ACTH-SECRETING PHEOCHROMOCYTOMA
AACE ePoster Library. Kalia-Reynolds M. 05/13/15; 97748; 100
Maitri Kalia-Reynolds
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TITLE: ACTH-SECRETING PHEOCHROMOCYTOMA
Kalia-Reynolds, Maitri S.1; Giordano, Jennifer M.1; Khanna, Ila2; Houston, Caroline1
ABSTRACT BODY:
Objective: We present a case of ACTH-producing pheochromocytoma, which is an extremely rare but important cause
of ectopic Cushing’s syndrome.
Results or Case Presentation: A 56 year old female presented with 3 years of progressively worsening diabetes
mellitus, hypertension, hypokalemia, facial swelling, insomnia, and irritability. Her blood pressure was 160/94, pulse
was 98, and BMI was 36.5. She was noted to have typical Cushingoid features of moon facies, facial plethora,
hirsutism, cervicodorsal and supraclavicular fat deposition, purple abdominal striae, and lower extremity edema.
Serum sodium was 142 mEq/L and potassium was 2.7 mEq/L. Random serum cortisol was 101.9 ug/dL with ACTH of
299 pg/mL. Urinary free cortisol was 11,870 mcg/24 hrs.
She was hospitalized and received potassium repletion, spironolactone, and ketoconazole to treat her
hypercortisolemia. Pituitary MRI revealed no abnormalities and other pituitary hormone levels were normal. CRH
stimulation testing yielded a 10.4% rise in serum cortisol and 49.4% rise in ACTH. High-dose (8 mg) dexamethasone
suppression testing resulted in a 2.7% decrease in serum cortisol. Together, these results were suggestive of ectopic
ACTH secretion.
Abdominal CT revealed a 5.6 cm heterogeneous right adrenal mass with postcontrast enhancement of 4 Hounsfield
units. Serum aldosterone, plasma renin activity, testosterone, and DHEAS were normal. Urine and plasma
metanephrines were greater than 4 times the upper limit of normal. Right adrenalectomy was performed following the
initiation of alpha and beta blockade. Pathology confirmed pheochromocytoma with positive ACTH staining.
The patient's Cushingoid features rapidly improved; her diabetes mellitus, hypertension, and hypokalemia resolved;
and her plasma metanephrines normalized. Glucocorticoid replacement was tapered over the ensuing 3 months with
eventual recovery of her HPA axis and sustained normalization of her ACTH and cortisol.
Discussion: Pheochromocytoma is a rare source of ectopic ACTH production, with fewer than 30 cases described in
the medical literature. This condition has protean manifestations and the clinical features of catecholamine excess
may be obscured. Our patient had symptoms (hypertension, insomnia, irritability) that were initially attributed to
hypercortisolemia, but may also have been due to her hyperadrenergic state. Recognition of her pheochromocytoma
was critical to the implementation of preoperative measures to avert hypertensive crisis.
Conclusion: Our case adds to the small body of literature on this topic and underscores the need for clinicians to be
alert to the possibility of pheochromocytoma in patients with Cushing’s syndrome.
Kalia-Reynolds, Maitri S.1; Giordano, Jennifer M.1; Khanna, Ila2; Houston, Caroline1
ABSTRACT BODY:
Objective: We present a case of ACTH-producing pheochromocytoma, which is an extremely rare but important cause
of ectopic Cushing’s syndrome.
Results or Case Presentation: A 56 year old female presented with 3 years of progressively worsening diabetes
mellitus, hypertension, hypokalemia, facial swelling, insomnia, and irritability. Her blood pressure was 160/94, pulse
was 98, and BMI was 36.5. She was noted to have typical Cushingoid features of moon facies, facial plethora,
hirsutism, cervicodorsal and supraclavicular fat deposition, purple abdominal striae, and lower extremity edema.
Serum sodium was 142 mEq/L and potassium was 2.7 mEq/L. Random serum cortisol was 101.9 ug/dL with ACTH of
299 pg/mL. Urinary free cortisol was 11,870 mcg/24 hrs.
She was hospitalized and received potassium repletion, spironolactone, and ketoconazole to treat her
hypercortisolemia. Pituitary MRI revealed no abnormalities and other pituitary hormone levels were normal. CRH
stimulation testing yielded a 10.4% rise in serum cortisol and 49.4% rise in ACTH. High-dose (8 mg) dexamethasone
suppression testing resulted in a 2.7% decrease in serum cortisol. Together, these results were suggestive of ectopic
ACTH secretion.
Abdominal CT revealed a 5.6 cm heterogeneous right adrenal mass with postcontrast enhancement of 4 Hounsfield
units. Serum aldosterone, plasma renin activity, testosterone, and DHEAS were normal. Urine and plasma
metanephrines were greater than 4 times the upper limit of normal. Right adrenalectomy was performed following the
initiation of alpha and beta blockade. Pathology confirmed pheochromocytoma with positive ACTH staining.
The patient's Cushingoid features rapidly improved; her diabetes mellitus, hypertension, and hypokalemia resolved;
and her plasma metanephrines normalized. Glucocorticoid replacement was tapered over the ensuing 3 months with
eventual recovery of her HPA axis and sustained normalization of her ACTH and cortisol.
Discussion: Pheochromocytoma is a rare source of ectopic ACTH production, with fewer than 30 cases described in
the medical literature. This condition has protean manifestations and the clinical features of catecholamine excess
may be obscured. Our patient had symptoms (hypertension, insomnia, irritability) that were initially attributed to
hypercortisolemia, but may also have been due to her hyperadrenergic state. Recognition of her pheochromocytoma
was critical to the implementation of preoperative measures to avert hypertensive crisis.
