子宫外脉管内平滑肌瘤病的诊治

脉管内平滑肌瘤病发生与雌激素密切相关。该类子宫肌瘤可以超出子宫范围,可在静脉内延伸达下腔静脉,甚至心脏。由于临床症状多变且不典型,早期诊断十分困难。病变侵及下腔静脉者及右心或肺动脉者可引起充血性右心衰的表现和猝死。此时需要与静脉血栓栓塞症、心房黏液瘤等鉴别。CT和MRI是重要的影像诊断方法。治疗原则是手术彻底切除所有病变,以降低复发率。如病变局限于盆腔,建议行全子宫切除术+盆腔病灶切除术+双侧附件切除,如果累及大血管甚至心腔,完整手术切除病灶需要多学科合作完成。     

Intracardiac leiomyomatosis

We describe a case of intracardiac leiomyomatosis originating from the uterus, growing up in the inferior vena cava, and extending into the right ventricle. She also found to have lung metastasis. As she declined for further operative intervention, Tamoxifen was given for the control of disease. This case represented an unusual rapid recurrence of intravenous leiomyomatosis which was potentially preventable. To investigate for the appropriate management, a review of the literature concerning this rare condition was made. Received: 5 June 2000 / Accepted: 26 July 2000     

Intravenous leiomyomatosis with cardiac extension

Intravenous leiomyomatosis with cardiac extension is an extremely rare uterine tumor. We report here a case of a patient with a uterine leiomyoma which extended into the right atrium through the left ovarian vein, progressing into the left renal vein along the inferior vena cava. Complete one-stage removal of the tumor was performed using cardiopulmonary bypass, and the patient has shown a favorable outcome. Successful therapy for intravenous leiomyomatosis is dependent on total surgical excision of the tumor, cessation of ovarian function and avoidance of postoperative estrogen replacement therapy.     

GnRH agonist for intravenous leiomyomatosis with cardiac extension. A case report.

BACKGROUND: Intravenous leiomyomatosis with cardiac extension is an extremely rare disease. CASE: We recently treated a case of intravenous leiomyomatosis with extension from the inferior vena cava into the right atrium. Three operations--exploratory laparotomy, debulking of the pelvic mass and resection of the intracardiac leiomyoma--were performed. Since cells of the resected leiomyomatosis were estrogen receptor positive, we postoperatively administered GnRH agonist (leuprorelin acetate) for six months to prevent regrowth of the residual mass in the pelvis. The residual mass began to enlarge immediately after cessation of leuprorelin acetate. The same medication was readministered, and regrowth of the residual mass was completely inhibited for 15 months, until this writing. CONCLUSION: Intravenous leiomyomatosis seems to be hormone dependent, as in the case of uterine leiomyomas. In the absence of total resection, functioning ovarian tissue may remain. Therefore, long-term treatment with GnRH agonist may be useful in preventing recurrence of this disease.     

Successful One-Stage Complete Removal of an Entire Intravenous Leiomyomatosis in the Heart, Vena Cava, and Uterus

The patient was a 61-year-old woman who had intravenous leiomyomatosis (IVL) extending into the right ventricle of the heart from the uterus. Tumors in the heart and the inferior vena cava were removed under cardiopulmonary bypass and then after terminating the cardiopulmonary bypass, tumors in the abdominal cavity including uterine leiomyoma were removed. This is the first case of extended IVL in which all tumors were successfully removed at one time. If a tumor is found in the right ventricle in a woman who had or currently has uterine leiomyoma, IVL should be suspected. Complete one-stage removal of tumors can be performed using cardiopulmonary bypass, and the present patient has shown a favorable outcome.     

