Aim: Diffuse sclerosing variant of papillary thyroid carcinoma (DSPC) is rare and occurs in young patients. This is a single institute retrospective analysis to report the clinical features and outcome of DSPC.
Method: DSPC constituted 8 (0.74%) of 1086 papillary thyroid carcinomas (PTC) referred to the department of Clinical Oncology, Queen Elizabeth Hospital Hong Kong from 1960 to 2000.
Results: The mean follow-up was 8 years (range: 1.4 to 15.2 years). Six were females and two were males, with age ranging from 11 to 48 years. All were ethnic Chinese. Compared with the whole cohort of PTC followed in the same period, these patients showed younger age at presentation (mean 27.4 vs 45.3 years), larger tumor size (mean 6.9 cm vs 2.4 cm), higher incidence of lymph node metastasis (100% vs 32.4%), and more frequent presence of serum anti-thyroglobulin autoantibody (75% vs 11.3%). The patients were managed as for differentiated thyroid carcinoma according to the institute's protocol, including total thyroidectomy followed by radioiodine (RAI) treatment. External radiotherapy was given to two patients as primary treatment and one patient after regional relapse. One patient had distant metastases at presentation and she was successfully treated by surgery followed by RAI, remaining in complete remission at 12.1 years. One patient had lymph node recurrence after primary total thyroidectomy and RAI treatment and was successfully salvaged by surgery and external radiotherapy. At last follow-up, all eight patients were alive with no evidence of disease.
Conclusions: Although DSPC is associated with some unfavourable features at presentation (such as large tumor size, extensive lymph node metastasis), the prognosis appears to be as good as classical PTC. After aggressive treatment by radical surgery, RAI ablation and/or external radiotherapy, the outcome and survival was excellent.