Gastrointestinal Stromal Tumor (GIST)-Clinical Profile & Management of Patients Admitted To a Tertiary Care Hospital in Odisha

Gastrointestinal stromal tumor (GIST) is the commonest mesenchymal tumor of gastrointestinal tract. Gastrointestinal bleeding, obstruction, pain and abdominal lump are the common clinical manifestations. Local or segmental resection provides satisfactory results. Aim: Our aim was to report our experience of gastrointestinal stromal tumors (GISTs) during the last 2 years. Methods: Between January 2017 and June 2019, we performed surgery for 12 cases of GIST. Metastases, recurrence and survival data were collected in relation to age, history, clinical presentation, location, size, resection margins and cellular features. Results: Resection was completed in 11 cases. In one case definitive surgery was abandoned due to local invasion and metastasis. Three patients with high risk GIST were treated with imatinib mesylate. Conclusion: Non-radical surgery in the form of local or segmental resection is the standard surgical approach for GIST management. Pathological and biological features of the neoplasm represent the most important factors predicting the prognosis.

Gastrointestinal Stromal Tumor (GIST)-Clinical Profile & Management of Patients Admitted To a Tertiary Care Hospital in Odisha

Gastrointestinal stromal tumor (GIST) is the commonest mesenchymal tumor of gastrointestinal tract. Gastrointestinal bleeding, obstruction, pain and abdominal lump are the common clinical manifestations. Local or segmental resection provides satisfactory results. Aim: Our aim was to report our experience of gastrointestinal stromal tumors (GISTs) during the last 2 years. Methods: Between January 2017 and June 2019, we performed surgery for 12 cases of GIST. Metastases, recurrence and survival data were collected in relation to age, history, clinical presentation, location, size, resection margins and cellular features. Results: Resection was completed in 11 cases. In one case definitive surgery was abandoned due to local invasion and metastasis. Three patients with high risk GIST were treated with imatinib mesylate. Conclusion: Non-radical surgery in the form of local or segmental resection is the standard surgical approach for GIST management. Pathological and biological features of the neoplasm represent the most important factors predicting the prognosis.

Tumor estromal gastrointestinal como causa infrecuente de hemoperitoneo / Gastrointestinal Stromal Tumor as a Rare Cause for Hemoperitoneum

RESUMEN El tumor estromal gastrointestinal es el tumor mesenquimático más frecuente y se caracteriza por la expresión de un receptor de factor de crecimiento tirosina kinasa, CD117 c-KIT/CD 117. Se diferencia del resto de los tumores mesenquimáticos en que no expresa esta proteína. Alrededor del 70 - 80 por ciento de estos tumores son benignos, la mayoría se localizan en estómago e intestino delgado (> 90 por ciento). Los tumores estromal gastrointestinal malignos son generalmente de gran tamaño (> 5 cm), con índice mitótico alto y pueden dar metástasis a hígado y peritoneo. El tratamiento es la resección quirúrgica. Presentamos una paciente con 65 años de edad que acudió al cuerpo de guardia por sufrir caída de sus pies y quejarse de dolor abdominal. Como datos positivos al examen físico se constató palidez cutáneo mucosa y los complementarios de urgencia. El ultrasonido y la tomografía axial computarizada informaron líquido libre en cavidad con cifras de hemoglobina en 6,4 g/L. La punción abdominal constató sangre roja que no coagulaba. Se realizó laparotomía exploratoria encontrando hemoperitoneo y gran tumoración en cara anterior del antro gástrico, con otra pequeña en la porción alta del cuerpo; se realizó resección local con bordes libres de tumor y se suturaron los bordes gástricos. El resultado de la biopsia informó tumor del estroma gastrointestinal de células fusiformes con bajo índice mitótico, que midió 13 x 8 x 8 cm, con marcada angiogénesis y zonas de calcificación(AU)

