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.     

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.     

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.     

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。     

Isolated cardiac metastasis of cervical carcinoma presenting as disseminated intravascular coagulopathy. A case report

BACKGROUND: An isolated cardiac metastasis from cervical carcinoma is very rare. This report describes the unusual presentation in a patient diagnosed and successfully treated for stage IB squamous cell carcinoma of the cervix, presenting six months later with disease metastatic to the heart. CASE: A 44-year-old woman presented with hand swelling and bruising. She had undergone successful surgical treatment of a stage IB squamous cell cervical carcinoma six months previously. Computed tomography revealed a large mass in the right ventricle, confirmed by echocardiography. The patient underwent surgery, where the mass was biopsied and debulked under a cardiopulmonary bypass. Frozen section confirmed metastatic squamous cell carcinoma. The patient was discharged with follow-up radiation and chemotherapy. CONCLUSION: All women with myocardial abnormalities and a history of squamous cell carcinoma of the cervix should be suspected of developing a myocardial metastasis until proven otherwise.     

Asymptomatic intracardiac metastasis from a low-grade endometrial stromal sarcoma with successful surgical resection

BACKGROUND: The endometrial stromal sarcoma (ESS) is a rare neoplasm of the uterine origin. Intracardiac metastasis from the low-grade ESS is an extremely rare event. A case of a patient who successfully underwent surgical extraction of metastatic tumors of the low-grade ESS in the right ventricle is described in the present report. CASE: A 48-year-old woman was considered recurrence of the low-grade ESS 4 years after the initial operation for this disease. The CT scanning and magnetic resonance imaging demonstrated solid masses in the pelvis, the lungs, the inferior vena cava, and the right ventricle. The chemotherapy was effective against the recurrent tumors except for intracardiac site. Although she had no symptoms of right-sided heart failure, surgical resection of the intracardiac masses was successfully performed. Pathological examination confirmed intracardiac recurrent low-grade ESS. CONCLUSION: Surgical approach to intracardiac metastasis of the low-grade ESS is considered viable because of an excellent long-term prognosis in this disease and the likelihood of fatal heart failure or sudden death in untreated cases.     

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。     

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.     

Cavernous hemangioma of the heart: report of a case

Cavernous hemangioma of the heart is a very rare disease. A 57-year-old male patient was admitted due to frequent onset of dull chest pain, which had been occurring for about 1 year. The pain was not related to exercise and was not relieved with nitroglycerin. On echocardiographic examination, a tumor was shown in the outflow tract of the right ventricle and was confirmed with computer tomography. He underwent open heart surgery for resection of the tumor. After a median sternotomy and opening of the pericardial cavity, a reddish-brown-colored tumor, 3 cm in diameter, was found protruding from the epicardial layer of the right ventricular outflow tract. The tumor involved all layers of the ventricle and could be resected only with the help of the cardiopulmonary bypass technique. The defect in the right ventricle was repaired with a woven dacron patch. The patient recovered without incident after the operation, and experienced no chest pain during 7 months of follow-up. Histology showed it to be a cavernous hemangioma.     

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。     

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.     

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.     

Severe Life-threatening Hemoperitoneum from Posttraumatic Avulsion of a Pedunculated Uterine Leiomyoma

Uterine leiomyomas are common tumors in women, and most of their complications are well known. Rupture of a uterine leiomyoma is an unusual source of severe hemoperitoneum. The cause seems to be spontaneous rupture of a leiomyoma vessel or is secondary to abdominal trauma. Herein, we describe the case of a 46-year-old woman who had a history of uterine myomas and came to the emergency department with acute abdominal pain after she fell while jogging. The patient's hemodynamic parameters were stable. An ultrasound examination and computed tomography scan showed multiple uterine myomas and free fluid in the peritoneum without signs of splenic rupture. Laparoscopy was performed, which revealed active bleeding from the base of a pedunculated myoma. The patient required transfusion of 2 units of packed red blood cells, and a subtotal hysterectomy via laparotomy was performed, with an uneventful postoperative course. Because surgical management is needed in an emergency, physicians should be aware of this rare but severe complication in patients with known uterine myomas.     

