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Thymic Carcinoid
Christopher M. Walker, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Thymic neuroendocrine tumor (NET)
              • Well-differentiated neuroendocrine carcinoma of thymus
                • Typical carcinoid of thymus
                • Atypical carcinoid of thymus
            • Definitions

              • Primary thymic neuroendocrine neoplasm, often hormonally active
              • 1-2% of all carcinoids
              • 2-5% of all thymic epithelial neoplasms
            • Associated Syndromes

              • Cushing syndrome: Excess secretion of adrenocorticotrophic hormone (ACTH) (25-40%)
              • Multiple endocrine neoplasia (MEN) type 1, Wermer syndrome (15-25%)
                • Autosomal dominant inheritance, variable penetrance
                • 3 Ps: Hyperparathyroidism (90-95%), pancreatic islet cell tumors (40-80%), pituitary adenomas (30-65%)
                • Multiple facial angiofibromas in 85-90% of patients
                • Less common associations: Thymic carcinoid, adrenal adenoma, adrenal carcinoma, lipoma, and follicular thyroid adenoma
                • Thymic carcinoid usually affects men with MEN type 1 and is hormonally inactive
              • Less common associations including MEN type 2, inappropriate secretion of antidiuretic hormone (SIADH), polymyositis, and polyarthropathy
              • Questionable rare reports of carcinoid syndrome 

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • Nuclear Medicine Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Allan BJ et al: An analysis of 73 cases of pediatric malignant tumors of the thymus. J Surg Res. 184(1):397-403, 2013
                                        2. Ahn S et al: Clinicopathological analysis of 21 thymic neuroendocrine tumors. Korean J Pathol. 46(3):221-5, 2012
                                        3. Elsayes KM et al: Imaging of carcinoid tumors: spectrum of findings with pathologic and clinical correlation. J Comput Assist Tomogr. 35(1):72-80, 2011
                                        4. Kawaguchi K et al: Multiple thymic carcinoids. Ann Thorac Surg. 91(6):1973-5, 2011
                                        5. Nasseri F et al: Clinical and radiologic review of the normal and abnormal thymus: pearls and pitfalls. Radiographics. 30(2):413-28, 2010
                                        6. Intenzo CM et al: Scintigraphic imaging of body neuroendocrine tumors. Radiographics. 27(5):1355-69, 2007
                                        7. Scarsbrook AF et al: Anatomic and functional imaging of metastatic carcinoid tumors. Radiographics. 27(2):455-77, 2007
                                        8. Scarsbrook AF et al: Multiple endocrine neoplasia: spectrum of radiologic appearances and discussion of a multitechnique imaging approach. Radiographics. 26(2):433-51, 2006
                                        9. Groves AM et al: Positron emission tomography with FDG to show thymic carcinoid. AJR Am J Roentgenol. 182(2):511-3, 2004
                                        10. Tamura M et al: Thymic carcinoid tumor. Jpn J Thorac Cardiovasc Surg. 51(1):29-31, 2003
                                        11. Rosado de Christenson ML et al: Thoracic carcinoids: radiologic-pathologic correlation. Radiographics. 19(3):707-36, 1999
                                        12. Birnberg FA et al: Thymic carcinoid tumors with hyperparathyroidism. AJR Am J Roentgenol. 139(5):1001-4, 1982
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Clinical Issues

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Synonyms

                                                    • Thymic neuroendocrine tumor (NET)
                                                    • Well-differentiated neuroendocrine carcinoma of thymus
                                                      • Typical carcinoid of thymus
                                                      • Atypical carcinoid of thymus
                                                  • Definitions

                                                    • Primary thymic neuroendocrine neoplasm, often hormonally active
                                                    • 1-2% of all carcinoids
                                                    • 2-5% of all thymic epithelial neoplasms
                                                  • Associated Syndromes

                                                    • Cushing syndrome: Excess secretion of adrenocorticotrophic hormone (ACTH) (25-40%)
                                                    • Multiple endocrine neoplasia (MEN) type 1, Wermer syndrome (15-25%)
                                                      • Autosomal dominant inheritance, variable penetrance
                                                      • 3 Ps: Hyperparathyroidism (90-95%), pancreatic islet cell tumors (40-80%), pituitary adenomas (30-65%)
                                                      • Multiple facial angiofibromas in 85-90% of patients
                                                      • Less common associations: Thymic carcinoid, adrenal adenoma, adrenal carcinoma, lipoma, and follicular thyroid adenoma
                                                      • Thymic carcinoid usually affects men with MEN type 1 and is hormonally inactive
                                                    • Less common associations including MEN type 2, inappropriate secretion of antidiuretic hormone (SIADH), polymyositis, and polyarthropathy
                                                    • Questionable rare reports of carcinoid syndrome 

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • CT Findings

                                                        • Nuclear Medicine Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Staging, Grading, & Classification

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Allan BJ et al: An analysis of 73 cases of pediatric malignant tumors of the thymus. J Surg Res. 184(1):397-403, 2013
                                                                              2. Ahn S et al: Clinicopathological analysis of 21 thymic neuroendocrine tumors. Korean J Pathol. 46(3):221-5, 2012
                                                                              3. Elsayes KM et al: Imaging of carcinoid tumors: spectrum of findings with pathologic and clinical correlation. J Comput Assist Tomogr. 35(1):72-80, 2011
                                                                              4. Kawaguchi K et al: Multiple thymic carcinoids. Ann Thorac Surg. 91(6):1973-5, 2011
                                                                              5. Nasseri F et al: Clinical and radiologic review of the normal and abnormal thymus: pearls and pitfalls. Radiographics. 30(2):413-28, 2010
                                                                              6. Intenzo CM et al: Scintigraphic imaging of body neuroendocrine tumors. Radiographics. 27(5):1355-69, 2007
                                                                              7. Scarsbrook AF et al: Anatomic and functional imaging of metastatic carcinoid tumors. Radiographics. 27(2):455-77, 2007
                                                                              8. Scarsbrook AF et al: Multiple endocrine neoplasia: spectrum of radiologic appearances and discussion of a multitechnique imaging approach. Radiographics. 26(2):433-51, 2006
                                                                              9. Groves AM et al: Positron emission tomography with FDG to show thymic carcinoid. AJR Am J Roentgenol. 182(2):511-3, 2004
                                                                              10. Tamura M et al: Thymic carcinoid tumor. Jpn J Thorac Cardiovasc Surg. 51(1):29-31, 2003
                                                                              11. Rosado de Christenson ML et al: Thoracic carcinoids: radiologic-pathologic correlation. Radiographics. 19(3):707-36, 1999
                                                                              12. Birnberg FA et al: Thymic carcinoid tumors with hyperparathyroidism. AJR Am J Roentgenol. 139(5):1001-4, 1982