scholarly journals Systemic Therapy for Microsatellite Instability Small Bowel Adenocarcinoma With Mesenteric Vascular Embolism as Initial Symptom: A Case Report

2021 ◽  
Vol 8 ◽  
Author(s):  
Zhongyi Dong ◽  
Xiang Xia ◽  
Zizhen Zhang
Keyword(s):  
Small Bowel ◽  
Complete Remission ◽  
Microsatellite Instability ◽  
Stage Iv ◽  
Vascular Occlusion ◽  
Palliative Surgery ◽  
Screening Methods ◽  
Initial Symptom ◽  
Small Bowel Adenocarcinoma ◽  
Vascular Obstruction

Background: Small bowel adenocarcinoma are relatively rare tumors of the digestive system. Due to the lack of specific screening methods, patients are often diagnosed at an advanced stage. At present, there is no specific surgical guidance and chemotherapy regimen for small bowel adenocarcinoma. Here, we report a rare small bowel adenocarcinoma case with mesenteric vascular embolization and microsatellite instability, in which palliative surgery combined with chemotherapy and anti-Programmed cell death protein 1(PD-1) therapy resulted in complete remission.Case Presentation: The patient was a 55-year-old man who was admitted for suspected small bowel adenocarcinoma combined with incomplete ileus, mesenteric vascular occlusion and distant metastasis. We performed palliative surgery to remove adenocarcinoma as well as relieve obstruction. Then according to the pathological and immunohistochemical results (Stage IV and microsatellite instability), we used XELOX regimen combined with anti-PD-1 therapy. In last 2 years follow up, this patient achieved complete remission.Conclusions: The possibility of small intestinal tumor should be considered in patients with mesenteric vascular obstruction. PD-1 blockade is an effective therapy for small bowel adenocarcinoma with microsatellite instability.

scholarly journals Pembrolizumab for Microsatellite Instability-High or Mismatch Repair Deficient Small Bowel Adenocarcinoma or Appendiceal Adenocarcinoma

2021 ◽  
Vol 1 (10) ◽  
Author(s):  
Keeley Farrell ◽  
Jennifer Horton
Keyword(s):  
Small Bowel ◽  
Microsatellite Instability ◽  
Mismatch Repair ◽  
Progression Free Survival ◽  
Clinical Effectiveness ◽  
Complete Response ◽  
Small Bowel Adenocarcinoma ◽  
Appendiceal Adenocarcinoma ◽  
The Cost ◽  
Evidence Based Guidelines

Some adult patients with microsatellite instability-high (MSI-H)/mismatch repair deficient (dMMR) small bowel adenocarcinoma might benefit from pembrolizumab in controlling the disease (i.e., some patients achieved a partial or complete response after treatment). These findings are based on 2 single-arm studies (i.e., no comparator) with fewer than 20 patients in each study, which limits the certainty of the findings. The longer-term benefit of pembrolizumab is unclear, as some outcomes (e.g., progression-free survival, overall survival) were not reached at the time of data analysis. The safety of pembrolizumab in patients with MSI-H/dMMR small bowel adenocarcinoma is unknown (no evidence was found for this population). No evidence was identified regarding the clinical effectiveness of pembrolizumab monotherapy for patients with MSI-H/dMMR appendiceal adenocarcinoma. No evidence was identified regarding the cost-effectiveness of pembrolizumab monotherapy for patients with MSI-H/dMMR small bowel adenocarcinoma or appendiceal adenocarcinoma. No evidence-based guidelines were identified regarding pembrolizumab monotherapy for patients with MSI-H/dMMR appendiceal adenocarcinoma. One guideline was identified that recommends pembrolizumab as an option for initial or subsequent therapy in patients with advanced or metastatic MSI-H/dMMR small bowel adenocarcinoma.

scholarly journals Correction: PD-L1 in small bowel adenocarcinoma is associated with etiology and tumor-infiltrating lymphocytes, in addition to microsatellite instability

