endocrine cancer

内分泌癌
  • 文章类型: Case Reports
    重症肌无力(MG)是一种影响神经肌肉接头的自身免疫性疾病,其特征是无力和疲劳。如果延迟治疗,发病率很高。与胸腺瘤的明确关联导致胸腺切除术作为一种常见做法,但很少报道出现胸腺切除术后的MG。我们提出了一个82岁女性发展疲劳的案例,胸腺切除术后3个月的下垂和构音障碍。经过MG的临床诊断,她对泼尼松龙和吡啶斯的明的迅速治疗反应良好。她的抗乙酰胆碱受体抗体(抗AChR)随后恢复为阳性。我们的系统评价显示,胸腺切除术后MG可以分为不同病因的早发性或晚发性形式,并证明了术前抗AChR滴度与胸腺切除术后MG之间的相关性。胸腺切除术后MG的假定机制围绕持久的外周自身抗体。临床医生应积极寻找胸腺瘤患者的MG症状,并在术前测量抗AChR以帮助预后。
    Myasthenia gravis (MG) is an autoimmune condition affecting the neuromuscular junction characterised by weakness and fatiguability, carrying a high morbidity if treatment is delayed. A clear association with thymoma has led to management with thymectomy as a common practice, but MG presenting post-thymectomy has rarely been reported. We present a case of an 82- year-old woman developing fatigue, ptosis and dysarthria 3 months after thymectomy. After a clinical diagnosis of MG was made, she responded well to prompt treatment with prednisolone and pyridostigmine. Her anti-acetylcholine receptor antibody (anti-AChR) subsequently came back positive. Our systematic review reveals that post-thymectomy MG can be categorised as early-onset or late-onset form with differing aetiology, and demonstrated correlation between preoperative anti-AChR titres and post-thymectomy MG. The postulated mechanisms for post-thymectomy MG centre around long-lasting peripheral autoantibodies. Clinicians should actively look for MG symptoms in thymoma patients and measure anti-AChR preoperatively to aid prognostication.
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  • 文章类型: Case Reports
    生长抑素瘤是罕见的神经内分泌肿瘤,主要位于胰腺或十二指肠,估计发病率为4000万分之一。十二指肠生长抑素瘤(DSs)通常与1型神经纤维瘤病(NF1)有关。结节性硬化症和VonHippel-Lindau综合征。NF1中也描述了胃肠道间质瘤(GIST),但与生长抑素瘤的关联非常罕见。我们报告了一例NF1患者,该患者因Vater壶腹周围多个牢固结节而出现阻塞性黄疸。进行了胰十二指肠切除术,发现1厘米的十二指肠/壶腹部肿块,生长抑素染色呈阳性,在十二指肠壁上还发现了一个GIST。尽管它很罕见,在NF1患者的胆道扩张的鉴别诊断中,应考虑壶腹生长抑素瘤。
    Somatostatinomas are rare neuroendocrine tumours, mostly located in the pancreas or duodenum, with an estimated incidence of 1 in 40 million. Duodenal somatostatinomas (DSs) are usually found in association with neurofibromatosis type 1 (NF1), tuberous sclerosis and Von Hippel-Lindau syndrome. Gastrointestinal stromal tumours (GIST) have also been described in NF1, but the association with somatostatinoma is very uncommon. We report the case of a patient with NF1 who presented with obstructive jaundice due to multiple firm nodules around the ampulla of Vater. A pancreaticoduodenectomy was performed and revealed a 1 cm duodenal/ampullary mass which stained positive for somatostatin, together with a GIST also found on the duodenal wall. Despite its rarity, ampullary somatostatinomas should be considered in the differential diagnosis of biliary tract dilation in patients with NF1.
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  • 文章类型: Journal Article
    Parathyroid carcinoma (PC) is one of the rarest known types of cancer and has a moderate prognosis, with estimated 5- and 10-year overall survival rates between 78-85% and between 49-70%, respectively. To raise awareness of this disease, and to optimize its diagnosis, clinical management and prognosis, the present study retrospectively reviewed 234 cases of PC. A total of 226 cases of PC, which were archived between 1984 and 2015 in the three major databases of the Chinese population, were retrieved and pooled with the 8 cases diagnosed and treated at the Department of Thyroid Surgery of The First Hospital of Jilin University (Changchun, China) between June 2008 and December 2015. The clinicopathological features, diagnosis, surgical procedures and outcomes of these cases of PC were investigated. The review revealed that misdiagnosis has been a considerable issue, with >80% of the patients misdiagnosed prior to surgery, and the accuracy of intraoperative diagnosis based on frozen sections was only 15.04%. The use of radical resection as first-line therapy significantly improved the disease-free survival by ~8 years (log-rank, 20.956; P<0.001); and, at relapse, reoperation prolonged patient survival by ~7 years (log-rank, 35.322; P<0.001). Consistently, a Cox proportional hazards analysis indicated that radical resection as a first-line therapy reduced the risk of postoperative recurrence (P=0.030), and that reoperation following recurrence significantly improved patient survival (P=0.030). The 5- and 10-year cumulative disease-specific survival rates of the cases of PC were 83 and 67%, respectively. Notably, an increased mortality rate was observed among males with PC compared with female patients with PC. In summary, in the past 32 years (1984-2015), the majority of patients with PC have been misdiagnosed. Performing radical resection as the first-line therapy significantly reduces recurrence and improves patient survival time; and, following relapse, subsequent surgery has also been demonstrated to be an effective approach.
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