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Description
A 40-year-old woman under investigation for a pelvic mass presented following a syncopal episode. A large fibroid-like pelvic mass and venous thrombus, extending from the left gonadal vein into the inferior vena cava and right atrium, were noted on CT scan (figures 1 and 2). She was referred to the nearest Sarcoma Unit; single-stage excision was planned.
At laparotomy, a 28-week uterus was found. Total abdominal hysterectomy with bilateral salpingo-oophrectomy was performed. The full length of the inferior vena cava and left renal vein were exposed while median sternotomy was undertaken. Following aorto-atrial cannulation, the patient was cooled to 18°C to induce cardioplegia. In the chest, cavotomy was performed inferior to the right atrium. Simultaneously, another cavotomy was performed at the level of the left renal vein. The tumour was thus exposed, it was smooth and non-adherent, allowing it to be easily grasped and delivered through the lower cavotomy. Finally, more distal tumour thrombus, extending down the internal iliac vessel ramifications, was removed via the third, midcaval, incision. Histological analysis revealed uterine leiomyoma with intravenous leiomyomatosis.
Uterine leiomyomatosis is a common condition among women with 30% having extrauterine involvement and of these 10% are intracardiac1 It is benign and characterised histologically by the proliferation of smooth muscle cells arising from either the uterine myoma or the vessel wall. Patients commonly present with gynaecological symptoms such as abnormal uterine bleeding and pelvic pain.2 They may also complain of cardiac symptoms such as syncope, dysponea, chest pain and peripheral odema.
Learning points
Uterine leiomyomatosis with cardiac extension can be suspected in patients presenting with abnormal uterine bleeding, palpable pelvic mass and cardiac symptoms.
Extrauterine leiomyomatosis may be easily mistaken for a malignant condition due to the clinical findings and radiological appearances. It is, however, histologically benign, and radical surgery is therefore justified.
Footnotes
Contributors ATT drafted the article for submission and was involved in the patient's care. AD and DG were the lead surgeons in the case, the former being the admitting consultant. SJF, AD and DG reviewed the draft article and made amendments for submission.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.