2020 ◽  
Vol 33 (7) ◽  
pp. 1453-1453
Author(s):  
Paolo Giuffrida ◽  
Giovanni Arpa ◽  
Federica Grillo ◽  
Catherine Klersy ◽  
Gianluca Sampietro ◽  
...  
Keyword(s):  
Small Bowel ◽  
Microsatellite Instability ◽  
Tumor Infiltrating Lymphocytes ◽  
Small Bowel Adenocarcinoma ◽  
Infiltrating Lymphocytes

Microsatellite instability and mutations in BRAF and KRAS in small bowel adenocarcinoma.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e15037-e15037
Author(s):  
Vasiliki Michalaki ◽  
Andreas Polydorou ◽  
Theodosios Theodosopoulos ◽  
George Frangulidis ◽  
Nikolaos Dafnios ◽  
...  
Keyword(s):  
Overall Survival ◽  
Small Bowel ◽  
Microsatellite Instability ◽  
Small Bowel Adenocarcinoma ◽  
Targeted Agents ◽  
First Line ◽  
Braf Mutations ◽  
Platinum Based Chemotherapy ◽  
Molecular Targeted Agents ◽  
Line Chemotherapy

e15037 Background: Small-bowel adenocarcinomas (SBAs) are rare cancers with a significantly lower incidence, later stage at diagnosis, and worse overall survival than other intestinal-derived cancers. Activating KRAS and/or BRAF mutations have been identified as predictors of resistance to anti-epidermal growth factor receptor (EGFR) The aim of this study was to perform a comprehensive immunohistochemical.analysis of KRAS, NRAS, V600E BRAF mutations and microsatellite instability using a cohort of surgically resected cases in our institution. Methods: A total of 17 patients (10 males and 7 females; mean age, 56.2 years old; range, 45-75 years old) received chemotherapy due to non-curative tumor resection, unresectable tumor or post-operative recurrence. Twelve patents received fluoropirimidine and oxaliplatin based first line chemotherapy. Molecular targeted agents were administered to 15 patients, for whom it was their first- or second-line therapy. Results: KRAS mutations were found in 7 cases (41%), out of which 5 (29%) were in exons 12/13. BRAFV600E mutation was observed in 1/17 pt. Microsatellite instability was identified in 3/17pt (MSI; 18%), mainly related to a loss of expression of MLH1 protein. Univariate analysis revealed a PS of 0 (P=0.0226) and treatment with platinum-based chemotherapy (P=0.0047) were significant factors for an improved prognosis. Among the 12 patients who received oxaliplatin-based chemotherapy as a first-line chemotherapy, a PS of 0 (P=0.0255) and treatment with anti-EGFR agents (P=0.0127) were significant positive prognostic factors. Toxicities due to the molecular targeted agents were not experienced. The median overall survival time was 14.3 months (range, 3-52 months), the median DFS was 14.2 months and the median OS was 32 months. Conclusions: To date, there is no standard chemotherapy regimen for advanced SBAs and little is known about their molecular characteristics. The results of the present study indicate that oxaliplatin based chemotherapy containing molecular targeted agents is a well-tolerated and effective treatment option for SBA. A better understanding of disease biology may help to identify therapeutic targets and advance precision medicine.

Small bowel adenocarcinoma with high levels of microsatellite instability in Crohn's disease

2006 ◽  
Vol 37 (5) ◽  
pp. 631-634 ◽  
Author(s):  
Renee Choy Foong Chan ◽  
Peter H. Katelaris ◽  
Peter Stewart ◽  
Betty P.C. Lin
Keyword(s):  
Crohn’S Disease ◽  
Small Bowel ◽  
Crohn's Disease ◽  
Microsatellite Instability ◽  
Small Bowel Adenocarcinoma

DNA mutation frequencies in metastatic small bowel adenocarcinoma (mSBA) in comparison to gastric (mGC), colon (mCC), and rectal cancer (mRC): Continuum or cutpoint?

