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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 -
View Open Access Histological Variants of Cutaneous Kaposi Sarcoma Wayne Grayson1 and Liron Pantanowitz*2
Diagnostic Pathology BioMed Central Review Open Access Histological variants of cutaneous Kaposi sarcoma Wayne Grayson1 and Liron Pantanowitz*2 Address: 1Histopathology Department, Ampath National Laboratory Support Services, Johannesburg, South Africa and 2Department of Pathology, Baystate Medical Center, Tufts University School of Medicine, Springfield, Massachusetts, USA Email: Wayne Grayson - [email protected]; Liron Pantanowitz* - [email protected] * Corresponding author Published: 25 July 2008 Received: 23 July 2008 Accepted: 25 July 2008 Diagnostic Pathology 2008, 3:31 doi:10.1186/1746-1596-3-31 This article is available from: http://www.diagnosticpathology.org/content/3/1/31 © 2008 Grayson and Pantanowitz; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Abstract This review provides a comprehensive overview of the broad clinicopathologic spectrum of cutaneous Kaposi sarcoma (KS) lesions. Variants discussed include: usual KS lesions associated with disease progression (i.e. patch, plaque and nodular stage); morphologic subtypes alluded to in the older literature such as anaplastic and telangiectatic KS, as well as several lymphedematous variants; and numerous recently described variants including hyperkeratotic, keloidal, micronodular, pyogenic granuloma-like, ecchymotic, and intravascular KS. Involuting lesions as a result of treatment related regression are also presented. Introduction progression, (2) KS variants alluded to in the older litera- Kaposi sarcoma (KS) is a vascular lesion of low-grade ture, (3) more recently described KS variants, and (4) KS malignant potential that presents most frequently with lesions as a consequence of therapy. -
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 -
Aggressive Angiomyxoma in Pregnancy: a Rare and Commonly Misdiagnosed Entity Kamal Malukani, Amit V
Published online: 2020-02-19 Case Report Access this article online Quick Response Code: Aggressive angiomyxoma in pregnancy: A rare and commonly misdiagnosed entity Kamal Malukani, Amit V. Varma, Devashish Choudhary, Shilpi Dosi Website: www.jlponline.org Abstract: DOI: 10.4103/JLP.JLP_179_17 Aggressive angiomyxoma (AAM) is an uncommon mesenchymal tumor that predominantly involves the pelvis and perineum of young females. It is often clinically mistaken for more common superficial lesions such as vaginal cysts, labial cysts, and lipomas. A review of the medical literature reveals very few cases of AAM reported in pregnancy. We describe a rare case of AAM in pregnancy, clinically misdiagnosed as prolapsed cervical fibroid. Key words: Aggressive angiomyxoma, mesenchymal tumors, pregnancy Introduction (G2 P1 L1), married for 5 years, presented with bleeding per vagina for 3 days. She ggressive angiomyxoma (AAM) is also complained of huge mass coming out Aa rare mesenchymal neoplasm of of vagina, difficulty in walking, and pain vulvo‑perineal region and usually found in abdomen and lower back. There was [1] in women of reproductive age group. It no history of fever, weight loss, trauma, is a slow‑growing, low‑grade neoplasm bowel or bladder disturbance, and use of but is locally infiltrative, and relapse any contraceptive. Her menstrual history has been reported in about 30%–40% of and past history were unremarkable. Local cases even after many years of complete examination showed a large pedunculated, resection.[2] Rarely, AAM can metastasize well‑circumscribed, ulcerated mass coming to lungs, peritoneum, and lymph nodes.[3] out of the vagina. Only a few cases of AAM coexistent Ultrasound of abdomen showed with pregnancy are reported in medical bulky anteverted uterus, measuring literature.[4,5] AAM grows to a huge size 9.6 cm × 7.6 cm × 5.4 cm. -
Desterrando El Término Ewing-Like Sarcoma: ¿Qué Entidades Se Cobijaban Bajo Esa Denominación?
