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The Thoracic and Cardiovascular Surgeon Official Organ of the German Society for Thoracic and Cardiovascular Surgery
Editor M. Heinemann, Mainz, Germany
Associate Editors A. Beckmann, Berlin, Germany F. Beyersdorf, Freiburg, Germany J. Cremer, Kiel, Germany A. Diegeler, Bad Neustadt/Saale, Germany F.-W. Mohr, Leipzig, Germany J. Rein, Stuttgart, Germany M. Roth (locum), Bad Nauheim, Germany
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Former Editors H. G. Borst, 1978 – 1986 K. Stapenhorst †, 1987 – 1995 W. P. Klövekorn †, 1996 – 2010
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Case Reports/Cardiac
b Introduction
This is a copy of the authorʼs personal reprint
F. Lococo 1, A. Cesario 1, 2, E. Meacci 3, L. M. Vita 1, V. Porziella 1, S. Margaritora 3, P. Granone 1 1 Department of Thoracic Surgery, Catholic University of the Sacred Heart, Rome, Italy 2 IRCCS-San Raffaele La Pisana-Rome, Rome, Italy 3 Department of Thoracic Surgery, Catholic University of the Sacred Heart, Rome, Italy
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Liposarcomas are the second most common soft tissue sarcoma in the adult population and develop from primitive mesenchymal cells [1]. Liposarcomas located in the mediastinum are extremely rare (< 1% of mediastinal tumors). Overall, only about 150 cases have been reported so far in the English literature [1]. Liposarcomas typically grow to a very large size (often invading the pleural space) while remaining asymptomatic. The late clinical syndrome is characterized by respiratory distress, cough, and chest pain, and is mostly due to the direct infiltration and compression of mediastinal structures.
Abstract
Case Description
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Liposarcomas are the second most common soft tissue sarcoma in adults. They occur predominantly in the lower limbs and retroperitoneum, whereas primary mediastinal liposarcomas are extremely rare. Liposarcomas are often asymptomatic and may reach a considerable size before causing any symptoms related to direct invasion or compression of other thoracic organs. We report a case of a 69-year-old woman with a giant primary pericardial liposarcoma causing cardiac tamponade and discuss its clinical and imaging features and surgical treatment and review the literature.
Key words Thoracic surgery · liposarcoma · pericardial tumor
Worsening effort dyspnea was the presenting symptom of a 69year-old woman with an unremarkable recent clinical history. However, careful questioning revealed that symptoms had potentially begun at least one year previously. Admitted to the emergency department, chest X-ray revealed a large mass in the anterior mediastinum. This was confirmed by a CT scan, which showed a large and well-defined mediastinal mass (21 × 16 cm) with a fat-equivalent density (− 89 HU) invading the median and " Fig. 1). posterior regions of the mediastinum (l Fine needle (CT-guided) aspiration cytology (FNAC) was consistent with the diagnosis of liposarcoma: closely packed atypical lipoblasts and fragments of cells embedded in myxoid background material.
Fig. 1 A and B Mediastinal, well-defined mass with fat-equivalent density (dimensions: 21 × 16 cm) invading the median and posterior regions of the mediastinum.
Fig. 2 A and B A Despite preoperative findings, the mass was found to have developed entirely within the pericardial sac. B Mass debulking was performed, and the final pathology confirmed the diagnosis of myxoid liposarcoma.
