Coexistence Of Gastrointestinal Stromal Tumor Research Articles

The coexistence of gastrointestinal stromal tumors and malignancies: Our 10-year results

Objective: Gastrointestinal stromal tumor (GIST) is one of the most common mesenchymal tumors in the gastrointestinal system, occurring frequently after epithelial tumors. Although rare, secondary epithelial malignancies can be associated with GISTs. It was planned to conduct a retrospective cohort study evaluating the coexistence of GISTs with malignancies and their long-term outcomes through clinical and pathological findings. Material and Methods: Demographic and clinicopathological data of 69 patients who underwent surgery for GIST between January 2011 and November 2021 were retrieved from the patient database. Variables between the groups with only GIST and those with a secondary malignancy alongside GIST were analyzed using the Chi-square test and Mann-Whitney U test. Long-term survival analyses were conducted using the Kaplan-Meier test. A p-value of <0.05 was considered statistically significant. Results: Out of the 69 patients in our population, 40 (58%) were male, and the median age was 65 years (interquartile range= 56-75). GIST was the most commonly located in the stomach (59.4%), and nine (13%) patients had a secondary malignancy. Tumor size, smooth muscle antibody (SMA), and S100 antibody expression showed significant differences between the groups (p< 0.001, p= 0.015, p= 0.006). Shorter survival was observed in patients with GIST plus secondary malignancy (p= 0.005). Conclusion: The incidence of other intraabdominal malignancies occurring alongside GISTs is more common than have been previously thought. While the presence of a secondary malignancy does not impact the overall survival (OS) in GISTs, it was observed that survival is dependent on the primary malignancy. Patients diagnosed with GISTs require thorough investigation and close monitoring for secondary malignancies.

Gastrointestinal Stromal Tumor Coexistence with Multiple Myeloma: A Case Report

Objectives: Gastrointestinal stromal tumor (GIST) is a tumor that is rarely found and is characterized by positive KIT (CD117) and DOG1 results. Multiple myeloma (MM) is characterized by malignant proliferation of uncontrolled plasma cells in the bone marrow which produces abnormal proteins in plasma or urine. The coexistence of GIST and other solid cancers in the gastrointestinal tract and urinary tract have been widely reported, but GIST coexistence with hematologic malignancies is a scarce case.Methods: Case Report. Results: Immunochemistry plays an important role in determining the diagnosis and treatment of GIST. Concomitant therapy between GIST dan MM gives good results. Conclusion: The management of both diagnoses is carried out simultaneously while considering the possibility of various side effects that arise. For GIST, given target therapy with Imatinib mesylate which is the selected target therapy, while for MM given conventional therapeutic regimens according to the patient's age and ability. In the condition of coexistence of the two types of disease, the management of the disease that most influence the quality of life with a worse prognosis, is made as the main target of initial treatment.

Coexistence of gastrointestinal stromal tumor of the stomach and small bowel adenocarcinoma: A case report

The coexistence of gastrointestinal tumors (GIST’s) with other several primary malignant neoplasms has been demonstrated. However, there is no study that reported simultaneous occurrence of primary small bowel adenocarcinoma (SBA) and a GIST located in the stomach. Here, we report the first case of SBA and a synchronous gastric GIST. Surgical resection and clinicopathological characterization were performed for the masses identified through endoscopic and radiologic studies. Computed tomography scan showed a 13 mm mass located at the gastric antrum and another mass lesion partially occluding the jejunum. A biopsy specimen was obtained from the mass at the proximal jejunum by enteroscopy. The tumor was reported as adenocarcinoma. The patient was operated by the general surgery team. The features of the submucosal lesion resected from the antrum were consistent with a diagnosis of a GIST which contained spindle cells and showed positive staining with CD-117 and CD-34. Mitosis was not observed and Ki67 index was below 10%. This is the first case of coexistence of GIST of the stomach and SBA to be reported in English-language literature. The prominent characteristics of our case include its antral localization, male sex, absence of mitosis and incidental diagnosis.

Significance of Primary Malignant Tumors on the Outcome of Patients With Resected Gastrointestinal Stromal Tumors.

