Tumors of the Bones and Joints

Total Page:16

File Type:pdf, Size:1020Kb

Tumors of the Bones and Joints TUMORS OF THE BONES AND JOINTS 1. Introduction. 1 Structure of Normal Bone . 1 Development of Bone . 2 Intramembranous Ossification . 2 Endochondral Ossification. 3 Classification of Bone Tumors. 3 Incidence of Bone Tumors. 3 Methods of Biopsy. 4 Open Biopsy. 4 Needle Biopsy. 5 Fine-Needle Aspiration Biopsy. 5 Handling of Specimens . 6 Decalcification. 7 Assessment of Chemotherapy Effect. 7 Grading of Bone Tumors . 8 Staging of Bone Tumors. 8 2. Genetics of Bone Tumors. 11 Introduction. 11 Basic Principles of Cancer Cytogenetics . 11 Cytogenetic Analysis . 11 Specimen Requirements. 11 Cell Culture and Chromosome Banding. 11 Nomenclature. 12 Molecular Cytogenetics. 13 Specimen . 13 Probes. 15 Technical Variations. 15 Molecular Biology. 16 Oncogenes. 16 Oncogene Detection . 17 Gene Amplification. 17 Tumor Suppressor Genes. 20 DNA Mismatch Repair Gene Defects. 21 Microarray Technology . 22 3. Radiographic Appearance of Bone Tumors . 27 Location. 27 ix Tumors of the Bones and Joints Size . .. 28 Internal Margins. 28 Matrix Pattern . 29 Proliferative Bone Induction. 30 Interrupted Periosteal Reaction. 32 Radionuclide Bone Imaging. 32 Computerized Tomograms. 33 Magnetic Resonance Imaging . 34 4. Cartilaginous Lesions. 37 Osteochondroma. 37 Multiple Hereditary Exostoses. 46 Enchondroma. 46 Multiple Chondromas. 52 Periosteal Chondroma. 56 Soft Tissue Chondroma . 59 Chondroblastoma. 61 Chondromyxoid Fibroma . 67 Chondrosarcoma. 73 Chondrosarcoma of the Small Bones of the Hands and Feet. 86 Periosteal Chondrosarcoma. 87 Secondary Chondrosarcoma . 89 Dedifferentiated Chondrosarcoma . 91 Mesenchymal Chondrosarcoma . 99 Clear Cell Chondrosarcoma. 104 5. Bone-Forming Lesions . 119 Benign Bone-Forming Tumors. 119 Osteoid Osteoma. 119 Osteoblastoma. 126 Malignant Bone-Forming Tumors. 135 Osteosarcoma of Bone. 135 Medullary Osteosarcomas . 136 Conventional Osteosarcoma. 136 Osteosarcoma of Jawbones. 149 Osteosarcoma in Paget’s Disease . 150 Postradiation Sarcoma. 151 Osteosarcoma in Other Benign Condtions. 152 Multicentric Osteosarcoma . 154 Telangiectatic Osteosarcoma. 154 x Contents Small Cell Osteosarcoma. 158 Low-Grade Osteosarcoma . 160 Genetics and Other Special Techniques. 162 Genetic Predisposition to Osteosarcoma. 162 Prognostic Markers. 165 Other Potential Genetic Lesions . 168 Tumor Cytogenetics. 168 Treatment and Prognosis for Patients with Osteosarcomas. 169 Osteosarcomas of the Surface of Bone. 171 Parosteal Osteosarcoma. 171 Periosteal Osteosarcoma. 176 High-Grade Surface Osteosarcoma. 180 6. Fibrogenic Tumors . 193 Desmoplastic Fibroma. 193 Fibrosarcoma . 196 7. “Histiocytic” Tumors . 201 Benign Fibrous Histiocytoma. 201 Malignant Fibrous Histiocytoma. 202 8. Small Cell Malignancies. 209 Ewing’s Sarcoma. 209 Myeloma. 222 Multiple Myeloma. 222 Solitary Myeloma (Plasmacytoma) . 229 Osteosclerotic Myeloma. 230 Malignant Lymphoma. 232 9. Notochordal Tumors . 249 Chordoma . 249 Chondroid Chordoma. 256 Dedifferentiated Chordoma. 257 10. Vascular Tumors. 261 Benign Vascular Tumors. 261 Hemangioma. 261 Massive Osteolysis. 264 Glomus Tumor. 265 Malignant Vascular Tumors. 266 Angiosarcoma. 266 Epithelioid Hemangioendothelioma. 273 Hemangiopericytoma. 276 xi Tumors of the Bones and Joints 11. Giant Cell Tumor. 281 Giant Cell Tumor. 281 Malignancy in Giant Cell Tumors. 292 12. Adamantinoma of Long Bones. 299 Adamantinoma. 299 13. Miscellaneous Tumors . 309 Neurilemmoma of Bone. 309 Lipoma of Bone . 311 Liposarcoma. 313 Clear Cell Sarcoma. 314 Alveolar Soft Part Sarcoma. 315 Leiomyosarcoma. 316 14. Conditions that Simulate Primary Neoplasms of Bone. 321 Metastatic Malignancy. 321 Cystic Lesions of Bone that Simulate Neoplasms . 324 Aneurysmal Bone Cyst. 324 Simple Cyst. 330 Intraosseous Ganglion. 330 Epidermoid Cyst. ..
