DOCSLIB.ORG

SOFT TISSUE SARCOMA • Soft Tissues Include: • Muscles • Tendons • Fat • Blood Vessels • Lymph Vessels • Nerves • Tissues Around Joints

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
SOFT TISSUE SARCOMA • Soft Tissues Include: • Muscles • Tendons • Fat • Blood Vessels • Lymph Vessels • Nerves • Tissues Around Joints NAACCR Webinar Series 2017-2018 1/11/18 COLLECTING CANCER DATA: GIST/SARCOMA 2017‐2018 NAACCR WEBINAR SERIES Q&A • Please submit all questions concerning webinar content through the Q&A panel. • Reminder: • If you have participants watching this webinar at your site, please collect their names and emails. • We will be distributing a Q&A document in about one week. This document will fully answer questions asked during the webinar and will contain any corrections that we may discover after the webinar. 2 Uterus 1 NAACCR Webinar Series 2017-2018 1/11/18 Fabulous Prizes 3 AGENDA • Overview • Epi Moment • Quiz 1 • Staging • Bone • Soft Tissue • GIST • Treatment • Quiz 2 • Case Scenarios 4 Uterus 2 NAACCR Webinar Series 2017-2018 1/11/18 SARCOMA ANATOMY 5 SOFT TISSUE SARCOMA • Soft tissues include: • Muscles • Tendons • Fat • Blood vessels • Lymph vessels • Nerves • Tissues around joints 6 Uterus 3 NAACCR Webinar Series 2017-2018 1/11/18 SOFT TISSUE SARCOMA • Over 50 different types • Angiosarcoma – blood vessels or lymph vessels; following radiation • Undifferentiated pleomorphic sarcoma – most often in arms or legs (previously malignant fibrous histiocytoma MFH) • Spindle cell sarcoma – descriptive name based on the appearance of the cells • Liposarcoma – fat cells; most frequently seen 7 SOFT TISSUE SARCOMA • Pleomorphic dermal sarcoma • Atypical Fibroxanthoma (AFX) • Arise in sun‐damaged skin • Arise in sun‐damaged skin • Head, Neck, Scalp • Ear, Nose, Forehead, Cheek • Negative: S100, Multiple CK, • Negative: S100, multiple CK, Desmin, CD34 Desmin, CD34 • Behavior likely more aggressive • Behavior is almost invariably a but limited follow up due to benign behavior with only rare patient advanced age at local recurrence presentation 8 Uterus 4 NAACCR Webinar Series 2017-2018 1/11/18 SOFT TISSUE SARCOMA • Question • What is the appropriate histology code for a final diagnosis or undifferentiated pleomorphic sarcoma and/or pleomorphic sarcoma, undifferentiated? Does the Other Sites MP/H Rule H17 apply in this case, which results in coding higher histology 8805/3? Or does the “undifferentiated” statement only refer to grade, which results in coding histology to 8802/3 (pleomorphic sarcoma)? 9 ANSWER • SINQ 20160044 • Assign 8802/34 to pleomorphic cell sarcoma/undifferentiated pleomorphic sarcoma. Pleomorphic is more important than undifferentiated when choosing the histology code in this case. Undifferentiated can be captured in the grade code. 10 Uterus 5 NAACCR Webinar Series 2017-2018 1/11/18 SARCOMA • 2018 Histology – New Terms • 8571/3 Carcinoma with chondroid differentiation (C50._) Carcinoma with osseous differentiation (C50._) Metaplastic carcinoma with chondroid differentiation (C50._) Metaplastic carcinoma with osseous differentiation (C50._) 11 SARCOMA • 2018 Histology – New Terms • 8801/3 Undifferentiated spindle cell sarcoma • 8802/3 Undifferentiated pleomorphic sarcoma • 8803/3 Undifferentiated round cell sarcoma • 8804/3 Undifferentiated epithelioid sarcoma • 8805/3 Undifferentiated uterine sarcoma • 8830/3 Undifferentiated high‐grade pleomorphic sarcoma 12 Uterus 6 NAACCR Webinar Series 2017-2018 1/11/18 OSTEOSARCOMA • Most common malignant bone tumor • Arise from osteoblasts • Typically occurs in long bones • Mutation in TP53 are most common • Distant metastasis occur in ~20% • Lung is most common site 13 OSTEOSARCOMA • Periosteum • 2 layers • Fibrous connective tissue • Inner osteogenic • Medullary cavity • Bone marrow https://seer.cancer.gov/tools/ssm/musculoskel.pdf 14 Uterus 7 NAACCR Webinar Series 2017-2018 