ABSTRACT Gastrointestinal stromal tumor is the most frequent mesenchymal tumor and is characterized by expression of a tyrosine kinase growth factor receptor, CD117 c-KIT/CD 117. It is different to the rest of mesenchymal tumors in that it does not express this protein. About 70-80 percent of these tumors are benign. The majority are located in the stomach and small intestine (more than 90 percent). Malignant gastrointestinal stromal tumors are usually large (over 5 cm in size), with a high mitotic index, and can metastasize to the liver and peritoneum. The treatment is surgical resection. We present a 65-year-old patient who came to the emergency room due to having fallen on her own feet and complaining of abdominal pain. As positive data to the physical examination, mucous and skin whitening and the emergency complement tests were analyzed. Ultrasound and CT scan reported free fluid in cavity with hemoglobin values at 6.4 g/L. The abdominal puncture showed red blood that did not clot. The exploratory laparotomy showed a hemoperitoneum and a large tumor in the anterior face of the gastric antrum, with a small one in the upper portion of the body; local resection with tumor-free borders was performed and the gastric borders were sutured. The result of the biopsy reported a spindle cell gastrointestinal stromal tumor with a low mitotic index, measuring 13x8x8 cm, with marked angiogenesis and areas of calcification(AU)

Progress in molecular targeting therapy for gastrointestinal stromal tumors / 中国肿瘤生物治疗杂志

@#Gastrointestinal stromal tumors (GISTs) are the most common malignant tumor of abdominal soft tissue. It originates from Cahal (Cajal) interstitial cells or common precursor cells, and is driven by the mutated KIT gene or platelet-derived growth factor receptor alpha (PDGFRa) gene, all expressing type Ⅲ tyrosine kinase receptors. Imatinib mesylate, a tyrosine kinase receptor inhibitor, has been used for the standard treatment of advanced GIST, which has achieved remarkable results. Thus, GIST has become the most successful example of target therapy for solid tumors. In the context of the era of precision medicine, with the deepening in research of GISTs molecular biology，the molecular targeted treatment of GISTs has obtained a clear venation from the first-line, second-line and third-line of the advanced stage to the postoperative auxiliary and preoperative treatment, providing significant survival benefits for GISTs patients. This article systematically and comprehensively combed the preoperative and postoperative molecular targeting therapy from advanced GIST to early GIST, and analyzed the problems, proposed solutions and prospects for the future, aiming to provide reference for clinical application of molecular targeting drug therapy for GIST.

Strongyloidiasis in a Diabetic Patient Accompanied by Gastrointestinal Stromal Tumor: Cause of Eosinophilia Unresponsive to Steroid Therapy

We report here a case of strongyloidiasis in a 72-year-old diabetic patient (woman) accompanied by gastrointestinal stromal tumor receiving imatinib therapy, first diagnosed as hypereosinophilic syndrome and treated with steroids for uncontrolled eosinophilia. She suffered from lower back pain and intermittent abdominal discomfort with nausea and diagnosed with gastrointestinal stromal tumor. After post-operative imatinib treatment eosinophilia persisted, so that steroid therapy was started under an impression of hypereosinophilic syndrome. In spite of 6 months steroid therapy, eosinophilia persisted. Stool examination was performed to rule out intestinal helminth infections. Rhabditoid larvae of Strongyloides stercoralis were detected and the patient was diagnosed as strongyloidiasis. This diagnosis was confirmed again by PCR. The patient was treated with albendazole for 14 days and her abdominal pain and diarrhea improved. This case highlights the need for thorough investigation, including molecular approaches, to test for strongyloidiasis before and during steroid therapies.

