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      Cherubism: A Rare Fibro-Osseous Disorder Characterized and Diagnosed by one Stop Imaging with Technetium-99m Methylene Diphosphonate Bone Scintigraphy Integrated with Single-Photon Emission Computed Tomography-Computed Tomography

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          Abstract

          Cherubism, a rare hereditary fibro-osseous lesion characterized by painless expansion of jaws, starts early in life manifesting itself fully in the second decade of life and is almost regressed in the third decade. Here, we report a sporadic case of Cherubism with clinico-radiological and scintigraphic presentation of its classical features for its disease rarity and single-photon emission computed tomography/computed tomography findings with review the literature.

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          Familial Multilocular Cystic Disease of the Jaws

          W. Jones (1933)
          Article 0 comments Cited 21 times – based on 0 reviews     Review now
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            Clinical and radiological evaluation of cherubism: a sporadic case report and review of the literature.

            S Basa, A Varol, N Turker … (2003)
            Many cases have been published on cherubism since Jones described it first time in three children of the same family [Am. J. Cancer 17 (1933) 946]. Cherubism is an autosomal-dominant disorder in which the normal bone is replaced by cellular fibrous tissue and immature bone. Extracranial skeletal involvement is rarely seen in hereditary and non-hereditary forms of the disorder. The mandible is the most severely affected craniofacial component, in which uncontrolled growth of the malady deteriorates the aesthetic balance of the face. Bilateral swelling of the cheeks, mandibular enlargement and maxillary spongious hypertrophy cause orbital manifestations and tendency of eyes looking up to the sky. Thus, the pathognomic clinical feature resembles the appearance of "raised to heaven" Renaissance cherubs. The sporadic case concerns a child affected by cherubism. Radiographic and clinical data of the patient are presented and brief review of the literature is included.
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              Cherubism: clinicopathologic features.

              Gary Yamaguchi, S Eisig, H Dorfman (1999)
              A case of cherubism in 6-year-old boy is reported. He presented with bilateral symmetrical enlargement of the jaw in addition to medially dislocated premature teeth, narrow V-shaped palatal vault, and mild upward turning of the eyes. Radiographs showed multiloculated osteolysis in both the mandible and maxilla. Histology revealed a non-neoplastic fibrous lesion, rich in multinucleated giant cells, consistent with giant-cell reparative granuloma. Since the original description of cherubism, various histologic interpretations have been proposed, particularly that of fibrous dysplasia. However, it should be emphasized that cherubism is a disease histologically indistinguishable from giant-cell reparative granuloma.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Indian J Nucl Med
                Journal ID (iso-abbrev): Indian J Nucl Med
                Journal ID (publisher-id): IJNM
                Title: Indian Journal of Nuclear Medicine : IJNM : The Official Journal of the Society of Nuclear Medicine, India
                Publisher: Medknow Publications & Media Pvt Ltd (India )
                ISSN (Print): 0972-3919
                ISSN (Electronic): 0974-0244
                Publication date (Print): Jan-Mar 2019
                Volume: 34
                Issue: 1
                Pages: 62-65
                Affiliations
                [1] Department of Nuclear Medicine and PET-CT, Apollo Hospitals, Chennai, Tamil Nadu, India
                Author notes
                Address for correspondence: Dr. Jaykanth Amalachandran, Department of Nuclear Medicine and PET-CT, Apollo Hospitals, 21, Greams Lane Off Greams Road, Chennai - 600 006, Tamil Nadu, India. E-mail: jaykanthamal@ 123456gmail.com
                Article
                Publisher ID: IJNM-34-62
                DOI: 10.4103/ijnm.IJNM_90_18
                PMC ID: 6352643
                SO-VID: efd66ef9-1ada-427c-9414-380eb48dba89
                Copyright © Copyright: © 2019 Indian Journal of Nuclear Medicine
                License:

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

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                Subject: Case Report

                ScienceOpen disciplines: Radiology & Imaging
                Keywords: cherubism,maxillary lesion,scintigraphy,single-photon emission computed tomography/computed tomography
                Data availability:
                ScienceOpen disciplines: Radiology & Imaging
                Keywords: cherubism, maxillary lesion, scintigraphy, single-photon emission computed tomography/computed tomography

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