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Thyroid Malignancies In Children

Thyroid Malignancies In Children. Bhaskar N. Rao, M.D. St. Jude Children’s Research Hospital 10/03. THYROID CANCER Staging. T 0 No evidence of tumor T 1 tumor <1 cm T 2 tumor 1-4 cm T 3 tumor >4 cm T 4 tumor any size beyond capsule N 0 No nodal mets N 1 regional nodes

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Thyroid Malignancies In Children

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  1. Thyroid MalignanciesIn Children Bhaskar N. Rao, M.D. St. Jude Children’s Research Hospital 10/03

  2. THYROID CANCERStaging T0 No evidence of tumor T1 tumor <1 cm T2 tumor 1-4 cm T3 tumor >4 cm T4 tumor any size beyond capsule N0 No nodal mets N1 regional nodes N1a ipsilateral cervical nodes N1b bilateral or mediastinal M0 N0 distant mets M1 distant mets

  3. THYROID CANCERStaging (Pap/follicular) Age <45 Stage 1 Any T Any N M0 Age >45 Stage 1 T1 N0 M0 Stage 2 T2/T3 N0 M0 Stage 3 T4 or N0 M0 T1-4 N1 M0 Stage 4 T1-4 Any N M1 Anaplastic All cases are stage IV Staging (Medullary) Stage 1 T1 N0 M0 2 T2-4 N0 M0 3 Any T N1 M0 4 Any T Any N M1

  4. Thyroid CancerEpidemiology • 20,000 new cases/year in the US • more often in women and whites • Peak incidence: 40 (women), 60 (men) • Lifetime risk: 1% • Histology: Papillary - 80% Follicular - 11% Hurthle cell - 3% Medullary - 4% Anaplastic - 2%

  5. Thyroid CancerEpidemiology - Children • Low incidence in childhood • 1.5% of all tumors < 15 years • peak 7-12 years • 10% of all head and neck cancer • 10% are diagnosed in childhood • 2/3 in girls • Indolent course, even with metastases • Survival > 90% • Up to 8% of secondary pediatric cancers

  6. Thyroid CancerEpidemiology - Children • Low dose RT used for Thymus, Hemang, Acne • Average dose 600cgy. One million people at risk • One fourth will develop nodules • Most (75%) Benign Hyperplasia, Adenoma, Fibrosis • Treatment Lobectomy – Post-op Hormones

  7. Thyroid CancerEpidemiology - Children • Increased risk of Carcinoma • Most are Papillary Carcinomas (20-50%) • Latency median 20 years • Most are multicentric, with lymph nodes • Other tumors – Salivary Gland, Parathyroid, Bone, Soft tissue Sarcomas, Thyroid lymphomas

  8. Thyroid Cancer Histology • Papillary • 80% incidence increases with younger age • High incidence of bilaterality, regional nodes • Follicular • Rare in children • Distinguished from adenoma by vascular or capsular invasion • Medullary • arise from calcitonin-secreting c-cells • Anaplastic • Extremely aggressive, high mortality Tumor Variable Affecting Prognosis • Histology • Size • Local invasion • Lymph node • Distant metastases

  9. Thyroid CancerEpidemiology - Children • Thyroid cancer has proven to be a common SNM • Between 1980 & 1987 58 centers in Europe reported 239 SMN’s • 18 of 239 (7.5%) were thyroid cancers • 6 / 18 primary was Hodgkins all received chemo + RT (25-42gy) • 7 / 18 primary was ALL all had CS RT (18-24gy) • 2 Ewings, 1 Wilms, 1 NB and 1 NPC

  10. Thyroid nodules • By far most thyroid nodules are benign and are either colloid nodules, adenomas or manifestations of thyroiditis • They may be cystic or solid • Most cystic are generally benign (degenerated colloid) • They may be toxic or non toxic Thyroid CancerPediatric vs. Adult • Thyroid masses more likely to be cancer • 50% of solitary nodules are malignant • More often larger, multicentric • Higher rate of metastasis at diagnosis • regional lymph nodes: 65% (35% adult [papillary]) • distant: 20% (10% adult [follicular]) • Higher rate of recurrence • 40% <20y (also >60y); 20% adults • 80% locoregional, 20% distant (similar)

  11. Thyroid CancerDiagnostic Imaging • Traditionally I131 Now I123 or Technitium scans • Nodules hyperfunctional (hot) with increased avidity • Functional cold same as rest of gland • Minimum 1 cm diameter for cold nodules • Hot functional nodules practically benign • Cold – incidence of malignancy higher

  12. Thyroid CancerDiagnostic Imaging • USG – differentiate multinodular vs solitary • CT or MRI – invasive lesion or sub-sternal location • Specific / sensitive is F.N.A. • Malignant, suspicious, benign or inadequate • If it is suspicious I123 , hot, rarely malignant cold 20% or higher

