an adrenal incidentaloma ( ai ) is defined as an adrenal lesion that is discovered when a radiological study is performed for indications other than suspected adrenal disease .
all patients with an ai should be evaluated for hypercortisolism , pheochromocytoma and ( if hypertensive ) hyperaldosteronism .
a combination of pheochromocytoma and cushing 's syndrome due to pathology in same adrenal gland is extremely rare .
there are few case reports suggesting the different etiologies for this association , namely , pheochromocytoma secreting adrenocorticotropin hormone ( acth ) or its precursors , corticomedullary mixed tumors and focal adrenocortical hyperplasia . here
, we report a case of ai with the clinical and biochemical evidence of both pheochromocytoma and sub - clinical cushing 's syndrome .
a 42-year - old female patient was admitted under department of gastroenterology with abdominal pain for 1 week .
routine biochemical evaluation including complete blood count , renal function tests ( including serum electrolytes ) and liver function tests were normal .
abdominal computed tomography ( ct ) revealed a right supra - renal mass ( 40 mm  39 mm  40 mm ) , which was heterogeneously enhancing with multiple cystic areas and a homogenously enhancing solid component within the anterior limb of the right adrenal gland .
as per the ct findings , right - sided adrenal adenoma with the possibility of pheochromocytoma was suspected .
computed tomography image of abdomen showing 4.0 cm  3.9 cm  4.0 cm heterogeneously enhancing right suprarenal mass ( a ) coronal section and ( b ) sagittal section on endocrine assessment , she had a complaint of intermittent palpitations and headache for 4 years .
there was no history of fractures , muscle weakness , easy bruisability , menstrual irregularity and hirsutism .
she was on treatment for hypertension and diabetes for 4 years with an adequate control .
she was not on any exogenous steroids . on examination , her height was 155 cm , weight was 60 kg , body mass index was 24.97 kg / m , blood pressure was 130/80 mmhg and pulse rate was 82/min .
absence of abdominal striae , moon facies and hyperpigmentation her endocrine investigations revealed raised 24-h urinary metanephrines and nor - metanephrines with value of 3268 g/24-h ( normal range 52 - 341 ) and 4591 g/24-h ( normal range 88 - 444 ) , respectively ; raised 8 a.m. serum cortisol with value of 80 g / dl ( normal range 5 - 25 ) ; and normal plasma aldosterone level of 32.84 pg / ml ( normal range 34 - 273 ) .
she had non - suppressible serum cortisol of 56.91 g / dl and 25.86 g / dl after 1 mg - overnight dexamethasone suppression test ( dst ) and 48 h - 2 mg / day dst , respectively .
after pre - operative management with alpha and beta blockers , she underwent laparoscopic right adrenalectomy . during the post - operative period
, she was watched for hypotension and hypoglycemia , but the entire course remained uneventful . repeat 8 a.m. serum cortisol on 1 post - operative day was 11.20 g / dl and she was started on intravenous hydrocortisone 50 mg tid . the excised mass measured 4 cm  3.5 cm  3 cm with weight of 12 g. the pathologist concluded that the lesion was pheochromocytoma staining positive for chromogranin and synaptophysin with hyperplastic adrenal cortex [ figure 2 ] .
two weeks later , she was re - evaluated and her 24-h urinary fractionated metanephrines came out to be in normal range and fasting 8 a.m. serum cortisol level was 10 g / dl ( prednisolone stopped 2-day prior to testing ) .
prednisolone dose was tapered slowly over 2-month and presently ; she is not on any medication and doing well .
this patient represents a rare case of ai with evidence of both pheochromocytoma and sub - clinical cushing 's syndrome .
patient 's condition improved with adrenalectomy of involved side and there was immediate fall in serum cortisol levels reflecting that the same adrenal gland was the source of excess cortisol .
pheochromocytoma may secrete various substances such as somatostatin , enkephalines , calcitonin , vasoactive intenstinal peptide , neuropeptide - y , renin , acth , parathyroid hormone , erythropoietin , adrenomedullin and dihydroxyphenylalanine ( dopa ) in addition to catecholamines .
there are about thirty cases reported in the literature of cushing 's syndrome due to acth - secreting pheochromocytoma . in some of these cases
, there was pre - dominance of hyperpigmentation , hypokalemic alkalosis and evidence of contralateral adrenal gland hyperplasia on imaging , but these features were absent in our case .
mixed tumors involving the cortical and medullary components of the adrenal gland are quite rare . on histochemical staining , patient 's tumor was not a corticomedullary mixed tumor , but a pure pheochromocytoma .
there was prominent cortex showing hyperplasia on staining with no evidence of neoplastic changes [ figure 3 ] .
( a ) h and e stain ( low power ) showing hyperplastic adrenal parenchyma with pheochromocytoma cells ( b ) high power view of tumor cells , arranged in nests , round to polygonal with scanty cytoplasm , vesicular nucleus and prominent nucleoli ( c and d ) low and high power view of immunohistochemical staining showing tumor cells stained positive for chromogranin and absence of stain in the adrenal cortex in this case , we speculate that adrenocortical hyperplasia , which was possibly because of paracrine stimuli from the pheochromocytoma , was the source of autonomous cortisol production . although , the possibility of acth - secreting pheochromocytoma can not be completely ruled out as we do not have plasma acth levels .
furthermore , we have not observed until now such high levels of serum cortisol ( 8 a.m. value of 80 g / dl ) , in cases of sub - clinical cushing 's syndrome without any cushignoid features .
the clinical aspects of this case suggest the importance of proper pre - operative recognition of the dual hormone secretion from adrenal mass , which , if goes unnoticed could lead to adrenal or hypertensive crisis in perioperative period . hence , to conclude , we think all patients with adrenal mass should undergo evaluation based on the guidelines for management of ai .
 adrenal incidentalomas ( ais ) are a cluster of different pathologies , but ais with dual functional aspects are very rare . 
 we report a case of ai with the evidence of both pheochromocytoma and sub - clinical cushing 's syndrome . a 42-year - old female patient presented with the history of abdominal pain . 
 abdominal computed tomography revealed right adrenal mass suggestive of pheochromocytoma . on endocrine evaluation , she admitted history of intermittent headache and palpitations for 4 years and was on treatment for hypertension and diabetes . 
 there were no signs and symptoms suggestive of cushing 's syndrome . 
 the laboratory data demonstrated 10 times raised 24-h urinary fractionated metanephrines with non - suppressible serum cortisol after 2-day low - dose dexamethasone suppression test . 
 she underwent right - sided adrenalectomy with subsequent resolution of both pheochromocytoma and hypercortisolism . 
 patient was discharged in good clinical condition . 
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