1. Management of Pediatric Thyroid Nodules Erin Kirkham, MD Resident Conference September 23, 2010

2. Case: H.M. 12 yo F with neurofibromatosis type 1 CC: thyroid nodule Noticed in the mirror one month prior. Had not increased in size. Nontender. No skin changes. No dysphagia or stridor. Occassional “froggy voice” (due to tonsillitis?) Denied sx of hyper/hypoTH

3. Case: H.M. PMH: neurofibromatosis 1, scoliosis, migraines, recurrent tonsillitis – No h/o previous radiation PSH: none Meds: none FH: mother with NF-1 and s/p total thyroidectomy for “precancerous nodules”

4. Case: H.M. PE: Unremarkable aside from a roughly 3 cm round, firm, nonmobile nodule in area of the L thyroid lobe that elevated upon swallowing. No hoarseness noted.

5. Case: H.M. Thyroid US: – R lobe normal in size – L lobe Complex cystic mass 3.5 x 4.6 x 5.4 cm – Scattered subcentimeter cystic nodules in both lobes Labs: – CBC, TFTs WNL, negative anti-TH antibodies

6. Thyroid Nodules in Children Incidence of nodules: < 2% Of those, an estimated 15-20% are malignant – Compared to 5% of adult nodules High incidence of LN (40-80%) & distant (25%) metastasis Outcomes for carcinoma are generally positive – Overall mean survival 30.5 years – 30 year survival probability: 90%

7. Differential Diagnosis Adenoma Cysts (colloid, simple, hemorrhagic) Inflammatory lesions (thyroiditis) Carcinoma (in kids) – Papillary (85%) – Follicular (10%) – Medullary (5%)

8. Different Differential? Could this be a thyroid neurofibroma? – Documented in scattered case reports Anagnoustouli M et al. Thyroid gland neurofibroma in a NF1 patient. Acta Neurol Scand 2002; 106:58-61. Kandil E et al. Primary Peripheral Nerve Sheath tumors of the Thyroid Gland. Thyroid 2010; 6:583-6. – Unlikely to be consistent with a cystic lesion located within the gland ? +

9. Workup Thyroid function tests – Low TSH  radionucleotide scan “Hot” generally “not” cancer – High/NL TSH  US Ultrasound – Indicated for any cold nodule – Suspicious features: Microcalcifications Hypoechoic Increased vascularity Taller than wide

10. Workup U/S guided FNA – Indications Nodules > 0.5 cm if suspicious or with high risk history Nodules > 1 cm if solid Nodules >1.5 cm with any suspicious features – Four categories Malignant Suspicious/indeterminate Benign Nondiagnostic – Utility in pediatric patients Sensitivity 94% Specificity 81%

11. Surgical Management Total or near-total thyroidecomy – Carcinomas > 1 cm – Indeterminate nodules >4 cm – Pathologically suspicious lesions – Patients with a family history – Patients with a radiation history Selective neck dissection – Indications: involvement of central/lateral nodes – Preop U/S should be obtained to visualize and biopsy any suspicious nodes Radioablation – Indications: tumors > 4 cm, extraglandular invasion, distant metastases

12. Surgical Management Lobectomy – Carcinomas <1 cm – Unifocal – Intrathyroid – Low-risk history – No involved nodes

13. Case: H.M. Plan: – Repeat thyroid US with attention to LN – Parathyroid studies – U/S guided FNA – Scope by ENT to assess VC function

14. Sources Tufano R, Glazer C, Randolf G. Thyroid and Parathyroid Glands. Essential Otolaryngology Head and Neck Surgery. McGraw Hill Medical, 2008, p603-634. Hogan AR, Zhuge Y; Perez EA; Koniaris LG; Lew JI; Sola JE. Pediatric thyroid carcinoma: incidence and outcomes in 1753 patients. J Surg Res. 2009 Sep; 156(1): 167-72. Bargren AE, Meyer-Rochow GY, Sywak MS, Delbridge LW, Chen H, Sidhu SB. Diagnostic utility of fine-needle aspiration cytology in pediatric differentiated thyroid cancer. World J Surg. 2010 Jun;34(6):1254-60. Surgery. 2010 Sep;148(3):532-7. Epub 2010 Mar 16. Stevens C, Lee JK, Sadatsafavi M, Blair GK. Pediatric thyroid fine-needle aspiration cytology: a meta- analysis. J Pediatr Surg. 2009 Nov;44(11):2184-91. Cooper, DS, Doherty, GM, Haugen, BR, et al. Revised American Thyroid Association management guidelines for patients with thyroid nodules and differentiated thyroid cancer. Thyroid 2009; 19:1167. Hung, W, Sarlis, NJ. Current controversies in the management of pediatric patients with well-differentiated nonmedullary thyroid cancer: a review. Thyroid 2002; 12:683. Handkiewicz-Junak, D, Wloch, J, Roskosz, J, et al. Total thyroidectomy and adjuvant radioiodine treatment independently decrease locoregional recurrence risk in childhood and adolescent differentiated thyroid cancer. J Nucl Med 2007; 48:879. Anagnoustouli M et al. Thyroid gland neurofibroma in a NF1 patient. Acta Neurol Scand 2002; 106:58-61. Kandil E et al. Primary Peripheral Nerve Sheath tumors of the Thyroid Gland. Thyroid 2010; 6:583-6.