Ann Thorac Surg 2008;86:1008-1011. doi:10.1016/j.athoracsur.2008.02.069
© 2008 The Society of Thoracic Surgeons
Case Reports
Emergency Surgery for Left Atrial Metastatic Alveolar Rhabdomyosarcoma Manifesting as a Right Atrial Mass
Kalyana C. Javangula, FRCS C-Th*,
David J. O'Regan, FRCS C-Th
Division of Cardiothoracic Surgery, Yorkshire Heart Centre, Leeds General Infirmary, Leeds, England
Accepted for publication February 21, 2008.
* Address correspondence to Dr Javangula, Leeds General Infirmary, D Floor, Jubilee Building, Great George St, Leeds, LS1 3EX, England (Email: kalyanachakravarthi{at}hotmail.com).
 |
Abstract
|
|---|
We report the case of a 43-year-old woman with a history of alveolar rhabdomyosarcoma of the forearm, scalp, and paraspinal region who developed acute shortness of breath owing to a tumor arising from the left atrium and extending through the coronary sinus. This was causing partial obstruction across the tricuspid valve as a result of the ball-valve effect. Emergency resection of the atrial mass was performed, and histologic analysis confirmed the presence of metastatic rhabdomyosarcoma. To our knowledge, this is the first reported case of emergency surgical resection of intracardiac metastatic alveolar rhabdomyosarcoma.
|
Introduction
|
|---|
Primary tumors of the heart are rare [1]. At histologic analysis three-fourths of primary heart tumors are found to be benign, and almost half are myxomas [2]. Secondary or metastatic cardiac tumors occur more frequently, with a 100-fold higher incidence compared with primary tumors of the heart [3]. However, intracavitary growth of metastatic tumors is unusual, and the necessity for emergency surgical excision is extremely uncommon. To our knowledge, there is no report of excision of a metastatic alveolar rhabdomyosarcoma in the English literature [4, 5]. We report the case of a woman with a history of alveolar rhabdomyosarcoma and severe acute breathlessness caused by a left atrial mass extending into the right atrium through the coronary sinus, causing obstruction at the tricuspid valve.
A 43-year-old woman was admitted with severe shortness of breath. Three years previously she had undergone marginal excision of a right forearm mass. The histologic diagnosis at that time was alveolar rhabdoyosarcoma. She underwent chemotherapy and radiotherapy. She was well until earlier this year when a metastatic lesion developed on the scalp. Complete excision of the lesion was performed, after which a paraspinal mass (metastatic) developed, for which she was receiving radiotherapy. She was emergently admitted with dyspnea shortly after the course of radiotherapy was completed. The clinical diagnosis was pulmonary embolism. Findings at computed tomographic pulmonary angiography were normal, but an echocardiogram revealed a right atrial mass projecting out of the coronary sinus and prolapsing through the tricuspid valve into the right ventricle (Fig 1). There was evidence of a right-to-left shunt at bubble testing with agitated saline solution. At admission the patient had severe respiratory distress with oxygen saturation of 80% with 10 L of oxygen, heart rate of 120 beats per minute, and blood pressure of 100/60 mm Hg. A decision was made to remove the right atrial mass.
View larger version (65K):
[in this window]
[in a new window]
|
Fig 1. Preoperative echocardiogram shows the right atrial mass arising out of the coronary sinus. (Cs = coronary sinus; Ra = right atrium; RV = right ventricle.)
|
|
Oxygen saturation decreased to 60% with induction of anesthesia. The operative approach was a standard median sternotomy with aortic and bicaval venous cannulation and hypothermic (32°C) cardiopulmonary bypass. The aorta was cross-clamped, and antegrade cold blood cardioplegia was instituted. An oblique right atriotomy revealed a well-encapsulated, solid, 5 x 4-cm mass protruding through the coronary sinus (Fig 2). There was a large ostium secundum atrial septal defect. The coronary sinus was unroofed to trace the proximal extent of the tumor, which was merging into the posterior wall of the left atrium. To achieve near-complete excision, part of the posterior left atrial wall was excised. The left atrium was reconstructed, and the atrial septal defect was closed with a pericardial patch, leaving the spatulated coronary sinus draining into the left atrium. The right atrium was closed. Because radical wide excision of the tumor was not performed, the resected tumor margins were not sent for tumor clearance.
