Primary malignant gastrointestinal stromal tumor of the liver (original) (raw)
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An Unusual Gastrointestinal Stromal Tumor Presentain: Breast, Liver and Lymph Node Metastasis
European Journal of Breast Health
Stromal tumors are the prominent mesenchymal tumors of gastrointestinal tract (GIT) (1). They are more apparent in upper middle age, mostly in 5 th and 6 th decades. They occur equally in both sexes (1). Gastrointestinal Stromal Tumors (GISTs) are most commonly localized in the stomach (40-70%) and may be found in the intestine (20-32%), colon (10%) and esophagus (5%) (1, 2). They also can be seen in extra-GIT sites like mesentery, omentum and retroperitoneum (2). Major patient complaints regarding GISTs are upper abdominal pain, GIT bleeding and abdominal masses (1). Interstitial cells of Cajal (ICC), which GISTs are thought to originate from, both express tyrosine kinase receptors (c-kit) and stain with CD34 and Vimentin. KIT (CD117) is a transmembrane tyrosine kinase receptor, which is expressed in hematopoietic stem cells, germ cells, mast cells, melanocytes and ICC (2). C-Kit positivity is an important parameter in diagnosis of GIST. Recently, a specific marker, namely discovered on GIST1 (DOG-1), has been discovered for GIST diagnosis. It is shown to be positive on ICCs and gastric surface epithelium. DOG-1 is also expressed in high amounts in KIT mutation-negative cases besides typical GIST cases (3). Immunohistochemical diagnosis of GIST is adopted when mesenchymal tumors stain positive with c-Kit (CD117) and the DOG-1 where the rest of mesenchymal cell markers were negative. Demonstration of CD117 and recently DOG-1 positivity immunochemically stands for the standard technique of definitive diagnosis of GIST. CD34 is positive in 70-80% of the cases. Vimentin is diffuse and strong positive in many stromal tumors. SMA is positive in 30-40% and S100 in 5% of GISTs. Staining with desmin is rare (1). Malignant stromal tumors mostly metastasize to liver, peritoneum, lungs and bones (4). Brain and lymph node metastases are unusual (4). No breast metastasis was presented in literature before. Hereby, we present a case of GIST with liver, bone, lymph node and breast metastases and aimed to discuss its unusual metastatic reviewing literature. Case Presentation A 46 year-old woman was referred to us from another health facility with the complaint of back pain. The radiological imaging studies revealed multiple liver metastases with the largest being 5.5 cm in diameter as well as multiple bone involvements. Positron Emission
International journal of surgical pathology, 2015
Gastrointestinal stromal tumor (GIST) metastases are found most commonly in the liver, on average 16 to 38 months after resection of the primary tumor, even if some delayed hepatic metastases from GISTs have been described. We report a case of a man with a giant liver mass at computed tomography scan. In September 1984, the patient had undergone resection of a duodenal tumor, diagnosed as schwannoma. A liver biopsy revealed a neoplasm composed of epithelioid and spindled cells, immunohistochemically positive to c-kit and Dog-1. Reexamining the duodenal tumor resected in 1984, it was reclassified as GIST. Sequencing revealed the same mutation of the c-kit gene in both duodenal and hepatic lesions. To the best of our knowledge, this case represents the longest disease-free interval between a primary GIST and its metastasis. A brief review of the literature and an analysis of the potential prognostic role of particular c-kit mutations are also provided.
