Bone & Soft Tissue
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A Rare Bone Tumor
OPEN ACCESS L E T T E R T O T H E E D I T O R Periosteal Desmoplastic Fibroma of Radius: A Rare Bone Tumor Aniqua Saleem1,* Hira Saleem2 1 Radiology Department, District Head Quarters Hospital, Rawalpindi Medical University, Rawalpindi 2 Department of Surgery, Shifa International Hospital, Islamabad. Correspondence*: Dr. Aniqua Saleem, Radiology Department, District Head Quarters Hospital, Rawalpindi Medical University, Rawalpindi E-mail: [email protected] © 2019, Saleem et al, Submitted: 05-04-2019 Accepted: 09-06-2019 Conflict of Interest: None Source of Support: Nil This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. DEAR SIR Desmoplastic fibroma is an extremely rare tumor of enhancement on post contrast images and with adjacent bone with a reported incidence of 0.11 % of all primary bone involvement as was evident by focal cortical inter- bone tumors. The most common site of involvement is ruption, mild endosteal thickening and irregularity and mandible (reported incidence 22% of all Desmoplastic also mild ulnar shaft remodeling (Fig. 3a, 3b). To further fibroma cases) followed by metaphysis of long bones. characterize the lesion, Tc99 MDP (methylene diphos- Involvement of forearm especially involving periosteum phonate) bone scan was also performed which showed is seldom reported. Prompt diagnosis and adequate active bone involvement in left distal radial shaft. management is important for limb salvage and restora- tion of limb function. [1-3] An 11-year-old boy presented with painful mild swelling of left forearm for a month, with no significant past med- ical history or any history of trauma. -
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. -
Primary Desmoid Tumor of the Small Bowel: a Case Report and Literature Review
Open Access Case Report DOI: 10.7759/cureus.4915 Primary Desmoid Tumor of the Small Bowel: A Case Report and Literature Review Peter A. Ebeling 1 , Tristan Fun 1 , Katherine Beale 1 , Robert Cromer 2 , Jason W. Kempenich 1 1. Surgery, University of Texas Health Science Center at San Antonio, San Antonio, USA 2. Surgery, Keesler U.S. Air Force Medical Center, Biloxi, USA Corresponding author: Peter A. Ebeling, [email protected] Abstract Desmoid tumors, also known as aggressive fibromatosis, are fibromuscular neoplasms that arise from mesenchymal cell lines. They may occur in almost all soft tissue compartments. Primary desmoids of the small bowel are rare but potentially serious tumors presenting unique challenges to the general surgeon. We present one case of a 59-year-old man presenting with three months of abdominal distension secondary to a small bowel desmoid. Computed tomography of the abdomen showed an 18-cm mass in the mid-abdomen without obvious vital structure encasement. Percutaneous biopsy of the mass indicated a desmoid tumor. The patient underwent a successful elective exploratory laparotomy with resection and primary enteric anastomosis. Final pathology revealed the mass to be a primary desmoid of the small bowel. His post- operative course was uneventful. At two years after surgery, he is symptom free, and there is no evidence of disease recurrence. Due to the rare nature of primary small bowel desmoids, there are few specific care pathways outlined. This is a challenging pathology to treat that often requires a multidisciplinary team of surgical and medical oncologists. Categories: General Surgery, Oncology Keywords: desmoid, small bowel, resection, aggressive fibromatosis Introduction Desmoid tumors, also known as aggressive fibromatosis, are fibromuscular neoplasms that arise from mesenchymal cell lines. -
Soft Tissue Cytopathology: a Practical Approach Liron Pantanowitz, MD
