Junior Resident AHD Endocrine Surgery. Nov. 14, 2012 Dr. Adrienne Melck

Transcription

1 Junior Resident AHD Endocrine Surgery Nov. 14, 2012 Dr. Adrienne Melck

2 Case #1 52-year-old woman GP felt nodule on left thyroid at annual physical examination Assessment?

3 Assessment of Thyroid Nodules History Physical Exam Investigations Labs Imaging

4 Thyroid Nodule Key features on History: Nodule history: change in size over time? Compressive symptoms: dysphagia, dyspnea, hoarseness Thyroid cancer risk factors: family history, head and neck radiation Thyroid dysfunction: symptoms of hyper- or hypothyroidism

5 Thyroid Nodule Key features on Physical Exam: Characteristics of mass: size, mobility Cervical / supraclavicular lymphadenopathy Voice quality Tracheal displacement Substernal component Signs of hypo/hyperthyroidism

6

7 Thyroid Nodule Investigations: Ultrasound TSH Fine needle aspiration biopsy

8 Nuclear Scan? Only indicated if patient is hyperthyroid! hyperfunctioning nodules rarely harbor malignancy one group you can skip FNA: hyperthyroid patient with hot nodule

9 Thyroid Ultrasound must be done in all patients with known or suspected nodules i.e.: all nodules picked up on CT, MRI, PET, physical exam, Xray

10 Which nodules need investigation? All nodules >1-1.5cm <1-1.5cm nodules if: thyroid cancer in 1 st degree relative radiation history thyroid cancer found in previous hemithyroidectomy specimen associated lymphadenopathy FDG-avid on PET (cancer risk 33%) suspicious sonographic features

11 Suspicious ultrasound features: Nodules Taller than wide Hypoechoic Microcalcifications Increased intranodular vascularity Irregular margins **none of these alone or in combination is sensitive/specific enough to determine malignancy

12 Back to case TSH 2.26 (normal) Ultrasound right lobe and isthmus normal, left lobe 2.3cm isoechoic nodule in the lower pole What next?

13 FNAB gold standard for evaluation of thyroid nodules fast, safe, easy most accurate and cost-effective test

14 FNAB 4 Possible Outcomes Malignant 5% Benign 70% Non-diagnostic 10-15% Indeterminate 10-15%

15 Bethesda Criteria (2008) National Cancer Institute Thyroid FNA State of the Science Conference Proposed an expanded classification of FNA cytology 2 further categories suspicious for malignancy FLUS

16 Bethesda Criteria (2008) Category Cancer Risk Benign <1% FLUS 5-10% Indeterminate 20-30% Suspicious 50-75% Malignant 100% Unsatisfactory

17 Benign Nodules Cancer risk: <1% FNA has up to 4% false negative rate repeat U/S in 6-18 months, then longer intervals if size is stable repeat FNA if nodule growing for complex nodules, growth of solid component What s considered significant nodule growth? 50% change in nodule volume 20% increase in 2 dimensions of at least 2mm NO evidence for TSH suppression in developed world Good data that TSH suppression shrinks nodules in iodinedeficient regions

18 Nondiagnostic Nodules Repeat FNA wait ~8-12 weeks to repeat make sure repeat is U/S-guided if non-diagnostic a 2 nd or 3 rd time, consider diagnostic lobectomy

19 FLUS Cancer risk: 5-10% also called atypia of undetermined significance repeat FNA FLUS a 2 nd or 3 rd time diagnostic lobectomy

20 Indeterminate Cancer risk: 20-30% Follicular and Hurthle cell neoplasm Diagnostic lobectomy (Why?) When would you consider upfront TT? Family history/radiation exposure contralateral dominant nodule patient already hypothyroid

21 Suspicious Cancer risk: 50-75% Really mean suspicious for PTC Follicular, Hurthle cell can t be Dx d by FNA FNA should diagnose medullary, anaplastic 2 options Total thyroidectomy Lobectomy with intraoperative frozen section

22 Malignancy Total Thyroidectomy What is a near-total thyroidectomy?

23 Back to case FNAB Hurthle cell neoplasm Recommendation?

24 What are the risks of thyroidectomy? Hematoma Recurrent laryngeal nerve injury Superior laryngeal nerve injury Hypoparathyroidism

25 What are the steps of thyroidectomy? Best place to make incision?

26 Back to case 4 hours after left thyroid lobectomy you re called to ward to assess for hematoma Patient complains of dysphagia and dyspnea O2 sats 82%, obvious large hematoma and increased WOB Management?

