scholarly journals Malignant Extra-Gastrointestinal Stromal Tumor of the Mesentery

2019 ◽  
Vol 05 (03) ◽  
pp. e65-e68 ◽  
Author(s):  
Prakash K. Sasmal ◽  
Rakesh Sharma ◽  
Susama Patra ◽  
Tushar S. Mishra ◽  
Pritinanda Mishra ◽  
...  
Keyword(s):  
Small Bowel ◽  
Gastrointestinal Stromal Tumors ◽  
Alimentary Tract ◽  
Large Mass ◽  
Histopathological Study ◽  
Small Bowel Mesentery ◽  
Mesenchymal Tumors ◽  
Stromal Tumors ◽  
Cells Of Cajal

Gastrointestinal stromal tumors (GISTs), the commonest mesenchymal tumors of gastrointestinal tract are often described to take origin from the interstitial cells of Cajal (ICC) or its precursor cells. Rarely these tumors do arise in structures other than the alimentary tract like omentum, mesentery, retroperitoneum, etc., of varying malignant potential and are known as extra-gastrointestinal stromal tumors (eGISTs).This is a case report of a 70-year-old female with multicentric malignant eGISTs arising in the mesentery of ileum. On laparotomy, a large mass of 20 × 15 cm was found in the small bowel mesentery without involvement of the adjacent ileum, with multiple other small nodules resembling lymph nodes, present adjacent to it. Histopathological study of the excised lump, confirmed the mass to be malignant eGIST without involvement of the adjacent ileum, with cluster differentiation (CD)117 positive and of high-risk stratification. The mesenteric nodule was confirmed on histopathology to be malignant eGIST, similar to that of that of the primary, without any lymphoid tissue. Adjuvant imatinib mesylate treatment was started immediately postoperation with the patient doing well at 1 year of follow-up. We report this case, due to the rare occurrence of multifocal malignant eGISTS of small bowel mesentery, which is yet to be reported. The existing literature is unclear regarding the clinicopathology and management of multifocal malignant stromal tumors of the mesentery.

Multislice imaging of gastrointestinal stromal tumors

2018 ◽  
pp. 3-14
Keyword(s):  
Computed Tomography ◽  
Key Words ◽  
Gold Standard ◽  
Gastrointestinal Stromal Tumors ◽  
Mesenchymal Tumors ◽  
Stromal Tumors ◽  
Preferential Localization ◽  
Multislice Imaging ◽  
Computed Tomography Diagnosis

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the digestive tract (1%). These tumors express the CD 117 in 95% of cases. The stomach is the preferential localization (70%). Diagnosis is difficult and sometimes late. Progress of imaging has greatly improved the management and the prognosis. Computed tomography (CT) is the gold standard for diagnosis, staging, and treatment follow-up. The increasing recognition of GIST’s histopathology and the prolonged survival revealed some suggestive imaging aspects. Key words: gastro-intestinal stromal tumors; computed tomography; diagnosis

Imatinib in the Management of Multiple Gastrointestinal Stromal Tumors Associated With a Germline KIT K642E Mutation

2007 ◽  
Vol 131 (9) ◽  
pp. 1393-1396
Author(s):  
Janet Graham ◽  
Maria Debiec-Rychter ◽  
Christopher L. Corless ◽  
Robin Reid ◽  
Rosemarie Davidson ◽  
...  
Keyword(s):  
Gastrointestinal Stromal Tumors ◽  
Interstitial Cells Of Cajal ◽  
Germline Mutations ◽  
Mesenchymal Tumors ◽  
Stromal Tumors ◽  
Pdgfra Gene ◽  
Oncogenic Mutations ◽  
Esophageal Tumors ◽  
Exon 11 ◽  
Cells Of Cajal

Abstract Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gut and are distinguished by expression of CD117 (c-Kit). Oncogenic mutations in the KIT or PDGFRA gene are detected in approximately 85% of sporadic GISTs. In recent years, examples of familial GIST have been reported in which germline mutations of KIT or PDGFRA result in multiple GISTs, skin disorders, and other abnormalities. The most common germline mutations are in KIT exon 11, mutations in exons 8 and 17 have also been described, and there are 2 families with germline PDGFRA mutations. We present a case in which a germline KIT exon 13 mutation (K642E) was discovered in a patient with multiple GISTs of rectum, small intestine, and esophagus, as well as diffuse hyperplasia of the interstitial cells of Cajal. To our knowledge, this is only the second germline example of this particular mutation. The patient's esophageal tumors were stabilized with imatinib.

