Abstract
ObjectivesLittle is known about the etiology of pulmonary carcinoids (PC). Associations with other types of cancer may identify shared risk factors but results from earlier studies were inconclusive. The aim of the present study was to explore the association between PC and other primary malignancies for identifying risk factors. MethodsA query of the nationwide Netherlands Cancer Registry generated data about patients diagnosed with PC from 1989 to 2018. The occurrence of second primary malignancies was evaluated separately for year 1 and years 2−30. The expected numbers of second primary malignancies were calculated using incidence reference tables, controlling for age, gender and period. Confidence intervals (95 % CI) for the ratio between observed and expected numbers (SIR: standardized incidence ratio) were calculated using Poisson distributions. ResultsIn a total of 2933 patients with PC, 425 consecutive primary malignancies were observed in 376 patients. Concomitant diagnoses in the first year mainly comprised lung (n = 59) and renal cancer (n = 14). Metachronous malignancies beyond the first year were most common for breast (n = 50), colorectal (n = 41), prostate (n = 32), and lung cancer (n = 29). Beyond year 1, the overall risk of second primary cancer in patients with PC was similar to the risk within the general population (n = 256, SIR = 1.12, 95 % CI 0.99–1.27). Increased risks were observed for soft tissue sarcoma (n = 5, SIR = 3.52, 95 % CI 1.14–8.22) and GEPNET (n = 4, SIR = 4.30, 95 % CI 1.17–11.01). ConclusionsConcomitant diagnosis of PC with other cancers is common, reflecting surveillance diagnostics. Apart from MEN-1 family history, no shared risk factors could be identified.
Highlights
Typical and atypical carcinoid tumours of the lung are rare neuro endocrine tumours (NETs), accounting for less than 1 % of all lung cancers
Very little is known about the Abbreviations: Pulmonary carcinoids (PC), pulmonary carcinoids; standardized incidence ratios (SIR), standardized incidence ratio; NET, neuroendocrine tumour; NCR, Netherlands Cancer Registry; GEPNET, gastroentero-pancreatic neuroendocrine tumour
As far as we know, this is the largest series published in literature, comprising 256 PC patients with metachronous malignancies
Summary
Typical and atypical carcinoid tumours of the lung are rare neuro endocrine tumours (NETs), accounting for less than 1 % of all lung cancers. According to data from the US SEER (Surveillance, Epidemi ology, and End Results) database, the incidence of pulmonary NETs gradually increased in the previous century and remained stationary in the last decennium at 1.6 per 100.000 persons [1]. This increase can be partially explained by increased recognition of this entity by patholo gists and improvement of diagnostics. The pathologic clas sification of neuroendocrine tumours in the lung is based on the WHO criteria [2] This system is based on morphologic features and immu nohistochemistry; mitotic frequency and necrosis are used to divide tumours into low- and intermediate grade (typical and atypical) carci noids or into the more aggressive large cell neuroendocrine cancer (LCNEC) and small cell lung cancer (SCLC). Very little is known about the Abbreviations: PC, pulmonary carcinoids; SIR, standardized incidence ratio; NET, neuroendocrine tumour; NCR, Netherlands Cancer Registry; GEPNET, gastroentero-pancreatic neuroendocrine tumour
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