Further Supporting Evidence to Q4 (Part 3) - Dr MJ Devlin
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  • 1. * Michael-John Devlin (CT1)
  • 2. * * Discuss the individual presentation, investigation, treatment and prognosis of the following endocrine cancers: * Thyroid * Adrenal * Neuroblastoma * Also highlight MEN and other syndromes associated with endocrine cancers
  • 3. * * 1% of all UK malignancies and 0.5% of all cancer deaths * More common in women 2.5:1 * Preponderance in 4th and 5th decades with median age of presentation 47 years * Relatives of patients with thyroid cancer have a x10 increased risk
  • 4. * Differentiated * Papillary and Follicular * Only well established RF is previous H&N irradiation * Few rare inherited syndromes like FAP and Gardners * Undifferentiated * Anaplastic * Typically older population with 20% having previous differentiated thyroid cancer * Up to 50% have history of multinodular goitre * Medullary * 75% sporadic * If more than one case in a family think of familial disease eg MEN 2a and 2b or isolated familial medually thyroid cancer. * Others * Lymphoma * Metastases from breast or colon cancer
  • 5. * * Medullary carcinoma * Prophylactic thyroidectomy at early age often appropriate if known carrier of predisposing gene (MEN 2) * Calcitonin problematic * Molecular analysis for germline RET
  • 6. * * Differentiated * Incidental microcarcinomas or painless lump * Clinical regional lymph node involvement in 50% children * Disseminated disease 2-10% * Clinically euthyroid * Undifferentiated * Rapidly enlarging neck mass which may be painful * Confluent bilateral lymphadenopathy * 90% regional or distant spread at diagnosis * Medullary * Painless neck lump * 50% cervical lymph nodes
  • 7. * * FBP, LFT and U&E * TFT * Calcitonin * HR thyroid ultrasonography with fine needle aspiration * ? CT/MRI neck * Staging with CT chest/liver and skeletal survey
  • 8. * * Differentiated * Based on TNM staging and takes into account age of patient * Undifferentiated * Very poor prognosis and all effectively stage IV * Medullary * Staging based on tumour size, local invasion, nodal disease and presence of metastasis. Unlike differentiated classification age is not a factor.
  • 9. * * Surgery * Radio-Iodine * Radiotherapy * Chemotherapy
  • 10. * * Differentiated * Unilateral total lobectomy * Total thyroidectomy * More extensive resections * Complications: hypoparathyroidism, recurrent laryngeal nerve injury and superior laryngeal nerve injury * Undifferentiated * Usually inoperable at presentation * Medullary * Preoperative screening for phaechromocytoma and hyperparathyroidism * Total thyroidectomy, central and ipsilateral neck node dissection
  • 11. * * 131I is used for ablation of residual thyroid tissue, assessment of diseases recurrence and treatment of residual or recurrent disease * Reduced risk of local recurrence by 60% * Prolongs disease free survival * Increases sensitivity and specificity of subsequent screening * Preparation for 131I includes: * Total thyroidectomy one month previous * No thyroid hormone replacement * Low iodine diet and avoidance of iodine medications * Confirm biochemically hypothyroid * Men should be offered sperm storage
  • 12. * Post ablation diagnostic 131I scans preformed and serum thyroglobulin should become undetectable. * Replacement of thyroid hormone with lifelong suppression of TSH
  • 13. * * Differentiated * * * * * Those tumours that don’t concentrate radio-iodine, if the primary tumour is large (pT4), extracapsular spread or lymph node involvement or other poor prognostic features Typically 60 Gy in 30 fractions in two phases with shielding in second phase to avoid exceeding spinal cord tolerance. Also Palliative role Undifferentiated * Most commonly palliative intent for local control of inoperable tumours or symptomatic mets Medullary * * Post-operatively for macroscopic remnant to maximise local control. Can be used pre-operatively to make an inoperable tumour operable.
