Morphology of gastrointestinal stromal tumors: Historical perspectives (original) (raw)
Gastrointestinal Stromal Tumors: A Focus on Diagnosis and Management
Gastrointestinal stromal tumors (GISTs) are rare tumors of the gastrointestinal (GI) tract that usually start in very early forms of special cells found in the wall of the GI tract, called the interstitial cells of Cajal (ICCs). Symptoms are usually variable, depending on tumor size and location, but many patients are asymptomatic. Most gastrointestinal stromal tumors (GISTs) occur in the stomach or small intestine. These tumors might not cause any symptoms unless they are in a certain location or grow to a certain size. Small tumors might not cause any symptoms and may be found accidentally when the physician is looking for another problem. These tumors are often benign. The main treatment for GIST that hasn't spread is usually surgery to remove all of the tumors. The above mentioned topics, as well as classification, causes, clinical presentation, diagnosis and prognosis of GISTs were discussed in this review.
Gastrointestinal stromal tumors: A clinicopathologic and immunohistochemical study of 136 cases
Pathology & Oncology Research, 2005
The clinicopathologic features of 136 gastrointesti-primary cases. Ulceration observed by microscopic nal stromal tumors were analyzed. The tumors examination was common (36 of 110 cases, 32.7%), occurred in 60 women and 76 men, ranging in age explaining the clinically frequently observed gasfrom 19 to 88 years (median 59 years, mean 59.2 trointestinal bleeding. Unusual histological feayears). Sixty-one cases arose from stomach, 38 from tures such as stromal hyalinization and nuclear palsmall intestine and 11 from colon or rectum.
Gastrointestinal Stromal Tumors, Interstitial Cells of Cajal and their Nomenclature
Journal of Gastrointestinal & Digestive System, 2014
Currently, gastrointestinal stromal tumors (GIST) have been emphasized considerably in the literature. Following the date of the description of interstitial cells of Cajal (ICC) by Santiago Ramon y Cajal in the late 19th century, this issue has been very popular. Lately, discovery of the association of GISTs with c-Kit mutations in their development, and the significance of drugs such as imatinib, that inhibit c-Kit mutations in their treatment, has increased the interest of researchers. Our aim is to review the nomenclature about ICC and GISTs in the light of literature, to discuss the definition of GIST, which is a heterogeneous, pleomorphic tumor, in its historical progression and in the light of new data, and to suggest the naming these tumors as "tumor of Cajal", ""Cajal tumor" or "Cajal cell tumor" instead.
Gastrointestinal Stromal Tumors: Are They of Cajal Cell Origin?
Experimental and Molecular Pathology, 2002
Recently some reports have suggested that gastrointestinal stromal tumors (GIST) might originate from the interstitial cells of Cajal or differentiate into them because they express c-kit and/or CD34 and indicated that the majority of previously diagnosed smooth muscle tumors (SMT) actually belong to GIST, but are not true SMT. We, therefore, detected c-kit, CD34, SMA, and S-100 in 106 Chinese cases of gastrointestinal tumors, which were histopathologically diagnosed as smooth muscle tumors originally, to demonstrate the immunophenotypes of these tumors. The results showed that 73 cases had immunoreaction with c-kit and/or CD34, of which 48 cases showed coexpression with either SMA or S-100 or with both. A correlation between the immunophenotypes and known histopathological parameters was also shown here based on follow-up data. We suggest that the concept of GIST should not be used as an umbrella to cover all gastrointestinal mesenchymal tumors, but be defined in a narrow term as differing from true smooth muscle tumors.
