ResearchPad - adrenal-case-reports-iii Default RSS Feed en-us © 2020 Newgen KnowledgeWorks <![CDATA[MON-LB44 Cushing’s Syndrome and Illegal Receptors]]> Case presentation: A 48-year-old female with HTN presented to the endocrinology clinic for the evaluation of incidental bilateral adrenal masses noted on chest CT for dyspnea workup. At the time of the presentation, she reported generalized fatigue, significant weight gain in the past year and shortness of breath. Her physical exam was remarkable for central obesity. Lab work showed elevated cortisol after 1 mg dexamethasone suppression test x 2 and elevated 24- hour urine cortisol. Plasma free metanephrine levels and aldosterone/ renin ratio were normal. MRI abdomen was done and showed bilateral adrenal masses (left: 5.6 cm, right: 3.2 cm). Patient was diagnosed with Cushing’s syndrome secondary to primary bilateral adrenal hyperplasia and was referred to endocrine surgery who recommended unilateral adrenalectomy. The decision was made to remove the larger left side adrenal mass. On post-operative day one her am cortisol decreased to 2.1 and she was started on hydrocortisone 20 mg in the morning and 10 mg in the evening.

Discussion: Primary bilateral adrenal hyperplasia is a rare cause (< 2 %) of endogenous Cushing’s syndrome, usually occurs in a bimodal age distribution, in childhood and in the fifth- sixth decades. Presentation is variable with most patients having no symptoms or subclinical Cushing’s. The theory is the larger nodule size corelates with the higher cortisol production. Studies have shown between 60-70% of cases has aberrant ectopic hormone receptors which leads to increased cortisol production not only from ACTH but also from other ligands such as serotonin and vasopressin. Aberrant receptor testing examines whether cortisol or other steroid production increases in response to either physiologic or pharmacologic stimulus. Multiple genetic mutations have been associated, the most frequent is mutations in the Armadillo repeat- containing 5 gene identified in 2013. Treatment can either be medical or surgical. Medical therapy can be initiated if testing for an aberrant receptor is positive. In recent years there has been a trend towards doing unilateral adrenalectomy instead of bilateral, with initial remission of symptoms reported in about 84% of cases after unilateral adrenalectomy although there is a small risk of recurrence. Post operatively after unilateral adrenalectomy patients should be monitored for adrenal insufficiency. Our patient declined aberrant receptor testing and opted for surgery and is doing well post operatively.

Conclusion: Primary bilateral adrenal hyperplasia is a rare cause of endogenous Cushing’s syndrome which can be treated either medically or surgically.

<![CDATA[MON-LB042 A Rare Case of Adrenocortical Carcinoma Arising From Ectopic Adrenal Tissue in the Mesentery]]> Background: ACTH independent Cushing’s syndrome usually arises from benign or malignant tumors of the adrenal gland. Ectopic adrenal tissue can undergo malignant transformation resulting in the development of adrenocortical carcinoma (ACC) with normal adrenal glands. We present a unique case of ACTH independent Cushing’s syndrome from a cortisol and androgen producing ACC arising from the mesentery.

Clinical Case: A 72-year-old woman presented with a 6-month history of progressive weakness, lower extremity edema, worsening hypertension and uncontrolled DM. She had moon facies, hirsutism, multiple bruises, and proximal muscle weakness. Labs revealed hypokalemia (2.6 mmol/L, N 3.5 -5.1 nmol/L), elevated random cortisol (51.3 mcg/dL, Nl- 5-23), suppressed ACTH (<1 pg/mL, Nl-7.2-63.3) abnormal 8 mg dexamethasone suppression test (58.1 mcg/dL, N <1.8 mcg/dL), elevated DHEAS (538mcg/dL, N 10-90 mcg/dL), elevated testosterone (590.2 ng/dL, N <75 ng/dL) and elevated 11-deoxycortisol (4650 ng/dL N-<32 ng/dL). 24 hour urinary free cortisol was 2810mcg/mL (<45mcg/dL). MRI of pituitary was normal. CT scan of abdomen/pelvis showed normal bilateral adrenal glands and innumerable enhancing masses throughout the abdomen with the largest mass near the distal ileum and cecum. Biopsy of right lower abdominal mass revealed adrenocortical morphology with immunohistochemical staining positive for inhibin, synaptophysin and calretinin. Ki-67 index was 10-15%, suggestive of low-grade adrenocortical carcinoma. A CT scan done one and half years prior noted a 4.4 cm soft tissue mass in the right lower mesentery supporting origin of the tumor from the mesentery. Hypercortisolism was controlled with Metyrapone 250 mg BID. Mitotane 1000 mg bid was initiated but patient developed peritoneal carcinomatosis within 1 month. Conclusions: Our case is remarkable for the development of a metastatic ACC from an ectopic adrenal tissue with normal bilateral adrenal glands. Ectopic ACC is very rare with only a handful of cases reported in the literature. This is the first reported case of ACC arising from the mesentery. Ectopic adrenal tissue can be found close to the adrenal glands, along the path from gonads to adrenal glands or in association with the gonads. In the setting of ACTH independent Cushing’s syndrome with normal adrenal glands, physicians should direct their search to a functioning ectopic adrenocortical tissue. Concomitant DHEAS secretion suggests ectopic ACC. If surgery is not an option due to metastatic disease, a multidisciplinary approach should be adopted to control tumor growth and associated symptoms. In such cases, control of the hypercortisolemia can be achieved with adrenolytic medications such as Metyrapone, Ketoconazole or Mitotane. Adjuvant chemotherapy (Mitotane and combination of cytotoxic drugs) might be considered for metastatic ACC treatment.

