Calcitonin secreting neuroendocrine neoplasms of the lung: a systematic review and narrative synthesis

in Endocrine Connections
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  • 1 D Llewellyn, Endocrinology ASO/EASO COM, King's College Hospital NHS Foundation Trust, London, United Kingdom of Great Britain and Northern Ireland
  • 2 R Srirajaskanthan, Institute of Liver Studies, King's College London, London, United Kingdom of Great Britain and Northern Ireland
  • 3 R Vincent, Department of Pathology, King's College Hospital NHS Foundation Trust, London, United Kingdom of Great Britain and Northern Ireland
  • 4 C Guy, Department of Cellular Pathology, Royal Sussex County Hospital, Brighton, United Kingdom of Great Britain and Northern Ireland
  • 5 E Drakou, Department of Clinical Oncology - Guy's Cancer Centre, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom of Great Britain and Northern Ireland
  • 6 S Aylwin, Endocrinology ASO/EASO COM, King's College Hospital NHS Foundation Trust, London, United Kingdom of Great Britain and Northern Ireland
  • 7 A Grossman, Oxford Centre for Diabetes Endocrinology and Metabolism, University of Oxford, Oxford, United Kingdom of Great Britain and Northern Ireland
  • 8 J Ramage, Institute of Liver Studies, King's College London, London, United Kingdom of Great Britain and Northern Ireland
  • 9 G Dimitriadis, Endocrinology ASO/EASO COM, King's College Hospital NHS Foundation Trust, London, SE5 9RS, United Kingdom of Great Britain and Northern Ireland

Correspondence: Georgios Dimitriadis, Email: g.dimitriadis@warwick.ac.uk

Calcitonin-secreting neuroendocrine neoplasms of the lung are rare, with few cases reported in the literature. Differentiating between medullary thyroid carcinoma and an ectopic source of calcitonin secretion can represent a complex diagnostic conundrum for managing physicians, with cases of unnecessary thyroidectomy reported in the literature. This manuscript reports a case of ectopic hypercalcitonaemia from a metastatic neuroendocrine neoplasm of the lung with concurrent thyroid pathology and summarises the results of a systematic review of the literature. Medical Literature Analysis and Retrieval System Online, Excerpta Medica, Cochrane Central Register of Controlled Trials, ClinicalTrials.gov and SCOPUS databases were systematically and critically appraised for all peer reviewed manuscripts that suitably fulfilled the inclusion criteria established a priori. The protocol for this systematic review was developed according to the Preferred Reporting Items for Systematic review and Meta-Analysis Protocols, and followed methods outlined in The Cochrane Handbook for Systematic Reviews of Interventions. This systematic review is registered with PROSPERO.

It is vital to consider diagnoses other than medullary thyroid carcinoma when presented with a patient with raised calcitonin, as it is not pathognomonic of medullary thyroid carcinoma. Lung neuroendocrine neoplasms can appear similar to medullary thyroid carcinoma histologically, they can secrete calcitonin and metastasize to the thyroid. Patients with medullary thyroid carcinoma may show stimulated calcitonin values over two or more times above the basal values, whereas calcitonin-secreting neuroendocrine neoplasms may or may not show response to stimulation tests. The present review summarises existing evidence from cases of ectopic hypercalcitonaemia to lung neuroendocrine neoplasms.

 

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