This study aimed to clarify the significance of primary malignant tumors for the outcome of resected gastrointestinal stromal tumors (GISTs). The medical history, pathological findings and prognosis of 66 patients with GISTs resected at our institute between January 2003 and December 2018 were investigated retrospectively and compared statistically. Among 66 patients with GISTs, 24 (36%) had concomitant malignant tumors. In an average study period of 57 months, one patient died from GIST, seven from other malignant tumors, and one from another disease. Only coexistence of GIST and other malignant tumors was recognized as a prognostic factor. Increasing age was significantly correlated with other malignant tumor in combination with GIST. When comparing patients with GIST alone and GIST with other malignant tumors, the latter showed significantly poorer prognosis. Coexistence of other malignant tumors was commonly observed in patients with GIST, and was associated with poorer prognosis. This association should be carefully considered and monitored in patients with GISTs.

2697 Gastrointestinal Stromal Tumor Mimicking a Small Lymphocytic Lymphoma

INTRODUCTION: Gastrointestinal stromal tumors (GIST) have been associated with synchronous or metachronous secondary malignancies in up to 43% cases. The occurrence between GIST and hematological malignancies is approximately 7%. Here, we present a case of a GIST mimicking Small Lymphocytic Lymphoma (SLL). CASE DESCRIPTION/METHODS: A 70-year-old male presented with a one-month history of bilateral axillary lymphadenopathy. Physical exam revealed a conglomerate of lymph nodes of both axillary regions. CT of the chest-abdomen-pelvis showed diffuse axillary, mesenteric, retroperitoneal and inguinal lymphadenopathy. In addition, it showed a 40 mm mass in the lesser curvature of the stomach (Figure 1). FNA of the axillary region showed findings consistent with SLL. Upper endoscopy showed a large subepithelial mass in the stomach (Figure 2). Biopsies were taken with cold forceps showing reactive white blood cells, this was suspected to be lymphoma. The patient was diagnosed with Stage IV SLL and started on Ibrutinib. Three months after therapy was started, CT revealed significantly improved on adenopathy but an increase in the size of the gastric mass. A repeat upper endoscopy with endoscopic ultrasound (EUS) showed a hypoechoic, heterogeneous round mass with well-defined borders, originating from muscularis propria layer (Figure 3). Fine needle biopsy (FNB) was performed and showed clusters of neoplastic cells, positive for CD117 and DOG1, negative for CK7, CK20, and CDX2, consistent with a GIST. DISCUSSION: Little is known about the coexistence of GIST and other metachronous tumors of different anatomic location. Several theories have been proposed to explain this link. These theories include pro-oncogenic changes in the tissue microenvironment, carcinogenic effect by chemotherapy and desmoid-type fibromatosis after trauma or surgery. When located in the stomach, GISTs most commonly present as subepithelial lesions. Since mucosal biopsies have a false negative rate of almost 50%, additional evaluation with EUS and EUS guided tissue acquisition is of essential importance. Obtaining core samples by FNB is useful for tissue architecture evaluation and immunohistochemistry assessment of the sample. In this particular case, EUS was not performed initially; instead, mucosal biopsies were obtained leading to a false negative result. This case highlights the rare association between GISTs and other malignancies, as well as, the importance of EUS for the evaluation and accurate diagnosis of different subepithelial lesions.

Italian survey of second tumors in patients with diagnosis of GIST (gastrointestinal stromal tumor).

11032 Background: GISTs are the most common mesenchymal tumors of the digestive tract. As of recent, new links are being made between GISTS and secondary malignancies. However, whether the coexistence of GISTs with other tumors is stochastic, or the result of related pathogenetic mechanisms is still unknown. Methods: We retrospectively reviewed clinical and molecular features from all GIST patients with second tumors treated in seven Italian GIST reference centers. Qualitative variables were compared using the Fisher exact test. Results: Clinical data of 184 patients with diagnosis of GIST were evaluated. Median age at diagnosis was 66 years, KIT exon 11 resulted the most frequent mutation (73%) while seven patients (3.8%) had a genetic syndrome. The most common primary GIST localizations were stomach (54%) and small intestine (33%). Second tumors arose mostly from gastrointestinal and genitourinary tract. Fourtythree patients had two primary tumors other than GIST and five patients had three other primary malignancies. According to Miettinen criteria, 45% of non-metastatic patients at diagnosis belong to low or very low-risk classes. We highlighted a significant correlation (P=0.002) between risk class and second/third tumor localization, with considerably high percentage of GI second malignancies in low/very low risk GISTs (table). Conclusions: The high frequency of second/third tumors reported in low and very low GIST calls for a careful follow-up also in these patients. Furthermore, this population requires further genetic investigation, NGS analysis is ongoing. [Table: see text]

Gastrointestinal stromal tumors during pregnancy: a systematic review of an uncommon but treatable malignancy.

