ACTH-secreting Pheochromocytoma Research Articles

Preoperative management of patients with ectopic Cushing's syndrome caused by ACTH-secreting pheochromocytoma: a case series and review of the literature.

ACTH-secreting pheochromocytoma is a rare cause of ectopic Cushing's syndrome, posing a clinical challenge for the severity of its clinical presentation, the difficulty in the prevention and the management of surgical complications. Sparse data are currently available about the optimal preoperative management of the severe symptoms due to both hypercortisolism and catecholamine excess, especially regarding the role and timing of medical therapies. We present a series of three patients with ACTH-secreting pheochromocytoma. A brief review of the available literature evidence on the preoperative management of this rare clinical condition is also conducted. Patients with ACTH-secreting pheochromocytoma show peculiarities as compared to other forms of ACTH-dependent Cushing's syndrome, in terms of clinical presentation, preoperative management, and peri- and post-surgical short-term outcome. Pheochromocytoma should be ruled out in patient with ectopic CS of unknown origin because of the high anesthesiologic risk of proceeding to surgery with an undiagnosed pheochromocytoma. Proper preoperative recognition of complications of both hypercortisolism and catecholamines excess is the key to prevent the morbidity and mortality of an ACTH-producing pheochromocytoma. In these patients the absolute priority is to control excessive cortisol secretion since the rapid correction of the hypercortisolism is the most effective treatment of all the related comorbidities and it is mandatory to prevent severe complications during surgery, opting if necessary for a "block-and-replace" regimen. Our additional cases and this literature review could provide a better understanding of the complications to be evaluated at diagnosis and some suggestions on their management during the preoperative period.

Cushing syndrome due to ACTH-secreting pheochromocytoma

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Catastrophic ACTH-secreting pheochromocytoma: an uncommon and challenging entity with multifaceted presentation.

Cushing's syndrome due to ectopic adrenocorticotropic hormone (ACTH) secretion (EAS) by a pheochromocytoma is a challenging condition. A woman with hypertension and an anamnestic report of a 'non-secreting' left adrenal mass developed uncontrolled blood pressure (BP), hyperglycaemia and severe hypokalaemia. ACTH-dependent severe hypercortisolism was ascertained in the absence of Cushingoid features, and a psycho-organic syndrome developed. Brain imaging revealed a splenial lesion of the corpus callosum and a pituitary microadenoma. The adrenal mass displayed high uptake on both 18F-FDG PET/CT and 68Ga-DOTATOC PET/CT; urinary metanephrine levels were greatly increased. The combination of antihypertensive drugs, high-dose potassium infusion, insulin and steroidogenesis inhibitor normalized BP, metabolic parameters and cortisol levels; laparoscopic left adrenalectomy under intravenous hydrocortisone infusion was performed. On combined histology and immunohistochemistry, an ACTH-secreting pheochromocytoma was diagnosed. The patient's clinical condition improved and remission of both hypercortisolism and catecholamine hypersecretion ensued. Brain magnetic resonance imaging showed a reduction of the splenial lesion. Off-therapy BP and metabolic parameters remained normal. The patient was discharged on cortisone replacement therapy for post-surgical hypocortisolism. EAS due to pheochromocytoma displays multifaceted clinical features and requires prompt diagnosis and multidisciplinary management in order to overcome the related severe clinical derangements. A small but significant number of cases of adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome are caused by ectopic ACTH secretion by neuroendocrine tumours, which is usually associated with severe hypercortisolism causing severe clinical and metabolic derangements. Ectopic ACTH secretion by a pheochromocytoma is exceedingly rare but can be life-threatening, owing to the simultaneous excess of both cortisol and catecholamines. The combination of biochemical and hormonal testing and imaging procedures is mandatory for the diagnosis of ectopic ACTH secretion, and in the presence of an adrenal mass, the possibility of an ACTH-secreting pheochromocytoma should be taken into account. Immediate-acting steroidogenesis inhibitors are required for the treatment of hypercortisolism, and catecholamine excess should also be appropriately managed before surgical removal of the tumour. A multidisciplinary approach is required for the treatment of this challenging entity.