27 Indications for Thyroidectomy Malignancy/suspicion of malignancy Cosmesis Compressive symptoms Retrosternal goiter Hyperthyroidism

28 Thyroid Cancer Types Papillary Follicular Medullary Anaplastic Lymphoma Metastases

29 Preop Workup for Cancer Make sure U/S looks at cervical LNs Not easy to see central compartment nodes Looking for lateral compartment nodes PTC most commonly involves level III/IV/VI Level II/V involvement less common Sensitivities of other modalities (CT, MRI, PET) to detect cervical LNs relatively low compared to U/S Consider CT if tumor very large, retrosternal, rapidly growing

30 Suspicious U/S features: LNs Loss of fatty hilum Cystic change Round (rather than oval) Peripheral vascularity Hypoechoic Calcifications **none of these alone or in combination is sensitive/specific enough to determine malignancy FNA any suspicious LNs to confirm Look at cytology but also can measure Tg

31 Other preop workup Laryngoscopy Endoscopy

32 Surgery for DTC >1cm tumors TT or NT Subtotal thyroidectomy is not acceptable

33 Other components of Thyroid Cancer Rx: TSH suppression RAI Prepare with T4 withdrawal or Thyrogen Surveillance with Tg and ultrasound Sometimes EBRT Chemo for anaplastic

34 Medullary Thyroid Cancer Familial syndromes (25%) MEN2, FMTC Preop workup RET/PTC testing, 24 urine cats and mets, calcitonin, CEA, calcium Minimum Operation TT and formal CCND Prophylactic lateral node dissection controversial

35 Case #2 43-year-old woman incidental finding of hypercalcemia on recent blood work Assessment?

36 Assessment of Hypercalcemia History Physical Exam Investigations Labs Imaging

37 Hypercalcemia Symptoms Osteopenia / osteoporosis Nephrolithiasis Mood disturbance, STML, poor concentration, confusion Fatigue Bony/joint aches and pains GERD/PUD Pancreatitis Polyuria / polydipsia Abdominal pain Constipation HTN, dyslipidemia

38 Other History Family History Head and neck irradiation Concomitant thyroid problem Compressive symptoms

39 Hypercalcemia Physical Exam Neck exam

40 Hypercalcemia - Physical Exam

41 Differential for Hypercalcemia? Primary Hyperparathyroidism Malignancy Drugs thiazides, lithium Granulomatous disease TB, Sarcoidosis Lymphoproliferative Diseases Vitamin A/D Toxicity Tertiary Hyperparathyroidism FHH Non-parathyroid endocrinopathies

42 Back to case Complains of: Increasing fatigue Decreasing energy Bony pain Nocturia Forgetfulness Worsening depression/anxiety No H&N radiation exposure

43 Case #2 PMHx: osteopenia, hypertension PSHx: hysterectomy, 2 C sections Meds: HCTZ Allergies: Sulfa SocHx: smokes half a pack/day Fam Hx: how do you screen for MEN-1?

44 How to screen for MEN 1? 6 Questions: Do you have any blood relatives with: Brain tumors/brain surgery? High calcium levels? Neck surgery? Kidney stones? Stomach ulcers? Pancreas tumors/pancreas surgery? Blood work Prolactin, gastrin

45 Familial PH MEN 1 MEN 2a Isolated familial hyperparathyroidism Familial hyperparathyroidism-jaw tumor syndrome

46 Case #2 Physical Exam: non contributory? Investigations

47 Investigations Calcium Parathyroid hormone Phosphate Vitamin D 24 hour urine for calcium and creatinine Bone density

48 Case #2 PTH = 15 ( Normal <6.9)

49 Case #2

50 What is FHH? Familial Hypocalciuric Hypercalcemia autosomal dominant inheritance Defect: CASR calcium-sensing receptor gene High PTH and Calcium (mild) Differentiate from PH with urine studies Urine calcium : creatinine ratio <.01 Only morbidity unnecessary parathyroid surgery

51 Parathyroid Anatomy Embryologic origin? 3 rd pharyngeal pouch inferior paras 4 th pharyngeal pouch superior paras Blood supply? inferior thyroid artery