1/11/18 SOFT TISSUE SARCOMA GRADE • FNCLCC grading system • Differentiation • Necrosis • Mitotic rate • Histologic Grade: GX Grade cannot be assessed G1 FNCLCC grade score of 2 or 3 G2 FNCLCC grade score of 4 or 5 G3 FNCLCC grade score of 6, 7, or 815 GIST ANATOMY 16 Uterus 8 NAACCR Webinar Series 2017-2018 1/11/18 WHAT IS GIST? • Rare type of soft tissue sarcoma • Develop in muscle layer of gut rather than mucosa • Grow outward (exophytic) • Described as a distinct entity in 1998 • Umbrella term for most mesenchymal tumors of stomach and intestine • Most tumors historically called leiomyosarcoma are now classified as GISTs 17 GIST • Interstitial cells of Cajal • “Pacemaker cells” • Sends signals to move food and liquid through system (peristalsis) http://www.gistsupport.org/media/Understanding%20Pathology%20Report/Fig‐1‐ 400pix.jpg 18 Uterus 9 NAACCR Webinar Series 2017-2018 1/11/18 ONCOGENIC MUTATIONS • ~85% of GIST contain oncogenic mutations in one of two receptor tyrosine kinases • KIT‐Mutant GIST • PDGFRA (Platelet‐derived Growth Factor Receptor Alpha) • Wild Type GIST • ~12‐15% GIST contain no genetic mutation of KIT or PDGFRA 19 TUMOR LOCATION Stomach 60% Small Intestine 30% Rectum 3% Colon 1‐2% Esophagus <1% Omentum/Mese Rare ntery 20 Uterus 10 NAACCR Webinar Series 2017-2018 1/11/18 GIST • How do you determine if a GIST is malignant i.e. reportable? • GIST, NOS is a borderline tumor (/1) • If your state or facility requires collection of these GISTs, you should follow their requirements • Don’t determine reportability based on staging • AJCC staging forms are used on all GISTs 21 Theme Song: The Skeleton Dance COLLECTING CANCER DATA: SARCOMA EPI MOMENT: RECINDA SHERMAN JANUARY 11, 2018 Uterus 11 NAACCR Webinar Series 2017-2018 1/11/18 EPIDEMIOLOGY OF SARCOMAS • Large grouping of distinct cancers • 50+ distinct histologies; putative mesenchymal origin • Combined & studied as group • Rare in adults (<2%); Top 5 for Pedi (21%) • Majority soft tissue (87%); malignant bone (13%) • Soft: muscles, joints, fat, nerves, deep skin, blood vessels • Bone: commonly in cartilage • Prognosis generally poor; esp soft • Delayed diagnosis: arise anywhere, lack of specific symptoms • No population based screening • Poor survival adults; better for pedi Figures from: Burningham, Zachary, Mia Hashibe, Logan Spector, and Joshua D. Schiffman. "The Epidemiology of Sarcoma." Clinical Sarcoma Research (2012). BioMed Central Ltd. Web. 10 Feb. 2016. 23 Sarcoma (Soft), 0‐19, CiNA 2014 PEDIATRIC SARCOMAS (SOFT) 12% Rhabdomyosarcomas 36% • Rhabdomyosarcoma – most common soft tissue for peds Fibrosarcomas, peripheral nerve & other fibrous • Skeletal muscle Other specified soft tissue 40% sarcomas • Unspecified soft tissue 50% occur <10; slightly more common in males 12% sarcomas • Often presents as painless mass; risk factor Li‐Fraumeni syndrome • 5 year survival 70%; dependent upon location, stage, and histology—often lymph node involvement • Embryonal better prognosis than alveolar subtype; 20% present metastatic with 5 year survival 30‐40% vs 80% for local • Other Sarcomas (Soft), Rate per 1,000,000, CiNA 2014 • Fibrosarcoma – historically 2/3rds of sarcomas 1.4 1.2 1.2 • now 12% due to better classification (proportion changed but not risk) 1 0.8 0.5 • Liposarcoma – often large tumors; common among adults but <5% of ped sarcomas 0.6 0.4 0.4 th • Synovial sarcoma –4 most common; 2x more common in males 0.2 0.1 0.1 0 • origin mesenchymal not synovium; largely genetic • Malignant peripheral nerve sheath tumors – grouped into Brain CNS category for Epi • Alveolar Soft Part Sarcoma (ASPS) – rare, slow growing; generally mets at dx • Mesenchymoma – rare but highly aggressive 24 Uterus 12 NAACCR Webinar Series 2017-2018 1/11/18 PEDIATRIC SARCOMAS (BONE) • Osteosarcoma – most common • Generally on edges of “long” bones; 2nd most common location upper arm near shoulder • Surgery & Chemo • Chondrosarcomas • Ewing Family – 10‐15% of bone sarcomas in peds Sarcomas (Bone), Rate per 1,000,000, CiNA 2014 • Ewing sarcoma, extraosseous Ewing, PNET, Askin) 10 9.3 9 • Impacts teens; responsive to radiotherapy 8 7 Sarcomas (Bone), 0‐19, CiNA 2014 6 5.1 5 3% 6% 4 3.0 3 2 1 0.3 0.5 0.2 32% 0 55% 4% Osteosarcomas Chondrosarcomas Ewing Family Other specified malignant bone tumors Unspecified malignant bone tumors 25 RISK FACTORS • Varied causes; distinct disease; limited studies & understanding • Environmental, genetic (synergistic) • Genetic • Age: Soft: Rate high <5 for soft, lower but steady increase 6‐49; 50+ high; Bone: Rate stable across ages; Rate high in YA (osteosarcoma, Ewing) • Race: Ewing (9x more common white vs black); but soft tissue higher for blacks • Hx of hernia ‐ Ewing sarcoma (children) • Growth “spurts” – osteosarcoma (children) • Genetic syndromes (Li‐Fraumeni; neurofibromatosis/von Recklinghausen dx, retinoblastoma) • Non currently described for Ewing but likely genetic • Environmental • HIV+ for KS (but HHV8 is causal, HIV & EBV)) • Radiation exposure ‐ Bone cancer; Tx and atomic (Japanese) • Occupational exposures ‐‐herbicides (soft tissue) 26 Uterus 13 NAACCR Webinar Series 2017-2018 1/11/18 THE GIST ON G.I.S.T.h/t Brad Wohler, FCDS • 2001 ICD‐O‐3 specific code • Rare, digestive tract soft tissue sarcoma • Adult cancer; 50+ • Used to believe origin was nerve or muscle cells • Now understood to arise from interstitial cells of Cajal (ICC) or precursor—the “pacemakers” of digestion; occur from esoph to anus but over ½ in stomach • Moving away from “benign” designation = increase rates but not risk GIST, over time (CiNA 1995‐2014) 1 0.8 APC 7.8%* 0.6 0.4 0.2 0 27 QUESTIONS? QUIZ 1 28 Uterus 14 NAACCR Webinar Series 2017-2018 1/11/18 STAGING SUMMARY STAGE AJCC 29 SUMMARY STAGE 2000 & 2018 30 Uterus 15 NAACCR Webinar Series 2017-2018 1/11/18 SUMMARY STAGE 2000‐MUSCULOSKELETAL SYSTEM • Bones, joints, and articular cartilage • C40.0‐C40.3, C40.8‐C40.9, C41.0‐C41.4, C41.8‐C41.9 • Peripheral nerves and autonomic nervous
Load more

Recommended publications
  • A Case Report1 양측 흉막에 발생한 결합조직형성 소원형세포종양의 증례 보고1
    Case Report pISSN 1738-2637 / eISSN 2288-2928 J Korean Soc Radiol 2015;72(4):295-299 http://dx.doi.org/10.3348/jksr.2015.72.4.295 Bilateral Presentation of Pleural Desmoplastic Small Round Cell Tumors: A Case Report1 양측 흉막에 발생한 결합조직형성 소원형세포종양의 증례 보고1 You Sun Won, MD1, Jai Soung Park, MD1, Sun Hye Jeong, MD1, Sang Hyun Paik, MD1, Heon Lee, MD1, Jang Gyu Cha, MD1, Eun Suk Koh, MD2 Departments of 1Radiology, 2Pathology, Soonchunhyang University College of Medicine, Bucheon Hospital, Bucheon, Korea Desmoplastic small round cell tumor (DSRCT) is a highly aggressive malignant small cell neoplasm occurring mainly in the abdominal cavity, but it is extremely rare in Received October 13, 2014; Accepted December 21, the pleura. In this case, a 15-year-old male presented with a 1-month history of left 2014 chest pain. Chest radiographs revealed pleural thickening in the left hemithorax and Corresponding author: Jai Soung Park, MD Department of Radiology, Soonchunhyang University chest computed tomography showed multifocal pleural thickening with enhance- College of Medicine, Bucheon Hospital, 170 Jomaru-ro, ment in both hemithoraces. A needle biopsy of the left pleural lesion was performed Wonmi-gu, Bucheon 420-767, Korea. and the final diagnosis was DSRCT of the pleura. We report this unusual case aris- Tel. 82-32-621-5851 Fax. 82-32-621-5874 E-mail: [email protected] ing from the pleura bilaterally. The pleural involvement of this tumor supports the hypothesis that it typically occurs in mesothelial-lined surfaces. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) Index terms which permits unrestricted non-commercial use, distri- Pleura bution, and reproduction in any medium, provided the Thickened Pleura original work is properly cited.
