Paraganglioma: Gallium-68 DOTOTATE PET CT scan superior to MIBG scanning in metastatic disease.
J Joharatnam, R W Carroll, A Ghaffar, R Sharma, K Meeran, J F Todd
Imperial Centre for Endocrinology
FC is a 31 year old male who presented to his local hospital in May 2010 with a two month history of right sided abdominal pain, profuse night time sweating, and a mass in his supraclavicular area. After several presentations to A&E he had neck/thoracic/abdominal /pelvic CT scan showing extensive para-aortic, neck, retroperitoneal and supra-clavicular lymphadenopathy. His supraclavicular node was biopsied urgently and revealed a metastatic paraganglioma. There was no family history of neuroendocrine tumours.
24h urine collection revealed a raised total urinary noradrenaline of 2.88 umol/total (NR <0.5), and total urine dopamine 36.41 umol/total (NR <2.7) with normal total urine adrenaline 0.03 umol/total (NR <0.1). His total urine normetanephrine was 11.97 umol/total (NR <1.95), total urine metaneprine was 12.7 umol/total (NR <3.47) with a calcitonin of 1.7 ng/L (NR <11.8). His blood pressure was 142/54 and he was commenced on phenoxybenzamine 10mg bd.
An MIBG scan was performed showing widespread uptake in the supraclavicular fossae, mediastinum and retroperitoneum in keeping with extensive lymphadenopathy demonstrated on the CT scan. A subsequent gallium-68 DOTOTATE PET CT scan showed very avid nodal disease and uptake above and below the diaphragm including pulmonary and bone lesions. Interestingly, several of these lesions were not seen on MIBG scanning.
After discussion at an Endocrine MDT in conjunction with the oncologists, radiologists and histopathologists the options of MIBG therapy, Lutetium-177 therapy and chemotherapy were discussed. It was decided that the first step in management should be conventional chemotherapy in the form of cisplatin and etoposide. He is also awaiting the results of genetic screening to assess whether he has an SDHB or D mutation known to cause paraganglinoma/pheochromcytoma susceptibility syndromes.
This case highlights the multiisciplinary approach to the complex management of these patients and also suggests that gallium-68 DOTOTATE PET CT scan is more sensitive than MIBG in patients with metastatic paraganglioma syndromes and also gives a potential treatment option in the form of Lutetium 177 therapy in the future.