Background: Pheochromocytomas and paragangliomas are rare neuroendocrine tumors. Furthermore, only about 10-20% of these tumors are diagnosed in children, who usually present with symptoms from catecholamine hypersecretion or... [ view full abstract ]
Background: Pheochromocytomas and paragangliomas are rare neuroendocrine tumors. Furthermore, only about 10-20% of these tumors are diagnosed in children, who usually present with symptoms from catecholamine hypersecretion or tumor mass effect (1, 2). We present a report of laparoscopic appendectomy in a healthy child complicated by perioperative hypertension later attributed to an undiagnosed paraganglioma.
Case Description: A previously healthy 10-year-old (30.5kg) female presented to PMD with 1-day history of headache and RLQ pain. She was sent to ED from clinic for BPs as high as 170s/130s. Patient reported headache and vomiting for 2 days 2 weeks ago and had not returned to baseline health since. She also endorsed intermittent night sweats. ROS was otherwise negative and labs only showed elevated WBC. Abdominal ultrasound in ED revealed significant right hydronephrosis and hydroureter, patent bilateral renal arteries, and acute appendicitis. TTE showed mild LVH. Patient was admitted for further hypertension work-up and acute appendicitis. Her BP improved to 120s/70s with scheduled oral isradipine. She was added on for laparoscopic appendectomy on hospital day 2. Immediately after induction using modified RSI, BP was 204/145 and she received nitroglycerine 100 mcg, additional propofol bolus, and high sevoflurane. Immediately after intubation, BP was 130s/60s. Additionally, EKG was noted to be irregular with evidence of sinus rhythm, junctional rhythm, and possible delta wave with HR in the 80s, which resolved prior to incision. Throughout the surgery, her BP remained labile and persistently elevated ranging 130s-170s/70s-120s with HR ranging 80s-100s requiring hydralazine and esmolol boluses. A vascular mass behind the uterus was later noted by surgeon. After extubation in the OR, she had persistent hypertension, tachycardia, and episodes of desaturation when attempting to wean from nonrebreather facemask; decision was made to admit patient to the PICU. Nicardipine drip was also started post-operatively with improved BP control. She was conservatively managed with oral CCB and ACEI, which only controlled her BP marginally, while undergoing further evaluations. CT urogram showed an enhancing mass, possibly arising from the adnexa, abutting the uterus and bladder wall and compressing the right distal ureter. A week later her serum and urine catecholamines, urine metanephrines and VMA returned consistent with paraganglioma. She also underwent nuclear imaging, which ruled out metastasis. Alpha and beta blockers were started and uptitrated. She returned for tumor excision 33 days later. The surgery was uneventful and pathology confirmed extra-adrenal paraganglioma. Patient continues to do well postoperatively.
Discussion: Reports of intra-operative presentation of undiagnosed pheochromocytoma and paragangliomas are relatively rare. Even fewer descriptions are found for the pediatric population. Furthermore, not all of these patients have a history of hypertension pre-operatively. Fifty percent of deaths associated with undiagnosed pheochromocytoma occur peri-operatively or during parturition. Twenty-seven percent of patients with undiagnosed pheochromocytoma died during or shortly after surgery in a case series on the topic. Though quite rare, given these facts it is always important to consider undiagnosed catecholamine secreting tumors in the event of otherwise unexplained severe intra-operative hypertension.
