Imboden Petra Nadja Elsässer, Borruat François-Xavier, De Tribolet Nicolas, Meagher-Villemure Kathleen, Pica Alesia, Gomez Fulgencio
Service of Endocrinology, Diabetology and Metabolism, Department of Internal Medicine, University Hospital, Lausanne, Switzerland.
Department of Ophthalmology, Hôpital Ophtalmique Jules-Gonin, Lausanne, Switzerland.
Pituitary. 2004;7(3):149-156. doi: 10.1007/s11102-005-3411-3.
Null-cell carcinomas of the pituitary are extremely rare. We describe a 41-year-old woman with a large adenohypophyseal neoplasm presenting as a primary nonfunctioning tumor without pituitary insufficiency. Signs of mass effect with progressive unilateral ocular motility disorders and anterior pituitary failure developed rapidly. Histopathological examination of the trans-sphenoidally removed tumor showed a primary pituitary null cell tumor with high mitotic index. Pituitary carcinoma was suspected because of rapid relapse of ocular motility disorders and of intra-sellar tumor growth after surgery. Radiotherapy of the sellar and parasellar area with a total dose of 59.4 Gy was performed, achieving marked tumor reduction and a significant improvement of ocular motility disorders. However, 6.5 months after presentation the patient rapidly declined and died of carcinomatous meningitis. Less than 100 pituitary carcinomas have been published so far, most of them as single-case reports, and endocrine, immunohistochemical, and ultrastructural data have not been described in the majority of cases. At presentation, there are no specific symptoms that allow to distinguish benign from malignant tumor. Prognosis is poor, since no curative treatment has been established, but aggressive surgery and radiotherapy has been recommended. Our case highlights the poor prognosis of nonfunctioning pituitary carcinomas.
垂体无细胞瘤极为罕见。我们描述了一名41岁女性,患有巨大的腺垂体肿瘤,表现为原发性无功能性肿瘤且无垂体功能减退。出现了占位效应的体征,伴有进行性单侧眼球运动障碍和垂体前叶功能减退,且进展迅速。经蝶窦切除的肿瘤组织病理学检查显示为原发性垂体无细胞瘤,有高有丝分裂指数。由于眼球运动障碍迅速复发以及术后鞍内肿瘤生长,怀疑为垂体癌。对鞍区和鞍旁区域进行了总剂量为59.4 Gy的放射治疗,肿瘤明显缩小,眼球运动障碍显著改善。然而,就诊6.5个月后,患者病情迅速恶化,死于癌性脑膜炎。迄今为止,发表的垂体癌病例不到100例,大多数为个案报道,大多数病例未描述内分泌、免疫组化和超微结构数据。就诊时,没有可用于区分良性与恶性肿瘤的特异性症状。由于尚未确立治愈性治疗方法,预后较差,但推荐积极的手术和放疗。我们的病例突出了无功能性垂体癌的不良预后。