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Juvenile Hyaline Fibromatosis
Catherine C. Roberts, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Molluscum fibrosum, mesenchymal dysplasia, fibromatosis hyalinica multiplex juvenilis, puretic syndrome, systemic hyalinosis
            • Definitions

              • Rare congenital disease producing subcutaneous tumors, gingival hypertrophy, osteolytic bone lesions, and flexion joint contractures

            IMAGING

            • General Features

              • Radiographic Findings

                • MR Findings

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Gross Pathologic & Surgical Features

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  Selected References

                                  1. Çam B et al: Juvenile hyaline fibromatosis: a case report follow-up after 3 years and a review of the literature. Int J Dermatol. 54(2):217-21, 2015
                                  2. Jaouad IC et al: Hyaline fibromatosis syndrome with mutation c.1074delT of the CMG2 gene: a case report. J Med Case Rep. 8:291, 2014
                                  3. Raeeskarami SR et al: Infantile Systemic Hyalinosis: Report of 17-year Experience. Iran J Pediatr. 24(6):775-8, 2014
                                  4. Van Raak SM et al: Hyaline fibromatosis of Hoffa's fat pad in a patient with a mild type of hyaline fibromatosis syndrome. Skeletal Radiol. 43(4):531-4, 2014
                                  5. Weiss SW et al: Fibrous tumors of infancy and childhood. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Philadelphia: Elsevier. 269-72, 2008
                                  6. Kransdorf MJ et al: Benign fibrous and fibrohistiocytic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Philadelphia: Lippincott Williams & Wilkins. 207-9, 2006
                                  7. O' Connell J: Juvenile hyaline fibromatosis. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press. 63-4, 2002
                                  8. Keser G et al: Two siblings with juvenile hyaline fibromatosis: case reports and review of the literature. Clin Rheumatol. 18(3):248-52, 1999
                                  Related Anatomy
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                                  Related Differential Diagnoses
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                                  References
                                  Tables

                                  Tables

                                  KEY FACTS

                                  • Terminology

                                    • Imaging

                                      • Top Differential Diagnoses

                                        • Pathology

                                          • Clinical Issues

                                            TERMINOLOGY

                                            • Synonyms

                                              • Molluscum fibrosum, mesenchymal dysplasia, fibromatosis hyalinica multiplex juvenilis, puretic syndrome, systemic hyalinosis
                                            • Definitions

                                              • Rare congenital disease producing subcutaneous tumors, gingival hypertrophy, osteolytic bone lesions, and flexion joint contractures

                                            IMAGING

                                            • General Features

                                              • Radiographic Findings

                                                • MR Findings

                                                  DIFFERENTIAL DIAGNOSIS

                                                    PATHOLOGY

                                                    • General Features

                                                      • Gross Pathologic & Surgical Features

                                                        • Microscopic Features

                                                          CLINICAL ISSUES

                                                          • Presentation

                                                            • Demographics

                                                              • Natural History & Prognosis

                                                                • Treatment

                                                                  Selected References

                                                                  1. Çam B et al: Juvenile hyaline fibromatosis: a case report follow-up after 3 years and a review of the literature. Int J Dermatol. 54(2):217-21, 2015
                                                                  2. Jaouad IC et al: Hyaline fibromatosis syndrome with mutation c.1074delT of the CMG2 gene: a case report. J Med Case Rep. 8:291, 2014
                                                                  3. Raeeskarami SR et al: Infantile Systemic Hyalinosis: Report of 17-year Experience. Iran J Pediatr. 24(6):775-8, 2014
                                                                  4. Van Raak SM et al: Hyaline fibromatosis of Hoffa's fat pad in a patient with a mild type of hyaline fibromatosis syndrome. Skeletal Radiol. 43(4):531-4, 2014
                                                                  5. Weiss SW et al: Fibrous tumors of infancy and childhood. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Philadelphia: Elsevier. 269-72, 2008
                                                                  6. Kransdorf MJ et al: Benign fibrous and fibrohistiocytic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Philadelphia: Lippincott Williams & Wilkins. 207-9, 2006
                                                                  7. O' Connell J: Juvenile hyaline fibromatosis. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press. 63-4, 2002
                                                                  8. Keser G et al: Two siblings with juvenile hyaline fibromatosis: case reports and review of the literature. Clin Rheumatol. 18(3):248-52, 1999