Intravascular leiomyomatosis (A rare case report) Prepared by: Dr. Susan Thabet Resident at KHMC Supervised by: Dr. Maher Maaita

Introduction Intravascular leiomyomatosis (IVL) is a rare benign smooth muscle tumor, growing within systemic veins. It is typically associated with mesenchymal tumors of the uterus. It may occasionally exhibit malignant behavior.

Case 42 years old female, multiparous, previously healthy, presented with history of prolonged heavy periods over the past two years. Pelvic ultrasound complex left adnexal mass with a large uterine fibroid. D&C biopsy no malignant changes. Blood and biochemical markers including tumor markers normal. She underwent TAH + left SO with omental biopsy.

Histopathological Exam. 1- Gross description: the cut surface of the uterus showed a fungating submucosal greyish mass projecting into the endometrial cavity (8x3x2 cm). Multiple nodular tumor growths involving the outer lateral wall of the uterus with extension to the broad ligament. The left parametrium showed dilated vascular spaces containing an intravascular fleshy tumor similar to that in the broad ligament.

Histopathological Exam. 2- Microscopic description: Submucosal uterine leiomyoma + Multiple foci of adenomyosis . Sections from outer myometrial wall, left broad ligament and left parametrial tissue showed widespread intravenous spindle cell tumor growths compatible with benign intravascular leiomyomatosis. Normal left ovary, tube and omentum.

Case cont. One week post operatively the patient developed fever as a result of a pelvic abscess that was drained through a small opening in the vaginal vault and she subsequently improved.

Imaging Studies Echocardiography was normal. Contrast enhanced CT angiography from the chest to the pelvis.

Literature Review Between Jan.2000 and Jan 2015, 52 cases of IVL with CT and MRI description identified. Median age was 44.8 years old. The majority of cases diagnosed intraoperatively. Almost always accompanied by uterine leiomyoma or a history of uterine leiomyoma.

The clinical presentation is unspecific. In the majority of the reviewed cases, the initial pathway of extension for IVL was unilateral through the common iliac veins, while certain cases exhibited intracardiac extension. Fatal intracardiac obstruction may occur.

Diagnosis In addition to symptoms and past medical history, imaging studies are necessary. Ultrasound and echocardiography. Enhanced CT . MRI. PET- CT.

Differential Diagnosis Thrombi within the systemic veins, Leiomyosarcoma arising from the wall of IVC, Right atrial myxoma and Malignant thrombosis with carcinoma, as these entities have similar CT and MRI characteristics to IVL.

Options of Management Operative approach: One stage complete resection. Two stage complete resection. 2. Hormonal therapy: Progesterone. Selective Estrogen Receptor Modulators (SERMs) Gonadotropin-Releasing Hormone (GnRH) Agonists. Aromatase inhibitors.

Follow Up Recurrence of IVL appears to be a rare event when complete resection is thought to have been achieved. Incomplete resection has a recurrence rate of over 30%. The most common imaging modality for follow up in reported papers is CT scan. Complete resection repeated imaging on 6-monthly to yearly basis, and to investigate symptoms is appropriate. Resection is incomplete radiologic follow up should be more frequent.

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