A 21-Year Old Woman with Pheochromocytoma Associated with Hypercalcemia: A Case Report

Review Article

J Endocr Disord. 2014;1(2): 1009.

A 21-Year Old Woman with Pheochromocytoma Associated with Hypercalcemia: A Case Report

Evgeny Farber, Nakhoul Nakhoul, Gershovich Maria, Ben-Itzhaq Ofer, Klouger Yoram and Nakhoul Farid*

Nephrology & Hypertension Division, Bar Ilan University, Israel

*Corresponding author: Farid Nakhoul, Nephrology and Hypertension Division, Baruch padeh poriya Medical center, Faculty of Medicine in Galilee, Bar Ilan University, Israel

Received: Aug 13, 2014; Accepted: Aug 30, 2014; Published: Aug 30, 2014


We experienced a case of Pheochromocytoma (PCC), which was found in a 21-year-old woman with hypercalcemia. The leading symptom was abdominal pain and dysmenorrheal. Large ovarian cyst was diagnosed by ultrasound. During induction anesthesia for ovarian cystectomy, the woman developed tachycardia and severe hypertension. Her familial histories were unremarkable. Laboratory examinations showed hypercalcemia (12.5 mg/dl) which was diagnosed 3 years before her last admission to our hospital. Computed tomography showed a heterogeneous mass measuring 12 cm in the left adrenal gland, which had abnormal uptake with 123-I Metaiodobenzylguanidine scintigraphy (MIBG). Serum/urine catecholamines were highly elevated, and serum PTH was mildly increased. The patient underwent open abdominal surgery with En bloc surgical resection of PCC with left adrenalectomy and ovarian cystectomy. The tumor was histological diagnosed as typical pheochromocytoma. Blood pressure was normal with serum calcium slightly decreased after surgery. She was free of disease postoperatively after 6 months of follow up.

Keywords: Pheochromocytoma; Hypercalcemia; Ovarian Cyst


Pheochromocytoma and paraganglioma represent the catecholamine-producing tumor arising from the chromaffin cells of the sympathetic ganglia in the adrenal medulla and extra-adrenal tissue, respectively [1-3]. These infrequent tumors are classically known to present with episodic hypertension, tachycardia, headache, and diaphoresis. Pheochromocytoma is diagnosed by elevated catecholamine and metanephrines in the serum/urine [4-6], and imaging studies including Computed Tomography scanning (CT) and 123-I Metaiodobenzylguanidine (MIBG) scintigraphy [7-9]. Although the majority of pheochromocytomas are sporadic/nonfamilial, a growing population of them has recently been found with involvement in familial genetic syndromes, such as multiple endocrine neoplasia type 2, von Hippel–Lindau disease, neurofibromatosis type 1, and paraganglioma syndrome types 1, 3 and 4 [11,12]. Although pheochromocytoma in childhood is rare, it represents the most common pediatric endocrine tumor.

The only definitive treatment consists of surgical resection. During manipulation of the tumor, dangerous amounts resulting in life-threatening events, including hypertensive crises, cardiac arrhythmias, myocardial infarction or ischemia, pulmonary edema and multiorgan failure [13,14]. Furthermore, the rapid decrease of catecholamine after surgery may result in severe hypotension. To prevent these life-threatening events from happening, preoperative management has been recommended. One of these therapies is the use of alpha-adrenoceptor blockers, which can counter the adrenergic effects of catecholamines. In addition, alpha- blockade permits intravascular volume expansion. Nowadays, some centers use the non-selective alpha-blocker phenoxybenzamine, while others use the selective alpha-blocker doxazosin [14,15]. A beta-blocker is often added to phenoxybenzamine therapy in order to decrease chronotropic activity.

Herein, we describe a case of active pheochromocytoma, which was found in a 21-year-old woman with persistent humeral hypocalcaemia, without any evidence of primary hyperparathyroidism by the routine screening. After adrenalectomy blood pressure was normalized but with persistent hypocalcaemia and high plasma PTH. Her family history was negative for arterial hypertension and syndromes [16].

Case Report

A 21-year-old woman with an unremarkable medical history, a part from taking hormonal treatment because of dysmenorrheal six month before admission. She denied any palpitations, chest pain or panic attacks. Her family history was negative for arterial hypertension. On 2010 she was diagnosed as suffering from hypocalciuric hypocalcaemia, but she was asymptomatic and no further investigation or follow up was done. She was admitted to the gynecological ring because of dysmenorrheal and abdominal pain after 3 month of hormonal treatment. Elective laparoscopic ovarian cystectomy by general anesthesia was recommended.

At admission blood pressure was 120 mmHg systolic and 80 mmHg diastolic with normal sinus rhythm of 72 beats/minute. Blood test showed plasma Creatinine of 0.7 mg/dl and normal electrolytes. Plasma calcium was 12 mg/dl with PTH of86 pg/ml. Tran abdominal sonography showed large ovarian cyst of 8cm.Chest X-Ray and normal electrocardiogram without left ventricular hypertrophy. Blood and urine tests are summarized in Table 1A &B. Serum level of 1, 25-dihydroxyvitamin D3 was normal.