Published online Sep 16, 2024. doi: 10.12998/wjcc.v12.i26.5990
Revised: June 8, 2024
Accepted: July 3, 2024
Published online: September 16, 2024
Processing time: 95 Days and 16.6 Hours
Extragastrointestinal stromal tumors (EGIST) and gastrointestinal stromal tumors are of similar pathological type and form. Here we report a rare case of EGIST diffusely distributed in membranous tissue in abdominal cavity, the feature of which included diffuse tumors at membranous tissue in entire abdominal cavity and spontaneous bleeding of the tumors.
The patient was a 71-year man and hospitalized due to continuous pain at lower abdomen for more than 10 days. Upon physical examination, the patient had flat and tough abdomen with mild pressing pain at lower abdomen, no obvious abdominal mass was touchable, and shifting dullness was positive. Positron emission tomography-computed tomography (CT) showed that in his peritoneal cavity, there were multiple nodules of various sizes, seroperitoneum, multiple enlarged lymph nodes in abdominal/pelvic cavity and right external ilium as well as pulmonary nodules. Plain CT scanning at epigastrium/hypogastrium/pelvic cavity + enhanced three-dimensional reconstruction revealed multiple soft tissue nodules in abdominal/pelvic cavity, peritoneum and right groin. Tumor marker of carbohydrate antigen 125 was 808 U/mL, diffuse tuberous tumor was seen in abdominal/pelvic cavity during operation with hematocelia, and postoperative pathological examination confirmed EGIST. Imatinib was administered with better therapeutic effect.
Gene testing showed breast cancer susceptibility gene 1 interacting protein C-terminal helicase 1 and KIT genovariation, and the patient was treated with imatinib follow-up visit found that his clinical symptoms disappeared and the tumor load alleviated obviously via imageological examination.
Core Tip: Extragastrointestinal stromal tumors (EGIST) are less common in comparison with gastrointestinal stromal tumors. EGIST tumors in the membranous tissue are rare and tumors independently developing in membrane tissues such as greater omentum, mesentery or peritoneum are occasionally reported. Diffuse membranous tumor in entire abdominal cavity is extremely rare worldwide. We report a case of EGIST with the tumors like cobblestone being diffusely distributed in entire abdominal cavity accompanied by spontaneous bleeding. The tumors may not be fully resected by surgery, hence we made biopsy, pathological examination and gene detection to determine the therapy. Treatment with imatinib achieved better outcome.