Pheochromocytoma and paraganglioma in children: a report of 24 cases of the French Society of Pediatric Oncology
| Autor: | Martin Schlumberger, Jean Lemerle, D Sommelet, Yann Revillon, Yves Perel, Patrick Lutz, L. de Lumley, J. F. Dyon, G. Marguerite, H Heloury, Françoise Flamant, Nathalie Alos |
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| Rok vydání: | 1997 |
| Předmět: |
Male
medicine.medical_specialty Adolescent Adrenal Gland Neoplasms Pheochromocytoma Neuroendocrine tumors Paraganglioma Medicine Humans Risk factor Multiple endocrine neoplasia Child Survival rate medicine.diagnostic_test business.industry Magnetic resonance imaging Hematology medicine.disease Combined Modality Therapy Surgery Treatment Outcome Oncology Tumor progression Pediatrics Perinatology and Child Health Female business |
| Zdroj: | Pediatric hematology and oncology. 14(5) |
| ISSN: | 0888-0018 |
| Popis: | Pheochromocytoma and paraganglioma of childhood are rare neuroendocrine tumors. Urinary catecholamine measurements, metaiobenzylguanidine (MIBG) scanning, computed tomographic scanning, and magnetic resonance imaging have greatly facilitated diagnosis. Prognosis after surgical resection is excellent. In this retrospective series collected from French oncology centers, the risk of tumor progression was studied in order to assess prognostic factors and the optimal diagnostic and therapeutic management. Medical records of 24 children with paraganglioma were reviewed. This tumor occurred at a median age of 12.5 years and in most cases was revealed by arterial hypertension. The diagnosis was made by the demonstration of urinary excretion of catecholamines and their metabolites. Six patients had bilateral adrenal pheochromocytomas; two patients had extra-adrenal paragangliomas. In eight patients, the paraganglioma occurred as a familial disease. Surgical excision was the only therapeutic procedure. With a follow-up of 5.2 years, 14 of the patients are still in first complete remission and 6 have developed metastases or shown tumor progression. Despite a high long-term survival rate, the risk of malignancy and of multifocal involvement is of concern and is associated with a significant rate of late events. The outcome depends on adequacy of tumor resection and must be serially assessed. |
| Databáze: | OpenAIRE |
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