Large Omental Metastases of a Gastrointestinal Stromal Tumor

Medicina ◽  
2011 ◽  
Vol 47 (11) ◽  
pp. 86
Author(s):  
Povilas Ignatavičius ◽  
Tomas Petraitis ◽  
Žilvinas Saladžinskas ◽  
Lilija Butkevičienė ◽  
Kristina Žvinienė
Keyword(s):  
Case Report ◽  
Gastrointestinal Tract ◽  
Gastrointestinal Stromal Tumor ◽  
Gastrointestinal Stromal Tumors ◽  
Interstitial Cells Of Cajal ◽  
Metastatic Tumor ◽  
Stromal Tumor ◽  
Mesenchymal Tumors ◽  
Stromal Tumors ◽  
Cells Of Cajal

Gastrointestinal stromal tumors are rare tumors, originating from the interstitial cells of Cajal. They are the most common mesenchymal tumors of the gastrointestinal tract. Metastatic tumor is treated with imatinib mesylate. A case of large metastases of a gastrointestinal stromal tumor to the omentum, diagnosis and treatment principles are presented in this case report.

Gallbladder GIST: A review of literature

2019 ◽  
Vol 92 (1) ◽  
pp. 1-5
Author(s):  
Ashish Gupta
Keyword(s):  
Gastrointestinal Stromal Tumors ◽  
Interstitial Cells Of Cajal ◽  
Case Reports ◽  
Gallbladder Wall ◽  
Postoperative Adjuvant Therapy ◽  
Review Of Literature ◽  
Mesenchymal Tumors ◽  
Stromal Tumors ◽  
Extensive Search ◽  
Cells Of Cajal

Mesenchymal tumors of the gallbladder are rarely encountered in clinical practice. The Gastrointestinal Stromal tumors(GIST) of the gallbladder are 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 form of GIST .The literature on GIST arising from the gallbladder wall is limited to few case reports only.on 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 are presenting a review of the available literature on this rare pathology.

scholarly journals Gastrointestinal Stromal Tumors of Small Intestine

2019 ◽  
Vol 05 (03) ◽  
pp. e92-e95 ◽  
Author(s):  
Tanweerul Huda ◽  
Mahendra Pratap Singh
Keyword(s):  
Gastrointestinal Tract ◽  
Interstitial Cells Of Cajal ◽  
Surgical Excision ◽  
Biological Behavior ◽  
Pacemaker Cells ◽  
Mesenchymal Tumors ◽  
Stromal Tumors ◽  
Cells Of Cajal ◽  
Oncogene Protein

AbstractGastrointestinal stromal tumor (GIST) is defined as mesenchymal tumors of the gastrointestinal tract expressing proto-oncogene protein CD117. They are the most common sarcomatous tumors of the gastrointestinal tract. GISTs are presumed to arise from interstitial cells of Cajal or gastrointestinal pacemaker cells which control gut motility. They have unpredictable biological behavior. Prognosis is dependent on tumor size as well as mitotic count. Radical surgical excision is the treatment of choice. They rarely metastasize to lymph nodes. Imatinib therapy is used as an adjuvant therapy. The follow-up of patients postsurgery is not standardized.

Lack of Prognostic Significance of Connexin-43 Labeling in a Series of 46 Gastrointestinal Stromal Tumors

2011 ◽  
Vol 26 (2) ◽  
pp. 124-128 ◽  
Author(s):  
Marko Boban ◽  
Neven Ljubicic ◽  
Marko Nikolic ◽  
Davor Tomas ◽  
Mario Zovak ◽  
...  
Keyword(s):  
Gastrointestinal Stromal Tumors ◽  
Connexin 43 ◽  
Prognostic Significance ◽  
Tumor Grade ◽  
Time Frame ◽  
Mesenchymal Tumors ◽  
Stromal Tumors ◽  
Junction Protein ◽  
Significant Difference

