Miura Noriyoshi, Ide Takehiro, Uda Takashi, Noda Terutaka, Asai Seiji, Nishimura Kenichi, Shirato Akitomi, Yanagihara Yutaka, Miyauchi Yuki, Kikugawa Tadahiko, Tanji Nozomu, Yokoyama Masayoshi
Nihon Hinyokika Gakkai Zasshi. 2014 Jul;105(3):79-84. doi: 10.5980/jpnjurol.105.79.
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.
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.
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%.
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.
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。
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。
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%。
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。