Giant Gastrointestinal Stromal Tumors Research Articles

Gastric volvulus associated with shrinkage of a gastrointestinal stromal tumor by neoadjuvant imatinib: a case report

BackgroundDuring neoadjuvant chemotherapy for giant gastrointestinal stromal tumors, changes in gastrointestinal stromal tumor size are rarely associated with events such as perforation and bleeding that require emergency surgery. Moreover, it is very rare for gastrointestinal stromal tumors to shrink and become mobile, resulting in gastric volvulus. Herein, we report a case of gastrointestinal stromal tumor shrinkage during neoadjuvant imatinib treatment, resulting in gastric volvulus that required surgery. To the best of our knowledge, this is the first reported occurrence of gastric volvulus during neoadjuvant imatinib treatment for a giant gastrointestinal stromal tumor.Case presentationA 58-year-old Japanese woman who was diagnosed with a giant gastric gastrointestinal stromal tumor and administered neoadjuvant imatinib presented to our hospital with complaints of abdominal pain and retching. Enhanced computed tomography revealed that the gastrointestinal stromal tumor had shrunk and shifted in position, and the stomach had organoaxially twisted. Accordingly, the patient was diagnosed with gastric volvulus caused by a gastric gastrointestinal stromal tumor. Conservative treatment did not improve the volvulus; hence, laparotomy was performed. The tumor developed from the lesser curvature of the stomach and caused rotation of the gastric body. The local gastric wall was resected. Histopathological examination confirmed the diagnosis of gastrointestinal stromal tumor. The patient received adjuvant imatinib for 3 years and has been alive for 5 years without recurrence.ConclusionsGastric volvulus can be caused by the laxity of the ligaments that hold the stomach and gastric ptosis or esophageal hernia and diaphragmatic hernia; therefore, gastric gastrointestinal stromal tumors rarely cause gastric volvulus. However, a risk of torsion exists if the gastrointestinal stromal tumor develops extramural to lesser curvature and attains a certain size.

Gastric GIST with 13 kg in Asymptomatic Patient: A Rare Case of Giant GIST and Literature Review

Gastrointestinal stromal tumors (GIST) account for 85% of all mesenchymal neoplasms of the digestive tract. Of these, two thirds are found in the stomach. GISTs usually present as small lesions, often incidentally discovered. On the other hand, the presence of an abdominal mass, a very infrequent finding, can produce symptoms: Abdominal pain, digestive bleeding, nausea, vomiting, in addition to weight loss and intestinal constipation.

Liver transplantation combined with tyrosine kinase inhibitors for the treatment of hepatic metastatic giant gastrointestinal stromal tumors: A case report and literature review

BackgroundSurgical resection combined with oral tyrosine kinase inhibitors(TKI) is the most effective treatment for gastrointestinal stromal tumor(GIST) liver metastases. Liver transplantation (LT) is the last resort for the treatment of terminal liver malignancy. Whether it can be a potential treatment option for liver metastases from unresectable GIST is worth exploring.Case presentationWe report a 38-year-old woman who underwent jejunal stromal tumor resection and TKI(imatinib) therapy 15 years ago for jejunal stromal tumor liver metastases. During the period from 2015 to 2018, the liver metastases continued to grow after the patient stopped taking imatinib voluntarily, and the patient subsequently underwent multiple interventional surgeries and drug treatments, which were still poorly curative. The tumor was deemed unresectable because it filled the entire liver, and the patient subsequently underwent LT and was treated with imatinib post-operatively, which resulted in no recurrence of the tumor within 18 months of follow-up.Literature reviewThere are few reports in the literature on LT for the treatment of liver metastases from GIST. A systematic review and summary of the current literature by literature search revealed that LT as a last resort for metastatic GIST of the liver remains a major challenge.ConclusionsLT combined with TKI-targeted therapy is a potential therapy worth exploring for patients with unresectable metastatic GIST.

Intestinal Preserving Giant Mesenteric GIST Excision – A Rare Case

Gastrointestinal Stromal Tumors (GISTs) were originally believed to have originated from the mesenchymal cells of the gastrointestinal tract. These tumors actually originate from the intestinal cells of Cajal. A 26-year-old female came with complaints of mass per abdomen since 3 months which was insidious in onset, slowly progressive, complaining of vomiting,3-4 episodes since the last 4 days, non-blood tinged,non-projectile,non-bilious,non-foul smelling containing food particles associated with pain in the upper abdomen dull aching type,non-radiating, and non-migrating. On per abdominal examination — There was an oval-shaped mass that was firm in consistency, measuring about 15x10cm in size located in the left lumbar extending into the umbilical area which was mobile horizontally but vertical mobility was restricted. Routine investigations, USG, and CECT abdomen & pelvis were done which was pointing the diagnosis towards mesenteric GIST. Exploratory laparotomy and resection of GIST were scheduled. The resected tumor was sent for histopathological examination and a CD-117(IHC) marker was sent, which confirmed the mass as a mesenteric GIST. Literature shows that only a few cases of mesenteric GIST have been reported to date. Incidence of such giant mesenteric GISTs is very rare and intestinal preserving giant mesenteric gist excision is one of the firsts. Keywords: Mass per abdomen, Mesenteric GIST

