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An unusual case of Carney triad with high level catecholamine- secreting but no existence of extra-adrenal paraganglioma

An unusual case of Carney triad with high level catecholamine- secreting but no existence of extra-adrenal paraganglioma
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摘要 In 1977, Carney originally described the association of .gastric epithelioid leiomyosarcoma, pulmonary chondroma and extra-adrenal paraganglioma, and this unusual syndrome was subsequently called "Carney triad". At present, the diagnosis of Carney triad, requires at least two of three components-gastrointestinal stromal tumor (GIST), pulmonary chondroma and extra-adrenal paraganglioma. Up to now, fewer than 80 cases of Carney triad have been reported worldwide.1 ^We described a case of incomplete Carney triad with multiple pulmonary chondromas and GIST. Although the patient was detected in high level secreting of catecholamine, no extra-adrenal paraganglioma has been found. In 1977, Carney originally described the association of .gastric epithelioid leiomyosarcoma, pulmonary chondroma and extra-adrenal paraganglioma, and this unusual syndrome was subsequently called "Carney triad". At present, the diagnosis of Carney triad, requires at least two of three components-gastrointestinal stromal tumor (GIST), pulmonary chondroma and extra-adrenal paraganglioma. Up to now, fewer than 80 cases of Carney triad have been reported worldwide.1 ^We described a case of incomplete Carney triad with multiple pulmonary chondromas and GIST. Although the patient was detected in high level secreting of catecholamine, no extra-adrenal paraganglioma has been found.
出处 《Chinese Medical Journal》 SCIE CAS CSCD 2010年第4期510-512,共3页 中华医学杂志(英文版)
关键词 Carney triad pulmonary chondroma gastrointestinal stromal tumor PARAGANGLIOMA Carney triad pulmonary chondroma gastrointestinal stromal tumor paraganglioma
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参考文献9

  • 1Rodriguez FJ, Aubry MC, Tazelaar HD, Slezak J, Carney JA. Pulmonary chondroma: a tumor associated with Carney triad and different from pulmonary hamartoma. Am J Surg Pathol 2007; 31: 1844-1853.
  • 2Matyakhina L, Bei TA, McWhinney SR, Pasini B, Cameron S, Gunawan B, et al. Genetics of Carney triad: recurrent losses at chromosome 1 but lack of germline mutations in genes associated with paragangliomas and gastrointestinal stromal tumors. J Clin Endocrinol Metab 2007; 92: 2938-2943.
  • 3Kiryu T, Kawaguchi S, Matsui E, Hoshi H, Kokubo M, Shimokawa K. Multiple chondromatous hamartomas of the lung: a case report and review of the literature with special reference to Carney syndrome. Cancer 1999; 85: 2557-2561.
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