Development of a pediatric differentiated thyroid carcinoma registry within the EuRRECa project: rationale and protocol.

Background: Although differentiated thyroid carcinoma (DTC) is the most frequent endocrine pediatric cancer, it is rare in childhood and adolescence. While tumor persistence and recurrence are not uncommon, mortality remains extremely low. Complications of treatment are however reported in up to 48% of the survivors. Due to the rarity of the disease, current treatment guidelines are predominantly based on the results of small observational retrospective studies and extrapolations from results in adult patients. In order to develop more personalized treatment and follow-up strategies (aiming to reduce complication rates), there is an unmet need for uniform international prospective data collection and clinical trials. Methods and analysis: The European pediatric thyroid carcinoma registry aims to collect clinical data for all patients ≤18 years of age with a confirmed diagnosis of DTC who have been diagnosed, assessed, or treated at a participating site. This registry will be a component of the wider European Registries for Rare Endocrine Conditions project which has close links to Endo-ERN, the European Reference Network for Rare Endocrine Conditions. A multidisciplinary expert working group was formed to develop a minimal dataset comprising information regarding demographic data, diagnosis, treatment, and outcome. We constructed an umbrella-type registry, with a detailed basic dataset. In the future, this may provide the opportunity for research teams to integrate clinical research questions. Ethics and dissemination: Written informed consent will be obtained from all participants and/or their parents/guardians. Summaries and descriptive analyses of the registry will be disseminated via conference presentations and peer-reviewed publications.

[Evaluation of surgical treatment results in patients with differentiated thyroid cancer from materials in the Oncologic Institute in Gliwice]. / Ocena wyników leczenia operacyjnego chorych na zróznicowane raki tarczycy w materiale Instytutu Onkologii w Gliwicach.

The optimal surgical treatment for patients with differentiated thyroid carcinoma is still controversial and discussed. The aim of this study was to analyze the effectiveness of treatment in differentiated thyroid carcinoma, to establish the prognostic value of histopathological type, clinical advancement, the extent of surgery and to estimate the therapeutic benefit rate of the radical approach. The randomized group of 768 patients with differentiated thyroid carcinoma, aged between 16 to 40 (median age 44), treated in Oncology Center Institute in Gliwice between 1986 and 1997 were analyzed. They were operated in different surgical clinics in Poland. The primary total thyroidectomy was performed in 153 of patients (20%), the secondary radical operation in 474 (62%). 136 persons were operated by less radical approach. Cervical lymph node metastases were observed in 26% of patients. Distant metastases occurred in 8% of cases. 610 persons were treated with 131I. All patients were administered hormonal therapy to suppress TSH. Locoregional recurrence occurred in 7.5% of patients. Complete remission was observed in 71.5% of persons (548 patients). The influence of distant metastases lymph node metastases and tumor size on the long-term prognosis was analyzed in univariate statistic analysis. There were no differences in survival in patients with papillary and follicular carcinoma. The 10-year survival was statistically significantly longer in patients who underwent radical surgical treatment (92%) than after non-total thyroidectomy (80%). Despite the relatively high risk of surgical complications the benefits of the radical approach were significant.

[Early evaluation of results after total thyroidectomy in patients with thyroid cancer]. / Wczesna ocena wyników pierwotnego calkowitego wyciecia gruczolu tarczowego u chorych na raka tarczycy.

UNLABELLED: 219 patients (159 females, 60 males) operated in Clinic of Oncological Surgery in Gliwice between 1995 and 1999 were analyzed in this study. In 89% of cases differentiated thyroid carcinoma (DTC) was diagnosed. In other patients medullary cancer (8%) and anaplastic one (3%) were diagnosed. Total thyroidectomy with removal of the central cervical lymph nodes compartment was performed in all cases. In 86% of operations bilateral nodal biopsy was done. In 27% of cases modified cervical lymph node dissection was performed. 44 patients were in T4 stage. In 51% of cases cervical nodal metastases were observed. The operation was estimated as radical in 195 patients. Distant metastases occurred in 15 patients. Bilateral paresis of recurrent laryngeal nerve was not observed, unilateral was noticed in 8.7% of cases, permanent hypoparathyroidism appeared in 15% of patients. In postoperative scintigraphy radioiodine uptake was in the range of 0-10% in 206 cases and in the range of 0-3% in 2/3 of operated patients. 125 patients with DTC were treated with 131I. All patients with DTC were administered L-thyroxine to suppress TSH. In other types of cancer substitutive hormone therapy was introduced. In the entire group 8 deaths occurred and 13 cases of local recurrences were observed during the observation. CONCLUSION: The results prove the high effectiveness of the radical thyroidectomy performed in thyroid cancer.