Recommended publications
  • Infiltrating Myeloid Cells Drive Osteosarcoma Progression Via GRM4 Regulation of IL23
    Published OnlineFirst September 16, 2019; DOI: 10.1158/2159-8290.CD-19-0154 RESEARCH BRIEF Infi ltrating Myeloid Cells Drive Osteosarcoma Progression via GRM4 Regulation of IL23 Maya Kansara 1 , 2 , Kristian Thomson 1 , Puiyi Pang 1 , Aurelie Dutour 3 , Lisa Mirabello 4 , Francine Acher5 , Jean-Philippe Pin 6 , Elizabeth G. Demicco 7 , Juming Yan 8 , Michele W.L. Teng 8 , Mark J. Smyth 9 , and David M. Thomas 1 , 2 ABSTRACT The glutamate metabotropic receptor 4 (GRM4 ) locus is linked to susceptibility to human osteosarcoma, through unknown mechanisms. We show that Grm4 − / − gene– targeted mice demonstrate accelerated radiation-induced tumor development to an extent comparable with Rb1 +/ − mice. GRM4 is expressed in myeloid cells, selectively regulating expression of IL23 and the related cytokine IL12. Osteosarcoma-conditioned media induce myeloid cell Il23 expression in a GRM4-dependent fashion, while suppressing the related cytokine Il12 . Both human and mouse osteosarcomas express an increased IL23:IL12 ratio, whereas higher IL23 expression is associated with worse survival in humans. Con- sistent with an oncogenic role, Il23−/− mice are strikingly resistant to osteosarcoma development. Agonists of GRM4 or a neutralizing antibody to IL23 suppressed osteosarcoma growth in mice. These fi ndings identify a novel, druggable myeloid suppressor pathway linking GRM4 to the proinfl ammatory IL23/IL12 axis. SIGNIFICANCE: Few novel systemic therapies targeting osteosarcoma have emerged in the last four decades. Using insights gained from a genome-wide association study and mouse modeling, we show that GRM4 plays a role in driving osteosarcoma via a non–cell-autonomous mechanism regulating IL23, opening new avenues for therapeutic intervention.
    [Show full text]
  • 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.
    [Show full text]
  • Primary Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: the MD Anderson Cancer Center Experience
    cancers Article Primary Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: The MD Anderson Cancer Center Experience Nidale Tarek 1,2,*, Rabih Said 3, Clark R. Andersen 4, Tina S. Suki 1, Jessica Foglesong 1,5 , Cynthia E. Herzog 1, Nizar M. Tannir 6, Shreyaskumar Patel 7, Ravin Ratan 7, Joseph A. Ludwig 7 and Najat C. Daw 1,* 1 Department of Pediatrics, the University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA; [email protected] (T.S.S.); [email protected] (J.F.); [email protected] (C.E.H.) 2 Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center, Beirut 1107, Lebanon 3 Department of Investigational Cancer Therapeutics, the University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA; [email protected] 4 Department of Biostatistics, the University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA; [email protected] 5 Division of Hematology, Oncology, Neuro-Oncology & Stem Cell Transplant, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL 60611, USA 6 Department of Genitourinary Medical Oncology, the University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA; [email protected] 7 Department of Sarcoma Medical Oncology, the University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA; [email protected] (S.P.); [email protected] (R.R.); [email protected] (J.A.L.) * Correspondence: [email protected] (N.T.); [email protected] (N.C.D.); Tel.: +1-713-792-6620 (N.C.D.) Received: 27 August 2020; Accepted: 5 October 2020; Published: 11 October 2020 Simple Summary: The Ewing sarcoma family of tumors (ESFT)s rarely originate in the kidneys and their treatment is significantly different from the other common kidney tumors.
