CASE REPORT Malignant gastroin- testinal stromal cell tumour (GIST) of the stomach W Spies MSChS C S de Vries MMedRad(D) Department of Diagnostic Radiology University of the Free State Bloemfontein Case presentation A 55-year-old female patient pre- sented with a painful mass in the left hypochondrium. Sonar revealed a well-demarcated round thin-walled lesion with internal echoes with a homogeneous pattern (Fig. I). The mass measured approxi- mately 15 x 13 cm. Duplex Doppler revealed a low venous flow pattern, and a sonar-guided fine needle aspira- tion (FNA), revealed a bloody aspirate (Fig. 2). Computerised tomography (CT) was done immediately after ultrasound investigation. No oral con- trast was given. The cystic lesion (Hounsfield unit (HU) = 18 on pre- contrast study) was situated adjacent to the stomach in the lesser omental sac. It was difficult to differentiate the cystic lesion from either the stomach or the pancreas on CT. The splenic artery could also not be differentiated from the cystic lesion. The post-con- .trast CT showed rim enhancement, Fig. 1. Sonar of a thin-walled lesion with internal echoes. Fig. 2. Duplex Doppler revealing low venous flow peNem. Fig. 3, Pre-contrast CT. but no enhancement of the contents was apparent (Figs 3 and 4). 31 SA JOURNAL OF RADIOLOGY • June 2002 Fig. 4, Post-contrast CT. The preliminary differential diag- nosis included a haemorrhagic pseudocyst, thrombosed splenic artery aneurysm, subacute haematoma, or abscess. A splenic, hepatic and superior mesenteric artery (SMA) angiogram revealed no significant pathology, splenic artery aneurysm, or neovascularity. A surgi- cal exploration and resection was then done. Stomach wall slices showed the presence of a cellular tumour with small areas of necrosis. Evidence of spindle-shaped and epithelioid cells was found. A slight pleomorphism was present with an average of 2 mito- sis per 10 high-field magnification. The final diagnosis was a malig- nant gastrointestinal stromal cell tumour (GIST). Discussion GISTs are uncommon mesenchy- mal tumours of the gastrointestinal (GI) tract. GISTs include tumours previously designated as leiomyoma, cellular leiomyoma, leiomyoblastoma and leiomyosarcoma. The oesophagus is the exception, with leiomyoma the most common mesenchymal tumour. Studies show that phenotypically undifferentiated GISTs are also genet- ically different from leiomyomas and schwannomas and support their das- CASE REPORT sification apart from leiomyornas.' GISTs are composed of spindle (70%) or epithelioid (30%) cells. 10 - 30% are malignant, showing intra-abdom- inal spread and/or liver metastasis. GISTs are both malignant and benign. They may be submucosal, subserosal or intraluminal. The CT appearance of a GIST may include a sharply defined mass with homoge- neous attenuation, sometimes with clarification. A study by Kim et al? analysed the clinical presentation, pathology and long-term follow-up of 19 patients with GIST tumours (12 gastric, 2 duo- denal, 3 jejunal and 2 rectal). The most common clinical presentation was GI bleeding. CT, contrast studies, and endoscopy were used to identify tumour mass. In only two instances could the diagnosis of GIST be made preoperatively. Histology was variable and the size of the tumours ranged from 0.8 to 23 em. The clinical course is favourable with complete resection. Needle biopsy can be used for the diagnosis of GIST tumours preopera- tively. GIST can be classified as benign, borderline, or malignant using immunochemistry, size, mitotic activity and clinical outcome. Spindle cell and epithelioid types can be dis- tinguished. Cytomorphology alone cannot be used to assess malignancy and predictions of aggressiveness but must be interpreted with a gross and histological examination of the resect- ed specimen. Immunocytochemical staining for CD is helpful in confirm- ing the diagnosis. Care must be taken to differentiate epithelioid-type GISTs from adenocarcinoma. GISTs are also immunohistochernically positive for C-kit (CDl17), CD34 and sometimes for actin, but are mostly negative for desmin and S-l 00 protein. The malig- nant GIST especially,shows activating mutations in the C-kit gene. GIST and GI autonomic nerve tumours (GANTs) can overlap. The cell of ori- gin is not well understood, but resem- blance to the interstitial cells of Gajal, expression of the smooth muscle markers and occurrence outside of the GI tract suggest an origin from multipotential cells that can differen- tiate into Cajal and smooth muscle cells. References 1. Dodd LG, Nelson RC, Mooney EE, Gottfried M. Fine-needle aspiration of gastrointestinal stromal tumours. Am] Clin Patho/199B; 109: 439-443. 2. Kim CJ, Day S,YehKA.Gastrointestinal stromal tumours: analysis of clinical and pathologic fac- tors. Am Surg2001; 67: 135-137. 32 SA JOURNAL OF RADIOLOGY'. June 2002