IMPE Abstracts

Introduction: Graves’ disease (GD) is an immune-mediated thyrotoxicosis characterized by hypertrophy and hyperplasia of the thyroid follicles and accounts for 50-80% of all cases of pediatric hyperthyroidism. Thyroid nodularity is a common feature of GD, with an estimated prevalence of 25% – 53%. Thyroid nodules are less common among children than adults, being 20% malignant. The presence of GD and toxic nodular goiter is a rare finding, known as Marine-Lenhart syndrome. The incidence of malignancy in patients with toxic nodular goiter in children and adolescents has been reported as 5-29%, being a rarity in Marine-Lenhart syndrome.

Objective: to describe a patient with Marine-Lenhart syndrome and papillary thyroid carcinoma.

Case: a 16-year-old female patient with a history of autoimmune hepatitis (AIH) and celiac disease was referred for thyroid function screening. She had a positive family history of autoimmunity. Thyroid function tests one year prior were normal. Physical examination: weight -1.7 SDS, height -1.1 SDS, showed goiter and tachycardia, jaundice, and clinical signs of iatrogenic Cushing syndrome (longstanding corticosteroid treatment for AIH). Laboratory showed hyperthyroidism with TSH <0.01 uUI/ml (RV 0.5-6.5), fT4 3.07 ng/dl (RV 0.8-2.2), TPOAb and TGAb negative, TRAb 5.52 UI/l (RV 0 – 1.75). Thyroid ultrasound informed the presence of a solid, hypoechoic nodule (17.8*12*9mm), with irregular borders, calcifications, and increased vascularization in the upper right lobe and 2 pathological lymph nodes. Treatment was started with methimazole (0.2mg/kg/day). Scintigraphy was performed and described a “hot” nodule in the right lobe with diffuse faint uptake in the thyroid gland. The patient received Lugol´s solution to normalize promptly thyroid function. Total thyroidectomy was indicated without fine needle aspiration biopsy because of the instable clinical condition of the patient. Frozen section showed a thyroid papillary carcinoma, central and bilateral lymph node dissection was done. Histopathology confirmed a classic papillary thyroid carcinoma of the right lobe (initial ATA stratification: T1b, N1b, Mx – High risk) and follicular hyperplasia with lymphocytic inflammatory infiltrate of the left lobe. She received radioactive iodine ablation therapy and suppressive treatment with levothyroxine. She is now euthyroid but in hepatic failure.

Conclusion: the co-existence of a toxic nodular goiter with malignancy in an adolescent with GD is very rare and the finding of a thyroid nodule in GD deserves an exhaustive workup. Our case adds evidence to recent communications that alert on the risk that this entity entails.