Intravenous leiomyomatosis with internal iliac vein extension: Case report Orhan Ünal, Osman Köse, Selçuk Özden, Metin Ercan, Mehmet Sühha Bostancı, Nermin Akdemir, Aslihan Kal Kaymaz, Erdem Varol Sakarya University, Medical Faculty, Department of Obstetrics And Gynecology, Sakarya, Turkey Dr Merve KESKİN PAKER

Intravenous leiomyomatosis (IVL) was first described by Hirschfield and represents a rare clinical entity which could be defined as the extrauterine extension of smooth-muscle cells to the venous system. The primary source of these extensions could be mainly intrauterine leiomyomas. Although histologically benign, IVL may penetrate the inferior vena cava (IVC) reach the right heart chambers which may have catastrophic results. The disease may be localized to the uterine veins alone, and be an incidental finding on a hysterectomy specimen, but it may also be found extending to the parametrial veins at the time of a hysterectomy indicated for other reasons.

CASE REPORT A 28-year-old woman was referred to our clinic with the diagnosis of pelvic mass. Transvaginal ultrasound revealed five leiomyomas uterine of five to twelve cm in diameter.

Laparotomy was performed with the plan of myomectomy. Hysterectomy and bilateral salpengectomy was performed because of atypical presentation of leiomyoma tissues. A diagnosis of IVL was established on her pathology specimen according to tumor was filling the intrauterine veins.

POSTOP PATOLOJİ

CD31 and CD34 immunohistochemical staining (streptavidin-bioptin 40x) for confirmation of vascular vasculature

Further abdominal imaging with CT identified no pathologic findings at common iliac veins, and internal iliac veins. At the follow up, CT identified a distended right internal iliac vein, filled with low-density intraluminal material and a soft tissue density mass in the pelvis at the end of 20th month. Relaparatomy was performed, pelvic mass and, right common iliac vein were excised. Also the diagnosis of IVL was established according to tumor was filling right common iliac vein at pathology specimen

excisional pathological specimen of the intravenous leiomyoma

Intravenous leiomyomatosis (IVL) is a rare benign tumor characterized by the growth of smooth muscle cells within the venous system. It is most commonly seen in premenopausal women with an average age of 44 years. Clinical presentation is initially nonspecific, asymptomatic or related to concomitant processes. IVL is also characterized by a relatively increased possibility of recurrence.

In the majority of cases, only the pelvic veins are involved; however, in rare instances, tumor growth may extend into the IVC. Diagnosis is usually made in advanced stages, when symptoms are more evident. Hysterectomy provides a good opportunity for intraoperative detection of IVL.

Radical excision of IVL is essential for a good prognosis. There is no consensus about optimal timing and technique for follow up. When a recurrence is detected, accurate and radical surgical resection has been shown to improve prognosis significantly. It seems that surgical resection of IVL either in one-step or in multiple-step strategy remains the optimal therapeutic approach regarding a clinical entity with few reported cases in which research about optimal diagnosis and treatment still leaves a lot to be desired. However, usage of selective estrogen receptor modulators has been recently proposed as an alternative approach.

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