Leiomyomas are benign tumors arising from smooth muscle of the uterus. Intravenous leiomyomatosis is characterized by intraluminal growth of benign smooth muscle into either venous or lymphatic vessels outside the limits of myoma. It commonly extends into the pelvic veins and manifests asworm-like protrusions of tumor emanating from veins at the parametrial margins of hysterectomy specimen. The tumor can cause life-threatening symptoms if it involves inferior vena cava or right atrium. We report a case of intravenous leiomyomatosis of the uterus managed at our institution.Keywords: Intravenous leiomyomatosis, myometrium, uteru

Agarwal, Y

Krishnamurthy, D

Manikyam, UK

Mariyappa, N

Prakar, U

Preeti, K

Nigerian Journal of Surgery

English

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AJOL - African Journals Online

105Nigerian Journal of Surgery Jul-Dec 2012 | Volume 18 | Issue 2Address for correspondence: Dr. Narayanaswamy Mariyappa, Prakruthi, No. 254, 1st Cross, 1st Main, New Extension, Near Doomlight Circle, Kolar - 563 101, Karnataka, India. E-mail: drnswamy12@rediffmail.comIntroductIonLeiomyomas are benign tumors arising from smooth muscle of  the uterus.Although the radiological diagnosis is straight forward in most cases, Leiomyomas can occur in unusual locations or present with unusual growth patterns that make the diagnosis more challenging. Intravenous leiomyomatosis (IVL) is a rare tumour characterized by intraluminal growth of  benign smooth muscle into either venous or lymphatic vessels outside the limits of  myoma.cAse reportA 32-year-old woman was admitted with complaints of  abdominal swelling and pain since 6 months. Pain abdomen was intermittent and confined to lower abdomen. She also had feeling of  incomplete voiding of  urine with overflow and stress incontinence since 3 months. Menstrual cycles were normal and there was no menorrhagia. She has one living child and underwent tubectomy 6 years ago. Abdominal examination revealed irregular, nontender uterine mass of  24 weeks pregnant size. Pelvic ultrasound showed bulky uterus measuring 9×6.8×4.3 cm, with multiple hypoechoic lesions in anterior and posterior myometrium, the largest one measuring 11×8.8×7 cm. Computed tomography (CT) scan was not done due to unavailability of  the facility in the rural set-up as well as the unaffordability of  the patient. As the mass was very big and the patient was symptomatic and had already completed her family, the decision of  total abdominal hysterectomy was taken. Abdomen was opened by midline vertical incision. During surgery, multiple large subserosal, intramural, and submucosal fibroids ranging from 5×4 cm to 20×30 cm were seen. During surgical removal, the tumor showed worm-like projections into the pelvic veins [Figure 1]. A presumptive diagnosis of  intravenous leiomyomatosis was made. Ovaries appeared healthy and were retained. There were no technical difficulties in removing the uterus. The tumor was very vascular; it started bleeding profusely from the cut margins. Bilateral hypogastric artery ligation was done which reduced uterine blood supply and enabled easy removal of  the uterus. Hemostasis was well achieved. The tumor weighed 6.7 kg. The postoperative course Intravenous LeiomyomatosisNarayanaswamy Mariyappa, Uday Kumar Manikyam1, Dinesh Krishnamurthy2, Preeti K, Yamini Agarwal, Prakar UDepartments of Obstetrics and Gynaecology, 1Pathology and 2Anaesthesia, Sri Devaraj Urs Academy of Higher Education and Research Center, Tamaka, Kolar, Karnataka, IndiaAbstrActLeiomyomas are benign tumors arising from smooth muscle of the uterus. Intravenous leiomyomatosis is characterized by intraluminal growth of benign smooth muscle into either venous or lymphatic vessels outside the limits of myoma. It commonly extends into the pelvic veins and manifests as worm-like protrusions of tumor emanating from veins at the parametrial margins of hysterectomy specimen. The tumor can cause life-threatening symptoms if it involves inferior vena cava or right atrium. We report a case of intravenous leiomyomatosis of the uterus managed at our institution.Keywords:  Intravenous leiomyomatosis, myometrium, uterusAccess this article onlineQuick Response