[Neural crest derived retroperitoneal tumors. General review].
Presentation of a series of 14 cases of neural crest derived tumours located in the retroperitoneal space in adult patients (five pheochromocytoma, six paraganglioma, two ganglioneuroma, and one neuroblastoma), and review and update of the diagnostic and therapeutic aspects. All pheochromocytoma cases presented high BP and the classic triad of sudation, tachycardia and headaches, as well as high levels of blood and urine catecholamines and/or their metabolites. CAT, ultrasound scanning and 123MIBG were the main diagnostic techniques used. All four paraganglioma were functioning and generally located surrounding both kidneys (one case was paired). No malignancy was found in any of the 11 tumours while controls remain with normal BP and normal levels of urine catecholamine metabolites. None of the two ganglioneuromas showed specific signs and symptoms but were diagnosed accidentally. The one neuroblastoma was juxtavesical showing a highly unfavourable evolution in spite of radical surgery, radiotherapy and multiple chemotherapy and the patient died within 16 months with local recurrence and haematogenous dissemination to bones and lungs.
Rodríguez García
J
J
Servicio de Urología, Hospital Juan Canalejo, La Coruña.
Lancina Martín
A
A
Gómez Veiga
F
F
Chantada Abal
V
V
Picallo Sánchez
J
J
González Martín
M
M
D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009380:Neoplasms, Nerve Tissue / Q000175:diagnosis / Q000628:therapy; D012186:Retroperitoneal Neoplasms / Q000175:diagnosis / Q000628:therapy
Tumores retroperitoneales derivados de la cresta neural. Revisión de conjunto.