ESPE Abstracts (2023) 97 P1-165

1Corporate Fund University Medical Center, Astana, Kazakhstan. 2Corporate Fund University Medical Center, Astana, Kazakhstan. 3National Research Cardiac Surgery Center, Astana, Kazakhstan


Intoduction: Pheochromocytoma is a neuroendocrine disease rarely encountered in childhood, and it’s complications are even less frequent among children. Catecholamine-induced cardiomyopathy (CICMPs) is a rare complication of pheochromocytoma with a reported incidence of 8–11 % of all patients. Symptoms are often accompanied by hypertension, which is most common among children. However, classical symptoms such as hypertension, paroxysmal headache, sweating, palpitation do not necessarily occur in all patients with CICMPs. In absence of clear signs of catecholamine excess, patients with non-ischemic and non-valvular cardiomyopathy should be considered as possible cases of CICMP.

Case Presentation: 14-year-old girl was admitted to the pediatric cardiology department with complaints of severe shortness of breath, excessive sweating, fatigue and weakness. According to outpatient records, EchoCG revealed a marked decrease in the contractility of the left ventricular myocardium with an ejection fraction of 17-19%. Initial observation and tests were consistent with diagnosis of dilated cardiomyopathy and heart failure NYHA Class 3:

Observation and tests Results
Subjective signs Severe shortness of breath during minor physical activity, profuse sweat
Objective signs Blood pressure 90/55mmHg, breath rate 25 per minute, heart rate 120 per minute
Fasting glucose 119.9 mg/dl
Insulin 10.94 mcU/mL
HOMA-IR 3.2
NT-proBNP 3858 pg/ml.
Cardiac MRI with contrast Fibrous subendocardial and intramural changes in the myocardium of the left ventricle, dilation and dysfunction of the left ventricle
Thoracic CT Hpertrophy of the left ventricle, limited pneumofibrosis of the lower lobe of the left lung
Abdominal ultrasound Neoplasm (5,2x3,9cm) in the projection of the the right adrenal gland

Consequently, patient was consulted by endocrinologist. Secondary survey:

Tests Results
Urine Metanephrines 21.21 mcg/day
Urine Normetanephrine 1447.20 mcg/day
Cortisol 929.50 nmol/l
ACTH 126.30 pg/ml
Aldosterone 810.34 pg/ml
NSE 13.58ng/ml
Abdominal MRI with contrast Volumetric formation (3.8x4.0х4.3cm, adrenal incidentaloma?) of the right adrenal gland and hyperplasia of the left adrenal gland.

Therefore, patient was diagnosed with pheochromocytoma, catecholamine-induced cardiomyopathy, heart failure NYHA Class 3. Subsequently, laparoscopic tumoradrenalectomy was performed. Patient was later transferred back to pediatric cardiology department, for further heart failure and DCM treatment. Tertiary survey:

Tests Results
Pathohistological examination of the neoplasm corresponds to a pheochromocytoma.
Immunohistochemical examination corresponds to a benign pheochromocytoma.

Discussion: This case shows necessity of alertness for early detection of pheochoromocytoma and it’s complications in children. Considering, the size of the tumor, and hyperplasia of the left adrenal gland, genetic study for MEN2 is necessary.

Volume 97

61st Annual ESPE (ESPE 2023)

The Hague, Netherlands
21 Sep 2023 - 23 Sep 2023

European Society for Paediatric Endocrinology 

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