ABSTRACT. Leiomyosarcomas of the ovarian vein are extremely rare tumours. Most tumours are hypervascular and typically have a large avascular centre of necrosis. We experienced a case of a retroperitoneal leiomyosarcoma that arose from the ovarian vein with marked vascular proliferation and dilatation within the tumour. To the best of our knowledge, this is the first case report of a retroperitoneal leiomyosarcoma with an atypical vascular structure in the clinical literature. A leiomyosarcoma is a rare malignant tumour of the smooth muscle that arises in the alimentary tract, retroperitoneum, genitourinary tract or soft tissue. A primary leiomyosarcoma of vascular origin is a rare tumour, which arises most often from the inferior vena cava [1]. A few cases of leiomyosarcoma of the ovarian vein have been reported in previous studies [2][3][4]. Commonly, a retroperitoneal leiomyosarcoma is seen as a large mass containing a prominent cystic necrotic area. Recently, we experienced a case of a retroperitoneal leiomyosarcoma that arose from the right ovarian vein and had multiple intratumoural dilated vessels. In this case report, we describe the imaging findings of this tumour and review the relevant literature.
Case reportA 58-year-old woman visited our hospital because of the presence of a palpable mass in the right lower abdomen. Laboratory findings, including tumour markers, were all normal.Ultrasound of the right lower quadrant showed the presence of a large echogenic, partially cystic mass. A colour Doppler sonogram demonstrated increased blood flow within the mass. A duplex Doppler sonogram also demonstrated increased blood flow and pulsatile venous waveforms within the tumour (Figure 1). Contrastenhanced CT revealed the presence of a 9 cm wellcircumscribed, heterogeneous retroperitoneal mass containing central low attenuation. Characteristically enhancing tortuous tubular structures, thought to be dilated vessels, were seen within the mass (Figure 2a). The vascular structures seemed to be connected to the right ovarian vein, and the upper portion of the right ovarian vein was distended (Figure 2b). The mass seemed separated from the kidney, small bowel and inferior vena cava. The differential diagnoses should include a retroperitoneal mass with a large area of central low attenuation and intratumoural vessels, such as a haemangiopericytoma, angiosarcoma or leiomyosarcoma.The patient underwent resection of the retroperitoneal mass. The mass was attached and was connected to the right ovarian vessels (Figure 3a). There was no definite invasion of the adjacent organs and the mass was found to originate in the right ovarian vein. Upon examination of a gross specimen, abundant large-calibre, thin-walled vascular spaces were found throughout the mass (Figure 3b). A microscopic examination revealed a tumour composed of interlacing fascicles of spindle cells with blunt-ended nuclei. The tumour cells showed moderate to severe nuclear atypia and frequent mitoses. The central portion of the tumour showed necrosis, hyal...