Understanding abdominal mesenchymal tumors

  The concept of gastrointestinal mesenchymal tumor was first introduced by Mazur and Clark in 1983 as a non-epithelial mesenchymal tumor of the gastrointestinal tract originating from Cajal cells. 95% of GISTs originate in the gastrointestinal tract: stomach (50%-60%), small intestine (20%-30%), large intestine (10%), esophagus (5%), and less than 10% in other sites such as mesentery, omentum and retroperitoneum. GIST is a gastrointestinal tumor with malignant potential. With the improvement of diagnostic imaging technology, especially the development of gastrointestinal ultrasonography and ultrasound endoscopy, more and more subclinical symptoms and non-clinical manifestations, <5cm or even 1~2cm microscopic gastric mesenchymal tumors have been detected. Our ultrasound colleagues said that in the past 5 years, they found 10 or more cases of suspected gastric mesenchymal tumor each year.  Of course, the diagnosis of this disease must be confirmed by pathology and immunohistochemistry, and a large number of microscopic spindle cell distribution, positive CD117 (tyrosine kinase growth factor receptor) and CD34 (bone marrow stem cell antigen) tests are the "gold standard" for the diagnosis of this disease. However, as the understanding of this disease has improved, more and more surgeons have been able to think of mesenchymal tumor diagnosis in the face of doubtful gastrointestinal and abdominal tumors.  In the past two years, I have encountered patients with mesenchymal tumors in the abdominal cavity, basically of gastric origin, which can be completely resected, but I have also encountered huge gastric mesenchymal tumors that densely invade and infiltrate the surrounding tissues and organs, bleeding at every turn and even helplessness, and finally can only hastily close the abdomen.  The accepted principles of surgical treatment for GIST should be as follows: complete tumor resection with negative margins is required as the standard. The extent of resection should be at least 1 to 2 cm from the tumor. Lymph node dissection is not routinely advocated. In cases where the GIST invades the surrounding tissues and can be resected "in its entirety", combined organ resection can be performed according to the principle of complete tumor resection. The depth of resection prefers total resection to endoscopic or laparoscopic enucleation of the GIST mass, and intraoperative manipulation should be performed to avoid tumor rupture, leading to abdominal implantation and liver metastasis.  The previous year I saw a patient with a widely distributed abdominal mesenchymal tumor whose shape could be compared to a dense abdominal cavity like a toad's skin. If it had not been for the bleeding tumor that caused the acute abdomen I don't know how long it would have lasted until it was discovered, but when asked about the medical history, he was sure that he had been working normally before the disease and had not felt any significant discomfort. But I believe it will not drag on for long! But doesn't it also suggest to us that non-gastrointestinal endogenous mesenchymal tumors have more insidious symptoms?  In the past 5-6 years, the efficacy of targeted therapy with Imatinib (trade name: Gleevec) for gastrointestinal mesenchymal tumors has been recognized. Gleevec has achieved a tumor control rate of up to 85% in the treatment of GIST by blocking the tyrosine kinases in the Kit and PDGFR α membranes. treatment.  However, it is important to recognize that Gleevec is mostly in partial remission or stable disease in the treatment of GIST, with few complete remissions (CR rates of about 2% to 3%). This means that in most GISTs, Gleevec can only control tumor growth but cannot completely destroy the tumor. Mesenchymal tumors are insensitive to both chemotherapy and radiotherapy, and the only effective treatment is to remove the tumor.