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 had been unremarkable).

Further imaging demonstrated MIBG uptake in keeping with extensive lymphadenopathy above and below the diaphragm. In addition, Gallium-68 DOTATATE scanning showed uptake in lesions which were not MIBG avid.

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 four cycles at intervals of 2–3 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 therapy of a metastatic paraganglioma, refractory to conventional chemotherapy, with Lutetium- DOTATATE. Significant clinical improvement could be achieved with very low toxicity and treatment side effects.

68Ga-DOTATATE PET-CT Scan. Maximum intensity projection a) and fused coronal PET-CT image b) shows the extent of disease above and below the diaphragm. Multiple lymph nodes, both lungs and bones are involved. Lung metastases are demonstrated on axial PET c), CT d) and fused e) images.

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.