[Evaluation of results after repeated radical surgery for differentiated thyroid cancer]. / Ocena wyników wtórnego radykalnego leczenia chirurgicznego chorych na zróznicowane raki tarczycy.

Total thyroidectomy is the treatment of choice in thyroid cancer. Unfortunately, the diagnosis is not always established before the first operation and the secondary radical surgery is necessary. The aim of this study was to analyze the effectiveness of secondary surgery in differentiated thyroid carcinoma. The randomized group of 768 patients with differentiated thyroid carcinoma, treated in Oncology Center Institute in Gilwice between 1986 and 1997 was analyzed. Among them 474 persons (56%) - 398 females and 76 males (median age 44 years) were operated more than once in different clinics in Poland. They were compared with 136 persons operated by less radical approach. Negative histobiological risk factors were noted in 4% to 33% of cases. Cervical lymph node metastases were observed in 18% of patients. Distant metastases occurred in 3% of cases. 373 persons were treated with 131(I). All patients administered hormonal therapy to suppress TSH. After secondary operation the rate of cancer foci was 28% (when radicalization was performed durning one year after primary surgery) and 56% when it was done later. This difference was statistically significant (p=0,0002). Locoregional recurrence was observed in 5% of patients. In the entire examined group the actual 5-year-survival was 98% and 10-year-survival was 92%. Complete remission was observed in 80% of patients. The incidence of multifocality in 1/3 of cases and the presence of neoplasm in the re-operated thyroid gland in 1/4 of cases have substantiated the decision for secondary surgery. The delay in radicalization surgery of more than 12 months has doubled the risk of multifocality in thyroid gland remnants. Our results have confirmed the necessity of the secondary surgery after nonradical primary thyroid resection in differentiated thyroid carcinoma.

[Clinical picture of differentiated thyroid carcinoma in the T1 stage]. / Obraz kliniczny zróznicowanego raka tarczycy w stopniu zaawansowania T1.

UNLABELLED: Patients with thyroid microcarcinoma have usually excellent prognosis. However, recurrences in neck and distant metastases have been reported. The optimal treatment for this group of patients is still discussed. The aim of study was to present the clinical and histopathological data of our group of patients with differentiated thyroid carcinoma of 1 cm or less in size diagnosed from 1990 to 2000. 146 patients (137 females and 9 males, mean age 47 years) with differentiated thyroid carcinoma up to 1 cm in the greatest dimension were analyzed. The median follow-up time was 3.5 years. The diagnosis was incidental (made after primary surgery) in 82% of cases. In 85% of patients papillary thyroid carcinoma was diagnosed (in 6% of them as follicular variant). Histopathological risk factors were noticed in 32 patients (21%). The patients were operated in different surgical clinics in Poland. 5% (8) of primary operations and 51% (33) of secondary ones were performed in our center. The most frequent primary operation was bilateral, subtotal strumectomy (62%). Total thyroidectomy was made in 14% of cases (21). Secondary radical operation was performed in 65 patients (44%). Residual cancer was found in 9% of reoperated patients. 47% of patients (69) were treated with 131I and all of them were administered hormonal therapy to suppress TSH. No recurrence and distant metastases were observed. Lymph node metastases were diagnosed and treated in 10 patients (7%). During observation period 1 patient died because of breast cancer. No statistically significant influence of clinical and histopathological risk factors or operation type on clinical outcome was observed. In the analyzed group secondary surgery was connected with the higher risk of complications. CONCLUSIONS: In thyroid papillary microcarcinoma total thyroidectomy is recommended for patients with multiple foci of neoplasm or with metastases (lymph node or distant). Establishment of optimal treatment in minimal follicular thyroid cancer is not possible on the basis of our data and published literature.