Clinico-pathologic pattern of gastrointestinal stromal tumors (GIST) in southern India: A single-institution experience

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 20531-20531
Author(s):  
M. Lamba ◽  
G. Mukherjee ◽  
K. Saini ◽  
C. Ramachandra ◽  
C. Rao ◽  
...  

20531 Background: There have been major advances in understanding the behavioral pattern, pharmacological intervention, and clinical response of GIST; yet Indian data in this regard is sparse. This study analyses the clinico-pathologic features in 36 patients (21 male, 15 female) of GIST seen at our institution. Methods: GIST was defined as a mesenchymal spindle or epithelioid cell lesion arising in the GI tract with CD117 immuno-reactivity. Retrospective data from January 03 to March 06 was analyzed for age, tumor site, morphology, immuno- reactivity, prognostic factors, response to treatment (by RECIST), and recurrence or metastasis. All patients had surgery; those with residual, recurrent, or metastatic disease got imatinib till tumor progression. Results: GIST presented at a mean age of 48.2 yrs (SD 6.4, range 34–65). The mean tumor size was 13.9 cm (range 2–42). The most common site was the small intestine (ileum 8, jejunum 7, duodenum 4). 24 patients (66.7%) had localized disease at baseline. Of these, 14 had local recurrence after surgery, and were given imatinib. 5 of them are in complete remission, 4 had partial response (PR), 3 patients died, and 2 had stable disease. Most patients with recurrent GIST had a mitotic rate of >10/50hpf. 8 patients developed metastasis, and received imatinib. Of these, 2 got a PR, 3 had progressive disease and died, and 3 had stable disease. 12 patients (33.3%) had metastasis at baseline (to liver and abdominal cavity), and underwent debulking. Of these, 6 patients with stable disease are on treatment with imatinib, 3 died and 3 were lost to follow-up. Conclusions: Average age of presentation was less than in Western reports. The commonest site was the small intestine as opposed to stomach in western literature. Mitotic rate was a better prognostic factor than gross tumor size. GIST with a mixed cell morphology showed aggressive behavior. Imatinib mesylate is useful in the post-operative management of GIST. [Table: see text] No significant financial relationships to disclose.

VASA ◽  
2011 ◽  
Vol 40 (6) ◽  
pp. 495-498 ◽  
Author(s):  
Rajkovic ◽  
Zelic ◽  
Papes ◽  
Cizmek ◽  
Arslani

We present a case of combined celiac axis and superior mesenteric artery embolism in a 70-year-old patient that was examined in emergency department for atrial fibrillation and diffuse abdominal pain. Standard abdominal x-ray showed air in the portal vein. CT scan with contrast showed air in the lumen of the stomach and small intestine, bowel distension with wall thickening, and a free gallstone in the abdominal cavity. Massive embolism of both celiac axis and superior mesenteric artery was seen after contrast administration. On laparotomy, complete necrosis of the liver, spleen, stomach and small intestine was found. Gallbladder was gangrenous and perforated, and the gallstone had migrated into the abdominal cavity. We found free air that crackled on palpation of the veins of the gastric surface. The patient’s condition was incurable and she died of multiple organ failure a few hours after surgery. Acute visceral thromboembolism should always be excluded first if a combination of atrial fibrillation and abdominal pain exists. Determining the serum levels of d-dimers and lactate, combined with CT scan with contrast administration can, in most cases, confirm the diagnosis and lead to faster surgical intervention. It is crucial to act early on clinical suspicion and not to wait for the development of hard evidence.


1999 ◽  
Vol 8 (4) ◽  
pp. 538-538 ◽  
Author(s):  
MARK G. KUCZEWSKI

The patient was born at 29 weeks gestation. There was a prenatal diagnosis that the child's small intestine had developed outside of the abdominal cavity. The length of gestation had made the initial prognosis good. But after birth, surgery to place the intestine back into the abdominal cavity found that the baby actually had very little small intestine and a diagnosis of “dead gut syndrome” was made. The amount of small intestine was not compatible with survival. The transplant service saw the baby twice and each time said the baby's profile did not meet the transplant protocol.