ODP061 When cortisol and catecholamines combine in a severe endocrine condition

Abstract Introduction While the majority of ACTH dependent Cushing's Syndrome cases are the result of over secretion of ACTH from the pituitary gland, ectopic ACTH production can occur. Pheochromocytoma, a tumor originating from the adrenal chromaffin cells and classically associated with catecholamine excess, has also been very rarely shown to secrete ACTH. A 54 year old male with a history of obesity, type 2 diabetes, and resistant HTN presented to ED with a tonic clonic seizure, hypoxia and altered mental status. Labs were notable for thrombocytopenia and hypokalemia. His thrombocytopenia was treated with IVIG and solumedrol. Renin activity and renal doppler were normal. He was discharged on additional blood pressure medication, antipsychotics and Keppra. After 2 weeks he was readmitted with hypertensive urgency, anemia and worsening thrombocytopenia. Bone marrow biopsy did not elucidate the etiology of thrombocytopenia. CT abdomen showed a 5.4 cm, 54 Hounsfield unit, right adrenal mass. MRI brain showed no intracranial pathology. MRI abdomen/pelvis showed bilateral adrenal thickening. The right adrenal mass had characteristics of hematoma as well. Additional labs showed urine metanephrines 1412mcg/24Hr and normetanephrines 978mcg/24Hr, Urine free cortisol was 4494 mcg/24h, random serum cortisol was 207mg/dl, and ACTH was 1091 pg/ml. Dexamethasone suppression test cortisol was 151 ug/dL. Interesting the patient lacked cushingoid phenotypical characteristicsexcept for truncal obesity. The patient was placed on doxazosin for alpha blockadeand etomidate drip to control cortisol. The diagnosis of ACTH secreting pheochromocytoma was reaffirmed after both octreoscan and dotatate PET CT showed the presence of somatostatin receptors in the rightadrenal mass. After 14 days with controlling treatment, the patient underwent successful right adrenalectomy. Pathology was notable for a well circumscribed dark brown mass that had replaced the adrenal medulla that stained patchy positive for ACTH and was positive for synaptophysin and chromogranin. Post surgical labs demonstrated cortisol of 26.3ug/dLand ACTH of 7pg/ml. The patient was discharged 14 days after surgery. Discussion and conclusion Pheochromocytomas are rare tumors for which classical features are often absent. Symptoms of palpitations, headache and sweating are only present in 17% of patients. ACTH secreting pheochromocytomas represent 5% of ectopic ACTH syndrome with less than 100 documented cases worldwide. While obesity, hypertension and diabetes are common in our society, this patient's rapid clinical deterioration was the key clue to this rare diagnosis. This case highlights how the decisive actions of a well-coordinated, multidisciplinary team can result in a good outcome for a very challenging and critically ill patient. Presentation: No date and time listed

SUN-168 Case Series of ACTH-Secreting Pheochromocytoma: Diagnosis and Management

INTROACTH-secreting pheochromocytoma is rare, posing challenges in diagnosis and management. Here we report two cases with different presentations and pre-operative approaches.CASES Patient 1 A 59 yo female who presented for basal cell carcinoma resection experienced hypertensive crisis intra-op and NSTEMI due to stress cardiomyopathy. The patient reported spells of palpitations, diaphoresis, tremors and pallor since 2015. She had elevated plasma metanephrine (M) 19.4 (0-0.49nmol/L), normetanephrine (NM) 24.7 (0-0.89nmol/L), 24-hour urine M 18711 (30-180mcg) and NM 11897 (128-484mcg). Imaging demonstrated a 5.6 x 5.5 x 5.8cm left adrenal mass. After pre-operative alpha and beta blockade, patient underwent left adrenalectomy.Pre-op 24-hour urine cortisol was checked as part of secondary HTN evaluation and found to be elevated at 219 (3.5-45mcg). She did not have any features of Cushing’s and this was initially felt to be an appropriate stress response. Due to persistent post-op hypotension and a low 10PM random cortisol level of 3.4mcg/dL, which was inappropriate given patient’s clinical status, hydrocortisone was started with subsequent hemodynamic improvement. Our suspicion was that she had an ACTH-secreting pheochromocytoma, which would have resulted in hypercortisolism pre-op, and secondary AI post-op. Pathology report later confirmed ectopic ACTH production with 25% cells positive with immunostaining. Patient 2 A 48 yo female was admitted for hypertensive urgency with symptoms of palpitations, facial and abdominal swelling, easy bruising and proximal muscle weakness. Her labs showed elevated 24-hour urine epinephrine 115 (0-20mcg), norepinephrine 366 (0-135mcg), plasma M 288 (0-62pg/mL) and NM 475 (0-145pg/mL), and an AM cortisol was 47.9mcg/dL. Aldosterone and renin levels were <1ng/dL and 0.775ng/mL/hr. Imaging revealed 3.4 x 3.1cm right adrenal mass with portal venous phase of 53 HU and no washout. AM cortisol level post 1mg dexamethasone was 62.6mcg/dL with an ACTH level of 222pg/mL. An ACTH-producing pheochromocytoma was suspected. Despite progressive uptitration of alpha and beta blockade, her BP remained poorly controlled. Her blood glucose also increased to 300s range. Ketoconazole 400mg BID was subsequently added to the regimen with rapid and marked improvement in BP and glycemic control. She underwent right adrenalectomy. Post-op plasma M, NM and ACTH were <0.20nmol/L, 0.36nmol/L and <5pg/mL.DISCUSSIONWithin the past 6 months, we have seen 2 cases of ACTH-secreting pheochromocytoma with different clinical symptoms and pre-op courses. Ectopic ACTH secretion may not cause classic Cushingoid features as in patient 1. Failure to recognize ectopic ACTH pre-op can lead to post-op complications such as AI. Patient 2 demonstrates that treatment of the hypercortisolemia may be necessary in order to adequately control BP and glucose levels prior to surgery.