Metastatic gastrointestinal stromal tumor of the greater omentum. Case report

Gastrointestinal stromal tumors (GIST) are the most common type of mesenchymal malignancies of the gastrointestinal (GI) tract. Almost 10% of them are originated outside of the GI tract (extra-GIST), while GIST of the greater omentum constitutes about 1% among stromal tumors. More than 80% of GIST have mutations in c-KIT and PDGFRA genes. Herein we demonstrate the case of successful treatment of patient with giant omental GIST with c-KIT exon 11 mutation. 64-years-old woman, was admitted to the Department of abdominal oncology with complaints of shortness of breath and abdominal enlargement in volume. CT-scan revealed a large tumor in the abdominal cavity with tumor size of 543422 cm. The patient underwent left thoraco-abdominal approach. It was found that the tumor was originated from the greater omentum with several metastases located on the peritoneum of the left lateral channel. Resection of the large omentum, splenectomy, liver resection and was done. Postoperative immunohistochemical study showed the expression of CD117, CD34 in tumor cells. Ki-67 index was 1215%. Genetic study revealed c-KIT exon 11 mutation. Treatment with imatinib 400 mg per day was started. Patient has been treated with imatinib for 12 years. On control examination we have found a metastasis in the anterior abdominal wall 3,532,5 cm in diameter. Afterwards we performed resection of anterior abdominal wall with metastasis on 9 November 2017. Immunohistochemical study confirmed metastasis of GIST. The index of tumor proliferation activity (Ki-67) was 45%. Patient prolonged imatinib treatment at the dose of 400 mg per day after operation. No signs of progression have been revealed on control examination 72 months after the operation. 12-year progression-free survival during imatinib treatment is unique in our practice. Moreover, in the case of further progression, we have second and third-line targeted therapy (sunitinib and regorafenib) and surgery treatment in local progression.

Surgical intervention of a giant gastric gastrointestinal stromal tumor following neoadjuvant therapy with imatinib

Gastrointestinal stromal tumors (GISTs) treatment has improved remarkably in recent years. However, giant and unresectable lesions could still be challenging, especially from the surgical aspect. We reported a case of a 44-year-old male patient who complained about abdominal pain and distention. Computed tomography scans, upper GI endoscopy, and tissue biopsy proved the diagnosis of a giant GIST which was considered unresectable. With the aid of neoadjuvant imatinib therapy, the tumor shrank tremendously, and we successfully performed en bloc resection with clean margins. Therefore, we suggested combining imatinib therapy and surgery in managing giant and unresectable GIST lesions.

Treatment and Prognoses in Patients With Primary Gastrointestinal Stromal Tumors ≥10 cm

Data on treatments and specific outcomes of primary gastrointestinal stromal tumors (GISTs) ≥10 cm are limited. We here report the treatments and survival outcomes concerning a subgroup of primary giant GISTs.Data of 83 consecutive patients with primary GISTs ≥10 cm in a single institution were retrospectively collected. Fifty-eight patients underwent surgery before imatinib mesylate (IM) treatment (Group A), 10 underwent surgical resection following IM therapy (Group B), whereas 15 patients took IM as drug therapy alone (Group C).The baseline clinical characteristics were similar among the 3 groups. However, a lower proportion in Group A had metastatic disease at the time of diagnosis or surgery compared with Groups B and C (8.6% vs 40.0% vs 40.0%, P < 0.05). The median follow-up duration was 21.5 months. No statistically significant differences were observed on progression-free survival (PFS) among the groups. However, patients in Group B showed significantly better overall survival (OS) compared with those in Group C (P = 0.044). Multivariate analysis showed that patients treated with adjuvant IM were associated with better PFS (hazard ratio [HR] 3.01; 95% confidence interval [CI] 1.13–7.97; P = 0.027) and OS (HR 29.11; 95% CI 3.32–125.36; P = 0.004). The subgroup with mitotic count >10/50 high-power fields (HPF) showed worse PFS (HR 3.50; 95% CI 1.19–10.25; P = 0.022) and OS (HR 20.04; 95% CI 1.67–143.79; P = 0.018) than that of mitotic count ≤5/50 HPF.Clinical treatment patterns for primary giant GISTs are different, and the outcomes of different interventions vary. The optimal treatments for these subgroup of patients still require further long-term investigation. Moreover, mitotic count and adjuvant IM are closely associated with PFS and OS in giant GISTs.

