# Medullary thyroid cancer

Source: https://onco.cc/cancers/medullary-thyroid-cancer/  
OnCo record `medullary-thyroid-cancer` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Medullary thyroid cancer comes from the calcitonin-making C cells, not the thyroid hormone cells, so radioactive iodine does not work. Surgery is the only cure, a quarter of cases run in families through the RET gene, and the RET-selective drug selpercatinib has transformed treatment of advanced disease.

## Summary

Medullary thyroid cancer arises from the parafollicular C cells and secretes calcitonin, which serves as its tumour marker. Germline RET mutations cause the hereditary forms (MEN2A, MEN2B and familial medullary thyroid cancer), and children who inherit them have the thyroid removed before cancer develops, timed by the mutation's risk level; most sporadic tumours carry a somatic RET M918T mutation, and a minority RAS mutations. Total thyroidectomy with central neck dissection is the only curative treatment, and calcitonin doubling time guides follow-up. For progressive advanced disease, vandetanib (2011) and cabozantinib (2012) were the first approved kinase inhibitors; selpercatinib, a RET-selective inhibitor, beat them in the LIBRETTO-531 trial and is now the standard for RET-mutant disease, with pralsetinib as an alternative. Phaeochromocytoma must be excluded before any thyroid surgery in MEN2.

## Fields

- Kind: Cancer
- Last checked: 2026-09-17
- Also known as: Medullary thyroid carcinoma; MTC
- Tags: subtype-page
- Group: endocrine
- Burden: Two to four percent of thyroid cancers; a quarter are inherited through a RET mutation in the multiple endocrine neoplasia type 2 syndromes; ten-year survival ranges from above 95 percent for disease confined to the thyroid to under half once distant spread has occurred.
- Subtypes: Sporadic (about 75 percent); Hereditary: MEN2A (with phaeochromocytoma and hyperparathyroidism); Hereditary: MEN2B (mucosal neuromas, marfanoid habitus, early aggressive disease); Familial medullary thyroid cancer only
- Biomarkers: Calcitonin and CEA (diagnosis, follow-up and doubling time); Germline RET testing in every patient; Somatic RET M918T; RAS mutations in RET-negative tumours

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/medullary-thyroid-cancer/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/medullary-thyroid-cancer/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/medullary-thyroid-cancer/#what-it-is [4 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/medullary-thyroid-cancer/#finding-it [4 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/medullary-thyroid-cancer/#treating-it [4 settings, 2 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/medullary-thyroid-cancer/#evidence [4 trials, 4 key papers, 6 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/medullary-thyroid-cancer/#science [4 targets]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/medullary-thyroid-cancer/where-you-are/ [1 centre]
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/medullary-thyroid-cancer/#living-with-it [15 questions, 6 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/medullary-thyroid-cancer/coming/ [4 medicines, 4 trials, 3 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/medullary-thyroid-cancer/data/ [32 connected records]

## Standard of care

- Hereditary RET carriers: Prophylactic total thyroidectomy in childhood, timed by the RET codon risk category (before age one in MEN2B). ([RET](https://onco.cc/targets/ret/), [Multiple endocrine neoplasia syndromes (MEN1, MEN2, MEN4)](https://onco.cc/cancers/multiple-endocrine-neoplasia/))
- Localised disease: Total thyroidectomy with central compartment dissection, lateral dissection when nodes are involved; no radioactive iodine; levothyroxine replacement only. ([Thyroid cancer](https://onco.cc/cancers/thyroid/))
- Advanced RET-mutant disease: Selpercatinib first line (LIBRETTO-531 beat cabozantinib and vandetanib); pralsetinib as an alternative. ([Selpercatinib](https://onco.cc/drugs/selpercatinib/), [Pralsetinib](https://onco.cc/drugs/pralsetinib/), [RET](https://onco.cc/targets/ret/))
- Advanced RET-negative disease: Cabozantinib or vandetanib; external radiotherapy for local control; somatostatin analogues for diarrhoea from calcitonin. ([Cabozantinib](https://onco.cc/drugs/cabozantinib/), [Vandetanib](https://onco.cc/drugs/vandetanib/))

## State of the art

- Selpercatinib gave a response rate near 70 percent and much longer progression-free survival than the older multikinase drugs, with fewer side effects.
- Genetic testing and prophylactic surgery have made hereditary medullary cancer a preventable disease in tested families.
- Calcitonin doubling time is one of the most reliable prognostic tools in any cancer.

