Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
Download Ectopic_Hormone_Syndromes
Document related concepts
Transcript
Ectopic Hormone Syndromes Case 1 65 y/o F presents with 20 lb weight loss over last 2 months, new onset hyperglycemia, HTN, and hypokalemia Pt is markedly hyperpigmented and cachectic Chest X-ray Ectopic Cushing’s Syndrome Due to production of ACTH, or rarely CRH, from source other than pituitary/hypothalamus 15% of all Cushing’s Syndrome Tumors can be very aggressive and therefore pt’s present more like cancer than Cushing’s, or can be indolent tumors and present like Cushing’s Tumors express POMC which is then processed to ACTH and MSH Ectopic Cushing’s Make diagnosis by documenting elevated cortisol levels: – 24 urine free cortisol – Low dose and high dose dex suppression – Inferior Petrosal Sinus Sampling Can be hard to detect – Chest/Abd/Pelvis imaging – Octreotide scans Treatment If find tumor, surgically resect If can’t find tumor or if tumor unresectable, can treat medically – Ketoconazole – Metyrapone – Ketamine – Aminoglutethamide – Mitotane Case 2 68 y/o M presents w/ dehydration and mental status changes Labs show Ca=16.5 mg/ dL PTH Related Protein A protein expressed in many cells whose main function is growth and differentiation Occupies PTH receptor to carry out it’s functions However, when make large amounts of it, acts like PTH to: – Increase osteoclast function – Increase conversion of 25 to 1,25 vitamin D leading to increased absorption of calcium through the gut – Increase renal resorption of calcium Tumors that make PTH-rp Renal Cell Squamous cell Bladder Melanoma Breast Prostate Treatment Hydration IV Bisphosphonates Subq calcitonin Treat underlying malignancy Case 3 A 25 y/o M presents with multiple stress fractures, bone pain, and muscle weakness He had a tender mass at the right posterior 9th rib Labs show Phosphate=0.8 mmol/dL Normal calcium Elevated alk phos CXR Bone Scan Oncogenic Osteomalacia Also called Tumor Induced Osteomalacia See renal phosphate wasting and vitamin D abnormalities Get inappropriate phosphate renal wasting due to excess production of fibroblast growth factors (phosphotonins), most common is FGF 23 FGFs inhibit phosphate reabsorption at the proximal tubule, and also inhibit 1 hydroxylation of 25 vit D Oncogenic Osteomalacia Tumors are usually benign and derived from bone or soft tissue Can be very hard to find—look in mouth, sinuses, and extremities Some reports that PET and octreotide scanning were able to localize the tumor Treatment is resection of tumor, but if can’t find treat w/ phosphate replacement and calcitriol Oncogenic Osteomalacia Case 4 65 y/o M presents with hypoglycemia. No hx of DM and no access to DM meds. CT scan looking for pancreatic mass finds a large retroperitoneal mass Insulin levels are Non-islet Cell tumor Usually bulky mesenchymal tumors – – – – – Fibrosarcomas Rhabdomyosarcomas Leiomyosarcomas Mesotheliomas hemangiopericytomas Make up 50% Hepatomas, carcinoid tumors, and adrenocortical carcinomas account for 25% See suppressed insulin, GH, and IGF binding proteins Why hypoglycemia? Tumors secrete increased amounts of IGFII There is altered binding increasing it’s bioavailability and effect More IGF-II goes to insulin receptors in tissues leading to: – Increased clearance of glucose from circulation to muscle – Decreased hepatic gluconeogenesis – Suppression of lipolysis Treatment Treat underlying malignancy GH, glucagon, glucocorticoids, or somatostatin have been effective in individual patients with unresectable tumors Case 5 An 81 y/o F presents w/ tachycardia, weight loss, tremors, and sweats. TSH<0.01 FT4=5.2 Neck exam is unremarkable Denies use of thyroid hormone Imaging I131 scan of neck I131 scan of pelvis Pelvic CT Struma Ovarii An ovarian tumor containing thyroid tissue as the predominant cell type Typically occur as part of a teratoma but may occasionally be encountered with serous or mucinous cystadenomas Malignant transformation is rare Most strumal tissue is not functionally active, and cases associated with thyrotoxicosis can be due to autoimmune stimulation of the normal thyroid gland Struma Ovarii Between 0.8-3% of teratomas contain functional thyroid tissue or thyroid tissue occupying most of the mass. They are thus classified as a struma ovarii. Approximately 15% of teratomas have a small, nonsignificant focus of thyroid tissue. The fifth and sixth decades are the ages of peak frequency Struma ovarii rarely occurs before puberty Case 6 A 25 y/o M presents with gynecomastia and symptoms of hyperthyroidism Has slight goiter on exam TSH<0.01, FT4-1.7 Testicular Ultrasound Germ Cell Tumor Can secrete HCG which leads to excess production of testosterone and estrogen Can also directly occupy TSH receptors causing hyperthyroidism similar to pregnancy Important to measure HCG in all men w/ gynecomastia Can also be secreted from hepatic, biliary, gastric, bladder, reanal, and pancreatic, but often not bioligically active