Desterrando el término Ewing-like sarcoma: ¿Qué entidades se cobijaban bajo esa denominación? Sílvia Bagué Servei de Patologia Hospital de la Santa Creu i Sant Pau Universitat Autònoma de Barcelona #SEOM20 No disclosures Small round cell sarcomas (SRCS) - Group of malignant neoplasms sharing histological similar features - Mainly in children & young adults - High-grade by definition. Often “translocated-sarcomas” • Ewing sarcoma • “Ewing-like” sarcomas • Desmoplastic SRCT • Alveolar rhabdomyosarcoma • Poorly diff sinovial sarcoma • Mesenchymal chondrosarcoma • High-grade myxoid liposarcoma • Small cell osteosarcoma • Undiff sarcoma with round cell morphology (USRCS) #SEOM203 Ewing sarcoma • Most common round cell sarcoma • 1st - 2nd decade • 85% diaphysis-metaphysis long bones > pelvis, ribs, chest wall (‘Askin’) • 15% extraeskeletal (adults) • Key morphologic features: uniform monotonous round cells, fine chromatin, inconspicious nucleoli, scant clear to pale cytoplasm • Key immunohistochemical stains: strong and diffuse membranous CD99 expression; NKX2+, PAX7+ • Genetics: fusion between EFT genes (FUS, EWSR1) and TAF15 genes and ETS transcription factor family members (Fli-1, ERG, ETV1, ETV4, FEV, E1AF, ZSG) 85-90% t(11;22)(q24;q12) EWSR1- FLI1 fusion gene 5-10 % t(21;22)(q22;q12) EWSR1-ERG fusion gene CD99 FISH EWSR1 #SEOM204 Desmoplastic small round cell tumor Clinical history Boy, 12 y-o. Intraabdominal mass #SEOM205 DSRCT: IHC & genetics Malignant round cell tumor with desmoplastic stroma, poliphenotypic differentiation and translocation -
The 2020 WHO Classification of Soft Tissue Tumours: News and Perspectives
PATHOLOGICA 2021;113:70-84; DOI: 10.32074/1591-951X-213 Review The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives Marta Sbaraglia1, Elena Bellan1, Angelo P. Dei Tos1,2 1 Department of Pathology, Azienda Ospedale Università Padova, Padova, Italy; 2 Department of Medicine, University of Padua School of Medicine, Padua, Italy Summary Mesenchymal tumours represent one of the most challenging field of diagnostic pathol- ogy and refinement of classification schemes plays a key role in improving the quality of pathologic diagnosis and, as a consequence, of therapeutic options. The recent publica- tion of the new WHO classification of Soft Tissue Tumours and Bone represents a major step toward improved standardization of diagnosis. Importantly, the 2020 WHO classi- fication has been opened to expert clinicians that have further contributed to underline the key value of pathologic diagnosis as a rationale for proper treatment. Several rel- evant advances have been introduced. In the attempt to improve the prediction of clinical behaviour of solitary fibrous tumour, a risk assessment scheme has been implemented. NTRK-rearranged soft tissue tumours are now listed as an “emerging entity” also in con- sideration of the recent therapeutic developments in terms of NTRK inhibition. This deci- sion has been source of a passionate debate regarding the definition of “tumour entity” as well as the consequences of a “pathology agnostic” approach to precision oncology. In consideration of their distinct clinicopathologic features, undifferentiated round cell sarcomas are now kept separate from Ewing sarcoma and subclassified, according to the underlying gene rearrangements, into three main subgroups (CIC, BCLR and not Received: October 14, 2020 ETS fused sarcomas) Importantly, In order to avoid potential confusion, tumour entities Accepted: October 19, 2020 such as gastrointestinal stroma tumours are addressed homogenously across the dif- Published online: November 3, 2020 ferent WHO fascicles. -
Multinucleate Cell Angiohistiocytoma
To protect the rights of the author(s) and publisher we inform you that this PDF is an uncorrected proof for internal business use only by the author(s), editor(s), reviewer(s), Elsevier and typesetter Toppan Best-set. It is not allowed to publish this proof online or in print. This proof copy is the copyright property of the publisher and is confidential until formal publication. These proofs may contain color(colour) figures. Those figures may print black and white in the final printed book if a color(colour) print product has not been planned. The color(colour) figures will appear in color(colour) in all electronic versions of this book. s0060 MULTINUCLEATE CELL ANGIOHISTIOCYTOMA s0065 Definition • Fibroblast-like and histiocyte-like mononuclear cells u0390 p0300 • A distinctive benign dermal proliferation composed • Thickened collagen bundles, frequently hyalinized u0395 of thin-walled capillaries and veins, admixed with • Occasional inflammatory cells, predominantly u0400 scattered multinucleated cells lymphocytes • Hemorrhage absent, no hemosiderin deposition u0405 s0070 Clinical features • Decreased elastic fibers in the dermis can be observed u0410 s0075 Epidemiology • Overlying epidermis normal, but can also be u0415 p0310 • Female predominance (F:M = 3 : 1) hyperplastic u0275 • Middle-aged adult patients • Proliferation restricted to upper and middermis u0420 s0080 Presentation Immunopathology/special stains s0100 p0325 • Slowly growing single or multiple firm, red-brown to • Multinucleated cells display variable CD68 positivity -