MALIGNANT GASTROINTESTINAL STROMAL TUMOUR WITH METASTASIS
ABSTRACT: - Gastrointestinal stromal tumors (GISTs) are the most usual mesenchymal neoplasms of the gastrointestinal tract. Ever since the classification of GIST as an entity distinct from leiomyoma’s, leiomyosarcomas, etc., there has been an increased concern in defining their imaging characteristics. It is estimated that approximately 5000-10,000 people are affected per year by this tumor all over the world. Most GISTs are benign (70-80%). However, these tumors have a spectrum ranging from benign to malignant lesions, depending on its anatomic site, tumor size, and mitotic frequency. We report a case of multiple malignant GIST with metastasis into Liver. KEYWORDS: – Hepatic metastasis, gastrointestinal stromal tumour, Liver
Diagnostic Cytopathology, 2002
Gastrointestinal stromal tumor (GIST) is the designation for a major subset of gastrointestinal mesenchymal tumors that histologically, immunocytochemically, and genetically differ from leiomyomas, leiomyosarcomas, and schwannomas. GISTs derive from the interstitial cells of Cajal and, in addition to variable expression of smooth muscle and neural markers, they characteristically express CD34 and CD117. The cytological appearance, including immunocytochemical and mutational analysis of c-kit gene in primary GIST has been well described. To our knowledge, only two cases of metastatic GIST diagnosed by fine-needle aspiration (FNA) have been reported. We illustrate three cases of metastatic GIST in the liver. Two cases had no prior history of gastrointestinal tumor and the third case had a 4-yr previous history of duodenal tumor. Consistent immunocytochemistry and ultrastructual studies supported the diagnosis of GIST. We emphasize that in the appropriate clinical and radiological setting, a confident diagnosis of GIST can be established by FNA of metastatic lesions.
Primary hepatic gastrointestinal stromal tumor
American Journal of Gastroenterology, 2003
Introduction: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors that mainly occur in the gastrointestinal tract. The GISTs that are sporadically reported in extra-gastrointestinal regions are named as extra-gastrointestinal stromal tumors (EGISTs). However, the primary EGISTs that originate from the liver are rare. Patient Concerns: A 64-year-old female presenting with right upper abdominal pain and thirsty for more than 20 days. Diagnosis: A diagnosis of a 15 Â 14 Â 7 cm liver mass located in the posterior right lobe of liver and spread to the right adrenal gland was confirmed. Pathological results showed that the tumor was mainly composed of epithelial cells and tested positive for CD117 and SDHB (succinate dehydrogenase complex iron sulfur subunit B). The gene mutational analyses for c-Kit and platelet-derived growth factor receptor alpha exons revealed negative results. Fluorescence in situ hybridization of murine double minute 2 produced negative fluorescence results which distinguished it from dedifferentiated liposarcomas. The postoperative gastroduodenal and colorectal endoscopy did not find any neoplastic lesions. To this end, the diagnosis of primary hepatic EGIST of wild type nature was confirmed. Interventions: The patient received right hepatectomy and adrenalectomy, no postoperative chemotherapy was administered. Outcomes: The patient died 11 months after surgery due to tumor metastasis. Conclusion: Primary hepatic EGIST is a rare and complicated disease of liver, a multidisciplinary team is necessary in diagnosis and treatment of primary hepatic EGIST. Abbreviations: EGISTs = extra-gastrointestinal stromal tumors, FISH = fluorescence in situ hybridization, HPF = high-power fields, ICCs = interstitial Cajal cells, MWA = microwave ablation, PDGFRA = platelet-derived growth factor receptor alpha, PET = positron emission tomography, RFA = radiofrequency ablation, sSNP = synonymous single nucleotide polymorphism.
Gastrointestinal stromal tumor of the stomach and hepatocellular carcinoma: An unusual association
International Journal of Surgery Case Reports, 2015
INTRODUCTION: Gastrointestinal stromal tumors (GIST) are uncommon, potentially malignant tumors, that arise in the wall of the gastrointestinal tract. Up to 50% can develop metastasis, mainly in the liver, but the occurrence of synchronous primary liver tumors is a rare event in these patients. PRESENTATION OF CASE: The authors report a case of the association of gastric GIST and hepatocellular carcinoma (HCC) in a non-cirrhotic liver in a 76 year-old patient. DISCUSSION: The appearance of an hepatic lesion in a GIST patient does not necessarily imply its secondary nature. CONCLUSION: In diagnosed GIST patients, all efforts should be pursued to characterize synchronous hepatic lesions, in order to plan a correct and tailored treatment of the patients.
Gastrointestinal stromal tumor (GIST) with hepatic metastases
2009
We discuss the case of a female patient, hospitalized in our clinic for pain in the right hypochondrium and in the lower part of the right hemithorax, following a thoracic trauma. Conventional and Sonovue contrast abdominal ultrasound revealed numerous hepatic tumours, while upper digestive endoscopy confirmed the existence of exulcerated gastric tumours. Endoscopy and CT scan helped in staging the tumour, while histopathological examination and immunophenotyping established the final diagnosis: GIST. After 3-months of daily treatment with Glivec 400 mg, we observed the regression of the tumoural diameter. This case is relevant for the diagnosis of a particular type of gastric tumour using modern imaging techniques and highlights the progress made in the GIST treatment.
