Comley.ai

Sudden Clinical Development Of Ectopic Cushing's Syndrome Due To A Non-Catecholamine Producing Pheochromocytoma Shannon Comley Sood, DO; Matthew Leinung, MD; Ming-Tseh Lin, MD; Timothy Jennings, MD; and Daniel W. Lee, MD Department of Medicine, Division of Endocrinology and Division of Pathology and Laboratory Medicine Albany Medical College, Albany, New York CASE HISTORY (continued): Her past medical history was significant for autoimmune hepatitis, Hashimoto's thyroiditis, lymphocytic inflammatory colitis, depression, and fibromyalgia. She was a former smoker To our knowledge, there is only one previous report of a patient with Cushing's syndrome caused by a non- but denied alcohol use and was on Synthroid, Wellbutrin, spironolactone, Toprol XL, secreting adrenal pheochromocytoma. We now present a 54 year old female who developed sudden ectopic Cushing's syndrome in a previously diagnosed but apparently nonfunctioning adrenal incidentaloma. The Aciphex, and potassium at time of presentation to our office after hospitalization. Spiro- incidentaloma was noted one year prior to her presentation with Cushing's syndrome. She was also eval- nolactone, metoprolol, and potassium had been started at the outlying hospital for uated by the authors in endocrine clinic four months after discovery of this lesion. Despite a search for signs refractory hypertension and hypokalemia.
and symptoms of Cushing's syndrome, none were identified. Seven months later the patient presented to an edicine 138(5): p 424, 2003. outlying hospital with rapid onset and worsening of swelling in her face, neck, and abdomen accompanied Upon initial physical examination, her blood pressure was 160/110 and her pulse was by a syncopal episode. Hypokalemia, hypertension, hyperglycemia, and Cushingoid habitus were noted. 110-120 and regular. Cushing habitus with facial and posterior cervical swelling was noted. Her cortisol was 119 mcg/dl; her ACTH was 518 pg/dl (nl 9-52 pg/ml). A 24-hour urine free cortisol was Lungs were clear. Abdomen was slightly distended. No violaceous striae were noted. nnals of Internal M elevated to 8479 ug/24 hr (nl 0-50 ug/24 hr). MRI of pituitary was negative and inferior petrosal sinus Lower extremities showed 1+ pitting edema and multiple ecchymoses. Proximal muscle sampling did not reveal an elevated central to peripheral ratio after CRH stimulation. The ACTH appeared weakness was also appreciated. There were no neurologic deficits appreciated. Skin ectopic in origin. A CT scan chest/abdomen/pelvis was repeated and the mass had increased in size to 3.7 showed mild acne and facial hirsutism.
Fig. 1. Pheochromocytoma. The medulla of the 42-gram left cm and the opposite adrenal was now noted to be hypertrophic. Urinary metanephrines and cate- adrenal gland is replaced by a 3.5 cm fleshy tan mass. cholamines were collected and levels were all within normal limits. During testing her clinical condition deteriorated rapidly with refractory hypertension and the development of psychosis. The patient underwent left adrenalectomy and a 3.5 cm tumor was removed. Pathologic examination was consistent with a Her white blood cell count was 12.6 thousand/ml, and her Hemoglobin/Hematocrit was linically Inapparent A pheochromocytoma. Immunostaining of the tumor showed focal staining for ACTH, and ACTH levels were 11.7/33%. Her potassium was 2.2 (normal 3.5-5.3), her bicarbonate was 40.1 (normal Serum ACTH was undetectable on postoperative day one. Her hypokalemia, hypergly 21-32), and her glucose was 186 (normal 60-110 A random cortisol was sent and found undetectable on postoperative day one. Her hypokalemia, hyperglycemia,hypertension, edema, and cemia, hypertension, edema, and psychosis all resolved within one week. Muscle psychosis all resolved within one week. In conclusion, we present a case of Cushing's syndrome due to a to be greater than 60 ug/dl (normal 8.7-22.4). A repeated cortisol value of 119 ug/dl was strength slowly improved over the next month. She remained on hydrocortisone noncatecholamine secreting, ACTH producing pheochromocytoma. Her rapid clinical presentation implies obtained. ACTH was also obtained and found to be 518 pg/dl (normal 9-52). A 24-hour replacement for six months post operatively and recently discontinued hydrocortisone.
