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Diffuse sclerosing variant of PTC (DSV-PTC) is an uncommon subtype of thyroid cancer. Although an aggressive behavior is often recognized, prognostic significance is still under debate.    Objectives  To describe the clinicopathological features and outcomes of a series of DSV-PTC patients.    Methods  Retrospective data collection involving 33 patients diagnosed with DSV-PTC followed at the Endocrine Department of the Portuguese Institute of Oncology in Lisbon between 1981 and 2020.    Results  Twenty-six patients (78.8%) were females with a mean age at presentation of 29.4 ± 11.7 years old. Mean time of follow-up was 19.5 ± 10.6 years (range 0.5–39). Histologically, bilateral tumors were present in 72.7% patients ( n   = 24), thyroid capsular invasion was documented in 57.6% ( n   = 19), 45.4% ( n   = 15) had extrathyroidal extension, and 42.4% ( n   = 14) had lymphovascular invasion. Most patients were staged pT3 (42.4%,  n   = 14) and pN1 (81.8%,  n   = 27). Median lymph nodes resected were 16. None of the patients showed distant metastases at presentation. All patients were treated at least once with  131 I. During follow-up, four patients (14.8%), with persistent neck disease, were diagnosed with distant metastases, all of them in the lung. Two patients (1.8%) presented recurrent disease in the neck after being considered with no evidence of disease. At the last appointment, 18 patients (54.5%) were in remission, 4 (12.1%) had biochemical evidence of disease, 6 had structural disease, and for 5 patients disease status was considered as undetermined. There was no disease related mortality.    Discussion/conclusion  Our study confirms that DSV-PTC is diagnosed more often in young patients and exhibits a local extensive disease at presentation. On the other hand, even in the presence of distant metastases, no patient died during follow-up.

Diffuse sclerosing variant of papillary thyroid carcinoma: outcomes of 33 cases