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[肾上腺皮质癌及其治疗]

[Adrenocortical carcinoma and its treatment].

作者信息

Tupikowski Wiesław, Bednarek-Tupikowska Grazyna, Florczak Agnieszka

机构信息

Dolnoślaskie Centrum Onkologii we Wrocławiu.

出版信息

Postepy Hig Med Dosw (Online). 2004 Feb 26;58:27-36.

Abstract

Adrenocortical carcinoma is a rare tumor with an annual incidence of 1 to 2 cases per million people. It is a very aggressive tumor, with a median survival of 28 months, and is slightly more common in women (58.6%) than in men (41.4%). The etiology of disease is still unknown. Most adrenocortical neoplasms are hormone functional. These tumors most commonly produce cortisol (30%), then androgens (20%), estrogens (10%) or aldosterone (2%). The rapid onset of Cushing 's syndrome, with its virilizing features, is characteristic of this cancer. Adrenal tumors are often detected at an advanced stage. In diagnostics, measurement of serum hormone levels and imaging studies, e.g. CT, or MRI, are essential. They also indicate the spread of disease. Complete surgical resection is the only curative treatment for adrenal cancer. Treatment also includes chemotherapy, especially with mitotane, usually in combination with doxorubicin, etoposide, and cisplatin. Results of treatment are not satisfying, so adjuvant multicenter trials are still underway.

摘要

肾上腺皮质癌是一种罕见肿瘤,年发病率为每百万人1至2例。它是一种侵袭性很强的肿瘤,中位生存期为28个月,在女性中(58.6%)比在男性中(41.4%)略为常见。疾病病因尚不清楚。大多数肾上腺皮质肿瘤具有激素功能。这些肿瘤最常分泌皮质醇(30%),其次是雄激素(20%)、雌激素(10%)或醛固酮(2%)。伴有男性化特征的库欣综合征快速起病是这种癌症的特点。肾上腺肿瘤常常在晚期才被发现。在诊断中,血清激素水平测定以及影像学检查,如CT或MRI,至关重要。它们还能显示疾病的扩散情况。完整的手术切除是肾上腺癌唯一的治愈性治疗方法。治疗还包括化疗,尤其是使用米托坦,通常与阿霉素、依托泊苷和顺铂联合使用。治疗结果并不令人满意,因此多中心辅助试验仍在进行中。

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