Giant Leiomyoblastoma Of The Ileum: Report Of A Rare Case And Review Of The Literature

摘要

A case of giant leiomyoblastoma of the ileum in a 60-year-old farmer is presented. The patient complained of 4 months and 2 months history of periumbilical pains and swelling respectively. There was no associated bleeding at presentation and no evidence of intestinal obstruction. The tumour at laparotomy was terminal ileal in origin, solitary, weighing 2.1kg, measuring 19X16X11cm and completely intramural with mucosal ulceration and microscopically showed features of leiomyoblastoma. The patient had resection and end-to-end anastomosis and remained well after 3 months of follow up. Introduction Leiomyoblastoma (Bizarre leiomyoma) was first reported by Martin et al in 19601. Two years later Stout proposed the term leiomyoblastoma to distinguish this new entity from the common smooth muscle tumours2. The lesion arises from a distinctive cell, the leiomyoblast2. The stomach accounts for the primary site in 93% of all the cases3. This case report is the first leiomyoblastoma of the ileum to be reported in Nigeria in an adult (60 years). We are highlighting this extremely rare case, with review of literature. Case Report A 60-years-old farmer from Tarmuwa L.G.A. of Yobe State of Nigeria presented in the surgical out patients' clinic of University of Maiduguri Teaching Hospital with 4 months of periumbilical pain and 2 months history of periumbilical swelling. The periumbilical pain was colicky, non-radiating, made worse by eating and no relieving factors. There was associated constipation, but no vomiting. Two months later, he noticed a lump in the umbilical area. The lump progressively increased in size to its size at presentation. There was no associated fever, jaundice, no chest pain, cough or difficulty in breathing. He had lost weight and had no urinary symptoms.On examination, he was ill looking, pale and moderately dehydrated, not in any form of distress. Other findings were on abdominal examination, which revealed a central intra-abdominal mass with smooth surface, moved perpendicular to the line of attachment of the mesentery but not along it. The liver and spleen were not enlarged and there was no ascitis. The external genitalia were normal and rectal examination was unremarkable. The biochemical parameters were also unremarkable. PCV-27%, ESR-130 mm/hr and stool microscopy was negative for melena. Abdominal ultrasound revealed a mesenteric solitary, soft tissue mass. The clinical diagnosis of mesenteric tumour to rule out lymphoma was made. He was prepared for explorative laparotomy. The intra-operative findings were that of a roundish mass involving the ileum and its mesentery about 50cm from the ileocaecal valve. The segment of the affected ileum was resected together with its mesentery and primary end-to-end anastomosis was carried out. Patient did well post-operatively and was discharged home on the 10th postoperative day and remained well after 3 months of discharge.Pathological findingsThe gross specimen was a huge, round mass, well circumscribed, weighing 2.1kg and measured 19x16x11cm.The cut surface is firm, solid, greyish white whorled appearance and showing a cavity of central ulcer crater and part of the bowel was ulcerated deep into the tumour (fig1).