Solitary Fibrous Tumour of Liver Presenting as Doege–Potter Syndrome – A Case Report

Abstract

Background: Doege–Potter syndrome is a rare syndrome which is defined as Solitary fibrous tumour (SFT) that is associated with NICTH (non-islet cell tumour hypoglycaemia). Limited case reports are available on Solitary fibrous tumour of liver presenting as Doege–Potter syndrome in published medical literature. Case report: A 60-year-old female presented with episodic weakness and incoherent behaviour since 3 months and during that episode her serum glucose were low which corrected on glucose administration and abdominal distension for 1 month. Abdominal lump was noted in right hypochondrium arising from liver. On investigation- fasting serum glucose levels, fasting insulin levels and fasting C peptide levels were low and serum IGFII levels were elevated. CT abdomen revealed heterogeneously enhancing solitary lesion of 23 x 18 x 14 cm arising from segment 5 ,6, 7, 8 of liver. AFP, CA19-9 levels were within normal range. Liver biopsy shows spindle cell tumour of fibroblastic origin. Anatomical right hepatectomy with MHV preservation was done. Postoperative course was uneventful with symptomatic improvement, serum glucose levels normalised, and patient was discharged on 8th POD. Discussion: Solitary fibrous tumours are uncommon benign or malignant mesenchymal cell tumours which originate from visceral pleura, pelvis, retroperitoneum and liver. Doege–Potter syndrome which is a rare paraneoplastic manifestation of Solitary fibrous tumour, when there is secretion of prohormone - insulin-like growth factor II (IGF-II), causing hypo insulinemic hypoglycaemia and suppressed serum insulin, C peptide and elevated IGFII/IGFI ratio . Definitive treatment is complete tumour resection.