There are two theories about IVL pathogenesis. The first theory claims that tumor arises from smooth muscle cells in uterine veins. According to the other, it appears as a vascular invasion of a leiomyoma[5].
The patients are generally 23-80 year-old women[6,7]. No correlation can be shown among race, parity and fertility[6,8]. Our case was diagnosed by intraoperative frozen section sampling.
Figure 1: Frozen section of the lesion (HE x100).
Figure 2: Macroscopic appearance of the well-circumscribed, granular, intramural lesion.
Figure 4: Widespread positivity on vascular walls detected with CD-34 antibody staining (CD-34 x40).
Although IVL has benign characteristics, it becomes fatal by growing into right ventricle through inferior vena cava[3,4]. Asymptomatic nodular lung metastases are also important. These lesions are generally detected accidentally by direct lung graphies taken for other reasons[9].
Total abdominal hysterectomy bilateral salphingo-oophorectomy is usually enough for treatment, but in some cases recurrent pelvic and cardiac tumors may occur after hysterectomy[8]. So the patients with IVL should be followed carefully and regularly because of recurrences and risk of tumor emboli. Our patient was diagnosed nearly 6 months ago and still been followed by our Obstetrics and Gynecology Department. Right ventricle failure occurred about 4 months after the operation.
CD-34 immunostaining was performed on sections in our laboratory and widespread positivity on vascular walls were detected (Fig. 4). Immunohistochemical analyses about estrogen and progesterone receptors, vimentin, desmin, smooth muscle actin, CD-10 and h-caldesmon were performed on these lesions by Kir et al. Weak to strong estrogen and progesteron receptor positivity were detected[10]. Because of estrogen and progesteron receptors, anti-estrogenic drugs and GnRH agonists can be used as adjuvant therapeutics in tumors that couldn't be excised totally[11]. Mitsuhashi et al. reported good results with GnRH agonists in residual pelvic lesions[12]. But some studies indicated that GnRH agonists were ineffective in IVL which had atypical histological features[2].
Hyalinization and hydropic degeneration are the most common features with dilated, thick walled veins as seen in our case[9]. Intravascular tumor resembles a typical leiomyoma or it may be a leiomyoma variant cellular, atypical, epithelioid, myxoid characteristics[11]. Differential diagnosis should include typical leiomyoma with artifactual retraction from the surrounding myometrium, leiomyoma with vascular invasion, leiomyoma with perinodular hydropic changes and low grade endometrial stromal sarcoma[11].
As a conclusion; cardiac and pulmonary systems should be examined carefully in patients with IVL. Postoperative follow-up is also important for prevention of unwanted problems.
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