Small Bowel Adenocarcinoma, Version 1.2020, NCCN Clinical Practice Guidelines in Oncology

Small bowel adenocarcinoma (SBA) is a rare malignancy of the gastrointestinal tract that has increased in incidence across recent years. Often diagnosed at an advanced stage, outcomes for SBA are worse on average than for other related malignancies, including colorectal cancer. Due to the rarity of this disease, few studies have been done to direct optimal treatment, although recent data have shown that SBA responds to treatment differently than colorectal cancer, necessitating a separate approach to treatment. The NCCN Guidelines for Small Bowel Adenocarcinoma were created to establish an evidence-based standard of care for patients with SBA. These guidelines provide recommendations on the workup of suspected SBA, primary treatment options, adjuvant treatment, surveillance, and systemic therapy for metastatic disease. Additionally, principles of imaging and endoscopy, pathologic review, surgery, radiation therapy, and survivorship are described.

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Ileal Adenocarcinoma with Liver Metastasis in Patient with Crohn’s Disease: A 9-Year Survival

Case Reports in Oncological Medicine ◽

10.1155/2019/8473829 ◽

2019 ◽

Vol 2019 ◽

pp. 1-5

Author(s):

Jiten P. Kothadia ◽

Deepa H. Nagaraju ◽

Seymour Katz ◽

Howard Bruckner ◽

Steven H. Itzkowitz ◽

...

Keyword(s):

Crohn’S Disease ◽

Liver Metastasis ◽

Small Bowel ◽

Crohn's Disease ◽

Poor Prognosis ◽

Treatment Options ◽

Small Bowel Adenocarcinoma ◽

Advanced Stage ◽

Advanced Malignancies ◽

Promising Treatment

Small bowel adenocarcinoma is a rare but well-known complication of Crohn’s disease. The diagnosis of small bowel adenocarcinoma remains difficult since its presentation is highly variable and mimics active or obstructive Crohn’s disease. The diagnosis is often delayed and typically detected only at surgery in an advanced stage with a poor prognosis. We report a case of metastatic ileal adenocarcinoma in a patient with Crohn’s disease with prolonged survival. Our case describes serial promising treatment options of these advanced malignancies and raises a possible role for checkpoint immunotherapy.

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

Frontiers in Oncology ◽

10.3389/fonc.2021.593561 ◽

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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Update on the role of pembrolizumab in patients with unresectable or metastatic colorectal cancer

Therapeutic Advances in Gastroenterology ◽

10.1177/17562848211024460 ◽

2021 ◽

Vol 14 ◽

pp. 175628482110244

Author(s):

Vanessa Wookey ◽

Axel Grothey

Keyword(s):

Colorectal Cancer ◽

Immune Checkpoint ◽

Checkpoint Inhibitors ◽

Treatment Options ◽

Standard Of Care ◽

Cancer Type ◽

Cancer Therapies ◽

Multiple Cancer ◽

Multiple Treatment

Colorectal cancer (CRC) is the third most common cancer type in both men and women in the USA. Most patients with CRC are diagnosed as local or regional disease. However, the survival rate for those diagnosed with metastatic disease remains disappointing, despite multiple treatment options. Cancer therapies for patients with unresectable or metastatic CRC are increasingly being driven by particular biomarkers. The development of various immune checkpoint inhibitors has revolutionized cancer therapy over the last decade by harnessing the immune system in the treatment of cancer, and the role of immunotherapy continues to expand and evolve. Pembrolizumab is an anti-programmed cell death protein 1 immune checkpoint inhibitor and has become an essential part of the standard of care in the treatment regimens for multiple cancer types. This paper reviews the increasing evidence supporting and defining the role of pembrolizumab in the treatment of patients with unresectable or metastatic CRC.

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Small-bowel adenocarcinoma diagnosed via capsule endoscopy in a patient found to have hereditary nonpolyposis colorectal cancer

Gastrointestinal Endoscopy ◽

10.1016/j.gie.2006.09.004 ◽

2007 ◽

Vol 65 (3) ◽

pp. 524-525 ◽

Cited By ~ 3

Author(s):

Riccardo Marmo ◽

Gianluca Rotondano ◽

Giovanni Riccio ◽

Rosario D'Angella ◽

Maria Rescinito ◽

...

Keyword(s):

Colorectal Cancer ◽

Small Bowel ◽

Capsule Endoscopy ◽

Hereditary Nonpolyposis Colorectal Cancer ◽

Small Bowel Adenocarcinoma ◽

Hereditary Nonpolyposis

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Trial in progress: A multicenter phase II study of pembrolizumab in patients with advanced small bowel adenocarcinomas.

Journal of Clinical Oncology ◽

10.1200/jco.2018.36.4_suppl.tps535 ◽

2018 ◽

Vol 36 (4_suppl) ◽

pp. TPS535-TPS535

Author(s):

Katrina Pedersen ◽

Michael J. Overman ◽

Nathan R. Foster ◽

Sunnie S. Kim ◽

Tanios S. Bekaii-Saab ◽

...

