Pediatrics Endocrine Masssssssssssssss.pptx

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1. Approach to Paediatrics Endocrine Masses Samuel Kefyalew (PSR4) Moderator : Dr. Fisseha Temesgen (Consultant General and Paediatrics Surgeon, FCOSECSA) September, 2022 1

2. Outline Introduction Thyroid Gland Parathyroid Glands Adrenal Glands References 2

3. Introduction Endocrine neoplasms are a rarity in childhood 1000 to 2000 per 100 000 for thyroid nodules 2 to 5 per 100 000 for hyperparathyroidism (HPT) 2 per 10 000 000 for adrenocortical tumors Superior outcomes when endocrine surgery is performed at a specialty center 3

4. Thyroid Gland 4

5. Thyroid Hormone Synthesis 5

6. Circulating Thyroid Hormones >99 % of circulating T4 and T3 is protein bound 0.02% of T4 and 0.4% of T3 is free Half life T4: 7 days T3: 12 -24 hours Control of thyroid function 6

7. NON-NEOPLASTIC THYROID CONDITIONS GOITER AND THYROIDITIS Goiter Etiology in children is broad and geographically different Many parts of the world : endemic goiter Developed countries : related to inflammatory conditions or nodular enlargement Children with thyroid enlargement are often euthyroid and asymptomatic 7

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10. CHRONIC LYMPHOCYTIC (HASHIMOTO) THYROIDITIS AKA “ chronic autoimmune thyroiditis” Most common cause of acquired hypothyroidism in children Prevalence of 1–2% F:M of 4:1 Goiter is the most common presentation 10

11. Diagnosis Laboratory workup TFT Antithyroid peroxidase (TPO) antibodies/ antithyroglobulin antibodies: 85–90% Ultrasound: non specific FNAB: solitary suspicious nodule or a rapidly enlarging goiter Management Euthyroid patients Expectant management Spontaneous resolution: 1/3 rd Hypothyroid patients Exogenous thyroid hormone Subclinical hypothyroidism Controversial Hyperthyroid patients Beta-adrenergic receptor blockers 11

12. SUBACUTE (DE QUERVAIN) THYROIDITIS ? a viral inflammation of the thyroid gland that is unusual in children Presentation: swollen, painful, and tender thyroid Diagnosis Mild thyrotoxicosis Decreased radioactive iodine uptake Histology: Granulomas and epithelioid cells Treatment : Supportive : Nonsteroidal anti-inflammatory agents or corticosteroids. Beta-adrenergic receptor blocker Thyroid hormone replacement Subacute thyroiditis usually lasts 2–9 months , with complete recovery being the norm . 12

13. ACUTE SUPPURATIVE THYROIDITIS A bacterial infection of the gland Characterized: fevers , chills, hoarseness, dysphagia , and an inflamed lobe Patients are usually euthyroid Management Intravenous antibiotics and abscess drainage, if necessary . Excision with left hemithyroidectomy Congenital pyriform sinus tract The thyroid gland typically recovers completely 13

14. GRAVES DISEASE Most common cause of hyperthyroidism in childhood Presentation : Unexplained weight loss and/or an increased rate of linear growth; onset of emotional lability, poor attention span, and hyperactivity with smooth, firm, non tender goiter Diagnosis TFT TSH receptor antibodies(definitive diagnosis): 94% to 100% 14

15. Treatment Goal: decrease the production and secretion of thyroid hormone Options : antithyroid medications, ablation with radioactive 131I, and thyroid resection Methimazole (MTH ) The dosing is 0.5–1 mg/ kg/day with a maximal dose of 30 mg/day The daily dose of MTH should be reduced to 10 mg when the patient is euthyroid , with normal T3 and T4 levels. Thyroid enlargement with therapy: an intensification of the disease or hypothyroidism due to overtreatment 15

16. The goal of definitive therapy is permanent hypothyroidism Preoperative preparation Antithyroid medication β- blocking agents Lugol’s solution 16

17. NEOPLASTIC THYROID CONDITIONS THYROID NODULES Less common in children than adults (incidence of 1% to 2 %) Higher incidence of malignancy (20–36 % vs 5% in adults) 17