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e14636-e14636
Author(s):  
Janet E. Murphy ◽  
Andrea Lyn Russo ◽  
Jackie Szymonifka ◽  
Eunice Lee Kwak ◽  
Jill N. Allen ◽  
...  
Keyword(s):  
Rectal Cancer ◽  
Small Bowel ◽  
Stage Iv ◽  
Unknown Primary ◽  
Small Bowel Adenocarcinoma ◽  
Exact Test ◽  
Dna Mutation ◽  
Genotype Frequencies ◽  
Mutational Profiling ◽  
Significant Difference

e14636 Background: The small intestine is in continuity with the stomach and colon/rectum. However mGC and mCC/mRC differ in oncogene profiles. No study has evaluated the oncogene profile of mSBA on the continuum of mGC and mCC/mRC. This may give insight into biology and have implications for treatment. Methods: Respective analysis was performed on GI cancer patients who underwent tumor mutational profiling since 2010. Genotyping was performed on formalin-fixed and paraffin-embedded tumor tissue using a multiplexed mutational profiling platform. Here we report results for APC, BRAF, EGFR, IDH1, KRAS, NRAS, PI3K, PTEN, and TP53. Results: We identified 12 patients with mSBA (11/12 with clinical data, 11/12 with genotype data). Median age at stage IV diagnosis was 61.1y (49.3-71.5y). Four patients had duodenal, 3 jejeunal, 2 ileal, and 2 multiple/unknown primary sites. All patients received 5FU and ox, while 8/11 received irino, 5/11 bev, 3/11 cetux, 1/11 regorafenib and 1/11 capecitabine. Two patients had adjuvant RT (both duodenal) while 4/11 received palliative RT. Survival was calculated from the date of stage IV diagnosis until death, with data censored on 2/1/2013. Median survival was 24.0 months (95% CI 6.4 – 60.0 mo). We compared genotype frequencies of mSBA patients (11) with mGC (41), mCC (161), and mRC (64) using Fisher’s exact test for pairwise comparisons. Conclusions: While the small bowel sample size was small, a significant difference in KRAS mutation frequency was found when compared to gastric cancer. No significant differences in mutation frequencies were found when compared with colon or rectal cancer. [Table: see text]

scholarly journals PD-L1 in small bowel adenocarcinoma is associated with etiology and tumor-infiltrating lymphocytes, in addition to microsatellite instability

2020 ◽  
Vol 33 (7) ◽  
pp. 1398-1409 ◽  
Author(s):  
Paolo Giuffrida ◽  
Giovanni Arpa ◽  
Federica Grillo ◽  
Catherine Klersy ◽  
Gianluca Sampietro ◽  
...  
Keyword(s):  
Small Bowel ◽  
Microsatellite Instability ◽  
Tumor Infiltrating Lymphocytes ◽  
Small Bowel Adenocarcinoma ◽  
Infiltrating Lymphocytes

scholarly journals Case Report: Molecular Features and Treatment Options for Small Bowel Adenocarcinoma

2021 ◽  
Vol 11 ◽  
Author(s):  
Miguel Cordova-Delgado ◽  
Gonzalo Pizarro ◽  
Mauricio P. Pinto ◽  
Maria Elisa Herrera ◽  
Marcelo Garrido
Keyword(s):  
Small Bowel ◽  
Treatment Options ◽  
Stage Iv ◽  
Tumor Type ◽  
Small Bowel Adenocarcinoma ◽  
Molecular Alterations ◽  
Molecular Features ◽  
Genetic Landscape ◽  
Rare Malignancy ◽  
Gene Alterations

Small bowel adenocarcinoma (SBA) is a rare malignancy characterized by poor prognosis. Recent efforts have sought to elucidate the genetic landscape and the molecular drivers behind this disease. Herein, we report the main molecular alterations in two metastatic (stage IV) SBA patients. Interestingly, one of them had gene alterations that affected signaling pathways previously described for SBA. However, a second patient displayed previously unreported alterations in this particular tumor type. Based on these findings we discuss potential treatment options for patients affected by this rare, aggressive disease.

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