    [Show full text]
  • Pathogenesis and Current Treatment of Osteosarcoma: Perspectives for Future Therapies
    Journal of Clinical Medicine Review Pathogenesis and Current Treatment of Osteosarcoma: Perspectives for Future Therapies Richa Rathore 1 and Brian A. Van Tine 1,2,3,* 1 Division of Medical Oncology, Washington University in St. Louis, St. Louis, MO 63110, USA; [email protected] 2 Division of Pediatric Hematology and Oncology, St. Louis Children’s Hospital, St. Louis, MO 63110, USA 3 Siteman Cancer Center, St. Louis, MO 63110, USA * Correspondence: [email protected] Abstract: Osteosarcoma is the most common primary malignant bone tumor in children and young adults. The standard-of-care curative treatment for osteosarcoma utilizes doxorubicin, cisplatin, and high-dose methotrexate, a standard that has not changed in more than 40 years. The development of patient-specific therapies requires an in-depth understanding of the unique genetics and biology of the tumor. Here, we discuss the role of normal bone biology in osteosarcomagenesis, highlighting the factors that drive normal osteoblast production, as well as abnormal osteosarcoma development. We then describe the pathology and current standard of care of osteosarcoma. Given the complex hetero- geneity of osteosarcoma tumors, we explore the development of novel therapeutics for osteosarcoma that encompass a series of molecular targets. This analysis of pathogenic mechanisms will shed light on promising avenues for future therapeutic research in osteosarcoma. Keywords: osteosarcoma; mesenchymal stem cell; osteoblast; sarcoma; methotrexate Citation: Rathore, R.; Van Tine, B.A. Pathogenesis and Current Treatment of Osteosarcoma: Perspectives for Future Therapies. J. Clin. Med. 2021, 1. Introduction 10, 1182. https://doi.org/10.3390/ Osteosarcomas are the most common pediatric and adult bone tumor, with more than jcm10061182 1000 new cases every year in the United States alone.
    [Show full text]
  • The Health-Related Quality of Life of Sarcoma Patients and Survivors In
    Cancers 2020, 12 S1 of S7 Supplementary Materials The Health-Related Quality of Life of Sarcoma Patients and Survivors in Germany—Cross-Sectional Results of A Nationwide Observational Study (PROSa) Martin Eichler, Leopold Hentschel, Stephan Richter, Peter Hohenberger, Bernd Kasper, Dimosthenis Andreou, Daniel Pink, Jens Jakob, Susanne Singer, Robert Grützmann, Stephen Fung, Eva Wardelmann, Karin Arndt, Vitali Heidt, Christine Hofbauer, Marius Fried, Verena I. Gaidzik, Karl Verpoort, Marit Ahrens, Jürgen Weitz, Klaus-Dieter Schaser, Martin Bornhäuser, Jochen Schmitt, Markus K. Schuler and the PROSa study group Includes Entities We included sarcomas according to the following WHO classification. - Fletcher CDM, World Health Organization, International Agency for Research on Cancer, editors. WHO classification of tumours of soft tissue and bone. 4th ed. Lyon: IARC Press; 2013. 468 p. (World Health Organization classification of tumours). - Kurman RJ, International Agency for Research on Cancer, World Health Organization, editors. WHO classification of tumours of female reproductive organs. 4th ed. Lyon: International Agency for Research on Cancer; 2014. 307 p. (World Health Organization classification of tumours). - Humphrey PA, Moch H, Cubilla AL, Ulbright TM, Reuter VE. The 2016 WHO Classification of Tumours of the Urinary System and Male Genital Organs—Part B: Prostate and Bladder Tumours. Eur Urol. 2016 Jul;70(1):106–19. - World Health Organization, Swerdlow SH, International Agency for Research on Cancer, editors. WHO classification of tumours of haematopoietic and lymphoid tissues: [... reflects the views of a working group that convened for an Editorial and Consensus Conference at the International Agency for Research on Cancer (IARC), Lyon, October 25 - 27, 2007]. 4. ed.