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DERMATOLOGY PRACTICAL & CONCEPTUAL www.derm101.com An unusual lesion on the nose: microvenular hemangioma A. Tulin Mansur1, Gulsen Tukenmez Demirci2, Eltaf A. Ozbal Koc3, Semsi Yildiz4 1 Dermatology Department, Istanbul Hospital, Baskent University, Istanbul, Turkey 2 Dermatology Department, Acibadem Altunizade Hospital, Acibadem University, Istanbul, Turkey 3 Ear, Nose and Throat Diseases Department, Istanbul Hospital, Baskent University, Istanbul, Turkey 4 Pathology Department, Istanbul Hospital, Baskent University, Istanbul, Turkey Key words: vascular anomaly, hemangioma Citation: Mansur AT, Tukenmez Demirci G, Ozbal Koc EA, Yildiz S. An unusual lesion on the nose: microvenular hemangioma. Dermatol Pract Concept. 2018;8(1):7-11. DOI: https://doi.org/10.5826/dpc.0801a02 Received: April 25, 2017; Accepted: October 6, 2017; Published: January 31, 2018 Copyright: ©2018 Mansur et al. 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 author and source are credited. Funding: None. Competing interests: The authors have no conflicts of interest to disclose. All authors have contributed significantly to this publication. Corresponding author: Gulsen Tukenmez Demirci, Associate Professor, Dermatology Department, Acibadem Altunizade Hospital, Üsküdar 34662, Istanbul,˙ Turkey. Email: [email protected] ABSTRACT Microvenular hemangioma (MVH) is an acquired, benign type of hemangioma that usually manifests itself as a solitary, slowly growing, red to violaceous, asymptomatic papule, plaque or nodule. It is typically located on the trunk or extremities of young adults. It can be difficult to differentiate MVH from other types of hemangioma and Kaposi sarcoma. Herein we report a case of MVH unusual for its location, age of onset, and morphologic features. -
MR Imaging of Aggressive Angiomyxoma of the Female Pelvis: Case Report
MR Imaging of Aggressive Angiomyxoma of the Female Pelvis: Case Report Hye Mi Kim1, Ji Eun Pyo2, Nam Hoon Cho2, Nam Kyu Kim3, and Myeong-Jin Kim1 Aggressive angiomyxoma is a rare tumor that predominantly occurs in the female genital tract. Because of its high tendency for local recurrence, preoperative diagnosis is important to ensure wide excision and reduce recurrence risk. But it is often misdiagnosed due to its rarity. We present two cases of aggressive angiomyxoma: a recurrent case that was initially misdiagnosed and a preoperatively diagnosed case. Index words : Myxoma Magnetic resonance (MR) Vulva Female Perineum Case 1 Introduction A 43-year-old woman presented with a non tender mass in the right perirectal area in 1999. Magnetic Aggressive angiomyxoma is a recently characterized resonance (MR) imaging showed a large soft-tissue, 12 benign mesenchymal tumor occurring predominantly ×8×5 cm mass, mainly located at the right perineum in the perineal area, with a strong female extending to the perirectal space. The mass displaced predominance in the 3rd-5th decades of life (1). It has a the adjacent soft tissue and splitted the internal and high propensity for local recurrence, thus preoperative external right levator ani sphincter muscle. It was diagnosis is important for planning surgical isointense to muscle on T1-wighted and hyperintense management (2). But it has been often misdiagnosed on T2-weighted images, with a whirling pattern (Fig. 1). due to its rarity (3). Differential diagnosis included a neurogenic tumor, To our knowledge, the imagings of aggressive myxoid liposarcoma, angiomyolipoma or other soft angiomyxoma have not been reported in Korea. -
Cellular Angiofibroma: a Rare Vulvar Neoplasm Distinct from Aggressive Angiomyxoma Jasvinder Kaur Bhatia*1, R Nangia2
Case Report Cellular Angiofibroma: A Rare Vulvar Neoplasm Distinct From Aggressive Angiomyxoma Jasvinder Kaur Bhatia*1, R Nangia2 1Department of Pathology, Armed Forces Medical college Pune, India 2SR Adv Pathology, CHCC Lucknow, India Keywords: Cellular Angiofibroma, Vulva, Immunohistochemistry, Aggressive Angiomyxoma. ABSTRACT Cellular angiofibroma (CA) is a rare benign mesenchymal neoplasm of the genital region of both the genders. In women, it arises in late reproductive age and can be cured by complete local excision. It is characterized by a bland spindle cell population and numerous small- to medium-sized vessels with hyalinization. We report a rare case in a postmenopausal woman with a view to highlight the importance of differentiating it from similar more aggressive tumours like aggressive angiomyxoma. A 56 year