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[Neural crest derived retroperitoneal tumors. General review]
J Rodríguez García1, A Lancina Martín, F Gómez Veiga
1Servicio de Urología, Hospital Juan Canalejo, La Coruña.
Actas Urologicas Espanolas
|January 1, 1992
Summary
This study reviews 14 retroperitoneal neural crest tumors in adults, including pheochromocytoma and paraganglioma. Early diagnosis and treatment are crucial for managing these rare neuroendocrine tumors.
Area of Science:
- Oncology
- Endocrinology
- Pathology
Context:
- Neural crest-derived tumors in the retroperitoneal space are rare in adults.
- This series includes pheochromocytoma, paraganglioma, ganglioneuroma, and neuroblastoma.
- Understanding their diagnostic and therapeutic aspects is essential.
Purpose:
- To present a series of 14 retroperitoneal neural crest tumors in adult patients.
- To review and update diagnostic and therapeutic strategies for these tumors.
- To highlight clinical presentations and outcomes.
Summary:
- Five pheochromocytomas presented with hypertension and classic symptoms, confirmed by elevated catecholamines and imaging (CAT, ultrasound, 123MIBG).
- Six paragangliomas were functioning, often perirenal. Eleven tumors were benign.
- Two ganglioneuromas were incidental findings. One aggressive neuroblastoma showed poor prognosis despite multimodal treatment.
Impact:
- Highlights the diverse clinical presentations of retroperitoneal neural crest tumors.
- Emphasizes the importance of advanced imaging and biochemical tests for diagnosis.
- Underscores the challenges in treating aggressive neuroblastoma, even with comprehensive therapy.