<![CDATA[MON-LB043 A Case of Renovascular Hypertension With Cortisol-Producing Adrenal Masses]]> Renovascular hypertension (RVHT) is an important and potentially treatable form of resistant hypertension. Hypercortisolemia could also cause hypertension and diabetes mellitus. We experienced a case wherein adrenalectomy markedly improved blood pressure and plasma glucose levels in a patient with RVHT and subclinical Cushing’s syndrome. A 62-year-old Japanese man had been treated for hypertension and diabetes mellitus for 10 years. He was hospitalized because of disturbance in consciousness. His blood pressure (BP) was 236/118 mmHg; pulse rate, 132 beats/min; and plasma glucose level, 712 mg/dl. Abdominal computed tomography scanning revealed the presence of bilateral adrenal masses and left atrophic kidney. Abdominal magnetic resonance angiography demonstrated marked stenosis of the left main renal artery. The patient was subsequently diagnosed with atherosclerotic RVHT with left renal artery stenosis. Bilateral adrenal masses were immunohistologically identified as potential sites for cortisol overproduction. Therefore, laparoscopic left nephrectomy and adrenalectomy were simultaneously performed resulting in improved BP and glucose levels. Pathological studies revealed the presence of multiple cortisol-producing adrenal nodules and aldosterone-producing cell clusters in the adjacent left adrenal cortex. In the present case, activated renin-angiotensin-aldosterone system and cortisol overproduction resulted in severe hypertension, which was managed with simultaneous unilateral nephrectomy and adrenalectomy.

<![CDATA[MON-LB034 A Severe Case of Pheochromocytoma Presenting as Classic Takotsubo’s Cardiomyopathy With Rapid Resolution]]> Background: Inverted Takotsubo’s cardiomyopathy requiring extra-corporeal membrane support (ECMO) with pheochromocytoma is well described. Classic Takotsubo’s cardiomyopathy, however, is rarely described in this setting.

Clinical Case: A 50-year old woman with no previous comorbidities presented with pulmonary edema and cardiogenic shock. She required rapid escalation of vasopressor, inotrope, and intra-aortic balloon pump then ECMO with consideration of cardiac transplant. Initial echocardiogram showed an ejection fraction of 17%. Coronary angiography showed apical ballooning in keeping with classic Takotsubo’s cardiomyopathy. Abdominal ultrasound for transplant screening showed a 4.6 cm abdominal mass. Computed tomography confirmed a 4.6 cm mass with classic radiologic features of pheochromocytoma. Plasma free normetaphrine and metanephrine were elevated at 2.34 nmol/L and 0.25 nmol/L. A 24-hour urine collection included urine volume of 3500 ml, urine creatinine 7.2 mmol/day, elevated urine norepinephrine 1199 nmol/day, elevated urine epinephrine 888 nmol/day, and normal urine dopamine 335 nmol/day. Parathyroid hormone and calcitonin were normal at 0.4 pmol/L and 4.8 pmol/L, respectively. Plasma aldosterone was normal at 87 pmol/L and plasma renin normal at 3.5 ng/L. Doxazosin 1 mg daily was initiated and she had clinical improvement next day and came off ECMO. Her ejection fraction improved to 55% subsequent week. She had retroperitoneoscopic adrenalectomy two weeks later with full recovery.

Conclusion: Classic Takotsubo’s cardiomyopathy is a possible presentation of pheochromocytoma with rapid resolution with alpha blockade.

