Pheochromocytoma Presenting Research Articles

MON-375 Chronic Hypertension Causing by Adrenal Medulla Hyperplasia Mimicking the Presentation of Pheochromocytoma

Background: Well-known causes of endocrine hypertension include primary hyperaldosteronism, pheochromocytoma, Cushing’s syndrome, etc. (1). In the international literature, adrenal medullar hyperplasia (AMH) has not been familiar for the clinicians as a cause of hypertension, and it possibly leads to misjudgment in the clinical treatment/outcomes. We reported an unusual case of resistant and prolonged hypertension caused by AMH. Clinical case: A 49-year-old female was referred to the Endocrinology clinic for possible having pheochromocytoma with prolonged hypertension. The patient had hypertension for 10-15 years which she is currently on metoprolol, losartan, clonidine, hydrochlorothiazide, and amlodipine for her home regimen. She also complained of having episodic hypertension which was associated with headaches, diaphoresis, palpitations, and chest pain for several years. Her blood pressure has been ranging from 100-160 over 60 -115 mmHg even though she is compliant to the medications. Also, she once was admitted to the hospital because of hypotension and needed to be taking care of in the intensive care unit. Her past medical history was significant for diabetes mellitus type 2 and anxiety. On physical examination, she had elevated blood pressure of 170/115 mmHg. The patient had no enlarged thyroid, no cushingoid appearance and denied using steroid supplements. Initial workup revealed elevated normetanephrines level in 24-hr urine and serum (1070 mcg/g; n 85-514 mcg/g and 477 pg/mL; n <148 pg/mL). In contrast with metanephrines in 24-hr urine and serum which were in normal range (154 mcg/g; n 33-192 mcg/g and <25 pg/mL; n <57 pg/mL). Serum aldosterone and cortisol level were within normal limit. An abdominal CT scan with contrast showed a 15 mm. mass was noted in the left adrenal gland with a density of -22 HU in the non-contrast image, 27 HU in portal venous phase, and 15 HU in the delay images. Pheochromocytoma was suggested at that time. The patient underwent a left adrenalectomy and pathology report revealed adrenal medullary hyperplasia. Postoperatively, her blood pressure is in the normal range, and she is able to discontinue taking all the anti-hypertensive medication. Discussion: AMH is a rare and frequently unrecognized disorder that causes hypertension. The etiology of AMH remains unknown and still unclear in etiology along with pathogenesis. Currently, there is no standard evaluation and treatment guideline for this condition. Researchers still debate that it may be a precursor lesion of pheochromocytoma since their presentation can be similar. Histopathology is still a definite diagnosis to determine the difference between both these conditions.

Phaeochromocytoma presenting as an adrenal haemorrhage

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

A dangerous master of disguise - An undiagnosed phaeochromocytoma presenting with pulmonary haemorrhage

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

Abstract #109 A Subtle Presentation of Pheochromocytoma: the Utility of Ambulatory Blood Pressure Monitoring

Abstract #109 A Subtle Presentation of Pheochromocytoma: the Utility of Ambulatory Blood Pressure Monitoring

Pheochromocytoma presenting with severe hyperglycemia and metabolic acidosis following intra-articular glucocorticoid administration: a case report