that the biologic behavior of the tumor had changed from when it was first discovered and 2.5 cm in size. urinary free cortisol was elevated to 8479 mcg/24 hr (normal 0-50 mcg/24 hr). Urinary elvin et al; M The explanation for this apparent transformation is unclear. One should be aware that while ACTH pro- metanephrines and catecholamines were collected. These levels were all within normal ACTH Levels (in PG/ML) ducing tumors of the adrenal are rare, it is possible that they can present without the usual biochemical limits; metanephrines 178 ug/24 hrs (normal 35-460), normetanephrines 138 ug/24 hr markers for pheochromocytoma. (normal 110-1050), epinephrine 6 ug/24 hr (normal 0-24), norepinephrine 21 ug/24 hr (normal 0-140), and dopamine 105 ug/24 hr (normal 65-610). Diagnostic Imaging Ectopic ACTH production is responsible for approximately 15% of ACTH-dependent Cushing's syndrome (1). Ectopic ACTH production can come from a variety of sources; one of the most rare MRI of the pituitary was completely normal and inferior petrosal sinus sampling was being pheochromocytomas, which are believed to cause only 3% of cases of ectopic ACTH pro- planned. A CT of the abdomen/pelvis was performed. The adrenal adenoma previously duction (1). In those that have been reported, approximately 90% of pheochromocytomas have 2.7 cm in size less than one year prior was now 3.7 cm in size and the right adrenal was catecholamine levels greater than two times the upper limit of normal (1). We now present a case now also noted to be hypertrophic. A chest CT was also done, but no mass was noted. of a non-catecholamine secreting, ACTH-producing pheochromocytoma.
Inferior petrosal sinus sampling did not reveal an elevated central to peripheral ratio after CRH stimulation.
POST OP DAY
LEFT INF PETROSAL A 54-year-old female was evaluated in endocrinology clinic for an incidental finding of an adrenal We present a case of Cushing's syndrome due to a noncatecholamine secreting, ACTH adenoma. Despite a search for signs and symptoms of Cushing's syndrome, none were identified. producing pheochromocytoma. Her rapid clinical presentation implies that the biologic Urinary catecholamines and metanephrines were ordered at the initial visit, but the patient did behavior of the tumor had changed from when it was first discovered and 2.5 cm in size. s Textbook of Endocrinology. tenth edition. Philadelphia: Saunders; 2003. p. 513, 555-562. 2. G not complete the urinary collection. The patient was to be followed for any change in size of the lesion and any new signs or symptoms. Several months later she was admitted to an outlying hospital after an episode of acute swelling of her face, neck, and abdomen accompanied by a syn- (All values of ACTH reported in pg/ml). An elevated central to peripheral ratio of ACTH after CRH stimulation is absent, indicating an ectopic source. copal episode while out in her yard gardening. At that time she also noted multiple ecchymoses We present a case of an apparent adrenal incidentaloma which rapidly became clinically life ed, Polonsky et al; W to her lower extremities despite a lack of trauma to the area. She had recently come off a steroid threatening. This appears to have been due to relatively sudden biochemical transformation of taper (prednisone 40 mg for 3 days, 10 mg for five days, and then 2.5 mg for seven days and then the adenoma. We agree with recent recommendations to obtain baseline urinary metanephrine The patient's clinical condition continued to deteriorate over the next several weeks. She and catecholamines as well as a 1 mg dexamethasone suppression test for screening in all discontinued) for a flare up of lymphocytic inflammatory colitis, but was otherwise in her usual remained hypertensive despite anti-hypertensive therapy, she was unable to ambulate or patients with adrenal adenoma, even those who are asymptomatic (2), and repeat testing if any state of health. She provided no other recent history of steroid use. She did complain of a sen- lift herself from a sitting position without assistance, and she became acutely psychotic change in symptoms occurs. We believe we have illustrated that it is possible for pheochromo- sation of excessive energy and had been suffering from insomnia for the last one month while on requiring medication. She was started on ketoconazole, and for lack of another potential cytomas to present without classic biochemical markers and yet be a source of ectopic ACTH. ES: 1. Larsen, Kronenberg, M source of ACTH, a left adrenalectomy was planned.
Designed by Michelle Brozowski

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