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Paraneoplastic Cushing Syndrome

Paraneoplastic Cushing Syndrome. Wael Batobara. History. 52 y Male Smoker 30 pack Seen in Thoracic Sx Clinic with 1/12 H/O Chest Pain bilateral non pleuritic lower costal 4/10 not related to exertion No Fever ,Wt loss , Cough , Hemoptysis No Leg pain , swelling

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Paraneoplastic Cushing Syndrome

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  1. ParaneoplasticCushing Syndrome Wael Batobara

  2. History • 52 y Male Smoker 30 pack • Seen in Thoracic Sx Clinic with 1/12 H/O Chest Pain bilateral non pleuritic lower costal 4/10 not related to exertion No Fever ,Wt loss , Cough , Hemoptysis No Leg pain , swelling • Trail Of Abx & NSAID  no effect

  3. History • No SOB , Orthopnea , PND • Associated flank pain , No dysuria ,hematuria • PMH : -ve IHD risk factor • Works as Plumber , +ve exposure to asbestos • No Rx , travel

  4. Examination • BP 150/80 HR 80 RR 18 Sat 93% Afebrile • Overweight • N JVP & cardiac exam • Chest N except bilateral tenderness lower ribs • ABD N ?LL edema

  5. Investigations • CBC WBC 16 Neut.13 Hb .Coagulation N • Lytes , BUN & Creat. N • LFT Alk Phos 170 ALT 180 LDH 650 • Cardiac Enzymes & EKG N • CXR & Chest CT

  6. Investigations • Brochoscopy  edema Lt main Endobronchial lesion Sup.LLL • Mediastinoscopy  Multiple LN • BAL & LN Bx Metastatic Small Cell CA • Bone Scan  Diffuse skeletal Mets

  7. The Story is not done Yet !!! Chest Medicine Has Not Been Involved Yet

  8. This Should Have Been Picked Up Earlier R3 Medical Resident

  9. New Complaint • Referred for work up of 1/12 H/O Bilateral Leg swelling Edema extending to Abdominal wall No New respiratory , cardiac symptoms No facial swelling NO decrease urine output , Leg Pain • Trial of Diuretics no improvement

  10. Any Suggestions?!

  11. Sequence Of Events • Patient was admitted to H6 • BP 150/85 • Not In CHF , No Signs of SVC obstruction • Pitting edema upto Ant Abd wall • No Leg Size Difference

  12. Investigation • CBC & Coagulation N • Na 150 Co2 40 Cl ,BUN , Creat N • K 2.2 in spite of >300 meq daily supplement • FBS 8.1 Mg N • ABG PH 7.51 PAO2 65 PCO2 48 HCO3 41 • Metabolic abnormalities persists after stopping the diuretics

  13. Investigation • CT Abd & Pelvis Multiple Mets Liver , spleen , kidneys Adrenal Looks Chubby No IVC obstruction • 2DE  N LV & RV function • 24 Urine Collection  High K

  14. Investigation • Persistent Hypokalemia 2.3 EKG only U wave • Nephrology Consult {Please help it is your game} • Next day while rounding we caught Nephrology Staff  Interesting Case!!!

  15. 24Hour Urine Cortisol 5250!!! Normal < 250

  16. Hospital Course • Overnight Dexamethasone suppression test -ve Serum Cortisol 17501400 • ACTH pending • Oncology Consult  Medical Resident Input Cis platinum & Etoposide • Endocrinology  Ketoconazole

  17. Investigation • Patient tolerated Chemo • Minimal K supplements with decrease CO2 • DM & HTN being treated • Follow up in Cancer Care

  18. ParaneoplasticCushing Syndrome Incidence Is the presentation different from Cushing Dis. Would prognosis differ in SCLC with Cushing Is Chemothherapyis enough ? Other Paraneoplastic syndromes

  19. Incidence • 20-30% of Cushing Synd. is 2ry to ectopic ACTH Lung Ca is the cause in 50% cases • Normal lung tissue secretes minimal amount of POMC proopiomelanocortin which is cleaved into different hormones including ACT { immunoreactive & not necessarily biologically active} • Up to 50% of Lung Ca will have High ACTH though 2-10% will have clinically significant disease

  20. Incidence • 3 Retrospective studies  SCLC had Cushing Synd • 14/840 1.6% Vs 5/157 3.2% Vs 10/126 2.6% • Dx clinical +High serum/urine cortisol • Majority Had extensive disease 60-90% • Cushing synd. Was diagnosed either with Ca Dx or shortly after Cancer Sept 81 & Mar 94 Arch Int Med Mar 93

  21. Clinical Presentation • Less prominent than Cushing Disease  shorter time of exposure to cortisol & the aggressive nature of tumor • Most common  LL edema ,Muscle weakness & moon faces 40-60% • Most common lab finding Hypokalemia ,Met.Alk & Hyperglycemia 100%

  22. Treatment • Majority required additional Rx to control hypercortosilemia • Worse consequence of febrile neutropenia in Patients whom hypercortisolemia was not controlled • Usual doses used to treat Cushing disease is not sufficient in Paraneoplastic Cushing • Rx used : Ketoconazole , Metyrapone ,Aminoglutethimide & Bilateral Adrenalectomy

  23. Prognosis • SCLC with Cushing Synd, have a shorter survival rates than SCLC without the Synd. 4-6 months Vs 8-11 months • 3 reasons  Larger tumor burden  Relative lack of responsiveness to Chemo  Tendency to develop serious infections • Infections  common in patients with higher cortisol levels with different sites & pathogens

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