    [Show full text]
  • Osteoid Osteoma: Contemporary Management
    eCommons@AKU Section of Orthopaedic Surgery Department of Surgery 2018 Osteoid osteoma: Contemporary management Shahryar Noordin Aga Khan University, [email protected] Salim Allana Emory University Kiran Hilal Aga Khan University, [email protected] Riaz Hussain Lukhadwala Aga Khan University, [email protected] Anum Sadruddin Pidani Aga Khan University, [email protected] See next page for additional authors Follow this and additional works at: https://ecommons.aku.edu/pakistan_fhs_mc_surg_orthop Part of the Orthopedics Commons, Radiology Commons, and the Surgery Commons Recommended Citation Noordin, S., Allana, S., Hilal, K., Lukhadwala, R. H., Pidani, A. S., Ud Din, N. (2018). Osteoid osteoma: Contemporary management. Orthopedic Reviews, 10(3), 108-119. Available at: https://ecommons.aku.edu/pakistan_fhs_mc_surg_orthop/92 Authors Shahryar Noordin, Salim Allana, Kiran Hilal, Riaz Hussain Lukhadwala, Anum Sadruddin Pidani, and Nasir Ud Din This article is available at eCommons@AKU: https://ecommons.aku.edu/pakistan_fhs_mc_surg_orthop/92 Orthopedic Reviews 2018; volume 10:7496 Osteoid osteoma: Contemporary management Epidemiology Correspondence: Shahryar Noordin, Orthopaedic Surgery, Aga Khan University, Osteoid osteoma accounts for around Karachi, Pakistan. Shahryar Noordin,1 Salim Allana,2 5% of all bone tumors and 11% of benign Tel.: 021.3486.4384. 4 Kiran Hilal,3 Naila Nadeem,3 bone tumors. Osteoid osteoma is the third E-mail: [email protected] Riaz Lakdawala,1 Anum Sadruddin,4 most common biopsy analyzed benign bone 5 tumor after osteochondroma and nonossify- Key words: Osteoid osteoma; tumor; benign; Nasir Uddin imaging; pathogenesis; management. 1 ing fibroma. Two to 3% of excised primary Orthopaedic Surgery, Aga Khan bone tumors are osteoid osteomas.5 Males University, Karachi, Pakistan; Contributions: SN, SA, study design, data col- are more commonly affected with an lection, manuscript writing; KH, NU, data col- 2 5 Department of Epidemiology, Rollins approximate male/female ratio of 2 to 1.
    [Show full text]
  • Leg Pain and Swelling in a 22-Year-Old Man
    CLINICAL ORTHOPAEDICS AND RELATED RESEARCH Number 448, pp. 259–266 © 2006 Lippincott Williams & Wilkins Orthopaedic • Radiology • Pathology Conference Leg Pain and Swelling in a 22-Year-Old Man Mustafa H. Khan, MD*; Ritesh Darji, MD†; Uma Rao, MD‡; and Richard McGough, MD* HISTORY AND PHYSICAL EXAMINATION which precluded him from participating in any sports, and night pain. The patient localized the pain over the anterior The patient was a 22-year man who presented with right aspect of the midtibia. He denied any history of trauma. He leg pain and swelling that had increased during the last 6 required regular doses of oxycodone for the past year to years. He complained of pain with walking and running, achieve adequate pain relief. His past medical history was unremarkable. From the *Departments of Orthopedic Surgery, †Radiology, and ‡Pathology; On physical examination a large anterior pretibial bony University of Pittsburgh Medical Center, Pittsburgh, PA. mass was palpable. No other masses were palpable in the Each author certifies that he or she has no commercial associations (eg, consultancies, stock ownership, equity interest, patent/licensing arrange- extremities and there was no evidence of lymphadenopa- ments, etc) that might pose a conflict of interest in connection with the thy. Active and passive range of motion testing and neu- submitted article. rovascular examination was in normal limits. Each author certifies that his or her institution has approved the reporting of this case report and that all investigations were conducted in conformity with Plain radiographs and MRI scans of the leg, along ethical principles of research. with CT scan of the leg and chest, were obtained Correspondence to: Richard McGough, MD, Department of Orthopedic Sur- (Figs 1–3).