    [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]
  • Appendix 4 WHO Classification of Soft Tissue Tumours17
    S3.02 The histological type and subtype of the tumour must be documented wherever possible. CS3.02a Accepting the limitations of sampling and with the use of diagnostic common sense, tumour type should be assigned according to the WHO system 17, wherever possible. (See Appendix 4 for full list). CS3.02b If precise tumour typing is not possible, generic descriptions to describe the tumour may be useful (eg myxoid, pleomorphic, spindle cell, round cell etc), together with the growth pattern (eg fascicular, sheet-like, storiform etc). (See G3.01). CS3.02c If the reporting pathologist is unfamiliar or lacks confidence with the myriad possible diagnoses, then at this point a decision to send the case away without delay for an expert opinion would be the most sensible option. Referral to the pathologist at the nearest Regional Sarcoma Service would be appropriate in the first instance. Further International Pathology Review may then be obtained by the treating Regional Sarcoma Multidisciplinary Team if required. Adequate review will require submission of full clinical and imaging information as well as histological sections and paraffin block material. Appendix 4 WHO classification of soft tissue tumours17 ADIPOCYTIC TUMOURS Benign Lipoma 8850/0* Lipomatosis 8850/0 Lipomatosis of nerve 8850/0 Lipoblastoma / Lipoblastomatosis 8881/0 Angiolipoma 8861/0 Myolipoma 8890/0 Chondroid lipoma 8862/0 Extrarenal angiomyolipoma 8860/0 Extra-adrenal myelolipoma 8870/0 Spindle cell/ 8857/0 Pleomorphic lipoma 8854/0 Hibernoma 8880/0 Intermediate (locally
    [Show full text]
  • The Best Diagnosis Is
    Dermatopathology Diagnosis Superficial Plantar Fibromatosis The best diagnosis is: H&E, original magnification 40. a. dermatofibroma b. keloid CUTISc. neurofibroma d. nodular fasciitis Do Note. superficialCopy plantar fibromatosis H&E, original magnification 400. PLEASE TURN TO PAGE 225 FOR DERMATOPATHOLOGY DIAGNOSIS DISCUSSION Luke Lennox, BA; Anna Li, BS; Thomas N. Helm, MD Mr. Lennox and Dr. Helm are from the Department of Dermatology, University at Buffalo, The State University of New York. Ms. Li is from the Department of Dermatology, Ross University, Dominica, West Indies. The authors report no conflict of interest. Correspondence: Thomas N. Helm, MD, Dermatopathology Laboratory, 6255 Sheridan Dr, Building B, Ste 208, Williamsville, NY 14221 ([email protected]). 220 CUTIS® WWW.CUTIS.COM Copyright Cutis 2013. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. Dermatopathology Diagnosis Discussion Superficial Plantar Fibromatosis lantar fibromatosis typically presents as firm represents a reactive proliferation of spindle cells most plaques or nodules on the plantar surface of the often encountered on the extremities of young adults. Pfoot.1 The process is caused by a proliferation of Spindle cells are loosely arranged in a mucinous fibroblasts and collagen and has been associated with stroma and are not circumscribed (tissue culture ap- trauma, liver disease, diabetes mellitus, epilepsy, and pearance). Vesicular nuclei are encountered, but there alcoholism.2 Unlike the fibromatoses associated with is no remarkable nuclear pleomorphism. Extravasated Gardner syndrome, superficial plantar fibromatosis has not been associated with abnormalities in the ade- nomatous polyposis coli gene or with the -catenin gene.3,4 Lesions typically present in middle-aged or elderly individuals and involve the medial plantar fas- cia.