SAT-202 Hypoglycemia Following Unilateral Pheochromocytoma Resection in the Immediate Post-Surgical Period

IntroductionHypoglycemia in the immediate post-resection period of unilateral pheochromocytoma is a potential complication but not very well recognized.Clinical CaseA 47 year old female with past medical history of Hypertension, coronary artery disease, Myocardial infarction, Depression, Systemic lupus erythematosus presented to the hospital initially for elective robotic assisted Left adrenalectomy. CT scan showed a big left adrenal mass with normal right adrenal gland. It was clinically diagnosed as Pheochromocytoma as outpatient by primary internist. Biochemical studies showed elevated serum metanephrines and normetanephrines, and urine normetanephrine.Post-surgery (< 24 hours) patient had episodes of fasting hypoglycemia with blood glucose levels as low as 68 mg/dl, accompanied with neuroglycopenic symptoms like tremors, sweating and palpitations.High dose ACTH stimulation test was performed. Serum cortisol levels were tested as 5.1, 11.7 and 14.4 mcg/dl within 0, 30 minutes and 60 minutes of Cosyntropin 250 mcg IV injection.The patient was started on Prednisone 5 mg daily to prevent any further episodes, which was successful, and was stopped by the patient one week after discharge, without any more hypoglycemia episodes. Further endocrinology work up could not be done as the patient did not follow up.Post-surgical pathology showed a 7x 5.5 x4 cm mass, which was confirmed as pheochromocytoma histopathologically and immunohistochemically by positive chromogranin, synaptophysin and BCl2 and negative for calretinin and S100.Discussion and ConclusionThe etiology of hypoglycemia after resection of unilateral pheochromocytoma can be explained by impaired glucagon secretion and decreased gluconeogenesis due to the suppression from higher catecholamine levels in the blood pre-operatively. The second mechanism is rebound insulin secretion from the pancreas due to sudden withdrawal of catecholamines.In our patient, the transient hypocortisolemia could be another reason. The lack of immunohistochemical evidence in post-surgical pathology report excluded cortisol- secreting tumor. Another rare situation, ACTH-secreting pheochromocytoma, has been reported but was not checked in the case.In a word, hypoglycemia is common after surgical removal of unilateral pheochromocytoma. Careful monitoring of patients’ glucose level in immediate post-resection period is essential to prevent transient hypoglycemia

Somatostatin analog challenge test in the pre-surgical management of ACTH-secreting pheochromocytoma.

SummaryACTH-secreting pheochromocytoma is a very rare cause of Cushing’s syndrome, with a high morbidity and mortality risk due to both cortisol and catecholamines excess. We report the case of a 45-year-old female patient with a 3 cm, high-density, left adrenal mass, diagnosed as an ACTH-secreting pheochromocytoma. The biochemical sensitivity of the tumor to somatostatin analogues was tested by a 100 μg s.c. octreotide administration, which led to an ACTH and cortisol reduction of 50 and 25% respectively. In addition to alpha and beta blockers, preoperative approach to laparoscopic adrenalectomy included octreotide, a somatostatin analogue, together with ketoconazole, in order to achieve an adequate pre-surgical control of cortisol release. Histopathological assessment confirmed an ACTH-secreting pheochromocytoma expressing type 2 and 5 somatostatin receptors (SSTR-2 and -5).Learning points:ACTH-secreting pheochromocytomas represent a rare and severe condition, characterized by high morbidity and mortality risk.Surgical removal of the adrenal mass is the gold standard treatment, but adequate medical therapy is required preoperatively to improve the surgical outcome and to avoid major complications.Somatostatin analogs, in addition to other medications, may represent a useful therapeutic option for the presurgical management of selected patients.In this sense, the octreotide challenge test is a useful tool to predict favorable therapeutic response to the treatment.