Although modern social structure and medical advances have led to the increasing number of women childbearing in older age, cancer remains a rare diagnosis during pregnancy. There is little given information throughout the literature concerning gestation associated with the coexistence of gastrointestinal stromal tumor (GIST). In this review, we present 12 reported cases of GIST during pregnancy and we discuss the maternal and fetal outcome, as well as the therapeutic plan that was followed in each situation. From the collected data, 8 out of 12 cases had an uneventful outcome of their fetus. In 11 out of 12 cases surgical excision of the tumor was the treatment of choice, while seven women were treated with imatinib. Two of them were already on imatinib therapy during conception due to preexisting GIST diagnosis. Surgery remains the gold standard for the treatment of local or resectable GIST, while published data concerning the use of imatinib during pregnancy indicate that teratogenicity or fetal loss might be induced, especially if given during the first trimester of pregnancy. GIST during gestational period is a rare tumor in which a multidisciplinary approach should be designed, taking always into consideration that it has a favorable outcome on targeted treatment.

Adenosquamous carcinoma of the uncinate process of the pancreas with synchronous gastrointestinal stromal tumor of the stomach: Case report and review of the literature

Recently, the coexistence of gastrointestinal stromal tumors (GISTs) with other neoplasms has been studied with increasing frequency. Coexistence of pancreatic cancer with GISTs remains a rarity; however, here, we report a very rare case of adenosquamous carcinoma (ASC) of the uncinate process of the pancreas with synchronous GISTs of the stomach in a 62-year-old female. The patient presented with epigastric discomfort and vomiting. Radiographic imaging revealed two masses; one located at the body of the stomach and the other located at the uncinate process of the pancreas. Intraoperatively, a fine needle aspiration biopsy was conducted in the uncinate process of the pancreas, which revealed the malignancy of the masses. A pancreaticoduodenectomy and partial gastrectomy were then conducted, and subsequent pathological examinations identified an ASC of the pancreas and a GIST of the stomach. In our case, contrary to the majority of previous cases of synchronous GISTs and other malignancies, GIST was not an incidental finding. The initial suspicion on the GIST as the underlying cause of clinical symptoms led to the discovery of the ASC of the uncinate process of the pancreas.

Extragastrointestinal Stromal Tumor during Pregnacy

Extragastrointestinal stromal tumors (EGISTs) are mesenchymal neoplasms without connection to the gastrointestinal tract. Gastrointestinal stromal tumors (GISTs) and EGIST are similar according to their clinicopathologic and histomorphologic features. Both of them most often express immunoreactivity for CD-117, a c-kit proto-oncogene protein. The coexistence of GIST and pregnancy is very rare, with only two cases reported in the literature. In this paper, we presented the first EGIST case during pregnancy in the literature.

Coexistence of gastrointestinal stromal tumor (GIST) with desmoid tumor (DT): Case series of 22 patients.

