Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0406810 (NAME)
 13,345 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Gastrointestinal stromal tumors (GIST) represent an extremely rare group of tumors, which are mostly of smooth muscle origin like leiomyomas, leiomyosarcomas and leiomyoblastomas. With the introduction of immunohistochemical analysis an epithelioid and an autonomic nerve variant can be distinguished. The purpose of this review is to demonstrate the image morphological appearance of these rare tumors together with the pathology based upon a retrospective analysis of five of our own cases since 1997. There are no pathognomonic imaging findings for characterizing a gastrointestinal stromal tumor; however, it should be included in the differential diagnosis if one or multiple large, round or oval, well-delineated gastrointestinal tumors occur in combination with central necrosis. Carney's syndrome is characterized by the syndromal association of a gastrointestinal stromal tumor (originally: gastric leiomyosarcoma) with an extra-adrenal paraganglioma and a pulmonary chondroma. In this rare syndrome, the radiological approach is important to diagnose or rule out the--simultaneous or consecutive--appearance of at least two of the three tumor entities (GIST, extra-adrenal paraganglioma, pulmonary chondroma).
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PMID:[Radiology of gastrointestinal stromal tumors (GIST) and a case of Carney syndrome]. 1077 62

This is a report of a patient who manifests all of the features of Carney's syndrome, including gastrointestinal stromal tumors (GISTs), extra-adrenal paragangliomas, and pulmonary chondromas. The patient underwent surgical resection of a gastric GIST; a retroperitoneal, nonfunctional paraganglioma; and a mediastinal, catecholamine-secreting paraganglioma (pheochromocytoma). Recently, new gastric GISTs were diagnosed by endoscopic, ultrasound-guided, fine-needle aspiration (EUS-FNA) biopsy and were resected. Recurrence of stromal tumors following complete resection is common in Carney's syndrome and presents considerable management challenges. This case illustrates several important points: (1) Carney's syndrome, although rare, should be considered in patients with foregut GISTs; (2) GISTs associated with Carney's syndrome, like sporadic gastric GISTs, may have a more indolent clinical course; and (3) EUS-FNA may be useful for the diagnosis and management of GISTs in this syndrome.
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PMID:Endosonographic diagnosis of recurrent gastrointestinal stromal tumors associated with Carney's syndrome. 1563 34

New immunotherapy represented by immune checkpoint inhibitor therapy and chimeric antigen receptor T-Cell immunotherapy (CAR-T) has already become hot trend in the treatment of malignant tumors. In gastrointestinal stromal tumor (GIST), with notable tumor-infiltrating immune cells existing in GIST tissues and immunological effects reported in imatinib mesylate (IM) treatment, the clinicians and researchers started to realize the possibilities of immunotherapy in GIST. Recent studies reported that PD-1/PD-L1 or CTLA-4 blockade may enhance the T-cell activity and anti-tumor effect of targeted therapy, which can be applied in advanced GIST, and anti-KIT CAR T-cells indicated a new immunotherapeutic targeted strategy for GIST patients with TKI resistance. All the immunotherapies in GIST mentioned above are frontline researches but their efficacies still need more evidence from clinical trials to verify.
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PMID:[Targeted therapy combined with immunotherapy in gastrointestinal stromal tumor: a new era of hope and challenges]. 2890 Sep 84