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.     

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。     

Intravascular leiomyomatosis and benign metastasizing leiomyoma: an unusual case

Benign metastasizing leiomyoma (BML) and intravascular leiomyomatosis (IVL) are rare variants of uterine leiomyomas. In our search of available literature, there have been only two reports of these conditions occurring in the same patient. We report a case of a 42-year-old female presenting with a left L4 nerve root lesion, left paravesical lesion, left ovarian cyst, multiple pulmonary metastases, and an intracaval lesion. Histology confirmed these to be leiomyomata strongly positive for estrogen receptors. Treatment included surgery, in two stages, to remove the L4 nerve root, left paravesical lesion, intracaval lesion, and a single pulmonary nodule. The remaining tumor was treated with a gonadotrophin-releasing hormone agonist, resulting in significant reductions in tumor size. It was concluded that the lesions in the lungs were an example of BML arising from the initial diagnosis of uterine leiomyoma, and the caval lesion was an IVL. Long-term follow-up is recommended, and familiarity with rare forms of benign smooth muscle uterine tumors is essential in avoiding misdiagnosis and overtreatment.     

Rare tumours of myometrium--intravenous leiomyomatosis and benign metastatic leiomyoma

Leiomyomas are the most common benign uterine neoplasms of the myometrium. Rarely, can be observed unusual manifestations of these tumours. We present a case of intravenous leiomyomatosis in a 52-year-old woman and a case of benign metastasizing leiomyoma in a 45-year-old woman. Intravenous leiomyomatosis is an uncommon condition in which morphologically benign smooth muscle is present within the lumina of the veins. Benign metastasizing leiomyoma is a very rare phenomenon where histologically benign smooth muscle tumours are present at distant sites, particularly in the lungs, in women having histologically benign uterine leiomyoma. The diagnostic features, etiology, histology, therapeutic management and prognosis of these extremely rare conditions are discussed.     

Uterine intravascular leiomyomatosis: an update and report of seven cases

A clinicopathological study of seven cases of intravascular leiomyomatosis (IVL) was performed. The patients' ages ranged from 29 to 80 and all cases were associated with uterine leiomyomas. The intravascular extension involved preferentially uterine vessels, although the adnexa were also affected. Microscopically, the intravascular tumors resembled the primary leiomyomas. Mitotic count was low and never exceeded 3 per 10 high-power fields. Thick venous vessels, sometimes with thrombosis, were a conspicuous marker in intravascular locations. Necrosis and hemorrhage were found in four cases. Epithelioid IVL was found in one instance associated with a lipid cell tumor of the ovary. This series supports, for most cases, the notion of an origin from a preexisting leiomyoma. A review of treatment, prognosis, and histogenesis of IVL is performed.     

子宫平滑肌瘤长入右心腔一例报道

子宫平滑肌瘤是女性生殖器官中最常见的良性肿瘤,进入静脉或淋巴管内称为脉管内平滑肌瘤病,局限于子宫可出现月经量增多、经期延长,肿瘤过大压迫可导致下肢肿胀、下腹部不适,累及髂静脉可引起腹痛,肿瘤向上生长累及下腔静脉、右心房、右心室、肺动脉等可出现呼吸困难、心悸、胸痛、胸闷、晕厥等,甚至可出现突发性大出血及休克。报道1例49岁女性患者因子宫平滑肌瘤长入右心腔导致下肢水肿3年,胸闷、胸痛2个月的病例,入院完善检查后予经腹全子宫+双侧附件+盆腔包块切除术,开胸行下腔静脉、右心房、右心室、肺动脉肿物清除术及房间隔缺损修补术,结合术中及病理结果,确诊为子宫平滑肌瘤、血管内平滑肌瘤。好转出院,随访8个月,肿瘤未复发。子宫平滑肌瘤侵入血管长入右心室达肺动脉者极少,病情严重,早发现、早诊断、早治疗尤为重要。     

伴有心脏受累的静脉内平滑肌瘤病临床分析

目的:探讨侵入心脏的静脉内平滑肌瘤的临床特点和治疗方法。方法:回顾分析北京协和医院1983年1月至2004年10月收治的心脏受累的静脉内平滑肌瘤患者3例的临床资料。结果:3例患者有心慌、胸闷、晕厥或静脉栓塞等症状。影像学检查在右心房或右心室发现瘤栓。其中,1例2年前因子宫肌瘤行子宫切除术,2例合并有盆腔包块。3例均无肺转移,均接受了静脉瘤栓取出术,其中1例残留心房内瘤栓,1例手术前后接受大剂量孕激素治疗。结论:静脉内平滑肌瘤病的发生率低,侵入心脏的病例更为罕见。治疗以手术为主,抗雌激素治疗可能改善预后。     

