Prophylactic Thyroidectomy in 75 Children and Adolescents with Hereditary Medullary Thyroid Carcinoma: German and Austrian Experience

1998 ◽  
Vol 22 (7) ◽  
pp. 744-751 ◽  
Author(s):  
Henning Dralle ◽  
Oliver Gimm ◽  
Dietmar Simon ◽  
Karin Frank-Raue ◽  
Günter Görtz ◽  
...  
Keyword(s):  
Thyroid Carcinoma ◽  
Children And Adolescents ◽  
Medullary Thyroid Carcinoma ◽  
Prophylactic Thyroidectomy ◽  
Medullary Thyroid ◽  
Hereditary Medullary Thyroid Carcinoma

Early, Prophylactic Thyroidectomy in Hereditary Medullary Thyroid Carcinoma

2015 ◽  
Vol 38 (5) ◽  
pp. 508-513 ◽  
Author(s):  
Maria R. Pelizzo ◽  
Francesca Torresan ◽  
Isabella M. Boschin ◽  
Davide Nacamulli ◽  
Gianmaria Pennelli ◽  
...  
Keyword(s):  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Prophylactic Thyroidectomy ◽  
Medullary Thyroid ◽  
Hereditary Medullary Thyroid Carcinoma

Phase I/II trial of vandetanib in children and adolescents with hereditary medullary thyroid carcinoma

2009 ◽  
Vol 27 (15_suppl) ◽  
pp. 10014-10014 ◽  
Author(s):  
E. Fox ◽  
B. C. Widemann ◽  
P. O. Whitcomb ◽  
A. Aikin ◽  
E. Dombi ◽  
...  
Keyword(s):  
Thyroid Carcinoma ◽  
Children And Adolescents ◽  
Medullary Thyroid Carcinoma ◽  
Growth Plate ◽  
Dose Level ◽  
Tyrosine Kinases ◽  
Linear Growth ◽  
Medullary Thyroid ◽  
Hereditary Medullary Thyroid Carcinoma ◽  
Tumor Measurements

10014 Background: Mutations in the RET protooncogenecause hereditary medullary thyroid carcinoma (MTC) including Multiple Endocrine Neoplasia (MEN) Type 2A, Type 2B and familial MTC. Vandetanib inhibits VEGFR, EGFR, and RET tyrosine kinases and is active in adults with hereditary MTC. Methods: We are conducting a trial of vandetanib in children and adolescents with RET mutations and MTC. Safety is evaluated at each dose level in adolescents (13–18 years) prior to enrolling children (5–12 years). In the absence of dose limiting toxicity (DLT), intrapatient dose escalation is permitted after cycle 2. To assess bone toxicity, growth plate volume is measured using MRI. Response is monitored using tumor measurements (RECIST), serum biomarkers, calcitonin (CTN), and carcinoembryonic antigen (CEA). Results: Five adolescents and 2 children were enrolled at the 100 mg/m2 dose level, 3 adolescents were dose escalated to 150 mg/m2. Six have MEN2B (M918T RET mutation). Median (range) baseline CTN was 12,200 pg/mL (2,300–67,000) and CEA was 104 mcg/L (5–325). Dose limiting diarrhea was observed in 1/5 adolescents at the 100 mg/m2 and 1/3 adolescents escalated to 150 mg/m2. No DLTs were observed in children receiving 100 mg/m2. Non-DLT included elevated TSH (n = 6), rash (n = 5), anorexia (n = 3), diarrhea (n = 2), hypertension (n = 1), and fatigue (n = 1). Median (range) percent change in growth plate volume during therapy was -18% (-44% to + 50%). All patients had linear growth during therapy. Serum CTN and CEA decreased by ≥ 50% in 6/7 and 2/7 patients, respectively. Tumor size decreased in 6/6 patients with M918T RET mutations; 2 achieved RECIST partial response after 6 and 12 cycles. Conclusions: Preliminary results suggest that vandetanib has activity in children and adolescents with MEN2B associated MTC. Vandetanib 100 mg/m2 was well tolerated. Linear growth was not impaired. [Table: see text]