Journal of Clinical and Diagnostic Research, 2022
The GISTs arise from the interstitial cells of Cajal, the pacemaker cells, comprising <1% of all primary GIT tract neoplasms [1]. The peak age of occurrence is 60-65 years, equally affecting males and females, which may be detected incidentally radiologically or present with abdominal symptoms [2]. More than 60% of them take place in the stomach. EGIST occur in the omentum, mesentery, retroperitoneum, and perineum. Histomorphologically, GISTs are hypocellular to densely cellular lesions [1]. The identification of primary mutations in KIT (tyrosine-protein kinase) (75-80%) or Platelet-derived growth factor receptor alpha (PGFRA) (7-15%) genes by IHC has revolutionised the diagnosis and led to the development of targeted therapy [1]. The diagnosis and treatment of small GISTs are important as surgery is the mainstay of treatment in Asian countries as compared to medical therapy in western countries. Optimal treatment of GISTs requires a team effort, including gastroenterologists, surgical and medical oncologists, pathologists, and radiologists [3]. This case series was done to study the clinicopathological, histomorphological, and IHC spectrum of GISTs along with their risk stratification according to the modified Miettinen and Lasota's algorithm [4]. The American Joint Committee on Cancer (AJCC) Cancer Staging (TNM) 8 th edition was used for staging and grading [5]. Following permission from the Institutional Ethics Committee of Clinical Research (No. 289/2021) was approved, eight patients with GISTs diagnosed between January 2017 and December 2020 are presented in this case series. CASE SERIES Case 1 A 36-year-old man presented to the Department of Surgery with chief complaints of epigastric discomfort for a month and vomiting for five days. A well-defined, spherical, broad-based isoechoic lesion measuring 4.3x3.4 cm with central anechoic cystic regions was seen on Ultrasound Sonography (USG), emerging from the wall of the pylorus producing luminal compression and stomach dilatation. A comparable tumour was detected using Contrast-Enhanced Computer Tomography (CECT), which raised the probability of GIST. Based on clinical findings and radiological investigations, the possibility of GIST or leiomyoma was considered. The histopathology laboratory received a 0.5 cm size upper GI scopy biopsy which microscopically showed mainly moderate chronic inflammation with no submucosal tissue. Eventually, excision of mass with distal stomach, pylorus and omentum was performed. Grossly, a submucosal tumour in region of pylorus measuring 4.0x3.5x3.0 cm was seen. The cut surface of the tumour was well-circumscribed, greyish-white with a few cystic areas. Sections from the tumour showed spindle cells arranged in fascicles and whorls with a round to oval nucleus with minimal pleomorphism and eosinophilic cytoplasm with pointed ends [Table/Fig-1a]. Mitosis was <5 per 20-25 hpf (high power field). Omentum was free from metastasis. A diagnosis of spindle cell tumour, suggestive of GIST, grade G1, very low-risk category was given. Strong, diffuse IHC positivity for CD117 [Table/Fig-1b] and vimentin confirmed the provisional diagnosis. S-100 was weakly positive and desmin was negative, which excluded neural and smooth muscle origin tumours. For six months there was no recurrence after which the patient was lost to follow-up. Case 2 A 75 year-old-male consulted a general surgeon at the Outpatient Department (OPD) for symptoms of increased frequency of stool with difficulty in defecation for six months and weakness for one year. For 20 days he felt a mass in the left iliac fossa. On
Gastro intestinal stromal tumor: A case report
Indian Journal of Radiology and Imaging, 2006
Background: Gastrointestinal stromal tumor (GIST) is a rare gastrointestinal tumor but the most common mesenchymal tumor. It originates in the interstitial cells of Cajal, which on their own membranes have a specific antigen designated as CD117 or KIT receptor, a reliable indicator of tumor detection and therapy. Case: We reported a case of a 63-year-old male patient, who presented with symptoms of severe abdominal pain, discomfort, and secondary anemia. After a physical examination and a CT scan of the abdomen with angiography, the presence of a large tumor in the right hypochondrium was detected. Blood tests showed a high C-reactive protein and low hemoglobin. The patient had an exploratory laparotomy on his abdomen with complete removal of the tumor growth, which was then histologically analyzed. The obtained result from the histological analysis showed a high-degree of GIST. Conclusion: Early diagnosis of GISTs is of paramount importance to reduce mortality rate. This requires good background in pathobiology and knowledge of the physical manifestations of this malignant tumor. When this is combined with various imaging techniques, it becomes the gold standard method for GIST diagnosis.
