ACTH-secreting Crooke cell carcinoma of the pituitary

ACTH-secreting Crooke cell carcinoma of the pituitary
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DOI:
10.1111/eci.12010
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发表时间:
2013-01-01
影响因子:
5.5
通讯作者:
Korbonits, Marta
Korbonits, Marta
中科院分区:
医学3区
文献类型:
--
作者:
Kovacs, Gabor L.;Goth, Miklos;Korbonits, Marta

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目的垂体腺瘤是常见的垂体癌是罕见的。目前尚不清楚垂体癌是否出现从头或从adenoma.Methods演变,我们研究了临床特点和组织标本从8垂体手术和尸检垂体癌患者。一名16岁的女性患者被诊断为侵袭性克鲁克细胞大腺瘤。经蝶窦手术后,库欣病的临床体征很快再次出现。在14年的病程中,进行了八次垂体手术,三次颅外照射和两次(90)钇DOTATOC治疗。行双侧肾上腺切除术。患者死于转移性疾病和肾上腺残留导致的不受控制的皮质醇增多症。一个系统的形态学研究(组织学染色,电子显微镜)的所有可用的手术和尸检samples.Results轻快的有丝分裂活动,高Ki-67和p53免疫标记存在于垂体样品从发病。高比例的肿瘤细胞显示不规则的核和大的核仁,并观察到MGMT染色逐渐增加。肿瘤在整个过程中仍然是Crooke细胞型。结论垂体癌是原发性还是由腺瘤转化而来,目前尚不能肯定地回答。
Purpose While pituitary adenomas are common, pituitary carcinomas are rare. It is unclear whether pituitary carcinomas arise de novo or evolve from adenomas.Methods We studied the clinical characteristics and tissue samples from eight pituitary surgeries and the autopsy from a patient with pituitary carcinoma. A 16-year-old female patient was diagnosed with an aggressive Crooke cell macroadenoma. Following transsphenoidal surgery, clinical signs of Cushing disease quickly reappeared. During the 14-year course of the illness, eight pituitary surgeries, three courses of extracranial irradiation and two (90)Yttrium-DOTATOC treatments were undertaken. A bilateral adrenalectomy was performed. The patient died of metastatic disease and uncontrolled hypercortisolism due to an adrenal remnant. A systematic morphologic study (histologic staining, electron microscopy) of all available surgical and autopsy specimens was undertaken.Results Brisk mitotic activity, high Ki-67 and p53 immunolabelling were present in the pituitary samples from the onset. High proportion of tumour cells showed irregular nuclei and large nucleoli, and gradual increase in MGMT staining was observed. The tumour remained of Crooke cell type throughout the course. Autopsy disclosed a postirradiation sarcoma in the pituitary area.Conclusions The question whether pituitary carcinomas arise de novo or transform from an adenoma cannot be answered at present with certainty.