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Calcitonin-negative medullary thyroid carcinoma: the 'triple-negative' phenotype

Lookup NU author(s): Dr Sarah Johnson


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Calcitonin-negative medullary thyroid carcinoma is a rare, poorly understood primary neuroendocrine carcinoma of the thyroid characterised by classic medullary thyroid carcinoma morphology without raised serum calcitonin. A 24-year-old woman presented with a slow-growing, right-sided neck swelling. She underwent an ultrasound scan, cytopathological and histopathological examination, and tests for alternative diagnoses. The ultrasound showed a heterogeneous, hyperechoic nodule in the right thyroid lobe. Serum calcitonin was normal. Cytopathology and histopathology showed typical medullary thyroid carcinoma morphology but without calcitonin upon immunostaining and mRNA in situ hybridisation. A 'triple-negative' calcitonin-negative medullary thyroid carcinoma was diagnosed. A completion thyroidectomy with bilateral central lymph node dissection was performed. The patient remains well three-years post-surgery. When cytopathology suggests a medullary thyroid carcinoma, serum calcitonin, pro-calcitonin, carcinoembryonic antigen and calcitonin-gene-related peptide should be measured to identify cases of calcitonin-negative medullary thyroid carcinoma. They should also be measured post-treatment for monitoring purposes. This will aid future calcitonin-negative medullary thyroid carcinoma diagnoses and will inform prognostic stratification and influence treatment decisions.

Publication metadata

Author(s): Murphy DC, Johnson SJ, Aspinall S

Publication type: Article

Publication status: Published

Journal: Annals of the Royal College of Surgeons of England

Year: 2020

Volume: 102

Issue: 3

Pages: e63-e66

Print publication date: 01/03/2020

Online publication date: 16/12/2019

Acceptance date: 02/04/2016

ISSN (print): 0035-8843

ISSN (electronic): 1478-7083

Publisher: Royal College of Surgeons of England


DOI: 10.1308/rcsann.2019.0153

PubMed id: 31841033


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