结论:库欣病是一种罕见的疾病,其特征是由于促肾上腺皮质垂体肿瘤导致皮质醇产生过多。虽然主要治疗是手术切除,术后放射治疗可用于持续的激素控制不足或残留或进行性结构性疾病。尽管结果有所改善,垂体肿瘤的放疗与垂体功能减退有关,视觉缺陷和,很少,继发性恶性肿瘤。我们描述了一个不寻常的病例,一名67岁的女性在37岁时被诊断出患有库欣病,并接受了垂体肿瘤的经蝶入路切除术并进行了手术后外部束放疗(EBRT)。酮康唑用于类固醇生成抑制,最后双侧肾上腺切除术治疗难治性疾病。治疗30年后,她目睹了全身性强直阵挛性癫痫发作。放射学检查证实颅外肿块渗入颞骨并进入脑实质。由于复发性癫痫发作,患者被插管并开始接受地塞米松和抗癫痫治疗.肿瘤切除显示为高级成骨细胞骨肉瘤。不幸的是,患者在重症监护中恶化,并在可能的误吸事件后发生致命的心脏骤停。我们描述了风险因素,放射性骨肉瘤的患病率和治疗,垂体照射的极其罕见和晚期并发症。据我们所知,据报道,这是垂体照射和颅骨骨肉瘤发展之间最长的潜伏期。
结论:库欣病采用经蝶入路切除作为一线治疗,在不完全切除的情况下使用放射治疗,疾病复发或持续性皮质醇增多症。垂体瘤照射最常见的长期不良结局是30-60%的患者在10年时发生垂体功能减退,不太常见,视力丧失和动眼神经麻痹,辐射诱发的脑肿瘤和肉瘤。目前提出的辐射诱导骨肉瘤的特征包括:发现与原发性肿瘤不同的组织学类型,在辐射束的路径内或附近发展,和至少3年的潜伏期。颅骨骨肉瘤的治疗包括完全手术切除,其次是全身化疗和/或放疗。放射性骨肉瘤的总体预后较差。诸如立体定向放射外科之类的较新技术可以减少辐射引起的恶性肿瘤的发生率。
CONCLUSIONS: Cushing\'s disease is a rare disorder characterised by excessive cortisol production as a consequence of a corticotroph pituitary tumour. While the primary treatment is surgical resection, post-operative radiation therapy may be used in cases of ongoing inadequate hormonal control or residual or progressive structural disease. Despite improved outcomes, radiotherapy for pituitary tumours is associated with hypopituitarism, visual deficits and, rarely, secondary malignancies. We describe an unusual case of a 67-year-old female with presumed Cushing\'s disease diagnosed at the age of 37, treated with transsphenoidal resection of a pituitary tumour with post-operative external beam radiotherapy (EBRT), ketoconazole for steroidogenesis inhibition, and finally bilateral adrenalectomy for refractory disease. She presented 30 years after her treatment with a witnessed generalised tonic-clonic seizure. Radiological investigations confirmed an extracranial mass infiltrating through the temporal bone and into brain parenchyma. Due to recurrent generalised seizures, the patient was intubated and commenced on dexamethasone and anti-epileptic therapy. Resection of the tumour revealed a high-grade osteoblastic osteosarcoma. Unfortunately, the patient deteriorated in intensive care and suffered a fatal cardiac arrest following a likely aspiration event. We describe the risk factors, prevalence and treatment of radiation-induced osteosarcoma, an exceedingly rare and late complication of pituitary irradiation. To our knowledge, this is the longest reported latency period between pituitary irradiation and the development of an osteosarcoma of the skull.
CONCLUSIONS: Cushing\'s disease is treated with transsphenoidal resection as first-line therapy, with radiotherapy used in cases of incomplete resection, disease recurrence or persistent hypercortisolism. The most common long-term adverse outcome of pituitary tumour irradiation is hypopituitarism occurring in 30-60% of patients at 10 years, and less commonly, vision loss and oculomotor nerve palsies, radiation-induced brain tumours and sarcomas. Currently proposed characteristics of radiation-induced osteosarcomas include: the finding of a different histological type to the primary tumour, has developed within or adjacent to the path of the radiation beam, and a latency period of at least 3 years. Treatment of osteosarcoma of the skull include complete surgical excision, followed by systemic chemotherapy and/or radiotherapy. Overall prognosis in radiation-induced sarcoma of bone is poor. Newer techniques such as stereotactic radiosurgery may reduce the incidence of radiation-induced malignancies.