Clinicopathological and Genetic Characteristics of Patients of Different Ages with Diffuse Sclerosing Variant Papillary Thyroid Carcinoma.

Diffuse sclerosing variant papillary thyroid carcinoma (DSVPTC) is commonly observed in young patients, with a median age at diagnosis in the third decade of life. Further, the risk of recurrence is higher for DSVPTC than for classical PTC. Therefore, this study aimed to describe the clinicopathological and genetic characteristics of patients of different ages with DSVPTC. We retrospectively reviewed 397 patients who underwent thyroidectomy for DSVPTC at Gangnam Severance Hospital, Yonsei University, from January 2005 to December 2017. The mean age at diagnosis was 36.7 ± 11.6 years, with most patients (163, 41.1%) aged 31-40 years. DSVPTC was predominant in women (276, 69.5%). We observed recurrence in 46 (11.6%) patients, with regional nodal recurrence being the most common type of recurrence (32 patients, 69.6%). The mean tumour size was larger in younger patients than in older patients. DSVPTC was more aggressive in paediatric patients with a larger-sized tumour, more common multiplicity, and lateral neck metastasis. Through random sampling, we selected 41 patients by age group and examined the mutations in 119 genes using next-generation sequencing. BRAF, KRAS, and TERT displayed relatively higher mutation rates than other genes. DSVPTC displays different clinical, pathological, and molecular profiles than classical PTC. The BRAF, KRAS, and TERT mutations are the most important, with age-specific differences.

Diffuse sclerosing variant papillary thyroid carcinoma has worse survival than classic papillary thyroid carcinoma: a meta-analysis.

Diffuse sclerosing variant (DSV) of papillary thyroid carcinomais a rare form of thyroid cancer that demonstrates more aggressive histopathology than classical papillary thyroid carcinoma (c-PTC); however, if this leads to worse survival is debated. Many DSVs are driven by fusion events which are of recent clinical importance due to the advent of targeted RET inhibitors. A systematic search and meta-analysis of the literature was performed to compare outcomes of disease-specific mortality (DSM), metastatic and recurrent disease and the incidence of fusion events between DSV and c-PTC to July 2022. The Newcastle-Ottawa Quality Assessment studies was used to assess quality. An odds ratio (OR) was utilised to measure outcomes with 95% CIs. The Preferred Reporting Items for Systematic Reviews and Meta-analysis guideline was followed. Seventeen studies were included with 874 DSV patients compared to 76,013 c-PTC patients. DSV patients had worse DSM (OR=2.50, 95% CI 1.39-4.51) and presented with a higher rate of metastatic lymph nodes (OR = 5.85, 95% CI 2.73-12.53) and more distant metastases (OR = 3.83, 95% CI 2.17-6.77). DSV patients had higher odds of recurrent disease (OR = 3.23, 95% CI 2.00-5.23) and overall distant metastasis (OR = 2.70, 95% CI 1.74-4.17). Rates of RET fusion alterations for DSV ranged from 25 to 83%. DSV has a worse prognosis than c-PTC with higher rates of recurrent disease and distant metastasis. The high prevalence of RET fusions offers the potential to improve outcomes for patients with DSV.

Axillary lymph node metastasis from papillary thyroid carcinoma with elevated CA 19-9 and CA 242 levels: a case report and literature review.

Axillary lymph node metastasis is a rare event in thyroid carcinoma. The simultaneous expression of carbohydrate antigens 19-9 (CA 19-9) and 242 (CA 242) in thyroid tumors is also extremely rare. Herein, we report a case of axillary lymph node metastasis with elevated serum CA 19-9 and CA 242 in papillary thyroid carcinoma. In a 47-year-old woman with thyroid carcinoma, masses developed in the neck and axilla over a two-month period, which were surgically treated using total thyroidectomy, with neck and axillary lymph node dissection. Histopathological examination confirmed a diffuse sclerosing variant-papillary thyroid carcinoma, with 52 of 63 axillary lymph node metastases. Notably, serum CA 19-9 and CA 242 levels decreased from the initial values of 1,110 and 50 kU/L, respectively, to normal levels one month postoperatively and have remained stable for two years since. The aggressive biological behavior of diffuse sclerosing variant-papillary thyroid carcinoma and the abnormal anatomical distortion caused by tumors in this case most likely reflect the mechanisms underlying retrograde dissemination in lymphatic tubes. However, the mechanism leading to a simultaneous elevation of CA 19-9 and CA 242 secreted by the diffuse sclerosing variant-papillary thyroid carcinoma has not been elucidated. The patient has survived for two years suggesting that timely surgery can help such patients achieve a better prognosis.

Research progress of diffuse sclerosing variant papillary thyroid carcinoma / 国际外科学杂志

Diffuse sclerosing variant papillary thyroid carcinoma,one of the variants of papillary thyroid carcinoma,nearly account for 0.7％ to 6.6％ of papillary thyroid carcinoma.It is an aggressive tumor that shows higher rates of recurrence,metastasis and persistent disease compares to classic papillary thyroid carcinoma,thus diffuse sclerosing variant papillary thyroid carcinoma needs stricter and more exhaustive operation.Since the incidence of diffuse sclerosing variant papillary thyroid carcinoma is low,many physicians lack of understanding of the disease,it's often missed diagnosis or misdiagnosed.Consequently.In order to let physicians understand more about diffuse sclerosing variant papillary thyroid carcinoma,this paper will elaborate the epidemiology,clinic and pathology features of it.