scholarly journals A Malignant Gastrointestinal Stromal Tumor of the Gallbladder Immunoreactive for PDGFRA and Negative for CD 117 Antigen (c-KIT)

HPB Surgery ◽  
2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Athanasios Petrou ◽  
Pari Alexandrou ◽  
Alexandros Papalambros ◽  
Angelica Saetta ◽  
Paraskevi Fragkou ◽  
...  
Keyword(s):  
Gastrointestinal Stromal Tumor ◽  
Gastrointestinal Stromal Tumors ◽  
Growth Factor Receptor ◽  
Stromal Tumor ◽  
Platelet Derived Growth Factor ◽  
Stromal Tumors ◽  
Cd 117 ◽  
Factor Receptor Alpha ◽  
Malignant Gists ◽  
First Time

Gastrointestinal stromal tumors (GISTs) compose the largest category of well-recognized nonepithelial neoplasms of the gastrointestinal tract (GI). GISTs of the gallbladder are extremely rare tumors. Only four malignant, two benign and one GIST-like tumor of the gall bladder have ever been described. The four malignant GISTs were all positive for CD 117 antigen (c-kit). We present for the first time a malignant gastrointestinal stromal tumor of the gallbladder, immunoreactive for platelet-derived growth factor receptor alpha (PDGFRA) and negative for CD 117 antigen (c-KIT).

scholarly journals Gastrointestinal Stromal Tumors: Molecular Mechanisms and Targeted Therapies

2011 ◽  
Vol 2011 ◽  
pp. 1-7 ◽  
Author(s):  
Erinn Downs-Kelly ◽  
Brian P. Rubin
Keyword(s):  
Gastrointestinal Stromal Tumors ◽  
Molecular Mechanisms ◽  
Kinase Inhibitors ◽  
Growth Factor Receptor ◽  
Clinicopathological Features ◽  
Platelet Derived Growth Factor ◽  
Clinical Behavior ◽  
Stromal Tumors ◽  
Mesenchymal Neoplasms ◽  
Factor Receptor Alpha

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract and are diverse not only in their clinical behavior but also in their histologic appearance. GISTs are insensitive to conventional sarcoma chemotherapy and radiation. However GISTs are sensitive to small-molecule tyrosine kinase inhibitors as 85–90% of GISTs have KIT or platelet-derived growth factor receptor alpha (PDGFRA) mutations, which drive tumorigenesis. This review will briefly touch on the clinicopathological features of GIST, while the majority of the review will focus on the clinical and treatment ramifications of KIT and PDGFRA mutations found in GIST.

scholarly journals Case report of rhabdomyosarcomatous transformation of a primary gastrointestinal stromal tumor (GIST)

BMC Cancer ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Li Li ◽  
Marian Khalili ◽  
Gregg Johannes ◽  
Praneeth Baratam ◽  
William F. Morano ◽  
...  
Keyword(s):  
Growth Factor ◽  
Gastrointestinal Stromal Tumor ◽  
Kinase Inhibitors ◽  
Growth Factor Receptor ◽  
Stromal Tumor ◽  
Platelet Derived Growth Factor ◽  
Receptor Alpha ◽  
Additional Information ◽  
Factor Receptor Alpha ◽  
Mesenchymal Neoplasm

Abstract Background Gastrointestinal stromal tumor (GIST) is the most common primary mesenchymal neoplasm of the gastrointestinal tract. Mutations of KIT and platelet-derived growth factor receptor alpha have been well characterized in GISTs. Patients with KIT mutations are generally sensitive to treatment with tyrosine kinase inhibitors. However, some patients with GIST, while initially sensitive to TKIs, gain resistance in later stages of treatment. Heterologous rhabdomyomsarcomatous dedifferentiation of advanced GISTs after long-term imatinib mesylate (IM) therapy has been reported. In these cases, the underlying molecular mechanism of tumor progression and transformation is unclear. Case presentation We report one such patient with rhabdomyosarcomatous dedifferentiation of a GIST without metastatic disease after brief 3-month therapy with IM. The tumor was composed of two distinct phenotypes, a CD117 negative region with rhabdomyosarcomatous differentiation directly adjacent to a CD117 positive classic GIST region. Molecular analysis identified the activating KIT exon 11 mutation in both regions, indicating a common origin for both phenotypes. Additionally, the dedifferentiated component contained two synonymous variants in platelet-derived growth factor receptor alpha and KIT. The increased number of synonymous variants in the rhabdomyosarcomatous region may reflect increased genetic instability of this tumor that may have resulted in the loss of CD117 expression in the dedifferentiated component. Conclusion This study adds to the growing consensus that rhabdomyosarcomatous GIST progresses from a common GIST primary tumor. The role of IM in this progression is uncertain; however short duration of IM treatment in this study supports the hypothesis that rhabdomyosarcomatous GIST progression is not a consequence of IM therapy. Furthermore, we provide additional information supporting the observation that CD117 negative rhabdomyosarcomatous transformation maintains the activating KIT variant without KIT expression.

scholarly journals A Case of Gastric Gastrointestinal Stromal Tumor with Platelet Derived Growth Factor Receptor Alpha Mutation

2009 ◽  
Vol 42 (12) ◽  
pp. 1785-1790
Author(s):  
Hiroshi Nimura ◽  
Naoto Takahashi ◽  
Atsushi Watanabe ◽  
Sigeo Yamashita ◽  
Hironori Ohdaira ◽  
...  
Keyword(s):  
Growth Factor ◽  
Gastrointestinal Stromal Tumor ◽  
Growth Factor Receptor ◽  
Stromal Tumor ◽  
Platelet Derived Growth Factor ◽  
Receptor Alpha ◽  
Factor Receptor Alpha
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