4/1/2020 Soft Tissue Cytopathology: A Practical Approach Liron Pantanowitz, MD Department of Pathology University of Pittsburgh Medical Center [email protected] What does the clinician want to know? • Is the lesion of mesenchymal origin or not? • Is it begin or malignant? • If it is malignant: – Is it a small round cell tumor & if so what type? – Is this soft tissue neoplasm of low or high‐grade? Practical diagnostic categories used in soft tissue cytopathology 1 4/1/2020 Practical approach to interpret FNA of soft tissue lesions involves: 1. Predominant cell type present 2. Background pattern recognition Cell Type Stroma • Lipomatous • Myxoid • Spindle cells • Other • Giant cells • Round cells • Epithelioid • Pleomorphic Lipomatous Spindle cell Small round cell Fibrolipoma Leiomyosarcoma Ewing sarcoma Myxoid Epithelioid Pleomorphic Myxoid sarcoma Clear cell sarcoma Pleomorphic sarcoma 2 4/1/2020 CASE #1 • 45yr Man • Thigh mass (fatty) • CNB with TP (DQ stain) DQ Mag 20x ALT –Floret cells 3 4/1/2020 Adipocytic Lesions • Lipoma ‐ most common soft tissue neoplasm • Liposarcoma ‐ most common adult soft tissue sarcoma • Benign features: – Large, univacuolated adipocytes of uniform size – Small, bland nuclei without atypia • Malignant features: – Lipoblasts, pleomorphic giant cells or round cells – Vascular myxoid stroma • Pitfalls: Lipophages & pseudo‐lipoblasts • Fat easily destroyed (oil globules) & lost with preparation Lipoma & Variants . Angiolipoma (prominent vessels) . Myolipoma (smooth muscle) . Angiomyolipoma (vessels + smooth muscle) . Myelolipoma (hematopoietic elements) . Chondroid lipoma (chondromyxoid matrix) . Spindle cell lipoma (CD34+ spindle cells) . Pleomorphic lipoma . Intramuscular lipoma Lipoma 4 4/1/2020 Angiolipoma Myelolipoma Lipoblasts • Typically multivacuolated • Can be monovacuolated • Hyperchromatic nuclei • Irregular (scalloped) nuclei • Nucleoli not typically seen 5 4/1/2020 WD liposarcoma Layfield et al. -
Well-Differentiated Spindle Cell Liposarcoma
Modern Pathology (2010) 23, 729–736 & 2010 USCAP, Inc. All rights reserved 0893-3952/10 $32.00 729 Well-differentiated spindle cell liposarcoma (‘atypical spindle cell lipomatous tumor’) does not belong to the spectrum of atypical lipomatous tumor but has a close relationship to spindle cell lipoma: clinicopathologic, immunohistochemical, and molecular analysis of six cases Thomas Mentzel1, Gabriele Palmedo1 and Cornelius Kuhnen2 1Dermatopathologie, Friedrichshafen, Germany and 2Institute of Pathology, Medical Center, Mu¨nster, Germany Well-differentiated spindle cell liposarcoma represents a rare atypical/low-grade malignant lipogenic neoplasm that has been regarded as a variant of atypical lipomatous tumor. However, well-differentiated spindle cell liposarcoma tends to occur in subcutaneous tissue of the extremities, the trunk, and the head and neck region, contains slightly atypical spindled tumor cells often staining positively for CD34, and lacks an amplification of MDM2 and/or CDK4 in most of the cases analyzed. We studied a series of well-differentiated spindle cell liposarcomas arising in two female and four male patients (age of the patients ranged from 59 to 85 years). The neoplasms arose on the shoulder, the chest wall, the thigh, the lower leg, the back of the hand, and in paratesticular location. The size of the neoplasms ranged from 1.5 to 10 cm (mean: 6.0 cm). All neoplasms were completely excised. The neoplasms were confined to the subcutis in three cases, and in three cases, an infiltration of skeletal muscle was seen. Histologically, the variably cellular neoplasms were composed of atypical lipogenic cells showing variations in size and shape, and spindled tumor cells with slightly enlarged, often hyperchromatic nuclei. -
Advances in Immune Checkpoint Inhibitors for Bone Sarcoma Therapy T Pichaya Thanindratarna,B, Dylan C
Journal of Bone Oncology 15 (2019) 100221 Contents lists available at ScienceDirect Journal of Bone Oncology journal homepage: www.elsevier.com/locate/jbo Review Article Advances in immune checkpoint inhibitors for bone sarcoma therapy T Pichaya Thanindratarna,b, Dylan C. Deana, Scott D. Nelsonc, Francis J. Horniceka, ⁎ Zhenfeng Duana, a Department of Orthopedic Surgery, Sarcoma Biology Laboratory, David Geffen School of Medicine, University of California, 615 Charles E. Young. Dr. South, Los Angeles, CA 90095, USA b Department of Orthopedic Surgery, Chulabhorn hospital, HRH Princess Chulabhorn College of Medical Science, Bangkok, Thailand c Department of Pathology, University of California, Los Angeles, CA, USA ARTICLE INFO ABSTRACT Keywords: Bone sarcomas are a collection of sporadic malignancies of mesenchymal origin. The most common subtypes Immune checkpoint include osteosarcoma, Ewing sarcoma, chondrosarcoma, and chordoma. Despite the use