Approach to pheochromocytoma and paraganglioma in children
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Multidisciplinary approach is necessary to achieve safe surgical treatment and surveillance of pheochromocytoma (PCC) and paraganglioma (PGL), according to a study by Hacettepe University researchers.

They reviewed clinical records of all patients operated for PCC and PGL between 2000 and 2020 retrospectively, to present the clinical characteristics, surgical management and outcome of childhood PCC and PGL in a tertiary care centre.

During the study period, 18 children were operated for PCC and PGL. Both male and female were equally involved.
Participants’ median age at diagnosis was 13 years.

The most common presenting symptoms seen among the participants were headache and diaphoresis. Hypertension was the most common sign. Three patients had von Hippel-Lindau (VHL). Tumours of two patients with VHL were detected during routine follow-up. Three patients had multifocal disease.

Medical preparation for surgery was carried out in all patients. Antihypertensive treatments were administered preoperatively.High salt diet was recommended to the participants, since they were at risk for postoperative hypotension due to chronic vasoconstriction and blood volume contraction. Intravenous normal saline at a rate of 3000 ml/m 2 body surface area per day was started for intravascular volume expansion preoperatively.

The mean duration for preoperative medication to achieve normal blood pressure was 22 days.

A total of 25tumours were excised in eighteen patients. One patient who had bone metastases on diagnosis was on Iodine-131 meta-iodobenzylguanidine (I-131 MIBG)therapy.
The median follow-up time was 5.6 years.

During the study period, 5 patients reached adulthood. Among them, four had recurrent metastases and new tumours (pancreatic neuroendocrine tumour, and pancreatic neuroendocrine tumour and renal cell carcinoma) after the age of 18 years.

Based on these findings, researchers concluded, “Multidimensional approach is mandatory to achieve safe surgical treatment and surveillance of PCC and PGL.” “Detection of associated familial cancer susceptibility syndromes and long-term follow-up is essential to detect late recurrences and new tumours,” they added.

Source:
https://www.jpurol.com/article/S1477-5131(21)00074-7/pdf
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