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      Congenital diffuse infiltrating facial lipomatosis

      case-report

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          Abstract

          Congenital diffuse infiltrating lipomatosis of the face (CDIL-F) is a rare pathological entity belonging to the subgroup of lipomatous tumors. Till date only a handful of cases has been documented and known to occur exclusively in infancy. On microscopical examination, it is characterized by diffuse infiltration of mature adipose tissue over normal muscle fibers, rapid growth, associated osseous hyperplasia, and a high recurrence rate after surgical intervention. An attempt has been made to identify and characterize all the 49 documented cases of CDIL-F in literature along with describing a report of a male child with CDIL-F. Follow-up of 8 years has been documented. The pathogenesis and spectrum of treatment modality are discussed with identified clinical features.

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          Congenital infiltrating lipomatosis of the face: clinicopathologic evaluation and treatment.

          Congenital lipomatosis of the face is characterized by collections of nonencapsulated, mature lipocytes which infiltrate local tissues and tend to recur after surgery. These lesions represent a distinct clinicopathologic entity that has not been previously reported in this location in children. Three children with congenital lipomatosis of the face were treated at the Institute of Reconstructive Plastic Surgery over a 2- to 14-year follow-up period. In each instance, pathologic evaluation by light and electron microscopy revealed similar lesions sharing the following morphologic criteria: (1) nonencapsulated tumors containing mature fat cells, (2) infiltration of adjacent muscle and soft tissue, (3) absence of malignant characteristics, (4) absence of lipoblasts, (5) presence of fibrous elements in conjunction with increased numbers of nerve bundles and vessels, and (6) hypertrophy of subjacent bone. All three lesions recurred after numerous excisions, some of which were extensive. All were benign by histologic examination and remained so for as long as 14 years. Surgical treatment improved the aesthetic appearance of each child despite evidence of tumor persistence. Although these tumors are benign, we recommend an early aggressive surgical approach to control the infiltrative nature of their growth and to improve facial appearance.
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            Congenital infiltrating lipomatosis of the face: case report and literature review.

            Congenital infiltrating lipomatosis of the face comprises a subgroup of lipomatous tumors. While rare, it remains a definite clinical entity. Its etiology is unknown. The tumor is congenital in origin and occurs in infancy or early childhood. It is poorly enveloped and characterized by diffuse infiltration of mature adipose tissue over normal muscle fibers, rapid growth, associated osseous hyperplasia, and a high recurrence rate after surgical intervention. Due to its diffuse infiltration and involvement of important facial structures, complete surgical excision is often impossible. A total of 14 cases of congenital infiltrating lipomatosis of the face was found in a literature review. Herein we present an additional case who had both diffuse infiltrating lipomatosis and a well-encapsulated lipoma on her left cheek.
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              Congenital infiltrating lipomatosis of the face: report of cases and review of the literature.

              Three cases of infiltrating congenital lipomatosis of the face are presented. These lesions shared the following morphologic criteria: 1) nonencapsulated congenital fatty tumor; 2) infiltration of adjacent muscle and soft tissue; 3) absence of lipoblast and malignant characteristics; 4) presence of fibrous elements with numerous nerve bundles and vessels; and 5) hypertrophy of subjacent bone. Although these tumors are benign, all three recurred after surgical excision. Extensive specimen sampling is advisable to exclude infiltrating lipoma, diffuse angiomatosis, infiltrating angiolipoma, and well-differentiated liposarcoma. Relationship with lipoblastomatosis and histogenesis of infiltrating congenital lipomatosis of the face are discussed.
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                Author and article information

                Journal
                Ann Maxillofac Surg
                Ann Maxillofac Surg
                AMS
                Annals of Maxillofacial Surgery
                Medknow Publications & Media Pvt Ltd (India )
                2231-0746
                2249-3816
                Jul-Dec 2012
                : 2
                : 2
                : 190-196
                Affiliations
                [1]Balaji Dental and Craniofacial Hospital, Teynampet, Chennai, India
                Author notes
                Address for correspondence: Prof. S.M. Balaji, Consultant Craniofacial Surgeon and Director, Balaji Dental and Craniofacial Hospital, 30, KB Dasan Road, Teynampet, Chennai - 600 018, Tamil Nadu, India. E-mail: smbalaji@ 123456gmail.com
                Article
                AMS-2-190
                10.4103/2231-0746.101363
                3591063
                23483013
                557f8b42-e7c2-41cf-b960-ad601a7d7ff9
                Copyright: © Annals of Maxillofacial Surgery

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Categories
                Case Report - Developmental Disorder

                facial tumor,infiltrating lipomatosis,lipomatosis,osseous hypoplasia

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