Abstract: Intravenous leiomyomatosis (IVL) is a rare kind of uterine myoma. It is a benign smooth muscle tumor with invading growth pattern. The tumor extends into venous channels, but rarely invades tissues. It grows along the refluxing direction of the venous channels, uterine vein, ovarian vein, and beyond the uterus, extends into the inferior vena cava till the right atrium or pulmonary arteries, resulting in intracardiac leiomyomatosis (ICL). At present, the tumor can be detected by ultrasonic waves, computer tomography and magnetic resonance imaging. The main ICL therapy is surgery which is divided into onestage operation and twostage operation in which the key is the complete tumor excision. Most sufferers have a good prognosis, but there are possibilities of recurrence. Missed diagnosis and misdiagnosis are not uncommon, because the disease is rare with hided and diversified clinical manifestations. It is fatal without special characteristics. For a better understanding of ICL, the recent research and treatment of ICL are reviewed.
ObjectiveTo investigate the clinical and imaging features of patients with pulmonary lymphangioleiomyomatosis (PLAM),to improve the knowledge of this disease. MethodsEighteen patients with PLAM admitted into Zhongshan Hospital between 2003 and 2013 were retrospectively analyzed. Relevant literatures were reviewed. ResultsAll the 18 cases were female and had dyspnea on exertion. Three of them suffered from chylothorax,6 from pneumothorax and 5 from hemoptysis. The high resolution CT (HRCT) findings in all the 18 cases showed multiple small cysts with thin wall diffusely distributing in the bilateral lung. PLAM was confirmed by biopsy in 14 cases. Pulmonary function tests in 4 cases showed obstructive ventilatory dysfunction. Nine cases (50%) were misdiagnosed as other diseases. All the patients were given symptomatic and supportive treatment. Literatures review showed that as a rare diffuse lung disease,PLAM is more common in women of childbearing age,mainly presenting with dyspnea,hemoptysis,pneumothorax and chylothorax. Diagnosis of the disease relies on medical history,HRCT features and lung tissue biopsy pathology. HRCT examination has diagnostic value for PLAM. Although anti-estrogen treatment has certain effects,rapamycin may be a promising drug for PLAM therapy. ConclusionPatients with PLAM have characteristic clinical and radiographic manifestations. Early diagnosis and effective treatment are keys to the management of patients with PLAM.
ObjectiveTo summarize the diagnosis and management of intravenous leiomyomatosis, and to compare effect of the one-stage surgery and two-stage surgery. MethodsClinicopathological data of 18 patients hospitalized in Peking Union Medical College Hospital who were diagnosed as intravenous leiomyomatosis with intracaval and intracardiac extension during Jan. 2002 to Sep. 2013 were collected, and some indexes of the one-stage surgery group and two-stage surgery group were compared, including blood loss, blood transfusion, operation time, period of stay in ICU, hospital stay, and hospitalization expense. ResultsAll the patients were diagnosed as intravenous leiomyomatosis pathologically after operation. Of the 18 patients, 6 (33.3%) patients underwent one-stage surgery and 12 (66.7%) patients underwent two-stage surgery. There were no significant difference on blood loss, blood transfusion, operation time, period of stay in ICU, hospital stay, and hospitalization expense (P > 0.05). There were some patients suffered from complication, including 1 case of pleural effusion, 1 case of recurrent laryngeal nerve injury, 1 case of pulmonary infection in one-stage surgery group; 1 cases of arrhythmia, 2 cases of intestinal obstruction, 2 cases of pleural effusion in two-stage surgery group. No significance had be found in incidence rate of complication between one-stage surgery group and two-stage surgery group (P=1.000). Tumors of 2 patients who underwent two-stage surgery had developed before the second surgery, increasing the difficulty and risk of the second surgery. Three cases of one-stage group were followed-up for 48-63 month (the median time was 62.0 months), 10 cases in two-stage group were followed-up for 1-43 month (the median time was 19.5 months). During the followed-up period, occurrence happened in 1 case of two-stage group, but without death in all cases. ConclusionsBoth one-stage surgery and two-stage surgery are effective and safe. Taking physical and psychological endurance of patients into consideration, one-stage surgery is highly recommended if the patient is in good status and can tolerate the strike brought by the surgery.
Objective To evaluate the diagnostic value of vascular endothelial growth factor (VEGF)-D detection for the diagnosis of lymphangioleiomyomatosis (LAM) by Meta-analysis. Methods Literatures published before August 2017 were retrieved from PubMed, Embase, China Biology Medicine database, China National Knowledge Internet, Wangfang, and VIP database to retrieve the study about VEGF-D detection for LAM. The studies were screened according to the inclusive and exclusive criteria, the data were extracted, the quality was assessed and the Meta-analysis was performed with related statistical software. Results Six primary studies were included and 521 patients met the inclusion criterion. The Meta-analysis showed the pooled sensitivity, specificity, positive likelihood ratio, negative likelihood ratio and diagnostic odds ratio of 0.75 [95% confidence interval (CI) (0.70, 0.79)], 0.95 [95%CI (0.91, 0.98)], 16.20 [95%CI (8.70, 30.19)], 0.20 [95%CI (0.10, 0.40)] and 89.49 [95%CI (38.46, 208.22)], respectively. The area under the curve was 0.953 9. Conclusions VEGF-D detection showes a good diagnostic value for LAM. A positive result is more clinical meaningful compared with a negative result, helping for the confirmation of the disease.
Pulmonary lymphangioleiomyomatosis (PLAM) is a rare chronic multi-system neoplastic disease that occurs in women of childbearing age. It lacks specific clinical manifestations and requires reliable biomarkers to achieve precise management. In recent years, with the emergence of emerging biomarkers, the detection rate of PLAM has been significantly improved, which can better monitor disease progression and provide timely feedback on the efficacy. These emerging biomarkers mainly include vascular endothelial growth factor-D, vitamin D-binding protein, CT score and prostaglandins. This article will focus on the current research results, and summarize the research progress of emerging biomarkers in PLAM diagnosis, prognosis evaluation, and disease monitoring, aiming to provide new ideas for the research and treatment of PLAM.