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      • Article: found

      Inflammatory Myofibroblastic Tumor of the Orbit


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          The authors describe an unusual case of orbital inflammatory myofibroblastic tumor (IMT) in a 17-year-old patient who presented with a painful exophthalmos of the left eye. After complete surgical excision, the mass was diagnosed as an IMT based on morphological and immunohistochemical features. No tumor recurrence was evident during 28-month follow-up. The authors discuss histopathological and immunohistochemical characteristics and review the literature of orbital IMT.

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          Most cited references3

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          ALK1 and p80 expression and chromosomal rearrangements involving 2p23 in inflammatory myofibroblastic tumor.

          Inflammatory myofibroblastic tumor (IMT) is an uncommon tumor of extrapulmonary and pulmonary tissues with an unpredictable clinical course, occasional recurrences, and rare malignant transformation. Clonal abnormalities with rearrangements of chromosome of 2p23 and the ALK gene have been reported in a few cases. The purpose of this study is to investigate whether these are consistent abnormalities among IMTs or represent a distinct subset. Formalin-fixed, paraffin-embedded archival tissue sections from 47 IMTs in 40 patients were immunostained with monoclonal antibodies against ALK and p80. Fluorescence in situ hybridization for ALK rearrangements was done on 22 IMTs from 19 patients. Findings were correlated with clinical features and outcome. ALK positivity was observed in 17 of 47 IMTs (36%) and p80 positivity in 16 of 47 IMTs (34%). Fluorescence in situ hybridization showed ALK rearrangements in nine cases (47%), aneuploidy in three cases (16%), and no rearrangement in seven cases (37%). IMTs with ALK abnormalities by immunohistochemistry and/or fluorescence in situ hybridization originated in the abdomen/pelvis/retroperitoneum, chest, and extremities. The mean age was 6.6 years, with a male/female ratio of 1.3. 64% of patients had no evidence of disease at last follow-up, 45% had one or more recurrences, and 18% displayed histologic evidence of malignant transformation. The IMTs without ALK abnormalities occurred in older children, were more frequent in females, and had fewer recurrences. However, in this group of 40 patients, the differences between the groups with and without ALK abnormalities did not have statistical significance. Aneuploidy without ALK abnormalities was associated with malignant transformation in three of five cases. Abnormalities of ALK and p80 and evidence of chromosomal rearrangements of 2p23 occur in a significant proportion of IMTs. These changes are most frequent in abdominal and pulmonary IMTs in the first decade of life and are associated with a higher frequency of recurrence. These findings confirm the neoplastic nature of a subset IMT with ALK abnormalities and suggest that aneuploid IMT is a subset with more aggressive clinical behavior.
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            Anaplastic Lymphoma Kinase (ALK) Expression in the Inflammatory Myofibroblastic Tumor

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              Inflammatory myofibroblastic tumor of the orbit presenting as a subconjunctival mass.

              To present a case of inflammatory myofibroblastic tumor in the anterior orbit and to describe its clinical features, diagnosis, and management.

                Author and article information

                S. Karger AG
                August 2007
                30 August 2007
                : 221
                : 5
                : 353-355
                Departments of aOphthalmology and Neurosurgery and bPathology, University of Siena, Siena, Italy
                104767 Ophthalmologica 2007;221:353–355
                © 2007 S. Karger AG, Basel

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                : 21 September 2006
                : 22 February 2007
                Page count
                Figures: 2, References: 5, Pages: 3
                Case Report

                Vision sciences,Ophthalmology & Optometry,Pathology
                Inflammatory myofibroblastic tumor,Spindle cells,Orbit


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