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Case Reports
 
Gastric Stromal Tumour
Chirag C Parikh*, Dipesh D Dutta Roy**, Brijesh Madhok***, Sashidhar V Yeluri+, Adeesh Jain*
 
Gastric stromal tumour (GST) though a rare pathology, is being reported now with increasing frequency. We report two cases with diverse symptomatology managed successfully by surgical resection.
 
Introduction

Gastrointestinal stromal tumours (GIST) are uncommon nonepithelial mesenchymal tumours originating usually from the gastrointestinal tract (GIT) and rarely independently within the abdomen. Mazur and Clark coined the term stromal tumour in 1983. Earlier they were thought to arise from the smooth muscle layer of the GIT and because of predominant spindle or epithelioid cells seen on histopathology were labelled as leiomyoma, leiomyosarcoma and leiomyoblastomas. These heterogeneous group of neoplasms have a complicated unclear histogenesis and are now presumed to arise from the interstitial cell of Cajal, an intestinal pacemaker cell.1,2 The stomach is the most common site (50% to 70%) for these tumours followed by small intestine (20% to 30%).1-4
 
Case 1
A 24 year female presented with a dull aching pain in the epigastrium, exacerbated on swallowing solid food, for 3 months. Abdominal examination was insignificant. Hematological and biochemical tests were normal. Ultrasonography (USG) revealed a mixed echogenic mass of 4 cm x 4 cm at the cardia. Barium study showed a smooth filling defect along the cardia and lesser curve (Fig. 1). Computed tomography (CT) revealed a trifoliate heterogeneous mass at the cardia in close proximity to but free from the liver and great vessels. Endoscopy revealed a submucosal tumour involving the cardia and the lesser curve, with normal overlying mucosa. On exploration a 5 cm x 5 cm x 5 cm tumour was seen arising from the cardia involving the lesser curvature, with no regional lymphadenopathy or ascites. A proximal gastrectomy was performed. Intraoperative frozen section revealed spindle cells with acidophilic cytoplasm and oval centrally placed nucleus arranged in short fascicles, suggestive of benign GIST. Oesophagogastric anastomosis was performed with a temporary feeding jejunostomy. Histopathology (HP) confirmed GIST-Leiomyoma with no evidence of malignancy and clear surgical margins. Patient is doing well after 6 months of surgery.
Fig. 1 : Barium study showing a smooth filling defect along the cardia and the lesser curve of stomach delineated by multiple arrows
 
Case 2
A 58 year male presented with history of a single episode of haematemesis 15 days prior to hospitalization. There was no abdominal pain or weight loss. Patient was anaemic. Abdominal examination was normal. Haemogram confirmed anaemia (Hb-6 gm/dl). Barium examination showed a large gastric mass arising from the fundus. Endoscopy revealed a large intraluminal pedunculated fundic tumour with normal mucosa, which was, confirmed on CT (Fig. 2). After correction of anaemia by blood transfusions, patient underwent exploration, which revealed a 6 cm x 6 cm x 5 cm pedunculated tumour in the anterolateral wall of the fundus. Excision of the tumour with safe margins was done. Frozen section revealed benign GIST. HP report was benign GIST-leiomyoma with rare mitotic activity. Patient is well 5 months after surgery.  
Fig. 2 : Contrast CT scan of abdomen showing a pedunculated mass arising from the anterolateral wall of the stomach
 
Discussion
Gastric leiomyoma occurs commonly in 5th or 6th decade of life,2 though rarely they have been reported in children. Majority of patients with gastric stromal tumour (GST) present with manifestations of acute or chronic upper gastrointestinal haemorrhage in the form of haematemesis, melaena, or anaemia.2,3,5,6 This is common in submucosal tumours eroding the mucosa. Epigastric pain, upper abdominal lump and vomiting due to gastric outlet obstruction are the other presenting symptoms.2,6,7 GST at cardia may present with dysphagia as was seen in our patient. Asymptomatic GST may be diagnosed by imaging techniques, endoscopy or accidentally on exploration. On barium examination GST typically appears as a smooth rounded filling defect with normal mucosal pattern. Though endoscopy is an important diagnostic tool it may not always detect these tumours, as they may be submucosal or subserosal.3 Endoscopic biopsy is possible only in ulcerated tumours but based on it histological differentiation between benign and malignant GST may not be possible.3 Though Endoscopic ultrasound (EUS) is helpful in detecting the layer of origin of the tumour, EUS guided fine needle aspiration cytology is unreliable in distinguishing between malignant and benign varieties. Percutaneous fine needle biopsy is not recommended due to the risk of intraabdominal tumour spillage.2 Endoscopic sclerotherapy is an important adjunct in the management of bleeding lesions.3 CT and magnetic resonance imaging are equally accurate in diagnosing and localizing GST.2,4

