We present 51 yr. Female Caucasian patient who, in 8/14 while on vacation became aware that her clothes became tight. She was well, had consulted a Cardiologist 12 months previously with an episode of palpitations (assumed SVT), which did not recur with reduced caffeine.
She had no signs of cortisol or androgen excess. Abdominal examination, Imaging and Biochemistry LUQ 24x20x17 cm complex retroperitoneal vascular mass separate to normal solid abdominal organs with pulmonary nodules and elevated Plasma and urinary catecholamines. Imaging biomarkers showed a mixed pattern of MIBG, SSR (Dotatate) and FDG avidity in primary, lung, liver and bone metastases. Oncological resection of 26 cm tumour with extra-adrenal invasion, Ki67>3%, PASS 20/20. Genetic screening was negative for all mutations.
She remained well in full employment during 12 mo therapy with 4 x I131 MIBG (35 GBq) and Lu177 Dotatate (7.4 GBq) as normal marrow. The metanephrines normalized with Recist response to liver, lung and some bone metastases; a large lytic painful scapular metastasis was treated with radio- and cryo-therapy.
Sunitanib was declined for residual FDG positive small lung metastases due to her concerns over benefit and side effects; she remained clinically stable and continued to work.
At 20 mo. she contracted H1N1 influenza on vacation, became short of breath and at CTPA multiple large pulmonary metastases and mediastinal nodes. In absence of clinical trials Immunomodulating therapy was not offered, as the tumour was PDL1, PD1 negative.
She proceeded to palliative care and died 24 mo. after presentation.
This patient demonstrates the increasing importance of integrating all histological, biochemical and imaging biomarkers in therapy choice to optimize patient quality of life during survival. We postulate the precipitation of relapse by viral infection with possible immune suppression.