Gastrointestinal Stromal Tumor of the Stomach
Internal Medicine, 1997
We report a case of gastrointestinal stromal tumor (GIST) of the stomach. The patient was a 79-year-old womanwith two gastric submucosal tumors detected by ultrasonography. Proximal gastrectomy was carried out and the tumors were diagnosed as GIST by histological and immunohistochemical investigations. Mesenchymaltumors of the gastrointestinal tract have been traditionally regarded as largely leiomyomatous lesions. However, GIST and other tumors have been distinguished recently on the basis of tumor cell differentiation shown by immunohistochemical studies. Wediscuss the concept and the immunohistochemical characteristics of GIST.
Management of gastrointestinal stromal tumors: from diagnosis to treatment
Swiss Medical Weekly
Up to 20 years ago most mesenchymal tumours of the digestive tract were considered to be of smooth muscle or perineural origin. In 1983, Mazur and Clark [1] coined the term gastrointestinal stromal tumours (GIST) to indicate a distinctive subgroup of gastrointestinal mesenchymal tumours which could be classified neither as neurogenic-nor smooth muscle-derived. Kindblom et al. [2] proposed that these tumours may originate from the interstitial cell of Cajal, an intestinal pacemaker cell, and suggested the name gastrointestinal pacemaker cell tumour. This hypothesis is supported by the observation that GIST cells show ultrastructural features and express cell markers typical of the normal interstitial cell of Cajal [2]. Histological features of GIST may explain the past diagnostic confusion with other mesenchymal tumours. Today, on the basis of pathological features, most gastrointestinal mesenchymal tumours previously designated as smooth muscle tumours, 145
Stromal tumors of the gastrointestinal tract: a report of4 cases and a review of the literature
Ospedali d'Italia - chirurgia
Background Gastrointestinal tumors (GISTs) belong to a group of rare neoplasms of the gastrointestinal tract, generally not recognized as distinct pathological entities until recently. The only treatment for local disease has been radical surgical resection. No adjuvant therapy has proven effective. Purpose The purpose of the study was to establish distinct pathological entities among GISTs using new immunohistochemical markers and to determine the feasibility of other therapeutic options as an alternative to surgery. Methods In all cases in which a diagnosis of GIST was made, immunohistochemical examination of the tumor was carried out. In patients undergoing surgery the lesion was localized. Treatment consisted of radical resection. Results There was no evidence of recurrence of disease following surgery. Consequently, no pharmacological therapy or adjuvant radiotherapy was deemed necessary. Conclusions GISTs comprise biologically distinct tumors. Treatment of local lesions consis...
Gastrointestinal stromal tumors: a clinicopathological and immunohistochemical study of 121 cases
Indian Journal of Gastroenterology, 2010
Background Primary mesenchymal tumors of the gastrointestinal tract are a heterogeneous group of tumors with a wide clinical spectrum, of which gastrointestinal stromal tumors (GIST) typically occur in middle-aged to older individuals. This study evaluated the clinicopathological and morphological features of GIST of the intestinal tract. Methods The study included 108 gastrointestinal and 13 extra-gastrointestinal stromal tumors involving the mesentry and retroperitoneum between January 1989 and July 2007. Immunohistochemical expression of CD117, CD34, SMA, Desmin, S100, and Ki-67 were studied. Results GIST comprised 108 of 120 (90%) of the mesenchymal tumors. The tumor was located in the stomach in the majority (55%) of patients followed by small intestine (30%), retroperitoneum (7%) and the colorectum (4%). There was a significant correlation between tumor size and mitotic index, with larger tumors having higher mitotic index (p<0.001). Mitotic index per 5 mm 2 correlated with high cellularity (p<0.001), presence of necrosis (p<0.001) and presence of mucosal invasion (p=0.01). Expression of CD117 was seen in 94%, CD34 in 59%, SMA in 41%, S-100 in 33%, and desmin in 4% of tumors. Conclusion We found GIST to be the most common mesenchymal tumor of the gastrointestinal tract.