New presentation of familial medullary thyroid carcinoma in 87-year-old patient with high-riskRETproto-oncogene codon 620 mutation

2008 ◽  
Vol 123 (7) ◽  
pp. 796-800 ◽  
Author(s):  
M K J Jaggard ◽  
C MacRae ◽  
S Ifeacho ◽  
S Robinson ◽  
N S Tolley
Keyword(s):  
High Risk ◽  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Early Development ◽  
Prophylactic Thyroidectomy ◽  
Current Recommendation ◽  
Clinical Prognosis ◽  
Medullary Thyroid ◽  
Familial Medullary Thyroid Carcinoma ◽  
Hereditary Medullary Thyroid Carcinoma

AbstractObjective:We report a case of familial medullary thyroid carcinoma in an 87-year-old woman, despite the patient having a high-risk codon 620 mutation.Method:Medline and PubMed were searched for cases and literature reviews relating to the following keywords: ‘codon 620’, ‘medullary thyroid carcinoma’, ‘multiple endocrine neoplasia’ and ‘RET proto-oncogene’.Results:We report the case of an 87-year-old woman who presented with a goitre, later identified as medullary thyroid carcinoma. Genetic analysis revealed aRETproto-oncogene codon 620 mutation. Genetic testing has revolutionised the management of medullary thyroid carcinoma. The genetic basis of hereditary medullary thyroid carcinoma lies with theRETproto-oncogene. Several disease-causing mutations of this gene have been identified and their clinical prognosis described. The penetrance of these mutations is high; as such, carriers progress to develop medullary thyroid carcinoma at a young age. Mutations at the codon 620 position are classified as high-risk for early development of medullary thyroid carcinoma; thus, the current recommendation is for prophylactic thyroidectomy at five years of age.Conclusions:In this case, the progress of hereditary medullary thyroid carcinoma was unique, considering the late presentation of medullary thyroid carcinoma despite the presence of the high-riskRETproto-oncogene codon 620 mutation. The authors wish to highlight the importance of this case, as it may present a counter-argument to the current recommendations for early, prophylactic thyroidectomy in codon 620 mutation carriers in order to prevent early development of medullary thyroid carcinoma.

scholarly journals Outcomes of Children and Adolescents with Advanced Hereditary Medullary Thyroid Carcinoma Treated with Vandetanib

2017 ◽  
Vol 24 (4) ◽  
pp. 753-765 ◽  
Author(s):  
Ira L. Kraft ◽  
Srivandana Akshintala ◽  
Yuelin Zhu ◽  
Haiyan Lei ◽  
Claudia Derse-Anthony ◽  
...  
Keyword(s):  
Thyroid Carcinoma ◽  
Children And Adolescents ◽  
Medullary Thyroid Carcinoma ◽  
Medullary Thyroid ◽  
Hereditary Medullary Thyroid Carcinoma

Evaluation of treatment approaches to the sporadic and hereditary medullary thyroid carcinoma in Turkey

2013 ◽  
Author(s):  
Bagdagul Yuksel ◽  
Berna Imge Aydogan ◽  
Alptekin Gursoy ◽  
Mazhar Muslum Tuna ◽  
Mehtap Vardar Basaran ◽  
...  
Keyword(s):  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Treatment Approaches ◽  
Medullary Thyroid ◽  
Hereditary Medullary Thyroid Carcinoma

Polymorphisms of cdkn1b gene may influence hereditary medullary thyroid carcinoma aggressiveness

2015 ◽  
Author(s):  
Raquel Bueno Barbieri ◽  
Lucas Leite Cunha ◽  
Natassia Elena Bufalo ◽  
Danilo Villagelin ◽  
Ligia Vera Assumpcao ◽  
...  
Keyword(s):  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Medullary Thyroid ◽  
Hereditary Medullary Thyroid Carcinoma ◽  
Cdkn1b Gene
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