ESPE Abstracts

Introduction: Catecholamine-secreting tumors typically present with episodic palpitations, sweating, flushing, and hypertension. However, patients may infrequently complain of excessive water consumption. The symptoms of sweating, increased water intake, flushing, palpitations, and weight loss can be attributed to catecholamines produced by the tumor tissue.

Case Report: A seven-month-old male patient presented with episodes of excessive thirst and water consumption with sweating for three months. During these episodes, there was an increase in water consumption and sweating, without an associated increase in urine output. Physical examination revealed restlessness, increased thirst, flushing, and sweating. During an episode, the patient’s body temperature was 36.2°C, blood pressure was 100/60 mmHg, and pulse was 150 beats per minute. Diabetes insipidus was ruled out as the patient's urine density was 1020 and serum sodium level was 138 mEq/L as well as episodic nature of the disease. Plasma and urine samples were obtained during an episode of hypertension to assess for potential catecholamine release. Spot urine normetanephrine, noradrenaline, dopamine, homovanillic acid, vanillylmandelic acid levels, as well as plasma adrenaline and serum neuron-specific enolase levels, were quantified using liquid chromatography-tandem mass spectrometry (LC-MS) and elevated levels (Table 1). Plasma levels of normetanephrine and metanephrine could not be determined due to the unavailability of the kit. Abdominal ultrasonography (USG) and magnetic resonance imaging (MRI) revealed a suspicious tumor measuring 43x55 mm compressing the upper pole of the kidney. The mass exhibited hyperechoic areas on USG and heterogeneously hypointense areas on MRI. Two weeks prior to tumor excision, the patient, suspected of having either neuroblastoma or pheochromocytoma, began treatment with two doses of 0.5 mg prazosin. The pathological examination of the mass revealed "poorly differentiated type neuroblastoma, good histological prognostic category." Given the patient's age (<18 months), negative N-myc amplification, absence of bone marrow involvement, and lack of metastasis or residue, the neuroblastoma was classified as "stage L2" and "low-risk category" according to the Turkish Pediatric Oncology Group Neuroblastoma 2020 protocol. Consequently, the patient was followed up without chemotherapy.

Conclusion: Endocrine hypertension in children often presents without symptoms, but recurrent episodes are distinctive. In addition to hypertension, the presence of increased water intake during the attack warrants evaluation for catecholamine-secreting tumors.