Pheochromocytomas and paragangliomas in children: Data from the Italian Cooperative Study (TREP): Pediatric Blood and Cancer

C. Virgone, M. Andreetta, S. Avanzini, S. Chiaravalli, D. De Pasquale, A. Crocoli, A. Inserra, P. D'Angelo, R. Alaggio, G. Opocher, G. Cecchetto, A. Ferrari, G. Bisogno, P. Dall'Igna

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Background: Pheochromocytomas (PCs) are neuroendocrine tumors arising from the chromaffin cells of the adrenal gland, and paragangliomas (PGLs) are their extra-adrenal counterparts arising from ganglia along the sympathetic/parasympathetic chain. Surgery is the cornerstone of treatment. A sporatic or inherited germline mutation is commonly associated. Materials and methods: Among over 1000 patients registered into the Tumori Rari in Età Pediatrica—rare tumors in pediatric age project—from 2000 to 2019, 50 were affected by PC/PGL. All clinical and therapeutic data were evaluated. Results: Twenty-eight patients had PC and 22 had PGL. Age at diagnosis ranged between 5 and 17 years. Thirty-five patients had symptoms related to catecholamine hypersecretion; in 7 of 50 patients, diagnosis was incidental or done during assessment of a familial syndrome. In all cases, conventional imaging was effective to assess the presence of a tumor. In addition, 18 of 38 functional imaging studies were positive (61%). Forty-eight patients were eligible for surgery: a complete resection was more frequently achieved in PC than in PGL (26/28 vs 11/22). All relapses were treated with surgery alone, surgery plus medical treatment, or chemotherapy alone; one PC with metastasis at diagnosis received radiotherapy only. Forty-four patients were in the first, second, or third complete remission (10/50 recurred; 8/10 carried a germline mutation). Five of 50 patients were alive with disease. One patient died of disease. Conclusions: Surgery can be curative in most tumors but it may not be always effective in removing PGLs. Severe postsurgical sequelae may affect these patients. Genetic tests should always be considered in individuals affected, and genetic counseling should be offered to their families. © 2020 Wiley Periodicals, Inc.
Original languageEnglish
JournalPediatr. Blood Cancer
Issue number8
Publication statusPublished - 2020


  • children
  • paraganglioma
  • pheochromocytoma
  • rare cancer
  • (3 iodobenzyl)guanidine i 123
  • 6 fluorodopa f 18
  • catecholamine
  • cisplatin
  • dactinomycin
  • doxazosin
  • doxorubicin
  • etoposide
  • glucocorticoid
  • ifosfamide
  • prazosin
  • vincristine
  • abdominal pain
  • acute heart failure
  • adjuvant chemotherapy
  • adolescent
  • adrenal insufficiency
  • adrenalectomy
  • Article
  • asthenia
  • bilateral cancer
  • body weight loss
  • bone metastasis
  • bradycardia
  • cancer chemotherapy
  • cancer radiotherapy
  • cancer surgery
  • cardiomyopathy
  • catecholamine release
  • child
  • clinical article
  • clinical feature
  • conversion to open surgery
  • diaphoresis
  • dyspnea
  • event free survival
  • external beam radiotherapy
  • facial nerve paralysis
  • female
  • fever
  • follow up
  • germline mutation
  • headache
  • heart arrest
  • Horner syndrome
  • human
  • hypertension
  • hypertensive crisis
  • hypotension
  • laparoscopy
  • lung edema
  • male
  • neoadjuvant chemotherapy
  • neurofibromatosis type 1
  • neurogenic bladder
  • otalgia
  • overall survival
  • pancreas fistula
  • positron emission tomography-computed tomography
  • priority journal
  • relapse
  • seizure
  • spinal cord metastasis
  • tachycardia
  • thorax pain
  • treatment response
  • tumor regression
  • adrenal tumor
  • clinical trial
  • Italy
  • metastasis
  • multicenter study
  • preschool child
  • prospective study
  • tumor recurrence
  • Adolescent
  • Adrenal Gland Neoplasms
  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Neoplasm Metastasis
  • Neoplasm Recurrence, Local
  • Paraganglioma
  • Pheochromocytoma
  • Prospective Studies


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