Natural history and prognostic factors for localised small bowel adenocarcinoma

Abstract

ObjectiveSmall bowel adenocarcinoma (SBA) is a rare malignancy with limited evidence regarding outcomes after curative resection of localised disease. We aimed to evaluate presentation and prognostic factors affecting overall survival (OS), relapse-free survival (RFS) and recurrence of SBA.MethodsConsecutive patients with completely resected localised SBA (1979–2019) were retrospectively reviewed for presentation, patient and tumour characteristics, perioperative treatment, recurrence, outcomes, and prognostic factors.ResultsAmong 257 total patients, median age was 58 years. Primary location was in the duodenum, jejunum and ileum in 52%, 29%, and 19% of patients, respectively. Median OS was 57.5 months and median follow-up was 40 months. In multivariate analysis, lymph node involvement, lymphovascular invasion, histologic grade and race were independent predictors of RFS, while race, stage and histologic grade were independent predictors of OS. No significant difference in OS or RFS was seen when evaluating the role of perioperative treatment. Median time to diagnosis from first medical evaluation was 31 days and did not change over time. Overall recurrence rate was 56%. Recurrence rate was higher in ileal (77%), than duodenal (54%) and jejunal (65%) SBA (p=0.01). Recurrence presented most commonly as distant metastasis (71%). Proficient mismatch repair was associated with decreased risk of locoregional recurrence (LR) but increased risk of distant recurrence (DR) when compared with deficient mismatch repair (dMMR) in univariate analysis.ConclusionsDespite advances in diagnostic modalities, this study did not show any improvement in earlier diagnosis of SBA over the course of the past three decades. The predominant pattern of disease recurrence was distant across all SBA locations, but dMMR status demonstrated a robust predilection for LR as opposed to DR. Perioperative treatment did not improve outcomes; however, a lower stage disease was seen in patients that received neoadjuvant therapy, suggesting further exploration of this approach.

Small bowel adenocarcinoma a rare cause of upper gastrointestinal obstruction (a case report and literature review)

“…Primary SBA is rare and challenges physicians in both diagnosis and treatment. [9] . Its global annual incidence accounts for only 1–3% of all gastrointestinal cancers [10] .…”

“…The clinical manifestation of SBA is not specific (i.e., abdominal pain, weight loss, nausea and vomiting, occult GI tract bleeding), coupled with a limited sensitivity of conventional radiological imaging [11] . Historically, a significant delay from onset of symptoms to diagnosis has been described for small bowel adenocarcinoma (SBA), related to that vague of symptoms and the challenges of evaluating the small intestine [9] .…”

Minimally invasive surgery with adenocarcinoma of jejunum diagnosed pathologically before surgery: A case report

“…The most common histological types of small intestine tumors are adenocarcinoma, carcinoid tumor, lymphoma, and stromal cells. About one-third of malignant tumors in the small intestine are reported as adenocarcinoma, with a low 5-year overall survival rate (25%) [ [3] , [4] , [5] , [6] , [7] , [1] , [2] , [3] , [4] , [5] , [6] , [7] , [8] ]. In the absence of specific symptoms, more than 30% of small bowel adenocarcinoma (SBA) patients at the time of diagnosis are stage IV, contributing to the poor prognosis [ 9 , 10 ].…”

“…Diagnosis of SBA is often delayed due to nonspecific patient symptoms and the resulting difficulty in performing appropriate tests. This delay contributes to a poor prognosis despite surgery, adjuvant chemotherapy, and radiation therapy [ 7 ]. While some cancers, such as colorectal cancer, stomach cancer, and breast cancer, have well-established screening tests and well-established guidelines for treatment, such guidelines are poor for small intestine cancer.…”

Poorly Cohesive Carcinoma of the Nonampullary Small Intestine

“…6 Adverse prognostic factors for surgically resected SBAs include TNM stage III/IV, duodenal site, grade 3 histologic grade (ie, poorly differentiated carcinomas), lymphovascular and perineural invasion, high tumor budding, whereas the association with celiac disease, Afro-Caribbean descent, dMMR/MSI-H, and tumor-infiltrating lymphocytes have been reported to be related with a more favorable outcome. 4,[7][8][9][10][11][12] Moreover, recent studies suggest that SBA histologic subtypes may have prognostic significance. 7,13 In particular, patients with SBAs characterized by noncohesive histology appear to have a worse prognosis in comparison with those exhibiting cohesive histotypes.…”