Case Report : A Rare Cause of Retroperitoneal Fibrosis Responding to Steroids

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Introduction: Retroperitoneal fibrosis is a rare inflammatory disease. Neuroendocrine tumors are a heterogenous group that arise from enterochromaffin cells, most commonly in the gastrointestinal tract. Their capacity to secrete hormones, five HT, tachykinins etc, are responsible for symptoms known as carcinoid syndrome. However NETS, particularly arising from the midgut, can cause fibrosis. This can result in bowel obstruction, but also cause fibrosis in the retroperitoneum, lungs and cardiac valvular fibrosis.

Aim(s): To discuss a rare case of retroperitoneal fibrosis that responded to steroids.

Materials and methods: A 39-year-old male presented with a two-month history of upper abdominal pain and a 5-year history of back pain and acute renal failure. An USS of his liver demonstrated multiple lesions. His CT scan demonstrated multiple liver metastases and a diffuse 3 cm retroperitoneal mass encasing the aorta and the right ureter at L4 causing hydronephrosis. Biopsy of a liver lesion showed a metastatic well-differentiated neuroendocrine tumor. An Octreotide scan demonstrated significant uptake in both lobes of the liver but none in the retroperitoneal mass.

Conference: 9th Annual ENETSConcerence (2012)

Presenting Author:

Authors: Mandair D, Mangat K, Malaki M, Shah T,

Keywords: neuroendocrine tumor, fibrosis, steroids,