Approach to pheochromocytoma and paraganglioma in children and adolescents: A retrospective clinical study from a tertiary care center

Autor: Ayfer Alikasifoglu, Mithat Haliloglu, Zeynep Alev Ozon, Berna Oguz, Feridun Cahit Tanyel, İdil Rana User, Arbay O. Ciftci, Diclehan Orhan, İbrahim Karnak, Mustafa Tezer Kutluk, Canan Akyüz, Nazli Gonc, Burak Ardicli, Saniye Ekinci
Rok vydání: 2021
Předmět:
Zdroj: Journal of Pediatric Urology. 17:400.e1-400.e7
ISSN: 1477-5131
DOI: 10.1016/j.jpurol.2021.01.043
Popis: Summary Aim Pheochromocytoma (PCC) and paraganglioma (PGL) are rare tumors in childhood. They are catecholamine secreting tumors and present with signs or symptoms related to their excess. Most common signs and symptoms are hypertension, headache and diaphoresis. The management of children usually depend on experience of adulthood. This study is conducted to present the clinical characteristics, surgical management and outcome of childhood PCC and PGL in a tertiary care center. Material and methods We reviewed clinical records of all patients operated for PCC and PGL between 2000 and 2020 retrospectively. Results There were 18 children operated for PCC and PGL in the study period. The female to male ratio was 1:1. The median age at diagnosis was 13 (IQR, 9–15) years. The most common presenting symptoms were headache and diaphoresis. Hypertension was the most common sign. Three patients had von Hippel-Lindau (VHL). Tumors 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. Since the patients are at risk for postoperative hypotension due to chronic vasoconstriction and blood volume contraction, high salt diet was recommended. Intravenous normal saline at a rate of 3000 ml/m2 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 (range, 16–30). Twenty-five tumors were excised in eighteen patients. One patient who had bone metastases on diagnosis and is on I131MIBG therapy. The median follow-up time was 5.6 years (range, 1 months - 21 years). Five patients reached adulthood during the study period. Four of these had recurrent metastases (n = 2) and new tumors (pancreatic neuroendocrine tumor, n = 1 and pancreatic neuroendocrine tumor and renal cell carcinoma, n = 1) after the age of 18. Conclusion Multidisciplinary approach is necessary 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 tumors. Summary Table . The clinical characteristics of the patients The median age (years) 13 Sex Male 9 (50%) Female 9 (50%) Tumor Unilateral PCC 13 (72%) Unilateral PCC + PGL (3) 1 (6%) Bilateral PCC + PGL 2 (10%) Only PGL 2 (10%) PGL site Paraaortic 5 Aortocaval 1 Level of renal hilus 1 The median size of tumors (cm) 4.5 Surgery Total excision 18 (100%) Outcome Disease free 14 (78%) Recurrent disease 1 (6%) Reccurent and Metastatic disease 1 (6%) Other tumor 2 (10%) Abbreviations: PCC, pheochromocytoma: PGL, paraganglioma: VHL, von Hippel-Lindau syndrome.
Databáze: OpenAIRE
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