Background Gastro Intestinal Stromal Tumors (GISTs) are defined as mesenchymal tumours that develop within the wall of the gastrointestinal tract from the interstitial cells of Cajal. The oncogenic event that leads to GIST development is a gain of function gene mutation in one of the receptor protein tyrosine kinases KIT or PDGFRA. Clinical presentation depends on the location, size and growth rate of the tumor. In most cases, symptoms are absent or vague and the lesion is found incidentally during procedures or surgery executed for other reasons. Methods This study reviews clinical presentation, histo-pathological features and surgical treatment of 9 cases of GISTs treated at Emergency Surgery Department of Bologna between 2011 and 2016. Results Between June 2011 and March 2016, 9 patients were admitted at our institution and received surgical treatment for GISTs. All 9 were male. The mean age at diagnosis was 57 (range 37–75 years). In our study 4 patients were asymptomatic and diagnosis was an incidental finding during radiological workup for other diseases. Five patients were admitted for the onset of complications: 3 GI bleeding, 1 acute abdominal pain and 1 duodenal occlusion. Tumor site was stomach in 7 patients and duodenum in 2. Tumor size was > 2 and ≤ 5 cm in 3 cases, > 5 and ≤ 10 cm in 4 cases. Two patients presented with a giant GIST > 20 cm. Histopathological examination was carried out in all patients. GIST cell morphology was spindle in 6 and mixed (epithelioid and spindle cells) in 3 cases. CD117 and DOG1 were positive in all lesions. CD34 positivity occurred in 3. S-100, SMA, PDGFR and desmin were tested only in a few patients. According to Mienninen criteria, patients were stratified as high (3 patients), intermediate (1), low (2) and very low risk (3). One patient was treated with neoadiuvant Imatinib achieving a partial response and subsequently was able to underwent surgical resection. Laparoscopic wedge resection was performed in 1 patients and conventional open surgery in 8 patients (gastric wedge resection in 3 cases, partial gastrectomy in 2, total gastrectomy in 1. In the two patients with duodenal GIST duodenal resection was preferred to Whipple procedure. All patients in our study were enrolled in a oncological follow-up program and no recurrences were recorded. Conclusions Biopsy is mandatory for the preoperative diagnosis of GIST and useful to decide the therapeutical approach. Surgery is the treatment of choice for patients with localized and potentially resectable lesions and may be indicated for locally advanced or previously non resectable disease after a favorable response to preoperative therapy with tyrosine kinase inhibitors.
Alberici, L., Luppi, G., De Siena, N., Brighi, M., Cervellera, M., Tonini, V. (2016). Gastric and duodenal GIST: emergency and elective surgery.
Gastric and duodenal GIST: emergency and elective surgery
ALBERICI, LAURA;BRIGHI, MANUELA;CERVELLERA, MAURIZIO;TONINI, VALERIA
2016
Abstract
Background Gastro Intestinal Stromal Tumors (GISTs) are defined as mesenchymal tumours that develop within the wall of the gastrointestinal tract from the interstitial cells of Cajal. The oncogenic event that leads to GIST development is a gain of function gene mutation in one of the receptor protein tyrosine kinases KIT or PDGFRA. Clinical presentation depends on the location, size and growth rate of the tumor. In most cases, symptoms are absent or vague and the lesion is found incidentally during procedures or surgery executed for other reasons. Methods This study reviews clinical presentation, histo-pathological features and surgical treatment of 9 cases of GISTs treated at Emergency Surgery Department of Bologna between 2011 and 2016. Results Between June 2011 and March 2016, 9 patients were admitted at our institution and received surgical treatment for GISTs. All 9 were male. The mean age at diagnosis was 57 (range 37–75 years). In our study 4 patients were asymptomatic and diagnosis was an incidental finding during radiological workup for other diseases. Five patients were admitted for the onset of complications: 3 GI bleeding, 1 acute abdominal pain and 1 duodenal occlusion. Tumor site was stomach in 7 patients and duodenum in 2. Tumor size was > 2 and ≤ 5 cm in 3 cases, > 5 and ≤ 10 cm in 4 cases. Two patients presented with a giant GIST > 20 cm. Histopathological examination was carried out in all patients. GIST cell morphology was spindle in 6 and mixed (epithelioid and spindle cells) in 3 cases. CD117 and DOG1 were positive in all lesions. CD34 positivity occurred in 3. S-100, SMA, PDGFR and desmin were tested only in a few patients. According to Mienninen criteria, patients were stratified as high (3 patients), intermediate (1), low (2) and very low risk (3). One patient was treated with neoadiuvant Imatinib achieving a partial response and subsequently was able to underwent surgical resection. Laparoscopic wedge resection was performed in 1 patients and conventional open surgery in 8 patients (gastric wedge resection in 3 cases, partial gastrectomy in 2, total gastrectomy in 1. In the two patients with duodenal GIST duodenal resection was preferred to Whipple procedure. All patients in our study were enrolled in a oncological follow-up program and no recurrences were recorded. Conclusions Biopsy is mandatory for the preoperative diagnosis of GIST and useful to decide the therapeutical approach. Surgery is the treatment of choice for patients with localized and potentially resectable lesions and may be indicated for locally advanced or previously non resectable disease after a favorable response to preoperative therapy with tyrosine kinase inhibitors.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
