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恶性嗜铬细胞瘤的治疗

Treatment of malignant pheochromocytoma.

作者信息

Drasin H

出版信息

West J Med. 1978 Feb;128(2):106-11.

Abstract

Pheochromocytoma is a tumor derived from chromaffin tissue, which secretes catecholamines. Today, about 90 percent of patients with this tumor are cured by surgical procedures. In 8 to 15 percent of patients with this tumor there is unresectable, recurrent or metastatic disease, which causes significant morbidity and mortality. The natural history of metastatic disease includes long-term survivors; many, however, die early of disseminated disease. The most common site of metastatic lesions is the skeleton. Palliation for these lesions can often be achieved with the use of radiation therapy. Other sites are, in general, less responsive to radiation therapy. Chemotherapy has been used in combination with radiation therapy, but the results generally have been disappointing. Chemotherapy with doxorubicin hydrochloride and cyclophosphamide in combination with radiation therapy has provided good palliation for skeletal disease for about five months, when disease progression was again noted. Further information is needed concerning the optimal chemotherapeutic treatment of this unusual tumor.

摘要

嗜铬细胞瘤是一种起源于嗜铬组织的肿瘤,可分泌儿茶酚胺。如今,约90%的该肿瘤患者可通过外科手术治愈。在8%至15%的该肿瘤患者中,存在无法切除、复发或转移性疾病,这会导致显著的发病率和死亡率。转移性疾病的自然病程包括长期存活者;然而,许多患者会因播散性疾病而早期死亡。转移性病变最常见的部位是骨骼。使用放射治疗通常可以实现对这些病变的姑息治疗。一般来说,其他部位对放射治疗的反应较差。化疗已与放射治疗联合使用,但结果通常令人失望。盐酸阿霉素和环磷酰胺联合放射治疗,在疾病再次进展前,可为骨骼疾病提供约五个月的良好姑息治疗效果。关于这种罕见肿瘤的最佳化疗治疗,还需要更多信息。

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