Gastrointestinal Stromal Tumors: Molecular Mechanisms and Targeted Therapies

Downs‐Kelly, Erinn; Rubin, Brian P.

doi:10.4061/2011/708596

Cited by 15 publications

(17 citation statements)

References 93 publications

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“…Gastrointestinal stromal tumors (GISTs) were first described by Mazur et al in 1983 (1). GISTs are the most common mesenchymal neoplasms of the gastrointestinal tract and can have spindle-cell or epithelioid histology; 80% express the KIT protein and 10% express platelet-derived growth factor receptor alpha (PDGFRA) (2,3). Gain-of-function comprises approximately 5% of all GISTs (11).…”

Section: Introductionmentioning

confidence: 99%

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: Introductionmentioning

confidence: 99%

Management of rectal gastrointestinal stromal tumor

Kameyama¹,

Kanda²,

Tajima³

et al. 2018

Transl. Gastroenterol. Hepatol.

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“…They come about owing to a mutation in the KIT or PDGFRA genes—a very small percentage (9–15%) do not have a mutation in either gene, and they are known as ‘wild type’ 2. Both KIT and PDGFRA are on chromosome 4q12, and they both encode homologous transmembrane glycoproteins; these glycoproteins belong to a type 3 tyrosine kinase receptor family 3. These tumours usually occur in people over the age of 50, with a median of 55–65 years of age 1.…”

Section: Discussionmentioning

confidence: 99%

Gastrointestinal stromal tumour (GIST) in a young man with unprovoked haematemesis

Shahnazarian¹,

Fazio

2013

BMJ Case Reports

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This case involves a 27-year-old Hispanic man who presented to the emergency department with two episodes of haematemesis with no other associated symptoms. In addition, he has no medical history, denies alcohol abuse, denies non-steroidal anti-inflammatory drug use and has never experienced these episodes before. Esophagogastroduodenoscopy evaluation showed a large gastric mass, and histological results obtained from several biopsies of the mass supported a diagnosis of gastrointestinal stromal tumour. His abdominal CT scan also supported this finding. However, the unprovoked haematemesis and young age of the patient does not fit the typical presentation of a gastrointestinal stromal tumour.

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“…They are mostly of gastric origin (50-60%) and constitute 1% of all malignancies. GISTs that are equal to or smaller than 2 cm are usually asymptomatic and incidentally detected by endoscopic or radiological studies or during surgery performed for other indications [2]. Herein we report a case of GIST with mesenteric localization that was fistulized to the proximal jejunum and presented with massive rectal bleeding.…”

Section: Introductionmentioning

confidence: 94%

Gastrointestinal Stromal Tumor with Mesenteric Localization Fistulized to Proximal Jejunum Causing Massive Rectal Bleeding

2016

Ann Clin Anal Med

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ÖzetGastrointestinal stromal tümörler (GİST) gastrointestinal sistemin en yaygın mezenkimal tümörleridir. Epitelyal olmayan bu tümörler gastrointestinal traktus duvarının muskularis propria tabakasından çıkarlar. En sık lokalizasyonu mide ve ince barsaklardır. Nadiren gastrointestinal sistem ile bağlantısız olarak retroperitonda veya abdomende ortaya çıkabilirler. Sıklıkla gastrointestinal sistem hastalıkla-rının endoskopik ve radyolojik incelemelerinde veya hemoraji, obstrüksiyon ve organ perforasyonu gibi acil durumların cerrahi tedavisi sırasında, tesadüfen tanı konur. Bu yazıda proksimal jejunuma fistülize, nadir lokalizasyonuyla masif rektal kanamaya yol açan 59 yaşında bir GİST olgusu sunuldu. Histopatolojik inceleme ile kesin tanı alan olguya, total kitle eksizyonu ile birlikte yaklaşık 20 cm jejunum rezeksiyonu yapıldı. Anahtar KelimelerGastrointestinal Kanama; Jejunum; Stromal Tümör Abstract Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal system. These non-epithelial tumors originate from the muscularispropria layer of the wall of the gastrointestinal tract. Their most common locations of origin are the stomach and small intestine. Rarely, they may originate from the retroperitoneum or abdomen, and may have no connection with the gastrointestinal system. They are usually incidentally detected in endoscopic and radiological examinations of the gastrointestinal system or during surgical treatment of emergency conditions such as hemorrhage, obstruction, or organ perforation. In this paper, we report a 59-year-old man with GIST located in the proximal jejunum that caused massive bleeding owing to its rarely encountered location. Histopathological examination made the definitive diagnosis, and the patient underwent total excision of the mass and the resection of a 20-cm jejunal segment.

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Gastrointestinal Stromal Tumors: Molecular Mechanisms and Targeted Therapies

Cited by 15 publications

References 93 publications

Management of rectal gastrointestinal stromal tumor

Management of rectal gastrointestinal stromal tumor

Gastrointestinal stromal tumour (GIST) in a young man with unprovoked haematemesis

Gastrointestinal Stromal Tumor with Mesenteric Localization Fistulized to Proximal Jejunum Causing Massive Rectal Bleeding

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