Pulmonary Stenosis due to Right Ventricular Outflow Tract Metastasis from a Uterine Sarcoma 

MA. Kholeif1, AW. Qubbany1, MT. Mando2 , MB. Satti3

Departments of Medicine1, Radiology2 & Pathology3

King Khalid National Guard Hospital at King Abdul-Aziz Medical City, Jeddah,  Kingdom of Saudi Arabia

Corresponding Author: Mona A. Kholeif MD, FRCP, Consultant Cardiologist Cardiology Section, Department of Medicine, King Khalid National Guard Hospital at King Abdul-Aziz Medical City P.O. Box 9515 Jeddah 21423, Saudi Arabia 

e-mail: kholeifm@ngha.med.sa

 

Keywords

Sarcoma of the Right Ventricle, Uterine Sarcoma, Pulmonary Stenosis, Right Ventricular Outflow Obstruction, Cardiac MRI

 

SUMMARY

A 50 year-old woman presented with dyspnoea and symptoms and signs of systemic venous congestion. Examination revealed murmur of pulmonary stenosis. Echo-Doppler, CT scanning and Magnetic Resonance Imaging showed a large right ventricular tumour obstructing the right ventricular outflow tract. She had a hysterectomy for menorrhagia 9 years previously, and resection of a lump involving the right trapezius a month prior to her deterioration. Pathology demonstrated low-grade endometrial stromal sarcoma. She deteriorated acutely in hospital due to pulmonary embolism from superimposed thrombosis. Anticoagulation, followed by radiotherapy resulted in marked clinical improvement. Two years later, the patient remains well. Cardiac metastasis from ESS is rare. Diagnosis is established by echocardiography, CT and cardiac MRI, which allow structural and functional evaluation of the tumour. MRI seems to be the most useful modality. Treatment is palliative or curative resection. This is the first case report of cardiac ESS showing good response and prolonged survival after radiotherapy. Prophylactic anticoagulation should be considered as pulmonary embolism can cause marked deterioration.

 

CASE REPORT

A 52-year old lady was referred to Cardiology from the Oncology Department complaining of worsening dyspnoea for approximately 6 months. Two months previously she noted ankle swelling, increasing abdominal girth and her dyspnoea deteriorated to NYHA Class III-IV. Onemonth prior to this deterioration she had undergone resection of a soft tissue mass, which had developed over her back – right trapezius muscle - and had been excised elsewhere. Also she had a hysterectomy, nine years previously, when she presented with menorrhagia. She had no medical report but had been informed that pathology showed a low-grade uterine sarcoma.

 

On examination, she was dyspnoeic at rest, tachypnoeic, tachycardic with low-normal blood pressure. Her JVP was raised, she had mild lower limb oedema and mildly tender hepar. It was  noted that her face was swollen. Cardiac auscultation revealed a harsh ejection systolic murmur maximal at the left sternal border over the pulmonary area, consistent with pulmonary stenosis. Chest was clear and a chest x-ray showed no significant abnormality.

 

An ECG showed evidence of right ventricular hypertrophy. A transthoracic echo-Doppler study showed severe Right Ventricular Outflow Tract (RVOT) obstruction by a large homogenuous mass measuring 3.5 x 2.5 cm and creating a gradient of 84mm Hg across the RVOT. The mass was involving an extensive portion of the RV free wall and RVOT.

 

A diagnosis of RVOT tumour, probably metastasis from her previous neoplasm, was provisionally made (Figure 1). CT scanning confirmed the presence of a large mass in the RVOT (Figure 2). While in hospital, her symptoms deteriorated acutely, with a clinical picture consistent with pulmonary embolism (PE). This was confirmed at spiral CT Angiography, which showed a significant filling defect in the left lower lobe pulmonary artery with multiple subsegmental branch filling defects. There was no clinical evidence of deep vein thrombosis and a venogram of leg veins was negative.  The possibility of tumour embolism as well as embolism from a thrombus on top of the tumour was considered, and we opted to fully anticoagulate her with Enoxaparin and Warfarin with improvement in her symptoms. 

