Patients with distant metastatic medullary thyroid carcinoma (DMMTC) often undergo primary tumor resection (PTR) with or without neck lymph node dissection (NLND) before systemic therapy. However, whether these patients benefit from treatment remains unclear.

Patients with DMMTC were identified from the Surveillance, Epidemiology, and End Results (SEER) database between 2010 and 2020. The overall survival (OS) and disease-specific survival (DSS) were established by Kaplan–Meier curves and were compared using the log-rank test or two-stage test between different treatment modalities (surgery, non-surgery) after propensity score matching (PSM). We also analyzed the effects of surgical and non-surgical treatments on the OS and DSS of patients stratified by TNM stages T1–2 and T3–4 in this cohort.

Among the 2336 patients with medullary thyroid carcinoma from the SEER database, 186 were diagnosed with DMMTC, with an average follow-up period of 28.12 months. Pairwise analysis after PSM revealed that the surgery group had a significantly improved survival rate compared to the non-surgery group (OS p = 0.00039, DSS p = 0.001). The survival advantages of the above-mentioned surgery group were maintained when stratified by stages T1–2 and T3–4.

Our results demonstrate that PTR with or without NLND, as an initial therapy, can benefit the survival rate of patients with DMMTC.