29. National Pediatric Surgery Congress and 27. National Congress of The Egyptian Pediatric Surgical Association

Introduction: Gastrointestinal stromal tumors (GST) are rarely encountered neoplasms which have their origin from the connective tissue elements of the gastrointestinal tract. In this report, a very rare clinical presentation, a patient with GST taking it’s origin from the stomach is presented.

Case:  Six years old male patient who had been brought to a state hospital with the complaint of loss of apetite  was referred to our hospital as the result of the histopathologic examination of his endoscopic biopsy performed because of the findings of diffuse thickening of the gastric wall and an antral wall thickness of 2,5 centimeters at abdominal ultrasonography (USG) and abdominal computerized tomography (CT) respectively. At USG performed in our hospital, a vascular, solid mass lesion with heterogenic, hypoechoic and malignant appearance and the dimensions of 53x40milimeters  located at the posterior antral wall and a mass lesion located at the anterior antral wall just at this level with a similar appearance and dimensions of 37x18 milimeters were visualized, however, an extension beyond the antral wall or to the duodenum was not observed. Antrectomy, pylorectomy and excision of the first portion of the duodenum was performed and the histopathologic findings of the tissue specimen was found to be in accordance with malignant mesenchymal tumor; but at which differentiation between leiomyosarcoma and GST could not be achieved by immunophenotyping. As a result of the finding of neoplastic tissue at the surgical margin,  the proximal part of the second portion of the duodenum and anastomotic line was excised in order to take the residual tumor out totally. No neoplastic tissue was found at the frozen sections and at the regular histopathologic examination of the specimen obtained at the second operation. The patient received vincristin and actinomycin-D chemotherapy for a year. After two years have passed, recurrent mass at the stomach was observed. The polypoid mass were palpated  two centimeters  distally from the oesophagus at the minor curvature at the third operation and it was excised totally. The result of it’s histopathologic evaluation was reported to be that the mass showed chaaracteristics that were in accordance with malignant mesenchymal tumor.

Result:  Stomach masses larger than five centimeters, having exophytic component, polylobulated contour, heterogenous staining pattern, findings of mesenteric infiltration and ulceration are some of the criteria that suggest a more malignant character . It should be held in mind that GST’s can metastatize or make local recurrences; even if with a small risk; so patients operated for GST should be followed up for metastases or local recurrences.

Giriş: Gastrointestinal stromal tümörler(GST) gastrointestinal duvarın bağ doku elementlerinden köken alan nadir neoplazilerdir. Çocukluk çağında çok ender görülen mideden köken alan GST olgusu sunulmuştur.

Olgu: Altı yaşında erkek hasta, 1 yıldır olan iştahsızlık yakınması ile başvurdu. Abdominal ultrasonografide(US) mide duvarı kalınlığında diffüz artış, abdominal bilgisayarlı tomografide (BT) antrumda 2,5 cm duvar kalınlaşması saptandı. Yapılan endoskopik biyopsi sonucu gastrointestinal stromal tümörle uyumlu olan hasta merkezimize sevk edildi. Merkezimizde US’sinde mide antrum düzeyinde, posterior duvarda, lümene uzanan 53x40 mm. heterojen, hipoekoik, vasküler, solid, malign natürde kitle lezyonu, bu düzeyde anterior duvarda 37x18 mm benzer özellikte diğer bir kitle lezyonu tespit edildi. Kitlelerin mide duvarı ile sınırlı olduğu, duodenuma uzanım göstermediği izlendi. Hastaya antrektomi, pilorektomi ve duodenum 1. kısım eksizyonu yapıldı. Patoloji sonucu malign mezenkimal tümörle uyumlu olup, immünfenotiplendirme  leiomyosarkom-gastrik stromal tümör ayrımı yapılamadı. Distal cerrahi sınırda tümör devamlılığının rapor edilmesi üzerine rezidüel kitle eksizyonu yapıldı. Duodenum 2. kısmın proksimali ve anastomoz hattı eksize edildi. Frozen incelemelerde ve histopatolojik incelemelerinde tümör dokusuna rastlanmadığı rapor edildi. Hastaya vinkristin ve aktinomisin D'den oluşan  1 yıl süreli tedavi verildi. Operasyonundan 2 yıl sonra hastada nüks mide kitlesi tespit edildi. Eksplorasyonda özefagusun yaklaşık 2 cm distalinde küçük kurvatuar tarafında polipoide bir kitle palpe edildi. Kitle total olarak çıkarıldı. Histopatolojik incelemesi Nüks Malign mezenkimal tümörle uyumlu geldi.

Sonuç:Kitlenin 5cm’den büyük olması ve ekzofitik komponentinin olması, heterojen boyanma göstermesi, polilobüle kontur, mezenterik infiltrasyon ve ülserasyon varlığı maligniteye yaklaştıran kriterlerdir. Malign gastrik stromal tümörlerin düşükte olsa metastaz ve malign lokal rekürrens riski vardır. Hastalar aralıklı olarak bu açıdan takip edilmelidir.