View larger version (85K):
[in this window]
[in a new window]
|
Fig 2. Operative image of the right atrial mass protruding through the coronary sinus. (Cs = coronary sinus; Is = inter atrial septum; Pw = posterior wall; Ra = right atrium; Tu = tumor.)
|
|
Postoperatively there was immediate improvement in oxygen saturation and blood gas levels to the normal range. The patient was symptom-free and had an uneventful recovery. She was subsequently discharged to the care of oncologists. Findings at histopathologic analysis of the resected mass were similar to those of the previously resected tumor in this patient and were consistent with metastatic alveolar rhabdomyosarcoma. The tumor was restaged after 6 weeks with computed tomography and magnetic resonance imaging, which demonstrated a left atrial mass (confirmed at echocardiography) and multiple pelvic metastases. Symptoms recurred. Repeat surgical resection was not offered because of the short time to relapse and systemic disease. Palliative radiotherapy was administered to the pelvis and the left atrium, resulting in improvement in breathlessness. The patient has attended our follow-up clinic for 4 months since emergency resection of the tumor.
|
Comment
|
|---|
Secondary cardiac malignant lesions can be due to either local extension of the adjacent neoplastic processes or metastasis from a distant organ. Signs of cardiac involvement are often overlooked because the symptoms of disseminated tumor disease prevail. Cardiac metastases are usually small and multiple; however, single large tumors are also observed. Although it is assumed that the right side is more frequently involved than the left, in numerous cases left-sided involvement has been found [6, 7]. In descending order of frequency, the pericardium, myocardium, and endocardium are involved [8]. In a selected group of patients with well-controlled primary tumors, resection of the cardiac metastases can improve survival [9, 10].
Most often, cardiac metastases are manifested in patients with advanced tumor disease, with the heart involved in the generalized tumor spread. At this stage of the disease, many patients will already have undergone surgical treatment of the tumor of origin, and radiotherapy, chemotherapy, or both. Cardiac treatment is primarily confined to palliative measures because the prognosis in most cases is poor. Surgical treatment is indicated in exceptional cases of solitary intracavitary metastases, leading to obliteration of the chamber or obstruction of the valve.
There are reports in the literature of emergency cardiac operations performed because of intracardiac metastatic tumors [11, 12]. Our report is unique for various reasons. The alveolar subtype of rhabdomyosarcoma is common in children but relatively uncommon in adults. To our knowledge, there is only 1 report of primary alveolar rhabdomyosarcoma of the heart, in a 12-year-old patient [13], and several isolated reports of primary cardiac rhabdomyosarcoma [14, 15]. In our patient the interesting feature is the intracoronary location of the metastatic tumor. Although the emergency operation was an effective salvage procedure in the short term, the overall prognosis in this patient depends on further systemic therapy.
|
References
|
|---|
- Reynen K. Frequency of primary tumors of the heart Am J Cardiol 1996;77:107-109.[Medline]
- Reyen K. Cardiac myxomas N Eng J Med 1995;333:1610-1617.[Free Full Text]
- Burke A, Viramani R. Tumors of the cardiovascular system. Atlas of Tumor Pathology, third series, fascicle 16Washington, DC: Armed Forces Institute of Pathology; 1996.
- Charney DA, Charney JM, Ghali VS, Teplitz C. Primitive neuroendocrine tumor of the myocardium: a case report, review of the literature, immunohistochemical and ultrastructural study Hum Pathol 1996;27:1365-1369.[Medline]
- Reynen K, Kockeritz U, Strasser RH. Metastases to the heart Ann Oncol 2004;15:375-381.[Free Full Text]
- Benjamin HS. Primary fibromyxoma of the heart Arch Pathol 1939;27:950.
- Berge T, Sievers J. Myocardial metastases: a pathological and elctrocardiographic study Br Heart J 1968;30:383-390.[Free Full Text]
- Lam KY, Dickens P, Chan ACL. Tumors of the heart: a 20-year experience with a review of 12,485 consecutive autopsies Arch Pathol Lab Med 1993;117:1027-1031.[Medline]
- Lagrange JL, Despina P, Spelman M, et al. Cardiac metastases: case report on isolated cardiac metastasis of a myxoid liposarcoma Cancer 1986;58:2333-2337.[Medline]
- Gibbs P, Cebon JS, Calafiore P, Robinson WA. Cardiac metastases from malignant melanoma Cancer 1999;85:78-84.[Medline]
- Naseri E, Eralp B, Oztek I. Emergency management of severe right ventricular inflow obstruction secondary to a metastastic cardiac tumor Ann Thorac Surg 2005;79:709-711.[Abstract/Free Full Text]
- Chu MW, Aboguddah A, Kraus PA, Dewar LR. Urgent heart surgery for an atrial mass: metaststic hepatocellular carcinoma Ann Thorac Surg 2001;72:931-933.[Abstract/Free Full Text]
- Orsmond GS, Knight L, Dehner LP, Nicoloff DM, Nesbitt M, Bessinger FB. Alveolar rhabdomyosarcoma involving the heart: an echocardiographic, angiographic and pathologic study Circulation 1976;54:837-843.[Abstract/Free Full Text]
- Castorino F, Masiello P, Quattrocchi E. Primary cardiac rhabdomyosarcoma of left atrium: an unusual presentation Tex Heart Inst J 2000;27:206-208.[Medline]
- Malyshev M, Safuanov A, Gladyshev I. Primary left atrial leiomyosarcoma: literature review and lessons of a case Asian Cardiovasc Thorac Ann 2006;14:435-440.[Abstract/Free Full Text]
Top
Abstract
Introduction