<![CDATA[MON-LB036 Uncommon Findings in Beckwith-Wiedemann Syndrome]]> 1000 mcg/dL (30,0 - 250,0 mcg/dL), testosterone 637 ng/dL (60-400 ng/dL), leading to ACT suspicion. No suppression of cortisol (2,8 mcg/dL) after dexamethasone 10 mcg/kg. Urinary metanephrines were normal, plasmatic normetanephrine 554 pg/mL (<196 pg/mL) and plasmatic norepinephrine 2094 pg/mL (< 420 pg/mL). Abdominal CT showed increased adrenal glands, with normal contrast uptake. MRI and PET/CT DOTA-68Ga did not identify tumors. Eight months later: 17OHP 0,88 ng/mL (0,8-5ng/mL), DHEAS 37 mcg/dL (<30,0 mcg/dL), testosterone 13 ng/mL (< 30 ng/dL), normal urinary metanephrines and plasmatic catecholamines. Conclusion: In this case congenital adrenal hyperplasia was ruled out and ACT was suspected. Imaging did not identify tumors and biochemical findings (hypercortisolism and high androgens levels) had progressive improvement. Bilateral adrenal hyperplasia may be present in BWS as a result of fetal adrenal gland delay maturation. Permanent adrenal cortex cytomegaly may be responsible for hypercortisolism. Transient cortex persistence can lead to androgens elevation and produces large amounts of DHEA, that could explain DHEAS elevation similar to that in ACT.Bilateral adrenal hyperplasia is described as a feature of BWS, but, to our knowledge, there is no similar case in literature with these biochemical findings.References:1)Beckwith JB. Extreme cytomegaly of the adrenal fetal cortex, omphalocele, hyperplasia of kidneys and pancreas, and Leydig-cell hyperplasia: Another syndrome. West Soc Pediatr Res. 1963; 112)Carney JA, Ho J, Kitsuda K, et al. Massive neonatal adrenal enlargement due to cytomegaly, persistence of the transient cortex, and hyperplasia of the permanent cortex: findings in Cushing syndrome associated with hemihypertrophy. Am J Surg Pathol. 2012 ]]> <![CDATA[MON-LB035 Adrenal Venous Sampling in the Lateralization of Acth-Independent Cushing Syndrome With Bilateral Adrenal Masses: A Case With a 5-Year Follow-Up]]> <![CDATA[MON-LB038 Isolated Hypoaldosteronism Due to Autoimmune Adrenalitis in a Patient With Autoimmune Polyglandular Syndrome]]> <![CDATA[MON-LB040 Challenge in Diagnosing and Treating of Mediastinal Paraganglioma]]> <![CDATA[MON-LB037 Adrenocortical Carcinoma: A Case Report]]> <![CDATA[MON-LB039 Acute Paraneoplastic Cushing’s Syndrome in a Patient With Small Cell Lung Cancer and Co-Incidental Adrenal Adenoma: A Case Report]]> 4357 nmol/24h; RI: 99 - 378 nmol/24h) and serum ACTH (158 ng/l; RI: < 61 ng/l) were markedly elevated. While the MRI of the head demonstrated no pituitary pathology, the CT of thorax and abdomen revealed a pulmonary mass as well as an incidental right adrenal mass. Bronchoscopic biopsy of the pulmonary mass confirmed the suspected diagnosis of ACTH-producing small cell lung cancer. The dignity of the right adrenal mass remained unclear, since the radiologic features per se could not differentiate between adrenal adenoma and metastasis. Chemotherapy with cisplatin/etoposide and inhibition of steroidogenesis with ketoconazole were initiated, which largely controlled the hypercortisolism. Imaging studies after completion of two cycles of chemotherapy showed a tumor response with regression of the pulmonary mass. The right adrenal mass remained stationary under chemotherapy. Thus, an adrenal metastasis could be excluded as potential cause of the adrenal mass, suggesting an incidental adrenal adenoma as the most likely diagnosis. Conclusion: Paraneoplastic Cushing’s syndrome requires high clinical suspicion for early diagnosis, since many of the classical clinical features of hypercortisolism may still be absent, even if the underlying cancer is already advanced. In patients suspected to have Cushing’s syndrome a three-step diagnostic approach is recommended: (1) biochemical confirmation of hypercortisolism, (2) differentiation between ACTH-dependant and -independant hypercortisolism and (3) identification of its source. This approach helps avoiding misdiagnosis in patients who have both an ACTH-producing cancer and an adrenal adenoma [1]. References: [1] Lacroix et al. Cushing’s syndrome. Lancet 2015; 386: 913-927. ]]> <![CDATA[MON-LB041 Pheochromocytoma - Illusive Myriad of Symptoms]]>