Immunohistocytochemistry has an important role in understanding the histogenesis of these tumours, which still have plenty of gray areas. Immunohistocytochemistry and electron microscopy have established that GISTs may have smooth muscle or neural differentiation, a combination of the two, or may totally lack differentiation.8 The diverse properties of these tumours have compelled pathologists to label them as GISTs or GSTs. Differentiating benign GSTs from malignant remains a dilemma because of their diverse and unpredictable behavioural patterns. Tumours < 5 cm in size, with low cellularity, absent to minimum nuclear pleomorphism, absent necrosis, mitosis of < 0-1/10 HPF and absence of infiltrative growth patterns, metastasis and c - kit mutation are usually benign.2

Primary treatment of GIST is aggressive surgical resection.2,3,5-7,9,10 Intraoperative frozen section performed by an experienced pathologist helps in achieving a precise diagnosis as well as differentiating stromal tumours from adenocarcinoma and lymphoma.2 Resection with a negative tumour free margin of at least 2 cm is recommended.3,5,9 Large bulky tumours as well as those near the cardia and pylorus may require a formal total or partial gastrectomy or their variations. Invasion of an adjacent organ would warrant its enbloc resection along with the tumour.2 Lymphadenectomy is not indicated as lymhatic spread occurs infrequently and does not affect the long-term survival.5,11 Laparoscopic resection of “being gastric stromal tumours” have been documented.9,12 Postoperative recurrence is common and though most recur within 2 years, some may take > 10 years to metastasize.2 Hence a long-term follow up is recommended.2,5 Radiotherapy and chemotherapy used in palliation of advanced tumours have been found to be ineffective. Clinical trials of STI-571 (Imatinib Mesylate) a potent tyrosine kinase inhibitor have provided encouraging results in metastatic GIST.
 
References
1. Strickland L, Douglas Letson G, Muro-Cacho CA. Gastrointestinal stromal tumours, Cancer Control 2001; 8(3) : 252-61.
2. Pidhorecky I, Cheney RT, Kraybill WG, Gibbs JF. Gastrointestinal stromal tumours : Current diagnosis, biologic behavior, and management. Ann Surg Oncol 2000; 7(9) : 706-12.
3. Mihssin N, Moorthy K, Sengupta A, Houghton PWJ. Gastric stromal tumours : a practical approach. Ann R Coll Surg Encl 2000; 82 : 378-82.
4. Mak HKF, Leung CMY, Chan CW, et al. Gastrointestinal stromal tumours - A retrospective study of the clinical, endoscopic and computed tomographic features. J HK Coll Radiol 2002; 5 : 82-7.
5. Sanders L, Silverman M, Rossi R, Braasch J, Munson L. Gastric smooth muscle tumours : Diagnostic dilemmas and factors affecting outcome. World J Surg 1996; 20 : 992-5.
6. Katrak MP, Mehta NN, Sinha AS, et al. Extraserosal pedunculated leiomyoma of stomach. Indian J Gastroenterol 2002; 21 : 200-1.
7. Bhattacharjee PK, Chaudhuri T, Roy D. An unusual abdominal lump - A case report. JIMA 2003; 101(5) : 322-3.
8. Rosai J. Gastrointestinal tract - stromal tumors. In: Rosai J editor. Ackerman’s surgical pathology. 8th ed. St Louis, Missouri : Mosby 1996; pp.645-7.
9. Felicano DV. Image of the month, Gastric stromal tumor (Leiomyoma Vs Leiomyosarcoma). Arch Surg 2001; 136 : 597-99.
10. Gondal PK, Pradhan S, Chattoraj A, Mohanty R. Gastrointestinal stromal sarcomas. Indian J Surg 2001; 63(6) : 461-4.
11. Di Matteo G, Pescarmona E, Peparini N, et al. Histopathological features and clinical course of gastrointestinal stromal tumors. Hepato-Gastroenterology 2002; 49 : 1013-6.
12. . Motson RW, Fisher PW, Dawson JW. Laparoscopic resection of a benign intragastric stromal tumour. Br J Surg 1995; 82 : 1670.
 

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