2021 ◽  
Author(s):  
Weihang Wu ◽  
Mingwei Wang ◽  
Weikang Zhou ◽  
Yuewen Zhu ◽  
Tianyu Lin ◽  
...  

Abstract Background: We aimed to verify the feasibility of a novel temporary intestinal storage device (TISD) using a simple intestinal gunshot wound model. Methods: Ten female beagle dogs were fasted for 12 hours and anesthetized. An incision protector was inserted into a 10-cm abdominal incision. The small intestine was exposed to the body by natural drooping. An automatic rifle was used to shoot the intestine from a distance of 25 meters to introduce a simple intestinal gunshot wound. The three phases of first aid for war injuries were followed: Care Under Fire, Tactical Field Care, and Tactical Evacuation Care. For Tactical Field Care, a novel TISD was used to reconstruct the ruptured intestine, and necrotic intestinal tissue was stored. The abdominal cavity was temporarily closed, and the abdomen was opened for exploration 4 hours after surgery. Treatment time was observed during Care Under Fire, transfer time was observed from Tactical Field Care to Tactical Evacuation Care, rescue was observed during Tactical Evacuation Care, and the treatment time of each intestinal segment was measured. After 4 hours, intestinal vitality was observed, and the heart, liver, spleen, lung, kidney, stomach, normal intestine, and necrotic intestine were examined before and 4 hours after surgery by light microscopy. The broken ends of the intestine were connected to the intestinal reconstruction device before and 4 hours after surgery and were examined by transmission electron microscopy. Results: The processing time of Care Under Fire was 41.55 ± 10.46 seconds, which is shorter than the maximum time limit of the battlefield first aid principle. Transit time from Care Under Fire to Tactical Field Care transit was 60.78 ± 15.95 seconds, which is shorter than the battlefield first aid principle. The treatment time of Tactical Field Care was 29.75 ± 5.13 minutes, and the reconstruction time of each intestinal segment was 4.44 ± 0.31 minutes. One dog died of anesthetic overdose, two died of splenic bleeding, and the rest completed all phases. The abdominal cavity was explored 4 hours after surgery, and the TISD was positioned. Intestinal tract reconstruction was normal, and no obvious necrosis was observed. Necrotic intestine had the same vitality as before storage. With light microscopy, the heart, liver, spleen, lung, kidney, and stomach showed no obvious necrosis, inflammatory cell infiltration, or necrosis of normal intestine before and after surgery. Before and 4 hours after surgery, intestinal necrosis involved local necrosis of villi and tissues, and marked inflammatory cell infiltration. Transmission electron microscopy showed that the villi of the intestinal stump connected to the TISD before surgery were intact, and no obvious necrosis was observed. The villi of the intestinal stump were moderately damaged after surgery, and focal necrosis was observed. Conclusions: The novel TISD can be used in the emergency treatment of simple small intestine gunshot wounds in beagle dogs and can prevent further deterioration after intestinal injury. Background: We aimed to verify the feasibility of a novel temporary intestinal storage device (TISD) using a simple intestinal gunshot wound model.


1993 ◽  
Vol 8 (2) ◽  
pp. 113-123 ◽  
Author(s):  
R. Molina ◽  
J. Jo ◽  
X. Filella ◽  
G. Zanon ◽  
J.J. Grau ◽  
...  