    [Show full text]
  • The Role of Cytogenetics and Molecular Diagnostics in the Diagnosis of Soft-Tissue Tumors Julia a Bridge
    Modern Pathology (2014) 27, S80–S97 S80 & 2014 USCAP, Inc All rights reserved 0893-3952/14 $32.00 The role of cytogenetics and molecular diagnostics in the diagnosis of soft-tissue tumors Julia A Bridge Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE, USA Soft-tissue sarcomas are rare, comprising o1% of all cancer diagnoses. Yet the diversity of histological subtypes is impressive with 4100 benign and malignant soft-tissue tumor entities defined. Not infrequently, these neoplasms exhibit overlapping clinicopathologic features posing significant challenges in rendering a definitive diagnosis and optimal therapy. Advances in cytogenetic and molecular science have led to the discovery of genetic events in soft- tissue tumors that have not only enriched our understanding of the underlying biology of these neoplasms but have also proven to be powerful diagnostic adjuncts and/or indicators of molecular targeted therapy. In particular, many soft-tissue tumors are characterized by recurrent chromosomal rearrangements that produce specific gene fusions. For pathologists, identification of these fusions as well as other characteristic mutational alterations aids in precise subclassification. This review will address known recurrent or tumor-specific genetic events in soft-tissue tumors and discuss the molecular approaches commonly used in clinical practice to identify them. Emphasis is placed on the role of molecular pathology in the management of soft-tissue tumors. Familiarity with these genetic events
    [Show full text]
  • A Rare Case of Peripheral Primitive Neuroectodermal Tumor in Palate
    Surgery and Rehabilitation Case Report ISSN: 2514-5959 A rare case of peripheral primitive neuroectodermal tumor in palate Rajul Ranka1*, Anuj Jain2, Amol Gadbail3 and Minal Chaudhary1 1Oral Pathology and Microbiology, Sharad Pawar Dental College and Hospital, Sawangi(M), Wardha, India 2Department of Trauma and Emergency Medicine, All India Institute of Medical Sciences, Bhopal, India 3Department of Dentistry, Indira Gandhi Government Medical College, Nagpur, India Abstract Primitive Neuroectodermal Tumor (PNET) is an aggressive round cell malignant tumor which belongs to Ewing’s Sarcoma family. Peripheral PNET is rare in head and neck region and even rare in palate with handful of cases reported till date. A case of Peripheral PNET of palate in a 32-year-old female pa tient along with its clinico-pathologic, radiologic and immunohistologic features as well as its management is reported here. We emphasize the need of histopathology and immunohistochemistry (IHC) for early diagnosis of such aggressive tumors to improve the prognosis of patient by providing timely management. Introduction Case report A neuroectodermal tumor is a tumor of the central or peripheral A thirty-two years old female patient of Indian origin reported nervous system. They are divided into two categories viz. Group to the Out-Patient Department of our institute with a complaint of (I) tumors, such as the pituitary adenomas and carcinoid tumors, painful ulcer on her palate since five months. The ulcer was gradually represent tumors that show predominantly epithelial differenti ation. progressive but has now suddenly increased in size since few days. It Group (II) tumors, which incorporate malignant melanoma, olfactory was associated with dull aching, intermittent and localized pain which neuroblastoma, Ewing’s sar coma (EWS) and primitive neuroectodermal aggravated on mastication and relieved with time.
