Genes, Vol. 10, Pages 723: Thyroid Cancer in the Pediatric Population

Genes, Vol. 10, Pages 723: Thyroid Cancer in the Pediatric Population Genes doi: 10.3390/genes10090723 Authors: Vera A. Paulson Erin R. Rudzinski Douglas S. Hawkins Thyroid cancer is rare in the pediatric population, but thyroid carcinomas occurring in children carry a unique set of clinical, pathologic, and molecular characteristics. In comparison to adults, children more often present with aggressive, advanced stage disease. This is at least in part due to the underlying biologic and molecular differences between pediatric and adult thyroid cancer. Specifically, papillary thyroid carcinoma (which accounts for approximately 90% of pediatric thyroid cancer) has a high rate of gene fusions which influence the histologic subtypes encountered in pediatric thyroid tumors, are associated with more extensive extrathyroidal disease, and offer unique options for targeted medical therapies. Differences are also seen in pediatric follicular thyroid cancer, although there are few studies of non-papillary pediatric thyroid tumors published in the literature due to their rarity, and in medullary carcinoma, which is most frequently diagnosed in the pediatric population in the setting of prophylactic thyroidectomies for known multiple endocrine neoplasia syndromes. The overall shift in the spectrum of histotypes and underlying molecular alterations common in pediatric thyroid cancer is important to recognize as it may directly influence diagnostic test selection and therapeutic ...
Source: Genes - Category: Genetics & Stem Cells Authors: Tags: Review Source Type: research

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wkins Thyroid cancer is rare in the pediatric population, but thyroid carcinomas occurring in children carry a unique set of clinical, pathologic, and molecular characteristics. In comparison to adults, children more often present with aggressive, advanced stage disease. This is at least in part due to the underlying biologic and molecular differences between pediatric and adult thyroid cancer. Specifically, papillary thyroid carcinoma (which accounts for approximately 90% of pediatric thyroid cancer) has a high rate of gene fusions which influence the histologic subtypes encountered in pediatric thyroid tumors, are as...
Source: Genes - Category: Genetics & Stem Cells Authors: Tags: Review Source Type: research
Yan Ding, Jun Feng, Xun-hua Xu, Jun Yao, Rong-Biao YingIndian Journal of Cancer 2019 56(2):173-175 A 41 year old man presented with a familial history of multiple endocrine neoplasia type 2A (MEN2A) and severe hypertension. Rearranged during transfection (RET) gene sequencing confirmed a Cys634Tyr mutation of TGC to TAC. Total thyroidectomy and bilateral neck dissection were performed and the pathological assessment revealed a medullary thyroid carcinoma (MTC), 0.6 cm in size on the right side (number of lymph nodes: 0/2, 0/15, 0/12, and 0/8 in areas VI, II, III, and IV, respectively) and a papillary thyroid carcinoma (PT...
Source: Indian Journal of Cancer - Category: Cancer & Oncology Authors: Source Type: research
ConclusionsTo the best of our knowledge, it is the first case reported in the literature of a patient who has been affected by recurrent non-functional pituitary adenoma and medullary thyroid carcinoma, concomitantly. Although this association can be accidental, it emphasizes the fact that patients with a history of a neoplasm should be monitored regularly in order to diagnose and treat possible second primary cancers in a timely manner. Of note, this consideration is of great importance in patients whose first neoplasms have better prognosis and survival rates, which provide them more time to develop second primary cancer...
Source: Journal of Medical Case Reports - Category: General Medicine Source Type: research
Authors: Younis E Abstract Thyroid neoplasms encompass a variety of lesions that range from benign adenomas to malignancies. These latter can be well-differentiated, poorly differentiated or undifferentiated (anaplastic) carcinomas. More than 95% of thyroid cancers are derived from thyroid follicular cells, while 2-3% (medullary thyroid cancers, MTC) originate from calcitonin producing C-cells. Over the last decade, investigators have developed a clearer understanding of genetic alterations underlying thyroid carcinogenesis. A number of point mutations and translocations are involved, not only in its tumorigenesis,...
Source: Asian Pacific Journal of Cancer Prevention - Category: Cancer & Oncology Tags: Asian Pac J Cancer Prev Source Type: research
Conclusion: The genetic evaluation is strongly recommended for patients with a positive family history, early onset of age, or multiple sites of masses. If the results verified the mutations of RET gene, thyroidectomy should be undertaken as the guide for better prognosis.
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Trial/Experimental Study Source Type: research
The objectives of this study are to describe the rare M918V RET mutation discovered in 8 MTC kindreds from Brazil lacking the MEN 2B phenotype classically observed in M918T patients and to investigate the presence of a founder effect for this germline mutation. Eight apparently sporadic MTC cases were diagnosed with the germline M918V RET mutation. Subsequently, their relatives underwent clinical and genetic assessment (n = 113), and M918V was found in 42 of them. Until today, 20/50 M918V carriers underwent thyroidectomy and all presented MTC/C-cell hyperplasia; the remainder carriers are on clinical follow-up. None of the...
Source: Endocrine-Related Cancer - Category: Endocrinology Authors: Tags: Research Source Type: research
Conclusion Genetic counseling plays a key role in the management of such high-risk families and hence helps in avoiding or reducing disease recurrence in their future generations.
Source: Egyptian Journal of Medical Human Genetics - Category: Genetics & Stem Cells Source Type: research
We report the 5-year treatment of an 11-year-old patient, with vandetanib and without thyroid surgery. PMID: 27345554 [PubMed - as supplied by publisher]
Source: Archives de Pediatrie - Category: Pediatrics Authors: Tags: Arch Pediatr Source Type: research
Conclusions: In our cohort of pediatric MTC patients, athyreotic patients with preexisting hypothyroidism developed increased TSH and reduced free T4 during the first few months of treatment with vandetanib, necessitating an increase in LT dosage. Additional patients with normal thyroid function before treatment and intact glands (n = 2) maintained normal thyroid function tests during treatment. Elevated TSH in athyreotic patients may be due to an indirect effect of vandetanib on the metabolism of thyroid hormone, or to altered TSH sensitivity at the pituitary. Proper recognition and management of abnormal thyroid hormone ...
Source: International Journal of Pediatric Endocrinology - Category: Endocrinology Authors: Source Type: research
We present twenty-six of 33 MEN2 patients underwent prophylactic thyroidectomy with varying neck dissection and eight of 24 MEN2A patients with PHEO underwent adrenal-sparing surgery. Direct sequencing of entire RET exons was performed in all participants. Results The RET mutations (p.C634Y [n = 10], p.C634R [n = 9], p.C634F [n = 2], p.C618Y [n = 8], p.C618R [n = 3], and p.M918T [n = 1]) were confirmed in 20 symptomatic patients and identified in 13 at-risk relatives (RET carriers). Twenty-six of ...
Source: Hereditary Cancer in Clinical Practice - Category: Cancer & Oncology Source Type: research
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