18. In children , US characteristics and clinical context are more important than size 18

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20. THYROID CARCINOMA The incidence of thyroid carcinoma is increasing in both children and adults Histopathology of thyroid cancer in children is similar to that in adults : 80 % papillary thyroid cancer 10 % follicular thyroid cancer 5 % medullary thyroid cancer (MTC ) 2 % other types of thyroid cancer 20

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22. Children : more advanced-stage disease, a higher incidence of lymph node and pulmonary metastases, but have a lower mortality rate than adults Presentation: thyroid mass, sometimes with enlarged cervical lymph nodes The Bethesda classification for cytology, i n children Bethesda Category III–V: very high rates of malignancy in children Imaging Comprehensive neck US Cross-sectional imaging : bulky disease or vocal cord paralysis 22

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25. Management : Papillary thyroid cancer Total thyroidectomy CLND: evidence of invasion outside the thyroid gland, and/or if locoregional metastases Lateral neck dissection: cytologic evidence of metastases Levothyroxine Radioactive iodine Systemic treatment / clinical trials 25

26. Postoperative staging and surveillance Staging within 12 weeks after resection Includes : 123I diagnostic whole body scans, TSH-stimulated thyroglobulin, and neck US or positron emission tomography(PET )/computed tomography (CT ) 131I therapy Nodal or other locoregional disease that cannot be resected as well as distant metastases Neck US surveillance 6 months postoperatively, and then annually for ATA low-risk children 6- to 12-month basis for ATA intermediate and high-risk children Surveillance should be continued for at least 5 years 26

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28. Management : Follicular thyroid cancer More debatable Total thyroidectomy: clear vascular invasion , distant metastases, or tumor size > 4 cm Staged postoperatively with RAI Lobectomy : minimal or no vascular invasion (three or fewer vessels involved), < 4 cm in size Should be decided on a case-by-case basis Radioactive iodine therapy Genetic counselling and genetic testing for germline PTEN mutations 28

29. Management : Medullary thyroid cancer Work up: calcitonin and carcinoembryonic antigen (CEA ), DNA analysis for RET germline mutation, and metastatic workup should ensue (including CT neck, chest, and abdomen) Total thyroidectomy CLND: calcitonin <500 pg /mL and no suspicious US findings in the lateral neck LLND : US findings of suspicious nodes in the lateral neck External beam radiotherapy : extensive disease, residual disease, or extrathyroidal extension Prophylactic thyroidectomy 29

30. Parathyroid Glands 30

31. PHYSIOLOGY Physiologic effects of parathyroid hormone CaSR = Calcium sensing receptor 31

32. HYPERPARATHYROIDISM Rare in children: 2–5 per 100,000 More common in girl Presents with inappropriately elevated plasma PTH levels and hypercalcemia May be primary, secondary, or tertiary 32

33. In general, hyperparathyroidism presents clinically with symptoms related to elevated calcium or is suspected when hypercalcemia is found incidentally 33

34. PRIMARY HYPERPARATHYROIDISM Cause Solitary hyperfunctioning adenoma : most common Diffuse hyperplasia of all four glands Parathyroid carcinoma Presentation: asymptomatic (20%), urinary and bone impairment, as well as neurologic manifestations of hypercalcemia Diagnosis: elevated PTH levels (>65 pg /mL), in the presence of normal or elevated serum calcium (>10.2 mg/ dL ) Preoperative localization studies Management Unilateral neck exploration Neck exploration Inappropriate IO-PTH response with single gland excision, a single lesion is not identified, parathyroid gland hyperplasia, or suspected MEN syndromes Parathyroid hyperplasia involving all of the glands, operative management can include 3 ½ gland parathyroidectomy Total parathyroidectomy with heterotopic autotransplantation Parathyroid carcinoma : en -bloc hemithyroidectomy, parathyroidectomy, and lymph node dissection 34

35. A rapid intraoperative parathyroid hormone (IOPTH) assay A 50% decline in PTH level from baseline at 10 minutes indicates that the offending parathyroid gland or glands have been removed 35

