endocrine cancer/ diseases – Flashcards
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pituitary adenoma
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benign -anterior pituitary funcional (hormones) or non functional nonfunctional - bitemporal hemianopsia, hypopituitarism, headache
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prolactinoma
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females: galactorrhea and amenorrhea males: decreased libido tx: dopamine agonist to suppress prolactin (bromocriptine)
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growth hormone cell adenoma
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children: gigantism adults: acromegaly -secondary diabetes mellitus tx: somatostatin analogue (octreotide)
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hypopituitary
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dec ant pituitary hormones (FSH, LH, ACTH, TSH, prolactin, GH) causes: 1) pituitary adenoma 2) sheehan syndrome (infarction during parturition) (poor lactation, loss of pubic hair, fatigue) 3) empty sella syndrome - congenital defect (herniation of arachnoid)
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T1DM
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insulin defficiency -> hyperglycemia -autoimmune destruction of T lymphocytes (inflam of islets) -HLA-DR3 and DR4 symp: high serum glucose, weight loss, low muscle mass, polyphagia 3 P's (polyuria, polydipsia, glycosuria)
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diabetic ketoacidosis
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excessive serum ketones -arrises with stress, epinephrine stimulates glucagon secretion -> inc lipolysis, gluconeogenesis, glycogenolysis -causes increase ffa -> liver converts to ketone bodies symp: kussmaul respirations (trying to blow off metabolic acidosis), dehydration, naus/vom, fruity breath smell, mental changes
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T2DM
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-end organ insulin resistance (Decreased number of insulin rec) -early on insulin levels inc -> Beta cell exhaustion -> amyloid deposits random glucose >200 fasting glucose > 126
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central DI
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ADH deficiency symp: polyuria, polydipsia, hypernatremia, high serum osmolality, low urine osmolality -water deprivation test does NOT increase urine osmolality tx: desmopressin (ADH analog)
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nephrogenic DI
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impaired renal response to ADH symp: polyuria, polydipsia, hypernatremia, high serum osmolality, low urine osmolality -water deprivation test does NOT increase urine osmolality DOES NOT respond to desmopressin
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consequences of diabetes
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1) nonenzymatic glycosylation of vascular BM (atherosclerosis) large/med arteries ->cardio, peripheral vascular disease (hyaline arteriosclerosis) small arteries -> glomerulosclerosis, nephrotic syndrome (kimmelstiel-wilson nodules) 2) osmotic damage -glucose enters schwann cells -> aldose reductase converts glucose to sorbitol (peripheral neuropathy, blindness, impotence, cataracts)
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pancreatic endocrine neoplasms
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1) insulinomas - episodic hypoglycemia w/ mental status change (inc insulin and c-peptide) 2) gastrinoma - zollinger ellison - peptic ulcers 3) somatostatinoma- achlorhydria, cholelithiasis w/ steatorrhea 4) VIPomas -watery diarrhea, hypokalemia, achlorhydria
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SIADH
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-excessive ADH (ectopic production -small cell cancer) -retention of water symp: hyponatremia, low serum osmolality -mental status change and seizure (hyponatremia -> neuronal swelling -> cerebral edema) tx: water restriction, demeclocycline
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hyperthyroid
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-increase basal metabolic rate -increase sympathetic nervous system activity ex: Grave's disease, multinodular goiter
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Grave's
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autoAb (IgG) to TSH receptor -> inc synthesis and release of thyroid hormone women 20-40 -thyroid hyperplasia, exophthalmos, -pretibial myxedema -irregular follicles, scalped colloid, chronic inflam lab: inc total and free T4, dec TSH, hypocholesterolemia, inc serum glucose
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multinodular goiter
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enlarged gland w/ multiple nodules -due to iodine deficiency -usually nontoxic but may become TSH independent
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cretinism
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hypothyroid (neonates and infants) -mental retardation, short stature, skeletal abnormalities, coarse facial features, enlarged tongue, umbilical hernia cause: maternal hypothyroid, thyroid agenesis, dyshormonogenetic goiter, iodine deficiency
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myxedema
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hypothyroid (children or adults) -decreased basal metabolic rate and symp nervous system symp: accumulation of glycosaminoglycans in skin and soft tissue, results in deepening of voice and large tongue cause: iodine def, hashimoto's
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Hashimoto
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autoimmune destruction of thyroid (HLA-DR5) -initially can be hyperthyroid and then progresses to hypo -inflam w/ germinal centers and Hurthle cells labs: dec T4 and inc TSH
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De Quervain (subacute granulomatous thyroiditis)
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-thyroiditis following a viral infection TENDER thyroid (self-limiting)
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Riedel fibrosing thyroiditis
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chronic inflam w/ extensive fibrosis HARD "as wood" nontender thyroid
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131 I radioactive uptake
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increased uptake: "hot nodule" - graves or nodular decreased uptake: "cold nodule" - adenoma and carcinoma
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follicular adenoma
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benign -fibrous capsule -usually nonfunctional
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papillary carcinoma
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risk: radiation exposure, RET, BRAF -most common -orphan annie eye nuclei -nuclear grooves -psammoma bodies -often spreads to cervical nodes but good prognosis
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follicular carcinoma
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-malignant follicles surrounded by fibrous capsule w/ invasion through capsule -hematogenous metastasis
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medullary carcinoma