Increased hyaluronan and CD44 expressions in intravenous leiomyomatosis

BACKGROUND: To determine the influence of hyaluronan and its receptor CD44 in the angiogenesis and invasiveness of intravenous leiomyomatosis (IVL). METHODS: Paraffin-embedded sections from four IVL cases and 10 uterine leiomyoma cases were immunohistochemically stained for CD34, CD44, basic fibroblast growth factor (bFGF), vascular endothelial growth factor, and platelet-derived growth factor and assayed for microvessel densities. Hyaluronan was immunostained by biotinylated hyaluronan-binding peptide and the results were clinically correlated. RESULTS: CD34 labeling showed significantly increased microvessel counts in IVL (156.6+/-3.7), when compared to uterine leiomyomas (61.3+/-27.3; P<0.001). Hyaluronan and its receptor CD44 were prominently expressed in IVL when compared to leiomyomas and associated with an elevation in bFGF expression. CONCLUSIONS: IVL is a highly vascular neoplasm with elevated microvessel counts. The increase of hyaluronan and CD44 expression in IVL suggests that it is highly angiogenic and has an invasive potential. Elevation of hyaluronan may play a possible role in the pathogenesis of IVL.     

Leiomyoma of the umbilical cord artery: A case report

A leiomyoma is a benign tumor originating from non-striated muscle that is typically found in the uterus. Intravenous leiomyomatosis is a rare form found within the veins, usually associated with uterine fibroids, and tends to recur. These masses can spread from the uterus throughout the venous system. A rare case involving a subendothelial leiomyoma found in an umbilical cord artery is presented in this article. A 21-year-old patient presented with symptoms of preterm labor, which resulted in the premature birth of a female below the 10^th percentile for 24-wk gestational age. The newborn died three days later, and microscopic analysis of the umbilical cord revealed occlusion of the artery by nodular structures. The antepartum diagnosis of intravascular leiomyoma was identified by immunohistochemistry showing that approximately 70% of all tumor cells were diffusely positive for smooth muscle markers, including desmin and smooth muscle actin. These findings indicate the possibility of a pathologic association between the umbilical cord leiomyoma, restriction of fetal growth and preterm delivery due to impaired circulation of blood in the umbilical cord.     

Cotyledonoid leiomyoma of the uterus: report of a case.

A 46-year-old woman presented with a pelvic mass. At the time of operation a large, exophytic, multinodular tumor extended into the peritoneal cavity and right broad ligament from a pedunculated attachment to the uterus in the region of the right cornu. On external examination the lesion had the appearance of cotyledonoid dissecting leiomyoma. On microscopic examination bulbous processes were composed of benign smooth muscle arranged in interlacing fascicles or swirls; there was focal hydropic degeneration. Significant nuclear atypia, mitotic activity, and coagulative tumor necrosis were not encountered. No intravascular involvement was present. There was no demonstrable parent leiomyoma or intramural dissecting component, and thus the case differed from previously reported cases of both cotyledonoid dissecting leiomyoma and intramural dissecting leiomyoma. This tumor represents another variation in the group of benign uterine smooth muscle tumors with unusual growth patterns.     

Intraplacental smooth muscle tumor: a case report.

We present the second reported case of a smooth muscle neoplasm involving the placental parenchyma. On gross examination, the tumor easily separated from the uterus and had a whorled cut surface with finger-like extensions into the villous parenchyma, very similar to the previously described case. The differential diagnosis included a primary smooth muscle tumor of the placenta (placental leiomyoma), a primary uterine neoplasm incorporated into the placenta, and a metastatic sarcoma. In this case, the infant was male, and the polymerase chain reaction technique demonstrated the presence of Y chromosome gene in the placental parenchyma and its absence in the placental neoplasm. Thus, this neoplasm, despite its gross appearance of a primary placental tumor, actually represented an incorporated benign uterine leiomyoma.     

A case of cotyledonoid leiomyoma and review of the literature

We report a case of a rare, unusual benign tumor, the cotyledonoid leiomyoma or Sternberg tumor, detected incidentally in a 67-year-old woman who underwent total hysterectomy and bilateral salpingo-oophorectomy because of a persisting left ovarian cyst of 5 cm. This is the 14th case of cotyledonoid leiomyoma and 2nd case which had no intrauterine portion but had extrauterine extensions. At laparotomy, it was detected that a deep red nodular papillary tumor had been dissecting the posterior leaf of the left broad ligament, projecting into the abdominal cavity just near the left cornu of the uterus, and extending in downward direction. The remaining portion was lying between the layers of the left broad ligament and arose from the subserosa of the left side of the uterine isthmus. Although the bizarre and unusual shape was in favor of malignity, the frozen-section examination revealed a benign histology. Although the cotyledonoid leiomyoma is a benign entity, it may suggest a malignant disease owing to its unusual sarcomatoid appearance and its rarity. Therefore, awareness of obstetricians and gynecologists regarding this rare entity will prevent unnecessary anxiety and interventions.     

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.     

