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Neuroendocrine Carcinoma
Cristina Fuss, MD; Sonia L. Betancourt-Cuellar, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Obsolete synonyms for small cell lung cancer (SCLC)
              • Oat cell carcinoma
              • Small cell anaplastic carcinoma
              • Undifferentiated small cell carcinoma
              • Intermediate cell-type small cell carcinoma
              • Mixed small cell/large cell carcinoma
          • Definitions

            • Neuroendocrine (NE) neoplasms
              • Storage, synthesis, and secretion of peptide hormones or bioamines
              • Histologic patterns similar to nonneoplastic NE cells
                • Trabecular or nesting growth pattern
                • Cells with coarsely stippled nuclear chromatin
              • Neurosecretory protein production detectable on immunohistochemical stains
                • Typically chromogranin A and synaptophysin
              • Subdivided into well- and poorly-differentiated NE neoplasms
            • Pulmonary NE neoplasms (25% of all NE neoplasms)
              • Malignant pulmonary neoplasms of NE origin
              • Thought to arise from normal Kulchitsky and NE cells of bronchial mucosa
              • WHO classification of Neuroendocrine Tumors (NET)
                • Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)
                  • Pre-invasive lesion for lung carcinoid tumor
                  • Characterized by proliferation of NE cells in bronchial wall
                • Well-differentiated NE neoplasms
                  • NET grade 1: Typical carcinoid (TC); low grade
                  • NET grade 2: Atypical carcinoid (AC); intermediate grade
                • Poorly-differentiated NE neoplasms
                  • NET grade 3: Small cell lung carcinoma (SCLC); high grade
                  • NET grade 4: Large cell neuroendocrine carcinoma (LCNEC); high grade
            • SCLC
              • Most common and most aggressive NE carcinoma
              • 13-15% of all lung cancers
              • Thought to arise centrally in mainstem or lobar bronchi

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Nuclear Medicine Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • Staging, Grading, & Classification

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      Selected References

                                      1. Crown A et al: Impact of initial imaging with gallium-68 dotatate PET/CT on diagnosis and management of patients with neuroendocrine tumors. J Surg Oncol. 121(3):480-5, 2020
                                      2. Mengoli MC et al: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) Syndrome and carcinoid tumors with/without NECH: a clinicopathologic, radiologic, and immunomolecular comparison study. Am J Surg Pathol. 42(5):646-55, 2018
                                      3. Baxi AJ et al: Multimodality imaging findings in carcinoid tumors: a head-to-toe spectrum. Radiographics. 37(2):516-36, 2017
                                      4. Deppen SA et al: Safety and efficacy of 68Ga-DOTATATE PET/CT for diagnosis, staging, and treatment management of neuroendocrine tumors. J Nucl Med. 57(5):708-14, 2016
                                      5. Klimstra DS et al: The spectrum of neuroendocrine tumors: histologic classification, unique features and areas of overlap. Am Soc Clin Oncol Educ Book. 35:92-103, 2015
                                      6. Carter BW et al: Small cell lung carcinoma: staging, imaging, and treatment considerations. Radiographics. 34(6):1707-21, 2014
                                      7. Benson RE et al: Spectrum of pulmonary neuroendocrine proliferations and neoplasms. Radiographics. 33(6):1631-49, 2013
                                      8. Lu HY et al: Targeted therapies in small cell lung cancer. Oncol Lett. 5(1):3-11, 2013
                                      9. Gaspar LE et al: Small-cell lung cancer: prognostic factors and changing treatment over 15 years. Clin Lung Cancer. 13(2):115-22, 2012
                                      10. Kalemkerian GP: Staging and imaging of small cell lung cancer. Cancer Imaging. 11:253-8, 2012
                                      11. Travis WD: Advances in neuroendocrine lung tumors. Ann Oncol. 21 Suppl 7:vii65-71, 2010
                                      12. Vallières E et al: The IASLC Lung Cancer Staging Project: proposals regarding the relevance of TNM in the pathologic staging of small cell lung cancer in the forthcoming (seventh) edition of the TNM classification for lung cancer. J Thorac Oncol. 4(9):1049-59, 2009
                                      13. Franks TJ et al: Lung tumors with neuroendocrine morphology: essential radiologic and pathologic features. Arch Pathol Lab Med. 132(7):1055-61, 2008
                                      14. Chong S et al: Neuroendocrine tumors of the lung: clinical, pathologic, and imaging findings. Radiographics. 26(1):41-57; discussion 57-8, 2006
                                      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