52

53 1 0 vs. 2 0 vs. 3 0 Hyperparathyroidism Primary Secondary Tertiary PTH high high high Calcium high low high

54 Indications for Parathyroidectomy 2008 NIH Criteria Age <50 T-score < -2.5 GFR <60mL/min Serum calcium >.25mmol/L above normal Unreliable patient

55 Why operate? Decrease incidence of new kidney stones Symptom and QOL improvement 80% Prevent further bone loss / #s Improves VH and stabilizes valvular sclerosis

56 Causes for Primary Hyperparathyroidism Single adenoma 85% 4 gland hyperplasia 10-15% Double adenomata 2-3% Carcinoma 1%

57 Imaging for Localization Gold Standard: bilateral 4-gland exploration Imaging shown to: increase surgical success rate decrease operative time decrease surgical complications affords a more minimally invasive approach

58 Sestamibi

59 Ultrasound

60 Parathyroid Exploration Excision of single adenoma Subtotal Parathyroidectomy (3 ½) Total Parathyroidectomy with autotransplant **for renal/familial hyperparathyroidism, it s always a 4 gland exploration because it s always 4 gland hyperplasia

61 Parathyroid Exploration Intraoperative PTH monitoring

62 Risks of Surgery? Hematoma (1/300) RLN injury (<1%) Superior laryngeal nerve injury (1/20) Recurrent hyperparathyroidism (<1%) Permanent hypoparathyroidism (<1%)

63 Ectopic Parathyroid Glands Inferior glands more likely to be in ectopic location intrathymic / thyrothymic ligament carotid sheath anterior mediastinum intrathyroidal Superior Glands: retroesophageal retropharyngeal intrathyroidal (superior pole)

64 Treatment of Hypercalcemic Crisis? Correct hypovolemia Normal saline boluses Enhance renal excretion of calcium After adequate volume resuscitation, Lasix Inhibit bone resorption bisphosphonates, calcitonin, steroids (not for PH) Treat underlying disorder

65 Adrenal Incidentaloma Clinically inapparent adrenal mass detected incidentally with imaging studies conducted for other reasons Prevalence increases with age rare in patients < 30 yrs 7% of 70 year olds

66 adrenocortical cancer myelolipoma aldosteronoma adrenal hemorrhage adrenal cyst Cushing s syndrome ADRENAL INCIDENTALOMA benign adenoma metastases ganglioneuroma pheochromocytoma

67 First Question: Is it Functioning? Functional or Non-functional? Functional Nonfunctional Conn s Cushing s Pheo Benign Malignant

68 The classic presentation of Conn s is A. hypertension, hypokalemia, metabolic acidosis B. hypertension, hyperkalemia, metabolic alkalosis C. hypertension, hypokalemia, metabolic alkalosis D. hypertension, hyperkalemia, metabolic acidosis

69 The classic presentation of Conn s is A. hypertension, hypokalemia, metabolic acidosis B. hypertension, hyperkalemia, metabolic alkalosis C. hypertension, hypokalemia, metabolic alkalosis D. hypertension, hyperkalemia, metabolic acidosis

70 Conn s Syndrome = Primary Hyperaldosteronism Classic presentation: hypertension, hypokalemia, metabolic alkalosis, fatigue, muscle cramps, polydipsia, polyuria Labs: elevated Aldosterone Renin Ratio

71 Aldosterone

72 Conn s Unilateral or Bilateral? Unilateral disease adrenalectomy Bilateral disease medical therapy (spironolactone) 1 side or both?? CT or MRI adrenal venous sampling (AVS)

73 AVS Technique 1. R adrenal vein 2. L adrenal vein 3. IVC Blood sent from 3 sites for aldosterone and cortisol Positive Test: adrenal vein cortisol 2x IVC cortisol after ACTH aldosterone/cortisol ratio 4x higher on side of adenoma

74 The most common cause of Cushing s syndrome is: A. Cushing s disease B. Exogenous steroid use C. Ectopic ACTH production D. Glucocorticoid-producing adrenal adenoma

75 The most common cause of Cushing s syndrome is: A. Cushing s disease B. Exogenous steroid use C. Ectopic ACTH production D. Glucocorticoid-producing adrenal adenoma

76 patients rarely Cushingoid Classic presentation: moon facies, buffalo hump, purple striae, easy bruising, HTN, glucose intolerance, osteopenia, hirsutism, menstrual changes, truncal obesity, mood changes