of aggressive treatment Immunotherapy protocols consisting of extensive surgical resection, chemotherapy, and radiotherapy, outcomes have not sig- Bone sarcoma nificantly improved over the past few decades for osteosarcoma or Ewing sarcoma patients. In addition, chon- Anti-PD-1/PD-L1 drosarcoma and chordoma are resistant to both chemotherapy and radiation therapy. There is, therefore, an Anti-CTLA-4 urgent need to elucidate which novel new therapies may affect bone sarcomas. Emerging checkpoint inhibitors have generated considerable attention for their clinical success in a variety of human cancers, which has led to works assessing their potential in bone sarcoma management. Here, we review the recent advances of anti-PD-1/ PD-L1 and anti-CTLA-4 blockade as well as other promising new immune checkpoint targets for their use in bone sarcoma therapy. -
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. -
Lumps and Bumps of the Abdominal Wall and Lumbar Region—Part 2: Beyond Hernias
Published online: 2019-06-18 THIEME Review Article 19 Lumps and Bumps of the Abdominal Wall and Lumbar Region—Part 2: Beyond Hernias Sangoh Lee1 Catalin V. Ivan1 Sarah R. Hudson1 Tahir Hussain1 Suchi Gaba2 Ratan Verma1 1 1 Arumugam Rajesh James A. Stephenson 1Department of Radiology, University Hospitals of Leicester, Address for correspondence James A. Stephenson, MD, FRCR, Leicester General Hospital, Leicester, United Kingdom Department of Radiology, University Hospitals of Leicester, 2Department of Radiology, University Hospitals of North Midlands, Leicester General Hospital, Leicester, LE5 4PW, United Kingdom Royal Stoke University Hospital, Stoke-on-Trent, United Kingdom (e-mail: [email protected]). J Gastrointestinal Abdominal Radiol ISGAR 2018;1:19–32 Abstract Abdominal masses can often clinically mimic hernias, especially when they are locat- ed close to hernial orifices. Imaging findings can be challenging and nonspecific Keywords with numerous differential diagnoses. We present a variety of pathology involving ► abdominal wall the abdominal wall and lumbar region, which were referred as possible hernias. This ► hernia demonstrates the wide-ranging pathology that can present as abdominal wall lesions ► mimics or mimics of hernias that the radiologist should be alert to. Introduction well-differentiated liposarcomas are histologically identical. The term “atypical lipoma” was coined by Evans et al in 1979 to An abdominal hernia occurs when an organ of a body ca vity describe well-differentiated liposarcoma of subcutaneous and 1 protrudes through a defect in the wall of that cavity. It is a 6 intramuscular layers. The World Health Organization (WHO) common condition with lifetime risk of developing a groin has further refined the definition by using atypical lipoma to hernia being estimated at 27% for men and 3% for women; it has describe subcutaneous lesions only and well- differentiated 2 thus been covered extensively in the literature. -
New Jersey Chapter American College of Physicians
NEW JERSEY CHAPTER AMERICAN COLLEGE OF PHYSICIANS ASSOCIATES ABSTRACT COMPETITION 2015 SUBMISSIONS 2015 Resident/Fellow Abstracts 1 1. ID CATEGORY NAME ADDITIONAL PROGRAM ABSTRACT AUTHORS 2. 295 Clinical Abed, Kareem Viren Vankawala MD Atlanticare Intrapulmonary Arteriovenous Malformation causing Recurrent Cerebral Emboli Vignette FACC; Qi Sun MD Regional Medical Ischemic strokes are mainly due to cardioembolic occlusion of small vessels, as well as large vessel thromboemboli. We describe a Center case of intrapulmonary A-V shunt as the etiology of an acute ischemic event. A 63 year old male with a past history of (Dominik supraventricular tachycardia and recurrent deep vein thrombosis; who has been non-compliant on Rivaroxaban, presents with Zampino) pleuritic chest pain and was found to have a right lower lobe pulmonary embolus. The deep vein thrombosis and pulmonary embolus were not significant enough to warrant ultrasound-enhanced thrombolysis by Ekosonic EndoWave Infusion Catheter System, and the patient was subsequently restarted on Rivaroxaban and discharged. The patient presented five days later with left arm tightness and was found to have multiple areas of punctuate infarction of both cerebellar hemispheres, more confluent within the right frontal lobe. Of note he was compliant at this time with Rivaroxaban. The patient was started on unfractionated heparin drip and subsequently admitted. On admission, his vital signs showed a blood pressure of 138/93, heart rate 65 bpm, and respiratory rate 16. Cardiopulmonary examination revealed regular rate and rhythm, without murmurs, rubs or gallops and his lungs were clear to auscultation. Neurologic examination revealed intact cranial nerves, preserved strength in all extremities, mild dysmetria in the left upper extremity and an NIH score of 1. -