    [Show full text]
  • About Soft Tissue Sarcoma Overview and Types
    cancer.org | 1.800.227.2345 About Soft Tissue Sarcoma Overview and Types If you've been diagnosed with soft tissue sarcoma or are worried about it, you likely have a lot of questions. Learning some basics is a good place to start. ● What Is a Soft Tissue Sarcoma? Research and Statistics See the latest estimates for new cases of soft tissue sarcoma and deaths in the US and what research is currently being done. ● Key Statistics for Soft Tissue Sarcomas ● What's New in Soft Tissue Sarcoma Research? What Is a Soft Tissue Sarcoma? Cancer starts when cells start to grow out of control. Cells in nearly any part of the body can become cancer and can spread to other areas. To learn more about how cancers start and spread, see What Is Cancer?1 There are many types of soft tissue tumors, and not all of them are cancerous. Many benign tumors are found in soft tissues. The word benign means they're not cancer. These tumors can't spread to other parts of the body. Some soft tissue tumors behave 1 ____________________________________________________________________________________American Cancer Society cancer.org | 1.800.227.2345 in ways between a cancer and a non-cancer. These are called intermediate soft tissue tumors. When the word sarcoma is part of the name of a disease, it means the tumor is malignant (cancer).A sarcoma is a type of cancer that starts in tissues like bone or muscle. Bone and soft tissue sarcomas are the main types of sarcoma. Soft tissue sarcomas can develop in soft tissues like fat, muscle, nerves, fibrous tissues, blood vessels, or deep skin tissues.
    [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]
  • Pathology and Genetics of Tumours of Soft Tissue and Bone
    bb5_1.qxd 13.9.2006 14:05 Page 3 World Health Organization Classification of Tumours WHO OMS International Agency for Research on Cancer (IARC) Pathology and Genetics of Tumours of Soft Tissue and Bone Edited by Christopher D.M. Fletcher K. Krishnan Unni Fredrik Mertens IARCPress Lyon, 2002 bb5_1.qxd 13.9.2006 14:05 Page 4 World Health Organization Classification of Tumours Series Editors Paul Kleihues, M.D. Leslie H. Sobin, M.D. Pathology and Genetics of Tumours of Soft Tissue and Bone Editors Christopher D.M. Fletcher, M.D. K. Krishnan Unni, M.D. Fredrik Mertens, M.D. Coordinating Editor Wojciech Biernat, M.D. Layout Lauren A. Hunter Illustrations Lauren A. Hunter Georges Mollon Printed by LIPS 69009 Lyon, France Publisher IARCPress International Agency for Research on Cancer (IARC) 69008 Lyon, France bb5_1.qxd 13.9.2006 14:05 Page 5 This volume was produced in collaboration with the International Academy of Pathology (IAP) The WHO Classification of Tumours of Soft Tissue and Bone presented in this book reflects the views of a Working Group that convened for an Editorial and Consensus Conference in Lyon, France, April 24-28, 2002. Members of the Working Group are indicated in the List of Contributors on page 369. bb5_1.qxd 22.9.2006 9:03 Page 6 Published by IARC Press, International Agency for Research on Cancer, 150 cours Albert Thomas, F-69008 Lyon, France © International Agency for Research on Cancer, 2002, reprinted 2006 Publications of the World Health Organization enjoy copyright protection in accordance with the provisions of Protocol 2 of the Universal Copyright Convention.