77 Cushing s Syndrome Screening diagnostic test: 24 hour urine free cortisol overnight low dose dexamethasone suppression patient takes 1mg dexamethasone 11pm serum cortisol drawn at 8am next morning positive test: 8am cortisol does not suppress

78 Cushing s ~3 possible sources 1. Pituitary source (= Cushing s disease) 70% of cases too much ACTH bilateral adrenal hyperplasia 2. Adrenal source 30% of cases adenoma too much cortisol 3. Ectopic source <1% of cases paraneoplastic syndrome too much ACTH bilateral adrenal hyperplasia

79 Cushing s syndrome confirmed ~ What s the cause? ACTH dependent or independent? ACTH level low adrenal source CT/MRI high pituitary or ectopic source

80 When is adrenalectomy required? ACTH independent unilateral adenoma ACTH dependent uncontrolled Cushing s disease unresectable ectopic ACTH syndrome

81 Which familial syndrome is NOT associated with pheochromocytomas? A. Von-Hippel Lindau B. Multiple Endocrine Neoplasia Type 1 C. Multiple Endocrine Neoplasia Type 2 D. Neurofibromatosis Type I

82 Which familial syndrome is NOT associated with pheochromocytomas? A. Von-Hippel Lindau B. Multiple Endocrine Neoplasia Type 1 C. Multiple Endocrine Neoplasia Type 2 D. Neurofibromatosis Type I

83 Pheochromocytoma tumor of the catecholamine-secreting chromaffin cells of adrenal medulla Classic presentation: flushing, headaches, hypertension, anxiety, palpitations, diaphoresis, weight loss

84

85 Pheochromocytoma - Diagnosis 24 hour urine cats and mets 2-fold increase considered positive 4-fold increase has 100% diagnostic accuracy

86 Pheochromocytoma Rule of 10s? 10% malignant 10% bilateral 10% extraadrenal 10% pediatric 10% normotensive 10% familial

87 Pheochromocytoma Preoperative preparation is everything! 1. Alpha blockade 2. Fluid resuscitation 3. +/- Beta blockade

88 Adrenal Functional Workup 1. Conn s Syndrome plasma aldosterone concentration (PAC) plasma renin activity (PRA) serum potassium 2. Cushing s Syndrome 24-hr urine free cortisol low dose dexamethasone suppression test 3. Pheochromocytoma 24-hr urine catecholamines and metanephrines

89 Is it functioning? ~ Order These 3 Tests 24-hour Urine catecholamines and metanephrines cortisol Low dose dexamethasone suppression test ARR

90 2 nd Question: Is it Malignant? Functional or Non-functional? Functional Nonfunctional Conn s Cushing s Pheo Benign Malignant

91 2 nd Question: Is it Malignant? Consider.. 1. Size 2. Imaging characteristics

92 Size Matters! Adrenocortical carcinoma 2% tumors 4cm 6% tumors 4.1-6cm 25% tumors >6cm anything >6cm needs to come out!!

93 Imaging Characteristics BENIGN Hounsfield units <10 on noncontrast scan smooth borders 50% contrast washout in delayed images homogenous ATYPICAL Hounsfield units >10 irregular borders inadequate washout central necrosis heterogeneous local invasion

94 Benign Imaging

95 Atypical Imaging

96 Adrenocortical Carcinoma Rare! Very poor prognosis <5% 5-year overall survival Best chance for cure en bloc resection

97 Adrenal Metastases adrenalectomy if isolated metastases Melanoma Lung Breast Renal

98 Benign Adenoma Common! up to 30% increase in size by at least 1cm up to 20% become functional

99 When should you biopsy adrenals? ALMOST NEVER!!! cannot diagnose ACC with FNA small risk of seeding needle tract NEVER before ruling out pheochromocytoma Indication: metastatic w/u of known extraadrenal primary cancer

100 Indications for Adrenalectomy Functional unilateral lesion Suspicion of malignancy Any lesion >6cm Any growing lesion

101 Adrenalectomy open laparoscopic** **Faster recovery time, shorter LOS, lower morbidity

102 Right Adrenalectomy

103 Right Adrenalectomy

104 Right Adrenalectomy

105 Left Adrenalectomy

106 Left Adrenalectomy

107 Left Adrenalectomy

108 Resources