Bolnav cu Gist Gastric Gigant; Supraviețuire Nesperată Prezentare de Caz

Background: Gastrointestinal Stromal Tumors (GIST) are rare neoplasms, with sever prognosis in advanced cases. Until the discovery of Tyrosin Kinase Inhibitors (TKI), the life expectancy was very poor for patient with metastatic tumors, postoperative relapses or unresectable disease. Introduction of TKI therapy provided an unexpected survival rate, controlling the disease with very good tolerance. Method: We present a case report representative for GIST pathology. It is the first case of our GIST series of patients, operated in General and Esophageal Surgery Clinic of “St. Mary” Hospital Bucharest. A multidisciplinary approach and a complex, multimodal, surgical and oncological treatment was applied, with good response confirmed by the long-term follow-up. A 79 years old patient was diagnosed in 2004 (at the age of 70 years) with giant gastric GIST. He underwent a total gastrectomy, splenectomy and left pancreatectomy; the resection was considered as R1 because the tumor capsule was not complete. The adjuvant treatment with TKI was started postoperatively for 2 years. The recurrence was noted one year after the adjuvant therapy stop. Then the TKI treatment was restarted with very good tolerance and control of disease at 9 years. Conclusion: The TKI therapy allows long-term control of the malignant GISTs in term of overall survival and quality of life, even when the surgical procedure is not optimal.

Stage IV Gastrointestinal Stromal Tumor of Colonic Interposition in Esophagus

Purpose: Gastrointestinal stromal tumors (GISTs) is the most common mesenchymal tumor of the gastrointestinal tract. GISTs predominant in the stomach (60-70%) and intestine (20-25%), but rarely occur in the esophagus (5%) and colorectum. A 47-year-old man with a history of recurrent intermittent chest pain for >6 months who was found to have a widened mediastinum on chest film. He has a history of lye ingestion in 1981 with gastric pull up and colonic interposition. He reported a 40 lbs weight loss with decreased appetite for 8 months. Review of systems was otherwise negative. Patient had fever of 101°F. Blood pressure, pulses and respiratory rate were normal. Physical exam is non-contributory. Laboratory evaluation was normal except for urine toxicology which was positive for cocaine. Chest film showed prominence of the left hilum and multiple retrosternal lucencies are likely represent bowel containing anterior diaphragmatic hernia. CT scan of chest showed a giant mediastinal mass with vessels running through it in continuity with the stomach, heart, aorta and pulmonary artery. Multiple rounded enhancing hypodensities were found in the liver in CT. Upper endoscopy revealed a large, ulcerated and cratered mass measuring 7cm in diameter and involving 75% of luminal circumference of the colonic interposition was seen 29-36cm from the incisors. EUS of the liver lesions showed well circumscribed hypoechoic masses easily accessible for FNA. FNA biopsy revealed malignant cells positive for CD34, CD117 and DOG1, consistent with GIST. Most GISTs occur in the sub diaphragmatic gastrointestinal tract; only a few cases have been reported in the esophagus. Our patient has GIST in a segment of colon that was used as an esophageal graft. Patient has been treated with Gleevec (a targeted therapy that is directed to the exact molecular abnormality that drives the GIST tumor) and followed up with CT and PET scans, which are sensitive for evaluating the extent of tumor progression. This is the first case of a giant metastatic GIST that involved a colonic interposition.Figure: [577] CT scan of chest.

Surgical Management of Abdominal Manifestations of Type 1 Neurofibromatosis: Experience of a Single Center

Neurofibromatosis type 1 (NF1) is a genetic disease characterized by neoplastic and nonneoplastic disorders involving tissues of neuroectodermal and mesenchymal origin. The mainly involved districts are skin, the central nervous system, and eye and there is a wide range of severity of clinical presentations. Abdominal manifestations of NF1 include five kinds of tumors: neurogenic tumors (neurofibromas, malignant peripheral nerve sheath tumors [MPNSTs], and ganglioneuromas); neuroendocrine tumors (pheochromocytomas and carcinoids); nonneurogenic gastrointestinal stromal tumors (GISTs); embryonal tumors; and miscellaneous. The present experience depends on the participation in the National Project for Diagnosis and Treatment of Rare Diseases. In the group of patients with a diagnosis of von Recklinghausen disease, 10 patients underwent surgical treatment for gastrointestinal and retroperitoneal tumors associated with NF1. Three patients underwent adrenalectomy for pheochromocytoma (in one case associated with jejunal wall neurofibroma); two patients were found to be affected by MPNST (recurrent and unresectable in one case). One patient was affected by giant gastric GIST and jejunal neurofibroma; two patients were affected by extraperitoneal neurofibroma (pararenal and pararectal position); one patient was affected by giant colic neurofibroma and one patient was affected by retroperitoneal bilateral plexiform neurofibromas. Early diagnosis of these abdominal manifestations is very important because of the risk of malignancy, organic complications (such as pheochromocytoma), or hemorrhagic-obstructive complications such as in case of tumors of the gastrointestinal tract (GISTs and neurofibromas). The importance of an annual clinical evaluation on the part of a multidisciplinary pool of clinicians in highly specialized centers allows early detection of complications and of neoplastic transformation. Genetic screening allows preclinical diagnosis with a sensibility of 95 per cent. Further studies are necessary to detect predictive factors of malignant tumor development of severe clinical conditions.