  13. Better overall survival >95% for children 75-90% for adults Better survival with metastases 86% of children 32% of adults Thyroid CancerPediatric vs. Adult Thyroid malignancies in pediatric population – how is it different? • Papillary ca. constitutes 85-90% of all malignant lesions with medullary second, forming 5% • Unlike adults follicular not as common and when present it is usually in the adolescent population • Thyroid lymphomas and metastasis are hardly ever seen in pediatric population • In familial medullary ca. prophylactic thyroidectomy is done in kids before they attain age 5yr • PARADOX: • often presents with extensive disease and progression or recurrence in a significant number of patients • is rarely fatal • Suggests biologic rather than treatment factors have a greater effect on outcome

  14. Increased suspicion Male Nodule > 4cm Age < 15 yr H/O XRT exposure H/O Pheochromocytoma Hyperparathyroidism Gardner’s FAP Carney’s complex Cowden’s syndrome Highly suspicious Rapid nodule growth Fixation Family history V.C paralysis Lymph nodes Neck invasion Approach to a malignant thyroid noduleClinically suspicious nodule >1cm • TSH • FNA of nodule/ lymph nodes • If insufficient FNA → repeat FNA (imparts 50% extra chance) • US solid or cystic and assist in FNA and determining the size of the nodule • Cystic nodules may be followed

  15. Thyroid Carcinoma Fine needle aspiration important Distinguishing benign/malignant follicular difficult Thyroid nodules containing follicular cytopathologic features have 20-30% malignancy Thyroid malignancy rate is 6.8% without atypia and 44-50% with atypia Allows for conservative approach in selected patients

  16. Thyroid CancerSurgical Options • Total Thyroidectomy in patients with invasive or metastatic or bilateral or previous RT • For others – controversy varies with surgery and complication rates • Unilateral P.C. or F.C. < 1.5 cm lobectomy + isthmus • If > 1.5 cm opposite lobe 30-80% recurrence rate is 10% • Recurrence associated with 30% mortality with 50% desease found in central neck • Total Thyroidectomy recurrence less than 5%

  17. sup. parathyroid recurrent laryngeal n.

  18. Thyroid CancerSurgery Risk vs. Benefit • Total Thyroidectomy • High risk groups: radiation, MTC, Anaplastic • Simplifies use of radioiodine • Follow thyroglobulin levels • Increased risk without increased survival benefit • 15% each-recurrent laryngeal nerve injury, hypoparathyroidism • 30% higher than lobectomy

  19. Thyroid Cancer in Childhood sup. parathyroid Challenges of ThyroidCancer Management recurrent laryngeal n. • No prospective randomized trials of treatment • The prognosis is generally excellent

  20. Thyroid CarcinomaMinimally Invasive Surgery Criteria by Niccoli et al. (Am J Surg, 2001) Nodules less than 3.5 cm Total thyroid volume less than 15ml No previous neck surgery or irradiation Absence of thyroiditis/invasion Total 336 pts. One-third total thyroidectomy Conversion 4.5% • Yamashita et al. • 25-30 mm transverse upper lateral neck • Total 39 pts. Recurrent nerve injury one • Tumor size 1.9 – 5.5 cm • Surgery 56 mm (36-90 minutes) • Other approaches described Axillary Approach

  21. Papillary ca. (dx. By FNA) and high risk Total thyroidectomy If L.N positive Central neck disec Lateral neck disec.(level II-IV, sparing spinal accessory nerve, int. jugular, SCM) Approach to a thyroid nodule(Papillary on FNA-high risk)

  22. Management post lobectomy for papillary (<1cm- low risk) • Their recurrence and cancer specific mortality rates are almost zero • Supress TSH with thyroxine • Tg and whole body I scan are insensitive • Physical exam with local neck US seem to be the best suggested follow up

  23. Follow up papillary • P/E q 3-6 mo for 2yrs with periodic US • Tg @ 6 & 12mo then annually • RI scans q 12mo • Periodic CXR/ CT chest • For locoregional recurrences → surgery followed by RI • Tg rise >10ng/ml → RI therapy with 100-150mCi

  24. Thyroid Carcinoma Follicular lesion Follicular carcinoma represent 10-20% Prognostic factors include size, age, metastasis Witte et al., report L.N., size, stage, mets, sex Advised total thyroidectomy + L.N. dissection and ipsilateral or bilateral L.N. dissection for T3, T4 TSH high → Thyroxine/Surg Follicular TSH normal → Surgery TSH low → Thyroid scan hot cold

  25. Invasive Follicular carcinoma on lobectomy Further local and metastatic work up < 1cm → observe/ re-resect > 1 cm → completion thyroidectomy followed by I 131 Approach to thyroid nodule(Follicular on lobectomy)

  26. Medullary on FNA Calcitonin levels CEA Pheo screening Serum calcium Screen for RET proto-oncogene Neck US Approach to a thyroid noduleMedullary carcinoma

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