目的探讨腹膜假性粘液瘤的诊断和治疗。方法对本院1988~2002年收治的5例腹膜假性粘液瘤的临床资料进行回顾性分析,并结合文献加以讨论。结果该5例患者的主要临床表现为腹胀、腹痛、腹部包块,其中4例行减瘤手术治疗,并在术后辅以腹腔内化疗。术后随访,1例3年内死亡,1例7年内死亡,另2例现已分别存活9年零5个月和3个月。结论腹膜假性粘液瘤是一种少见疾病,其来源多见于阑尾,术前诊断困难,多次针吸和血清癌胚抗原检查对诊断可能有帮助。多数患者术中探查可见腹腔内广泛病变,一次清除很困难,往往需采取多次减瘤术。外科手术治疗仍是目前有效的治疗手段。
Objective To examine the effect and safety of thoracoscopic surgery for left atrium myxoma excision. Method Sixty-nine left atrial myxoma patients underwent excision of left atrial myxoma in our hospital between January 2012 and August 2014 year. The patients were divided into two groups according to the procedure. Thirty patients under-went thoracoscopic surgery, as a thoracoscopic group, with 8 males and 22 females, aged 47.36±13.02 years. Thirty-nine patients received median sternotomy surgery, as a median sternotomy group, with 10 males and 29 females, aged 49.17±13.09 years. The effect and safety between the two groups were compared. Results All patients survived after surgery without death and other serious complications. Compared with the median sternotomy surgery group, longer cardiopul- monary bypass and aortic cross clamp time, shorter ICU stay, ventilator support, and postoperative drainage time, shorter hospital stay time, less postoperative drainage, lower cost, and more higher rate of returning to work in 1 month after surgery were found in the thoracoscopic group with P value less than 0.05. There was no complication of stroke and other neurological complication in the two groups. All patients were followed up for 11 months to 4 years and 7 months, average age of 38.5±12.7 months. There was no recurrence in both groups. Conclusions The thoracoscopic left atrial myxoma excision cardiopulmonary is effective and safe. It can be used as a surgical treatment of left atrial myxoma preferred.
Carney complex (CNC) is a rare autosomal dominant syndrome, characterized by pigmented lesions of the skin and mucosa, cardiac, cutaneous and other myxomas and multiple endocrine tumors. The disease is caused by inactivating mutations or large deletions of the PRKAR1A gene located at 17q22–24 coding for the regulatory subunit type Ⅰ alpha of protein kinase A (PKA) gene. Most recently, components of the complex have been associated with defects of other PKA subunits, such as the catalytic subunits PRKACA (adrenal hyperplasia) and PRKACB (pigmented spots, myxomas, pituitary adenomas). We reviewed CNC’s clinical features, diagnosis, treatment and molecular etiology.
Objective To summarize the clinical characteristics and surgical treatment experience of 1 106 patients with cardiac myxoma. Methods Clinical data of 1 106 patients with cardiac myxoma who underwent surgical treatment in Beijing Anzhen Hospital from 2002 to 2018 were retrospectively analyzed. There were 749 (67.7%) females and 357 (32.3%) males. Results The highest incidence rate was at the age of 51-70 years. The location of the disease was: left atrium in 987 (89.2%) patients, right atrium in 99 (9.0%) patients, left ventricle in 10 (0.9%) patients, right ventricle in 8 (0.7%) patients. There were 1 013 patients of heart classification (NYHA) Ⅰ-Ⅱ and 93 patients of Ⅲ-Ⅳ. There were 301 patients with cerebral infarction and 57 patients with peripheral arterial embolism. Tumor size was closely related to hemodynamic symptoms (P≤0.05), but not to peripheral vascular embolism (P>0.05). Two (0.2%) patients died in hospital and 306 patients were followed up, with a follow-up rate of 27.7%. The median follow-up time was 7 years (range, 1-18 years). One patient died of all causes, and 23 patients recurred, with a recurrence rate of 2.1%. Among 23 recurrent patients, 15 (65.2%) patients were atypical myxoma and 8 (34.8%) patients were typical myxoma. There was no statistical difference in aortic clamping time, ICU stay time, ventilator-assisted breathing time, postoperative hospital stay time, postoperative mortality, or cardiac ejection fraction at discharge between the reoperation in 23 recurrent patients and the first operation in 1 083 non-recurrentpatients. Conclusion Cardiac myxoma is more common in middle-aged and elderly women, and it often occurs in the left atrium. The size of cardiac myxoma can affect the hemodynamic changes. Surgical treatment is effective. Atypical myxoma is more common in recurrent patients, and the effect is still satisfactory through surgical treatment.