9 year-old Female with Papillary Thyroid Cancer. Katie O Sullivan, M.D. Fellow Medicine/Pediatric Endocrinology Thursday, January 16 th, 2014

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1 9 year-old Female with Papillary Thyroid Cancer Katie O Sullivan, M.D. Fellow Medicine/Pediatric Endocrinology Thursday, January 16 th, 2014

2 Chief Complaint Mass on the right side of the neck x 2-3 weeks at 8yr 0 mo

3 History of Present Illness Right neck mass identified x 2-3 wks Tender x 1 day Treated with course of steroids and antibiotics General: Denies fever, fatigue, anorexia, weight loss, night sweats HEENT: Denies congestion, rhinorrhea, sore throat, dysphagia, hoarseness No history of radiation exposure

4 More History Birth History: Neonatal jaundice Developmental History: No delayed milestones Past Medical/Surgical History: Recurrent otitis media Medications: Clindamycin Allergies: Cephalosporins Immunizations: Up-To-Date Social History: 2 nd grade Lives in Indiana with family, 2 dogs. No exposure to cats. Family History: No family history of thyroid cancer.

5 Review of Systems Eyes: Negative for visual disturbance. Respiratory: Negative for cough or shortness of breath. Cardiovascular: Negative for palpitations or chest pain. Gastrointestinal: Negative for abdominal pain, nausea, vomiting, diarrhea, constipation. Genitourinary: Negative for urgency, frequency and enuresis. Musculoskeletal: Negative for arthralgias, edema. Skin: Negative for acne, rash, dry skin. Neurological: Negative for headaches. +intermittent tremulousness. Psychiatric/Behavioral: Negative for behavioral problems.

6 Physical Exam Vital Signs: BP 92/33, P 67, R 17, Wt 36.7kg (85%), Ht 131 cm (70 %), BMI 21.4 (96%) General: Well-developed, no distress. HEET: Conjunctiva clear, EOMI, PERRL, nasal turbinates normal, tongue normal, 2+ tonsils. Neck: supple, trachea midline, small jugulodigastric LN on left and 3 discrete firm, non-mobile LN on the right neck. No palpable thyroid or thyroid nodules. Axilla: No lymphadenopathy. CV: RRR, no murmur, no extremity edema. Pulmonary: CTAB, no crackles or wheezing. Abdomen: soft, non-tender, non-distended. Neuro: alert, 2+ patellar reflexes. Skin: warm, no diaphoresis.

7 Laboratory/Imaging Studies Ca CBC: WBC 11.8, Hgb 13, Plt %PMN, 18.3%Lymph, 6.9%Mono, 2.2%Eos CT Neck (outside film): Multiple matted LN extending under the SCM, some of which had a necrotic center Bilateral thyroid masses

8 Next Step: Neck LN biopsy under general anesthesia Findings: 3-4 hard LN anterior and beneath the right SCM Procedure: Excision of 2 right-sided LN measuring 1cm and 2cm. Pathology: Papillary thyroid carcinoma Extra-nodal extension present

9 Total Thyroidectomy 8/15/2012 Procedure: Total thyroidectomy, right modified radical neck dissection, paratracheal and pretracheal node dissection Pathology: Papillary thyroid cancer, chronic lymphocytic thyroiditis Thyroid gland: 2.5cm nodule and widely-invasive cancer in bilateral lobes and isthmus; capsular invasion, positive margins Lymph Nodes: 32/51 nodes positive Extranodal extension present

10 Post-Op TSH 13.3 FT Ca 7.8 Phos 4.4 PTH 4 Started: LT4 3mcg/kg (112mcg/d) Calcium carbonate 1250mg po q6h Cholecalciferol 2000IU daily Calcitriol 0.5mcg qd Vit D 25-OH 26

11 Next step in management?

12 Post-Operative Management Labs 12/2012: Anti-Tg AB 630 IU/mL (nl <22) Pre-RAI scan: 2.15mCi I-131 RAI ablation 1/2013: 60mCi I-131 Post-therapy RAI scan 6-month post-rai scan 7/2013: 1.5mCi I- 131

13 TSH and Free T4 Trend Oct-12 Nov-12 Dec-12 Jan-13 Feb-13 Mar-13 Apr-13 May-13 Jun-13 Jul-13 Aug-13 Sep-13 Oct-13 Nov-13 Dec-13 Date TSH Free T4

14 Clinical Questions How do you determine the prognosis of children with differentiated thyroid cancer? What is the standard goal thyrotropin for children s/p thyroidectomy for differentiated thyroid cancer?

15 Well-Differentiated Thyroid Cancer (WDTC) in Children Accounts for 3-13% of all WDTC Most common endocrine malignancy in children Children present with more extensive disease than adults Mortality is low Dinauer et al. Curr Opin Onc Rachmiel et al. Ped Endo Metab Shayota et al. Surgery Zimmerman et al. Surgery 1988.

16 Calculating Prognosis of Well- Differentiated Thyroid Cancer Cooper et al. Thyroid 2009.

17 Shayota et al. Surgery 2013.

18 Shayota et al. Surgery 2013.

19 Clinical Questions How do you determine the prognosis of children with differentiated thyroid cancer? What is the standard goal thyrotropin for children s/p thyroidectomy for differentiated thyroid cancer?

20 TSH Suppression Therapy Adults: Pediatrics:??? Cooper et al. Thyroid 2009.

21 Royal Marsden Hospital Landau et al. European Journal of Cancer 2000.

22 Royal Marsden Hospital Landau et al. European Journal of Cancer 2000.

23 Conclusion WDTC is the most common endocrine cancer in children Poor prognostic factors for children with WDTC include male gender, larger primary tumor size and presence of distant metastasis Surgical and RAI therapy in children with WDTC is still controversial TSH suppression therapy may be beneficial, however the goal TSH has not been welldescribed in children

24 Works Cited Cooper et al. Revised American Thyroid Association management guidelines for patients with thyroid nodules and differentiated thyroid cancer. Thyroid 2009;19(11):1-47. Dinauer et al. Differentiated thyroid cancer in children: diagnosis and management. Current Opinion in Oncology 2008;20: Landau et al. Thyroid cancer in children: the Royal Marsden Hospital experience. European Journal of Cancer : Rachmiel et al. Evidence-based review of treatment and follow-up of pediatric patients with differentiated thyroid carcinoma. Journal of Pediatric Endocrinology and Metabolism 2006; 19: Rapkin L and Pashankar FD. Management of thyroid carcinoma in children and young adults. Journal of Pediatric Hematology and Oncology 2012; 34(supp 2):S39-S46. Shayota et al. MeSS: A novel prognostic scale specific for pediatric welldifferentiated thyroid cancer: A population-based, SEER outcomes study. Surgery 2013; 154: Zimmerman et al. Papillary thyroid carcinoma in children and adults: long-term follow-up of 1039 patients conservatively treated at one institution during three decades. Surgery 1988;104:

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