Pathology slides from her recently resected trapezius muscle growth were obtained and studied (Figure 3). Slides form her uterine tumour could not be obtained.

 

Review of her sections showed vascularised soft tissue sarcoma formed of spindle and ovoid cells with plump hyperchromatic nuclei, separated by thin vascular channels, showing pleomorphism and infrequent mitosis (5/40 HPF). The cells were immunoreactive to Vimentin, WT1, bcl2 and strongly reactive for Estrogen/Progesterone Receptors, (ER / PR), and were negative for MSA, Desmin, CD34, CD117, CK, EMA and S100.  The features are compatible with metastasis of low-grade endometrial stromal sarcoma (ESS). The case was referred to the regional cardio thoracic surgical center but the patient was reluctant to undergo open-heart surgery.

 

She underwent a course of palliative radiotherapy using parallel opposed pairs of fields giving conformal treatment with 18MV X rays. The radiation dose was 3600cGy given in 12 fractions over 18 days. Concern about the risk of transient oedema post radiotherapy with temporary worsening of the RVOT gradient, and the uncertainty regarding adequate clinical response was explained to the patient. She was placed empirically on dexamethasone during her radiotherapy. The radiation was conformed to the right ventricle with a 2cm margin to allow for the changing position with cardiac contraction. Significant clinical and echo-Doppler improvement and some radiological improvement were noted following this treatment.

 

Sequential echo-Doppler studies demonstrated that the improvement in the gradient across her RVOT, (from 84 mmHg to 51mm Hg), was maximal at 1 month post radiotherapy, even though the tumour size was only slightly reduced. She was discharged home on warfarin. 2 years later she remains well, with mild dyspnoea, NYHA I-II, normal vital signs, and no recurrence of oedema. Her echo-Doppler follow up studies show that, the tumour size is increasing slowly, as is the RVOT gradient.

 

Discussion

Primary cardiac tumors are present in 0.0017 to 0.03 % of autopsy cases. Approximately 75 % of cardiac tumors in adults are benign and the majority are myxomas. Secondary tumours are far more common than primary, afflicting 10 to 25% of patients dying of cancer.1

 

However, cardiac metastases from an endometrial stromal sarcoma (ESS) is an exceedingly rare occurrence with very few cases previously reported.2-5

 

Spread of malignant tumors to the heart is known to occur by way of direct extension, extension along the inferior vena cava, hematogenous spread, lymphatic spread, and intracavitary extension.

 

Clinical presentation depends on the localization of the tumour. Tumours causing RVOT obstruction usually present with progressive dyspnoea, chest pain, arrhythmias and manifestations of systemic venous congestion. Intracardiac metastases are usually discovered during investigations for heart failure, or at autopsy.1-4

 

Diagnosis is usually confirmed by imaging modalities, particularly transthoracic and transoesophageal echocardiography, which are ideal non-invasive tools for the initial diagnosis of intracardiac masses and are of great value in evaluating the haemodynamic consequences. In addition transoesophageal echocardiographic-guided transvenous biopsy appears to be a safe and valuable technique for obtaining tissue samples for histological diagnosis.6 Limitations of ultrasound, however, often necessitate additional imaging modalities to improve preoperative diagnosis. 6-7

 

In a comparison between echo and MRI in the evaluation of cardiac masses, Gulati et al found that MRI is superior to a combination of transthoracic and transoesophageal echo. 7

 

Therefore, CT and MRI play a valuable part in the diagnosis of intracardiac tumors; the high quality images and multiple planes provide more complete characterization of the tumours. 6-7

 

In our patient, echocardiographic diagnosis was followed by CT and MRI studies, which confirmed the extent of the intracardiac and extracardiac tumour and demonstrated the pulmonary embolus. Biopsy and pathology of the trapezius muscle metastasis, was easily accessible and suggested the histological diagnosis. A panel of immunohistochemical stains was used to confirm the diagnosis and differentiate this tumour from smooth muscle tumours and hemangiopericytomas.8