Conclusion: Our case adds to the small body of literature on this topic and underscores the need for clinicians to be
alert to the possibility of pheochromocytoma in patients with Cushing’s syndrome.
TITLE: ACTH-SECRETING PHEOCHROMOCYTOMA
Kalia-Reynolds, Maitri S.1; Giordano, Jennifer M.1; Khanna, Ila2; Houston, Caroline1
ABSTRACT BODY:
Objective: We present a case of ACTH-producing pheochromocytoma, which is an extremely rare but important cause
of ectopic Cushing’s syndrome.
Results or Case Presentation: A 56 year old female presented with 3 years of progressively worsening diabetes
mellitus, hypertension, hypokalemia, facial swelling, insomnia, and irritability. Her blood pressure was 160/94, pulse
was 98, and BMI was 36.5. She was noted to have typical Cushingoid features of moon facies, facial plethora,
hirsutism, cervicodorsal and supraclavicular fat deposition, purple abdominal striae, and lower extremity edema.
Serum sodium was 142 mEq/L and potassium was 2.7 mEq/L. Random serum cortisol was 101.9 ug/dL with ACTH of
299 pg/mL. Urinary free cortisol was 11,870 mcg/24 hrs.
She was hospitalized and received potassium repletion, spironolactone, and ketoconazole to treat her
hypercortisolemia. Pituitary MRI revealed no abnormalities and other pituitary hormone levels were normal. CRH
stimulation testing yielded a 10.4% rise in serum cortisol and 49.4% rise in ACTH. High-dose (8 mg) dexamethasone
suppression testing resulted in a 2.7% decrease in serum cortisol. Together, these results were suggestive of ectopic
ACTH secretion.
Abdominal CT revealed a 5.6 cm heterogeneous right adrenal mass with postcontrast enhancement of 4 Hounsfield
units. Serum aldosterone, plasma renin activity, testosterone, and DHEAS were normal. Urine and plasma
metanephrines were greater than 4 times the upper limit of normal. Right adrenalectomy was performed following the
initiation of alpha and beta blockade. Pathology confirmed pheochromocytoma with positive ACTH staining.
The patient's Cushingoid features rapidly improved; her diabetes mellitus, hypertension, and hypokalemia resolved;
and her plasma metanephrines normalized. Glucocorticoid replacement was tapered over the ensuing 3 months with
eventual recovery of her HPA axis and sustained normalization of her ACTH and cortisol.
Discussion: Pheochromocytoma is a rare source of ectopic ACTH production, with fewer than 30 cases described in
the medical literature. This condition has protean manifestations and the clinical features of catecholamine excess
may be obscured. Our patient had symptoms (hypertension, insomnia, irritability) that were initially attributed to
hypercortisolemia, but may also have been due to her hyperadrenergic state. Recognition of her pheochromocytoma
was critical to the implementation of preoperative measures to avert hypertensive crisis.
Conclusion: Our case adds to the small body of literature on this topic and underscores the need for clinicians to be
alert to the possibility of pheochromocytoma in patients with Cushing’s syndrome.
Kalia-Reynolds, Maitri S.1; Giordano, Jennifer M.1; Khanna, Ila2; Houston, Caroline1
ABSTRACT BODY:
Objective: We present a case of ACTH-producing pheochromocytoma, which is an extremely rare but important cause
of ectopic Cushing’s syndrome.
Results or Case Presentation: A 56 year old female presented with 3 years of progressively worsening diabetes
mellitus, hypertension, hypokalemia, facial swelling, insomnia, and irritability. Her blood pressure was 160/94, pulse
was 98, and BMI was 36.5. She was noted to have typical Cushingoid features of moon facies, facial plethora,
hirsutism, cervicodorsal and supraclavicular fat deposition, purple abdominal striae, and lower extremity edema.
Serum sodium was 142 mEq/L and potassium was 2.7 mEq/L. Random serum cortisol was 101.9 ug/dL with ACTH of
299 pg/mL. Urinary free cortisol was 11,870 mcg/24 hrs.
She was hospitalized and received potassium repletion, spironolactone, and ketoconazole to treat her
hypercortisolemia. Pituitary MRI revealed no abnormalities and other pituitary hormone levels were normal. CRH
stimulation testing yielded a 10.4% rise in serum cortisol and 49.4% rise in ACTH. High-dose (8 mg) dexamethasone
suppression testing resulted in a 2.7% decrease in serum cortisol. Together, these results were suggestive of ectopic
ACTH secretion.
Abdominal CT revealed a 5.6 cm heterogeneous right adrenal mass with postcontrast enhancement of 4 Hounsfield
units. Serum aldosterone, plasma renin activity, testosterone, and DHEAS were normal. Urine and plasma
metanephrines were greater than 4 times the upper limit of normal. Right adrenalectomy was performed following the
initiation of alpha and beta blockade. Pathology confirmed pheochromocytoma with positive ACTH staining.
The patient's Cushingoid features rapidly improved; her diabetes mellitus, hypertension, and hypokalemia resolved;
and her plasma metanephrines normalized. Glucocorticoid replacement was tapered over the ensuing 3 months with
eventual recovery of her HPA axis and sustained normalization of her ACTH and cortisol.
Discussion: Pheochromocytoma is a rare source of ectopic ACTH production, with fewer than 30 cases described in
the medical literature. This condition has protean manifestations and the clinical features of catecholamine excess
may be obscured. Our patient had symptoms (hypertension, insomnia, irritability) that were initially attributed to
hypercortisolemia, but may also have been due to her hyperadrenergic state. Recognition of her pheochromocytoma
was critical to the implementation of preoperative measures to avert hypertensive crisis.
Conclusion: Our case adds to the small body of literature on this topic and underscores the need for clinicians to be
alert to the possibility of pheochromocytoma in patients with Cushing’s syndrome.
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