BackgroundThere are several reports of pheochromocytoma crisis triggered by systemic glucocorticoid administration. However, pheochromocytoma crisis after intra-articular glucocorticoid administration has been rarely reported.Case presentationA 45-year-old Japanese man presented to our hospital with a sudden, severe headache. He had no history of diabetes. He had received an intra-articular injection of betamethasone (2 mg) for joint pain, 2 days prior to his admission. On examination, his blood pressure was 240/126 mmHg and pulse was 120 beats/minute. The possibility of cerebrovascular events was ruled out by imaging studies and lumbar puncture. Blood tests revealed severe hyperglycemia (523 mg/dL) and metabolic acidosis (pH 7.21, anion gap 26.2 mEq/L, lactate 11.75 mmol/L) with a glycosylated hemoglobin level of 5.7%. Although a urine sample could not be obtained, fulminant type 1 diabetes mellitus and diabetic ketoacidosis were suspected based on these findings. However, after the initial treatment for diabetic ketoacidosis, his insulin secretion was found to be normal and the plasma levels of ketones were not elevated. This excluded the possibility of fulminant type 1 diabetes mellitus and diabetic ketoacidosis. Subsequently, a left adrenal gland tumor and elevated levels of serum catecholamine and urinary catecholamine metabolites were detected, while his other hormone levels were normal. Serum catecholamine levels did not decrease following the clonidine test, and a functional scintigraphy using iodine-131 metaiodobenzylguanidine showed strong uptake in the region of the left adrenal gland. Although no signs of pheochromocytoma crisis, such as paroxysmal hyperglycemia and hypertension, had been observed since admission, a pheochromocytoma was diagnosed based on the investigations. After controlling his blood pressure, a left adrenalectomy was performed.ConclusionsThis case illustrates that intra-articular glucocorticoid administration can induce a pheochromocytoma crisis and an increase in hyperglycemia, and that pheochromocytoma crisis can resemble the clinical picture of fulminant type 1 diabetes mellitus owing to severe hyperglycemia with metabolic acidosis and normal glycosylated hemoglobin levels, especially under the influence of glucocorticoid.

Synchronous renal cell carcinoma and pheochromocytoma presenting as acute decompensated heart failure

We report a 49-year-old woman who presented with a hypertensive crisis and acute heart failure and reduced left ventricular systolic function. An abdominal ultrasonography revealed a huge lobulated heterogeneous mass at the lower pole of the right kidney and a mass over the left suprarenal area, which were further delineated by magnetic resonance imaging. The patient underwent laparoscopic right radical nephrectomy and left adrenalectomy. Histopathological analysis confirmed the diagnoses of clear cell renal cell carcinoma of the right kidney with metastasis to the lung; and atypical pheochromocytoma of the left adrenal gland. Target therapy was initiated, which resulted in stabilization of the patient's tumors and the recovery of her heart function. To avoid a delayed diagnosis and catastrophic outcome, clinicians should consider such rare causes of acute decompensated heart failure.

Clinical Presentation, Diagnosis, and Management of Primary Aldosteronism and Pheochromocytoma

Clinical Presentation, Diagnosis, and Management of Primary Aldosteronism and Pheochromocytoma

Not So Incidental: Pheochromocytoma in an Adult with Unrepaired Cyanotic Heart Disease

Pheochromocytoma, a rare neuroendocrine tumour, is often encountered in the general internal medicine clinic as an adrenal incidentaloma. Even rarer is its presence in adult cyanotic heart disease, although there are a few documented reports of this association in various paediatric populations, with chronic hypoxia being the likely driving force. Here we present the case of a 38-year-old adult with unrepaired complex cyanotic congenital heart disease with biochemically proven pheochromocytoma presenting as an adrenal incidentaloma to a general internal medicine clinic.LEARNING POINTSThere is an association between pheochromocytoma and congenital cyanotic heart disease.It is important to maintain clinical vigilance for laboratory testing for pheochromocytoma in patients with cyanotic heart disease who are seen at general internal medicine clinics.

A Rare Location and Presentation of Pheochromocytoma.

Background: Pheochromocytomas typically are diagnosed in the adrenal gland and from the sympathetic nervous system. Bladder pheochromocytoma is a rare location for this tumor.Case Presentation: We describe a 67-year-old Afro Caribbean woman referred to our hospital for an asymptomatic bladder tumor. Preliminary transurethral resection revealed bladder pheochromocytoma. After a comprehensive endocrine evaluation, we performed a robot-assisted laparoscopic partial cystectomy with ureteral reimplantation.Conclusion: We present a rare case of bladder pheochromocytoma treated effectively with minimally invasive techniques. When confronted with a solid bladder mass, apart from the more common urothelial malignancies, a differential diagnosis of bladder pheochromocytoma should also be considered.