Mucin-like Carcinoma-associated Antigen (MCA) has been associated with many breast cancers. The aim of this study was to evaluate MCA in tumor tissue and serum as a potential tumor marker for prognosis and disease monitoring. MCA levels were determined in the tissue of 196 patients with primary breast cancer, 25 with metastatic disease and 25 patients with benign diseases, in pellet and/or cytosol. MCA levels were also determined in the serum of 50 patients with benign diseases, 148 with primary breast cancer (Mo), 150 with metastatic breast cancer (MT), and 200 with no clinical evidence of disease (NED). MCA tissue concentrations in pellet and cytosol were similar: 66.7 + 251 U/mg and 41.1 + 53 U/mg, respectively. No relationship between MCA levels and tumor size or nodal involvement was found. Higher MCA levels were observed in patients with ER + or PgR + tumors than in those with ER- or PgR- tumors (p < 0.01). Patients with MCA pellet concentrations lower than 10 U/mg of protein had shorter disease - free intervals (DFI) than those with higher values (p < 0.05). Abnormally high serum levels of MCA were found in 8% of patients with benign diseases, 4% of NED patients, 22% of Mo patients, and in 76% of MT cases. In primary breast cancer MCA values were related to tumor size and nodal involvement. A trend toward a lower DFI in patients with elevated presurgical MCA levels was observed but was of no statistical significance. These differences became statistically significant when patients were subdivided according to nodal status, with shorter DFI in those without nodal invasion (p < 0.05). In metastatic patients, changes in serum MCA were related to the tumor's response to treatment in 82% of cases. The highest MCA values were found in patients with liver or bone metastasis and the lowest values were found in those with locoregional recurrence. In conclusion, although MCA is not a specific tumor marker, it can be useful as a prognostic factor (tissue and serum) and in monitoring metastatic patients.


2003 ◽  
Vol 40 (1) ◽  
pp. 42-54 ◽  
Author(s):  
D. Frost ◽  
J. Lasota ◽  
M. Miettinen

Fifty canine gastrointestinal (GI) mesenchymal tumors were examined to determine the occurrence of leiomyomas (LM) and GI stromal tumors and to compare their clinicopathologic features. Twenty-one tumors (42%) were histologically reclassified as gastrointestinal stromal tumors (GISTs) and 29 tumors (58%) as LMs on the basis of their histologic similarity with homologous human tumors. The GISTs occurred equally in males and females, with a mean age of 11 years (range 5–14 years). Five GISTs (24%) were associated with clinical signs and six (29%) had metastasis in liver or abdominal cavity. The GISTs occurred in large intestine (10, 48%), small bowel (six, 29%), stomach (four, 19%), and mesentery of small intestine (one, 5%). Histologically, they were highly cellular spindle, or less commonly epithelioid tumors with mitotic rates ranging from 0 to 19 per 10 HPF. Eleven tumors (52%) were positive for CD117 (KIT); seven (33%) were positive for smooth muscle actin but none for desmin and S-100 protein. Sequences of KIT exon 11, often mutated in human GISTs, were evaluated from four GISTs. Deletion of Try556-Lys557 coexisting with duplication of Gln555 in one case of GIST and T to C transition resulting in substitution of Pro for Leu575 in another were identified. The LMs occurred predominantly in males (82%) with a mean age of 11 years (range 8–17 years). Nine tumors (31%) had associated clinical signs. They occurred in the stomach (22, 76%), esophagus (four, 14%), and intestines (three, 10%); all were paucicellular, had no mitoses, and were composed of mature smooth muscle cells. Twenty-eight (97%) were positive for smooth muscle actin and 18(62%) for desmin but none for CD117 and S-100. Both GISTs and true LMs occur in the GI tract of dogs. Both tumors have distinctive pathologic features.


2020 ◽  
Vol 19 ◽  
pp. 153303382097167
Author(s):  
Sahin Lacin ◽  
Suayib Yalcin

