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Macrodystrophia Lipomatosa
Eric Walker, MD, MHA, FACR, FACHE; Catherine C. Roberts, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Nerve territory-oriented macrodactyly, neural fibrolipoma with macrodactyly
          • Definitions

            • Progressive overgrowth of bone and adipose tissue (local gigantism) of single or multiple digits

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Other Modality Findings

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical Features

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    Selected References

                                    1. Southard EJ et al: Macrodystrophia lipomatosa of the foot: a case report of MRI and histologic findings including pacinian corpuscle abnormalities. JBJS Case Connect. 11(2), 2021
                                    2. Jacobs CJ et al: Surgical management for isolated macrodactyly in an adult PIK3CA mutant. JPRAS Open. 26:86-90, 2020
                                    3. Sharma R et al: X-ray and computed tomography findings in macrodystrophia lipomatosa of the foot with secondary osteoarthritic changes diagnosed in an elderly female: a case report. Radiol Bras. 50(2):132-4, 2017
                                    4. Majumdar B et al: Macrodystrophia lipomatosa: review of clinico-radio-histopathological features. Indian Dermatol Online J. 7(4):293-6, 2016
                                    5. Prasetyono TO et al: A review of macrodystrophia lipomatosa: revisitation. Arch Plast Surg. 42(4):391-406, 2015
                                    6. Siddiqui MA et al: Macrodystrophia lipomatosa with ulnar distribution in hand: MR evaluation of a rare disorder. JBR-BTR. 98(1):43-4, 2015
                                    7. Kransdorf MJ et al: Lipomatous tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott Williams & Wilkins. 105-9, 2014
                                    8. Weiss SW et al: Benign lipomatous tumors. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 460-1, 2008
                                    9. Fritz TR et al: Macrodystrophia lipomatosa extending into the upper abdomen. Pediatr Radiol. 37(12):1275-7, 2007
                                    10. Ho CA et al: Long-term follow-up of progressive macrodystrophia lipomatosa. A report of two cases. J Bone Joint Surg Am. 89(5):1097-102, 2007
                                    11. Turkington JR et al: MR imaging of macrodystrophia lipomatosa. Ulster Med J. 74(1):47-50, 2005
                                    12. Murphey MD et al: From the archives of the AFIP: benign musculoskeletal lipomatous lesions. Radiographics. 24(5):1433-66, 2004
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Pathology

                                          • Clinical Issues

                                            TERMINOLOGY

                                            • Synonyms

                                              • Nerve territory-oriented macrodactyly, neural fibrolipoma with macrodactyly
                                            • Definitions

                                              • Progressive overgrowth of bone and adipose tissue (local gigantism) of single or multiple digits

                                            IMAGING

                                            • General Features

                                              • Radiographic Findings

                                                • CT Findings

                                                  • MR Findings

                                                    • Other Modality Findings

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • General Features

                                                          • Gross Pathologic & Surgical Features

                                                            • Microscopic Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      Selected References

                                                                      1. Southard EJ et al: Macrodystrophia lipomatosa of the foot: a case report of MRI and histologic findings including pacinian corpuscle abnormalities. JBJS Case Connect. 11(2), 2021
                                                                      2. Jacobs CJ et al: Surgical management for isolated macrodactyly in an adult PIK3CA mutant. JPRAS Open. 26:86-90, 2020
                                                                      3. Sharma R et al: X-ray and computed tomography findings in macrodystrophia lipomatosa of the foot with secondary osteoarthritic changes diagnosed in an elderly female: a case report. Radiol Bras. 50(2):132-4, 2017
                                                                      4. Majumdar B et al: Macrodystrophia lipomatosa: review of clinico-radio-histopathological features. Indian Dermatol Online J. 7(4):293-6, 2016
                                                                      5. Prasetyono TO et al: A review of macrodystrophia lipomatosa: revisitation. Arch Plast Surg. 42(4):391-406, 2015
                                                                      6. Siddiqui MA et al: Macrodystrophia lipomatosa with ulnar distribution in hand: MR evaluation of a rare disorder. JBR-BTR. 98(1):43-4, 2015
                                                                      7. Kransdorf MJ et al: Lipomatous tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott Williams & Wilkins. 105-9, 2014
                                                                      8. Weiss SW et al: Benign lipomatous tumors. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 460-1, 2008
                                                                      9. Fritz TR et al: Macrodystrophia lipomatosa extending into the upper abdomen. Pediatr Radiol. 37(12):1275-7, 2007
                                                                      10. Ho CA et al: Long-term follow-up of progressive macrodystrophia lipomatosa. A report of two cases. J Bone Joint Surg Am. 89(5):1097-102, 2007
                                                                      11. Turkington JR et al: MR imaging of macrodystrophia lipomatosa. Ulster Med J. 74(1):47-50, 2005
                                                                      12. Murphey MD et al: From the archives of the AFIP: benign musculoskeletal lipomatous lesions. Radiographics. 24(5):1433-66, 2004