Cushings syndrome

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Cushings syndrome : 

Cushings syndrome Murali A

introduction : 

introduction HARVEY CUSHINGS-1932 Central obesity,glucose intolerance,HT, plethora,hirsutism,osteoporosis, nephrolithiasis,menstrual irregularity, muscle weakness &emotional liability…….Pituitary Basophilism. Today …refers to a complex of symptoms & signs resulting from hypersecretion of cortisol, regardless of etiology M:F=1:8,in pituitary dependant-M:F=1:3 Mc endogenous cause-ACTH producing pit tumour

etiology : 

etiology ACTH dep cushings d/s 70 % ectopic ACTH 10% ectopic CRH 1% ACTH indep adrenal adenoma[10-15 %]&Ca[5- 10 %] adrenal hyperplasia 5 % OTHERS pseudo cushings iatrogenic

c/f : 

c/f General Integumentary CVS Musculoskeletal Neuropsychiatric Metabolic Renal gonadal

diagnosis : 

diagnosis Step 1—demonstration of cortisol excess &loss of diurnal variation 24 hr cortisol free level-sensitivity 95- 100%,specificity 98% >100microgm/day in 2 independent collections-diagnostic Overnight DST- S cortisol <3-5microgm/dl [normally],some endocrinologists-<1.8mi g

Step 1 …. : 

Step 1 …. Late night salivary cortisol-[11 pm] >6 nmol/L diagnostic,3-6 nmol/L indeterminate.[high sensitivity] CRH/DST 0.5mg dexamethasoneQ6H x 2 days + 8 am CRH bolus,15 min cortisol >1.4 microgm/dl is diagnostic

Inv…. : 

Inv…. Step 2 –determining the cause-ACTH dep or not plasma ACTH level –[nl 10-60 pg/ml] suppression to <5 pg/ml is diagnostic of adrenocortical neoplasms High dose DST-2 gmQ6H x 2 days nl & pituitary ACTH neoplasm pt respond-reduction of urinary free cortisol &17-OH steroids<50% of basal value urinary cortisol is suppresed to <90% from baseline in 60 – 70% of pit adenoma

Inv…. : 

Inv…. CRH test 1 microgm/kg,iv over 30 sec,plasma ACTH & S cortisol at 15,30,60 min pl ACTH rise of >35-50 % in pit tumours blunted peak ACTH response in adrenal cushings No response in ectopic cushing

Inv… : 

Inv… Metyrapone test-700 mg Q4H x 24 hrs,increase in plasma ACTH ,urinary corticosteroids confirm cushings disease B/L Inf petrosal sinus sampling.. 1 micro gm CRH iv,a ratio of inf petrosal sinus to peripheral blood ACTH of 2 at basal &3 after CRH administration -100% sensitive & specific for pit adenoma

Slide 10: 

Confirm diagnosis Overnight DST 24 HR uri cortisol 11 pm salivary cortisol Source of cortisol Plasma ACTH High dose DST& uri cortisol ACTH,sup- ^ACTH,+ve ^ACTH,-ve equivocal Adrenal source Pit source ectopic B/L PETROSAL SAMPLING ACTH GRADIANT NO YES CT SCAN ADRENAL

RADIOLOGY : 

RADIOLOGY CT & MRI of abd identifies adrenal tumours with 95% sensitivity NP-59[I131-6 b iodomethyl noriodo cholestrol] schintigraphy-distinguish adenoma from hyperplasia.cold nodules-malignant. Thin section CT -22% sensitive at identifying pit tumours Gado enhanced MRI-33-67 % sensitivity

Inv… : 

Inv… In suspected ectopic tumour ,CT or MRI of chest & ant mediastinum performed 1st,followed by imaging of neck,abd &pelvis if initial study is neg. Those suspecting ectopic ACTH, S calcitonin &urine catecholamines to be done

treatment : 

treatment Cushings d/s-transsphenoidal resection,80% success. Pituitary irradiation ,Steroetactic radiosx for persistent &recurrent d/s Those who fail-adrenal inhibitors like ketoconazole,metryapone,aminoglutethemide,b/l adrenalectomy ?b/l lap adrenalectomy Nelsons syn

Rx……. : 

Rx……. Ectopic ACTH tumours-removal of primary Debulkingof unresectable/recurrent lesions +/- b/l adrenaletomy provides palliation in some Medical therapy in inoperable conditions-ketoconazole,aminoglutethemide,metryapone,mitotane ?b/l adrenalectomy in uncontrolled hypercortisolism or unsuccessfull localisation Those rx with ketoconazole &metryapone should be given 100mg hydrocortisone on induction

Rx,….. : 

Rx,….. Adrenal adenoma-<6 cm lap adrenalectomy,>6 cm –open SX Perioperative & post op steroids.parenteral hydrocortisone 100 mg Q6H for 24 hr then gradually reduced. Replacement therapy with prednisolone 5 mg in morning and 2.5 mg in the evening for 6-18 months

Rx…. : 

Rx…. Primary adrenal hyperplasia- Sx-total b/l adrenalectomy Adrenocortical carcinoma –aggressive surgical resection,though cure is infrequent Preclinical or subclinical cushings-in 5 -20 % incidentally detected adrenal masses adrenalectomy in <20 yrs,presence of diseaserelatedHT,obesity,DM,osteoporosis.^plasma ACTH,^ urinary free cortisol

Rx…. : 

Rx…. Cushings in children –girls 3 times more,mc is adrenal neoplasms in <7 yr [70%], Adrenalectomy in adreno cortical neoplasms,trans sphenoidal resection in cushings d/s. Ectopic ACTH production rare.

Adrenocaortical carcinoma : 

Adrenocaortical carcinoma 0.2 % of all cancers,bimodal-1st & 5th decade,functioning tmr mc in female & non functional in males. 50 % non functional,30 % secrete cortisol, Rapid onset cushings syn 92 % >6cm. CT-heterogeneity,irregular,hge,LN,liver mets Moderately bright T2 signal intensity[adrenal mass:liver=1.2 :2.8] Once tumour diagnosed,CT chest & pelvis

Adre….ca : 

Adre….ca T1 -<5 Cm without local inv T2 ->5 cm without local inv T3 –any size with local inv,no adj organ inv T4 –any size with adj organ inv N0 –no regional nodes N1 – regional nodes + M1 –distant mets

Rx adr..ca : 

Rx adr..ca Most imp predictor of survival is adequacy of resection With complete resection 5 yr survival-32 to 48 %,with incomplete resection median survival 1 yr Open adrenalectomy,excision of tumour en bloc,with any contiguously involved LN or organs like diaphraghm,pancreas,liver,IVC. Ext to IVC is not a CI.

Adre..ca : 

Adre..ca Mitotane DOC in advanced cases,30 % response. Surgical debulking is recommended for isolated,recurrent d/s..prolong survival Systemic CT-ETOPOSIDE,CISPLATIN ,DOXORUBICIN,…PACLITAXEL SURAMIN..a growth factor inhibitor has shown minimal partial response Ext RT for bone mets Ketoconazole,aminoglutethemide, metryapone for controlling hyper cortisolism