Challenges for endocrine surgeons: management of a colossal normotensive pheochromocytoma

Although the presentation of pheochromocytoma is remarkably protean, it usually presents as sustained or paroxysmal hypertension with triad of headache, palpitation and diaphoresis. The Incidence of serendipitously discovered pheochromocytoma is approximately 4% to 8%. Perioperative management of such cases are not only controversial but also poses infallible challenges to the surgeon. This case report intends to highlight an unusual presentation of asymptomatic, normotensive pheochromocytoma as a huge abdominal mass and its management.

Genetic background as a predictive factor of pheochromocytoma and paraganglioma presentation

Genetic background as a predictive factor of pheochromocytoma and paraganglioma presentation

An atypical pheochromocytoma presenting with clinical sign and symptoms of non-mechanical bowel obstruction

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

Unusual presentation of pheochromocytoma: thirteen years of anxiety requiring psychiatric treatment

SummaryPheochromocytoma is a rare tumor of the adrenal gland. It often presents with the classic triad of headache, palpitations and generalized sweating. Although not described as a typical symptom of pheochromocytoma, anxiety is the fourth most common symptom reported by patients suffering of pheochromocytoma. We report the case of a 64 year old man who had severe anxiety and panic disorder as presenting symptoms of pheochromocytoma. After 13 years of psychiatric follow-up, the patient was diagnosed with malignant pheochromocytoma. After surgical resection of his pheochromocytoma and his hepatic metastases, the major panic attacks completely disappeared, the anxiety symptoms improved significantly and the psychiatric medications were stopped except for a very low maintenance dose of venlafaxine. We found in our cohort of 160 patients with pheochromocytoma 2 others cases of apparently benign tumors with severe anxiety that resolved after pheochromocytoma resection. These cases highlight that pheochromocytoma should be included in the differential diagnosis of refractory anxiety disorder.Learning points:Anxiety and panic disorder may be the main presenting symptoms of pheochromocytoma.The diagnosis of pheochromocytoma should be excluded in cases of long-term panic disorder refractory to medications since the anxiety may be secondary to a catecholamine-secreting tumor.Surgical treatment of pheochromocytoma leads to significant improvement of anxiety disorders.

A case of pheochromocytoma presenting as syncope due to long QT syndrome

Pheochromocytoma, a catecholamine secreting tumour, is rare and we are presenting such a case who presened with syncopal episodes due to arrhythmias associated with the tumour. The patient was managed with pharmacologic and surgical treatment.

A rare presentation of pheochromocytoma in pregnancy: a case report

BackgroundEarly diagnosis of pheochromocytoma and its proper management can lessen its mortality and morbidity. This case report describes a 24-year-old pregnant woman with an unusual presentation of pheochromocytoma.Case presentationAn Iranian 24-year-old primigravid woman from Kordistan province was referred to our center with left flank pain at 37 weeks of gestation. She had a history of gestational diabetes mellitus since the 12th week of gestation which was managed by insulin administration. She also had a history of pulsatile bi-temporal headache for 2 years prior to her referral to us. She underwent complete abdominal and pelvic ultrasound imaging for her flank pain. This examination revealed a heterogeneous mass of 119 × 87 × 79 mm above her left kidney, highly suspicious of being an adrenal-originating tumor. Subsequently, we consulted an endocrinologist. She underwent abdominopelvic magnetic resonance imaging and her 24-hour urine metanephrine, normetanephrine, and vanillylmandelic acid were assessed. Finally, the diagnosis of pheochromocytoma was confirmed. She underwent a cesarean section and adrenal mass excision at the 40th week of gestation. This timely diagnosis resulted in her proper management and good maternal and neonatal treatment outcomes.ConclusionsOur patient had pheochromocytoma during pregnancy. She had no complaints about hypertension before or during pregnancy until giving birth to her child; her only symptoms were a vague left flank pain, gestational diabetes, and headaches for the past 2 years. The unusual symptom of flank pain led to timely diagnosis and a good treatment outcome.

