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[肾上腺外嗜铬细胞瘤中肾上腺素能症状的高发生率]

[High percentage of adrenergic symptoms in extraadrenal chromaffin tumors].

作者信息

Hervás Abad E, Páramo Fernández C, Casteràs Román A, Hernández De Alonso E, Fajar Rodríguez L, Ramírez Muñoz L, García-Mayor R V

机构信息

Servicio de Endocrinología, Hospital Universitario Sta. M. del Rosell, Cartagena, España.

出版信息

Rev Clin Esp. 2010 Apr;210(4):163-7. doi: 10.1016/j.rce.2009.09.008. Epub 2010 Mar 26.

Abstract

UNLABELLED

Descriptive retrospective study of 14 patients with paragangliomas (PGL) attended in the University Hospital of Vigo (Pontevedra) during the last 25 years to evaluating their characteristics and neuroendocrine potential.

RESULTS

71.4% were diagnosed due to mass tumoral effect, 21.4% due to adrenergic symptoms and 7.1% incidentally. Regarding to symptoms and signs 66.7% of PGL Simpatic (PGLS) and 40% of PGL Parasimpatic (PGLPS) presented adrenergic symptoms. Urine catecholamine analysis was carried out to 4 PGLS and high levels were found in all patients. The tumoral size reached a mean value of 37.8+/-18.9 mm, there were not differences found between both types of tumors. Inmunohistoquimia showed positive Chromogranine A stain in all patients. Two PGLS were maligns. Eleven patients had positive outcome, 2 PGLS died, and 1 PGLPS remains not cured.

CONCLUSIONS

High percentage of patients had clinical manifestations related to catecholamine hyperproduction. We believe that due to the risk related to surgical treatment, malignance, multiple location and family associations, it would be advisable to carry out a complete examination prior to surgery.

摘要

未标注

对过去25年在维戈大学医院(庞特维德拉)就诊的14例副神经节瘤(PGL)患者进行描述性回顾性研究,以评估其特征和神经内分泌潜能。

结果

71.4%因肿瘤肿块效应被诊断,21.4%因肾上腺素能症状被诊断,7.1%为偶然发现。关于症状和体征,66.7%的交感神经型副神经节瘤(PGLS)和40%的副交感神经型副神经节瘤(PGLPS)出现肾上腺素能症状。对4例PGLS进行了尿儿茶酚胺分析,所有患者均发现高水平。肿瘤大小的平均值为37.8±18.9毫米,两种类型的肿瘤之间未发现差异。免疫组织化学显示所有患者嗜铬粒蛋白A染色呈阳性。2例PGLS为恶性。11例患者预后良好,2例PGLS死亡,1例PGLPS仍未治愈。

结论

高比例患者有与儿茶酚胺分泌过多相关的临床表现。我们认为,鉴于与手术治疗、恶性肿瘤、多部位发生和家族关联相关的风险,术前进行全面检查是可取的。

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