Background: Neovascularization plays a crucial pathogenic role in tumor development and vascular endothelial growth factor (VEGF-A) is a key signaling element that drives angiogenesis, thereby facilitating hepatocellular cancer (HCC) growth and metastasis. We aimed to define the relationship between serum VEGF-A levels and clinical outcomes in a cohort of Turkish patients with HCC. Methods: We enrolled and prospectively followed 84 patients with HCC in our study. Serum VEGF-A levels were measured and we assessed the association between VEGF-A levels and clinical features. Results: Forty-eight patients had cirrhosis while 35 patients were noncirrhotic. Serum VEGF-A levels were significantly lower in HCC patients with cirrhosis compared to non-cirrhotic HCC patients (p = 0.03).In terms of viral hepatitis subtype, 36 (%42.8) of patients were hepatitis B virus (HBV) positive and 8 (%9.5) of patients were hepatitis C virus (HCV) positive. Patients with serum VEGF-A levels ≥100 pg/mL had significantly lower OS rates than patients with serum VEGF-A level <100 pg/mL (p = 0.01). The OS rates were 5.8 and 14.2 months, respectively (p = 0.02). The median OS was 7.38 months (95% CI: 5.89-13.79 months). We observed a significant relationship between serum VEGF-A level and tumor size. Patients with tumor size ≤ 5cm had lower VEGF-A levels than patients with VEGF-A levels <5 cm. The VEGF-A levels were 132.7 and 342.1 pg/mL, respectively (p < 0.001). The median follow-up was 32 months. Conclusions: Serum VEGF-A level, a biological marker of angiogenesis, is an independent predictor of survival in patients with HCC. Serum VEGF-A levels may be utilized to predict response to treatment targeting serum VEGF-A in patients with HCC.


2015 ◽  
Vol 49 (3) ◽  
pp. 279-285 ◽  
Author(s):  
Viljem Kovac ◽  
Metoda Dodic-Fikfak ◽  
Niko Arneric ◽  
Vita Dolzan ◽  
Alenka Franko

AbstractBackground.Fibulin-3 is a new potential biomarker for malignant mesothelioma (MM). This study evaluated the potential applicability of fibulin-3 plasma levels as a biomarker of response to treatment and its prognostic value for progressive disease within 18 months. The potential applicability of fibulin-3 in comparison with or in addition to soluble mesothelin-related peptides (SMRP) was also assessed.Patients and methods.The study included 78 MM patients treated at the Institute of Oncology Ljubljana between 2007 and 2011. Fibulin-3 levels in plasma samples obtained before treatment and in various responses to treatment were measured with the enzyme-linked immunosorbent assay.Results.In patients evaluated before the treatment, fibulin-3 levels were not influenced by histopathological sub-types, tumour stages or the presence of metastatic disease. Significantly higher fibulin-3 levels were found in progressive disease as compared to the levels before treatment (Mann-Whitney [U] test = 472.50, p = 0.003), in complete response to treatment (U = 42.00, p = 0.010), and in stable disease (U = 542.00, p = 0.001). Patients with fibulin-3 levels exceeding 34.25 ng/ml before treatment had more than four times higher probability for developing progressive disease within 18 months (odds ratio [OR] = 4.35, 95% confidence interval [CI] 1.56–12.13). Additionally, patients with fibulin-3 levels above 34.25 ng/ml after treatment with complete response or stable disease had increased odds for progressive disease within 18 months (OR = 6.94, 95% CI 0.99–48.55 and OR = 4.39, 95% CI 1.63–11.81, respectively).Conclusions.Our findings suggest that in addition to SMRP fibulin-3 could also be helpful in detecting the progression of MM.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 5079-5079
Author(s):  
Rafael F. Duarte ◽  
Julio Delgado-Gonzalez ◽  
Bronwen E. Shaw ◽  
David Wrench ◽  
Mark Ethell ◽  
...  