Case of uterine leiomyoma showing fludeoxyglucose uptake on F-18 fludeoxyglucose positron emission tomography/computed tomography

In order not to over treat uterine leiomyoma and to avoid overlooking uterine leiomyosarcoma, a highly reliable diagnostic method has been thought. Occasionally, it is difficult to discriminate uterine leiomyoma from uterine leiomyosarcoma. Recently positron emission tomography/computed tomography (PET/CT) has been proved useful in assessing pelvic malignancies. We experienced a case of uterine leiomyoma showing increased F-18 fludeoxyglucose uptake on PET/CT in a postmenopausal woman. However, histological analysis demonstrated benign leiomyoma by the hysterectomy. Immunohistochemical analysis of glucose transporter-1 showed negative in leiomyoma. Our case indicates that uterine leiomyoma in a postmenopausal woman may show false positive result of PET/CT.     

Uterine smooth-muscle tumor of uncertain malignant potential metastasizing to the humerus as a high-grade leiomyosarcoma

BACKGROUND: Smooth-muscle tumors of uncertain malignant potential represent a variant of uterine leiomyoma and remain a dilemma due to their uncertain clinical behavior. Most of these tumors have a benign clinical course, and coagulative tumor cell necrosis has usually been associated with worse outcome. CASE: We present a case of a uterine smooth-muscle tumor (SMT) of uncertain malignant potential without coagulative tumor cell necrosis that has metastasized to the humerus. The metastatic lesion was a high-grade leiomyosarcoma consistent with uterine origin. CONCLUSIONS: Smooth-muscle tumors of uncertain malignant potential that demonstrate a lack of coagulative tumor cell necrosis do not necessarily insure benign clinical outcome. These lesions can recur and metastasize to distant sites, thus requiring long-term follow-up.     

不孕症合并子宫肌瘤的治疗进展

子宫肌瘤是女性生殖系统最常见的良性肿瘤。随着生育年龄的推迟以及中国二胎政策的放开,不少子宫肌瘤患者在就诊时仍有生育要求,但此类患者的最佳治疗方式至今尚无定论。尽管国内外相关共识或指南中对不孕症合并子宫肌瘤的处理给予了一定的建议,但是这些建议也常模糊不清,有时甚至相互矛盾。有指南指出有生育需求的子宫肌瘤患者不推荐孕前行子宫肌瘤剔除术,除非子宫肌瘤曾导致妊娠并发症;有指南认为子宫肌瘤合并不孕就是治疗指征;而另有指南建议对于不孕症合并子宫肌瘤的治疗要根据子宫肌瘤的类型而定,建议对黏膜下肌瘤或引起宫腔形态改变的肌壁间肌瘤行手术治疗,其他部位肌瘤不论其大小均不建议治疗。现结合文献报道及相关指南建议,探讨不孕症合并子宫肌瘤的基本处理原则,为其规范化治疗提供参考。     

Myoma and pregnancy: when medical treatment is not sufficient

The leiomyoma is the most common benign uterine tumor in population of childbearing age women. We report a patient case with uterine myomas threatening the pregnancy continuation and causing a real problem of maternal tolerance. In presence of a 22 cm subserosal fibroma which is responsible of a severe abdominal pain syndrome resistant to level 3 analgesics, the use of surgical treatment was considered, even if current recommendations are for an optimal medical management. Myomectomy by laparotomy has been performed after 13 weeks of gestation.     

子宫肌瘤的非手术治疗

子宫肌瘤是女性生殖器官最常见的良性肿瘤。其治疗包括手术治疗和非手术治疗。介绍了药物治疗,高强度聚焦超声（high intensity focused ultrasound, HIFU）及子宫动脉栓塞（uterine artery embolization, UAE）3种非手术疗法的应用范围及利弊,为子宫肌瘤的非手术治疗方法的选择提供参考。     

子宫肌瘤的非手术治疗

子宫肌瘤是女性生殖器官最常见的良性肿瘤。其治疗包括手术治疗和非手术治疗。介绍了药物治疗,高强度聚焦超声（high intensity focusedultrasound,HIFU）及子宫动脉栓塞（uferine artery embolization,UAE）3种非手术疗法的应用范围及利弊,为子宫肌瘤的非手术治疗方法的选择提供参考。     

Hypercalcemia associated with a uterine leiomyoma: a case report and review of the literature

BACKGROUND: Humoral hypercalcemia is known to be an important cause of hypercalcemia associated with malignancy. Humoral hypercalcemia due to benign conditions has been rarely reported. CASE: We report on a 45-year-old woman with humoral hypercalcemia secondary to uterine leiomyoma. Following the surgical removal of leiomyoma by hysterectomy, serum calcium, suppressed parathyroid hormone (PTH) and elevated parathyroid hormone-related protein (PTH-rP) levels were normalized. CONCLUSIONS: To our knowledge, this is the second reported case of humoral hypercalcemia associated with uterine leiomyoma. We suggest that for women with hypercalcemia who had suppressed PTH and elevated PTH-rP levels, uterine leiomyoma should be included in the differential diagnosis.