Gallbladder GIST: A review of literature

2019 ◽  
Vol 92 (1) ◽  
pp. 1-5
Author(s):  
Ashish Gupta

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.

2007 ◽  
Vol 131 (9) ◽  
pp. 1393-1396
Author(s):  
Janet Graham ◽  
Maria Debiec-Rychter ◽  
Christopher L. Corless ◽  
Robin Reid ◽  
Rosemarie Davidson ◽  
...  

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.


Medicina ◽  
2011 ◽  
Vol 47 (11) ◽  
pp. 86
Author(s):  
Povilas Ignatavičius ◽  
Tomas Petraitis ◽  
Žilvinas Saladžinskas ◽  
Lilija Butkevičienė ◽  
Kristina Žvinienė

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.


Cancers ◽  
2019 ◽  
Vol 11 (5) ◽  
pp. 679 ◽  
Author(s):  
Chiao-En Wu ◽  
Chin-Yuan Tzen ◽  
Shang-Yu Wang ◽  
Chun-Nan Yeh

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.


Author(s):  
Tommaso Marcucci ◽  
◽  
Elena Falsetti ◽  
Chiara Genzan ◽  
Lorenzo Pandolfini ◽  
...  

Gastrointestinal Stromal Tumors (GISTs) are rare neoplasms but they represent the most common type of mesenchymal tumors found in the Gastrointestinal (GI) tract [1-5]. GISTs arise from the interstitial cells of Cajal, “Pacemaker Cells”, that play a neuromotor role in normal gut motility [3,6]. They account for < 1% of gastrointestinal tumors with an estimated annual incidence of 10 to 20 cases for one million of the general population [1,2]. Treatment has not yet been standardized, but surgical resection remains the mainstay for non-metastatic GISTs [5]. The use of laparoscopic techniques has been widely debated.


Oncotarget ◽  
2017 ◽  
Vol 8 (25) ◽  
pp. 41026-41043 ◽  
Author(s):  
Pierre Vandenberghe ◽  
Perrine Hagué ◽  
Steven C. Hockman ◽  
Vincent C. Manganiello ◽  
Pieter Demetter ◽  
...  

2009 ◽  
Vol 136 (5) ◽  
pp. A-51
Author(s):  
Michael R. Bardsley ◽  
Laura Popko ◽  
David L. Young ◽  
Gianrico Farrugia ◽  
Brian P. Rubin ◽  
...  

2001 ◽  
Vol 158 (3) ◽  
pp. 817-823 ◽  
Author(s):  
Tohru Tsujimura ◽  
Chiaki Makiishi-Shimobayashi ◽  
Johan Lundkvist ◽  
Urban Lendahl ◽  
Keiji Nakasho ◽  
...  

2019 ◽  
Vol 05 (03) ◽  
pp. e92-e95 ◽  
Author(s):  
Tanweerul Huda ◽  
Mahendra Pratap Singh

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.


2007 ◽  
Vol 64 (9) ◽  
pp. 597-603
Author(s):  
Gorana Rancic ◽  
Vuka Katic ◽  
Ljubinka Jankovic-Velickovic ◽  
Milan Rancic

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. .


2019 ◽  
Vol 05 (03) ◽  
pp. e65-e68 ◽  
Author(s):  
Prakash K. Sasmal ◽  
Rakesh Sharma ◽  
Susama Patra ◽  
Tushar S. Mishra ◽  
Pritinanda Mishra ◽  
...  

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.


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