A case of metastatic neuroendocrine tumour illustrating that a conservative approach to the management of such tumours can be justified


Ghaffar AH, Carroll RW, Tan T, Meeran K.

Imperial Centre for Endocrinology.

A 49 year old lady presented to the general surgeons  in 1989 with a 2 month history of a painless mass in the left supraclavicular fossa.  FBC, U&Es, CXR and mammography were normal and she underwent excision of this mass in early 1990. The histology demonstrated an APUDoma. A subsequent CT scan showed a large tumour posterior to the pancreatic head, liver lesions, paraaortic lymph nodes and lymph nodes around the porta hepatis. She underwent a laparotomy, at which the pancreatic mass was found to be adherent to the portal vein and inoperable. Multiple liver cysts were seen. A gastrojejunostomy was fashioned, and a biopsies were taken. A lymph node biopsy was consistent with an APUDoma, while the liver biopsies were normal. Her recovery was complicated by a sub-phrenic abscess which had to be drained. She was referred for assessment to a tertiary centre, when she was well and on examination had a left sided supraclavicular lymph node.  Fasting gut hormones were normal as were urinary 5HIAA measurements. From 1991 onwards her main problem was pain from the original surgery for which she was on morphine. A repeat CT demonstrated stable disease. She underwent an octreotide scan in 1992, and this demonstrated her pancreatic lesion and her neck metastasis. The liver lesions were not octreotide avid. A few months later she returned with an enlarging left supraclavicular mass. Fasting gut hormones remained normal. A CT demonstrated a 3.5x3x5cm mass arising from behind the left internal jugular vein and displacing it forward. She underwent resection of the lesion in March 1993. Histology confirmed it to be a neuroendocrine tumour invading a lymph node. The tumour stained positive for chromogranin. She continued to be followed up 6-12 monthly and remained well. There was no evidence of disease progression either clinically, biochemically or radiologically. In 2000, she was found to have a 3cm non-tender left supraclavicular mass. It was managed conservatively as it was not causing symptoms. The masses in the neck and the abdomen have remained stable until the present time. The lady is being followed-up annually with clinical assessment and biochemistry and with cross-sectional imaging every 2 years.