Do patients with familial nonmedullary thyroid cancer present with more aggressive disease? Implications for initial surgical treatment

Surgery. 2019 Jan;165(1):50-57. doi: 10.1016/j.surg.2018.05.075. Epub 2018 Oct 14.

Abstract

Background: There are conflicting reports on whether familial nonmedullary thyroid cancer is more aggressive than sporadic nonmedullary thyroid cancer. Our aim was to determine if the clinical and pathologic characteristics of familial nonmedullary thyroid cancer are different than nonmedullary thyroid cancer.

Methods: We compared patients with familial nonmedullary thyroid cancer to a cohort of 53,571 nonmedullary thyroid cancer patients from the Surveillance, Epidemiology, and End Results database.

Results: A total of 78 patients with familial nonmedullary thyroid cancer from 31 kindreds presented at a younger age (P = .04) and had a greater rate of T1 disease (P = .019), lymph node metastasis (P = .002), and the classic variant of papillary thyroid cancer on histology (P < .001) compared with the Surveillance, Epidemiology, and End Results cohort. Patients with ≥3 affected family members presented at a younger age (P = .04), had a lesser female-to-male ratio (P = .04), and had a greater rate of lymph node metastasis (P = .009). Compared with the Surveillance, Epidemiology, and End Results cohort, we found a higher prevalence of lymph node metastasis in familial nonmedullary thyroid cancer index cases (P = .003) but not in those diagnosed by screening ultrasonography (P = .58).

Conclusion: Patients with familial nonmedullary thyroid cancer present at a younger age and have a greater rate of lymph node metastasis. The treatment for familial nonmedullary thyroid cancer should be more aggressive in patients who present clinically and in those who have ≥3 first-degree relatives affected.

Publication types

  • Comparative Study
  • Observational Study

MeSH terms

  • Adult
  • Age Distribution
  • Carcinoma, Medullary / congenital*
  • Carcinoma, Medullary / epidemiology
  • Carcinoma, Medullary / pathology
  • Carcinoma, Medullary / surgery
  • Cohort Studies
  • Female
  • Humans
  • Lymphatic Metastasis
  • Male
  • Middle Aged
  • Multiple Endocrine Neoplasia Type 2a / epidemiology
  • Multiple Endocrine Neoplasia Type 2a / pathology*
  • Multiple Endocrine Neoplasia Type 2a / surgery
  • Neck Dissection / statistics & numerical data
  • SEER Program
  • Sex Distribution
  • Thyroid Cancer, Papillary / epidemiology
  • Thyroid Cancer, Papillary / pathology*
  • Thyroid Cancer, Papillary / surgery
  • Thyroid Neoplasms / epidemiology
  • Thyroid Neoplasms / pathology*
  • Thyroid Neoplasms / surgery
  • Thyroidectomy / statistics & numerical data
  • United States / epidemiology

Supplementary concepts

  • Familial medullary thyroid carcinoma