Surgical Management of Diffuse Sclerosing Variant of Papillary Thyroid Carcinoma. Experience in 25 Patients.

purposeTo delineate the clinicopathologic features and biologic behavior of the diffuse sclerosing variant of papillary thyroid carcinoma (DSV-PTC) and to report its outcome.

methodsThe clinical records of 25 patients who had surgery for DSV-PTC from 2004 to 2017 were retrospectively analyzed. Comparisons were made to similar studies in the literature reporting ≥8 cases and a cohort of classical PTC.

resultsThere were 20 females and 5 males with an average age of 23 years (range 10-39 years). Bilateral disease occurred in 80% of cases. The mean size of the dominant mass was 4.2 ± 1.92 cm. In 92% of cases, therapeutic neck dissection was performed. Male sex significantly correlated with a higher yield of positive lymphadenopathy (p = 0.045). 62% of patients had recurrent disease. Recurrence significantly correlated with male sex, the number of metastatic lymph nodes (cutoff: 22 lymph nodes), and multifocality (p = 0.044, p ˂ 0.008, p ˂ 0.003, respectively). However, it did not correlate with the age at presentation. No disease-specific mortality occurred after an average follow-up of 77 months (range 12-168 months). The two comparisons made demonstrated a statistically significant greater tendency of the current series of DSV-PTC toward more aggressive clinicopathologic features and biologic behavior. No differences in overall survival were observed.

conclusionThe DSV-PTC should be considered a high-risk PTC that mandates an aggressive therapeutic strategy with the intent of optimizing disease-free survival.