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risk: MEN 2A and 2B (RET oncogene) -proliferation of parafollicular C cells (neuroendocrine cells - secrete calcitonin) -hypocalcemia (calcitonin inc renal excretion of Ca) -calcitonin deposits in tumor as AMYLOID -hematogenous spread
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anaplastic carcinoma
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-elderly -invades locally -> dysphagia or respiratory compromise -undifferentiated poor prognosis
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thyroglossal duct cyst
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remnant of thyroid tissue along migration path from base of tongue
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lingual thyroid
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persistence of thyroid at base of tongue -mass at base of tongue
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parathyroid adenoma
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benign primary hyperparathyroidism -asymptomatic hypercalcemia -can have increased PTH and hypercalcemia problems 1) nephrolithiasis 2) nephrocalcinosis (calcification of renal tubules) 3) CNS disturbance 4) constipation, peptic ulcer, acute pancreatitis 5) osteitis fibrosa cystica (resorption of bone) inc PTH, Ca, urinary cAMP and alkaline phosphatase dec phosphate
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hypoparathyroid
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low PTH causes: autoimmune damage, surgical excision, DiGeorge syndrome symp: low serum Ca -numbness and tingling -muscle spasm (trousseau or chvostek sign)
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lymphoma
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assoiciated with hashimoto thyroiditis
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Neuroblastoma
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-adrenal tumor of CHILDREN - N-myc -from neural crest cells --> occur anywhere along sympathetic chain symp: -abdominal distention, firm mass that can cross the midline -opsoclonus-myoclonus dx: -HVA and VMA in urine -homer-wright rosettes -bombesin and enolase +
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pheochromocytoma
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-adrenal medulla tumor of ADULTS -NF1, MEN 2A, 2B, VHL -from chromaffin cells from neural crest symp: episodic HTN (Ep, Nep, Dopamine secretion) -5 Ps (pressure, pain, perspiration, palpitations, pallor) dx: inc catecholamins and metanephrines in urine (VMA) tx: a-antag (phenoxybenzamine) follow with b blocker
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insulinoma
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pancreatic B cell tumor -overproduction of insulin --> hypoglycemia -increased C-peptide level whipple triad - low blood glucose, hypoglycemia (syncope, diplopia), resolution of symptoms after normalization of glucose tx: surgery
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carcinoid syndrome
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-neuroendocrine -metastatic small bowel tumor to liver (secrete 5HT) symp: -diarrhea, flushing, wheezing, right side valve disease -rule of 1/3 -uniform shape and size w/ eosinophilic cyto and dense core granules in cytoplasm -rosettes dx: inc 5-HIAA, niacin deficiency (pellagra)
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zollinger-ellison
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gastrin secreting pancreatic or duodenum tumor MEN-1 symp: -recurrent ulcers in duodenum/jejunum (refractory to therapy and beyond duodenal bulb (H pylori before)), abdominal pain, diarrhea dx: + secretin test (gastrin levels remain elevated)
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MEN 1
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parathyroid, pituitary (prolactin, GH), pancreatic (zollinger, insulinoma, VIPoma, glucagonomas)
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MEN 2A
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RET parathyroid hyperplasia, pheochromocytoma medullary thyroid carcinoma
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MEN 2B
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RET pheochromocytoma medullary thyroid carcinoma, ganglioneuromatosis -marfan habitus
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adrenocortical adenoma
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cause primary hyperaldosteronism (conn syndrome) -well defined yellowish tumor of the adrenal cortex symp: renal Na retention and loos of K and H --> HTN, hypokalemia, metabolic alkalosis (paresthesia and muscle weakness)
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paraganglioma
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clusters of neuroendocrine cells -extra adrenal paragangliomas occur in head and neck nested zellballen pattern, benign but recur -may be familial (MEN) adrenal: pheochromocytoma (symp) nonadrenal: zucherhandle (symp) carotid body (parasymp)
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thymoma
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primary mediastinum tumor -epithelial tumor w/ lymphocytes (T cells) -round or spindle cells -anterior mediastinal mass - paraneoplastic MG ** (AcH rec autoAb)
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hyperaldosteronism
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HTN (inc Na expands plasma volume), hypokalemia, metabolic alkalosis -no edema due to aldosterone escape cause primary: adrenal hyperplasia, adrenal adenoma (conn's) cause secondary: activation of renin-angio system
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liddle syndrome
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decreased degradation of Na channels in collecting tubules -HTN, hypokalemia, metabolic alkalosis -low aldosterone and renin
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cushing
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excess cortisol -muscle weakness, thin extremeties, moon facies, buffalo hump, truncal obesity, abdominal striae, HTN, high insulin, osteoporosis, dx: 24 hr urine cortisol, late night salivary cortisol (both increased) -late night dexamethasone suppression test (fails to suppress cortisol)
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21-hydroxylase deficiency
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-aldosterone and cortisol decreased -androgens increased symp: hyponatremia, hyperkalemia, hypovolemia, salt wasting and life threatening hypotension -female neonates - genital ambiguity
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11-hydroxylase deficiency
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-cortisol decreased -weak mineralcorticoids increased -increased androgens -low aldosterone and renin
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17-hydroxylase deficiency
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-decreased cortisol and androgens -weak mineralcorticoids are increased -> HTN -renin and aldosterone are low -primary amenorrhea - lack of pubic hair in females or pseudohermaphroditism in males
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adrenal insufficiency
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1) waterhouse friderichsen - hemorrhagic necrosis (DIC with Neisseria Meningitidis) 2) chronic (Addison disease) - autoimmune, TB, metastatic carcinoma destruction of adrenal glands -hyperpigmentation and hyperkalemia (primary)