SUN-393 ACTH-secreting Pheochromocytoma - A Rare Cause of Cushing’s Syndrome

Background: We present a patient with a rare ACTH-secreting pheochromocytoma. Clinical Case: A 47-year-old woman with a 22-year history of hypertension presented to the emergency room complaining of worsening headaches. Despite being on five antihypertensives, she revealed that her blood pressure was still difficult to control. On presentation her BP 187/109mmHg and labs notable for K+ of 2.4 [3.5-5.0 mmol/L] and a random glucose of 700mg/dL. She was admitted for hypertensive emergency and new onset diabetes. Physical exam was pertinent for anasarca, hyperpigmentation of skin, facial plethora, hirsutism, and acne. Labs were remarkable for a random cortisol of 88 [5.0-25mcg/dL] and ACTH level of 276 [5-46pg/mL]. Cortisol post 1mg dexamethasone was 91 [5.0-25mcg/dL], post 8mg was 88 [5.0-25mcg/dL] and 24-hour free urine cortisol 5863 [3.5-45mcg/24hr]. Pituitary MRI showed a 0.4cm microadenoma and she underwent inferior petrosal sinus sampling that suggested ectopic production of ACTH. A chest CT showed a possible thyroid nodule. Abdominal CT showed bilateral thickened adrenals and a right adrenal nodule 2.9 x 2.0cm in size. Further relevant testing revealed showed: plasma fractionated metanephrine 195 [12-61pg/mL], normetanephrine 195 [18-112pg/mL]. 24-hour urine collections: metanephrine 969 [<400mcg/24hr], normetanephrine 1193 [<900 mcg/24hr], total metanephrines 2162 [<1300mcg/24hr], epinephrine 52 [<21mcg/24hr], norepinephrine 216 [15-80mcg/24hr], dopamine 61 [65-400 mcg/24hr]. Testosterone 225.7 [14-76 ng/dL], DHEAS 1217 [48-244ug/dL], PTH 110 [15-65pg/mL], Calcium 10.35 [8.2-10mg/dL], Vitamin D 25-OH 9 [ng/mL], calcitonin <5.0 [< 7.6pg/mL], CEA 7.9, chromagranin A 55 [<93 ng/mL], gastrin 40 [<100pg/mL], TSH 0.31 [0.27-4.20mclU/mL], and FT4 1.12 [0.93-1.70]. With clear evidence of increased ACTH and catecholamine production she was placed on ketoconazole and phenoxybenzamine, respectively. In attempts to localize the site of excess ACTH production a PET/CT Ga-68 DOTATE was suspicious for a right adrenal pheochromocytoma and left anterior pelvic mass with no evidence of significant SST-R2 expression. PET/CT F-18 F-DOPA exposed a right pheochromocytoma and left lower pelvic mass with mild F-DOPA uptake. She subsequently underwent a right adrenalectomy and postoperatively was treated with hydrocortisone. Need for further antihypertensive and insulin treatment ceased. Her facial roundness, lower extremity weakness and edema resolved. Facial acne persists despite normalization of her testosterone. Conclusion We suggest that patients with Cushing’s Syndrome and uncontrolled HTN be evaluated for ACTH-secreting pheochromocytomas. Although it is a very rare condition, making its diagnosis could be life-saving and achieve clinical remission.

Anesthetic management of a patient undergoing resection of ACTH-secreting pheochromocytoma: a case report

Anesthetic management of a patient undergoing resection of ACTH-secreting pheochromocytoma: a case report

Metyrapone-responsive ectopic ACTH-secreting pheochromocytoma with a vicious cycle via a glucocorticoid-driven positive-feedback mechanism.