Lococo F et al. Huge Primary Pericardial …
Thorac Cardiov Surg 2011; 59: 172–183
This is a copy of the authorʼs personal reprint
Huge Primary Pericardial Liposarcoma
Case Reports/Cardiac
b
Dyspnea progressed quickly, and rapid onset of cardiac tamponade made it necessary to perform emergency right-sided tho" Fig. 2 A). It was immediately clear that the mass, deracotomy (l spite the preoperative assessment findings, was not mediastinal but that it had developed entirely within the pericardial sac. This, in fact, was very tense and consistently huge. After incising the pericardium, the tumor appeared free from adhesions throughout its circumference except for a large area infiltrating the left " Fig. 2 B). The final ventricle. Mass debulking was performed (l pathology confirmed the diagnosis of primary pericardial myxoid liposarcoma. The postoperative course was uneventful. Adjuvant chemotherapy was not indicated due to direct involvement of the heart and the known cardiotoxicity pattern of doxorubicin, which would have been the first line and the only potentially effective treatment [2]. Seven months after surgery the patient died from untreatable local disease recurrence.
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received May 21, 2010
Bibliography DOI http://dx.doi.org/10.1055/s-0030-1250187 Thorac Cardiov Surg 2011; 59: 172–173 © Georg Thieme Verlag KG Stuttgart · New York · ISSN 0171-6425 Correspondence Dr. Filippo Lococo Department of Thoracic Surgery Catholic University of the Sacred Heart Largo F. Vito n 1 00168 Rome Italy Phone: + 39 32 94 13 12 02 Fax: + 39 0 63 01 51
[email protected]
This is a copy of the authorʼs personal reprint
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Liposarcomas are common and, regardless of gender, peak in the fifth decade with an age range of 20 to 70 years [3]. However, the anterior mediastinum location is very rare. At CT scan evaluation, the appearance of mediastinal liposarcomas varies from a predominantly fat-containing mass to a solid mass with low attenuation values between − 50 and − 150 HU. MRI represents the gold standard for diagnostic evaluation and preoperative planning due to its superior definition of the tumor invasion of vessels in the mediastinum and the thoracic inlet. In our case, the planned MRI was not performed due to the rapid onset of cardiac tamponade. Radical surgery remains the mainstay of therapy since liposarcomas are generally resistant to chemo- and radiation treatments. Doxorubicin has been weakly validated as the adjuvant treatment of choice [4]. Completeness of surgical resection is the main prognostic factor. Unfortunately, a radical operation is difficult to achieve due to the consistently late diagnosis and the invasiveness of this tumor. Local recurrence, which peaks on average in the sixth postoperative month, is therefore the main cause of death. However, fatal recurrence in the very long term after the initial excision (between 5 and 10 years later) is not uncommon [5]. In conclusion, a pericardial location of a liposarcoma is extremely rare. If possible, complete surgical excision is the treatment of choice. Debulking procedures should be considered only if lifethreatening conditions such as cardiac tamponade occur. Adjuvant treatments fail to control progression of the disease.
References
1 Grobmyer SR, Luther N, Antonescu CR, Singer S, Brennan MF. Multiple primary soft tissue sarcomas. Cancer 2004; 101: 2633–2635 2 Le Cesne A, Judson I, Crowther D et al. Randomized phase III study comparing conventional-dose doxorubicin plus ifosfamide versus highdose doxorubicin plus ifosfamide plus recombinant human granulocytemacrophage colony-stimulating factor in advanced soft tissue sarcomas: a trial of the European Organisation for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group. J Clin Oncol 2000; 18: 2676–2684 3 Caraglia M, Montella M, Addeo R, Costanzo R, Faiola V, Del Prete S, Baldi F, Baldi A, Abbruzzese A, Alloisio M. Mediastinal liposarcoma in a patient with previous testicular cancer. J Clin Oncol 2005; 05: 3844–3846 4 Enzinger FM, Weiss SW. Liposarcoma. In: Enzinger FM, Weiss SW, eds. Soft tissue tumors. 3rd ed. St. Louis: Mosby; 1995: 431–466 5 Mikkilineni RS, Bhat S, Cheng AW, Prevosti LG. Liposarcoma of the posterior mediastinum in a child. Chest 1994; 106: 1288–1289
Lococo F et al. Huge Primary Pericardial …
Thorac Cardiov Surg 2011; 59: 172–183
This is a copy of the authorʼs personal reprint
Discussion
