell population that future epidermal ectoderm. -
Orthopedic-Conditions-Treated.Pdf
Orthopedic and Orthopedic Surgery Conditions Treated Accessory navicular bone Achondroplasia ACL injury Acromioclavicular (AC) joint Acromioclavicular (AC) joint Adamantinoma arthritis sprain Aneurysmal bone cyst Angiosarcoma Ankle arthritis Apophysitis Arthrogryposis Aseptic necrosis Askin tumor Avascular necrosis Benign bone tumor Biceps tear Biceps tendinitis Blount’s disease Bone cancer Bone metastasis Bowlegged deformity Brachial plexus injury Brittle bone disease Broken ankle/broken foot Broken arm Broken collarbone Broken leg Broken wrist/broken hand Bunions Carpal tunnel syndrome Cavovarus foot deformity Cavus foot Cerebral palsy Cervical myelopathy Cervical radiculopathy Charcot-Marie-Tooth disease Chondrosarcoma Chordoma Chronic regional multifocal osteomyelitis Clubfoot Congenital hand deformities Congenital myasthenic syndromes Congenital pseudoarthrosis Contractures Desmoid tumors Discoid meniscus Dislocated elbow Dislocated shoulder Dislocation Dislocation – hip Dislocation – knee Dupuytren's contracture Early-onset scoliosis Ehlers-Danlos syndrome Elbow fracture Elbow impingement Elbow instability Elbow loose body Eosinophilic granuloma Epiphyseal dysplasia Ewing sarcoma Extra finger/toes Failed total hip replacement Failed total knee replacement Femoral nonunion Fibrosarcoma Fibrous dysplasia Fibular hemimelia Flatfeet Foot deformities Foot injuries Ganglion cyst Genu valgum Genu varum Giant cell tumor Golfer's elbow Gorham’s disease Growth plate arrest Growth plate fractures Hammertoe and mallet toe Heel cord contracture -
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. -
(Ollier Disease, Maffucci Syndrome) Is Not Caused by the PTHR1 Mutation
Enchondromatosis (Ollier Disease, Maffucci Syndrome) Is Not Caused by the PTHR1 Mutation p.R150C Bovée, J.V.M.G.; Rozeman, L.B.; Sangiorgi, L.; Briaire-de Bruijn, I.H.; Mainil-Varlet, P.; Bertoni, F.; ... ; Hogendoorn, P.C.W. Citation Bovée, J. V. M. G., Rozeman, L. B., Sangiorgi, L., Briaire-de Bruijn, I. H., Mainil-Varlet, P., Bertoni, F., … Hogendoorn, P. C. W. (2004). Enchondromatosis (Ollier Disease, Maffucci Syndrome) Is Not Caused by the PTHR1 Mutation p.R150C. Human Mutation, 24, 466-473. Retrieved from https://hdl.handle.net/1887/8144 Version: Not Applicable (or Unknown) License: Downloaded from: https://hdl.handle.net/1887/8144 Note: To cite this publication please use the final published version (if applicable). HUMAN MUTATION 24:466^473 (2004) RAPID COMMUNICATION Enchondromatosis (Ollier Disease, Maffucci Syndrome) Is Not Caused by the PTHR1 Mutation p.R150C Leida B. Rozeman,1 Luca Sangiorgi,2 Inge H. Briaire-de Bruijn,1 Pierre Mainil-Varlet,3 F. Bertoni,4 Anne Marie Cleton-Jansen,1 Pancras C.W. Hogendoorn,1 and Judith V.M.G. Bove´e1* 1Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands; 2Laboratory of Oncology Research, Rizzoli Orthopedic Institute, Bologna, Italy; 3Institute of Pathology, University of Bern, Bern, Switzerland; 4Department of Pathology, Rizzoli Orthopedic Institute, Bologna, Italy Communicated by Arnold Munnich Enchondromatosis (Ollier disease, Maffucci syndrome) is a rare developmental disorder characterized by multiple enchondromas. Not much is known about its molecular genetic background. Recently, an activating mutation in the parathyroid hormone receptor type 1 (PTHR1) gene, c.448C>T (p.R150C), was reported in two of six patients with enchondromatosis. -
This Guide to Chondrosarcoma Was Authored By
This Guide to chondrosarcoma was authored by DAVIDE DONATI M.D. LUCA SANGIORGI M.D., PH.D., Rizzoli Orthopaedic Institute, Bologna Italy & The MHE Research Foundation. What is chondrosarcoma? The term chondrosarcoma is used to define an heterogeneous group of lesions with diverse features and clinical behavior. Chondrosarcoma is a malignant cancer that results in abnormal bone and cartilage growth. People who have chondrosarcoma have a tumor growth starting from the medullary canal of a long and flat bone. However, in some cases the lesion can occur as an abnormal bony type of bump, which can vary in size and location. Primary chondrosarcoma (or conventional chondrosarcoma) usually develops centrally in a previously normal bone. Secondary chondrosarcoma is a chondrosarcoma arising from a benign precursor such as Exostoses, Osteochondromas or Enchondromas. Although rare, chondrosarcoma is the second most common primary bone cancer. The malignant cartilage cells begin growing within or on the bone (central chondrosarcoma) or, rarely, secondarily within the cartilaginous cap of a pre-existing Exostoses (peripheral chondrosarcoma). Cartilage is a type of dense connective tissue. It is composed of cells called chondrocytes which are dispersed in a firm gel-like substance, called the matrix. Cartilage is normally found in the joints, the rib cage, the ear, the nose, in the throat and between intervertebral disks. There are three main types of cartilage: hyaline, elastic and fibrocartilage. It is important to understand the difference between a benign and malignant cartilage tumor. Chondrosarcoma is a sarcoma, (i.e.) a malignant tumor of connective tissue. A chondroma, is the benign counterpart. Benign bone tumors do not spread to other tissues and organs, and are not life threatening.