Abstract Liver histology is regarded as inadequate for grading the severity of H-GVHD. Thus, liver biopsies (LB) are only performed to rule out other causes of liver injury when the results may lead to a radical therapeutic change. In this study we reviewed the pathologic features in LB from 33 consecutive evaluable patients with at least one LB, confirmed diagnosis of H-GVHD and no other concomitant causes of liver dysfunction. We asked whether the pathologic features in the first LB may predict their clinical outcome. Underlying diseases were AML (9), ALL (8), CML (7), MM (4), MDS (3), NHL (1) and AA (1). H-GVHD developed after BM (15) or PB (11) allo-HSCT, or DLI (7). Twenty-eight donors were related and 5 unrelated, 30 HLA-matched and 3 mismatched. All patients received myeloablative TBI-based (31) or chemotherapy only (2) conditioning regimens, and GVHD prophylaxis with cyclosporine-methotrexate (21) or T-cell depletion (12). The onset was acute in 22 (median day 35, range 5–86) and chronic in 11 (median day 139, range 103–545). The median overall survival (OS) from the onset of liver dysfunction was 6.2 months, with a non-relapse mortality (NRM) of 73.2% at 1 year (CI95=50.2–96.2). The causes of NRM were infection (8), liver failure (3), hemorrhage (3) and others (4). The first LB was performed at a median of 14 days (4–68) from the onset of abnormal liver function tests. The commonest pathologic finding in this series was bile duct damage, but ductopenia was uncommon (4). Neither the presence nor the degree of bile duct damage associated with the clinical outcome. Lobular inflammation (LI) was present in 25 cases (grade 1: 16; grade 2: 6; grade 3: 3). Higher degree of LI in the first LB associated with a reduced NRM (P=.001) and improved OS (P&lt;.001). Patients with LI grades 2/3 had higher median OS than those with grades 0/1 (not-reached vs 4.3 months; P=.012), and were more likely to respond to primary treatment (RPT) with steroids (P=.044), the most important clinical prognostic factor for OS in our series (HR=4.3; P=.001). Presentation of H-GVHD as hepatitis, with markedly increased aminotransferases levels, has been reported, in particular after DLI. In our group, there was no association between DLI-induced H-GVHD and LI, or between LI and aminotrasferases peak or biopsy-time levels. No study to date has shown that LI in the LB associated with clinical outcome in H-GVHD. In liver transplantation however, a histologically documented lobular hepatitic phase has been shown to anticipate ductopenia in chronic rejection, and associate with good response to treatment. In addition to LI, hepatocyte ballooning (HB) was associated with NRM (P=.003) and OS (P=.018) in our series. Patients with HB grades 0/1 had higher median OS than those with grades 2/3 (6.5 months vs 1.3 months; P=.004). HB did not associate with RPT. In multivariate analysis the effects of LI and HB on NRM (HR=5.1, P=.033; and HR=5.5, P=. 018, respectively) and OS (HR=4.0, P=.032; and HR=4.2, P=.037, respectively) remained significant. All other pathologic features examined had no association with patient clinical outcome. Our results suggest that LI and HB in the first diagnostic LB from patients with H-GVHD may predict the probability of RPT, NRM and OS. These results should be considered in the indications of LB and the selection of candidate patients with H-GVHD for new experimental therapies and in the design of future trials. Prospective validation of our findings is under way.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3892-3892
Author(s):  
Andres O. Soriano ◽  
Michael Thompson ◽  
Jorge Romaguera ◽  
Alma Rodriguez ◽  
Fredrick B. Hagemeister ◽  
...  

