Advanced Medullary Thyroid Carcinoma in an 11-Year Old Boy: Treatment with Vandetanib

G SANSO; I BERGADÁ; P PAPENDIECK ;; E ELIAS; A CHIESA ; A VIEITES; M GARCÍA

Cusco

Congreso; XXVIII Sociedad Latinoamericana de Endocrinología Pediátrica; 2018

Sociedad Latinoamericana de Endocrinologia Pediatrica SLEP

An 11 year old boy with a longstanding historyof hypotonia and diarrhea starting at 3 months of age Consultedat our center Because of a multinodular goiter (MNG) with multipleadenomegalies detected on a spinal MRI and CNS. Physicalexam Showed to read boy (BMI: 12.5), with average stature andmar-fanoid phenotype, thick lips and eyelids and multiple smallwhitish nodules on His tongue. Cervical examination revealedcervical MNG with adenomegalies. TSH was 25.8 mU/I Normalthyroid hormones with, negative thyroid antibodies, circulatingcalcitonin 58085 pg/ml (RV: 0?46) and the normal urinary catecholamines.Cervical ultrasound Showed a left dominant heterogeneousnodule with Increased vascularization center and rightsmall microcalcifications and 2 nodules with microcalcificationsand abnormal cervical multiple lymphadenopathies. FNAB waspositive for medullary carcinoma (MTC) with wash out positivefor calcitonin. Bone scan was negative but multiple pulmonarymetastases Were found on CT. Proto-oncogene RET mutationc.2753T>CpM918T was found in exon 16 confirming MEN 2BSyndrome. Partial resection of the thyroid tumor and neck dissectionwas left Decided to reduce tumor mass and adjacent tissuecompression. In the presence of a locally and distant metastaticMTC with vandetanib treatment was started. After the first monthDecreased by 90% calcitonin. Currently after 22 months of treatmentHis serum calcitonin is 1400 pg/ml. Dramatically improvedgastrointestinal symptoms and allowed school reinsertion. Photosensitivitywas the only adverse event and was managed withLocal treatment. His illness has stabilized vandetanib and alloweda better quality of life.