Clinical significance of preoperative imatinib therapy in stomach gastrointestinal stromal tumors / 实用肿瘤学杂志

Objective Gastrointestinal stromal tumor( GIST) is a rare mesenchymal tumor from gastroin-testinal tract,and surgery remains the only curative treatment.In order to improve the outcome of surgical treat-ment,reduce the risk of surgery,and increase the quality of life,preoperative imatinib( IM) treatment for GIST is investigated.Methods We retrospectively studied the multidisciplinary team model treatments for 8 GIST cases receiving preoperative IM treatment.The cases were prescribed IM of 400 mg daily for 12 to 40 weeks and exten-sively followed until surgery was considered feasibleg.The clinical significance and safety profile were analyzed. Results Partial responsive rate was 62.5% in this study.There was no intolerable sever adverse effects by IM preoperative treatment.All the cases received R0 dissection,with no intraoperative tumor rupture.The postopera-tive recovery was satisfied.Conclusion IM preoperative treatment brings significant clinical benefit to large stomach GISTs and cardiac region GISTs.The preoperative treatment should be monitored carefully under a multi-disciplinary team.Preoperative IM treatment is an evocative treatment strategy for high risk GIST.

Salvamento de extremidad con resección extraarticular de rodilla / Limb-salvage surgery with extra-articular resection of the knee

Cuando tenemos un tumor óseo maligno del fémur distal con contaminación de la articulación, ya sea por extensión del tumor, fracturas secundarias al tumor con trazo intraarticular o contaminación en una biopsia tomada de manera inadecuada, debemos realizar resecciones extraarticulares de rodilla para lograr un control primario del tumor. Podemos emplear varias técnicas para lograr este fin, y una buena reconstrucción; la técnica que empleamos es la resección extraarticular más la preservación del mecanismo extensor, poco conocida en nuestro medio y de la cual es pionera el instituto Nacional de Cancerología. Con ella, hemos logrado un adecuado control tumoral primario de las lesiones óseas malignas en el fémur distal, menor número de complicaciones, una rápida rehabilitación y retorno a sus actividades habituales. Por esto, el interés de dar a conocer dicha técnica en los grupos de ortopedia oncológica, para que haga parte de su armamentario quirúrgico habitual.

When we encounter a malignant bone tumor in the distal femur with contamination of the joint, whether caused by tumor extension, secondary tumor fractures with intra-articular traces, or by contamination produced by inadequate taking of biopsies, extra-articular knee resection must be performed in order to achieve primary tumor control. Several methods are at our disposal to do so; our reconstruction technique of choice is that of extra-articular resection plus preservation of the extensor mechanism. this method is not well known locally; however, the National Cancer institute of Colombia has pioneered its use, thus allowing us to attain adequate primary tumor control in malignant bone lesions in the distal femur, with fewer complications, rapid rehabilitation and more immediate return to daily activities. We are, therefore, interested in sharing our expertise on this method with orthopedic oncologists so that they may include it their armory of standard surgical procedures.

A case of exophytic pedunculated gastrointestinal stromal tumor with cystic changes / 대한내과학회지

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract, and cystic changes are commonly observed. However, there have been few reports of cases of exophytic pedunculated GIST with cystic changes. Here, we report a 45-year-old man who presented with a palpable mass in the left upper quadrant of the abdomen. The mucosal folds were endoscopically abnormal, but there was no mucosal lesion. Computed tomography demonstrated a large, low-density cystic lesion surrounding an enhanced nodule in the greater curvature of the gastric body, and there was no tumor infiltration to other organs. The patient underwent hemigastrectomy and the lesion was shown to be an exophytic pedunculated cystic tumor. Histopathological examination showed epithelioid cells with marked hemorrhaging. Immunohistochemical examination indicated that most of the tumor cell cytoplasm was positive for the c-kit protein (CD117) and CD34. The tumor was diagnosed as an exophytic pedunculated GIST of the stomach with cystic changes.