Initial clinical presentation and spectrum of pheochromocytoma: a study of 94 cases from a single center.

BackgroundWith the increasing access to imaging more pheochromocytomas are diagnosed in the workup of adrenal incidentalomas. This may have changed the occurrence of the classic presentation with hypertension and the classic triad (headaches, sweating and palpitation).MethodsWe reviewed 94 consecutive cases of pheochromocytomas. Two cases of ectopic ACTH-syndrome were subsequently excluded.ResultsOf the 92 cases included 64% had presented as an incidentaloma, 32% as a suspected pheochromocytoma and 4% had been screened because of previously diagnosed MEN2A. Those screened were youngest while those with incidentalomas were oldest. The females were more common in the incidentaloma and the screening groups, and males in the suspected pheochromocytoma group. Measurements of noradrenaline/normetanephrine levels were highest in the suspected pheocromocytoma group and lowest in the screening group. Hypertension was present in 63% of the incidentalomas, 79% of suspected pheochromocytomas and in none of the screening group. Paroxysmal symptoms were present in almost all with suspected pheochromocytoma while only in half of the other groups. The suspected pheocromocytoma group had most symptoms and the screening group least. The classic triad was present in 14% of the incidentalomas, in 28% of the suspected and in none of the screening group, while no symptoms at all was present in 12%, 0% and 25%, respectively. Pheochromocytoma crisis occurred in 5%. There was a positive correlation between tumor size vs hormone levels, and catecholamine levels vs blood pressure.ConclusionClinicians need to be aware of the modern presentation of pheochromocytomas since early identification can be life-saving.

Giant phaeochromocytoma presenting with an acute stroke: reappraising phaeochromocytoma surveillance for the neurofibromatosis type 1 phakomatosis

Neurofibromatosis type 1 (NF1) is a multisystem genetic disorder associated with reduced lifespan attributed largely to malignancy and vascular causes. One of the tumours associated with NF1 is phaeochromocytoma. The...

Evolving Clinical Presentation and Assessment of Pheochromocytoma: A Review

Evolving Clinical Presentation and Assessment of Pheochromocytoma: A Review

The Largest Malignant Pheochromocytoma: A Case Report and Literature Review

: Introduction: Malignant pheochromocytoma is a rare neuroendocrine tumor with an annual incidence of 3–8 cases per 1 million per year in the general population. Its clinical presentation can vary greatly. There are few published cases of giant pheochromocytomas. We report a case of the largest tumor in the world. The literature is reviewed to evaluate the spectrum of disease presentations and the available options for disease management and treatment. Case presentation: A 52-year-old male, originally from El Kelaa des Sraghna in the middle of Morocco, which had a 36cm mass of the left suprarenal gland with metastatic lesions. He was treated with a combination chemotherapy regimen of cyclophosphamide, vincristine and dacarbazine. Conclusion: A heterogeneous clinical presentation of malignant pheochromocytoma and a short response to chemotherapy had been observed in our case. However, controlled studies need to determine a standard of care.

Pheochromocytoma Presenting as Acute Cardiomyopathy and Multiorgan Failure in a Young Lady-Think Twice before Sending a Patient for Heart-Transplantation

We illustrate a case of pheochromocytoma-related cardiomyopathy in a young lady who presented with acute decompensated cardiomyopathy. Initially it was thought to be caused by mumps infection and heart-transplantation was planned. Through a collaborative effort among different specialists, the correct diagnosis was eventually reached. A novel management pathway lining up pre-operative embolization of tumor followed by adrenalectomy with ECMO was proven to be successful.