The patient is a 49-year-old woman with a past medical history significant for uterine fibroids and menorrhagia who presented with a complaint of heavy vaginal bleeding over the previous 8 days, progressive weakness, lightheadedness, palpitations, dyspnea on exertion and lower abdominal pain. She denied chest pain, cough, hemoptysis, fever and sick contacts. Her uterine fibroids were treated in the past by blood transfusion and bilateral uterine artery and left ovarian artery embolization. She was treated for tuberculosis and was hepatitis C positive. She was not allergic to any medications and denied smoking tobacco, consuming alcohol or using intravenous drugs. She was noted to have a hemoglobin level of 5.1 g/dl and received 5 units of packed red blood cells and levonorgestrel. Her shortness of breath improved marginally but she continued to have tachycardia. She was noted to have left lower extremity swelling which the patient admitted had started 4-5 days prior to admission.
A lower extremity Doppler ultrasound revealed extensive deep venous thrombosis and she was started on systemic anticoagulation with dalteparin. Her chest X-ray revealed multiple bilateral pulmonary modules (fig. 1a, b). A chest CT showed saddle embolus and multiple bilateral pulmonary emboli with evidence of right heart strain. Multiple bilateral pulmonary nodules suspicious for metastatic disease and a moderate right-sided pleural effusion and a trace left-sided pleural effusion were also noted (fig. 2). Her physical examination was pertinent for tachycardia, normal breath sounds, distended abdomen with tenderness to palpation in the lower quadrants (uterus was palpable) and bilateral calf muscle swelling with tenderness. She underwent placement of an inferior vena cava filter and an endometrial biopsy. However, the next day morning the patient was noted to become severely hypoxemic with an altered mental state. She was intubated and started on vasopressors for shock.
Due to persistent hypoxemia and hypotension she was taken to the operating room for thrombectomy. Intraoperative transesophageal echocardiography revealed an underfilled left ventricle with interventricular septum flattening, massively dilated right atrium and an extremely dilated right ventricle with reduced function. It also showed a mild-to-moderate pulmonary artery (PA) dilatation with a large mass in the right PA (possibly thrombus) and a severe tricuspid regurgitation with a large pedunculated, very mobile mass attached to the TV leaflets that moved from the right atrium to the right ventricle during the cardiac cycle (fig. 3). Intraoperatively, a large thrombus at the bifurcation of the PA which was extending into the PA branches was removed. The right atrium and the right ventricle were found to be devoid of any clots, but a worm-like mass attached to the anterior TV leaflet was identified and removed. Also a left lower lobe wedge resection was performed on a peripheral nodule and the right pleural effusion was drained.
The patient continued to require a maximal dose of vasopressors and had to be maintained on extracorporal membrane oxygenation and renal replacement therapy. She was successfully extubated on postoperative day 6 and was transferred to the medical floor. Histology of the mass attached to the TV and the pulmonary nodule revealed spindle cells with marked nuclear atypia, frequent mitoses, extensive necrosis and vascular invasion consistent with leiomyosarcoma (fig. 4a). Immunohistochemistry was positive for smooth muscle actin (SMA), desmin, ER, PR and cyclin-dependent kinase inhibitor 2A (p16) (fig. 4b-f). It was negative for myogenin, myogenic differentiation 1 (myoD1) and cytokeratins (AE1/3).
These findings support a diagnosis of leiomyosarcoma and likely represent metastasis from the uterus. The pleural fluid showed rare, isolated highly atypical cells of uncertain origin and scattered mesothelial cells. The endometrial biopsy revealed an early secretory endometrium. It is likely that this patient suffered from a massive pulmonary embolism due to a combination of a predisposed state of hypercoagulation due to malignancy and due to the possible obstructive nature of the mass on the TV. These findings suggest that uterine leiomyosarcoma may manifest itself as a mass on the heart valves, and early identification may help prevent life-threatening complications.
Chest CT with contrast showing pulmonary nodules and a moderate right-sided pleural effusion.