Endocrine Abstracts

A 31 year old man presented with a two month history of sweating, right-sided abdominal pain and supraclavicular swelling. CT scanning showed widespread lymphadenopathy with multiple retroperitoneal lymph nodes. Biopsy of a left supraclavicular node resulted in a diagnosis of metastatic paraganglioma. Raised urinary catecholamine as well as urinary metanephrine levels further confirmed the diagnosis. A normocytic anaemia requiring repeated blood transfusions was believed to be due to bone marrow dysfunction. He was also found to have pituitary insufficiency as evidenced by hypgonadotrophic hypogonadism, undetectable serum prolactin levels and a suboptimal cortisol increase in a glucagon suppression test (Pituitary MRI was unremarkable). Further imaging demonstrated MIBG uptake in keeping with extensive lymphadenopathy above and below the diaphragm. In addition, Gallium-68 DOTATATE showed uptake in non-MIBG avid lesions. He initially completed four cycles of conventional chemotherapy consisting of cisplatin and etoposide as well as palliative radiotherapy to para-aortic nodes. He continued, however, to suffer from extensive lymphoedema, abdominal distension and difficulty in breathing which had necessitated hospital admission and home oxygen therapy. Rising chromogranin A levels as well as repeated CT scans confirmed disease progression. Therefore, conventional chemotherapy had been discontinued and he was commenced on Lutetium-DOTATATE treatment. Up to now, he has received 4 cycles at intervals of two to three months. His clinical response was excellent with gradual improvement of both symptoms and quality of life. Prior to his third cycle, the lymphoedema had disappeared and there was significant reduction in the size of overall metastatic disease on repeat CT scanning. He is no longer transfusion-dependent and his only remaining symptom is of occasional night sweats. This is a unique case which demonstrates successful treatment of a metastatic paraganglioma, refractory to conventional chemotherapy, with Lutetium- DOTATATE. Disease remission could be achieved with very low toxicity and treatment side effects.

Declaration of interest: There is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported.

Funding: No specific grant from any funding agency in the public, commercial or not-for-profit sector.