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肾上腺皮质癌患者的临床病理特征及预后

[Clinicopathologic characteristics and prognosis of patients with adrenocortical carcinoma].

作者信息

Miura Noriyoshi, Ide Takehiro, Uda Takashi, Noda Terutaka, Asai Seiji, Nishimura Kenichi, Shirato Akitomi, Yanagihara Yutaka, Miyauchi Yuki, Kikugawa Tadahiko, Tanji Nozomu, Yokoyama Masayoshi

出版信息

Nihon Hinyokika Gakkai Zasshi. 2014 Jul;105(3):79-84. doi: 10.5980/jpnjurol.105.79.

Abstract

PURPOSE

Adrenocortical carcinoma (ACC) is a rare condition associated with poor prognosis. This study aimed to evaluate the clinicopathologic characteristics and prognosis of 7 patients with ACC.

PATIENTS AND METHODS

The clinicopathologic characteristics, treatment, and survival of 7 patients with pathologically confirmed ACC treated at our institution between January 2002 and December 2012 were retrospectively examined.

RESULTS

The study cohort comprised 4 male and 3 female patients (median age at diagnosis, 63 years [range, 36-71 years]). The median tumor size was 7.0 cm (range, 4.0-13.0 cm), and the median follow-up duration was 22 months (range, 9-107 months). One patient had stage I ACC, 4 had stage III, and 2 showed metastasis. The patient with stage I disease underwent laparoscopic adrenorectomy and those with stage III disease underwent adrenorectomy with the excision of adjacent organs. Four of these 5 patients are alive without recurrence at a median of 55 months (range, 22-107 months) after surgery. Of the 2 patients with metastases, 1 received combined chemotherapy with etoposide, adriamycin, and cisplatin plus mitotane without surgical resection but died 19 months later, and the other, with a solitary lung metastasis, underwent adrenorectomy and metastatectomy followed by adjuvant treatment with mitotane and is alive without recurrence at 9 months after treatment. The 3-year cause-specific survival rate was 56%.

CONCLUSIONS

Patients with advanced-stage tumors showed long-term survival with complete tumor resection at diagnosis; hence, this seems to be most beneficial treatment option for patients with ACC.

摘要

目的

肾上腺皮质癌(ACC)是一种预后较差的罕见疾病。本研究旨在评估7例ACC患者的临床病理特征及预后。

患者与方法

回顾性分析2002年1月至2012年12月在我院接受治疗的7例经病理确诊的ACC患者的临床病理特征、治疗情况及生存情况。

结果

研究队列包括4例男性和3例女性患者(诊断时的中位年龄为63岁[范围36 - 71岁])。肿瘤中位大小为7.0 cm(范围4.0 - 13.0 cm),中位随访时间为22个月(范围9 - 107个月)。1例患者为Ⅰ期ACC,4例为Ⅲ期,2例有转移。Ⅰ期疾病患者接受了腹腔镜肾上腺切除术,Ⅲ期疾病患者接受了肾上腺切除术及相邻器官切除。这5例患者中有4例在术后中位55个月(范围22 - 107个月)时存活且无复发。2例有转移的患者中,1例接受了依托泊苷、阿霉素和顺铂联合米托坦的化疗,未进行手术切除,但19个月后死亡;另1例有孤立性肺转移患者,接受了肾上腺切除术和转移灶切除术,随后接受米托坦辅助治疗,治疗后9个月存活且无复发。3年病因特异性生存率为56%。

结论

晚期肿瘤患者在诊断时通过完整切除肿瘤可获得长期生存;因此,这似乎是ACC患者最有益的治疗选择。

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