In ectopic ACTH-secreting pheochromocytoma, combined ACTH-driven hypercortisolemia and hypercatecholaminemia are serious conditions, which can be fatal if not diagnosed and managed appropriately, especially when glucocorticoid-driven positive feedback is suggested with a high ACTH/cortisol ratio. A 46-year-old man presented with headache, rapid weight loss, hyperhidrosis, severe hypertension and hyperglycemia without typical Cushingoid appearance. Endocrinological examinations demonstrated elevated plasma and urine catecholamines, serum cortisol and plasma ACTH. Moreover, his ACTH/cortisol ratio and catecholamine levels were extremely high, suggesting catecholamine-dominant ACTH-secreting pheochromocytoma. Computed tomography revealed a large right adrenal tumor. 18F-FDG positron emission tomography showed uptake in the area of the adrenal tumor, while 123I-metaiodobenzylguanidine scintigraphy showed no accumulation. His plasma ACTH level paradoxically became elevated after a dexamethasone suppression test. After metyrapone administration, not only serum cortisol but also plasma ACTH levels were exponentially decreased almost in parallel, suggesting a glucocorticoid-driven positive-feedback regulation in this rapidly exacerbated ectopic ACTH-producing pheochromocytoma. Interestingly enough, plasma catecholamine levels were also decreased by metyrapone, although they remained extremely high. He became severely dehydrated due to hypoadrenalism requiring hydrocortisone supplementation. His clinical signs and symptoms were improved, and right adrenalectomy was performed uneventfully, resulting in complete remission of pheochromocytoma and Cushing's syndrome. A glucocorticoid-driven positive-feedback regulation in this ectopic ACTH-secreting pheochromocytoma created a vicious cycle with rapid exacerbation of both hypercortisolemia and hypercatecholaminemia with extremely elevated plasma ACTH level. Metyrapone was clinically effective to stop this vicious cycle; nonetheless, great care must be taken to avoid hypoadrenalism especially when hypercatecholaminemia remained.

ACTH ectopic syndrome caused by corticomedullary mixed tumor

We report the case of a 61 year old woman with a typical clinical picture of ACTH dependent Cushing’s syndrome. Diagnostic tests confirmed ACTH dependent Cushing’s syndrome, which is caused by corticomedullary mixed tumor. ACTH-secreting pheochromocytoma are rarely described in accessible literature, there is no one case report about ACTH-secreting corticomedullary mixed tumor. Due to the rarity of the disease, we believe it appropriate to share our experience and consider all variants of interaction in hypothalamic-pituitary-adrenal axis, which could lead to morphology and clinical changes uncovered in this case.

Adrenocorticotropic hormone-producing pheochromocytoma: analysis of clinical cases

Ectopic secretion of ACTH from non-pituitary tumors, referred to as ectopic ACTH syndrome (EAS), accounts for about 10–20% of Cushing’s syndrome (CS). Ectopic hormone-secreting pheochromocytomas (Pheo) are rare. The first publication of association between pheochromocytoma and Cushing’s syndrome by Roux is dated 1955. Pheochromocytoma represents a rare cause of hypercortisolism, accounting for less than 5 % of ectopic Cushing’s syndrome while less than 1 % of pheochromocytomas is accompanied by Cushing’s syndrome.We are reporting 4 cases of ACTH-secreting pheochromocytoma presenting as Cushing’s syndrome. Data from 4 patients were analysed. There were 4 women from 50 to 63 years old. All patients had a clinical presentation of hypercorticoidism. Their levels of adrenocorticotropic hormone in plasma, 24-hour urinary free cortisol and urinary catecholamine were high. Computed tomography scan of the abdomen in all cases revealed a mass in the left adrenal gland. Left sided adrenalectomy was performed under treatment with a-blocker doxazosin and b-blocker atenolol. Histological examination revealed in 3 cases – pheocromocytoma and in 1 case corticomedullary mixed tumor of the adrenal gland. Additional immunostaining (IHC)of these tumors showed positive immunostaining for chromogranin and ACTH. The IHC search for somatostatin receptors of subtype 2 and 5 (SSTR2, SSTR5) was performed in 3 cases and showed predominately expression SSTR2. The case index of Ki-67 ranged, from 0,5 to 4%. Biochemical signs of hypercortisolism rapidly began to disappear after surgery. Follow up of the patients during the next 2 years on average was with disease remission.