Three isolated, large retroperitoneal leiomyomas

Leiomyomas are very rare as retroperitoneal neoplasms. The pathogenesis of retroperitoneal leiomyomas remains obscure. A 42-year-old multiparous woman presented with a large abdominopelvic mass. At surgery, the uterus was displaced laterally to the right and the right kidney was displaced medially by three isolated large retroperitoneal masses. A total abdominal hysterectomy and complete excision of the retroperitoneal masses was performed. The histopathologic findings of the resected three tumors revealed leiomyomas without evidence of malignancy and the remaining surgical specimens were normal. This is the first case of three isolated large retroperitoneal leiomyomas without a uterine leiomyoma. The distribution of retroperitoneal leiomyomas in our case suggests that these may be of primary multifocal origin, rather than metastatic or parasitic.     

Artificial insemination by donor and the view of man

A 36 year old woman with left heart failure and right-sided hemiparesis due to endocarditis of the aortic valve underwent urgent aortic valve replacement during the 24th week of gestation. High-flow high-pressure normothermic perfusion during cardiopulmonary bypass was performed. Peri-operative fetal heart rate and uterine contractions were monitored. Severe fetal heart rate decelerations and loss of variability as well as uterine contractions were observed during surgery. Uterine contractions were treated medically. Pregnancy was carried to term and a healthy baby was delivered vaginally. The effect of the nonpulsating cardiac pump during extracorporeal circulation on the fetus will be discussed. Recommendations for the peri-operative management of the fetal unit are made.     

Cutaneous malignant melanoma metastatic to a uterine leiomyoma: a case report

BACKGROUND: Malignant melanoma is the sixth-most-common cancer in the United States and can spread throughout the body. This report represents the second case of a cutaneous melanoma involving a uterine leiomyoma. CASE: A 49-year-old woman presented with an inguinal mass; biopsy revealed an undifferentiated neoplasm. Further examination and computed tomography revealed an enlarged, fibroid uterus. The patient underwent a total abdominal hysterectomy, bilateral salpingo-oophorectomy and right inguinal lymphadenectomy. Histologic examination of the inguinal lymph node and the leiomyoma revealed metastatic melanoma. CONCLUSION: As the incidence of melanoma increases, metastasis to unusual locations, such as uterine fibroids, probably will be encountered more frequently.     

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.     

Laparoscopic myomectomy in a patient with Mayer-Rokitansky-Kuster-Hauser syndrome

A 36-year-old woman had primary amenorrhea, pelvic pain, Mayer-Rokitansky-Kuster-Hauser syndrome, and an 8.5-cm, solid pelvic mass. The leiomyoma uteri was removed laparoscopically from the vestigial mullerian duct with secondary vaginopoiesis. The patient had a satisfactory clinical outcome. Finding of a leiomyoma in a patient with Rokitansky syndrome is rare. To our knowledge this is the first such case in which the myoma was removed by laparoscopy.     

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.     

Disseminated peritoneal leiomyomatosis coexisting with leiomyoma of the uterine body

The authors present an extremely rare case of disseminated peritoneal leiomyomatosis (DPL) coexisting with leiomyoma of the uterine body in a 32-year-old woman. The patient had undergone a caesarean section 12 years ago and used oral contraceptives since then. Before the present surgery leiomyoma of the left corner of the uterine body was diagnosed. DPL was found on the uterine serous membrane, Douglas’s cavity, vesicouterine recess, the great omentum and abdominal peritoneum. There were no DPL nodules on the surface of the liver, kidneys and bowels. Saving operation was performed and only leiomyoma and the great omentum were resected. Immunohistochemical analysis using smooth muscle actin and HHF-35 antibodies showed the same reactivity of leiomyoma and DPL cells and proved the intra-operative diagnosis. Our case corroborates that DPL may occur in women of reproductive age and in patients who use oral contraceptives over a long span of time. That both leiomyoma and DPL are hormonally dependent could also be proved.