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Duodenal Diverticulum Mimicking Duodenal Stromal Tumor

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EN
A patient with duodenal diverticulitis who was initially misdiagnosed with stromal tumor of the duodenum is presented. This case is of interest because it illustrates difficulties in the interpretation of auxiliary investigations and the choice of the best treatment option. US and CT images revealed two large ovoid masses of fluid density in the duodenal wall which may have suggested stromal tumor of the duodenum as well as periampullary cystic neoplasm, the more so because intramural tumor of the duodenum was seen in duodenoscopy. A similar picture may also be seen in duodenal diverticula, especially in diverticula which are not filled with gas or a combination of fluid and gas. This case demonstrates one such entity.
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Gists As a Cause of Massive Bleeding

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EN
This report describes two cases of GIST with bleeding as the predominant symptom. The first case comprised a patient with a gastric carcinoma and bleeding of the digestive tract. The second patient was diagnosed with mesenteric carcinoma and peritoneal cavity bleeding. Both patients underwent emergent surgical intervention. The first patient was subjected to gastric resection, while the latter to mesentery resection. The postoperative period proved uneventful. The excised specimens were evaluated at the Department of Pathomorphology, Institute of Oncology in Warsaw. The patients were directed to the Department of Soft Tissue Neoplasms, Institute of Oncology for registration and further treatment.
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Giant Abdominal Cyst - Case Report

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EN
We present a case of a 53-year old male patient who underwent elective surgery due to a giant abdominal cystic mass. Prior to the surgery, he complained of abdominal distention, growing abdominal mass and increasing nourishment problems. CT and ultrasonographic examinations revealed a giant abdominal cyst but failed to indicate the point of its origin. Intraoperatively a giant abdominal cyst was found, approximately 30x30x25 cm in size. The cyst was free-lying in the peritoneal cavity, except small area adherent to the stomach wall. Partial resection of the stomach wall was performed and the cyst was completely removed.The postoperative course was uncomplicated. The pathological examination did not give an unequivocal answer as to the origin of the cyst, suggesting differentiation between a tumor of vascular origin and of stromal origin.
PL
Guz stromalny (GIST) należy do rzadkiej patologii. Stanowi od 0,3 do 1% wszystkich guzów zlokalizowanych w przewodzie pokarmowym. Jednocześnie nowotwory tego typu są najczęstszymi, złośliwymi, nienabłonkowymi guzami cewy pokarmowej. W ponad 90% umiejscowienie dotyczy żołądka oraz jelita cienkiego. Z reguły nowotwór przebiega bez charakterystycznych objawów klinicznych oraz rozpoznawany jest przypadkowo. Sytuacja kliniczna, gdzie pierwszym objawem guza o utkaniu GIST jest pełnoobjawowa, niepowikłana przepuklina pępkowa jest anomalią niespotykaną. Praca przedstawia przypadek 77-letniej pacjentki operowanej w trybie planowym, gdzie zawartością worka przepukliny pępkowej był guz o utkaniu guza podścieliskowego. Śródoperacyjnie stwierdzono rozsiane, wieloogniskowe zaawansowanie choroby. Na podstawie pooperacyjnego badania histopatologicznego oraz immunohistochemicznego rozpoznano żołądkowo-jelitowy guz stromalny GIST z komórek wrzecionowatych, wykazujący ekspresję CD-117, CD-34, SMA, z prawdopodobnym punktem wyjścia z jelita cienkiego.
