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Lymphatic Malformations
Stephanie A. Bernard, MD; Catherine C. Roberts, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Cystic hygroma or lymphangioma
          • Definitions

            • Lymphatic malformation (LM): benign developmental lesion composed of dilated lymphatic channels
            • Lymphangiomatosis: multicentric or extensive infiltration; typically traversing tissue planes

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Angiographic Findings

                      • Nuclear Medicine Findings

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        Selected References

                                        1. de Keyser CE et al: A large skull defect due to Gorham-Stout disease: case report and literature review on pathogenesis, diagnosis, and treatment. Front Endocrinol (Lausanne). 11:37, 2020
                                        2. White CL et al: Low-flow vascular malformation pitfalls: from clinical examination to practical imaging evaluation--part 1, lymphatic malformation mimickers. AJR Am J Roentgenol. 206(5):940-51, 2016
                                        3. ISSVA: ISSVA classification for vascular anomalies. Published April 2014. Updated May 2018. https://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf
                                        4. Nikolaou VS et al: Vanishing bone disease (Gorham-Stout syndrome): a review of a rare entity. World J Orthop. 5(5):694-8, 2014
                                        5. Behr GG et al: Vascular anomalies: hemangiomas and beyond--part 2, slow-flow lesions. AJR Am J Roentgenol. 200(2):423-36, 2013
                                        6. Flors L et al: MR imaging of soft-tissue vascular malformations: diagnosis, classification, and therapy follow-up. Radiographics. 31(5):1321-40; discussion 1340-1, 2011
                                        7. Yoo JC et al: OK-432 sclerotherapy in head and neck lymphangiomas: long-term follow-up result. Otolaryngol Head Neck Surg. 140(1):120-3, 2009
                                        8. Weiss SW et al: Tumors of lymph vessels. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 734-43, 2008
                                        9. Kransdorf MJ et al: Vascular and lymphatic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Lippincott Williams & Wilkins. 165-8, 2006
                                        10. Beham A: Lymphangioma. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 162-3, 2002
                                        11. Hardegger F et al: The syndrome of idiopathic osteolysis. Classification, review, and case report. J Bone Joint Surg Br. 67(1):88-93, 1985
                                        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

                                                  • Cystic hygroma or lymphangioma
                                                • Definitions

                                                  • Lymphatic malformation (LM): benign developmental lesion composed of dilated lymphatic channels
                                                  • Lymphangiomatosis: multicentric or extensive infiltration; typically traversing tissue planes

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • CT Findings

                                                      • MR Findings

                                                        • Ultrasonographic Findings

                                                          • Angiographic Findings

                                                            • Nuclear Medicine Findings

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Gross Pathologic & Surgical Features

                                                                    • Microscopic Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              Selected References

                                                                              1. de Keyser CE et al: A large skull defect due to Gorham-Stout disease: case report and literature review on pathogenesis, diagnosis, and treatment. Front Endocrinol (Lausanne). 11:37, 2020
                                                                              2. White CL et al: Low-flow vascular malformation pitfalls: from clinical examination to practical imaging evaluation--part 1, lymphatic malformation mimickers. AJR Am J Roentgenol. 206(5):940-51, 2016
                                                                              3. ISSVA: ISSVA classification for vascular anomalies. Published April 2014. Updated May 2018. https://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf
                                                                              4. Nikolaou VS et al: Vanishing bone disease (Gorham-Stout syndrome): a review of a rare entity. World J Orthop. 5(5):694-8, 2014
                                                                              5. Behr GG et al: Vascular anomalies: hemangiomas and beyond--part 2, slow-flow lesions. AJR Am J Roentgenol. 200(2):423-36, 2013
                                                                              6. Flors L et al: MR imaging of soft-tissue vascular malformations: diagnosis, classification, and therapy follow-up. Radiographics. 31(5):1321-40; discussion 1340-1, 2011
                                                                              7. Yoo JC et al: OK-432 sclerotherapy in head and neck lymphangiomas: long-term follow-up result. Otolaryngol Head Neck Surg. 140(1):120-3, 2009
                                                                              8. Weiss SW et al: Tumors of lymph vessels. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 734-43, 2008
                                                                              9. Kransdorf MJ et al: Vascular and lymphatic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Lippincott Williams & Wilkins. 165-8, 2006
                                                                              10. Beham A: Lymphangioma. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 162-3, 2002
                                                                              11. Hardegger F et al: The syndrome of idiopathic osteolysis. Classification, review, and case report. J Bone Joint Surg Br. 67(1):88-93, 1985