Primary Ovarian Leiomyosarcoma: A Review of the Clinical and Immunohistochemical Features of the Rare Tumor

Abstract

After completion of this article, the reader should be able to state how rare primary ovarian leiomyosarcoma (POLMS) is, explain that because of its rarity the best diagnostic and treatment modalities are not conclusive, and recall that the authors reviewed the literature to bring the readership current on POLMS.

Double ovarian tumour in the mare: Case report

“…The present study is the first report of an ovarian leiomyosarcoma in mares. In women, tumours from ovarian smooth muscle are extremely rare, accounting for <1% of the clinical cases (Anderson, Turner, & Benda, 1987;Taşkin, Taşkin, Uzüm, Ataoğlu, & Ortaç, 2007). According to Lerwill et al (Lerwill et al, 2004), the leiomyosarcoma in human ovary can vary in size with the average of 14 cm, present a greyish-white or yellowish colour, solid and fleshy, cystic, with focal haemorrhage and necrosis, which corroborates with the macroscopic evaluation of the neoplasm described by this study.…”

Leiomyosarcoma and Squamous Cell Carcinoma Arising in Mature Cystic Teratoma of the Ovary

“…Leiomyosarcoma of the ovary is a very rare tumor, with approximately 70 cases reported in the English literature [16]. The diagnostic criteria for ovarian leiomyosarcoma are similar to those used for the uterine counterpart [17]. Ovarian leiomyosarcoma typically occurs in postmenopausal women with a mean age of 52.6 years and has an unfavorable prognosis [16].…”

A rare presentation of primary leiomyosarcoma of ovary in a young woman

“…Immunohistochemical markers used in the diagnosis of leiomyosarcoma of the ovary include desmin, vimentin, smooth muscle actin, S-100 [1, 6, 7]. It is also positive for oestrogen, progesterone receptors, p53, and bcl-2 in some cases [1, 7, 8].…”

“…The main treatment modality is surgery which ranges from fertility sparing surgery to debulking surgery consisting of total abdominal hysterectomy, bilateral salpingooopherectomy, and excision of the tumour masses. Cytoreductive surgery can be performed during relapses and prognosis is good if the total tumour surgical resection is possible [1, 6, 9, 7, 10, 8, 11]. …”

“…Even though chemotherapy and radiation therapy have been used in the adjuvant setting they do not provide any additional benefits [12, 13, 7]. The disease is associated with a poor prognosis [10].…”