JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY
REFERENCES
One of our patients was known to have Gorlin-Goltz syndrome (patient 3), also known as nevoid basal cell carcinoma syndrome (NBCCS). NBCCS represents a series of multi-organ abnormalities known to be the consequence of abnormalities in the PTCH gene.13 These include basal cell carcinomas, skeletal abnormalities, facial dysmorphism, odontogenic keratocysts, medulloblastoma, ovarian fibromas, and less frequently, increased risk of all malignancies, including thyroid carcinoma.¹⁴ This patient, was referred for endocrine evaluation incidental to the finding of multiple small thyroid cysts discovered on a neck imaging study for a non-thyroid indication. She has been followed on levothyroxine therapy for three years without changes in thyroid gland size or cysts. Benign thyroid cystic changes in NBCCS patients is not a previously reported part of the syndrome. As PCT may be a more commonly encountered incidental finding than previously appreciated the multiple small thyroid cysts discovered in our patient may not be part of the NBCCS syndrome. There are several limitations of this case study. As the patients were otherwise healthy and there was no clinical progression, thyroid biopsies were not performed as had been previously done in the reports of Kubota et al.³ In Japanese adult patients with PCT, hypothyroidism was associated with high dietary iodine intake. We did not assess iodine intake in our patients. However, dietary iodine content among children in the U.S. is generally not excessive¹⁵ and potentially prevents deterioration to hypothyroidism. The period of follow up in our patient cohort was relatively brief. As most patients reported with overt hypothyroidism were adults, PCT may require a long period of time before hypothyroidism occurs and/or high dietary iodine. Many of the patients in our series were prophylactically started on thyroid replacement which may have prevented progression of pathology during follow-up. In conclusion, we speculate that PCT has not been previously described as a distinct entity in pediatrics due to infrequent occurrence, subtlety of the clinical thyroid enlargement, and absence of overt clinical and biochemical hypothyroidism. Although pediatric PCT may be infrequent, non-progressive, and benign there has been no follow up of this entity from childhood into adulthood. Until there is further information on the long-term natural history of PCT discovered in the pediatric patients, we recommend periodic long-term follow up through adulthood.
1. Avula S, Daneman A, Navarro OM, Moineddin R, Urbach S, Daneman D: Incidental thyroid abnormalities identified on neck us for non-thyroid disorders. Pediatric radiology 2010;40:1774-1780. 2. Kubota S, Maruta T, Fujiwara M, Hagiwara H, Tsujimoto N, Kudo T, Nishihara E, Ito M, Amino N, Miyauchi A: The prevalence of polycystic thyroid disease in hypothyroid patients with negative thyroid autoantibodies. Thyroid : Thyroid 2010;20:1205-1208. 3. Kubota S, Hirokawa M, Takamura Y, Ito Y, Tamai H, Kudo T, Nishihara E, Ito M, Amino N, Miyauchi A: Pathologic features of polycystic thyroid disease: Comparison with benign nodular goiter. Endocr J 2011;58:783-788. 4. Naranjo ID, Robinot DC, Rojo JC, Ponferrada MR: Polycystic thyroid disease in pediatric patients: An uncommon cause of hypothyroidism. J Ultrasound Med 2016;35:209-211. 5. Lucas KJ: Use of thyroid ultrasound volume in calculating radioactive iodine dose in hyperthyroidism. Thyroid 2000;10:151-155. 6. Recommended normative values for thyroid volume in children aged 6-15 years. World health organization & international council for control of iodine deficiency disorders. Bull World Health Organ 1997;75:95-97. 7. Kubota S, Fujiwara M, Hagiwara H, Tsujimoto N, Takata K, Kudo T, Nishihara E, Ito M, Amino N, Miyauchi A: Multiple thyroid cysts may be a cause of hypothyroidism in patients with relatively high iodine intake. Thyroid : Thyroid 2010;20:205-208. 8. Leung AM, Braverman LE: Iodine-induced thyroid dysfunction. Current opinion in endocrinology, diabetes, and obesity 2012;19:414-419. 9. Bahn RS, Castro MR: Approach to the patient with nontoxic multinodular goiter. J Clin Endocrinol Metab 2011;96:1202-1212. 10. Gutekunst R, Hafermann W, Mansky T, Scriba PC: Ultrasonography related to clinical and laboratory findings in lymphocytic thyroiditis. Acta endocrinologica 1989;121:129-135. 11. Marcocci C, Vitti P, Cetani F, Catalano F, Concetti R, Pinchera A: Thyroid ultrasonography helps to identify patients with diffuse lymphocytic thyroiditis who are prone to develop hypothyroidism. J Clin Endocrinol Metab 1991;72:209-213. 12. Kobayashi K, Hirokawa M, Yabuta T, Fukushima M, Kihara M, Takamura Y, Ito Y, Miya A, Amino N, Miyauchi A: Papillary thyroid carcinoma with honeycomb-like multiple small cysts: Characteristic features on ultrasonography. Eur Thyroid J 2013;2:270-274. 13. Scully C, Langdon J, Evans J: Marathon of eponyms: 7 gorlin-goltz syndrome (naevoid basal-cell carcinoma syndrome). Oral diseases 2010;16:117-118. 14. Gorlin RJ: Nevoid basal cell carcinoma (gorlin) syndrome. Genet Med 2004;6:530-539. 15. Perrine CG, Sullivan KM, Flores R, Caldwell KL, Grummer-Strawn LM: Intakes of dairy products and dietary supplements are positively associated with iodine status among U.S. Children. J Nutri 2013;143:1155-1160. Eileen Báez, MD, Liladhar Kashyap, MD, Laurie Finger, APRN, and Stuart Chalew, MD are affiliated with Louisiana State University Health Sciences Center-New Orleans, Department of Pediatric Endocrinology and Diabetes in NewOrleans, Louisiana. MarieHaymon, MD is affiliatedwith Children's Hospital, Department of Pediatric Radiology in New Orleans, Louisiana.
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