Guía de práctica clínica para el diagnóstico y tratamiento de la apoplejía hipofisaria

Classic pituitary apoplexy (PA) is an acute, life-threatening clinical syndrome caused by acute hemorrhage and/or infarction of the pituitary gland. PA is considered a neuroendocrinological emergency. However, there is no consensus about the best options for PA diagnosis and management.ObjectiveTo develop a clinical practice guideline with a number of recommendations for diagnosis and treatment of patients with PA based on the medical evidence available, in order to help clinicians involved in their care.MethodsThe clinical guideline for diagnosis and treatment of pituitary apoplexy issued in 2006 by the Neuroendocrinology Working Group of the Spanish Society of Endocrinology and Nutrition (SEEN) and the British Clinical Practice Guideline published in 2011 were taken as the basis. The text has been adapted to the format used in most international medical journals. For this, after updated medical literature, the quality of evidence and the strength of the recommendations were evaluated using the system proposed by the Agency for Health Care Policy and Research (AHCPR).ConclusionsDiagnosis of pituitary apoplexy should be considered in all patients with acute severe headache with or without neuro-ophthalmic signs. Patients with PA must undergo a complete history and physical examination. All patients with suspected pituitary apoplexy should have urgent blood samples drawn to test electrolytes, renal function, liver function, coagulation screen, complete blood count, and basal levels of pituitary and peripheral hormones, and to rule out adrenocorticotropic hormone (ACTH) deficiency. Formal visual field assessment should be performed when the patient is clinically stable. Magnetic resonance imaging (MRI) is the imaging test of choice to confirm diagnosis. Indications for empirical urgent corticosteroid therapy in patients with PA include hemodynamic instability, impaired consciousness, reduced visual acuity, and severe visual field defects. In patients with these severe neuro-ophthalmic signs, surgery should be considered. Surgery should preferably be performed within seven days of the onset of symptoms. Patients with mild and stable signs may be managed conservatively with careful monitoring. Treatment and long-term follow-up of patients with PA should be conducted by a multidisciplinary team consisting, amongst others, of an experienced pituitary neurosurgeon, an ophthalmologist, and an endocrinologist.     

22例子宫卒中性平滑肌瘤的临床病理特征分析

目的:探讨卒中性平滑肌瘤的临床病理特征、免疫表型及预后。方法:回顾性分析22例子宫卒中性平滑肌瘤的临床特点、病理形态学特点及免疫表型,并复习相关文献。结果:卒中性平滑肌瘤患者的临床症状主要为异常子宫出血,腹痛等。肿瘤大体常伴有以下特征,多灶性出血、坏死、囊性变、水肿变性及质地变软等,镜检可见多灶出血区,呈放射状、卵圆形或不规则形,其中央常可伴有坏死、玻璃样变或肿瘤细胞减少,周边肿瘤细胞富集。22例卒中性平滑肌瘤中,15例核分裂增加(最高达13个/10HPF),但细胞均未见病理性核分裂及显著细胞异型性;22例肿瘤组织弥漫强表达Desmin、SMA、H-caldesmon,Ki67指数范围(3%-15%);18例出血及凝固性坏死区及其周围的肿瘤组织不同程度表达CD10。22例患者均获得完整随访资料,平均随访30个月(10~110个月),均无瘤生存。结论:子宫卒中性平滑肌瘤预后较好,但由于其具有一系列异常的病理形态学特征,如多发出血灶,出血灶中央可伴有坏死,其周围富于肿瘤细胞区细胞可见轻度非典型性且核分裂不同程度增加,易被误诊为子宫恶性潜能未定的平滑肌瘤甚至是子宫平滑肌肉瘤。正确认识该类肿瘤宽广的形态学谱系,有助于临床病理医师做出正确诊断。