10052 Background: Both gastrointestinal stromal tumor (GIST) and desmoid tumors (DT) are mesenchymal tumors. Anecdotal reports of individuals with both diseases led us to evaluate a larger cohort of patients with both tumors including clinical, molecular and histopathologic features. Methods: We identified 22 patients with GIST associated with DT between 1985 and 2008 at 9 institutions in U.S., Australia and Europe. Standardized Incidence Ratio (SIR) and its 95% confidence intervals were calculated comparing the incidence of DT in GIST patients for 2000 through 2008 in U.S. population. Results: We found 13 men and 9 women aged from 39-88 yrs. In 15 cases, the GIST was diagnosed prior to the DT, 6 cases had both tumors synchronous at presentation, and 1 patient had DT prior to GIST. The primary site of the GIST was gastric in 14 cases, small intestine in 8 cases, and 1 was mesenteric. Imatinib mesylate was administered to 11 patients, all with partial response. In 17/22 cases KIT expression was observed immunohistochemically for GIST but 0/22 for DT. Additionally, 15/22 cases of DT but 0/22 cases of GIST had to have B-Catenin expression by immunohistochemistry. When tissues were available KIT, PDGFRA and CTNNB1 were genotyped for both tumors. KIT mutations were observed in 7 of 10 GIST, all in exon 11. CTNNB1 mutations were observed in 4 of 9 DT, all in exon 3 (T41A and S45P). In GIST patients, the estimated risk of DT was significantly higher (SIR of 43; 95% CI, 19-74) than that expected in the general population. Conclusions: Preliminary evidence suggests that the relationship between the development of GIST and DT is not coincidental. The precise relationship between these tumors remains unknown. No significant financial relationships to disclose.

Abstract 4683: Coexistence incidence and clinicopathologic characteristics of gastrointestinal stromal tumors with gastrointestinal carcinoma

Abstract Objective Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumors of the digestive tract. The coexistence with other gastrointestinal carcinoma is unique. The aim of this study was to investigate the coexistence of GIST with other primary gastrointestinal carcinoma and its clinicalpathologic features. Methods 113 patients had been diagnosed as GIST in three local hospitals from 2002 to 2008. The surgical specimens were analyzed with an immunohistochemical panel that included staining for CD117, CD34, SMA and Ki-67. Results 16.8 % of (19 of 113) patients with GIST were co-diagnosed as gastrointestinal carcinoma. The average age at diagnosis was 57 years (range, 43 to 66), two years older than that of the patients only with GIST (range, 43 to 71). The patients with the coexistence of GIST and gastrointestinal carcinoma included 11 males and 8 females. The male-to -female ratio was 1.38:1, which is higher than that of the patients of GIST without the carcinoma (1.24:1). 26.3% GIST with the carcinoma was found in the esophagus (5 of 19) and 73.7% in the stomach (14 of 19) and non in the intestine. In contrast, only 5.3% GIST without the carcinoma was found in the esophagus (5 of 94) which was less incidence in the esophagus than that of coexistence of GIST and the carcinoma (P < 0.05), 84.0% in the stomach and 10.6% in the intestine, which higher incidence in the intestine than that of coexistence of GIST and the carcinamo (P < 0.05). The mean diameter of 19 GISTs with the carcinoma was 1.91 cm (range, 0.6 to 3.8 cm) smaller than that of 94 GISTs without the carcinoma (5.42 ± 6.17 cm, p < 0.05). 15.8% of the patients with coexistences of GIST and the carcinoma showed lower dysplasia, whereas 41.5 % of GIST patients without the carcinoma had the dysplasia and 33.0 % was malignant (p < 0.05). The average mitotic count in GIST with the carcinoma was 0.74/high-power field (HPF) (range, 0-4/HPF), and much less than that (3.78/HPF) of GIST without the carcinoma (range, 0 to 53/HPF, P < 0.05). The average proliferative index (positive number of Ki-67/HPF) of GIST with the carcinoma was 7.16/HPF (range 0 to 22) and less than that (19) of GIST without the carcinoma (range, 0 to 107). Conclusion 16.8% of GIST was co-diagnosed with gastrointestinal carcinoma. More coexistence of GIST and the carcinoma occurred in the esophagus. The GIST with the carcinoma was less proliferative and aggressive than the GIST without the Carcinoma. [Key words] gastrointestinal stromal tumor; pathology; carcinoma Citation Format: {Authors}. {Abstract title} [abstract]. In: Proceedings of the 101st Annual Meeting of the American Association for Cancer Research; 2010 Apr 17-21; Washington, DC. Philadelphia (PA): AACR; Cancer Res 2010;70(8 Suppl):Abstract nr 4683.