36. Neonatal Severe Hyperparathyroidism and Familial Hypocalciuric Hypercalcemia Neonatal severe hyperparathyroidism Management Parathyroidectomy with transplantation of one gland Potent intravenous bisphosphonates Familial hypocalciuric hypercalcemia Managed medically 36

37. SECONDARY HYPERPARATHYROIDISM Occurs in children with renal insufficiency resulting in reduced renal vitamin D activation and therefore decreased GI absorption of calcium Total parathyroidectomy with autotransplantation Severe cases of secondary hyperparathyroidism/ severe renal osteodystrophy TERTIARY HYPERPARATHYROIDISM Parathyroid glands are exposed to prolonged stimulation from long-term hypocalcemia and begin autonomous PTH production, even after the inciting stimulus has been removed. Management Total parathyroidectomy with autotransplantation . 37

38. Adrenal Glands 38

39. PHYSIOLOGY Adrenal cortex: Aldosterone Cortisol Androgens Adrenal medulla: Epinephrine and norepinephrine 39

40. ADRENAL MASSES Neuroblastoma: >90 % of adrenal masses in children Incidental adrenal mass: prompt hormone evaluation All functioning adrenal cortical tumors and pheochromocytomas should undergo resection 40

41. ADRENAL CORTEX HYPERCORTISOLISM (CUSHING SYNDROME) Iatrogenic Cushing syndrome is the most common cause Clinical presentation Weight gain and growth failure 41

42. Diagnosis Laboratory evaluation Midnight plasma cortisol : >2 mg/ dL 24-hour urinary 17-hydroxycorticosteroid or free cortisol : >is >150 mg/day Overnight dexamethasone suppression test Specific cause High dose dexamethasone suppression test Plasma ACTH level Management Resection of primary lesion Pituitary irradiation Medical therapy 42

43. PRIMARY HYPERALDOSTERONISM Adrenocortical hyperplasia is the most common cause in children Others causes: adrenal adenoma, adrenal carcinoma Presentation : hypertension and hypokalemic alkalosis Confirmation: saline load challenge 43

44. Aldosterone-secreting adenoma or bilateral adrenal hyperplasia MRI or CT Selective adrenal vein sampling Inconclusive image Scintigraphy with 131I-iodomethylnorcholesterol (NP-59) Management Functioning adrenal adenoma: adrenalectomy Bilateral adrenal hyperplasia: spironolactone 44

45. ADRENOCORTICAL CARCINOMA 1–1.5 per million population per year Hormonally functional in 80–100% of patients usually present with steroid overproduction About 50% occur in children <5 years old Management Resection offers the only chance for cure Adjuvant therapy Mitotane: metastatic disease, incompletely excised tumors, and to counteract the hormonal effects from the tumor ?Radiation therapy Prognosis in children depends on age and resectability of the tumor 45

46. ADRENAL MEDULLA PHEOCHROMOCYTOMAS Children : higher incidence of extra-adrenal pheochromocytoma (18–61 % ), multifocal, and familial disease , but a lower incidence of malignancy compared with adults The presentation in adults and children is similar Pheochromocytoma should be considered in any child with hypertension Biochemical Test: plasma free metanephrines or urinary fractionated metanephrines Imaging : MRI, CT, and 123I- or 131I-metaiodobenzylguanidine (MIBG) scanning MRI is recommended in children 46

47. Perioperative management An α-adrenergic antagonist Volume expansion β-Adrenergic blockers Alternatively Calcium channel blockers Operative management Laparascopy Open : >6 cm tumors and invasive tumors Bilateral pheochromocytoma Cortical-sparing adrenalectomies 47

48. References Kundel A, Thompson GB, Richards ML, et al. Pediatric endocrine surgery: a 20-year experience at the Mayo Clinic. J Clin Endocrinol Metab . 2014;99(2):399-406. doi:10.1210/jc.2013-2617 Tuggle CT, Roman SA, Wang TS, et al. Pediatric endocrine surgery: who is operating on our children?. Surgery. 2008;144(6):869-877. doi:10.1016/j.surg.2008.08.033 Holcomb and Ashcraft’s Pediatric Surgery, 7 th edition Coran Pediatric Surgery, 7th edition Daniel J. Ledbetter Endocrine Surgery in Children Uptodate 48

49. 49 Thank YOU