Keyword(s):

Clinical Trial ◽

Small Bowel ◽

Checkpoint Inhibitors ◽

Standard Of Care ◽

Bowel Function ◽

Primary Objective ◽

High Rate ◽

Small Bowel Adenocarcinoma ◽

First Line ◽

Colon Cancers

TPS535 Background: Small bowel adenocarcinoma (SBA) is a rare malignancy, possibly because of the high rate of immunosurveillance in baseline small bowel function, and is frequently diagnosed in late stages. Due to these factors, there is no consistent standard of care for metastatic disease beyond first-line where several prospective studies have confirmed activity of FOLFOX/CAPOX. Historically, second-line treatment of SBA has been extrapolated from colon/gastric cancer. Recent studies suggest that SBA has a distinct genomic profile, including higher incidence of microsatellite instability, compared to gastric and colon cancers. Additionally, the tumor microenvironment reflects a greater degree of PD-L1 expression and infiltration by lymphocytes. Immune checkpoint inhibitors have not previously been tested in SBA unselected for MSI status. Methods: The study is a phase 2, multicenter, single-arm clinical trial of pembrolizumab (200 mg IV every 3 weeks) for patients with unresectable or metastatic SBA refractory to first-line chemotherapy. Forty patients with any tumor mismatch repair status will be enrolled at 8 US sites as part of the ACCRU network and International Rare Cancer Initiative (IRCI) with a primary objective to assess the confirmed response rate (H0 p < 10%, HA: p > 30%) to pembrolizumab. Secondary endpoints include PFS, OS, and adverse events. Correlative aims include assessing blood and tissue biomarkers (i.e. PD-L1, MSI-H/MSS status, cfDNA, mutation burden, etc.) for association with clinical benefit. An interim analysis will be performed to assess efficacy after 18 patients become evaluable. The study is open with 16 patients enrolled at time of submission. Clinical trial information: NCT02949219.

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Elevating the standard of care: Impact of a regimen-level prior authorization tool on provider adherence to clinical guidelines.

Journal of Clinical Oncology ◽

10.1200/jco.2020.38.15_suppl.e19235 ◽

2020 ◽

Vol 38 (15_suppl) ◽

pp. e19235-e19235

Author(s):

Rogelio Alberto Brito ◽

Geri Kuklinski ◽

Patricia Angelica ◽

Anne Claussen ◽

Diana Fischer ◽

...

Keyword(s):

Treatment Guidelines ◽

Quality Care ◽

Standard Of Care ◽

Clinical Decision ◽

Cost Of Care ◽

Evidence Based ◽

Prior Authorization ◽

Total Cost ◽

Nccn Guidelines ◽

The Impact

e19235 Background: New developments in oncology therapy continue to grow in complexity, fueling a dramatically rising cost of care. Traditional care models present opportunities to streamline plan sponsor management efforts, expedite therapy, and improve health outcomes. Studies suggest adherence to evidence-based standards results in higher quality care. Current plan sponsor management platforms match medical policy to individual drugs, not to combination therapy regimens and lack real-time access to standard treatment guidelines. 70% of precertification requests are submitted via antiquated, cumbersome methods such as paper and fax. Methods: CVS Health/Aetna developed a comprehensive oncology solution featuring an enterprise web-based clinical decision support prior authorization tool (Novologix) at the regimen level to reduce administrative burden and support quality care. Novologix regimens were updated via collaboration with the National Comprehensive Cancer Network (NCCN) evidence-based guidelines. Groups also entered a value-based payment (VBP) model to help support quality of care by promoting adherence to NCCN guidelines when clinically appropriate and tool utilization. Eligible members were Commercial, fully-insured members newly diagnosed with breast, colorectal, or lung cancer. Providers were offered dedicated, individual training sessions to provide education on the Novologix tool. NCCN-aligned regimens requested through the platform were automatically certified. Any non-NCCN aligned regimens received accelerated medical review by a board-certified medical oncologist with the option for an external peer-to-peer review upon denial. Providers received ongoing quality and cost of care reporting. Results: Primary in progress. N of precertification requests submitted via Novologix ( 28 requests as of 1/23/2020) - (will include graph displaying N of requests by month). N of regimens submitted via Novologix that were automatically certified (46% as of 1/23). Avg turnaround times for modified regimen requests requiring clinical review (TBD). Avg % adherence to NCCN guidelines (100% as of 1/23/20) Secondary: Total cost of care (preliminary/other leading indicator). Conclusions: By engaging oncology practices through an enhanced payer-provider collaboration and implementing an automated regimen-level precertification process we can facilitate higher-quality oncology care. Future studies will be needed to measure the impact of this program on total cost of care.

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ERBB2 gene as a potential therapeutic target in small bowel adenocarcinoma.

