Clinical Presentation of Gastrointestinal Stromal Tumors

被引:59
作者
Menge, Franka [1 ]
Jakob, Jens [2 ]
Kasper, Bernd [3 ]
Smakic, Armand [4 ]
Gaiser, Timo [5 ]
Hohenberger, Peter [1 ]
机构
[1] Heidelberg Univ, Med Fac Mannheim, Div Surg Oncol, Mannheim Univ,Med Ctr, Theodor Kutzer Ufer 1-3, D-68167 Mannheim, Germany
[2] Heidelberg Univ, Med Fac Mannheim, Dept Surg, Mannheim Univ,Med Ctr, Mannheim, Germany
[3] Heidelberg Univ, Med Fac Mannheim, Interdisciplinary Tumor Ctr, Mannheim Univ,Med Ctr, Mannheim, Germany
[4] Heidelberg Univ, Med Fac Mannheim, Inst Clin Radiol & Nucl Med, Mannheim Univ,Med Ctr, Mannheim, Germany
[5] Heidelberg Univ, Med Fac Mannheim, Inst Pathol, Mannheim Univ,Med Ctr, Mannheim, Germany
关键词
Gastrointestinal stromal tumors; GIST; Clinical presentation; GERMLINE MUTATION; CARNEY TRIAD; C-KIT; RECURRENCE; EMERGENCIES; RESECTION; PREDICT; GISTS; SIZE; ERA;
D O I
10.1159/000494303
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. They constitute 1-2% of all gastrointestinal neoplasms but are the most common subtype of soft tissue sarcomas, accounting for 20-25%. In the late 1990s, GISTs were more and more recognized as a particular tumor entity. The tumors are supposed to originate from the interstitial pacemaker cells of Cajal. They are usually well circumscribed and can be located in every part of the tubular gastrointestinal tract. Most often GISTs occur in the stomach, followed by the small bowel and colon/rectum. In contrast to epithelial tumors, GISTs grow transmurally and submucosal. GISTs can be found with highly variable growth features including tumors with intraluminal, intra- or transmural, and pedunculated appearance. Here we describe the most common clinical presentation of GISTs on the basis of our 809 patients managed from 2004 to 2017. The median age of our patients was 59 years and the average size of GIST was 75 mm (range: 4 mm to 35 cm). The clinical presentation is very heterogeneous, depending on tumor site, size, and growth pattern. GISTs of the stomach is the group with the lowest rate of acute or emergency symptoms with 31%, followed by GISTs of the duodenum with 42%, whereas GISTs of the small bowel show acute symptoms in more than 50% of the cases and have an emergency surgery rate of almost 15%. Many patients are diagnosed accidentally, through screening examinations, or with latent, unspecific symptoms. (c) 2018 S. Karger GmbH, Freiburg
引用
收藏
页码:335 / 340
页数:6
相关论文
共 29 条
[1]   Microscopic gastrointestinal stromal tumors in Esophageal and intestinal surgical resection specimens -: A clinicopothologic, immunohistochemical, and molecular study of 19 lesions [J].
Agaimy, Abbas ;
Wuensch, Peter H. ;
Dirnhqfer, Stephan ;
Bihl, Michel P. ;
Terracciano, Luigi M. ;
Tornillo, Luigi .
AMERICAN JOURNAL OF SURGICAL PATHOLOGY, 2008, 32A (06) :867-873
[2]  
Agaimy A, 2012, INT J CLIN EXP PATHO, V5, P852
[3]   Gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma (Carney triad): Natural history, adrenocortical component, and possible familial occurrence [J].
Carney, JA .
MAYO CLINIC PROCEEDINGS, 1999, 74 (06) :543-552
[4]   Gastrointestinal stromal tumors: correlation between symptoms at presentation, tumor location and prognostic factors in 47 consecutive patients [J].
Caterino, Salvatore ;
Lorenzon, Laura ;
Petrucciani, Niccolo ;
Iannicelli, Elsa ;
Pilozzi, Emanuela ;
Romiti, Adriana ;
Cavallini, Marco ;
Ziparo, Vincenzo .
WORLD JOURNAL OF SURGICAL ONCOLOGY, 2011, 9
[5]  
Constantin VD, 2014, ROM J MORPHOL EMBRYO, V55, P619
[6]   Tumor mitotic rate, size, and location independently predict recurrence after resection of primary gastrointestinal stromal tumor (GIST) [J].
DeMatteo, Ronald P. ;
Gold, Jason S. ;
Saran, Lisa ;
Goenen, Mithat ;
Liau, Kui Hin ;
Maki, Robert G. ;
Singer, Samuel ;
Besmer, Peter ;
Brennan, Murray F. ;
Antonescu, Cristina R. .
CANCER, 2008, 112 (03) :608-615
[7]   Two hundred gastrointestinal stromal tumors - Recurrence patterns and prognostic factors for survival [J].
DeMatteo, RP ;
Lewis, JJ ;
Leung, D ;
Mudan, SS ;
Woodruff, JM ;
Brennan, MF .
ANNALS OF SURGERY, 2000, 231 (01) :51-58
[8]   Remarkable effects of imatinib in a family with young onset gastrointestinal stromal tumors and cutaneous hyperpigmentation associated with a germline KIT-Trp557Arg mutation: case report and literature overview [J].
Farag, S. ;
van der Kolk, L. E. ;
van Boven, H. H. ;
van Akkooi, A. C. J. ;
Beets, G. L. ;
Wilmink, J. W. ;
Steeghs, N. .
FAMILIAL CANCER, 2018, 17 (02) :247-253
[9]   Diagnosis of gastrointestinal stromal tumors: A consensus approach [J].
Fletcher, CDM ;
Berman, JJ ;
Corless, C ;
Gorstein, F ;
Lasota, J ;
Longley, BJ ;
Miettinen, M ;
O'Leary, TJ ;
Remotti, H ;
Rubin, BP ;
Shmookler, B ;
Sobin, LH ;
Weiss, SW .
HUMAN PATHOLOGY, 2002, 33 (05) :459-465
[10]   Pathology of Gastrointestinal Stromal Tumors [J].
Foo, Wai Chin ;
Liegl-Atzwanger, Bernadette ;
Lazar, Alexander J. .
CLINICAL MEDICINE INSIGHTS- PATHOLOGY, 2012, 5 :23-33