A Case of Small Bowel GIST Initially Suspected as Peritoneal Seeding of Gastric Cancer

Gastrointestinal stromal tumors (GISTs) constitute the most common primary mesenchymal tumors of the digestive tract and characteristically express c-kit (CD117). GISTs are the most common non-epithelial tumor of the GI tract and frequently originate from the stomach and small bowel. Specifically, the synchronous occurrence of a GIST with other epithelial tumors is rarely reported. Recently, we discovered one case of a concurrent gastric cancer and a small bowel GIST that was initially suspected to be peritoneal seeding from gastric cancer. The patient was initially admitted with epigastric pain. Gastric cancer with peritoneal seeding was suspected after an evaluation. Following a laparoscopic examination, a distal gastrectomy with D2 lymph node dissection and small-intestine segmental resection was performed. The final pathologic diagnosis was early gastric cancer and high-risk small bowel GIST. The patient refused adjuvant therapy for the GIST, and currently shows no other marked indisposition. He has been disease-free for 14 months.

A Case of Gastrointestinal Stromal Tumor with Recurrent Hypoglycemia

Non-islet cell tumor induced hypoglycemia (NICTH) is attributable to overproduction of insulin-like growth factor-II (IGF-II) by solid tumors, and these tumors usually originate from mesenchymal or epithelial cells. Gastrointestinal stromal tumor (GIST) is a rare mesenchymal tumor and most commonly find in the gastrointestinal tract. It is usually expresses the CD117 (stem cell factor receptor, c-kit) detected by immunohistochemistry. Hypoglycemia associated with GIST is very rare and this has not yet been reported in Korea. A 72-year-old man was hospitalized due to frequent episodes of confusion. It was observed that non-hyperinsulinemic hypoglycemia, an elevated serum IGF-II level and a huge liver mass. The histology of liver mass showed c-kit (CD117) positivity, which was consistent with GIST, but it was surgically unresectable. He was treated with imatinib mesylate. Although he recieved palliative treatment, he still experienced intermittent fasting hypoglycemia. After 2 months, the serum IGF-II level was even higher than before. We changed imatinib mesylate to sunitinib malate and performed radiotherapy on the liver mass. Although the change of the liver mass was not significant, he did not suffer from hypoglycemia for three months afterwards.

A Case of Duodenal GIST Accompanied with Neurofibromatosis-1, Presenting with Gastrointestinal Bleeding / 대한소화기내시경학회지

Gastrointestinal stroma tumors (GISTs) are CD117- positive primary mesenchymal tumors of the gastrointestinal tract and are noted to have a possible non-random association with neurofibromatosis-1 (NF-1, Von Recklinghausen disease). We report a case of a duodenal GIST presenting with gastrointestinal bleeding in a 74-year-old female, and this condition was accompanied with NF-1. A upper gastrointestinal endoscopy and abdominal computed tomography scan revealed several submucosal tumors in the duodenum, jejunum and ileum. Histological and immunohistochemical studies on the surgical resection specimen revealed gastrointestinal stromal tumors of an uncommitted type. The patient was treated with local excision of the tumors and is now in a favorable state.

Gastrointestinal Stromal Tumor of the Rectum in a Pediatric

Gastrointestinal stromal tumors are the most common subset of mesenchymal tumors that arise from the gastrointestinal tract. They most commonly arise in the stomach, followed by the small bowel and, rarely, the rectum, esophagus and colon. Gastrointestinal stromal tumors and the specific c-kit protein (CD117)-positive mesenchymal tumors, have been sporadically reported in the rectum. We report here on a 12-year-old boy who underwent low anterior resection of huge gastrointestinal stromal tumor of the rectum. The diagnosis was confirmed by immunohistochemical staining of the tumor. The specimen was revealed to be a polypoid fungating tumor that, measured 6.0 x 6.0 cm in size, which consisted histologically of spindle-shaped cells. The tumor cells were positive for c-kit, vimentin, S-100, desmin, and SMA, although the results of the CD34 test were negative. In general, Gastrointestinal stromal tumors of the rectum predominantly occur between the sixth and seventh decades and they are rarely seen in individuals less than 40 years of age. To the best of our knowledge, our case is the youngest reported patient in the English literature who had as gastrointestinal stromal tumor of the rectum.