                                              TERMINOLOGY

                                              • Synonyms

                                                • Obsolete synonyms for small cell lung cancer (SCLC)
                                                  • Oat cell carcinoma
                                                  • Small cell anaplastic carcinoma
                                                  • Undifferentiated small cell carcinoma
                                                  • Intermediate cell-type small cell carcinoma
                                                  • Mixed small cell/large cell carcinoma
                                              • Definitions

                                                • Neuroendocrine (NE) neoplasms
                                                  • Storage, synthesis, and secretion of peptide hormones or bioamines
                                                  • Histologic patterns similar to nonneoplastic NE cells
                                                    • Trabecular or nesting growth pattern
                                                    • Cells with coarsely stippled nuclear chromatin
                                                  • Neurosecretory protein production detectable on immunohistochemical stains
                                                    • Typically chromogranin A and synaptophysin
                                                  • Subdivided into well- and poorly-differentiated NE neoplasms
                                                • Pulmonary NE neoplasms (25% of all NE neoplasms)
                                                  • Malignant pulmonary neoplasms of NE origin
                                                  • Thought to arise from normal Kulchitsky and NE cells of bronchial mucosa
                                                  • WHO classification of Neuroendocrine Tumors (NET)
                                                    • Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)
                                                      • Pre-invasive lesion for lung carcinoid tumor
                                                      • Characterized by proliferation of NE cells in bronchial wall
                                                    • Well-differentiated NE neoplasms
                                                      • NET grade 1: Typical carcinoid (TC); low grade
                                                      • NET grade 2: Atypical carcinoid (AC); intermediate grade
                                                    • Poorly-differentiated NE neoplasms
                                                      • NET grade 3: Small cell lung carcinoma (SCLC); high grade
                                                      • NET grade 4: Large cell neuroendocrine carcinoma (LCNEC); high grade
                                                • SCLC
                                                  • Most common and most aggressive NE carcinoma
                                                  • 13-15% of all lung cancers
                                                  • Thought to arise centrally in mainstem or lobar bronchi

                                              IMAGING

                                              • General Features

                                                • Radiographic Findings

                                                  • CT Findings

                                                    • MR Findings

                                                      • Nuclear Medicine Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • Staging, Grading, & Classification

                                                              • Gross Pathologic & Surgical Features

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          Selected References

                                                                          1. Crown A et al: Impact of initial imaging with gallium-68 dotatate PET/CT on diagnosis and management of patients with neuroendocrine tumors. J Surg Oncol. 121(3):480-5, 2020
                                                                          2. Mengoli MC et al: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) Syndrome and carcinoid tumors with/without NECH: a clinicopathologic, radiologic, and immunomolecular comparison study. Am J Surg Pathol. 42(5):646-55, 2018
                                                                          3. Baxi AJ et al: Multimodality imaging findings in carcinoid tumors: a head-to-toe spectrum. Radiographics. 37(2):516-36, 2017
                                                                          4. Deppen SA et al: Safety and efficacy of 68Ga-DOTATATE PET/CT for diagnosis, staging, and treatment management of neuroendocrine tumors. J Nucl Med. 57(5):708-14, 2016
                                                                          5. Klimstra DS et al: The spectrum of neuroendocrine tumors: histologic classification, unique features and areas of overlap. Am Soc Clin Oncol Educ Book. 35:92-103, 2015
                                                                          6. Carter BW et al: Small cell lung carcinoma: staging, imaging, and treatment considerations. Radiographics. 34(6):1707-21, 2014
                                                                          7. Benson RE et al: Spectrum of pulmonary neuroendocrine proliferations and neoplasms. Radiographics. 33(6):1631-49, 2013
                                                                          8. Lu HY et al: Targeted therapies in small cell lung cancer. Oncol Lett. 5(1):3-11, 2013
                                                                          9. Gaspar LE et al: Small-cell lung cancer: prognostic factors and changing treatment over 15 years. Clin Lung Cancer. 13(2):115-22, 2012
                                                                          10. Kalemkerian GP: Staging and imaging of small cell lung cancer. Cancer Imaging. 11:253-8, 2012
                                                                          11. Travis WD: Advances in neuroendocrine lung tumors. Ann Oncol. 21 Suppl 7:vii65-71, 2010
                                                                          12. Vallières E et al: The IASLC Lung Cancer Staging Project: proposals regarding the relevance of TNM in the pathologic staging of small cell lung cancer in the forthcoming (seventh) edition of the TNM classification for lung cancer. J Thorac Oncol. 4(9):1049-59, 2009
                                                                          13. Franks TJ et al: Lung tumors with neuroendocrine morphology: essential radiologic and pathologic features. Arch Pathol Lab Med. 132(7):1055-61, 2008
                                                                          14. Chong S et al: Neuroendocrine tumors of the lung: clinical, pathologic, and imaging findings. Radiographics. 26(1):41-57; discussion 57-8, 2006