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Uterine Sarcomas: a Review
ARTICLE IN PRESS YGYNO-973334; No. of pages: 9; 4C: 3, 6 Gynecologic Oncology xxx (2009) xxx–xxx Contents lists available at ScienceDirect Gynecologic Oncology journal homepage: www.elsevier.com/locate/ygyno Review Uterine sarcomas: A review Emanuela D'Angelo, Jaime Prat ⁎ Department of Pathology, Hospital de la Santa Creu i Sant Pau, Autonomous University of Barcelona, Sant Antoni M. Claret, 167, 08025 Barcelona, Spain article info abstract Article history: Objective. Uterine sarcomas are rare tumors that account for 3% of uterine cancers. Their histopathologic Received 29 June 2009 classification was revised by the World Health Organization (WHO) in 2003. A new staging system has been Available online xxxx recently designed by the International Federation of Gynecology and Obstetrics (FIGO). Currently, there is no consensus on risk factors for adverse outcome. This review summarizes the available clinicopathological data Keywords: on uterine sarcomas classified by the WHO diagnostic criteria. Uterine sarcomas Methods. Medline was searched between 1976 and 2009 for all publications in English where the studied Leiomyosarcoma population included women diagnosed of uterine sarcomas. Endometrial stromal sarcoma fi Undifferentiated endometrial sarcoma Results. Since carcinosarcomas (malignant mixed mesodermal tumors or MMMT) are currently classi ed Adenosarcoma as metaplastic carcinomas, leiomyosarcomas remain the most common uterine sarcomas. Exclusion of Carcinosarcoma several histologic variants of leiomyoma, as well as “smooth muscle tumors of uncertain malignant potential,” frequently misdiagnosed as sarcomas, has made apparent that leiomyosarcomas are associated with poor prognosis even when seemingly confined to the uterus. Endometrial stromal sarcomas are indolent tumors associated with long-term survival. Undifferentiated endometrial sarcomas exhibiting nuclear pleomorphism behave more aggressively than tumors showing nuclear uniformity. -
Mr Leiomyoma Vs Leiomyosarcoma
2 0 SCBT· MR 1 LEIOMYOMA VS LEIOMYOSARCOMA 5 Susan M. Ascher, MD Professor & Co-Director of Abdominal Imaging Georgetown University Hospital, Washington, DC T2-W MRI: Normal Uterus, Leiomyoma and Leiomyosarcoma NORMAL LEIOMYOMA LEIOMYOSARCOMA LEIOMYOMA or LEIOMYOSARCOMA LEIOMYOMA LEIOMYOSARCOMA LEIOMYOMA or LEIOMYOSARCOMA LEIOMYOMA LEIOMYOSARCOMA LEIOMYOMA or LEIOMYOSARCOMA LEIOMYOMA LEIOMYOSARCOMA DEGENERATED LEIOMYOMA vs LEIOMYOSARCOMA Distinguishing the two can be challenging Laparoscopic Power Morcellators • Hysterectomy • Myommectomy Prognosis is significantly worse in women who had leiomyosarcomas morcellated than women who underwent standard abdominal hysterectomy Park JY, et al. Gynecol Oncol 2011; 122:255-259. Perri T, et al. Int J Gyencol Cancer 2009; 19:257-260 DEGENERATED LEIOMYOMA vs LEIOMYOSARCOMA Distinguishing the two can be challenging 4/17/14: FDA safety warning on LPM for hysterectomy & myomectomy • Prev of unsuspected uterine sarcoma: 1 in 352 • Prev of unsuspected uterine LMS: 1 in 498 • Upstaging sarcoma 1 in 7000 Pritts et al (open source) 7/10 -11/14: FDA OB-GYN Devices Panel FDA: Quantitative Assessment of the Prevalence of Unsuspected Uterine Sarcoma in Women undergoing Treatment of Uterine Fibroids. Summary and Key Findings http://www.fda.gov/downloads/MedicalDevices/Safety/AlertsandNotices/UCM393589. 7.11.14: “Fate of Uterine Device Now in Hands of FDA: Panel's Recommendations Run From Outright Ban to 'Black Box' Warning to Limited Use” Ethicon voluntarily suspend sales and recalls devices worldwide 9.22.14: “Gynecologists Resist FDA Over Popular Surgical Tool: Doctors Continue to Use Morcellators Months After Regulator Warned They Can Spread Undetected Cancer” 11.24.2014: FDA Black Box Warning & IIE “Warning Prompts Shift in Surgeries on Women” A Yale University study found that 84% of gynecological surgeons at large U.S. -
Mixed Hepatoblastoma in the Adult: Case Report and Review of the Literature
J Clin Pathol: first published as 10.1136/jcp.33.11.1058 on 1 November 1980. Downloaded from J Clin Pathol 1980;33:1058-1063 Mixed hepatoblastoma in the adult: case report and review of the literature RP HONAN AND MT HAQQANI From the Department of Pathology, Walton Hospital, Rice Lane, Liverpool L9 JAE, UK SUMMARY A case of mixed hepatoblastoma in a woman is described. A survey of the English literature reveals 13 cases acceptable as mixed hepatoblastoma; these have been described and published under a variety of names. Difficulties in nomenclature and the histology of these cases are discussed. Diagnosis depends on the identification of both malignant mesenchymal and malignant epithelial elements. The former include myxoid connective tissue resembling primitive mesenchyme and areas resembling adult fibrosarcoma. Mature fibrous tissue with calcification and bone for- mation may be seen. Epithelial areas show tissue resembling fetal liver, poorly differentiated epithelial cells, and/or areas of adenocarcinoma. The current view on histogenesis is also given. Most hepatoblastomas