A twenty-year experience with thyroid carcinoma in children

Desjardins, Jean G.; Bass, Juan; Leboeuf, G; Lorenzo, Maria Di; Letarte, Jacques; Khan, Abid H.; Simard, Pierre

doi:10.1016/s0022-3468(88)80407-x

Cited by 41 publications

(23 citation statements)

References 39 publications

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“…Young females appear to be more often affected than males, but this prevalence is com- parable to that observed in the adult population (F/ M = 3.8:1 vs 4.1:1, respectively). The differentiated forms of thyroid carcinoma tend to prevail in childhood and adolescence, from a minimum of 48% to a maximum of 89.8% for the papillary form and from 7.4% to 19.2% for the follicular histological subtype [4,7,21,28]. The undifferentiated form is rare despite the high frequency reported in some studies [32].…”

Section: Discussionmentioning

confidence: 99%

Thyroid carcinoma in children and adolescents

Danese

Gardini

Farsetti³

et al. 1997

European Journal of Pediatrics

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Section: Discussionmentioning

confidence: 99%

Thyroid carcinoma in children and adolescents

Danese

Gardini

Farsetti³

et al. 1997

European Journal of Pediatrics

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“…6 The malignant transformation of the C-cells begins very early in life: in MEN-2B patients, MTC can occur in infancy, whereas MTC is less aggressive in MEN 2A. [7][8][9][10] Nevertheless, in children of MEN-2A families thyroidectomized at 1 to 6 years of age, histologic analyses revealed all stages of C-cell disease, even metastatic MTC. [7][8][9][10][11][12] The only potentially curative treatment for medullary thyroid carcinoma is surgical removal of all thyroid tissue, [13][14][15] a goal that is not regularly achieved in patients with clinically manifest MTC.…”

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confidence: 99%

“…[7][8][9][10] Nevertheless, in children of MEN-2A families thyroidectomized at 1 to 6 years of age, histologic analyses revealed all stages of C-cell disease, even metastatic MTC. [7][8][9][10][11][12] The only potentially curative treatment for medullary thyroid carcinoma is surgical removal of all thyroid tissue, [13][14][15] a goal that is not regularly achieved in patients with clinically manifest MTC. 16 The molecular bases of MEN-2A and MEN-2B are missense mutations in the RET protooncogene, a transmembrane tyrosine kinase receptor located on chromosome 10q11.2.…”

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confidence: 99%

Review of Multiple Endocrine Neoplasia Type 2A in Children: Therapeutic Results of Early Thyroidectomy and Prognostic Value of Codon Analysis

et al. 2003

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ABSTRACT. Objectives. The aim of this study was first to investigate whether early total thyroidectomy (ETT; 1-5 years of age) can prevent medullary thyroid carcinoma with persistent or recurrent disease (PRD) in pediatric patients with multiple endocrine neoplasia type 2A (MEN-2A) and second, to evaluate the strength of codon analysis in children with MEN-2A as prognostic parameter.Methods. Case reports and review of the literature for pediatric patients with MEN-2A were conducted. Inclusion criteria were age (0 -20 years) and histologic degree of C-cell disease (normal ‫؍‬ N, C-cell hyperplasia ‫؍‬ CCH, medullary thyroid carcinoma ‫؍‬ MTC, metastatic MTC ‫؍‬ MMTC). To evaluate therapeutic results of ETT (1-5 years) versus late total thyroidectomy (LTT; 6 -20 years), age-dependent histologic stages of C-cell disease and postoperative occurrence of PRD were compared. Prognostic value of specific codons, age-dependent histologic distribution, and long-term outcome were analyzed.Results. In a total of 260 cases, 42 (16%) underwent ETT, and 218 (84%) underwent LTT. Histologic analysis showed significant difference between ETT versus LTT (57% vs 76%) regarding malignant stage of C-cell disease (of combined rate of MTC and MMTC). Long-term outcome was documented in 74 patients (28%). During a median follow-up period of 2 years (range: 0 -15 years), 21 of 65 of the LTT group versus 0 of 9 of the ETT group suffered PRD. Information about codon analysis was available in 150 patients (58%). Mutated codons were c634 (63%), c618 (19%), c620 (9%), and c804 (6%). Codonrelated histologic analysis resulted in prognostic differences: 81% of patients with c634-mutation had MCT or MMTC in contrast to c804 (44%), c618 (34%), and c620 (7%). Fifteen of 17 MMTC and 7 of 9 PRD occurred in patients with c634-mutation. 4 Usually, the first tumor occurring during life is medullary thyroid carcinoma (MTC), 5 a neoplasm of parafollicular C-cell origin. Malignant transformation of C-cells is multifocal in both thyroid lobes and characterized by different histologic stages: Diffuse C-cell hyperplasia (carcinoma in situ), uni-and multifocal MTC with or without local (lymph nodes), or distant metastasis (liver, bone). 6 The malignant transformation of the C-cells begins very early in life: in MEN-2B patients, MTC can occur in infancy, whereas MTC is less aggressive in MEN 2A. 7-10 Nevertheless, in children of MEN-2A families thyroidectomized at 1 to 6 years of age, histologic analyses revealed all stages of C-cell disease, even metastatic MTC. [7][8][9][10][11][12] The only potentially curative treatment for medullary thyroid carcinoma is surgical removal of all thyroid tissue, 13-15 a goal that is not regularly achieved in patients with clinically manifest MTC. 16 The molecular bases of MEN-2A and MEN-2B are missense mutations in the RET protooncogene, a transmembrane tyrosine kinase receptor located on chromosome 10q11.2. In MEN 2A, the most frequent germ line mutations involve the extracellular domain of rearranged during transfection (RET...

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“…The excellent prognosis creates a therapeutic dilemma. Some authors have suggested that total thyroidectomy reduces the incidence of relapse [2,[9][10][11], others appear to support a more conservative approach to the surgical management of well-differentiated thyroid carcinoma [12][13][14][15]. With more aggressive surgery, great care should be taken to avoid hypoparathyroidism or recurrent laryngeal nerve injury.…”

Section: Introductionmentioning

confidence: 99%