Two Cases of Jejunal Gastrointestinal Stromal Tumor Diagnosed by Capsule Endoscope / 영남의대학술지

Small bowel tumors have been difficult to diagnose because of low incidence and absence of specific symptoms. There are no efficient and accurate tests available for diagnosis. Capsule endoscopy is an efficient diagnostic tool for small bowel disease and obscure gastrointestinal bleeding. We diagnosed two cases of small bowel gastrointestinal stromal tumor (GIST) diagnosed by capsule endoscopy that were treated by surgery. A 68 year old male presented with abdominal pain. The capsule endoscopy showed fungating ulcer mass at the jejunum. A 55 year female presented with melena. The capsule endoscopy showed an intraluminal protruding mass with a superficial ulcer at the jejunum. Two cases were diagnosed with GIST after surgery. We report these two case diagnosed by capsule endoscopy and review the medical literature.

A Case of Rectal Gastrointestinal Stromal Tumor (GIST) Presenting with Hematochezia / 대한소화기내시경학회지

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal neoplasms affecting the gastrointestinal tract. The main sites for GIST are the stomach and small intestine but they can also occur in the rectum, albeit rarely. We report a 58-year-old woman who presented with hematochezia. Endoscopically, a submucosal tumor with mucosal ulceration was located approximately 2 cm from the anal verge. The pelvic CT and MRI scanning findings demonstrated a large low-density lesion with intraluminal growth from the lower rectum and an absence of tumor infiltrations to other organs. The patient underwent an abdominoperineal resection of the rectum. A histopathological examination showed fascicular proliferation of spindle shaped cells. The mitotic rate was more than 30 mitotic figures per 50 high-power fields. A immunohistochemical examination revealed most of the tumor cells to be positive for the c-kit protein, CD34 and SMA (alpha-smooth muscle action). The tumor was diagnosed as a GIST of the rectum.

A Case of Duodenal Gastrointestinal Stromal Tumor Presenting with Gastrointestinal Bleeding / 대한소화기내시경학회지

Gastrointestinal stromal tumor (GIST) is the most common non-epithelial tumor of the gastrointestinal tract. GISTs are most common in the stomach, followed by small intestine, colon and rectum, and esophagus. We report a case of duodenal GIST presenting with gastrointestinal bleeding in a 53-year-old male. Upper gastrointestinal endoscopy revealed a protruded mass with central ulceration on the second portion of the duodenum. Spontaneous spurting blood was encountered from the central ulcer of the mass. Abdominal computed tomography scan and celiac angiography revealed hypervascular tumor, located in the second portion of the duodenum. Laparotomy with wedge resection was performed. Histological and immunohistochemical studies on resected specimen revealed a duodenal GIST of a combined smooth muscle and neural type.

Multiple Small Intestinal Stromal Tumors Associated with Neurofibromatosis-1

Gastrointestinal stromal tumors (GISTs) are rarely noted in association with neurofibromatosis-1 (NF-1, von Recklinghausen disease) as an individual gastrointestinal manifestation. We report here a case of multiple GISTs with an abundant skeinoid fiber in the jejunum of a 43-year-old woman diagnosed as NF-1. Histologically, the tumors were composed of uniform spindle-shaped cells with a fascicular pattern, almost indistinguishable from the histology characteristic of usual GISTs. However, multiple synchronous tumor occurrence, abundant skeinoid fiber, and presence of microscopic miniatures of stromal tumors are additional characteristic features of this case.