Intravenous leiomyomatosis misdiagnosed with large thrombosis in inferior vena cava

ObjectiveThe aim of this report is to highlight the importance of a comprehensive preoperative evaluation in the case of intravenous leiomyomatosis.Case reportA 49-year-old women was presented with dyspnea and abdominal distension. Imaging studies revealed a large leiomyoma with intravenous leiomyomatosis from this mass to the right parauterine veins, right ovarian vein reaching the inferior vena cava. Complete resection was performed by a two-stage operation by a multidisciplinary team. Final pathology confirmed it to be intravenous leiomyomatosis and uterine leiomyomas.ConclusionIntravenous leiomyomatosis is a benign and rare disease that can be a fatal condition. Precise diagnosis and appropriate treatment are important for the best outcome. Gynecologists should consider this rare disease when a patient with a uterine tumor shows symptoms such as chest pain and dyspnea.     

Intravenous leiomyomatosis of the uterus

We present a case of intravenous leiomyomatosis (IVL) associated leiomyoma of the uterus in a 57-year-old woman. It is clinically and morphologically suspected to be leiomyoma. IVL is a rare benign neoplasm that commonly grows into the pelvic veins and the inferior vena cava and, rarely, the right side of the heart. Histologically benign smooth-muscle tumor arises from either a uterine myoma or the wall of a uterine vessel with extension into veins. The primary treatment of IVL is hysterectomy and the excision of any extrauterine tumor, when technically feasible. Antiestrogenic therapy has been suggested as potentially useful in controlling of the unresectable tumor.     

Nongenital pelvic leiomyosarcoma metastatic to the heart

A patient who had a 5-year history of a low-grade nongenital pelvic leiomyosarcoma was evaluated for worsening dyspnea, hypertension, and jugular venous distension. An echocardiogram revealed a large right atrial mass. At surgical exploration, metastatic leiomyosarcoma was found within the inferior vena cava extending from below the renal veins up into the right atrium. Using cardiopulmonary bypass with profound hypothermia and circulatory arrest, the inferior vena cava was opened below the renal veins, and the tumor was transected. That portion of the tumor above this transection was then extracted through a right atriotomy. Resection of the pelvic tumor was not thought to be feasible. The patient remains asymptomatic with stable pelvic tumor 1 year after the procedure.     

Metastatic tumor extending through the inferior vena cava into the right atrium: a case report of carcinoma of the uterine cervix with para-aortic lymph node metastases

Carcinoma of the uterine cervix with cardiac metastasis is not uncommon in autopsy cases. However, an intraatrial tumor extending through the inferior vena cava (IVC) from the site of para-aortic lymph node metastasis has never been reported. A 57-year-old Japanese woman was admitted to an emergency care unit complaining of mild chest pain and shortness of breath. She had progressive multiple lymphatic metastases of stage IIIB squamous cell carcinoma of the uterine cervix that had initially been treated with concurrent chemoradiation. Echocardiogram showed pedunculated tumor in the right atrium (RA), and computed tomography demonstrated multiple pulmonary tumor embolism. Surgical specimen from the RA showed squamous cell carcinoma resembling the primary cervical tumor, and the peduncle appeared to originate from within the IVC. Postoperative ultrasonography showed severe stenosis of the abdominal IVC due to the invasive growth of para-aortic lymph node metastases. The stalk of the tumor originated from this lesion. We present an extremely unusual case of intraatrial metastatic tumor originating from the para-aortic lymph nodes of cervical cancer.     

Leiomyosarcoma of the uterus with a florid intravascular component ("intravenous leiomyosarcomatosis").

A leiomyosarcoma of the uterus in a 54-year-old woman exhibited striking involvement of large vessels of the myometrium and broad ligament on both gross and microscopic examination. The pattern of vascular involvement resembled that seen in intravenous leiomyomatosis. Imaging studies showed recurrent tumor within the inferior vena cava 3 months after hysterectomy. To our knowledge, this is the first reported such case in the literature, for which we propose the designation intravenous leiomyosarcomatosis of the uterus.     

