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.
Objective To develop a novel methylene blue staining technique to localize small esophageal leiomyomas (<1.5 cm) and evaluate its feasibility. Methods Between January 2013 and October 2016, 9 patients with small esophageal leiomyomas (<1.5 cm) underwent thoracoscopic enucleation in Tongji Hospital. There were 5 males and 4 females with an average age of 51 years. We preoperatively injected 0.5–1.0 ml methylene blue in the submucosa adjacent to the tumors under the guidance of gastroscope. Then, we transferred the patients to the operating room. Results Staining was successful in 9 patients. The unstained tumor was exposed after the blue-stained mediastinal pleura and overlying muscle were incised longitudinally during video-assisted thoracoscopic surgery via one utility port. No abnormalities were detected in the esophageal mucosa. No major complications, such as esophageal leakage or esophageal diverticulum occurred. Conclusion Endoscopic methylene blue staining is safe and feasible for localizing small esophageal leiomyomas during video-assisted thoracoscopic surgery via one utility port. This method will enable enucleation precise and easy.
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.
【摘要】 目的 探讨子宫颈巨大平滑肌瘤在其诊断及治疗上的特殊性。 方法 对2007年10月-2010年3月收治的11例子宫颈巨大平滑肌瘤患者的临床资料进行分析，对其发病率，诊断和手术治疗进行评价。 结果 11例子宫颈巨大平滑肌瘤中黏膜下2例，腹膜后9例。术前9例出现误诊，其中误诊为盆腔包块5例，子宫体肌瘤3例，子宫肉瘤1例。6例行经腹子宫全切加双附件切除，2例行经腹子宫切除术，1例行经腹肌瘤挖除术，1例行经阴道肌瘤摘除术，1例行经腹肌瘤挖出加宫颈残端切除术。 结论 子宫颈巨大平滑肌瘤由于其位置的特殊性，尤其是凸向腹膜后的肌瘤，由于盆腔器官被挤压，使盆腔解剖结构发生改变，术前易被误诊。且手术过程中易出现损伤及出血，因此术前估计充分，术中仔细认清各器官解剖关系，可有效地减少术中损伤和控制出血。【Abstract】 Objective To investigate the particularity of diagnosis and treatment for giant uterine cervical leiomyoma. Methods We analyzed the clinical data of 11 patients with giant uterine cervical leiomyoma who were admitted in our hospital from October 2007 to March 2010. The incidence, diagnosis and surgical treatment of the disease were evaluated. Results Of the 11 cases, nine were retroperitoneal leiomyoma and two were submucous leiomyoma. There were nine misdiagnosed cases before operation, including five diagnosed as pelvic mass, one as uterine sarcoma and three as uterine corpus leiomyoma. Six patients underwent abdominal hysterectomy and bilateral salpingo-oophorectomy; two underwent abdominal hysterectomy; one underwent abdominal myomectomy; one underwent transvaginal myomectomy; and one underwent abdominal myomectomy with excision of cervical stump. Conclusion The giant uterine cervical leiomyoma is easily misdiagnosed preoperatively due to its special anatomic site. A good example is the retroperitoneal leiomyoma in which the pelvic anatomic structure is changed because of the extrusion of the tumor on other pelvic organs. Furthermore, injuries and bleeding often happen during the operation. Consequently, sufficient preoperative assessment and clearly identifying regional anatomical relations can effectively reduce the damage and bleeding during the operation.