EN
GIST is the most common mesenchymal tumour of gastrointestinal tract arising from mutation of KIT or PDGFRA gene. Surgery is the primary method of treatment, however a targeted therapy with imatinib is necessary due to recurrence. The aim of the study was to evaluate efficacy of the targeted chemotherapy in advanced gastrointestinal stromal tumours with non-exon 11 KIT mutations. Material and methods. Data from 279 patients with advanced GIST treated with imatinib between 2001 and 2011 were analysed in the study. Exon 11 KIT mutation was found in 192 patients (68.7%), non-exon 11 KIT mutation was found in 87 patients (31.3%): this group included lack of mutation - wild-type, exon 9 KIT mutations, exon 18 PDGFRA D842V mutations, non-D842V PDGFRA mutations as well as non-exon 9 and 11 KIT mutations. Analysis of progression-free survival and overall survival were done for the entire group of patients and for patients with particular mutations, and then effects on progression-free survival and overall survival of such factors as sex, age, imatinib dose were evaluated. Results. Occurrence of non-exon 11 KIT mutation increases the risk of disease progression by 20% in comparison to the presence of exon 11 KIT mutation, however it does not increase the risk of patient’s death. Percentage of 5-year progression-free survivals is the greatest in the case of PDGFRA mutation other than D842V mutation. Percentage of 5-year survivals in case of the presence of D842V PDGFRA mutation is more than twice worse than in the case of the other mutations. Lesion location in the gastrointestinal tract affected the risk of death, with the greatest percentage of 5-year survival for lesions located in the stomach. Such factors as sex, age at diagnosis (<50, ≥50 years) and imatinib dose did not affect the risk of disease progression and the risk of patient’s death. Conclusions. The ratio of overall survival of patients with advanced GIST with a mutation other than exon 11 KIT mutation treated with imatinib is similar to the ratio of overall survival of patients with GIST with exon 11 KIT mutation. An exception is the group of patients with GIST in whom the presence of D842V PDGFRA mutation was found. In general, longer survival has been found in patients with GIST located in the stomach in comparison to the small intestine or other less frequent locations. Percentage of 5-year progression-free survivals is the greatest in the case of PDGFRA mutation other than D842V mutation.
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vol. 85
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issue 5
284-288
EN
Both gastrointestinal stromal tumors (GIST) and liposarcoma originate from mesenchymal tissue. Their coincidence requires a specific expertise in the diagnostic and therapeutic management. An unusual exemplary case is described representing a 47-year old female patient with a gastric GIST and a monstrous retroperitoneal liposarcoma with infiltration of the left kidney. The gastric tumor lesion was removed with a tangential resection of the gastric wall; the retroperitoneal tumor lesion was resected including the left kidney. Both tumors were resected with no macroscopic tumor residual. The technically difficult surgical intervention did not show any postoperative complication, and the postoperative course was also uneventful. The complete tumor resection is the treatment of choice in mesenchymal tumors (aim: R0). Depending on histologic tumor classification, resection status and tumor sensitivity, a subsequent radiation and/or chemotherapy is necessary, which allowed to achieve a postoperative tumor-free survival of 6 years including a good quality of life.
EN
Meckel diverticulum is the most common congenital abnormality of gastrointestinal track (2-4%), however complications occur rarely (4-16%). We describe a case of 63- years old female presenting simultaneously two serious complications of Meckel diverticulum. Operated patient was diagnosed with perforated tumor of Meckel diverticulum. Segmental resection of small bowel including tumor was performed. Pathology examination revealed gastrointestinal stromal tumor (GIST) in Meckel diverticulum. No significant malignancy risk factors were found (low mitotic count). Consequently, computed tomography periodic surveillance was implemented. We report the possibility of simultaneous presentation of two serious complications of Meckel diverticulum. Tumors of Meckel diverticulum may mimic other abdominal pathologies and thus, they should be considered in differential diagnosis of abdominal tumors.
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Perforated Gist of Meckel's Diverticulum

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EN
Meckel diverticulum is the most common congenital abnormality of gastrointestinal track (2-4%), however complications occur rarely (4-16%). We describe a case of 63- years old female presenting simultaneously two serious complications of Meckel diverticulum. Operated patient was diagnosed with perforated tumor of Meckel diverticulum. Segmental resection of small bowel including tumor was performed. Pathology examination revealed gastrointestinal stromal tumor (GIST) in Meckel diverticulum. No significant malignancy risk factors were found (low mitotic count). Consequently, computed tomography periodic surveillance was implemented.We report the possibility of simultaneous presentation of two serious complications of Meckel diverticulum. Tumors of Meckel diverticulum may mimic other abdominal pathologies and thus, they should be considered in differential diagnosis of abdominal tumors.
