Carcinoid tumors outside the abdomen
Abstract Neuroendocrine tumors (NETs) are epithelial malignancies that can arise from multiple tissues. Gastrointestinal (GI) NETs are the most common; in this review of extra‐abdominal carcinoid tumors, we focus our discussion on bronchial and thymic carcinoid tumors. Bronchial carcinoid tumors com...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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Wiley
2023-04-01
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| Series: | Cancer Medicine |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/cam4.5564 |
| _version_ | 1827958715275280384 |
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| author | Kenna Koehler Wade T. Iams |
| author_facet | Kenna Koehler Wade T. Iams |
| author_sort | Kenna Koehler |
| collection | DOAJ |
| description | Abstract Neuroendocrine tumors (NETs) are epithelial malignancies that can arise from multiple tissues. Gastrointestinal (GI) NETs are the most common; in this review of extra‐abdominal carcinoid tumors, we focus our discussion on bronchial and thymic carcinoid tumors. Bronchial carcinoid tumors comprise a quarter of all NETs and less than 2% of all lung cancers. Thymic carcinoid tumors are extremely rare, accounting for 5% of thymic tumors. Both bronchial and thymic carcinoid tumors are histologically classified as either typical or atypical based on their mitotic rate (less than 2 or 2–10 mitoses per 10 high‐powered fields (HPF), respectively). Both bronchial and thymic carcinoids can present with symptoms of obstruction and potentially carcinoid syndrome. The gold standard of management of bronchial and thymic carcinoid tumors is surgical resection. For patients with advanced disease, first‐line systemic therapy is generally somatostatin analog monotherapy with octreotide or lanreotide. In patients with refractory disease, therapy generally involves peptide receptor radioligand therapy, everolimus, or cytotoxic chemotherapy. There are ongoing, prospective trials comparing the mainstays of systemic therapy for these patients, as well as ongoing evaluations of immune checkpoint inhibitors and multi‐kinase inhibitors. Prognosis for both bronchial and thymic carcinoid tumors depends on histologic grade, local versus invasive disease, and extent of metastases. Herein we provide a summary of the pathophysiologic and clinical background, the current state of the field in diagnosis and management, and note of key ongoing prospective trials for patients with bronchial and thymic carcinoid tumors. |
| first_indexed | 2024-04-09T15:40:44Z |
| format | Article |
| id | doaj.art-475883782ba14d7fa98bf9902c8c22fa |
| institution | Directory Open Access Journal |
| issn | 2045-7634 |
| language | English |
| last_indexed | 2024-04-09T15:40:44Z |
| publishDate | 2023-04-01 |
| publisher | Wiley |
| record_format | Article |
| series | Cancer Medicine |
| spelling | doaj.art-475883782ba14d7fa98bf9902c8c22fa2023-04-27T10:12:43ZengWileyCancer Medicine2045-76342023-04-011277893790310.1002/cam4.5564Carcinoid tumors outside the abdomenKenna Koehler0Wade T. Iams1Department of Medicine, Division of Hematology‐Oncology Vanderbilt University Medical Center Nashville Tennessee USADepartment of Medicine, Division of Hematology‐Oncology Vanderbilt University Medical Center Nashville Tennessee USAAbstract Neuroendocrine tumors (NETs) are epithelial malignancies that can arise from multiple tissues. Gastrointestinal (GI) NETs are the most common; in this review of extra‐abdominal carcinoid tumors, we focus our discussion on bronchial and thymic carcinoid tumors. Bronchial carcinoid tumors comprise a quarter of all NETs and less than 2% of all lung cancers. Thymic carcinoid tumors are extremely rare, accounting for 5% of thymic tumors. Both bronchial and thymic carcinoid tumors are histologically classified as either typical or atypical based on their mitotic rate (less than 2 or 2–10 mitoses per 10 high‐powered fields (HPF), respectively). Both bronchial and thymic carcinoids can present with symptoms of obstruction and potentially carcinoid syndrome. The gold standard of management of bronchial and thymic carcinoid tumors is surgical resection. For patients with advanced disease, first‐line systemic therapy is generally somatostatin analog monotherapy with octreotide or lanreotide. In patients with refractory disease, therapy generally involves peptide receptor radioligand therapy, everolimus, or cytotoxic chemotherapy. There are ongoing, prospective trials comparing the mainstays of systemic therapy for these patients, as well as ongoing evaluations of immune checkpoint inhibitors and multi‐kinase inhibitors. Prognosis for both bronchial and thymic carcinoid tumors depends on histologic grade, local versus invasive disease, and extent of metastases. Herein we provide a summary of the pathophysiologic and clinical background, the current state of the field in diagnosis and management, and note of key ongoing prospective trials for patients with bronchial and thymic carcinoid tumors.https://doi.org/10.1002/cam4.5564atypical carcinoidbronchial carcinoidthymic carcinoidtypical carcinoid |
| spellingShingle | Kenna Koehler Wade T. Iams Carcinoid tumors outside the abdomen Cancer Medicine atypical carcinoid bronchial carcinoid thymic carcinoid typical carcinoid |
| title | Carcinoid tumors outside the abdomen |
| title_full | Carcinoid tumors outside the abdomen |
| title_fullStr | Carcinoid tumors outside the abdomen |
| title_full_unstemmed | Carcinoid tumors outside the abdomen |
| title_short | Carcinoid tumors outside the abdomen |
| title_sort | carcinoid tumors outside the abdomen |
| topic | atypical carcinoid bronchial carcinoid thymic carcinoid typical carcinoid |
| url | https://doi.org/10.1002/cam4.5564 |
| work_keys_str_mv | AT kennakoehler carcinoidtumorsoutsidetheabdomen AT wadetiams carcinoidtumorsoutsidetheabdomen |