    [Show full text]
  • Practical Issues for Retroperitoneal Sarcoma Vicky Pham, MS, Evita Henderson-Jackson, MD, Matthew P
    Pathology Report Practical Issues for Retroperitoneal Sarcoma Vicky Pham, MS, Evita Henderson-Jackson, MD, Matthew P. Doepker, MD, Jamie T. Caracciolo, MD, Ricardo J. Gonzalez, MD, Mihaela Druta, MD, Yi Ding, MD, and Marilyn M. Bui, MD, PhD Background: Retroperitoneal sarcoma is rare. Using initial specimens on biopsy, a definitive diagnosis of histological subtypes is ideal but not always achievable. Methods: A retrospective institutional review was performed for all cases of adult retroperitoneal sarcoma from 1996 to 2015. A review of the literature was also performed related to the distribution of retroperitoneal sarcoma subtypes. A meta-analysis was performed. Results: Liposarcoma is the most common subtype (45%), followed by leiomyosarcoma (21%), not otherwise specified (8%), and undifferentiated pleomorphic sarcoma (6%) by literature review. Data from Moffitt Cancer Center demonstrate the same general distribution for subtypes of retroperitoneal sarcoma. A pathology-based algorithm for the diagnosis of retroperitoneal sarcoma is illustrated, and common pitfalls in the pathology of retroperitoneal sarcoma are discussed. Conclusions: An informative diagnosis of retroperitoneal sarcoma via specimens on biopsy is achievable and meaningful to guide effective therapy. A practical and multidisciplinary algorithm focused on the histopathology is helpful for the management of retroperitoneal sarcoma. Introduction tic, and predictive information based on a relatively Soft-tissue sarcomas are mesenchymal neoplasms small amount of tissue obtained
    [Show full text]
  • Aggressive Fibromatosis of the Chest Wall Mimicking Low Grade Fibrosarcoma - an Unusual Clinical Presentation
    International Journal of Health Sciences and Research www.ijhsr.org ISSN: 2249-9571 Case Report Aggressive Fibromatosis of the Chest Wall Mimicking Low Grade Fibrosarcoma - An Unusual Clinical Presentation Dr. Nimisha Sharma1*, Dr. Sumanashree Mallappa2**, Dr. Sujata Raychaudhuri3*, Dr Amit Yadav3**, Prof. Dr. A. K. Mandal4** 1Assistant Professor, 2Senior Resident, 3Associate Professor, 4Director Professor, *Dept. of Pathology, ESIC Medical College and Hospital, Faridabad **Dept. of Pathology, VMMC and Safdarjung Hospital, New Delhi Corresponding Author: Dr. Nimisha Sharma ABSTRACT Background: Aggressive fibromatosis (AF), also known as desmoid tumour or musculoaponeurotic fibromatosis is a monoclonal fibroblastic proliferative disease. It can present as abdominal and extra- abdominal fibromatosis. Extra abdominal deep fibromatosis represent 3.5% of fibrous tissue tumor and 0.03% of all neoplasms. Anterior chest wall forms 10% of Extra abdominal deep fibromatosis cases. Case history: Patient presented with a progressively increasing large swelling on the chest wall since 2 years. On examination, firm to hard ill defined growth was found. Microscopic examination showed a cellular spindle cell tumour. Strong positivity for VIMENTIN and focal positivity for SMA and DESMIN was seen on Immunohistochemistry. Final Diagnosis of aggressive fibromatosis was made. Conclusion: This case is being presented for its rarity, unusual clinical presentation and overlapping clinical and histopathological features with fibrosarcoma, which is a malignant
    [Show full text]
  • Primary Undifferentiated High-Grade Pleomorphic Sarcoma/Malignant
    Oral Science International 11 (2014) 70–75 Contents lists available at ScienceDirect Oral Science International j ournal homepage: www.elsevier.com/locate/osi Case Report Primary undifferentiated high-grade pleomorphic sarcoma/malignant fibrous histiocytoma arising from the mandible a,∗ b c d a Yoshiyuki Mori , Toru Motoi , Komei Ida , Junji Shibahara , Hideto Saijo , a a Takafumi Susami , Tsuyoshi Takato a Department of Oral-Maxillofacial Surgery, Dentistry and Orthodontics, The University of Tokyo Hospital, 7-3-1 Hongo Bunkyo-ku, Tokyo 113-8655, Japan b Department of Pathology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 18-22 Honkomagome 3chome, Bunkyo-ku, Tokyo 113-8677, Japan c Department of Pediatrics, Teikyo University Mizonokuchi Hospital, 3-8-3 Mizonokuchi, Takatsu-ku, Kawasaki City, Kanagawa 213-8507, Japan d Department of Pathology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo Bunkyo-ku, Tokyo 113-8655, Japan a r t i c l e i n f o a b s t r a c t Article history: A patient who had a primary undifferentiated high-grade pleomorphic sarcoma/malignant fibrous histio- Received 30 October 2013 cytoma that apparently arose in the mandible, but showed uncertain differentiation on histopathological Received in revised form 10 January 2014 examination, is described. Our regimen, a combination of pre- and postoperative chemotherapy and Accepted 15 January 2014 surgical resection, produced a good outcome. © 2014 Japanese Stomatological Society. Published by Elsevier Ltd. All rights reserved. Keywords: Undifferentiated high-grade pleomorphic sarcoma/malignant fibrous histiocytoma VAC therapy Rectus abdominis musculocutaneous flap 1. Introduction Computerized tomography (CT) scans revealed osteolytic changes and a mass measuring about 4 cm in diameter in the right Sarcomas arising in the oral region have a much lower incidence side of the mandible.
