Recent Progress and Challenges in the Diagnosis and Treatment of Gastrointestinal Stromal Tumors
Gastrointestinal stromal tumors (GISTs) are the most frequent malignant mesenchymal tumors in the gastrointestinal tract. The clinical incidence of GISTs is estimated 10/million/year; however, the true incidence is complicated by frequent findings of tiny GISTs, of which the natural history is unkno...
Main Authors: | , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
MDPI AG
2021-06-01
|
Series: | Cancers |
Subjects: | |
Online Access: | https://www.mdpi.com/2072-6694/13/13/3158 |
id |
doaj-93d9bac8a182441bb08c034b777d9cc1 |
---|---|
record_format |
Article |
spelling |
doaj-93d9bac8a182441bb08c034b777d9cc12021-07-15T15:31:24ZengMDPI AGCancers2072-66942021-06-01133158315810.3390/cancers13133158Recent Progress and Challenges in the Diagnosis and Treatment of Gastrointestinal Stromal TumorsToshirou Nishida0Shigetaka Yoshinaga1Tsuyoshi Takahashi2Yoichi Naito3Department of Surgery Japan, Community Health-Care Organization Osaka Hospital, Osaka 553-0003, JapanEndoscopy Division, National Cancer Center Hospital, Tokyo 104-0045, JapanDepartment of Gastroenterological Surgery, Osaka University Graduate School of Medicine, Osaka 565-0871, JapanDepartment of General Internal Medicine/Experimental Therapeutics/Medical Oncology, National Cancer Center Hospital East, Chiba 277-8577, JapanGastrointestinal stromal tumors (GISTs) are the most frequent malignant mesenchymal tumors in the gastrointestinal tract. The clinical incidence of GISTs is estimated 10/million/year; however, the true incidence is complicated by frequent findings of tiny GISTs, of which the natural history is unknown. The initial work-up with endoscopy and endoscopic ultrasonography plays important roles in the differential diagnosis of GISTs. Surgery is the only modality for the permanent cure of localized GISTs. In terms of safety and prognostic outcomes, laparoscopy is similar to laparotomy for GIST treatment, including tumors larger than 5 cm. GIST progression is driven by mutations in <i>KIT</i> or <i>PDGFRA</i> or by other rare gene alterations, all of which are mutually exclusive. Tyrosine kinase inhibitors (TKIs) are the standard therapy for metastatic/recurrent GISTs. Molecular alterations are the most reliable biomarkers for TKIs and for other drugs, such as NTRK inhibitors. The pathological and genetic diagnosis prior to treatment has been challenging; however, a newly developed endoscopic device may be useful for diagnosis. In the era of precision medicine, cancer genome profiling by targeted gene panel analysis may enable potential targeted therapy even for GISTs without <i>KIT</i> or <i>PDGFRA</i> mutations.https://www.mdpi.com/2072-6694/13/13/3158gastrointestinal stromal tumorsubmucosal tumorsubepithelial tumorgene panel analysisprecision medicine |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Toshirou Nishida Shigetaka Yoshinaga Tsuyoshi Takahashi Yoichi Naito |
spellingShingle |
Toshirou Nishida Shigetaka Yoshinaga Tsuyoshi Takahashi Yoichi Naito Recent Progress and Challenges in the Diagnosis and Treatment of Gastrointestinal Stromal Tumors Cancers gastrointestinal stromal tumor submucosal tumor subepithelial tumor gene panel analysis precision medicine |
author_facet |
Toshirou Nishida Shigetaka Yoshinaga Tsuyoshi Takahashi Yoichi Naito |
author_sort |
Toshirou Nishida |
title |
Recent Progress and Challenges in the Diagnosis and Treatment of Gastrointestinal Stromal Tumors |
title_short |
Recent Progress and Challenges in the Diagnosis and Treatment of Gastrointestinal Stromal Tumors |
title_full |
Recent Progress and Challenges in the Diagnosis and Treatment of Gastrointestinal Stromal Tumors |
title_fullStr |
Recent Progress and Challenges in the Diagnosis and Treatment of Gastrointestinal Stromal Tumors |
title_full_unstemmed |
Recent Progress and Challenges in the Diagnosis and Treatment of Gastrointestinal Stromal Tumors |
title_sort |
recent progress and challenges in the diagnosis and treatment of gastrointestinal stromal tumors |
publisher |
MDPI AG |
series |
Cancers |
issn |
2072-6694 |
publishDate |
2021-06-01 |
description |
Gastrointestinal stromal tumors (GISTs) are the most frequent malignant mesenchymal tumors in the gastrointestinal tract. The clinical incidence of GISTs is estimated 10/million/year; however, the true incidence is complicated by frequent findings of tiny GISTs, of which the natural history is unknown. The initial work-up with endoscopy and endoscopic ultrasonography plays important roles in the differential diagnosis of GISTs. Surgery is the only modality for the permanent cure of localized GISTs. In terms of safety and prognostic outcomes, laparoscopy is similar to laparotomy for GIST treatment, including tumors larger than 5 cm. GIST progression is driven by mutations in <i>KIT</i> or <i>PDGFRA</i> or by other rare gene alterations, all of which are mutually exclusive. Tyrosine kinase inhibitors (TKIs) are the standard therapy for metastatic/recurrent GISTs. Molecular alterations are the most reliable biomarkers for TKIs and for other drugs, such as NTRK inhibitors. The pathological and genetic diagnosis prior to treatment has been challenging; however, a newly developed endoscopic device may be useful for diagnosis. In the era of precision medicine, cancer genome profiling by targeted gene panel analysis may enable potential targeted therapy even for GISTs without <i>KIT</i> or <i>PDGFRA</i> mutations. |
topic |
gastrointestinal stromal tumor submucosal tumor subepithelial tumor gene panel analysis precision medicine |
url |
https://www.mdpi.com/2072-6694/13/13/3158 |
work_keys_str_mv |
AT toshirounishida recentprogressandchallengesinthediagnosisandtreatmentofgastrointestinalstromaltumors AT shigetakayoshinaga recentprogressandchallengesinthediagnosisandtreatmentofgastrointestinalstromaltumors AT tsuyoshitakahashi recentprogressandchallengesinthediagnosisandtreatmentofgastrointestinalstromaltumors AT yoichinaito recentprogressandchallengesinthediagnosisandtreatmentofgastrointestinalstromaltumors |
_version_ |
1721299933551656960 |