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Atypical Carcinoid
Carol C. Wu, MD; Emily Tsai, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Atypical carcinoid (AC)
              • Synonyms

                • "Bronchial adenoma" formerly used to describe tumors arising in central airways (trachea and mainstem bronchi)
                  • Encompassed several central airway malignant neoplasms
                    • Bronchial carcinoid
                    • Adenoid cystic carcinoma
                    • Mucoepidermoid carcinoma
                  • All carcinoid tumors are malignant; therefore, term "adenoma" should not be used
              • Definitions

                • Intermediate-grade malignant neuroendocrine neoplasm
                • Arises from normal neuroendocrine cells in tracheobronchial epithelium
                • 2-10 mitoses/10 high-power fields (HPF) or necrosis

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Nuclear Medicine Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Consider

                                              Selected References

                                              1. Caplin ME et al: Pulmonary Neuroendocrine (Carcinoid) Tumors: European Neuroendocrine Tumor Society Expert Consensus and Recommendations for Best Practice for Typical and Atypical Pulmonary Carcinoid. Ann Oncol. ePub, 2015
                                              2. Chong CR et al: Chemotherapy for locally advanced and metastatic pulmonary carcinoid tumors. Lung Cancer. 86(2):241-6, 2014
                                              3. Filosso PL et al: Clinical management of atypical carcinoid and large-cell neuroendocrine carcinoma: a multicentre study on behalf of the European Society of Thoracic Surgeons (ESTS) Neuroendocrine Tumours of the Lung Working Group. Eur J Cardiothorac Surg. 48(1):55-64, 2015.
                                              4. Lococo F et al: PET/CT assessment of neuroendocrine tumors of the lung with special emphasis on bronchial carcinoids. Tumour Biol. 35(9):8369-77, 2014
                                              5. Steuer CE et al: Atypical Carcinoid Tumor of the Lung: a Surveillance, Epidemiology, and End Results Database Analysis. J Thorac Oncol. ePub, 2014
                                              6. Travis WD: Pathology and diagnosis of neuroendocrine tumors: lung neuroendocrine. Thorac Surg Clin. 24(3):257-66, 2014
                                              7. Benson RE et al: Spectrum of pulmonary neuroendocrine proliferations and neoplasms. Radiographics. 33(6):1631-49, 2013
                                              8. Travis WD: Advances in neuroendocrine lung tumors. Ann Oncol. 21 Suppl 7:vii65-71, 2010
                                              9. Aubry MC et al: Significance of multiple carcinoid tumors and tumorlets in surgical lung specimens: analysis of 28 patients. Chest. 131(6):1635-43, 2007
                                              10. Davies SJ et al: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease. Thorax. 62(3):248-52, 2007
                                              11. Divisi D et al: Carcinoid tumors of the lung and multimodal therapy. Thorac Cardiovasc Surg. 53(3):168-72, 2005
                                              12. Renshaw AA et al: Distinguishing carcinoid tumor from small cell carcinoma of the lung: correlating cytologic features and performance in the College of American Pathologists Non-Gynecologic Cytology Program. Arch Pathol Lab Med. 129(5):614-8, 2005
                                              13. Jeung MY et al: Bronchial carcinoid tumors of the thorax: spectrum of radiologic findings. Radiographics. 22(2):351-65, 2002
                                              14. Fraser RS et al: Fraser and Pare's Diagnosis of Diseases of the Chest, vol. II. 4th ed. Philadelphia: W.B. Saunders. 1229-43, 1999
                                              15. Rosado de Christenson ML et al: Thoracic carcinoids: radiologic-pathologic correlation. Radiographics. 19(3):707-36, 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

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Abbreviations

                                                            • Atypical carcinoid (AC)
                                                          • Synonyms

                                                            • "Bronchial adenoma" formerly used to describe tumors arising in central airways (trachea and mainstem bronchi)
                                                              • Encompassed several central airway malignant neoplasms
                                                                • Bronchial carcinoid
                                                                • Adenoid cystic carcinoma
                                                                • Mucoepidermoid carcinoma
                                                              • All carcinoid tumors are malignant; therefore, term "adenoma" should not be used
                                                          • Definitions

