The Carney triad is a rare syndrome defined by the coexistence of three tumors: extra-adrenal paraganglioma (e.g. spinal paraganglioma) initially, only functioning extra-adrenal paragangliomas were included, but subsequent work includes non-functioning extra-adrenal paragangliomas 1. gastric gastrointestinal stromal tumors (GIST)

What is the prognosis of Carney’s triad with Gist?

GIST associated with Carney’s triad are mostly indolent. Recurrence after surgery is found in approximately 50% of patients, with a mean interval after the initial presentation of 12 years. Imatinib mesylate, an effective agent in the treatment of GIST, is under investigation as an adjuvant treatment.

What is the treatment of choice for Carney-Stratakis dyad?

The differential diagnosis should include Carney-Stratakis dyad. The treatment of choice for GIST and its metastases (overall rate: 50%) is surgical resection. GIST associated with Carney’s triad are mostly indolent. Recurrence after surgery is found in approximately 50% of patients, with a mean interval after the initial presentation of 12 years.

What is the Carney triad of sarcomas?

Stratakis CA, Carney JA. The triad of paragangliomas, gastric stromal tumours and pulmonary chondromas (Carney triad), and the dyad of paragangliomas and gastric stromal sarcomas (Carney-Stratakis syndrome): molecular genetics and clinical implications.

Do gastrointestinal stromal tumors (GISTs) in Carney triad lack CD117 (c-KIT) mutations?

Subsequent advances in molecular biology have led to the current terminology of gastrointestinal stromal tumors (GISTs). However, there is limited evidence to suggest that the gastrointestinal stromal tumors (GIST) in Carney triad lack CD117 (c-kit) mutations (i.e., they are wild-type), and hence these GISTs may prove unresponsive to Imatinib.