occur in children under the mixedtumour,6carcino-osteochondromyxosarcoma,5 copyright. age of 2 years.' Hepatoblastoma in adults is ex- and rhabdomyosarcohepatoma.7 tremely rare, and the prognosis is much worse than in the mixed hepatoblastoma of childhood. Case report The literature of mixed hepatoblastoma in adults has until recently been confused, and the true inci- CLINICAL PRESENTATION dence of the tumour obscured, owing to the various A Chinese woman aged 27 had been resident in names used by different authors to describe their England for eight years. She gave a history of cases. The commonest pseudonym is 'mixed malig- 18 months' intermittent right-sided chest pain http://jcp.bmj.com/ nant tumour',2-4 an ambivalent term which merely and upper abdominal discomfort. -
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. -
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. -
Angiomyolipoma of the Cervix – Report of a Rare Entity
Internet Journal of Medical Update. 2017 July;12(2):13-15. doi: 10.4314/ijmu.v12i2.4 Internet Journal of Medical Update Journal home page: http://www.akspublication.com/ijmu Case Report Angiomyolipoma of the cervix – report of a rare entity N. Hariharanadha Sarmaᴪ1, Rama Srivastava2, Smriti Agnihotri3 1Consultant Pathologist, Department of Pathology, RDT Hospital, Bathalapalli, Andhra Pradesh, India 2Professor, Department of Pathology, SSR Medical College, Belle Rive, Mauritius 3Professor, Department of Pathology, American University of Antigua College of Medicine, Antigua & Barbuda, WI (Received 22 September 2017 and accepted 04 October 2017) ABSTRACT: Angiomyolipoma (AML) is a mesenchymal neoplasm seen usually in the kidney. Few cases of extra renal AML have been documented in various organs including the female genital tract, where the uterus is the most common site. To the best of our knowledge, only 4 cases of AML in the cervix have been reported in the literature. Association of AML with tuberous sclerosis is well known. Presently AML is included in the spectrum of disease entities called PEComa. We report a case of AML without tuberous sclerosis arising from the uterine cervix, which has to be differentiated from lipoleiomyoma. KEY WORDS: Angiomyolipoma; Uterine cervix; PEComas; Uterine tumor INTRODUCTIONV localization8, and mostly in females over 409. The occurrence of AML in the cervix without Angiomyolipoma (AML) occurs most frequently in concurrent incidence in the kidney is extremely the kidney, where it is closely related to tuberous rare, and only four cases have been reported in the sclerosis complex (TSC)1,2, occasionally in other scientific literature10. We are reporting a case of organs, most commonly the liver, but occurrence at uterine cervix AML without tuberous sclerosis other sites is extremely rare3. -
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. -
Diagnostic Immunohistochemistry for Canine Cutaneous Round Cell Tumours — Retrospective Analysis of 60 Cases
FOLIA HISTOCHEMICA ORIGINAL PAPER ET CYTOBIOLOGICA Vol. 57, No. 3, 2019 pp. 146–154 Diagnostic immunohistochemistry for canine cutaneous round cell tumours — retrospective analysis of 60 cases Katarzyna Pazdzior-Czapula, Mateusz Mikiewicz, Michal Gesek, Cezary Zwolinski, Iwona Otrocka-Domagala Department of Pathological Anatomy, Faculty of Veterinary Medicine, University of Warmia and Mazury in Olsztyn, Olsztyn, Poland Abstract Introduction. Canine cutaneous round cell tumours (CCRCTs) include various benign and malignant neoplastic processes. Due to their similar morphology, the diagnosis of CCRCTs based on histopathological examination alone can be challenging, often necessitating ancillary immunohistochemical (IHC) analysis. This study presents a retrospective analysis of CCRCTs. Materials and methods. This study includes 60 cases of CCRCTs, including 55 solitary and 5 multiple tumours, evaluated immunohistochemically using a basic antibody panel (MHCII, CD18, Iba1, CD3, CD79a, CD20 and mast cell tryptase) and, when appropriate, extended antibody panel (vimentin, desmin, a-SMA, S-100, melan-A and pan-keratin). Additionally, histochemical stainings (May-Grünwald-Giemsa and methyl green pyronine) were performed. Results. IHC analysis using a basic antibody panel revealed 27 cases of histiocytoma, one case of histiocytic sarcoma, 18 cases of cutaneous lymphoma of either T-cell (CD3+) or B-cell (CD79a+) origin, 5 cases of plas- macytoma, and 4 cases of mast cell tumours. The extended antibody panel revealed 2 cases of alveolar rhabdo- myosarcoma, 2 cases of amelanotic melanoma, and one case of glomus tumour. Conclusions. Both canine cutaneous histiocytoma and cutaneous lymphoma should be considered at the beginning of differential diagnosis for CCRCTs. While most poorly differentiated CCRCTs can be diagnosed immunohis- tochemically using 1–4 basic antibodies, some require a broad antibody panel, including mesenchymal, epithelial, myogenic, and melanocytic markers. -
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. -
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 -
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.