Uterine leiomyosarcoma: a rare clinical entity

Abstract

Leiomyosarcoma is a rare aggressive malignant mesenchymal tumour, accounting for 1% of all uterine malignancies. It spreads rapidly through the intraperitoneal and haematogenous pathways. It is often diagnosed postoperatively following myomectomy, hysterectomy or supracervical hysterectomy for presumed benign disease. It has a predilection for perimenopausal women with a median age of 50 years. Individuals may describe symptoms of vaginal or abdominal pressure. Physical examination may reveal a large palpable pelvic mass, which may haemorrhage. Surgery remains the mainstay of treatment. Hysterectomy and a bilateral salpingo-oophorectomy may be considered, depending on the individual's menopausal status. Ovarian preservation can be considered in young patients. Adjuvant systemic treatment and radiotherapy are of no benefit. Gemcitabine/docetaxel and doxorubicin have shown benefit in the treatment of advanced or recurrent disease. The authors present the case of a 44-year-old woman with lower abdominal pain, vaginal bleeding and a uterine fibroid. Laboratory investigations confirmed a leucocytosis, neutrophilia and a thrombocythaemia. Further investigation with an MRI pelvis showed a very large, heterogeneous, malignant appearing pelvic mass compressing the right ureter and it appeared uterine in nature. Her staging CT showed multiple lung metastases. The diagnosis of uterine leiomyosarcoma was subsequently established. Due to the aggressive behaviour of this sarcoma subtype, novel early detection strategies and targeted therapies are required.

Keywords: cancer - see oncology; gynecological cancer.

Figure 1

There appeared to be a heterogeneous area within the posterior myometrium measuring 58×51×56 mm suggestive of a subserosal fibroid that appeared to impinge on the endometrium.

Figure 2

The posterior myometrium fibroid measured 71×58×68 mm. The fundal myometrium fibroid measured 64×76×52 mm. The appearances were suggestive of a degenerating fibroid.

Figure 3

Her chest radiograph showed multiple small nodular densities in both of her lungs. The differential diagnosis included multiple pulmonary nodules including tumour deposits, small abscesses and connective tissue diseases. L, left side; PA, posteroanterior.

Figure 4

Further investigation with a MRI pelvis demonstrated a huge heterogeneous mass measuring 21×15×19 cm, centred on and replacing the uterus and cervix was observed.

Figure 5

A staging CT chest, abdomen and pelvis showed a large mediastinal lymph node in the anterior mediastinum (8 mm) and a right pelvic nodal mass lesion (120×65 mm). A mixed solid cystic mass lesion within the uterus was observed.