Chondrosarcoma Masquerading as Cardiomyopathy

Ann Thorac Surg 2008;86:297–9

CASE REPORT MULLIGAN ET AL CHONDROSARCOMA PRESENTS AS CARDIOMYOPATHY

References

Post-op follow-up (months) Variable

Pre-op

1

3

9

Pulmonary annulus diameter, mm Pulmonary trunk diameter, mm Pulmonary valve insufficiency, grade Pulmonary transvalvular peak gradient, mm Hg

35 55 III-IV

22 33 I-II

25 33 II

23 32 II

15

20

40

40

a At the 9-month follow-up, the diameters of the pulmonary annulus and trunk, and also the pulmonary insufficiency, maintained largely stable. An unobstructed flow through the pulmonary valve still existed, indicating at the most a tolerable mild pulmonary stenosis.

monary vessel diameters, hemodynamic variables, and the pulmonary valve function are reported in Table 1. In the current 9-month follow-up, the diameters of the pulmonary annulus and trunk, and also the PI, maintained largely constant. Just like in the 3-month followup, an unobstructed flow still exists over the pulmonary valve, indicating not more than a tolerable mild pulmonary stenosis (Table 1).

Comment Even significant PI may be clinically absent for a long period of time before symptoms of RV dilatation and heart failure occur. Most patients with PI have an underlying congenital heart lesion, such as tetralogy of Fallot [2, 3], that was corrected in the past. It is quite common that patients are left with PI after surgical repair of obstructive lesions of the RVOT [2]. Although many patients with significant PI after relief of RVOT obstruction will tolerate this long term, a certain percentage will not. In those cases, the surgical repair consists of replacement of the incompetent pulmonary valve either with a homograft or with a bioprothesis. In a few patients, such as in isolated congenital absence of a single pulmonary valve cusp, the reconstruction of the incompetent pulmonary valve can be performed [4]. In our patient, we favored the reconstruction of the pulmonary valve by bicuspidization instead of replacement with a homograft. Perhaps the reason why this operation was possible was growth of the 2 functional cusps during the 19-year interval since the outflow tract patch procedure. This contrasts with the prior reports of this technique that were mainly applied in congenital forms with a single absent pulmonary valve cusp [1, 5]. The uneventful recovery, satisfactory pulmonary function, and unobstructed flow assessed by echocardiography and angiography postoperatively and in the 3- and 9-month follow-up visits justifies our regimen. The bicuspidization technique could be a satisfying alternative in selective patients presenting with 2 sufficient cusps and a dilated pulmonary valve annulus.

1. Kadri MA, Lazzara RR, McLellan BA, Starr A. Repair of congenital pulmonary incompetence by bicuspidization of the pulmonary valve. Ann Thorac Surg 1997;63:1482–3. 2. Kanter KR, Budde JM, Parks WJ, et al. One hundred pulmonary valve replacements in children after relief of right ventricular outflow tract obstruction. Ann Thorac Surg 2002; 73:1801–1806. 3. Tanabe Y, Takahashi M, Kuwano H, et al. Long-term fate of isolated congenital absent pulmonary valve. Am Heart J 1992;124:526 –9. 4. Sayger P, Lewis M, Arcilla R, Ilbawi M. Isolated congenital absence of a single pulmonary valve cusp. Pediatr Cardiol 2000;21:487–9. 5. Westaby S, Katsumata T. Congenital absence of a single pulmonary valve cusp. Ann Thorac Surg 1997;64:849 –51.

Chondrosarcoma Masquerading as Cardiomyopathy Charles R. Mulligan, Jr, MAJ, MC, Houman Tavaf-Motamen, LTC, MC, Robert Stewart, LTC, MC, and William C. Devries, COL, MC* Division of Cardiothoracic Surgery, Walter Reed Army Medical Center, Washington, DC

A 17-year-old male patient presented to his primary care provider with heart failure symptoms and was transferred to our hospital with the diagnosis of idiopathic cardiomyopathy. His workup identified a large mediastinal mass with right ventricular outflow obstruction, which was resected. The pathology of the mass was a low-grade chondrosarcoma. The patient currently remains disease free at 4 years. (Ann Thorac Surg 2008;86:297–9) © 2008 by The Society of Thoracic Surgeons

C

hondrosarcomas are the most common primary chest wall malignant tumors. They generally present with an enlarging, painful, chest wall mass. We present an unusual case of a chondrosarcoma presenting with symptoms related to cardiac compression. A 17-year-old male patient presented to his primary care provider with a 2-month history of cough, congestion, and a 50-pound weight loss. He also reported dyspnea at rest, 2-pillow orthopnea, and general fatigue. He denied any fever, chills, or night sweats. He denied any chest pain or paroxysmal nocturnal dyspnea. The physical examination revealed bilateral wheezing, distant heart sounds without murmur, and a faint gallop. Accepted for publication Jan 21, 2008. Address correspondence to Dr Mulligan, Division of Cardiothoracic Surgery, Rm 4655, Department of Surgery, Walter Reed Army Medical Center, 6900 Georgia Ave, NW, Washington, DC 20307-5001; e-mail: [email protected]. *The views expressed in this article are the views of the authors and should not be construed as official or as reflecting the policies of the Department, of the Army or Department of Defense.