                                                            • Intermediate-grade malignant neuroendocrine neoplasm
                                                            • Arises from normal neuroendocrine cells in tracheobronchial epithelium
                                                            • 2-10 mitoses/10 high-power fields (HPF) or necrosis

                                                          IMAGING

                                                          • General Features

                                                            • Radiographic Findings

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Nuclear Medicine Findings

                                                                    • Imaging Recommendations

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Staging, Grading, & Classification

                                                                            • Gross Pathologic & Surgical Features

                                                                              • Microscopic Features

                                                                                CLINICAL ISSUES

                                                                                • Presentation

                                                                                  • Demographics

                                                                                    • Natural History & Prognosis

                                                                                      • Treatment

                                                                                        DIAGNOSTIC CHECKLIST

                                                                                        • Consider

                                                                                          Selected References

                                                                                          1. Caplin ME et al: Pulmonary Neuroendocrine (Carcinoid) Tumors: European Neuroendocrine Tumor Society Expert Consensus and Recommendations for Best Practice for Typical and Atypical Pulmonary Carcinoid. Ann Oncol. ePub, 2015
                                                                                          2. Chong CR et al: Chemotherapy for locally advanced and metastatic pulmonary carcinoid tumors. Lung Cancer. 86(2):241-6, 2014
                                                                                          3. Filosso PL et al: Clinical management of atypical carcinoid and large-cell neuroendocrine carcinoma: a multicentre study on behalf of the European Society of Thoracic Surgeons (ESTS) Neuroendocrine Tumours of the Lung Working Group. Eur J Cardiothorac Surg. 48(1):55-64, 2015.
                                                                                          4. Lococo F et al: PET/CT assessment of neuroendocrine tumors of the lung with special emphasis on bronchial carcinoids. Tumour Biol. 35(9):8369-77, 2014
                                                                                          5. Steuer CE et al: Atypical Carcinoid Tumor of the Lung: a Surveillance, Epidemiology, and End Results Database Analysis. J Thorac Oncol. ePub, 2014
                                                                                          6. Travis WD: Pathology and diagnosis of neuroendocrine tumors: lung neuroendocrine. Thorac Surg Clin. 24(3):257-66, 2014
                                                                                          7. Benson RE et al: Spectrum of pulmonary neuroendocrine proliferations and neoplasms. Radiographics. 33(6):1631-49, 2013
                                                                                          8. Travis WD: Advances in neuroendocrine lung tumors. Ann Oncol. 21 Suppl 7:vii65-71, 2010
                                                                                          9. Aubry MC et al: Significance of multiple carcinoid tumors and tumorlets in surgical lung specimens: analysis of 28 patients. Chest. 131(6):1635-43, 2007
                                                                                          10. Davies SJ et al: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease. Thorax. 62(3):248-52, 2007
                                                                                          11. Divisi D et al: Carcinoid tumors of the lung and multimodal therapy. Thorac Cardiovasc Surg. 53(3):168-72, 2005
                                                                                          12. Renshaw AA et al: Distinguishing carcinoid tumor from small cell carcinoma of the lung: correlating cytologic features and performance in the College of American Pathologists Non-Gynecologic Cytology Program. Arch Pathol Lab Med. 129(5):614-8, 2005
                                                                                          13. Jeung MY et al: Bronchial carcinoid tumors of the thorax: spectrum of radiologic findings. Radiographics. 22(2):351-65, 2002
                                                                                          14. Fraser RS et al: Fraser and Pare's Diagnosis of Diseases of the Chest, vol. II. 4th ed. Philadelphia: W.B. Saunders. 1229-43, 1999
                                                                                          15. Rosado de Christenson ML et al: Thoracic carcinoids: radiologic-pathologic correlation. Radiographics. 19(3):707-36, 1999