Background Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors with variable malignant potential. Connexin-43 (C×43) is the commonest gap-junction protein and has been frequently investigated in oncology. Our aim was to establish the immunohistochemical expression of C×43 in relation to GIST location, size, Ki67 index, tumor grade and follow-up. Materials and methods The study included postoperative samples of 46 patients treated for GIST in the 1999–2010 time frame. Complete clinical workup was available for 38 patients (82.6%); total surgical resection was carried out in 32 (84.2%) patients, while 13 (34.2%) patients underwent chemotherapy. Median follow-up was 40.7 months (range, 1-134). Results The calculated incidence of GIST in our setting was 11.5 per million. C×43 was expressed in 43/46 (93.5%) GIST cases, with a significant difference between stomach- and small intestine-derived tumors (p=0.006). Ki67 was 10% on average (range, 1–22) and was not correlated with tumor location (p=0.194). C×43 did not show significance with regard to tumor size (p=0.264) or higher tumor grade (p=0.658), as opposed to Ki67, which significantly correlated with both (p=0.0048 and p<0.001, respectively). C×43 and Ki67 were not significantly correlated (p=0.708). Ki67 correlated with time to recurrence (p=0.022). Ki67 >11% was taken as the indication to start imatinib chemotherapy (sensitivity 61.5%, specificity 92.0%, p=0.022). Ten (66.7%) of 15 patients with long-term (>5 years) follow-up were in remission. Conclusion C×43 was frequently expressed in GISTs regardless of tumor site. However, no significant relationships to histopathological parameters suggestive for prognosis were found. Further investigations might clarify the roles of C×43 in GIST oncogenesis.

scholarly journals An unusual presentation of a retroperitoneal gastrointestinal stromal tumors

2021 ◽  
Vol 8 (4) ◽  
pp. 1325
Author(s):  
Samir Paruthy ◽  
Shivani B. Paruthy
Keyword(s):  
Gastrointestinal Stromal Tumors ◽  
Residual Disease ◽  
Histopathological Examination ◽  
Broad Ligament ◽  
Pelvic Mass ◽  
Stromal Tumors ◽  
Immune Histochemistry ◽  
Cells Of Cajal ◽  
Complete Tumor

Gastrointestinal stromal tumors (GIST) are soft tissue mesenchymal tumor originating from intestinal cells of Cajal which are pace makers of the intestine. GIST commonly arises from stomach and intestine although they seem to appear occasionally in mesentery, omentum and esophagus, but origin from retro-peritoneum is very infrequent.  We herein report case of a huge retroperitoneal GIST measuring 16x13x9 cm size, in a 52-year-old lady occupying whole abdomen arising as pelvic mass. GIST was initially mistaken with associated large intramural fibroid uterus and a large pedunculated sub-serosa broad ligament fibroid. Patient was taken for abdominal open hysterectomy with complete tumor excision after all adhesions from adjoining intestine was separated. Histopathological examination of the mass confirmed it was retroperitoneal GIST with positive immune-histochemistry. Patient responded well to Imatinib therapy, and for last 4 years there has been no residual disease or recurrence as evidenced with regular follow up. 

scholarly journals Gastrointestinal stromal tumors: Microscopic and immunihistochemical features

2007 ◽  
Vol 64 (9) ◽  
pp. 597-603
Author(s):  
Gorana Rancic ◽  
Vuka Katic ◽  
Ljubinka Jankovic-Velickovic ◽  
Milan Rancic
Keyword(s):  
Mitotic Index ◽  
Tumor Size ◽  
Gastrointestinal Stromal Tumors ◽  
Malignant Tumors ◽  
Smooth Muscle Actin ◽  
Biological Behavior ◽  
Mesenchymal Tumors ◽  
Stromal Tumors ◽  
S 100 ◽  
Cells Of Cajal

Background/Aim. Gastrointestinal (GI) stromal tumors (GIST) are the most common mesenchymal tumors of GI tract. The most frequent localization is gastric (60-70%) followed by intestinal localization (20-30%). The histogenesis, classification, diagnostic criteria and biological behavior of GIST are still discussable. Gastrointestinal stromal tumors are thought to originate from interstitial pacemaker intestinal cells of Cajal. Histologic appearance of a GIST is complicated and biologic potential unpredictable. The aim of of tha study was to investigate anatomic localization , the size of the tumor, incapsulation, microscopic and immunohistochemical characteristics. Methods. The study involved 21 GIST taken by a complete resection in the period from 1994-2006. The analysed parameters were the localization, size, microscopic (mitotic index, nectosis, bleeding, invasivity) and immunohistochemical characteristics (CD117 (ckit), CD34, desmin, vimentin, smooth muscle actin and s- 100 protein expression. Results. Gastrointestinal stromal tumors (n=21) size varied from 10-150 mm were most frequently gastric localised with predominance of malignant tumors (85.72%). Most GIST were comprised of a uniform spindle cell population, but some were dominated by epitheloid cells. Eosinophilic cells stained CD117, CD34 and vimentin positively, were usually arranged in fascicles with the presence of skeinoid fibers. Positive correlation of biologic potential and tumor size, haemorrhagia and mitotic index were found, so as negative correlation of biologic potential and incapsulation. Conclusion. The above results, a specially localization, tumor size, mitotic index, CD117, CD34 and vimentin positivity, may be helpful for setting of a widespread criteria for diagnostic and differential diagnosis of GIST and their use in practice and therapy. .