EN
Richter’s hernia (partial enterocele) is the strangulation/entrapment of only part of the circumference of the intestinal wall. It is relatively rare, and presents without mechanical obstruction – giving vague, non-specific symptoms and signs, and a threat of intestinal necrosis, gangrene, followed by perforation. A report of a case of entrapment of the jejunum in the femoral ring, which did not cause the gangrene, but symptoms of mechanical obstruction, is presented. Further inspection of the small bowel in this case revealed an extra-luminal tumour, which appeared to be a GIST. The entrapped part of the jejunum has been released and the tumour has been resected.
EN
Gastrointestinal stroma tumors (GIST) arise from the pacemaker, the interstitial Wells of Cajal. These tumors constitute 1 to 3% of gastrointestinal neoplasms, and may occur in each portion of the gastrointestinal tract. The most useful prognostic factors are tumor size, mitotic index, cell structure and location within the gastrointestinal tract.The aim of the study was to assess the chosen prognostic factors (location in the gastrointestinal tract and mitotic index) in patients with GIST.Material and methods. Between 1989 and 2002, 74 patients (37 men and 37 women) with an average age of 54.9 years (range from 13 to 89 years) were operated for GIST in the Department of Gastrointestinal Surgery. Two- and five-year survival rates during observation were analyzed, as well as the location within the gastrointestinal tract and mitotic index. Based on the intraoperative and postoperative investigations, the tumor size, presence of metastases and histological type of predominant cells were estimated in each patent. Results were subjected to statistics, where p≤0.05 was considered to be significant.Results. Of the 74 patients included in the study, 3 patients (4%) had a primary tumor located in the lower oesophagus, 42 patients (56.8%) in the stomach, 4 patients (5.4%) in the duodenum, and 13 patients (17.6%) had tumors originated from the small intestine. In an additional 12 patients (16.2%), the tumor originated from the large intestine. The most frequent (51%) mitotic index was 2, and 9/50 hpf was considered an intermediate malignant potential risk. Two-year survival was common in patients with GIST located in the oesophagus, stomach, and duodenum, totalling 34 (79%) patients. A lower than two-year survival rate was noted in patients with GIST arising from the small intestine: 7 (63.6%) patients had tumors arising from the colon and 4 (36.3%) patients had rectal tumors. Five-year survival was also the most frequent in patients with GIST located in the upper part of gastrointestinal tract (37.2%), in the median part of gastrointestinal tract (36.3%), and in the lower part of the gastrointestinal tract (27.7%). Correlation between location, mitotic index and survival of patients was assessed. The correlation studies showed a statistically significant influence of tumor location in the gastrointestinal tract (p=0.0264) and mitotic index (p=0.0003) with the survival of patients operated for GIST. Thus, the lower location and higher mitotic index of GIST are associated with shorter survival of patients.Conclusions. The mitotic index and location in the gastrointestinal tract are essential prognostic factors in analyzed patients with GIST. In the analyzed group, the lower locations and higher mitotic indices of GIST were associated with shorter survival of patients.
11
80%
EN
Mesenchymal tumors of the gallbladder are rarely encountered in clinical practice. The Gastrointestinal Stromal Tumor (GIST) of the gallbladder is rarely encountered. These tumors most commonly arise from the interstitial cells of Cajal (ICC), the pacemakers of the intestinal system. There can be benign as well as malignant forms of GIST. The literature on GIST arising from the gallbladder wall is limited to a few case reports only. In extensive search of the indexed literature, only 9 cases of gallbladder GIST were retrieved. Based on the available literature these tumors are commonly found in females. They usually present with hypochondrial pain with or without other features of cholangitis. These tumors are usually malignant and warrant a radical surgical excision. The data on postoperative adjuvant therapy and survival is limited. The authors presented a review of the available literature on this rare pathology.