 

Although cardiac metastases were first described in 1700, the first surgical intervention for this rare condition was not performed until the early 1950s.2 Advances in surgical techniques, along with a growing body of experience, have improved the prognosis for patients with cardiac metastases. Treatment however remains difficult and involves palliative or curative surgical resection, chemotherapy and radiotherapy. Of the different modalities, surgical resection seems to offer the best outcome. 1-3, 9-10

The type and extent of surgery for RVOT obstruction ranges from complete resection with reconstruction of the RVOT, to palliative modified Fontan procedure to bypass the right ventricle. The choice will depend on the type and extent of both intracardiac and extracardiac tumour. 9-10

 

A diagnosis of cardiac neoplasm, especially metastatic disease, generally carries a poor prognosis. Kamiya and colleagues9 reviewed the cases of 135 patients who had malignant cardiac tumors; only 3 patients remained alive 4 years after surgery. Most died within 12 months postoperatively. However, prolonged survival, up to 5.6 years, has also been reported. 2

 

Our case demonstrates 2 important management points; firstly, pulmonary embolism, presumably secondary to thrombosis on the tumour may cause catastrophic deterioration of a previously stable patient. Lifelong full anticoagulation should be considered for these patients. Secondly, radiotherapy can provide reasonable palliation for this type of tumour as even small reductions in tumour size can result in a substantial relief of obstruction. Our patient is likely to need further intervention in the future in the form of surgical resection or further palliative radiotherapy.

 

References

  1. Harting MT, Messner GN, Gregoric ID, Frazier OH.  Sarcoma metastatic to the right ventricle. Tex Heart Inst J  2004;31:93-5.1.  

  2. Yokoyama Y, Ono Y, Sakamoto T, Fukuda I, Mizunuma H. Asymptomatic intracardiac metastasis from a low-grade endometrial stromal sarcoma with successful surgical resection. Gynecol Oncol 2004;92:999-1001.

  3. Mikami Y, Demopoulos RI, Boctor F, Febre EF, Harris M, Kronzen I, et al. Low-grade endometrial stromal sarcoma with intracardiac extension. Pathol Res Pract 1999; 195:501-8.

  4. Val-Bernal JF, Hernandez-Nieto E. Symptomatic intracavitary cardiac metastasis from low grade endometrial stromal sarcoma of the uterus. Pathol Res Pract 1999;195:717-22.

  5.  Tabata T, Takeshima N, Hirai Y, Hasumi K. Low-grade endometrial stromal sarcoma with cardiovascular involvement: a report of three cases. Gynecol Oncol 1999;75:495-8.

  6. Lynch M, Clements SD, Shanewise JS, Chen CC, Martin RP. Right-sided cardiac tumors detected by transesophageal echocardiography and its usefulness in differentiating the benign and the malignant ones. Am J Cardiology 1997; 79:781-4.

  7. Gulati G, Sharma S, Kothari SS, Juneja R, Saxena A, Talwar KK. Comparison of Echo and MRI in the imaging evaluation of intra cardiac masses. Cardiovasc Intervent Radiol 2004; 27:459-69.

  8. Bhargava R, Shia J, Hummer AJ, Thaler HT, Tornos C, Soslow RA. Distinction of endometrial stromal sarcoma from hemangiopericytomatous tumours using a panel of immunohistochemical stains. Mod Pathol 2005; 18(1):40-7.

  9. Kamiya H, Yasuda T, Nagamine H, Sakakibara N, Nishida S, Kawasuji M, Watanabe G. Surgical treatment of primary cardiac tumors: 28 years experience in Kanazawa University Hospital. Jpn Circ J 2001; 65:315-9.

  10. Murphy MC, Sweeney MS, Putnam JB J, Walker WE, Frazier OH, Ott DA, Cooley DA. Surgical treatment of cardiac tumors: a 25-year experience. Ann Thorac Surg 1990;49:612-8.

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