ECE2018 ePoster Presentations Adrenal and Neuroendocrine Tumours (28 abstracts)
14th Department of Internal Medicine Hematology and Endocrinology Charles University Hospital and Faculty of Medicine in Hradec Králové, Hradec Králové, Czech Republic; 2Third Internal Clinic Clinic of Endocrinology and Metabolism, General University Hospital in Prague, Prague, Czech Republic; 3Charles University in Prague, Prague, Czech Republic; 4Department of Nuclear Medicine, Charles University Hospital and Faculty of Medicine in Hradec Králové, Hradec Králové, Czech Republic.
Introduction: Paragangliomas are rare neuroendocrine tumors that arise from the extraadrenal paraganglia. Sympathetic paragangliomas usually secrete catecholamines and are located in the sympathetic paravertebral ganglia of thorax, abdomen and pelvis.
Case report: We present a 66 year old patient with an incidentally found retroperitoneal mass on spine MRI. An open biopsy was performed (9/15) with the histological finding of benign paraganglioma (according to histopathology report from the local pathology). No further controls were provided. In 2017 during MR of the spine (after a sport trauma) progression of the tumor size was found and hormonal evaluation showed significantly increased norepinephrine, normetanephrine and chromogranin levels. Patient presented with paroxysmal hypertension, episodic sweating and tachycardia. 18F-FDG PET/CT showed numerous bone metastatic lesions of spine and ribs. No tumor lesion took up 123I-MIBG and somatostatin receptor scintigraphy is planned to decide if the patient is suitable for peptide receptor radiotherapy or systemic chemotherapy. Due to bone involvement, bisphosphonate therapy was initiated. Results of genetic examination are still pending.
Conclusion: Paragangliomas are rare neuroendocrine tumors. In this particular case, misleading pathological report led to incorrect therapeutic strategy since this tumor type can never be regarded as benign.