目的探討腹膜假性粘液瘤的診斷和治療。方法對本院1988~2002年收治的5例腹膜假性粘液瘤的臨床資料進行回顧性分析，并結合文獻加以討論。結果該5例患者的主要臨床表現為腹脹、腹痛、腹部包塊，其中4例行減瘤手術治療，并在術后輔以腹腔內化療。術后隨訪，1例3年內死亡，1例7年內死亡，另2例現已分別存活9年零5個月和3個月。結論腹膜假性粘液瘤是一種少見疾病，其來源多見于闌尾，術前診斷困難，多次針吸和血清癌胚抗原檢查對診斷可能有幫助。多數患者術中探查可見腹腔內廣泛病變，一次清除很困難，往往需采取多次減瘤術。外科手術治療仍是目前有效的治療手段。
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 explore the diagnosis accuracy of cardiac tumor and effectiveness of surgical treatment. Method We retrospectively analyzed the clinical data of 103 patients with cardiac tumor in our hospital from 2011 through 2014 year. There were 65 females and 38 males, aging from 3 months to 82 years (average age of 59.71±13.80 years). We analyzed age distribution and clinical manifestation of the patients, as well as size and location of tumors. Then we compared effects of different surgical procedures. Result There was no death during evaluation. Early postoperative complications included arrhythmia (47 patients), electrolyte disturbance (13 patients), and cardiac dysfunction (9 patients). One patient with B-cell non-Hodgkin's lymphoma auto-discharged because of cardiac dysfunction. No relapse was obse-rved in the patients with atrial myxoma or lipoma (2 patients) during follow-up. One patient with benign myogenic tumor was lost during the follow-up. Six patients with malignant tumor were with poor long-term effect including 2 patients lost in the follow-up and 4 deaths due to tumor relapse during 1 year after surgery. Conclusion Surgery is still the most effective and major therapy of cardiac tumor.
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.
ObjectiveTo investigate clinical outcomes and safety of minimally invasive left atrial myxoma (LAM) resection via right anterolateral minithoracotomy (ALMT). MethodsClinical data of 9 patients who underwent minimally invasive LAM resection via right ALMT in the Affiliated Hospital of Luzhou Medical College from January 2011 to October 2013 were retrospectively analyzed. There were 2 male and 7 female patients with their age of 37-62 (51±9) years. The operation was performed through a small (4-6 cm) right ALMT incision. Femoral artery and vein and superior vein were cannulated to establish cardiopulmonary bypass (CPB). Transthoracic clamp was used for ascending aortic clamping. Antegrade cold blood cardioplegia was infused for myocardial protection. LAM was resected through right atriotomy trans-septal approach. ResultsAll the operations were successfully performed without in-hospital death. Operation time was 210-310(260±33) minutes, aortic cross-clamping time was 23-50(37±9) minutes, CPB time was 60-87(71±9) minutes, postoperative mechanical ventilation time was 6-14(9.0±2.5) hours, and length of ICU stay was 17-26(20±3) hours. Postoperative mediastinum drainage was 100-650(376±190) ml. Mean length of right ALMT was 4.5-6.0 (5.3±0.6) cm. All the patients were followed up for 1 to 30 months,and echocardiography showed no LAM recurrence. ConclusionMinimally invasive LAM resection via right ALMT is safe and feasible with satisfactory clinical outcomes.
Objective To analyze the causes of cardiac myxoma recurrence and discuss its clinical classification. Methods We reviewed the data of two female patients, aged thirty three and forty two, with recurrent cardiac myxoma, who were admitted into our hospital separately in December 2004 and October 2005. We searched articles with “cardiac myxoma” as the key words at www.cqvip.com, and reviewed literature of big case groups having undergone surgical operations, case reports of recurrence and literature with a followup time longer than 4 years between January 1994 and December 2008. Results We reviewed a total of 1 969 cases of cardiac myxoma, in which there were 60 recurrent cases with a recurrence rate of 3.0%, and there were 15 recurrence case reports. Data analysis showed that single pedicle recurrence rate was 3.0% (3/99) and multiple pedicle recurrence rate was 35.7% (5/14); In two reports on cardiac myxoma distributed in multiple chambers, the recurrence rate was 41.7% (5/12) and 33.3% (3/9) respectively;Average interval of recurrence was 4.1 years; Repeated recurrence happened to 9 cases (12.0%) with the most repetition times of 4 in one case; Malignancy on recurrence was found in one case; Reoperation rate was 64.0% (32/50); Six cases (8.0%) were familial myxoma. Accordingly, we advocate a clinical classification of “typical” and “atypical” cardiac myxoma. The typical myxoma refers to the tumors located at left atrium with single pedicle, rooted at or around fossa ovalis, and without abnormal DNA, while the atypical myxoma are familial tumors and tumors stemming from multiple points or multiple chambers, rooted in abnormal position of the left atrium, arising from clear gene mutation, or with malignant tendency. Conclusion Myxomas with multiple pedicles, distributed in more than one chamber, and rooted in abnormal position of the left atrium have a much higher recurrence rate. Close follow-up is needed for abovementioned patients to achieve an optimal treatment results.