Endocrine Abstracts

Introduction: We report the unusual case of a young patient with micturition induced headache, in which contrast-enhanced ultrasound helped to assure diagnosis of paravesical paraganglioma.

Case: An 18-year-old male presented with attacks of severe headache lasting several minutes and occurring strictly after micturition. Hypertensive values up to 200/105 mmHg were detected subsequent to nearly every micturition while values before and some minutes after it were normal.

A paravesical tumor with regressive changes, measuring 3.7×3 cm, was seen by B-mode ultrasound and confirmed by MRI. Contrast-enhanced ultrasound was performed. After i.v. injection of 2.4 ml SonoVue, the tumor showed an early arterial contrast enhancement with peripheral to central contrast filling and a distinct, irregular hypervascularisation – signs which have been described as typical for pheochromocytomas before.

Plasmametanephrines revealed a distinct elevation of normetanephrine (1934 pg/ml, norm <180), so we diagnosed a functional paravesical paraganglioma. The patient had no family history for phaeochromocytoma or other endocrine tumors. A Fluorodopamine (F-Dopa)-PET CT was performed with uptake of F-Dopa confined to the paravesical tumor but not in further locations. After adrenergic blockage with Urapidil the tumor was successfully removed. Mutational analysis of SDHB, VHL, SDHD and RET was performed without any pathologies.

Conclusions: There are only few reports of functional paravesical paragangliomas which are very rare tumors (less than 1% of all pheochromocytomas). However, to avoid serious complications including hypertensive crisis during operation, it is important to consider these tumors in the differential diagnosis of paravesical tumors. Contrast-enhanced ultrasound can be a cost-effective, less-invasive method to support diagnosis.

Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.

Funding: This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.