Embryonal sarcoma of the liver in pregnancy, associated with HELLP syndrome

After cesarean section, a 30-year-old patient with HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count in association with preeclampsia) had fever, jaundice, loss of ascites, and a cardiac murmur. Magnetic resonance imaging showed a tumor extending from the left lobe of the liver into the inferior vena cava, right atrium, and ventricle. The biopsy specimen revealed an embryonal sarcoma of the liver. On the second day of chemotherapy, the patient died of intra-abdominal hemorrhage from the tumor.     

Intravenous leiomyomatosis

A case of intravenous leiomyomatosis is presented along with a review of the literature. The condition is a neoplasia of smooth muscle originating from the uterus, with vermiform extensions into veins. Although histologically benign, the clinical course can be fatal if intravenous tumor progresses to the heart. Ordinary leiomyomas of the uterus are frequently coexistent. The symptoms are usually vague. The diagnosis should be suspected when a worm-like, greyish tumor with a smooth surface is found in cut vessels or when right-sided cardiac tumors are detected. Radical excision of the uterus and ovaries, as well as of the intravenous tumor extensions is recommended. In our case the tumor extended into the vena cava. The diagnosis was incidental during nephrectomy due to hydronephrosis. Tumor tissue was positive for estrogen and progesterone cytosolic receptors.     

Absent ductus venosus in the fetus: review of the literature and first report of direct umbilical venous drainage to the coronary sinus

The ductus venosus connects the portal and umbilical veins with the inferior vena cava and acts as a sphincter to protect the fetus from placental overcirculation. Its absence usually causes hydrops fetalis and is associated with high mortality rate, chromosomal anomalies and congenital malformations. In this condition, the umbilical vein almost always drains directly into right-sided structures such as inferior vena cava or right atrium. We reviewed the literature and describe the first case of a fetus with absent ductus venosus and direct connection of the umbilical vein to the coronary sinus.     

Low-Grade Endometrial Stromal Sarcoma with Cardiovascular Involvement—A Report of Three Cases

Background. In low-grade endometrial stromal sarcoma, it has been reported that vascular space involvement in surgical specimens is found in over 50% of patients. However, in contrast to intravenous leiomyomatosis, it has been thought that further tumor extension to large vessels is rarely observed.Cases. We present three cases of low-grade endometrial stromal sarcoma with cardiovascular involvement by recurrent tumors observed on imaging studies. Two cases demonstrated tumor infiltration inside the inferior vena cava while the other case showed tumor growth in the left ventricle.Conclusion. This report suggests that attention should be paid to the possibility of cardiovascular invasion during the entire course of this disease.     

子宫静脉内平滑肌瘤病的诊治进展

子宫静脉内平滑肌瘤病（intravenous leiomyomatosis，IVL）是一种罕见的疾病，可沿血管生长，延伸到下腔静脉，甚至心脏。目前对其发病机制尚有争议，多数学者考虑其起源于子宫肌瘤直接侵入子宫肌层静脉并沿血管腔扩散所致。子宫IVL好发于40~50岁有生育史的女性，早期临床表现不典型，如累及下腔静脉或右心时，可出现腹水、肝脾肿大、呼吸困难、心力衰竭，严重者甚至猝死。手术是目前首选的治疗方法，但具体的手术方案尚无统一共识。该病复发率高，术后需长期严密随访。综述子宫IVL的发病机制及高危因素、临床及影像学表现、诊断与鉴别诊断、治疗、预后及随访，以期为该病的诊疗提供参考。     

Primary primitive neuroectodermal tumor of the urinary tract.

Primary primitive neuroectodermal tumor (PNET) of the urinary tract is a rare disease with aggressive behavior and poor prognosis. We analyzed 851 cases of urinary tract malignancies in our hospital between 1984 and 2004. Only three (0.035%) cases with PNET of the urinary tract were identified. Presenting symptoms included flank pain and hematuria. The first case was a 44-year-old man with left renal PNET who underwent hand-assisted laparoscopic radical nephrectomy and adjuvant chemotherapy. There was no recurrent tumor at the 4-year follow-up. The second case was a 75-year-old woman with right renal PNET with inferior vena cava (IVC) thrombosis extending to the right atrium. The patient underwent right radical nephroureterectomy and IVC thrombectomy with cardiopulmonary bypass. She died of metastatic disease 7 months later. The third case was a 45-year-old man with left ureteral PNET. Left ureteral segmental resection and partial cystectomy were performed. Tumor recurrence was noted 7 years later. The patient died of disseminated disease 1 year after the discovery of recurrence. Urinary tract PNET appears to be an aggressive malignancy. Long-term survival is possible if complete resection is performed at an early stage.     