Case series of gastrointestinal stromal tumors with lymph nodes metastasis: unusual presentations
International Surgery Journal, 2021
Gastrointestinal stromal tumor (GIST) is a rare cancer of the gastrointestinal tract, it occurred about 0.1-3.0% of all gastrointestinal neoplasms. Accounted about 10% of small-bowel tumors, and 10-15% of all sarcomas. Liver is a common hematogenous spread in GIST. However, metastasis to lymph nodes is consider extremely rare and routine lymph node dissection for GIST tumor was not recommended. Hence, we reported a case series of GIST that metastasize to lymph node along our experience in our center, hospital Taiping, Malaysia from 2010 until 2020. Hereby we report total of 3 out of 18 GIST cases that we encountered confirmed through histopathology the existence of lymph node metastasis.
Frequent occurrence of low grade cases among metastatic gastrointestinal stromal tumours
Journal of Clinical Pathology, 2003
Aim: Gastrointestinal stromal tumours (GISTs) are uncommon mesenchymal neoplasms. Some metastasise, whereas others remain asymptomatic for years, but it is difficult to distinguish between them histologically. This report analyses the characteristics of seven metastasising GISTs and compares clinicopathological parameters of the metastatic and non-metastatic groups. Methods/Results: Histology revealed typical GIST features with spindle, epithelioid, or mixed appearance. All seven cases were positive for vimentin, five for neurone specific enolase, six for c-kit, four for S-100, three for PGP-9.5, three for CD-34 and synaptophysin, but all were negative for cytokeratin, neurofilament, chromogranin A, and desmin. Four showed a focal reaction for smooth muscle actin. Three of the tumours were GI, and two each were GII and GIII. The Ki-67 index varied from 4% to 44%, the three GI cases had 4%, 10%, and 16%. Tumours from the metastatic GIST group were significantly larger than those from the non-metastatic group. Conclusions: Three cases exhibited bland, GI histological features with moderate or low proliferative activity. Among the c-kit positive metastasising stromal tumours, some were low grade, with moderate or low mitotic and Ki-67 indices, emphasising the necessity to develop a reliable grading system for GIST to predict clinical behaviour, the importance of careful analysis of "benign looking" tumours, and the key role of c-kit status in identifying patients who could benefit from treatment with STI-571. Larger tumours had a higher chance of metastasising, and only the size of the primary tumour played a role in predicting metastatic potential.
ABSTRACT ObjectiveThe aim of the study was to review the clinical records of 122 patients with gastrointestinal stromal tumors (GISTs) and analyze their clinicopathologic and immunohistochemical characteristics. MethodsThe medic records of 122 patients with GISTs during the periods from January 2002 to May 2010 were reviewed. All tumors were confirmed by histological and immunohistochemical analyses. ResultsThe tumors occurred in 59 males and 63 females, ranging from 25 to 77 years. Of all cases, 46 cases originated from stomach, 42 from small intestine, 17 from colon and rectum and 9 from retroperitoneal cavity and 4 cases from extra-gastrointestinal site. Liver was the most common organ that tumors metastases involved. Immunohistochemically, there were 114 tumors being positive for CD117 while 8 tumors negative for it. The frequencies of CD34 positive were higher in the stomach and rectum (89.1% and 86.7% respectively) than in the small intestine (64.3%, P < 0.05). Higher expression of SMA was in the tumors located in small intestine (54.8%) while the expressions of SMA in the gastric and rectal tumors were relatively low (21.7% and 20.0% respectively, P < 0.05). ConclusionGastrointestinal stromal tumors can occur in the gastrointestinal tract as well as in the extra-gastrointestinal sites. The frequencies of CD34 and SMA expression vary significantly with different locations. Key wordsgastrointestinal stromal tumors (GISTs)-c-kit protein (CD117)-surgery-imatinib