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

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

SUBMITTER: Li L 

PROVIDER: S-EPMC6743131 | biostudies-literature | 2019 Sep

REPOSITORIES: biostudies-literature