Leiomyosarcoma of the inferior vena cava: clinical experience with four cases

Abstract

Background: Leiomyosarcoma of the inferior vena cava is a rare tumor that presents in an insidious manner with non-specific symptoms. Given its rarity, there are no consensus guidelines to its management. The aim of this study was to report the clinical experience in the management of patients presenting to our institution during a 12 year period.

Patients and methods: Four patients with leiomyosarcomas of the inferior vena cava were managed at our institution during the period reviewed. Patient details were identified through a search of the pathology department computerized database, and case notes were retrospectively reviewed to obtain details of presentation and management.

Results: There were 3 females and 1 male with a mean age of 59 years. All tumors were identified within 2 months of first symptoms. Three of the 4 had localized tumors whilst 1 patient had lung metastases at presentation. The three patients with resectable tumors underwent radical surgical excision of the tumor, and two patients had postoperative radiotherapy. One patient died of recurrence at 7 months, and another at 30 months. The third patient is currently well and disease free at 16 months. The fourth patient with metastatic disease was treated with chemotherapy alone and survived 36 months.

Conclusion: Leiomyosarcoma of the inferior vena cava is an uncommon tumor that presents with non-specific symptoms. At the time of presentation, tumors are usually large and resection is challenging but probably offers the best opportunity for long-term survival.

Figure 1

Computerized tomographic scan of patient 1 demonstrating a large tumor.

Figure 2

Gross appearance of the tumor.

Figure 3

Photomicrograph of the edge of the tumour (× 40). (A). The tumour is composed of spindle cells which show nuclear pleomorphism with occasional giant nuclei (Hematoxylin and eosin × 200), (B). Immunohistochemistry for epithelial marker AE1/AE3 (C), and for S100 (D) is negative, but tumor is strongly positive for smooth muscle marker smooth muscle actin(E).