old woman presented with superficial painless swelling of the vulva and the mass was surgically excised. Histological examination revealed spindle cell proliferation and hyalinised blood vessels in a loosely cellular stroma. Immunohistochemistry revealed positivity for vimentin and CD34 and positivity for ER & PR. *Corresponding author: Dr Jasvinder Kaur Bhatia, Department of Pathology, Armed Forces Medical College, Pune-40, India Phone: +91 - 8552825142 E-mail: [email protected] This work is licensed under the Creative Commons Attribution 4.0 License. Published by Pacific Group of e-Journals (PaGe) C-214 Cellular Angiofibroma Introduction Immunohistochemistry: The tumour cells were positive Cellular Angiofibroma (CA) is a rare benign mesenchymal for vimentin and CD34, ER and PR. (Fig 4, 5, 6, 7) The neoplasm of the genital region of both the genders. It arises tumour was negative for S-100 protein, actin, desmin and in women of late reproductive age [1, 2] and can be cured EMA. -
Conversion of Morphology of ICD-O-2 to ICD-O-3
NATIONAL INSTITUTES OF HEALTH National Cancer Institute to Neoplasms CONVERSION of NEOPLASMS BY TOPOGRAPHY AND MORPHOLOGY from the INTERNATIONAL CLASSIFICATION OF DISEASES FOR ONCOLOGY, SECOND EDITION to INTERNATIONAL CLASSIFICATION OF DISEASES FOR ONCOLOGY, THIRD EDITION Edited by: Constance Percy, April Fritz and Lynn Ries Cancer Statistics Branch, Division of Cancer Control and Population Sciences Surveillance, Epidemiology and End Results Program National Cancer Institute Effective for cases diagnosed on or after January 1, 2001 TABLE OF CONTENTS Introduction .......................................... 1 Morphology Table ..................................... 7 INTRODUCTION The International Classification of Diseases for Oncology, Third Edition1 (ICD-O-3) was published by the World Health Organization (WHO) in 2000 and is to be used for coding neoplasms diagnosed on or after January 1, 2001 in the United States. This is a complete revision of the Second Edition of the International Classification of Diseases for Oncology2 (ICD-O-2), which was used between 1992 and 2000. The topography section is based on the Neoplasm chapter of the current revision of the International Classification of Diseases (ICD), Tenth Revision, just as the ICD-O-2 topography was. There is no change in this Topography section. The morphology section of ICD-O-3 has been updated to include contemporary terminology. For example, the non-Hodgkin lymphoma section is now based on the World Health Organization Classification of Hematopoietic Neoplasms3. In the process of revising the morphology section, a Field Trial version was published and tested in both the United States and Europe. Epidemiologists, statisticians, and oncologists, as well as cancer registrars, are interested in studying trends in both incidence and mortality. -
UC Davis Dermatology Online Journal
UC Davis Dermatology Online Journal Title Erythematous asymptomatic nodule on the sole Permalink https://escholarship.org/uc/item/9969k7kp Journal Dermatology Online Journal, 23(10) Authors Paret-Sanz, Cristina del Alcázar-Viladomiu, Elena Pérez-Muñoz, Noelia et al. Publication Date 2017 DOI 10.5070/D32310037003 License https://creativecommons.org/licenses/by-nc-nd/4.0/ 4.0 Peer reviewed eScholarship.org Powered by the California Digital Library University of California Volume 23 Number 10 | October 2017 Dermatology Online Journal || Case Presentation DOJ 23 (10): 12 Erythematous asymptomatic nodule on the sole Cristina Paret-Sanz1 MD, Elena del Alcázar-Viladomiu MD, Noelia Pérez-Muñoz2 MD, Ana Iglesias-Plaza1 MD, Montserrat Salleras-Redonnet1 MD PhD Affiliations: 1Department of Dermatology, Hospital Universitari Sagrat Cor, Barcelona, Spain, 2Department of Pathology, Hospital Universitari Sagrat Cor, Barcelona, Spain Corresponding Author: Cristina Paret Sanz MD, Department of Dermatology, Hospital Universitari Sagrat Cor, Carrer de Viladomat, 288, 08029 Barcelona, Spain, Email address: [email protected] Abstract A 75-year-old man presented to the dermatology clinic with an asymptomatic lesion on his right plantar surface. The lesion had progressively grown for two months. Physical examination revealed an erythematous and slightly scaly nodule measuring 10x10 mm. Dermoscopy examination showed central diffuse erythema with small red globules. A punch- biopsy revealed a proliferation of irregularly branched small vessels with collapsed lumen, extending in an infiltrative pattern in the superficial and deep dermis. Although this is a rare location, a diagnosis of microvenular hemangioma was made. Keywords: vascular neoplasm; microvenular hemangioma, hemangioma Introduction Microvenular hemangioma is a rare, benign, acquired vascular tumor, which usually occurs as a solitary slowly enlarging, red, purple, or reddish-blue plaque or nodule of less than 30 mm in diameter.