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Table 1. Preoperative and Postoperative Development of Pulmonary Vessel Diameters and Pulmonary Valve Functiona

© 2008 by The Society of Thoracic Surgeons Published by Elsevier Inc

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CASE REPORT MULLIGAN ET AL CHONDROSARCOMA PRESENTS AS CARDIOMYOPATHY

Ann Thorac Surg 2008;86:297–9

Fig 1. The patient’s chest roentgenogram shows a mediastinal mass masquerading as cardiomegaly.

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A chest roentgenogram revealed cardiomegaly, with no evidence of pleural effusion (Fig 1). An electrocardiogram demonstrated low voltage and a sinus tachycardia. A transthoracic echocardiogram was limited due to poor windows but found a moderately sized pericardial effusion, without evidence of hemodynamic compromise, and a dilated right atrium and ventricle with mild tricuspid regurgitation. A subsequent computed tomography (CT) scan demonstrated a large anterior mediastinal mass compressing the trachea and right ventricular outflow tract (RVOT; Fig 2). In the operating theater, an awake, fiberoptic double-lumen tube was placed, and he underwent an en bloc sternectomy and chest wall resection. The tumor had not invaded the pericardium or the heart. The patient experienced transient right heart dysfunction upon removal of the outflow obstruction, which responded to dobutamine.

Fig 2. A chest computed tomography scan demonstrates extrinsic compression of the right ventricular out flow tract with posterior cardiac displacement.

Fig 3. Pathologic specimen tumor and surrounding chest wall en bloc.

The anterior chest wall was reconstructed with Dualmesh (W. L. Gore & Associates, Flagstaff, AZ) and methylmethacrylate and was covered with pectoralis major flaps. Histopathology revealed a low-grade chondrosarcoma, 17.0 ⫻ 15.0 ⫻ 14.0 cm, without invasion of anterior ribs or sternum (Fig 3). He has been followed up with serial chest CT scans and remains disease free at 4 years.

Comment Chondrosarcoma is the most common primary tumor of the chest wall. It generally presents as a painful, slowly enlarging chest wall mass. Chondrosarcomas rarely present with manifestations of cardiac compression. Castillo and colleagues [1] published the only case report in the English literature of extrinsic cardiac compression from chondrosarcoma in 1966. Although uncommon, other neoplasms can present with symptoms of cardiac compression. Shaver and colleagues [2] presented a patient with Hodgkin disease of the thymus that caused extrinsic compression of the RVOT. Their review of the literature found 7 additional case studies of cardiac compression, with 3 caused by teratomas, 2 by lymphomas, 1 pericardial mesothelioma, and 1 lymphatic cyst [2]. Marshall and colleagues [3] found 35 case studies, predominantly teratomas and Hodgkin disease, causing extrinsic RVOT obstruction. More recent literature examining the benefit of echocardiography in the diagnosis of mediastinal masses revealed additional cases of teratoma and lymphoma causing extrinsic compression of the RVOT [4 – 6]. The management of this and all patients presenting

with tumors compressing the heart requires a thoughtful and multidisciplinary approach from diagnosis, to anesthetic management, to surgical approach and subsequent medical management.

References 1. Del Castillo JJ, Gianfrancesco H, Mannix EP Jr. Pulmonic stenosis due to extrinsic compression by sternal chondrosarcoma. J Thorac Cadiovasc Surg 1966;52:255– 60. 2. Shaver VC, Bailey WR Jr, Marrangoni AG. Acquired pulmonic stenosis due to external cardiac compression. Am J Cardiol 1965;16:256 – 61. 3. Marshall ME, Trump DL. Acquired extrinsic pulmonic stenosis caused by mediastinal tumors. Cancer 1982;49:1496 –9. 4. Canedo MI, Otken L, Stefadouros MA. Echocardiographic features of cardiac compression by a thymoma simulating cardiac tamponade and obstruction of the superior vena cava. Br Heart J 1977;39:1038 – 42. 5. Baduini G, Paolillo V, Di Summa M. Echocardiographic findings in a case of acquired pulmonic stenosis from extrinsic compression by a mediastinal cyst. Chest 1981;80:507–9. 6. Mandysova E, Neuzil P, Niederle P, Belohlavek O, Kozac T, Mandys V. Pulmonary stenosis caused by extrinsic compression of non-Hodgkin’s lymphoma. Echocardiography 2004;21: 564 –7.

