Morphology of gastrointestinal stromal tumors: Historical perspectives (original) (raw)
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Gastrointestinal Stromal Tumors: An Ultrastructural Study
International Journal of Surgical Pathology, 2002
Gastrointestinal stromal tumors (GISTs) represent an enigmatic group of lesions of uncertain phenotype and biologic potential. Although earlier studies suggested smooth muscle cells, schwann cells, or neuronal differentiation, more recent evidence indicates that these tumors show phenotypic features that are similar to the interstitial cells of Cajal. Recently, investigators have begun to evaluate these lesions in a site-specific manner and have found that, in addition to morphologic differences between them, their biologic behavior also appears to be linked to their anatomic location. Many of these studies have emphasized the histologic and immunophenotypic features of GISTs in relation to their sites of origin, however, their site-specific ultrastructural characteristics have received little attention in the literature. In this study, we evaluated 34 GISTs (15 gastric, 12 small intestinal, 4 colonic, and 3 omental) for a variety of ultrastructural features in an effort to identify site-specific similarities and differences. Tumors predominantly composed of epithelioid cells were more commonly seen in gastric (60%) and omental (67%) tumors than in those of the small intestine (33%) and colon (0%). Cytoplasmic filaments and intercellular junctions were commonly seen in tumors from all locations, the filaments frequently forming paranuclear aggregates in the epithelioid cells. Tumors from all sites were composed of cells with surface filopodia and interdigitating cell processes, but in tumors of the stomach and omentum the filopodia were usually short and minimally intertwined, whereas those of small and large intestinal GISTs were characteristically long and complex. Basal lamina, though poorly formed, was present only in tumors of gastric and omental origin (13% and 67%, respectively). Pinocytotic vesicles were also seen in tumors from these sites (33% of gastric tumors and 67% of omental lesions) as well as those of the small intestine (17%) and the colon (25%). None of the gastric or omental tumors had microtubules; they were, however, seen in small intestinal (33%) and colonic (25%) stromal tumors. Skenoid fibers were seen in 33% of small intestinal GISTs and 1 metastatic gastric GIST. Overall, gastric and omental tumors have better developed features of myogenic differentiation and have blunt filopodia and minimally intertwined cell processes. Indeed, these 2 groups are indistinguishable ultrastructurally, raising the possibility that the genesis of omental GISTs is similar to that of gastric stromal tumors. Small intestinal stromal tumors have characteristic interdigitating cell processes and numerous elongate filopodia-like structures harboring intercellular junctions as well as microtubules and extracellular skenoid fibers. The constituent cells in colonic stromal tumors, while more reminiscent of small intestinal stromal, were frequently more primitive in appearance. In conclusion, GISTs from different anatomic locations share many overlapping ultrastructural characteristics; however, a few features are distinctive. It is hoped that these findings will aid in their recognition and contribute to the classification of this heterogeneous group of neoplasms. Int J Surg Pathol 10(2): [101][102][103][104][105][106][107][108][109][110][111][112][113] 2002
Gastrointestinal Stromal Tumors—A Morphological and Immunohistochemical Study
Journal of Gastrointestinal Cancer, 2009
Purpose The rationale of this study was to assess the morphological and immunohistochemical characteristics of gastrointestinal stromal tumor (GIST) along with their risk stratification. Methods Record of 36 cases diagnosed as GIST over a period of 2 years (January 2007 to December 2008) was retrieved. Slides were reviewed for histological typing, immunohistochemical staining, and mitotic count. Cases were divided into very low, low, intermediate, and high-risk groups according to the Fletcher method of risk stratification (Table 1; Fletcher et al. (Int J Surg Pathol 10:81–89, 2002)). Mean, median, and mode were calculated for quantitative variables like age, tumor size, and mitotic count by using SPSS version 14. Frequencies and percentages were also calculated for qualitative variables like results of immunohistochemistry, tumor site, and histological subtypes. Results Out of 36 patients, 14 patients were male, and 22 were female. A total of 14 (39%) patients had tumor size between 2 and 5 cm, 13 (36%) patients had size between 5 and 10 cm, and 9 (25%) patients had size >10 cm. There was no tumor less than 2 cm in size. Twenty-one patients (58%) had mitoses <5/50 high power fields (HPF) while seven (20%) had mitoses between 5 and 10/50 HPF and eight (22%) >10/50 HPF. Thirty-one (86%) of cases were strongly positive for CD117 while CD34 was positive in 81% of the cases. Most frequent histological type was hypercellular spindle cell type, and most frequent site of presentation was stomach. Seven patients fell into low risk, ten patients intermediate risk, and 19 patients in high risk groups. There were no patients in very low risk group. Conclusion By using microscopy and immunohistochemical techniques, GISTs can be diagnosed accurately and treated efficiently. Risk stratification and histological subtyping have emerged as efficient tools to predict malignant behavior.
Gastrointestinal Stromal Tumors—A Mini Review
2021
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. They are potentially malignant, and have an unpredictable evolution. The origin of these tumors is in the interstitial cells of Cajal, which are cells that are interposed between the intramural neurons and the smooth muscle cells of the digestive tract. GISTs are characterized by mutations in the gene c-Kit, but also other mutations, such as those of the platelet-derived growth factor receptor alpha. The most common locations of these tumors are the stomach and small intestine, although they can occur at any level of the digestive tract and occasionally in the omentum, mesentery and peritoneum. Most cases of GISTs are sporadic, and about 5% of cases are part of family genetic syndromes. The correct diagnosis of GIST is determined by histopathological examination and immunohistochemistry. According to histopathology, there are three main types of GISTs: spindle cell type, ...
Gastrointestinal stromal tumors: A clinical and histopathological presentation of 27 cases
Turkish Journal of Surgery
Objective: Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors that express type 3 tyrosine kinase receptors and are thought to develop from the neoplastic transformation of the interstitial Cajal cells. The present study was performed to morphologically and immunohistologically evaluate GISTs, to compare their qualities using a GIST risk categorization system, and to identify the diagnostic and prognostic parameters of GISTs. Material and Methods: A total of 27 patients with GISTs underwent treatment and were followed up at the Gaziosmanpaşa Taksim Training and Research Hospital. Descriptive statistics was used to calculate the mean and median values. Survival analysis was performed by the Kaplan-Meier method. The analyses were performed using the SPSS version 22.0 software. Results: The mean follow-up time was 3.5 (5 months to 13 years) years. The mean age was 60.4 (29-82) years. The tumors were localized in the stomach (62.9%), extraintestinal areas (14.8%), intestine (7.4%), esophagus (7.4%), and rectum (7.4%). Twenty-four patients were classified according to the Fletcher system. Of these patients, 7 (25.9%) were classified as very low risk, 8 (29.6%) as low risk, 7 (25.9%) as intermediate risk, and 2 (7.4%) as high risk. Twenty-four patients underwent surgery. Of the 3 patients who did not undergo surgery, 1 had metastatic disease at the time of diagnosis, and 2 had mini-or micro-GISTs in the stomach. On endoscopic surveillance, all tumors remained stable. Three out of the 27 patients were lost to follow-up. Two patients developed recurrence, and 1 patient died of GIST. Conclusion: We analyzed the clinical and pathological characteristics of GIST. The most common site of tumor origin was the stomach. The size, mitotic index, and Ki-67 values were to be found high in intermediate-and high-risk groups and metastatic diseases.
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