Synchronous colorectal adenocarcinoma and gastrointestinal stromal tumor in Meckel's diverticulum; an unusual association

BackgroundCoexistence of gastrointestinal stromal tumor with synchronous or metachronous colorectal cancer represents a phenomenon with increasing number of relative reports in the last 5 years. Synchronous occurence of GISTs with other gastrointestinal tumors of different histogenesis presents a special interest. We herein report a case of GIST in Meckel's diverticulum synchronous with colorectal adenocarcinoma.Case presentationA 69 year old man, presented with abdominal distension and anal bleeding on defecation. Colonoscopy revealed colorectal cancer and a low anterior resection was performed, during which a tumor in Meckel's diverticulum was discovered. Histologic examination revealed GIST in Meckel's diverticulum and a rectosigmoid adenocarcinoma.ConclusionWhenever GIST is encountered, the surgeon should be alert to recognize a possible coexistent tumor with different histological origin. Correct diagnosis of synchronous tumors of different origin is the cornerstone of treatment.

Association of Gastrointestinal Stromal Tumor and Acute Myeloid Leukemia Preceded by Myelodysplastic Syndrome with Refractory Anemia

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. GISTs are believed to be related to mutational activation of receptor tyrosine kinases, KIT, or platelet-derived growth factor receptor-alpha. The coexistence of GISTs with other neoplasms has been extensively addressed in the literature. The most common second neoplasms are colorectal cancer, prostate cancer, and neoplasms derived from lymphoid tissue. In this case report, we describe a patient affected by GIST and acute myeloid leukemia preceded by myelodysplastic syndrome with refractory anemia. The clinicopathological characteristics of the patient are discussed and the literature is reviewed.

When is a GIST not a GIST? A case report of synchronous metastatic gastrointestinal stromal tumor and fibromatosis

BackgroundA number of non-malignant diseases that share similar morphological features as gastrointestinal stromal tumor (GIST) have been reported. Co-existence of GIST with these other diseases is rarely recognized or reported.Case presentationWe report a case of a 62 year-old man with long-term stable control of metastatic GIST with systemic therapy, presented with an apparent intra-abdominal progression but not supported by imaging with positron emission tomography. Subsequent resection of the intra-abdominal tumor identified a non-malignant fibroid.ConclusionDifferentiating localized progression of GIST from other diseases has important prognostic and therapeutic implications. The potential for co-existence of non-malignant soft tissue neoplasm should always be considered.

Coexistence of gastrointestinal stromal tumors with other neoplasms

The purpose of this study was to assess the prevalence of other neoplasms in patients with gastrointestinal stromal tumors (GISTs) and to compare clinical and histopathological data in patients with a GIST and accompanying neoplasms and in patients with GIST only. The analysis encompassed 82 patients with a GIST from among 330 300 patients whose surgical specimens, biopsies, and autopsies were evaluated between January 1989 and June 2006. A subgroup of patients with other types of neoplasms was selected. Other neoplasms in patients with a GIST were diagnosed in 22 of the 82 (26.8%) patients. The most common accompanying neoplasms were colorectal (nine cases) and gastric (four cases) adenocarcinoma, as well as pancreatic adenocarcinoma (three cases). There was a tendency toward more common localization of a GIST in the small intestine in patients with other neoplasms than in patients with a GIST alone (P < 0.09). Tumors with very low risk of aggressive behavior were more frequent in patients with a GIST accompanied by other neoplasms than in the other group (P < 0.05). No phenotypic differences in GIST cells were found between the two groups. In almost 27% of the study population, GISTs coexisted with other neoplasms. A greater proportion of patients with a GIST localized in the small intestine and/or characterized by a very low risk of aggressive behavior and accompanying other neoplasms, compared with a GIST alone, most likely reflects the fact that in the first group, GISTs tended to be an incidental finding during surgery. The results were affected by patient selection and the type of tissue material available.

About a case of GIST occurring during pregnancy with immunohistochemical expression of epidermal growth factor receptor and progesterone receptor

The coexistence of gastrointestinal stromal tumors (GISTs) and pregnancy is very rare. We are the first to add to the literature a case report of GIST occurring during pregnancy with immunohistochemical staining for epidermal growth factor receptor (EGFR) and progesterone receptor (PgR). A role of PgR and EGFR in tumor growth should not be excluded, and these findings indicate that the expression of these receptors could provide pertinent biological information required to determine adequate therapeutic regimens. In conclusion, considering that GIST occurring during pregnancy is a rare event, with frequent delay in diagnosis, it is important to consider this diagnosis for early recognition, correct diagnosis, and a better outcome.