Holthouse D J, Robbins P D, Kahler R, Knuckey N, Pullan P
Department of Neurosurgery, Sir Charles Gairdner Hospital, Perth, Western Australia.
Endocr Pathol. 2001 Fall;12(3):329-41. doi: 10.1385/ep:12:3:329.
Pituitary carcinomas, currently defined as primary adenohypophyseal neoplasms with evidence of either brain invasion or metastatic spread, are exceptionally rare. A case of corticotroph pituitary carcinoma is reported. A 17-year-old female first presented with an invasive and clinically non-functioning pituitary macroadenoma. The primary pituitary lesion lacked atypical histological features and retrospective immunohistochemical studies confirmed its corticotrophic nature. Repeated episodes of local recurrence followed together with the acquisition of severe Cushing's disease. Local disease control was not obtained despite repeated surgical decompression and courses of radiation therapy. Systemic dissemination with multiple bone metastases became manifest eleven years after the first presentation. Atypical histological features and Crooke's hyaline change were evident in both the recurrent and metastatic disease. She remains alive at last follow-up with severe complications relating to persisting sellar and metastatic disease and ongoing endocrine dysfunction. A further 32 cases of pituitary corticotroph carcinoma reported in English are reviewed to highlight the clinicopathological features of this rare form of pituitary neoplasm. Difficulties associated with the diagnosis and management of pituitary carcinoma are also discussed.
垂体癌目前被定义为具有脑侵犯或转移扩散证据的原发性腺垂体肿瘤,极为罕见。本文报告一例促肾上腺皮质激素细胞垂体癌。一名17岁女性最初表现为侵袭性且临床上无功能的垂体大腺瘤。原发性垂体病变缺乏非典型组织学特征,回顾性免疫组化研究证实其为促肾上腺皮质激素细胞性质。随后出现多次局部复发,并伴有严重库欣病。尽管反复进行手术减压和放射治疗疗程,但仍未实现局部疾病控制。首次就诊11年后出现多发骨转移的全身播散。复发和转移疾病中均可见非典型组织学特征和克鲁克透明变性。在最后一次随访时她仍然存活,但存在与持续的鞍区和转移性疾病相关的严重并发症以及持续的内分泌功能障碍。本文复习了另外32例英文报道的垂体促肾上腺皮质激素细胞癌,以突出这种罕见垂体肿瘤形式的临床病理特征。还讨论了与垂体癌诊断和管理相关的困难。