    [Show full text]
  • Craniofacial Chondrosarcomas: Imaging Findings in 15 Untreated Cases
    165 Craniofacial Chondrosarcomas: Imaging Findings in 15 Untreated Cases Ya-Yen Lee1 Radiographic findings of 15 untreated chondrosarcomas of the cranial and facial Pamela Van Tassel bones were reviewed. These tumors have a propensity to occur in the wall of a maxillary sinus, at the junction of sphenoid and ethmoid sinuses and vomer, and at the undersur­ face of the sphenoid bone. Because of its slow-growing nature, chondrosarcomas tend to be large, multi lobulated, and sharply demarcated when detected. Frequent bone changes are a combination of erosion and destruction, with sharp transitional zones and absent periosteal reaction. Tumor matrix calcifications, not necessarily chondroid, are almost always present. Both CT and MR may be necessary for thorough evaluation of tumor extent. Chondrosarcoma, a malignant but usually slow-growing cartilaginous tumor, constitutes approximately 11 % of malignant bone tumors [1] but rarely occurs in the craniofacial region . Because of its propensity to occur in the deep facial structures or base of the skull, the true extent and origin of the tumor may be overlooked if not properly evaluated radiographically. We review a relatively large series of craniofacial chondrosarcomas and discuss the differential diagnosis and choice of imaging technique. Materials and Methods This retrospective radiologic review was based on the pretreatment radiographic studies of 15 patients with craniofacial chondrosarcomas seen at our institution over a period of 40 years , excluding three intracranial dural chondrosarcomas, which are to be reported sepa­ rately. An attempt was also made to correlate the radiographic findings with the hi stologic grades of the tumors. The ages of the patients ranged from 10 to 73 years , with a mean of 40 years.
    [Show full text]
  • Ewing-Like Adamantinoma
    Orthopaedics & Traumatology: Surgery & Research (2012) 98, 845—849 View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Elsevier - Publisher Connector Available online at www.sciencedirect.com CASE REPORT Ewing-like adamantinoma a,∗ a a b a M.M. Hamdane , L. Charfi , M. Driss , H. Nouri , R. Sellami-Dhouib , a b a K. Mrad ,M. Mestiri , K. Ben Romdhane a Histopathology Department, Salah Azaiez Institute, Bab Saâdoun, 1006 Tunis, Tunisia b Adult Orthopaedics Department, Mohamed-Kassab Orthopaedics Institute, Ksar Saïd, 2010 Mannouba, Tunisia Accepted: 6 June 2012 KEYWORDS Summary The Ewing-like variation of adamantinoma is a rare entity, leading to challenge its Adamantinoma; differential diagnosis, notably with Ewing’s sarcoma. We are reporting a case of a 20-year-old Sarcoma; male who presented with swelling in the left leg that had progressed over a 2-year period. X-rays Ewing; revealed a tumour in the tibia that was intracortical, osteolytic, multilocular and invaded the Pathology; soft tissues. A surgical biopsy was performed. Histopathology examination showed a tumour Immunohistochemistry growth with small round cells expressing CD99. A diagnosis of Ewing’s sarcoma was made. Since the patient declined surgical treatment, chemotherapy was administered. Tw o years later, the patient returned because the tumour had grown in size. A second biopsy was performed. Microscopic evaluation showed a tumour growth with osteofibrous and epithelial components, which expressed pankeratin and vimentin, but was negative for CD99. A diagnosis of Ewing-like adamantinoma was made. © 2012 Published by Elsevier Masson SAS. Introduction We are reporting here on a rare case of Ewing-like adamanti- noma.
    [Show full text]
  • Adamantinoma
    Cancer Association of South Africa (CANSA) Fact Sheet on Adamantinoma Introduction Bone cancer develops in the skeletal system and destroys tissue. It can spread to distant organs, such as the lungs. The usual treatment for bone cancer is surgery, and it has a good outlook following early diagnosis and management. Adamantinoma The two main types are primary and secondary bone cancer. In primary bone cancer, cancer develops in the cells of the bone. Secondary bone cancer occurs when cancers that develop elsewhere spread, or metastasize, to the bones. Adamantinoma Adamantinoma is a rare bone cancer. It makes up less than 1% of bone cancers. Most of the time, adamantinoma grows in the lower leg. It often starts as a lump in the middle of the shinbone (tibia) or the calf bone (fibula). Adamantinoma can also occur in the jaw bone (mandible) or, sometimes, the forearm, hands, or feet. An adamantinoma lump can be painful, swollen and red, and can cause movement problems. [Picture Credit: Adamantinoma] Adamantinoma mostly occurs in the second to fifth decade. The median patient age is 25 to 35 years, with a range from 2 years to 86 years. It is slightly more common in men than women, with a ratio of 5:4. It rarely occurs in children. Adamantinoma is a serious condition. Treatment is important for survival but it is possible to make a full recovery. Limaiem, F., Tafti, D. & Malik, A. 2020. “Adamantinoma is a rare low-grade malignant bone tumor of uncertain histogenesis which occurs commonly in the diaphyses and metaphyses of the tibia.
    [Show full text]