Epidemiology and therapy of thyroid cancer in childhood and adolescence

Abstract

Thyroid cancer is a rare disease in childhood and adolescence. However, it represents the most frequent cancer type in this age group. Thyroid cancer amounts about 0.5%-1.5% of all malignancies in children and adolescents. In Germany 10-30 cases could be expected in a year. The most common histologic type for this age group is the differentiated thyroid cancer (DTC), i.e. the papillary and follicular subtypes (90%). In 10% of patients medullary thyroid cancer (MTC) will be diagnosed. DTC occur more often in girls than in boys (female/male-ratio 2:1), with a median age of about 12-13 years (yrs). An important aetiological factor of DTC is a former exposition to different kinds of radiation. In childhood and adolescence MTC mostly appears in patients suffering from the syndrome MEN-2, whereas the sporadic form is rare. As in DTC, more girls than boys can be diagnosed for MTC (female/male-ratio 2-3:1), with a median age about 10 yrs. The anaplastic/undifferentiated subtype occurs extremely rare. For all types of thyroid cancer the most important therapeutic approach is the surgical intervention. In most cases of DTC an optimal disease control could be achieved by radioiodine therapy. Prognosis of DTC in children and adolescents is favourable. However, therapeutic strategies and modalities reported in the literature are very different. In contrast, prognosis of MTC is rather poor, that of the anaplastic type is infaust. No other effective therapy option than surgery is available. To evaluate the therapeutic efficacy of a combined modality therapy by both radiotherapy and cytostatic drugs in children and adolescents with poor prognosis types of thyroid cancer, an interdisciplinary multicenter therapy study will now be started in Germany.

Revisiting the genotype‐phenotype correlation in children with medullary thyroid carcinoma: A report from the GPOH‐MET registry

“…This is the first report on children and adolescents diagnosed with MTC and CCH reported to the German GPOH-MET registry. In line with previous reports on patients with MTC, 1,2,4,6,8,9,11,[19][20][21][22] children and adolescents presenting with localized disease at diagnosis of MTC had an excellent outcome, whereas children with metastatic disease did worse.…”

“…Medullary thyroid carcinoma (MTC) accounts for 3% to 5% of all thyroid cancers in children and adults with an incidence in children of 0.03 per 100.000 population per year . In most cases, MTC is hereditary .…”

“…Medullary thyroid carcinoma (MTC) accounts for 3% to 5% of all thyroid cancers in children and adults with an incidence in children of 0.03 per 100.000 population per year. 1,2 In most cases, Abbreviations: (M)MTC, (metastatic) medullary thyroid carcinoma; ATA, American Thyroid Association; CCH, C-cell hyperplasia; CI, confidence interval; CPS, cancer predisposition syndrome; CR, complete remission; EFS, event-free survival; H, high risk; HST, highest risk; MEN, multiple endocrine neoplasia; MET, malignant endocrine tumor; MOD, modest risk; OS, overall survival; PET, positron emission tomography; PHEO, pheochromocytoma; PHPT, primary hyperparathyroidism; RLN, recurrent laryngeal nerve; SD, standard deviation; TE, thyroidectomy. others a marfanoid habitus (referred to as MEN2B).…”

β1 integrin expression on endothelial cells is required for angiogenesis but not for vasculogenesis

“…Thyroid carcinoma is a rare malignancy of childhood yet among all cancers (relative incidence of 1.3%) it accounts for 0.7% and thus encompasses the largest carcinoma entity in children and adolescents 1, 2. In order to define appropriate treatment strategies, accurate staging and classification of the tumour is needed.…”

Pediatric Thyroid Disease: When is Surgery Necessary, and Who Should be Operating on Our Children?

“…MTC can occur sporadically or as part of a spectrum of familial MTC syndromes. In adults, sporadic MTC accounts for 65% to 75% of MTC (36), but in the pediatric population, sporadic carcinomas are exceedingly rare; the vast majority of MTC diagnosed in childhood is hereditary (37,38). Hereditary MTC can occur independently as familial MTC (FMTC).…”