Clinicopathologic and Immunohistochemical Features of Gastrointestinal Stromal Tumors (GISTs) in the Colon & Rectum

PURPOSE: A gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the gastrointestinal tract. There are only few reports in the literature describing colorectal GISTs. The aim of this study was to evaluate the clinicopathologic features of colorectal GISTs and to verify prognostic factors. METHODS: We reviewed 14 patients diagnosed as having primary colorectal GISTs between 1992 and 2003. Clinicopathologic variables and immunohistochemical expressions were analyzed. The diagnostic criteria and grading system for the GISTs were based on the proposal by the National Institutes of Health in April 2001. The median follow-up period was 27 (1~137) months. RESULTS: The male-to-female ratio was 9 : 5, and the mean age was 61 (37~76) years. The primary location was the rectum (11 cases, 78.6%). The mean tumor size was 7.7 (1.5~17) cm, and the mean number of mitoses was 33.4 (1~150) per 50 HPF. Of the 14 patients, 10 patients (71.4%) were regarded as a high-risk group and four patients as an intermediate-risk group. KIT protein and CD34 were expressed in 92.9% and 78.6% of the cases, respectively. The patients were subclassified based on immunohistochemical expressions as an uncommitted type in 11 cases (78.6%), a combined type in 2 cases (14.3%), and a myoid type in 1 (7.1%) case. Recurrence occured in three patients (21.4%) who were in the high-risk group. CONCLUSIONS: Colorectal GISTs occurred predominantly in the rectum and tended to be classified as high risk, which was the most important risk factor for recurrence. Accurate diagnosis and grading are important for adequate treatment and accurate prognosis.

A Case of Gastrointestinal Stromal Tumor of the Stomach with a Gastric Fistula / 대한소화기내시경학회지

Gastrointestinal stromal tumors (GIST) are mesenchymal gut tumors that lack markers of myogenic differentiation, but express CD34 and CD117, which are products of the c-kit oncogene. They present commonly gastrointestinal bleeding and abdominal pain and/or palpable mass. The other symptoms may include anorexia, weight loss, obstruction, perforation or fever. Fever due to abscess formation is rare complaint of GIST. We report a case of GIST of stomach with abscess and gastric fistula in a 54-year-old male who presented a three-week history of abdominal pain and fever. Fistula opening site was observed by endoscope, and abdominal CT showed multiple air pockets within the mass representing communication with bowel lumen by fistula.

Double Primary Gastrointestinal Stromal Tumor Associated with Upper Gastrointestinal Bleeding / 대한소화기내시경학회지

The terms "gastrointestinal stromal tumor" (GIST) have been applied to mesenchymal tumos that represent neither typical leiomyoma nor schwannoma. The majority of GISTs are Located in the stomach and small intestine, and only 4% of GISTs are found in duodenum. The most important characteristic is their indolent, slow-growing nature, rendering the most common definitions of malignancy invalid and inapplicable. Clinical and pathological criteria to differentiate benign from malignant GISTs are not well established. Tumor size and mitoic activity are commonly considered as important features, allowing for the prediction of biological behaviour and outcome. The case of a 87-year-old male patient who was presented with melena and acute anemia is herein reported. Esophagogastroduodenoscopy and computed tomography of the abdomen revealed two submucosal tumors in the stomach and duodenum. Histological and immunohistochemical studies on the surgical resection specimen revealed a gastrointestinal stromal tumor of an uncommiteed type, with no evidence of increased mitotic activity. The patient was treated with local excision of the tumors and is now in a favorable state.

A Case of Subserosal Stromal Tumor of the Stomach / 대한소화기내시경학회지

Gastrointestinal stromal tumor (GIST) were previously known as smooth muscle tumor like leiomyoma or leiomyosarcoma. Most of them were histologically spindle cell tumors, usually of mesenchymal origin. Stromal tumors account for about 1% to 2% of gastric malignant tumor, and the most important characteristics is their indolent, slow-growing nature, rendering most common definitions of malignancy invalid and inapplicable. The tumors are generally found deep within the stroma and submucosa, and only about half have an intragastric component, detectable by radiography, endoscopy or endoscopic ultra-sonography (EUS). Especially EUS is a valuable technique for diagnosing GIST. We experienced a 35-year-old female patient who complained right upper quadrant pain. She was diagnosed as subserosal stromal tumor by gastrofiberscopy, EUS, abdominal CT and operation. We report a case of gastric subserosal stromal tumor with a review of relevant literatures.