Characteristics and prognosis of rectal gastrointestinal stromal tumors: an analysis of registry data

Yasui, Masayoshi; Tsujinaka, Toshimasa; Mori, Masaki; Takahashi, Tsuyoshi; Nakashima, Yoko; Nishida, Toshirou

doi:10.1007/s00595-017-1524-8

Cited by 22 publications

(41 citation statements)

References 23 publications

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“…Rectal GISTs are also rare in eastern countries, accounting for approximately 0.1% of all rectal neoplasms in South Korea (10). Yasui et al reported that of 737 GIST patients evaluated between 2003 and 2007 in Japan, 24 (3.3%) were rectal GISTs (21). All were in the lower rectum, within a median of 2.5 cm from the anal verge (21).…”

Section: Epidemiologymentioning

confidence: 99%

“…Complete curative resection of rectal GISTs is difficult because of its anatomical features (35), and choice of the surgical procedure may be difficult in patients with large tumors close to the anal verge. Rectal GISTs have a high rate of local recurrence regardless of the surgical procedure (21). Surgical treatment is yet to be standardized (20), but local resection, low anterior resection, abdominoperineal resection (APR), and pelvic exenteration are performed.…”

Section: Surgerymentioning

confidence: 99%

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Management of rectal gastrointestinal stromal tumor

Kameyama¹,

Kanda²,

Tajima³

et al. 2018

Transl. Gastroenterol. Hepatol.

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Gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the gastrointestinal tract. However, rectal GIST is rare, the incident rate of it is approximately 5% of all GISTs. Rectal GIST symptoms generally include bleeding and/or pain and occasionally, urinary symptoms. Immunohistochemical evaluation finds that most rectal GIST tumors are CD117 (KIT) positive, and are sometimes CD34, platelet-derived growth factor receptor alpha (PDGFRA), smooth muscle actin, S-100, or vimentin positive. The National Institutes of Health (NIH) classifies rectal GIST as very-low risk, low risk, intermediate risk, or high risk, and the frequencies have been estimated as 0-23.8% for very-low risk, 0-45% for low risk, 0-34% for intermediate risk, and 21-100% for high risk tumors. The first-line treatment for localized GIST is curative resection, but is difficult in rectal GIST because of anatomical characteristics such as the deep, narrow pelvis and proximity to the sphincter muscle or other organs. Several studies noted the efficacy of the minimally invasive surgery, such as trans-anal, trans-sacral, trans-vaginal resection, or laparoscopic resection. The appropriate surgical procedure should be selected depending on the case. Imatinib mesylate (IM) is indicated as first-line treatment of metastatic or unresectable GIST, and clinical outcomes are correlated with mutation genotype. However, the mutation genotypes in rectal GIST are not well known. In this review, as in other GISTs, a large proportion (59-100%) of rectal GISTs carry exon 11 mutations. Although curative resection is indicated for localized rectal GIST, a high rate of local recurrence is a problem. Multimodal therapy including perioperative IM may improve postoperative outcomes, contributing to anus-preserving surgery. Moreover, mutation analysis before IM treatment is important. This review summarizes current treatment strategies for rectal GIST.

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Section: Epidemiologymentioning

confidence: 99%

Section: Surgerymentioning

confidence: 99%

Management of rectal gastrointestinal stromal tumor

Kameyama¹,

Kanda²,

Tajima³

et al. 2018

Transl. Gastroenterol. Hepatol.

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“…Approximately 75% of GISTs harbor oncogenic mutations in the receptor tyrosine kinases KIT and PDGFRA (platelet‐derived growth factor receptor alpha) . As compared to gastric GIST, rectal GIST are mostly caused by KIT mutations . Many of these KIT mutations are responsive to imatinib, a tyrosine kinase inhibitor (TKI).…”

Section: Introductionmentioning

confidence: 99%

“…Resection is generally the initial therapy for localized GIST without metastasis, but the rectum represents an especially difficult region for tumor removal . Yasui et al found that rectal GISTs frequently recur and most often in a locoregional distribution. Thus, perioperative imatinib has been shown to be effective in reducing locoregional and distant recurrences after tumor resection, even in patients with positive margins .…”

Section: Introductionmentioning

confidence: 99%

“…Yasui et al found that rectal GISTs frequently recur and most often in a locoregional distribution. Thus, perioperative imatinib has been shown to be effective in reducing locoregional and distant recurrences after tumor resection, even in patients with positive margins . More recently, imatinib has been studied as a neoadjuvant treatment before surgery to reduce tumor burden and aid in operative success .…”

Section: Introductionmentioning

confidence: 99%

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Frequent rectal gastrointestinal stromal tumor recurrences in the imatinib era: Retrospective analysis of an International Patient Registry

Stuart

Banerjee

Torre

et al. 2019

Journal of Surgical Oncology

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Introduction: Rectal gastrointestinal stromal tumor (GIST) is rare and comprises about 3% of GIST. Methods: Registry data was collected by the Life Raft Group June 1976 to November 2017. All patients had a histologic GIST diagnosis. Demographic, clinicopathologic, and clinical outcome data were patient reported. Recurrence-free survival (RFS) was analyzed using the Kaplan-Meier method and Cox regression analysis.Results: Of 1798 patients in the database, 48 had localized rectal GIST (2.7%).Patients were frequently male (58.3%) and non-Hispanic whites (58.3%). Median age at diagnosis was 52 years. Most patients (77%) were diagnosed in the imatinib era (2001 to current). Over half (54.2%) of the cohort had mutation testing and all profiled tumors possessed KIT mutations (exon 9: 7.7%, exon 11: 88.5%, and exon 13: 3.8%). Most evaluable patients (26/28; 92.9%) had high-risk disease (modified NIH criteria) and nearly all patients (95.8%) received imatinib. Median follow-up was 8.8 years (range, 0.3-30.7) and overall RFS was 8.0 years (95% CI, 2.9-13.1). Thirty-two percent (12/37) of patients in the post-imatinib era developed recurrent disease.Diagnosis in the imatinib era was associated with improved RFS (HR = 0.22, 95% CI, 0.08-0.62; P = .004) in the multivariable model. Conclusion:We find that disease recurrence remains prevalent in one-third of patients treated during the imatinib-era. K E Y W O R D S gastrointestinal stromal tumor, imatinib, rectal mass, submucosal tumor

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The effect of neoadjuvant imatinib therapy on outcome and survival in rectal gastrointestinal stromal tumors: A multiinstitutional study

Yang

Liu

Lin

et al. 2021

Journal of Surgical Oncology

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Characteristics and prognosis of rectal gastrointestinal stromal tumors: an analysis of registry data

Abstract: Rectal GISTs showed high rates of local recurrence regardless of the surgical procedure. Perioperative treatment with imatinib may improve outcomes.

Cited by 22 publications

References 23 publications

Management of rectal gastrointestinal stromal tumor

Management of rectal gastrointestinal stromal tumor

Frequent rectal gastrointestinal stromal tumor recurrences in the imatinib era: Retrospective analysis of an International Patient Registry

The effect of neoadjuvant imatinib therapy on outcome and survival in rectal gastrointestinal stromal tumors: A multiinstitutional study

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