scholarly journals Clinical Diagnosis of Gastrointestinal Stromal Tumor (GIST): From the Molecular Genetic Point of View

Cancers ◽  
2019 ◽  
Vol 11 (5) ◽  
pp. 679 ◽  
Author(s):  
Chiao-En Wu ◽  
Chin-Yuan Tzen ◽  
Shang-Yu Wang ◽  
Chun-Nan Yeh
Keyword(s):  
Gold Standard ◽  
Gastrointestinal Stromal Tumors ◽  
Interstitial Cells Of Cajal ◽  
Molecular Genetic ◽  
Therapeutic Strategies ◽  
Point Of View ◽  
Protein Markers ◽  
Mesenchymal Tumors ◽  
Stromal Tumors ◽  
Cells Of Cajal

Gastrointestinal stromal tumors (GISTs) originating from the interstitial cells of Cajal are mesenchymal tumors of the gastrointestinal tract and have been found to harbor c-KIT mutations and KIT (CD117) expression since 1998. Later, PDGFRA mutations, SDH alterations, and other drive mutations were identified in GISTs. In addition, more and more protein markers such as DOG1, PKCθ were found to be expressed in GISTs which might help clinicians diagnose CD117-negative GISTs. Therefore, we plan to comprehensively review the molecular markers and genetics of GISTs and provide clinicians useful information in diagnostic and therapeutic strategies of GISTs. Twenty years after the discovery of KIT in GISTs, the diagnosis of GISTs became much more accurate by using immunohistochemical (IHC) panel (CD117/DOG1) and molecular analysis (KIT/PDGFRA), both of which constitute the gold standard of diagnosis in GISTs. The accurately molecular diagnosis of GISTs guides clinicians to precision medicine and provides optimal treatment for the patients with GISTs. Successful treatment in GISTs prolongs the survival of GIST patients and causes GISTs to become a chronic disease. In the future, the development of effective treatment for GISTs resistant to imatinib/sunitinib/regorafenib and KIT/PDGFRA-WT GISTs will be the challenge for GISTs.

scholarly journals Jejunal gastrointestinal stromal tumors masquerading as an appendicular mass: an unusual presentation

2020 ◽  
Vol 7 (8) ◽  
pp. 2769
Author(s):  
Rajat R. Choudhari ◽  
Annagiri Raghavendra ◽  
Harish Rao Kalbavi
Keyword(s):  
Small Bowel ◽  
Gastrointestinal Stromal Tumors ◽  
Case Reports ◽  
Iliac Fossa ◽  
Unusual Presentation ◽  
Gi Bleeding ◽  
Stromal Tumors ◽  
Cells Of Cajal ◽  
Resection And Anastomosis ◽  
Appendicular Mass

Gastrointestinal stromal tumors (GISTs) are a rare variety of tumors of mesenchymal origin found in the gastrointestinal (GI) tract forming about 1% of all GI tumors. These originate from the interstitial cells of Cajal. Small bowel GISTs have been shown to present as obscure GI bleeding, obstruction and perforation in literature. We report a 57 years old female patient presenting with pain abdomen, fever and vomiting and palpable right iliac fossa (RIF) mass diagnosed as an appendicular mass and managed conservatively. She was planned interval appendicectomy and was discovered to have a jejunal GIST at laparotomy treated with resection and anastomosis. There are case reports of small bowel GISTs presenting as sources of obscure or overt GI bleeding and luminal or extra luminal mass causing small bowel obstruction. Surgery is mainstay of treatment with imatinib for adjuvant or neoadjuvant therapy. This case highlights an unusual presentation of a jejunal GIST with a sealed off perforation mimicking an appendicular mass in the RIF treated by surgical resection followed by adjuvant Imatinib therapy. GIST being an uncommon tumor with varied presentations can lead to misdiagnosis and delays in treatment. This differential should be kept in mind while evaluating small bowel pathologies to aid a timely diagnosis.   

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