Ectopic Cushing's syndrome caused by ACTH-secreting non-functioning pheochromocytoma

Ectopic Cushing's syndrome caused by ACTH-secreting non-functioning pheochromocytoma

Mini-review: pheochromocytomas causing the ectopic ACTH syndrome

Cushing's syndrome (CS) is a condition associated with high cortisol levels and affects around 0.7-2.4 cases per million population per year [1-3]. Approximately 80 % of cases of CS are secondary to excess adrenocorticotrophin (ACTH) secretion, while in around 20 % the primary abnormality lies in the adrenal, most often an adrenal adenoma or carcinoma. Of the ACTH-dependent causes, some 80-90 % are due to a pituitary adenoma-Cushing's disease-but in a significant proportion the cause is ectopic secretion from a non-pituitary source, the ectopic ACTH syndrome (EAS) [4]. The commonest source of ACTH secretion in these patients is a bronchial carcinoid. However, many other tumors are capable of secreting ACTH, and in most series a small but significant number are secondary to an ACTH-secreting pheochromocytoma. Amalgamating the data from 6 large series of patients with ectopic ACTH, pheochromocytoma was the source of ACTH secretion in 19 out of 363 patients (5.2 %) [5-11]. In this mini-review, we present a patient presenting to our department with a pheochromocytoma as well as Cushing's syndrome due to ectopic ACTH secretion by the pheochromocytoma, and we discuss published cases in the world literature to assess its significance. We emphasize the problems in the simultaneous management of these two serious endocrine conditions.

Ectopic ACTH syndrome caused by pheochromocytoma: Computed tomography-guided percutaneous ethanol injection as an alternative treatment

Ectopic ACTH secretion represents 8-18% of the cases of endogenous hypercortisolism. Pheochromocytomas correspond to 2-25% of the cases and surgery is the indicated treatment. We describe a case of ACTH-secreting pheochromocytoma treated with percutaneous ethanol injection (PEI) guided by computed tomography (CT). A 71-yr-old man presented with diabetes, severe hypokalemia, weight loss, muscle weakness, and hypertension. Hormonal evaluation revealed elevated levels of urinary cortisol, ACTH, catecholamines, and urinary metanephrines. There was no cortisol or ACTH response to desmopressin stimulation test. Magnetic resonance revealed bilateral adrenal nodules, larger on the left side. The suspected diagnosis was ectopic ACTH syndrome caused by pheochromocytoma. Ketoconazole treatment resulted in reduction of urinary cortisol levels but was followed by severe cholestasis and hepatic dysfunction, preventing surgery; it was substituted by octreotide with reduction of ACTH and cortisol levels, but without improvement of cholestasis. The patient presented cachexia and developed multiple pulmonary abscesses that also prevented surgical treatment, thus he was treated with percutaneous ethanol injection guided by CT of the left adrenal tumor. During the procedure, the patient had an increase in blood pressure controlled by the infusion of sodium nitroprusside followed by hypotension that required infusion of dopamine and volume expansion. Afterwards, he presented hormonal normalization, normal catecholamines levels, and clinical improvement. Histological tissue analysis confirmed pheochromocytoma. We concluded that CT-guided PEI represents an efficient alternative therapy to ectopic ACTH-secreting pheochromocytomas in patients without clinical conditions for surgery.

Cushing's syndrome due to ACTH-secreting pheochromocytoma

Pheochromocytomas are rare; less than 1% of hypertensive patients harbor a chromaffin tumour as the cause of increased blood pressure. The rarity of these tumours should not belie their importance. Correctly diagnosed and properly treated, pheochromocytoma is curable; misdiagnosed or improperly treated, it is fatal. 90% of pheochromocytomas are benign; most are diagnosed during life, although many cases are found unexpectedly at post mortem examination. Pheochromocytomas are dangerous because of their capacity to store and release catecholamines in large amounts (Wilson et al.'98).

Cushing′s syndrome in a patient with ACTH secreting pheochromocytoma

Cushing′s syndrome in a patient with ACTH secreting pheochromocytoma

ACTH Secretion from a Functioning Pheochromocytoma

A PATIENT with hypertension, hirsutism, hyperglycemia, hypokalemic alkalosis and a transient paranoid psychosis was found to have hypercortisolism and increased excretion of catecholamines secondary to an ACTH-secreting pheochromocytoma. Fluctuations in plasma ACTH levels coincided with variations in urinary catecholamine excretion, suggesting that a common factor regulated peptide and catecholamine secretion in this patient. Adrenal-vein sampling revealed fourfold elevations in left-adrenal-vein ACTH and 10-fold elevations in left-adrenal-vein norepinephrine, when these values were compared with samples from the right adrenal vein and peripheral blood. After surgical removal of the left adrenal gland, urinary catecholamine levels returned to the normal range, whereas levels . . .