Recurrent Myasthenia Gravis Due to a Pleural Implant 3 Years After Radical Thymectomy Stijn R. G. Heyman, MD, Hendrik De Raeve, MD, PhD, Rudy Mercelis, MD, PhD, Christel De Pooter, MD, PhD, and Paul Van Schil, MD, PhD Departments of Thoracic and Vascular Surgery, Pathology, Neurology, Antwerp University Hospital, and Department of Radiotherapy, St. Augustinus Hospital, Antwerp, Belgium

Although recurrence of a thymoma is rare, pleural dissemination or local relapses have been described. We present a patient who underwent complete thymectomy for a thymoma, type AB according to the World Health Organization classification and stage II according to Masaoka, followed by adjuvant radiotherapy. Three years later, a relapse of the myasthenic symptoms occurred. An isolated pleural implant above the left diaphragm was removed by video-assisted thoracoscopy. Pathology confirmed the recurrence of the thymoma. As this is a rare occurrence, no precise therapeutic guidelines exist. In our case, surgical resection of the recurrence with adjuvant immunomodulating therapy for myasthenia provided good results. (Ann Thorac Surg 2008;86:299 –301) © 2008 by The Society of Thoracic Surgeons

A

thymoma is a low-grade malignant tumor that is the most frequent neoplasm in the anterior mediastinum in adults. The most common clinical manifestation

Accepted for publication Jan 10, 2008. Address correspondence to Dr Heyman, Graaf Van Hoornstraat 23, Antwerp, 2000, Belgium; e-mail: [email protected].

© 2008 by The Society of Thoracic Surgeons Published by Elsevier Inc

CASE REPORT HEYMAN ET AL RECURRENT MYASTHENIA GRAVIS

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is myasthenia gravis. Complete radical thymectomy is the treatment of choice. Although recurrences after complete resection are rare, pleural dissemination or local relapses have been described. Treatment of recurrent thymomas is not standardized due to the small number of patients reported in the literature. We present a patient who underwent complete thymectomy for thymoma and was treated for recurrent myasthenia 3 years later. A 40-year-old man presented with ptosis, dysarthria, and difficulties with chewing at the department of neurology in 2004. His medical history revealed urge incontinence, stress-induced hematuria, diabetes mellitus type II, and obesity. Electromyography showed disturbed neuromuscular transmission, and anti-acetylcholine receptor antibodies were elevated at 7.49 nmol/L, confirming the diagnosis of myasthenia gravis. On chest computed tomographic scan a polylobular, soft tissue tumor suggestive of thymoma was found in the anterior mediastinum measuring 60 ⫻ 30 mm. Because of the severity of the myasthenic symptoms, preoperative plasmapheresis was applied. By median sternotomy, a radical thymectomy was performed with partial resection of the pericardium and the left phrenic nerve due to local invasion. The patient recovered well without respiratory problems. Final pathologic diagnosis was mixed thymoma according to the Muller-Hermelink classification, AB thymoma according to the World Health Organization classification and stage II according to Masaoka. Approximately 90% of the tumor cells stained positively for the proliferation marker Ki-67. All removed lymph nodes were tumor free. Because there was a positive anterior section margin, postoperative radiotherapy was indicated. A total dose of 60 Gy was delivered and was given in 30 sessions over a time period of 6 weeks. The patient was further treated with a cholinesterase inhibitor and steroids. Clinical symptoms gradually disappeared and complete remission was obtained without any medication. In April 2007, he was reinvestigated because of recurrent myasthenic symptoms. On computed tomographic (Fig 1) and positron emission tomographic (Fig 2) scans, a pleural nodule was detected originating from the parietal pleura above the left hemidiaphragm. There was a slight uptake of the isotope on the positron emission tomographic scan, but there was no evidence for local recurrence in the mediastinum or distant metastases. The lesion was resected by video-assisted thoracoscopy. No other pleural lesions were found. Pathologic findings were similar to the initial diagnosis with free resection margins. One week postoperatively, an increase in bulbar symptoms with difficulties in speaking and eating were noted, and the steroid dose was increased, which resulted in gradual improvement. Follow-up at 6 months showed some minor remaining difficulties with chewing and 0003-4975/08/$34.00 doi:10.1016/j.athoracsur.2008.01.030

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Ann Thorac Surg 2008;86:299 –301