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. -
Dedifferentiated Extraskeletal Myxoid Chondrosarcoma of the Masticator Space - a Case Report
The Korean Journal of Pathology 2011; 45(S1): S101-105 DOI: 10.4132/KoreanJPathol.2011.45.S1.S101 Dedifferentiated Extraskeletal Myxoid Chondrosarcoma of the Masticator Space - A Case Report - Geunyoung Jung · Kyung-Ja Cho We describe a 69-year-old woman who presented with a dedifferentiated extraskeletal myxoid Seung-Ho Choi1 · Mi-Jung Kim chondrosarcoma arising in the left masticator space. Computed tomography and magnetic reso- nance imaging revealed a 5 cm sized mass in the left masticator space. Histologically, the tumor Departments of Pathology and consisted of two distinct areas. The less cellular area was a low-grade extraskeletal myxoid chon- 1Otorhinolaryngology, Asan Medical Center, drosarcoma, composed of strands or cords of uniform spindle cells and abundant myxoid stro- University of Ulsan College of Medicine, Seoul, ma. The more cellular, dedifferentiated area corresponded to a high grade myxofibrosarcoma, Korea consisting of anaplastic tumor cells in myxoid stroma and geographic necrosis. The tumor cells of the former area were positive for S-100 protein, microtubule-associated protein-2 (MAP-2) and Received: October 25, 2010 class III β-tubulin, but negative for cytokeratin, smooth muscle actin, and desmin. The tumor cells November 23, 2010 Accepted: in the latter, pleomorphic area showed MAP-2 and β-tubulin immunoreactivity with a high Ki-67 Corresponding Author labeling index. Based on its histologic and immunohistochemical features, the tumor was consid- Mi-Jung Kim, M.D. ered a dedifferentiated extraskeletal myxoid chondrosarcoma. Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, 388-1 Pungnap 2-dong, Songpa-gu, Seoul 138-736, Korea Tel: +82-2-3010-4557 Fax: +82-2-472-7898 E-mail: [email protected] Key Words: Extraskeletal myxoid chondrosarcoma; Cell dedifferentiation; Masticator space Extraskeletal myxoid chondrosarcoma (EMC) is a rare malig- CASE REPORT nant soft tissue sarcoma, distinguished from its skeletal coun- terpart by cytogenetic and histologic features. -
Soft Tissue and Bone Tumors
Soft tissue and bone tumors Zoltán Sápi MD, PhD 1st Department of Pathology and Experimental Cancer Research • Large and heterogeneous group with more than 200 entity and more than 50 sarcoma types • Previously: classified according to a histogenetic concept (fibrosarcoma from fibroblast) • Now: primitive multipotential stem cells differentiate along one or more lines Pluripotent mesenchymal stem cell, may differentiate into different direction lipogenic, neurogenic, fibro-myofibroblastic, smooth muscle, vascular, uncertain • Light microscopic evaluation (H&E) + many ancillary techniques Principles Characteristic morphology with corresponding biological behavior Variants (with the same biological behavior) Similar morphology but different biological behavior (MFS – LGFMS) Different morphology but the same genetic background (Giant cell fibroblastoma – DFSP) Genetic promiscuity (partly or totally) Molecular complexity complex karyotype Recurrent cytogenetic alteration, genetic instability Translocations fusion genes, proteins no fusion gene de novo formation de novo formation, rarely fusion gene is the initial step/driving force „dysplastic-precursors” synovial sarcoma, myxoid liposarcoma MPNST, leiomyosarcoma FISH probes Break-apart SS18 (SYT) Synovial sarcoma FOXO1 Alveolar rhabdomyosarcoma DDIT3 (CHOP) Myxoid liposarcoma FUS Myxoid liposarcoma, Angiomatoid fibrosus histiocytoma, LGFMS EWSR1 Ewing sc, AFH, DSRCT, EMC, MC, Clear cell sc EWSR1 COL1A1 Dermatofibrosarcoma protuberans USP6 Nodular fasciitis, Aneurysmal bone cyst ETV6 Infantile