Intravenous leiomyomatosis

Abstract

Intravenous leiomyomatosis is a rare tumor in which benign smooth muscle cells grow into the pelvic venous channels of female patients. A case of intravenous leiomyomatosis with cardiac extension in a 45-year-old woman is described. The patient was diagnosed with cardiac syncope 3 months after total abdominal hysterectomy and was successfully treated with a two-stage approach consisting of sternotomy followed by laparotomy. The cause, disease, presentation, diagnosis, treatment and recurrence are reviewed.

Leiomyomas beyond the uterus; benign metastasizing leiomyomatosis with paraaortic metastasizing endometriosis and intravenous leiomyomatosis: a case series and review of the literature

“…The true rate of recurrence of completely resected leiomyomatosis is unknown, but regrowth of the tumor has been documented in up to 30 % of patients, from 7 months to 15 years after primary surgery [48]. It may be due to incomplete debulking or microscopic metastases and usually presents with a recurrent pelvic mass or signs of heart failure and venous obstruction [42]. One of the largest series of cases, published by Du et al in 2011 (18 patients), shows a recurrence rate of 16.6 %.…”

“…Diagnosis is usually made in advanced stages, when symptoms are more evident and suggest the need for imaging techniques [44]. Microscopically, the intravascular tumor is variably cellular with spindle-shaped cells that contain elongated nuclei lacking pleomorphism with mitotic figures that are usually fewer than 10/high power field [42]. The mainstay of treatment is surgical including total hysterectomy (TH), bilateral salpingo oophorectomy(BSO), and complete resection of the intravenous and intracardiac extension, if present.…”

“…Successful management relies on total surgical excision. However, tumors recur in as many as 30 % of cases, and long-term follow up imaging at 3-to 6-month intervals is necessary [41,42]. Histologically, it appears as a benign smooth muscle cell tumor growing within the uterine venous system.…”

“…One-or two-stage surgery has been used in cases of thoracic involvement [45,46], requiring a multidisciplinary team to perform the procedure. Given the fact that estrogen and progesterone receptors are present in both the tumor cells and the vessel walls [42], BSO is essential and exogenous estrogens must be avoided to prevent subsequent growth of microscopic or unresected foci of IVL. Gonadotropins releasing hormone (GnRH) analogs and antiestrogen therapy have also been used both to prevent tumor growth and to reduce the tumor mass [47] but no efficacy has been demonstrated to date.…”

Vascular problems of the pelvis

“…These tumors typically occur in patients who are approximately 50 years of age and is diagnosed by CT scan, MRI, and inferior vena cavography. The treatment of choice is surgical resection, with total abdominal hysterectomy and salpingo-oophorectomy [57][58][59].…”