EN
Gastrointestinal stromal tumor is a rare pathology. GISTs account for 0.3-1% of all tumors of the gastrointestinal tract. At the same time, this type of cancer is the most common, malignant, non-epithelial tumor of the gastrointestinal tube. Over 90% of GISTs are found in the stomach and small intestine. This cancer usually develops without characteristic clinical symptoms and is diagnosed incidentally. This clinical situation, in which the first symptom of a GIST-pattern tumor includes a fully-symptomatic, non-complicated umbilical hernia, is an unprecedented anomaly. This work presents a case report of a 77-year old female patient undergoing elective surgery, in which the contents of the hernial sac included a stromal tumor. Disseminated, multi-focal progression of the disease was found intraoperatively. Postoperative histopathology and immunohistochemistry revealed a gastrointestinal stromal tumor GIST of the spindle cell type, showing a CD-117, CD-34, SMA expression with possible starting point in the small intestine.
OncoReview
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2013
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vol. 3
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issue 4
232-342, 343-354
EN
Background: Adjuvant therapy is recommended for the population of patients with high risk of recurrence of gastrointestinal stromal tumour after resection. The aim of the study: Evaluation of the clinical efficacy and safety profile of imatinib used in adjuvant therapy in patients after complete resection of a gastrointestinal stromal tumour. Material and methods: A systematic review of the literature published up to 30.03.2012 was performed, and a meta-analysis of identified studies was carried out. Databases were searched: PubMed, EMBASE, The Cochrane Library and others. Results: Two randomised clinical trials regarding comparisons of: imatinib vs. placebo and 12 months of adjuvant imatinib vs. 36 months of adjuvant imatinib as well as 20 non-randomised trials fulfilled the established criteria. Adjuvant imatinib statistically significantly improves recurrence-free survival compared with placebo. Patients with high risk of recurrence benefit most from assigned treatment. Three years of adjuvant imatinib therapy improves recurrence-free state and overall survival compared with 1 year of imatinib in patients after resection of a gastrointestinal stromal tumour. The safety profile of imatinib in the analyzed population is acceptable. Conclusions: For patients with a significant risk of recurrence adjuvant therapy with imatinib should be considered for every patient, due to the clinical benefits it brings.
PL
Wstęp: Zastosowanie terapii adiuwantowej jest zalecane u pacjentów ze znaczącym ryzykiem nawrotu choroby po zabiegu usunięcia nowotworu podścieliskowego przewodu pokarmowego. Cel pracy: Ocena skuteczności klinicznej i profilu bezpieczeństwa imatynibu stosowanego w ramach terapii adiuwantowej u pacjentów po zabiegu całkowitej resekcji nowotworu podścieliskowego przewodu pokarmowego. Materiał i metody: Przeprowadzono systematyczny przegląd literatury opublikowanej do 30 marca 2012 r. oraz metaanalizę zakwalifikowanych do opracowania badań. Przeszukano następujące bazy danych: PubMed, EMBASE, The Cochrane Library oraz inne. Wyniki: Odnaleziono 2 randomizowane badania kliniczne, dotyczące porównania: imatynibu z placebo oraz imatynibu stosowanego przez 12 miesięcy z imatynibem podawanym przez 36 miesięcy, oraz 20 nierandomizowanych badań klinicznych dotyczących stosowania imatynibu w analizowanym wskazaniu. Zastosowanie imatynibu w terapii adiuwantowej prowadzi do istotnego statystycznie wydłużenia przeżycia wolnego od nawrotu choroby względem placebo. Największe korzyści z zastosowanej terapii odnoszą chorzy z wysokim ryzykiem nawrotu choroby. Przedłużenie 12-miesięcznej terapii imatynibem do 36 miesięcy zwiększa prawdopodobieństwo przeżycia wolnego od progresji choroby oraz przeżycia całkowitego pacjentów po zabiegu resekcji nowotworu podścieliskowego przewodu pokarmowego. Profil bezpieczeństwa imatynibu w analizowanej populacji okazał się korzystny. Wnioski: U pacjentów o znaczącym ryzyku nawrotu terapia adiuwantowa imatynibem powinna być rozpatrywana w przypadku każdego pacjenta ze względu na korzyści kliniczne, jakie przynosi.
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