Clinical features and prognostic factors analysis of intravenous leiomyomatosis

Uterine leiomyomas affect 20%-30% of women older than 35 years. Extrauterine leiomyomas are rarer, and they present a greater diagnostic challenge: These histologically benign tumors, which originate from smooth muscle cells, usually arise in the genitourinary tract (in the vulva, ovaries, urethra, and urinary bladder) but may arise in nearly any anatomic site. In addition, unusual growth patterns may be seen, including benign metastasizing leiomyoma, disseminated peritoneal leiomyomatosis, intravenous leiomyomatosis, parasitic leiomyoma, and retroperitoneal growth. In the presence of such a pattern, a synchronous uterine leiomyoma or a previous hysterectomy for removal of a primary uterine tumor may be indicative of the diagnosis. However, some extrauterine leiomyomas may mimic malignancies, and serious diagnostic errors may result. The most useful modalities for detecting extrauterine leiomyomas are ultrasonography, computed tomography, and magnetic resonance (MR) imaging. The superb contrast resolution and multiplanar capabilities of MR imaging make it particularly valuable for characterizing these tumors, which usually show low signal intensity similar to that of smooth muscle on T2-weighted images. The radiologist's recognition of this and other characteristic features may help steer the clinician toward timely, appropriate management and away from unnecessary, potentially harmful treatment. Show more

Intracardiac leiomyomatosis is rare but has been increasingly reported in recent years. Owing to its rarity, intracardiac leiomyomatosis has been reported only as isolated case reports and case series. This disorder is thought to be underestimated and easily overlooked in the clinic, while it is dangerous owing to the risk of sudden death caused by total outflow tract obstruction. We performed an electronic literature search for intracardiac leiomyomatosis and identified 194 cases that were reported in English from 1974 (the first reported case) to September 2012. Our aim is to provide a detailed and comprehensive review of the clinical presentation, diagnosis, histopathological characterization, treatment and prognosis of this disorder. According to our analysis, intracardiac leiomyomatosis is most common in the fifth decade, and the mean age of detection is ~50 years. Most patients had undergone previous hysterectomy/myomectomy or had a coexisting uterine leiomyoma when admitted. The most common clinical presentations were dyspnoea, syncope, oedema of the lower extremities and palpitation. Transoesophageal echocardiography, computed tomography and magnetic resonance imaging are helpful in the preoperative diagnosis and to guide the surgical management. Complete removal guarantees an excellent outcome, with no recurrence or postoperative death, while incomplete removal leads to recurrence in one-third of patients. Anti-oestrogen therapy is not imperative after incomplete removal owing to its inability to prevent recurrence. Show more

Abstract Intravenous leiomyomatosis (IVL) is a rare benign tumor. The study aimed to assess outcomes of patients treated surgically for IVL. Between November 2002 and January 2015, 76 patients were treated for IVL. The stage of IVL was evaluated preoperatively by echocardiography and enhanced computerized tomography (CT) scan, and graded into 4 stages according to intravascular tumor progression. We recorded age, lower limb edema before surgery, surgical parameters, and hospitalization expenses. Patients were followed up every 6 months and tumor recurrence was assessed by CT and ultrasound. Patients were followed up for a mean of 4.5 ± 2.5 years (range 1–13 years) and there was no operative, hospital, or long-term mortality or were lost to follow-up. The rate of lower extremity edema, amount of blood loss, postoperative transfusion, length of intensive care unit (ICU) stay, postoperative hospitalization, and hospitalization expenses differed significantly between patients at different presurgery stages. Tumors recurred in 4 of 7 patients with stage I IVL that opted for surgery that preserved the ovaries and uterus. No recurrence was observed in patients graded stage II or more, in all of which the uterus and ovaries were removed. Recurrence was observed in only 4 of 76 cases of IVL, all of whom opted for surgery that spared the ovaries and uterus. Different surgical strategies should be decided based on the staging to completely remove the tumor and ensure the safety of patients. Removal of both ovaries is necessary for inhibiting tumor growth and avoiding recurrence. Show more