Giant gastrointestinal stromal tumor of the esophagus presenting with dyspnea

Gastrointestinal stromal tumors (GISTs) are rare neoplasms thought to arise from mesenchymal cells of the gastrointestinal tract. These tumors demonstrate a pathobiology and clinical behavior different from those of smooth muscle and Schwann cell tumors. GISTs account for 0.1% to 3% of all tumors in the gastrointestinal tract. Two thirds of these tumors arise from the stomach, 25% arise from the small intestine, and 5% arise from the esophagus. We describe a case of giant GIST of the esophagus that compressed the right main bronchus and caused dyspnea. Clinical Summary A 33-year-old woman was admitted to our hospital with a 6-year history of dyspnea and 2-year history of dysphagia and back pain. She had been treated for asthma for 6 years. On admission, chest radiograph showed a widened mediastinum. Esophagogram showed a dilated thoracic esophagus and irregular mucosa in two thirds of the thoracic esophagus. Chest computed tomography revealed massive circumferential thickening of the esophageal wall extending from the level of the thoracic inlet to the cardia of the stomach. The giant mass compressed the right main bronchus (Figure 1). A mucosal ulceration 33 cm from the incisors and external compression were confirmed by esophagoscopy. Endoscopic ultrasonography was undertaken, which confirmed circumferentail thickening of the esophageal wall 6 to 8 cm along the thoracic esophagus. Proximal gastrectomy, subtotal esophagectomy, and esophagogastrostomy were performed through the abdominothoracic approach. The tumor weighed 2800 g and was 27 cm long (Figure 2). The postoperative course was uncomplicated, and the patient was discharged on the 12th postoperative day. The patient has been doing well with no recurrence 3 years after the operation. On histopathologic examination, we observed a tumoral mass in the esophageal wall between the submucosal and subserosal layer. We diagnosed this tumor as a GIST of the esophagus. The tumor was composed of spindle cells with the following features: mitotic count, 3 to 5/50 high-power field; CD117 staining, 10% to 50%/ ; CD34 staining, 10% to 50%/ . From the Ondokuz Mayis University, Medical School, Department of Thoracic Surgery, Samsun; Uludag University, Medical School, Department of Surgery, Bursa; and Ondokuz Mayis University, Medical School, Department of Pathology, Samsun, Turkey.

Giant malignant gastrointestinal stromal tumors: Recurrence and effects of treatment with STI-571

Malignant gastrointestinal stromal tumors (GISTs) are rare. Tumors larger than 10 cm tend to recur earlier: the larger the volume of the tumor, the worse the prognosis. We hypothesized that treatment with imatinib mesylate (Gleevec; STI-571), a c-kit tyrosine kinase inhibitor, as palliative therapy would prolong the survival of patients with recurrent giant malignant GISTs after resection. We performed a retrospective analysis of the effects of resection on patients with giant GISTs (>10 cm in diameter) to determine the overall survival and recurrence rates. Twenty-three patients diagnosed with giant GISTs were included from June 1996 to December 2003. STI-571 was not available until January 2000. After that time, 9 patients received this drug. The factors of age, sex, tumor location, histological surgical margin, and STI-571, tumor size changes and drug side effects were reviewed. We compared the survival rate to determine the prognostic factors and the effects of STI-571 on patients with recurrent malignant gastrointestinal stromal tumor. The positive surgical margin group had a significantly higher recurrence rate than the negative margin group (P = 0.012). A negative surgical margin and palliative treatment with STI-571 were significant prognostic variables (Log-rank test, P<0.05). Age, sex and tumor location were not significant prognostic variables. The 5-year survival rate of the surgical margin free patients was 80% and the 2-year survival rate of the surgical margin positive patients was 28%. The 5-year survival rate was 80% for the patients given STI-571 and 30% for the patients not given STI-571. The use of STI-571 gave a significant tumor shrinkage (6/9) rate in patients with giant GIST recurrence after resection. A negative surgical margin and the use of STI-571 after surgical resection were good prognostic indicators. Achieving a tumor-free surgical margin is still the best primary treatment for patients with such tumors. If STI-571 is used immediately when the surgical margin is positive and the tumor recurs after resection, then the prognosis of patients with giant GISTs can be improved.