Gastrointestinal stromal tumor overview: Difference between revisions
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==Historical Perspective== | ==Historical Perspective== | ||
Prior to the advent and use of electron microscopy, gastrointestinal stromal tumors (GIST) were classified as smooth muscle tumors such as leiomyomas or leiomyosarcomas. In 1983, Mazur and Clark and Schaldenbrand and Appleman in 1984 were the first to describe gastrointestinal stromal tumors as an independent entity of intra-abdominal tumors that were neither carcinomas nor exhibit histologic features of smooth muscle or nerve cells. In 1998, Kindblom et al described the origin of GIST as pluripotential mesenchymal stem cells which were programmed to differentiate into the interstitial cell of Cajal. In 1998 Hirota and others were the first to describe c-kit (proto-oncogene) mutations as the cause of GIST. In 2001, Joensuu was the first to report successful treatment of patients with advanced GIST on molecular-targeted therapy (imatinib). | Prior to the advent and use of [[electron microscopy]], gastrointestinal stromal tumors (GIST) were classified as [[smooth muscle]] [[tumors]] such as [[Leiomyoma|leiomyomas]] or [[Leiomyosarcoma|leiomyosarcomas]]. In 1983, Mazur and Clark and Schaldenbrand and Appleman in 1984 were the first to describe gastrointestinal stromal tumors as an independent entity of intra-abdominal tumors that were neither [[carcinomas]] nor exhibit [[histologic]] features of [[smooth muscle]] or [[nerve cells]]. In 1998, Kindblom et al described the origin of GIST as pluripotential mesenchymal stem cells which were programmed to differentiate into the [[interstitial cell of Cajal]]. In 1998 Hirota and others were the first to describe c-kit [[Proto-oncogene|(proto-oncogene]]) mutations as the cause of GIST. In 2001, Joensuu was the first to report successful treatment of patients with advanced GIST on molecular-targeted therapy ([[imatinib]]). | ||
==Pathophysiology== | ==Pathophysiology== | ||
Revision as of 19:40, 18 December 2017
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Gastrointestinal stromal tumor Microchapters |
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Differentiating Gastrointestinal stromal tumor from other Diseases |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]
Overview
In medical oncology, gastrointestinal stromal tumors (GIST) are a rare tumor of the gastrointestinal tract. GIST is a form of connective tissue cancer, or sarcoma. GISTs are therefore non-epithelial tumors, separate from more common forms of bowel cancer. Majority of the cases occur in the stomach(70%), 20% of cases in the small intestine and less than 10% in the esophagus. Small tumors are generally benign, especially when cell division rate is slow, but large tumors disseminate to the liver, omentum and peritoneal cavity. They rarely occur in other abdominal organs. Gastrointestinal stromal tumor affects men and women equally.
Historical Perspective
Prior to the advent and use of electron microscopy, gastrointestinal stromal tumors (GIST) were classified as smooth muscle tumors such as leiomyomas or leiomyosarcomas. In 1983, Mazur and Clark and Schaldenbrand and Appleman in 1984 were the first to describe gastrointestinal stromal tumors as an independent entity of intra-abdominal tumors that were neither carcinomas nor exhibit histologic features of smooth muscle or nerve cells. In 1998, Kindblom et al described the origin of GIST as pluripotential mesenchymal stem cells which were programmed to differentiate into the interstitial cell of Cajal. In 1998 Hirota and others were the first to describe c-kit (proto-oncogene) mutations as the cause of GIST. In 2001, Joensuu was the first to report successful treatment of patients with advanced GIST on molecular-targeted therapy (imatinib).
Pathophysiology
On microscopic histopathological analysis, spindle cells or plump epithelioid cells are characteristic findings of gastrointestinal stromal tumor.
Causes
There are no established causes for gastrointestinal stromal tumor.
Epidemiology and Demographics
Gastrointestinal stromal tumor affects men and women equally.
Risk factors
The most potent risk factor in the development of GISTs are age 50-80 and certain genetic syndromes like neurofibromatosis type 1, Carney-Stratakis syndrome and familial gastrointestinal stromal tumor syndrome.
Screening
Differential Diagnosis
Gastrointestinal stromal tumor must be differentiated from gastrointestinal leiomyoma, gastrointestinal leiomyosarcoma, gastrointestinal lymphoma / gastric lymphoma, gastrointestinal schwannoma and gastrointestinal carcinoid.
Natural History, Complications and Prognosis
Most common site of involvement of GIST is stomach(70%).
Staging
According to the American Joint Committee on Cancer, there are 4 stages of gastrointestinal stromal tumor based on the tumor spread.
History and Symptoms
Symptoms of gastrointestinal stromal tumor include dysphagia, gastrointestinal hemorrhage, and vague abdominal pain.
Physical Examination
Laboratory Examination
Abdominal X-ray
On abdominal X-ray, gastrointestinal stromal tumor is characterized by soft tissue density displacing bowel loops.
CT scan
Abdominal CT scan may be helpful in the diagnosis of gastrointestinal stromal tumor.
MRI
MRI scan may be helpful in the diagnosis of gastrointestinal stromal tumor.
Ultrasound
Other Imaging Findings
Fluoroscopy may be helpful in the diagnosis of gastrointestinal stromal tumor.
Other Diagnostic studies
Medical Therapy
The predominant therapy for gastrointestinal stromal tumor is surgical resection. Adjunctive chemotherapy/tyrosine Kinase Inhibitor therapy may be required.
Surgical Therapy
The predominant therapy for gastrointestinal stromal tumor is surgical resection. Adjunctive chemotherapy/tyrosine Kinase Inhibitor therapy may be required.