British Journal of Radiology 75 (2002),987-989 © 2002 The British Institute of Radiology
Gastroduodenal intussusception of a gastrointestinal stromal tumour
K S Crowther, MBChB
1
L Wyld, MBChB, PhD, FRCS
1
Q Yamani, BSc, MRCP, FRCR
2 and
G Jacob, MC BCH, MCH, FRCS
1
Departments of 1 Surgery and 2 Radiology, Doncaster Royal Infirmary, Armthorpe Road, Doncaster DN2 5LT, UK
Correspondence: Mr G Jacob, MC BCH, MCH, FRCS
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Abstract
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Gastrointestinal stromal tumours (GISTs), previously termed leiomyomas and leiomyosarcomas are relatively common tumours of the gastrointestinal tract, most commonly found in the stomach. Most GISTs are asymptomatic but may cause abdominal pain or bleeding from ulceration of the overlying mucosa. A rare case of gastroduodenal intussusception of a large gastric stromal tumour, which presented with intermittent abdominal pain and gastric outlet obstruction, is reported. Pre-operative diagnosis was made on abdominal CT and confirmed at laparotomy. Pre-operative diagnostic difficulties and management are discussed.
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Introduction
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Gastrointestinal stromal tumours (GISTs) are relatively common tumours of the stomach, occurring in up to 46% of stomachs in some post mortem series [1]. These tumours were previously referred to as leiomyomas and leiomyosarcomas, but have recently been reclassified as they arise from undifferentiated stromal fibroblasts rather than mature smooth muscle cells [2, 3]. GISTs can range in size from under 0.5 cm diameter to 30 cm diameter. Three-quarters of these tumours are benign, but as size increases the risk of malignancy rises such that 60% of tumours over 10 cm are malignant (usually determined by the histological presence of more than five mitotic figures per 10 high power fields). Age is also a relevant factor, with GISTs common in those between 50 and 60 years of age, and less common in those aged 40 years or younger. The incidence of malignancy is higher in the younger age group.
The majority of GISTs are asymptomatic, with a large proportion being found incidentally at autopsy or during other surgical procedures. Macroscopically GISTs are smooth submucosal projections into the stomach lumen. Ulceration of the apical mucosa may occur resulting in abdominal pain and bleeding, which is present in up to 50% of benign tumours and 85% of malignant tumours [4]. Less commonly, patients may complain of weight loss, a palpable mass, early satiety, dysphagia and vomiting [5].
A very rare complication of GISTs is gastroduodenal intussusception caused by prolapse of the tumour and subsequent invagination of a portion or the full thickness of the gastric wall into the duodenum [6–8]. This may present with intermittent vague epigastric pain or as an acute gastrointestinal emergency with severe sudden pain, shock and intractable vomiting. Intermittent symptoms due to recurrent, spontaneously reducible intussusceptions have been reported [7, 8]. An epigastric mass may be palpated on physical examination.
Endoscopy, ultrasound and barium studies have previously been used to demonstrate this rare complication of a GIST but, until this case, it has never been demonstrated by CT.
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Case report
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A 59-year-old female presented with a 1-year history of indigestion and a 3-week history of intermittent severe epigastric pain, premature fullness and vomiting bile-stained fluid and undigested food. Between these episodes the patient was well. On examination, the patient was mildly tender in the epigastrium but no mass was palpable. All haematological and biochemical indices were normal. An upper gastrointestinal endoscopy performed at the referring hospital was reported as showing a dilated stomach, pylorus and proximal duodenum, thought to be consistent with small bowel obstruction. There was no evidence of stromal tumour. Abdominal ultrasound revealed a dilated stomach, despite fasting, and an unusual echo pattern in the region of the duodenum, suggesting gastric outflow or duodenal obstruction. The barium meal also performed at the referring hospital showed a dilated stomach and an intramural mass lesion between the first and second parts of the duodenum (Figure 1
). This was reported as a possible tumour but in retrospect demonstrated the intussusception.
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Figure 1. Barium meal demonstrating a dilated stomach with an intramural mass lesion between the first and second parts of the duodenum.
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As a result of the discrepancy between these initial investigations, CT was requested for further clarification. The examination confirmed partial gastric outflow obstruction with a characteristic pattern of intussusception, four layers of bowel wall ("partially inverted sock"), at the pylorus. A well defined 5 cm diameter lesion, which contained a rim of low density tissue compatible with fat, was present in the region of the second part of the duodenum (Figure 2 and Figure 3). Although extremely rare, a diagnosis of intussusception of a GIST was suggested.
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Figure 2. CT scan at the level of the intussusception. G, fluid in body of stomach; a, wall of the antrum; P, pyloric wall; D1, first part of the duodenum.
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Figure 3. CT section below the level of Figure 2 . L, gastrointestinal stromal tumour containing low density areas consistent with fat in the lesion; G, fluid in body of stomach.
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At laparotomy the gastric wall was distorted and the third part of the duodenum was distended by a hard mass. Manipulation of the mass delivered it into the stomach and a simple gastrotomy revealed a 7 cm stromal tumour on a long pedicle derived from the mucosa of the anterior gastric antrum. This had been intermittently prolapsing into the duodenum. The base of the tumour was excised and the gastrotomy closed. There was no evidence of metastatic spread. The patient made an uneventful post-operative recovery.
Histology confirmed a smooth, 60 mm diameter GIST with minimal nuclear pleomorphism, no necrosis and only one mitotic figure per 10 high power fields. Despite these good prognostic features, the large size of the tumour placed it into a borderline category for malignant potential.
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Discussion
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Gastroduodenal intussusception of a stromal tumour of the stomach is a very rare cause of duodenal obstruction, which unlike the most common causes, periampullary and pancreatic carcinomas, has a good prognosis. Pre-operative diagnosis was difficult to make and, although suspected, was not confirmed until laparotomy. Previous cases of stromal tumours intussuscepting into the duodenum have been demonstrated on barium studies, ultrasound and endoscopy, but this is the first case demonstrated by CT. Due to the CT findings, the patient went to theatre and was operated on successfully. This unusual case demonstrates the value of pre-operative cross-sectional imaging, particularly when there is a discrepancy between the initial first line investigations.
Received for publication December 13, 2001.
Revision received June 24, 2002.
Accepted for publication July 5, 2002.
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Abstract
Introduction