Pleural effusion with parenteral nutrition solution: an unusual complication of an "appropriately" placed umbilical venous catheter

Pleural effusion is not an uncommon complication of percutaneous intravenous catheters in neonates. Umbilical venous catheters (UVCs) are associated with pleural effusion following abnormal placement in the left atrium or pulmonary veins due to venous obstruction. We report for the first time a case of right-sided pleural effusion with parenteral nutrition solution following a UVC that appeared to be positioned appropriately in the inferior vena cava.     

Experience with the Cardial inferior vena cava filter as prophylaxis against pulmonary embolism in pregnant women with extensive deep venous thrombosis.

OBJECTIVE: To report the use of the Cardial inferior vena caval filter as prophylaxis against pulmonary embolism in pregnant women with extensive iliofemoral thrombosis. SETTING: Leicester Royal Infirmary. SUBJECTS: Four pregnant women with extensive iliofemoral thrombosis, deemed to be at high risk of pulmonary embolism, managed over a period of one year. TECHNIQUE: In addition to standard full anticoagulation with heparin, the Cardial inferior vena cava filter was introduced percutaneously under local anaesthesia through the unaffected contralateral femoral vein and positioned in the inferior cava below the renal veins. RESULTS: The procedure was uncomplicated and did not compromise feto-maternal condition. There was no evidence of pulmonary embolism after filter insertion. CONCLUSION: The use of inferior vena cava filters should be considered as an adjunct to intravenous anticoagulation in pregnant women with extensive deep vein thrombosis of the lower limbs.     

Late intracaval and intracardiac leiomyomatosis following hysterectomy for benign myomas treated by surgery and GnRH agonist

BACKGROUND: The aim of this study was to report an exceptional case of a patient presenting with intracaval and intracardiac leiomyomatosis treated by combined surgical and medical treatment. CASE: A 48-year-old presented with intracaval and intracardiac leiomyomatosis (IL) discovered 6 years following a total hysterectomy with ovarian conservation for myomas. Surgical resection of the pelvic myomas and intracaval leiomyomatosis was performed during the same surgical procedure. Given the presence of a small tumor residuum in the pelvic cavity, postoperative medical treatment based on a gonadotropin-releasing hormone (GnRH) agonist was delivered for 1 year. The patient was followed-up using clinical examination and systematic CT scan. Ten months following the end of medical treatment, she is still in good health and the pelvic residuum has stabilized. CONCLUSIONS: Patients with pelvic tumor combined with IL could be treated using a one-stage surgical procedure. In cases of incomplete surgical resection, medical treatment based on GnRH agonist could be successfully delivered.     

Intravascular leiomyomatosis: unusual variant of leiomyoma

Leiomyomas (fibroid) arise from the smooth muscle of the myometrium and are benign in nature. Intavascular leiomyomatosis is an unusual and rare condition where the fibroid grows into the pelvic veins and inferior vena cava and sometimes extends into the heart chambers. We present a case where a woman presented to us with multiple fibroids and underwent an abdominal hysterectomy. The diagnosis was not suspected before the surgery but diagnosed on histopathology. The patient underwent further surgery to remove the fibroids from the pelvic veins and the inferior vena cava. This condition has been reported in only few case reports around the world. In view of the rarity of this condition, the diagnosis can be easily missed. We suggest an increased vigilance in women who have large fibroids with unusual features in symptoms or during surgery. Pre-surgical imaging will help to make diagnosis, and good surgical outcomes can be achieved by a multidisciplinary surgical approach.