Journal of Clinical Oncology ◽

10.1200/jco.2014.32.3_suppl.205 ◽

2014 ◽

Vol 32 (3_suppl) ◽

pp. 205-205

Author(s):

Anais Laforest ◽

Thomas Aparicio ◽

Aziz Zaanan ◽

Fabio pittella Silva ◽

Audrey Didelot ◽

...

Keyword(s):

Small Bowel ◽

Somatic Mutations ◽

Large Data ◽

Standard Of Care ◽

Tumor Location ◽

Small Bowel Adenocarcinoma ◽

Data Set ◽

Mutational Hotspots ◽

Low Incidence ◽

Poor Outcomes

205 Background: Small bowel adenocarcinoma (SBA) is an aggressive tumor responsible for poor outcomes with an expected median OS at 5 years inferior to 30%. Because of its low incidence, few prospective studies have been performed leading to insufficient knowledge and absence of standard of care. Aiming to better understand the small bowel carcinogenesis process we screened for somatic mutations a large data set of patients in more than 740 mutational hotspots among 46 genes. Methods: In total, 83 SBA cases were selected from 2 European databases. The sequencing was performed using the Ion 316 Chip. We additionally looked into MSI status and ERBB2 expression (immunochemistry and FISH). Results: Tumors were mostly located in the duodenum (47%) and stage ≥ 3 (63%). Among the 46 genes investigated, 8 were mutated in more than 5% of the cases: KRAS, TP53, APC, SMAD4, PIK3CA, ERBB2, BRAF, and FBXW7. Compelling mutations (7 cases) and amplifications (3 cases) 12% of the patients had an ERBB2 alteration. In this group, ERBB2 alterations were positively associated with dMMR status (p=0.006) and APC mutations (p=0.02) but negatively associated with p53 mutations (p=0.038). Interestingly, ERBB2 mutations were mainly detected within tumors derived from the foregut while ERBB2 amplifications were limited those derived from the midgut. Conclusions: This study describes the first large screening of somatic mutations in SBA using next generation sequencing. ERBB2 mutation was revealed to be one of the most frequent alterations in SBA with a distribution dependent on tumor location. In most cases patients harbored the same p.L755S-ERBB2 mutation. In clinical practice, this study may suggest that more than 10% of the patients with SBA could be treated using anti-ERBB2-targeted agents.[Table: see text]

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Racial disparities in treatment and outcomes of colorectal cancer in young adults.

Journal of Clinical Oncology ◽

10.1200/jco.2017.35.15_suppl.6559 ◽

2017 ◽

Vol 35 (15_suppl) ◽

pp. 6559-6559

Author(s):

Olatunji Boladale Alese ◽

Renjian Jiang ◽

Walid Labib Shaib ◽

Christina Sing-Ying Wu ◽

Madhusmita Behera ◽

...

Keyword(s):

Colorectal Cancer ◽

Colon Cancer ◽

Young Adults ◽

Proportional Hazards ◽

Standard Of Care ◽

Cox Proportional Hazards ◽

Care Utilization ◽

Nccn Guidelines ◽

Rectal Cancers ◽

The Impact

6559 Background: The incidence of colorectal cancer (CRC) in young adults is increasing. Minority populations with CRC are known to have worse outcome. The objective of this study is to evaluate the impact of race on the outcome of young adults with CRC. Methods: Data were obtained from all US hospitals that contributed to the National Cancer Database (NCDB) between 2004 and 2013. Univariate and multivariate testing was done to identify factors associated with patient outcome. Kaplan-Meier analysis and Cox proportional hazards models were used for association between patient characteristics and survival. Results: A total of 83,449 patients between 18 and 50 years of age were identified. The mean age was 43.6 years (SD±6), with a male preponderance (53.9%). About 72% were non-Hispanic Whites (NHW) while African Americans (AA) made up 15.1%. Distribution across stages I-IV was 15.6%, 22.4%, 33.9% and 27% consecutively, similar among the races. 41.8% of NHW and 28.4% of AA had rectal cancers (p<0.001). Despite equally receiving standard of care (SOC) as per NCCN guidelines, AA had significantly lower 5year survival rates (58.8%) compared to Hispanics (64.8%) and NHW (66.9%; HR 1.42; 1.38-1.46; p<0.001). Patients with colon cancer had worse outcome compared to rectal cancer (HR 1.21; 1.18-1.24; P<0.001). In terms of survival, NHW (HR 0.85; 0.81-0.88; p<0.001) and Hispanics (HR 0.75; 0.70-0.79; p<0.001) were more likely to benefit from chemotherapy compared to AA. As expected, SOC utilization was associated with improved survival across all racial groups, especially in AA with HR of 0.64 (0.60 – 0.69; p<0.001). Conclusions: Despite comparable rates of standard of care utilization, AA young adults with CRC had worse outcomes compared to other races. Colon cancer was significantly more common in AA than rectal cancers, which may have contributed to their worse outcomes. [Table: see text]

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