## Open problems

- No cure once the disease has spread beyond the neck.
- Resistance mutations to RET inhibitors (G810) are emerging.
- RET-negative sporadic disease has no targeted option.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Medullary_thyroid_cancer
- Wikipedia: https://en.wikipedia.org/wiki/Medullary_thyroid_cancer

## Connected records

- cancers: [Anaplastic thyroid cancer](https://onco.cc/cancers/anaplastic-thyroid-cancer/), [Follicular thyroid cancer](https://onco.cc/cancers/follicular-thyroid-cancer/), [Hereditary pheochromocytoma and paraganglioma (SDHx, VHL, RET, NF1, MAX and TMEM127)](https://onco.cc/cancers/hereditary-ppgl/), [Multiple endocrine neoplasia syndromes (MEN1, MEN2, MEN4)](https://onco.cc/cancers/multiple-endocrine-neoplasia/), [Multiple endocrine neoplasia type 2 (MEN2A and MEN2B)](https://onco.cc/cancers/men2-syndrome/), [Papillary thyroid cancer](https://onco.cc/cancers/papillary-thyroid-cancer/), [Thyroid cancer](https://onco.cc/cancers/thyroid/)
- trials: [A Study of Selpercatinib (LOXO-292) in Participants With Advanced Solid Tumors, RET Fusion-Positive Solid Tumors, and Medullary Thyroid Cancer (LIBRET](https://onco.cc/trials/nct03157128/), [A Study of Selpercatinib (LY3527723) in Participants With Advanced Solid Tumors Including RET Fusion-positive Solid Tumors, Medullary Thyroid Cancer a](https://onco.cc/trials/nct04280081/), [EXAM](https://onco.cc/trials/exam/), [ZETA](https://onco.cc/trials/zeta/)
- key papers: [EXAM: cabozantinib in progressive medullary thyroid cancer](https://onco.cc/key-papers/paper-exam-cabozantinib-mtc-elisei-jco-2013/), [LIBRETTO-531: selpercatinib versus cabozantinib or vandetanib in advanced RET-mutant medullary thyroid cancer](https://onco.cc/key-papers/paper-libretto-531-nejm-2023/), [Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma](https://onco.cc/key-papers/paper-ata-medullary-thyroid-guideline-wells-thyroid-2015/), [ZETA: vandetanib in locally advanced or metastatic medullary thyroid cancer](https://onco.cc/key-papers/paper-zeta-vandetanib-mtc-wells-jco-2012/)
- targets: [RET](https://onco.cc/targets/ret/)
- drugs: [Cabozantinib](https://onco.cc/drugs/cabozantinib/), [Pralsetinib](https://onco.cc/drugs/pralsetinib/), [Selpercatinib](https://onco.cc/drugs/selpercatinib/), [Vandetanib](https://onco.cc/drugs/vandetanib/)
- people: [Julien Hadoux](https://onco.cc/people/julien-hadoux/), [Rossella Elisei](https://onco.cc/people/rossella-elisei/), [Samuel A. Wells Jr.](https://onco.cc/people/samuel-wells/), [Vivek Subbiah](https://onco.cc/people/vivek-subbiah/)
- institutions: [University of Pisa / Azienda Ospedaliero-Universitaria Pisana](https://onco.cc/institutions/university-of-pisa/)
- biomarkers: [RET fusion and RET mutation](https://onco.cc/biomarkers/ret-fusion/)
- technologies: [Serum tumour markers: proper use and misuse](https://onco.cc/technologies/serum-tumour-markers/), [Thyroglobulin, calcitonin and CEA in thyroid cancer follow-up](https://onco.cc/technologies/thyroid-cancer-markers/)

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JSON: https://onco.cc/api/v1/entities/medullary-thyroid-cancer.json