Dilemmas in Managing Metastatic Paraganglioma.

Thein Htay1, Sushmita Chakrabati1, Alison Wren1, Asha Miah2, Yong Du3, Daniel Morganstein1,2

1.Endocrinology Department Chelsea and Westminster Hospital

2.The Sarcoma Unit, The Royal Marsden Hospital

3. Nuclear Medicine Department, The Royal Marsden Hospital 


A 56 year old lady presented with increasing abdominal distension and constipation with episodes of intermittent sweats, anxiety and palpitations since the menopause. She has had hypertension for many years, alongside asthma, previous eclampsia, post-partum cardiac arrest which left her some degree of memory impairment. Of note she had a glomus jugular tumour which was resected in 2008. She was taking amitriptyline and felodipine. Her BP was 124/840 on this therapy

Initial CT imaging revealed a large right sided retroperitoneal soft tissue mass (18.9x 13,2x22.6 cm), inseparable from the Inferior Vena cava and the head and neck of pancreas with a large central area of necrosis, with lung findings consistent with metastases. Biopsy showed this to be a paraganglioma and it was subsequently demonstrated to take up In111-octerotide peripherally with central photopenia.  Additionally, there was a focal octreotide accumulation area at the level of deep cervical paracervical region adjacent to C7. MRI confirmed a neck mass consistent with a small paraganglioma. A 24 hour urine collection of catecholamine’s showed that mildly raised noradrenaline at 8.94 µmol/day whilst taking Amitriptyline. Elevated noradrenaline at 0.73 µmol/day (Ref:0,00-0.50) normetadrenaline at 8.79µmol/day ( Ref: <1.95) and metanephrine at 9.03 µmol/day ( Ref: < 3.47) were confirmed on repeat urine collection after discontinuing amitriptyline. She also had raised Chromogranin A at182 pmol/l  ( Ref:0-60) and Chromogranin B 379 pmol/l ( Ref: 0-150)

The abdominal paraganglioma is felt to be unresectable, and the morbidity of surgical treatment for the neck mass was felt to be high. She currently has well controlled blood pressure whilst taking doxazosin. However she is symptomatic from the abdominal distension. Genetics results are pending.

What are the management options for this lady? Is radiolabelled octreotide therapy likely to reduce the size of her abdominal mass and improve her symptoms or should she be managed conservatively?

This case highlights that there is little consensus on the optimal management of non-resectable paraganglioma.