    [Show full text]
  • Synovial Sarcoma- an Unusual Cause of Heel Pain
    Malaysian Orthopaedic Journal 2008 Vol 2 No 2 MY Norhamdan, et al Synovial Sarcoma- An unusual cause of Heel Pain MY Norhamdan, MS (Ortho), Y Shahril, MS (Ortho), O Masbah, MS (Ortho), Siti Aishah MA*, MIAC Department of Orthopaedics & Traumatology, UKM Medical Centre, Kuala Lumpur, Malaysia * Department of Pathology, UKM Medical Centre, Kuala Lumpur, Malaysia ABSTRACT imaging revealed a well-circumscribed heterogeneous lesion, isointense to muscle on T(1)-weighted image and We report a case of 29-year-old female who presented with hyperintense on T(2) image indicating a benign lesion right heel pain that worsened over a period of two years. The (Figure 2). Due to close proximity to the posterior tibial onset of pain was followed by swelling at the medial aspect nerve, the patient was diagnosed with neuroma. of right ankle. She was initially treated for plantar fasciitis with multiple steroid injections over the heel. Subsequent The patient underwent excision biopsy and intra-operatively MRI revealed a well-defined heterogeneous lesion in the lesion was noted to be a soft, friable mass encasing the continuity with the medial plantar nerve. Excision biopsy medial plantar nerve (Figure 3). Histopathological was performed and histopathological evaluation revealed evaluation revealed monophasic synovial sarcoma as monophasic synovial sarcoma. The patient subsequently evidenced by spindle-shaped and neoplastic cells. underwent wide resection and free tissue transfer followed by Imunohistochemically the neoplastic cells were positive for radiotherapy and chemotherapy. This case highlights an vimentin and focally positive for EMA, CK, and CD 99 but unusual site and presentation of synovial sarcoma which led negative for CD 34, CD 31, desmin, myoglobin, smooth to delayed diagnosis and treatment.
    [Show full text]
  • High-Grade Soft Tissue Sarcoma Arising in a Desmoid Tumor
    Bertucci et al. Clin Sarcoma Res (2015) 5:25 DOI 10.1186/s13569-015-0040-0 Clinical Sarcoma Research CASE REPORT Open Access High‑grade soft tissue sarcoma arising in a desmoid tumor: case report and review of the literature François Bertucci1,2*, Marjorie Faure1, Maria‑Rosa Ghigna3, Bruno Chetaille4, Jérôme Guiramand5, Laurence Moureau‑Zabotto6, Anthony Sarran7 and Delphine Perrot1 Abstract Desmoid tumors are rare benign monoclonal fibroblastic tumors. Their aggressiveness is local with no potential for metastasis or dedifferentiation. Here we report on a 61-year-old patient who presented a locally advanced breast desmoid tumor diagnosed 20 years after post-operative radiotherapy for breast carcinoma. After 2 years of medical treatment, a high-grade undifferentiated pleomorphic soft tissue sarcoma arose within the desmoid tumor. Despite extensive surgery removing both tumors, the patient showed locoregional relapse by the sarcoma, followed by multimetastatic progression, then death 25 months after the surgery. The arising of a soft tissue sarcoma in a desmoid tumor is an exceptional event since our case is the fourth one reported so far in literature. It reinforces the need for timely and accurate diagnosis when a new mass develops in the region of a preexisting desmoid tumor, and more generally when a desmoid tumor modifies its clinical or radiological aspect. Keywords: Breast cancer, Desmoid tumor, Soft tissue sarcoma Background classification of sarcomas, they are classified as tumors Desmoid tumors (DT) are rare benign monoclonal fibro- with intermediate malignancy potential. Locally, they can blastic tumors representing less than 5 % of all soft tissue damage vital structures and threaten life [7], notably in tumors [1].
    [Show full text]