  • 14. * * Very limited role * Only if other modalities are no longer appropriate and patient remains fit * Response rates poor (<30%), incomplete and of short duration * Doxorubicin
  • 15. * * Differenatied * Most recurrance within 5 years * Regular examination, serum T4, TSH and TG on each visit. Increased TG in presence of supressed TSH requires investigation * Medullary * Clinical examination, serum calcitonin levels and screening for familial diseases
  • 16. * * Differentiated * >90% 10 year survival (98% Papillary, 92% follicular) * Undifferentiated * Aggressive cancer with very poor prognosis * Time from first symptoms to death is 3-7 months * 5-14% 5 year survival * Medullary * No effective treatment for advanced disease but may live for years despite high metastatic load (median survival of stage IV disease is 5 years)
  • 17. * * Adrenocortical * 1 per 106 population * Unknown aetiology, rare familial cases * Bimodal age distribution; peaks before age of 5 and then in 4th-5th decades * 60% non-functioning, 40% secreting oestrogens, testosterone and/or aldosterone * Medullary * 0.6 per 106 population * 10% familial including MEN2 and occasionally VHL * Commonest is phaeochromocytoma which exhibits the “Rule of 10”
  • 18. * * Adrenocortical * Incidental * Pressure symtoms * Virilization, Cushings, feminization and hypertension * Medullary * Incidental * During screening of familial syndromes * Classical presentation of headache, sweating, tachycardia/palpitations
  • 19. * * Family history * FBP, U&E, LFT * Plasma catecholamines * Plasma renin and aldosterone activity ratio * Urinary VMA and catecholamines * Chromogranin assays * Serum and urinary cortisols * Blood oestrogen and testosterone * CXR * USS, CT, MRI abdomen to assess for potential metastatic disease * USS thyroid (MEN) * Selenocholestrol imagine – cortical tumour
  • 20. * * Surgery * Only treatment to achieve cure n benign disease or patients with localised malignant disease * Open or laprascopic * Preoperative need to correct electrolyte abnormalities and control hypertension * Non-surgical
  • 21. * * Adrenocortical tumours * Mitotane * Metyraone, Aminoglutethamide and ketoconazole * Chemotherapy * Radiotherapy * Medullary * Anti-hypertensive * 131I-MIBG * Chemotherapy (dacarbazine, vincristine and cyclophosphamide) * Radiotherapy
  • 22. * * Adrenocortical * Almost all benign are cured with surgery * Poor prognosis with malignant disease, untreated survival being 3-9 months improving to 14-36 months with surgery * Medullary * Surgery is not always curative even in benign disease * Long term monitoring required * 5 year survival for malignant phaeochromocytoma is <50% however some patients may live for many years without significant symptoms.
  • 23. * * Commonest extracranial solid tumour in childhood * Peak at 1-3 years * Arise in sympathetic nervous tissue (60% adrenal) * Pathology ranges from undifferentiated, small, round cell and highly differentiated ganglioneuroblastoma * Investigated with abdominal USS, CT/MRI Chest and abdomen, MRI spine, urinary catecholamines, bloods including serum LDH and serum neurone-specific enolase (NSE)
  • 24. * * Depends on stage and presence of certain genetic features that may indicate a less good prognosis (n-myc oncogene) * Watch and wait * Surgical clearance * Neoadjuvant Chemotherapy * Radiotherapy
  • 25. * * Depends on stage, genetics and age of diagnosis. * Children <1 year have good prognosis even in presence of widely disseminated disease (85% overall survival for stage 4S) * Children >5 have an overall survival of <40% with neuroblastoma of any age
  • 26. * * Syndromes with multiple benign or malignant endocrine neoplasms * MEN-1 * Menin gene on C11 * Parathyroid, Pituitary and Pancreas * MEN-2a * Ret gene on C10 * Parathyroid, Phaeochromocytoma, Medullary Thyroid * MEN-2b * Ret gene on C10 * Parathyroid, Phaeochromocytoma, Medullary Thyroid, Marfanoid and mucosal neuromas
  • 27. * * Carney Complex * Gardners Syndrome * Beckwith-Wiedemann Syndrome * Li–Fraumeni syndrome * Cowen Syndrome * Tuberous sclerosis