Case Report Fatal hypoglycaemia from IGF II hyperproduction as a complication of a mesenteric gastrointestinal stromal tumour Zouhour Samlani-Sebbane a,⇑ , Azeddine Diffaa a , Khadija Krati a , Abdennasser Finech b , Khalid Rabbani b , Youssef Narjis b , Nawal El Ansari c , Fadoua El Mansouri d , Badia Belaabidia d a Gastroenterology Department, King Mohamed VI Hospital, FMPM, UCAM, King Mohamed VI University Hospital, Medical School of Marrakech, Cadi Ayyad University, Marrakech, Morocco b Surgery Department, King Mohamed VI Hospital, FMPM, UCAM, King Mohamed VI University Hospital, Medical School of Marrakech, Cadi Ayyad University, Marrakech, Morocco c Endocrinology Department, King Mohamed VI Hospital, FMPM, UCAM, King Mohamed VI University Hospital, Medical School of Marrakech, Cadi Ayyad University, Marrakech, Morocco d Anatomical Pathology Department, King Mohamed VI Hospital, FMPM, UCAM, King Mohamed VI University Hospital, Medical School of Marrakech, Cadi Ayyad University, Marrakech, Morocco Introduction Gastrointestinal stromal tumours (GISTs) are rare mesenchymal tumours. One of their characteristics is the immunohistochemical expression of a c-kit proto-oncogene (CD117) [1]. They primarily develop in the stomach and small intestine [2]. The main clinical signs are associated with the mass effect and exceptionally parane- oplastic manifestations [3]. We report on a case of non-islet-cell-tumour-hypoglycaemia (NICTH)-associated mesenteric GIST. The originality of this obser- vation lies in the unusual location of the tumour and in the excep- tional paraneoplastic syndrome associated with it. Case report A 47-year-old female patient presented with vomiting unre- lated to meals and associated with a typical ulcer-like epigastric pain. Upon examination, the patient was found to be in a fairly good general condition, conscious and haemodynamically stable, with a weight of 65 kg. Abdominal examination revealed an abdominopelvic mass with firm consistency, a regular surface and was not tender. The rest of the physical examination was unremarkable. Computed tomogra- phy (CT) scan (Fig. 1) showed a bulky, cystic abdominopelvic mass that had developed over the mesentery, suggesting a schwannoma. The rest of the examinations were found to be normal. Surgical exploration revealed an enormous and encapsulated mesenteric mass. The histopathological appearance and the immunohistochemical profile were those of a GIST marked by anti-CD117 antibodies with high mitotic index (20 mitoses for 50 high-magnification fields) (Figs. 2 and 3). The resection was incom- plete. The operative follow-up was uncomplicated, and the patient was put on imatinib. Five months after the intervention, the patient presented in the emergency department with deterioration of conscious level, sweating and palpitations. Upon examination, the pulse was rapid at 120 min À1 . Laboratory findings revealed a blood-glucose level of <0.45 g l –1 , hypokalaemia at 2.7 g l –1 , peptide C of 0.0 pmol l –1 (normal = 0.3–1.4) and insulin level of 0.6 pmol l –1 (normal = 13–169). NICTH was suspected after the insulin-like growth factor (IGF) hormone assay: IGF I was dramatically reduced (4 ng l –1 ; normal between 81 and 267) and IGF II was very high (2003 ng; normal between 459 and 1123). A follow-up CT scan was done; it revealed an abdominopelvic mass without local–regional or distant invasion. The diagnosis of paraneoplastic hypoglycaemia was established, and the patient was treated with continuous intravenous infusion of 30% glucose with corticosteroid therapy at 1 mg kg –1 . Due to the non-response to this treatment, and the need for continuous intra- venous infusions of 30% glucose solution, surgery was decided upon for tumour reduction. Two 15 Â 20-cm and 10 Â 4-cm tumour masses were found closely adherent to the colon and the mesentery. The patient died on postoperative day 2 from fatal hypoglycaemia. Discussion The originality of this observation lies in the rarity of NICTH occurring with stromal tumours and in the uncommon mesenteric location. Gastrointestinal tumours are often asymptomatic or mani- fest by digestive symptoms. Presentation by a paraneoplastic 1687-1979/$ - see front matter Ó 2011 Arab Journal of Gastroenterology. Published by Elsevier B.V. All rights reserved. doi:10.1016/j.ajg.2011.08.001 ⇑ Corresponding author. Address: 4, Residence ALI, IMM D, Avenue Mohamed VI, Marrakech, Morrocco. Tel.: +212 661 64 76 80. E-mail address: samlanizouhour@yahoo.fr (Z. Samlani-Sebbane). Arab Journal of Gastroenterology 12 (2011) 171–172 Contents lists available at SciVerse ScienceDirect Arab Journal of Gastroenterology journal homepage: www.elsevier.com/locate/ajg