Abstract Objectives: To describe the clinical characteristics, pathologic features, immunophenotype, treatment and outcome of patients with FDCS seen at MD Anderson Cancer Center in the past 10 years. Background: FDCS is grouped in the WHO classification of tumors with the histiocytic and dendritic cell neoplasms, this group also includes histiocytic sarcoma, Langerhans cell tumors and interdigitating dendritic cell tumors. Data on this disease is based on case reports and case series. Methods: After IRB approval, cases were identified from the files of the lymphoma and pathology departments at MD Anderson Cancer Center from 1995 to 2005. Results: Fourteen patients were identified. Median age was 48 years old (25–69). Three patients presented with cervical lymphadenopathy, five had abdominal lymphadenopathy, three had mediastinal adenopathy, two had nasopharyngeal disease and one had pleural involvement. Extranodal disease included liver, spleen, pancreas and pleura. Constitutional symptoms were reported in 2 patients. Median performance status was 1 (0–2). Histologically, five cases showed low grade cytology with proliferation of spindled cells, growth patterns included whorled, storiform, fasicular and nodular. Three cases showed low grade features with focal high grade cytology and a diffuse growth pattern. Five cases showed high grade cytology that also included areas of necrosis in 2 cases and a high mitotic rate (>20/10HPF) in one case. CD21, CD23 and CD35 were positive in 83%, 90% and 44% of the cases respectively. Epidermal growth factor receptor (EGFR) was strongly positive in 12/13 cases tested (92%). The case that tested negative had high grade features and high mitotic rate. One patient had coexistent Castleman’s disease. Information on initial treatment was available in 11 patients which included surgery alone in 1 patient, surgery and radiation in 2 patients, surgery and chemotherapy in 1, chemotherapy alone in 3, chemotherapy and radiation in 1, surgery followed by radiation and chemotherapy in 3 patients. The initial chemotherapy regimen was CHOP in 8 patients. Complete remission (CR) was achieved in 7/11 patients (63%), 3 patients had disease progression and 1 had stable disease. Relapse occurred in all the patients who had a CR. Salvage treatment included surgery in 2 patients and chemotherapy in 7 patients (including the 2 patients who progressed on initial treatment). Two patients underwent allogenic hematopoietic stem cell transplantation after salvage therapy. CR was achieved in 5 patients, partial remission in 2, progression in 1 and stable disease in 1. Information on disease status at last follow up was available in 13 patients. Ten patients were alive at a median follow up of 22 months, 3/13 patients (23%) had no evidence of disease and 7/13 patients (53%) were alive with disease. Conclusions: The pathologic characteristics and immunophenotype found in our series were similar to those previously reported. EGFR was strongly positive in all but one of the cases tested. Consistent with previous reports Castleman’s disease was found in one of the cases. Although most of the patients initially responded to treatment, all of them eventually relapsed, which is in contrast to previously reported relapse rates of 16–36%. A better understanding of the biology of FDCS could guide our efforts in the development of new treatment modalities for this rare disease.


2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 21109-21109
Author(s):  
J. D. Hainsworth ◽  
D. Talantov ◽  
T. Jatkoe ◽  
C. Meng ◽  
J. Baden ◽  
...  

21109 Background: Standard treatment for most patients with CUP involves empiric chemotherapy. Since specific treatment now exists for most types of advanced carcinoma, precise identification of the primary site could lead to improved therapy. Veridex developed an optimized set of 10 gene markers, for a qRTPCR assay to identify tissue of origin of metastatic carcinoma in formalin-fixed, paraffin-embedded (FFPE) tissue samples (J Mol Diagn 8:320, 2006). The assay includes markers for 6 primary sites: lung, pancreas, colon, breast, ovary, and prostate. In this retrospective study, we evaluated the Veridex assay in patients with CUP. Methods: We obtained FFPE tissue from diagnostic biopsies on 69 CUP patients previously enrolled in empiric chemotherapy studies. The Veridex assay was performed as previously described. Assay results were correlated with clinical features, pathologic features, and response to treatment. Results: The Veridex assay yielded provisional diagnoses in 42 of 69 patients (61%): lung (15), pancreas (11), colon (12 ), ovary (4), breast (0), and prostate, (0 ). Most patients with diagnoses of lung and pancreas cancer had clinical and pathologic features compatible with these diagnoses; response rates to empiric chemotherapy (usually taxane/platinum-based) in patients with these diagnoses were 29% and 9%, respectively. The 12 patients with colon cancer diagnoses had predominantly intra-abdominal metastases (liver, peritoneum); response rate to therapy (usually taxane/platinum- based) was low (8%). The 4 patients with ovarian cancer had atypical clinical and pathologic features, and only 1 of 4 had PR to first-line taxane/platinum therapy. Conclusions: In this retrospective study, the Veridex 10-gene molecular assay was feasible and provided provisional diagnoses in a majority of patients with CUP. The diagnoses made using this assay (except ovarian cancer) were compatible with clinicopathologic features. The efficacy of cancer-specific treatment in patients diagnosed by this assay will be evaluated in prospective studies. No significant financial relationships to disclose.


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