Code:Website: www.nigerianjsurg.comDOI:  10.4103/1117-6806.103122cAse RepORtFigure 1: Inravenous leiomyomataMariyappa, et al.: Intravenous leiomyomatosis106Nigerian Journal of SurgeryJul-Dec 2012 | Volume 18 | Issue 2was uneventful and she has been in good health for 16 months after the operation. Microscopic examination demonstrated the tumor cells invaginating the vascular tree with no evidence of  atypia.dIscussIonIntravenous leiomyomatosis was first described by Birch-Hirschfeld in 1896 and defined by Norris and Parmlyin in 1975.The neoplasm may result from the invasion of  myometrial sinuses by either leiomyomatosis cells or proliferating smooth muscle cells, arising directly from the venous wall of  the uterine or pelvic veins.[1]Most of  the patients present in the 5th and 6th decades of  life. Female gonadal steroids play a major role in the pathogenesis as the condition is associated with high estrogen levels.[2]Early diagnosis may be difficult because patients may be asymptomatic despite extensive intravenous extension.[3] Symptoms may be associated with uterine myomas. Although there may be some difficulties in distinguishing such lesions from low-grade sarcomas, they are different histologically from sarcomatosis uteri because the intravenous plug is mainly smooth muscle in origin. The tumor behaves like a benign tumor, and the mitotic index is low with most active lesions showing only one mitosis per 15 high-power fields. Conservative surgery is usually adequate as the intravenous extensions are probably incapable of  independent parasitic existence and remain dormant after removing the uterus. The prognosis is excellent even when tumor is left in pelvic veins. Extension of  benign leiomyomatosis up to vena cava and right atrium has been reported. Several cases requiring open heart surgery to remove the intracardiac thrombosis have been successful without recurrence.Intravenous leiomyomatosis is a rare but an important clinical situation. Knowledge about this condition and its recognition is important to provide the best treatment for the patients.conclusIonProphylactic hypogastric artery ligation is safe and effective to reduce uterine blood supply before attempting hysterectomy for vascular uterine tumors. Though intravenous leiomyomatosis imitates malignant neoplasm regarding its pattern of  growth and extension, it must be differentiated histologically from malignant tumors to avoid overtreatment. Since intravenous leiomyomatosis has a tendency for recurrence, long-term follow-up of  patients is recommended.reFerences1. Yaguchi C, Oi H, Kobayashi H, Miura K, Kanayama N. A case of intravenous leiomyomatosis with high levels of hyaluronan. J Obstet Gynaecol Res 2010;36:454-8.2. Quade BJ, Dal Cin P, Neskey DM, Weremowicz S, Morton CC. Intravenous leiomyomatosis: Molecular and cytogenetic analysis of a case. Mod Pathol 2002;15:351-6.3. Worley MJ Jr, Aelion A, Caputo TA, Kent KC, Salemi A, Krieger KH, et al. Intravenous leiomyomatosis with intracardiac extension: A single-institution experience. Am J Obstet Gynecol 2009;201:574.e1-5.How to cite this article: Mariyappa N, Manikyam UK, Krishnamurthy D, Preeti K, Agarwal Y, Prakar U. Intravenous leiomyomatosis. Niger J Surg 2012;18:105-6.Source of Support: Nil. Conflict of Interest: None declared.

Intravenous Leiomyomatosis

Leiomyomas are benign tumors arising from smooth muscle of the uterus. Intravenous leiomyomatosis is characterized by intraluminal growth of benign smooth muscle into either venous or lymphatic vessels outside the limits of myoma. It commonly extends into the pelvic veins and manifests as worm-like protrusions of tumor emanating from veins at the parametrial margins of hysterectomy specimen. The tumor can cause life-threatening symptoms if it involves inferior vena cava or right atrium. We report a case of intravenous leiomyomatosis of the uterus managed at our institution

Narayanaswamy Mariyappa

Uday Kumar Manikyam

Dinesh Krishnamurthy

K Preeti

Yamini Agarwal

U Prakar

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Intravenous leiomyomatosis

http://dx.doi.org/10.4103/1117-6806.103122

Intravenous Leiomyomatosis

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