Ann Thorac Surg 2004;77:1837-1839
© 2004 The Society of Thoracic Surgeons
Case report
Surgical resection of recurrent bilateral mediastinal liposarcoma through the clamshell approach
Yasuhiko Ohta, MDa*,
Tomomi Murata, MDa,
Masaya Tamura, MDa,
Hideo Sato, MDa,
Hiroshi Kurumaya, MDa,
Kazuyoshi Katayanagi, MDa
a Departments of Thoracic Surgery and Pathology, Ishikawa Prefectural Central Hospital, Kanazawa, Japan
Accepted for publication June 3, 2003.
* Address reprint requests to Dr Ohta, Department of Thoracic Surgery, Ishikawa Prefectural Central Hospital, Kanazawa 920-8530, Japan
e-mail: yohta{at}ipch.jp
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Abstract
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Primary mediastinal liposarcoma is an unusual variant of mediastinal neoplasms. We describe a long-term survivor who underwent repeated operations. After resections through a posterolateral thoracotomy and median sternotomy, a third operation was performed for recurrent bilateral huge tumors through a clamshell incision, and both tumors were removed en bloc. Results of pathologic examination showed that both tumors were well-differentiated liposarcoma. The patient is currently well 16 years after the first operation. Aggressive surgical intervention whenever possible appears to improve the quality of life and prolong the survival of patients with mediastinal liposarcoma.
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Introduction
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Liposarcoma is an unusual variant of mediastinal neoplasms, representing around 1% of all malignancies and 9% of primary sarcomas of the mediastinum [1, 2]. Although surgical resection is thought to be the optimal treatment for this neoplasm, the frequency of local recurrence is high (50% to 90%) [1, 3]. According to the very limited number of case reports [4, 5], some patients with mediastinal liposarcoma appear to benefit from repeated surgical resections to achieve long-term survival. We herein report a long-term survivor who underwent repeated triple operations for resection of mediastinal well-differentiated liposarcoma over about 16 years. At the third operation, recurrent tumors in the left and right sides of the thorax were successfully removed in one stage through the clamshell approach.
A 59-year-old woman underwent the first operation for mediastinal well-differentiated liposarcoma in November 1987. A total of 1,570 g of the tumor including thymic tissue was resected through a left posterolateral thoracotomy. In November 1995, a computed tomographic (CT) scan showed the presence of a recurrent mass lesion anterior to the main pulmonary artery. The tumor, which was 10.0 x 6.0 x 2.0 cm, was removed through a median sternotomy. The results of pathologic examination showed the tumor to be recurrent mediastinal well-differentiated liposarcoma. Follow-up CT scans and simple chest x-ray examinations were alternately performed every 3 months after the operation. In March 1997, a follow-up CT scan showed a mediastinal fatty lesion around the left pericardium. Because of the absence of symptoms, the patient decided not to undergo surgical resection, and outpatient follow-up was continued. In March 1998, a CT scan showed an anterior mediastinal fatty mass lesion also on the right side. In December 2002, the patient began to experience exertional dyspnea and was admitted to the hospital for a third operation. A chest x-ray at this time showed increased density around the bilateral pericardial lesions. Magnetic resonance imaging showed huge soft-tissue densities in the bilateral pleural cavities (Fig 1).
The results of a pulmonary function test showed slight restrictive ventilation impairment. The forced excretory volume in the 1st second was 2.10 L. The results of echocardiography showed that cardiac function, including wall motion, was relatively well preserved. Explorations of other sites, including the brain, bone, and abdomen, showed no abnormalities.
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Fig 1. Magnetic resonance imaging of the chest showing huge soft-tissue densities at the bilateral pleural cavities.
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The third operation was performed in January 2003. The patient was placed in the right hemilateral position, and a double-lumen endotracheal tube was inserted under general anesthesia. The chest was entered through a bilateral thoracosternotomy, the so-called "clamshell" incision, for simultaneous removal of the bilateral huge mediastinal tumors. The skin incision was made along the inframammary crease to the anterior axillary line on the right side and to the posterior axillary line on the left side. A thin capsule surrounded the tumor on the right side with no direct invasion into surrounding tissue such as the pericardium, lung, and chest wall. The tumor on the right side, measuring 12.0 x 7.8 x 4.2 cm (210 g) was removed en bloc. On the left side, the thoracotomy was started with dissection of dense adhesion due to the previous two operations. The tumor was dissected from the lung and pericardium with a cavitron ultrasonic surgical aspirator (CUSA). Because direct invasion into the lung and the posterolateral portion of pericardium was suspected, partial resection of the left lung and pericardium was performed. The defect of the pericardium was reconstructed with Dexon mesh (Sherwood-Davis & Geck, St. Louis, USA). The fatty tissue around the inferior pulmonary veins connected to the main tumor was resected piecemeal with CUSA. The surgically resected main tumor on the left side consisted of a mass of diameter 21.0 x 20.0 x 5.5 cm (922 g). Microscopic pathologic examination showed that both tumors were recurrent well-differentiated liposarcoma (Fig 2).
The mitotic rate was 1 to 2 per 10 high-power fields. There was no change in the number of mitotic figures in the recurrent tumor compared to the original one. The sampled hilar lymph nodes were free of cancer metastasis.
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Fig 2. Histologic section of the resected tumor shows appearance of the well-differentiated liposarcoma with focal lipoblasts. (Hematoxylin & eosin; original magnification, x200.)
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The postoperative course was uneventful and the patient was discharged from hospital 3 weeks after the operation. At the last follow-up examination, 6 months after the operation, the patient was in good health without exertional dyspnea and without any clinical evidence of recurrence.
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Comment
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Pathologic subtypes of liposarcoma generally include multiple variations, ie, well-differentiated, myxoid, pleomorphic, dedifferentiated, and round cell types [1, 6]. Among these, tumors consisting of pleomorphic and round cell types are most aggressive, with a high risk of local recurrence and distant metastasis [7, 8]. Even in patients with well-differentiated type, however, recurrence is common, and some liposarcomas, especially those without an encapsulated structure, carry a risk for repeated relapse after the surgical treatment [1, 4–6]. Because of the rarity of the disease, the effectiveness of chemotherapy for different pathologic subtypes has not been assessed in detail, and induction or adjuvant chemotherapy protocols have not established value for mediastinal liposarcoma. Based on currently available information, radiation also appears ineffective for treating this neoplasm [1]. In patients with retroperitoneal sarcomas, one report concluded that chemotherapy and radiation therapy actually have no effect on survival [9]. At present, therefore, radical surgical resection is believed to be the only curative intervention.
Although previous reports have emphasized the utility of surgical resection even if entire tumor removal is impossible [1, 6], the reported number of patients with mediastinal primary liposarcomas who actually underwent repeated surgical resections is limited, and only a few patients have undergone resections more than three times. In the present case, we selected the clamshell approach for simultaneous removal of the bilateral recurrent tumors at the third operation. Although the tumor on the left side had extended to the posterior mediastinum, the tumor was successfully removed with a relatively good operative view by taking the right hemilateral position with a skin incision to the posterior axillar line.
The most plausible explanation of relapse after resection is the presence of remnant or intrathoracic disseminated tumor cells at the operation. If the boundary between the surface of the tumor and the surrounding soft tissue is not clear, as much adipose tissue around the tumor as possible should also be removed. In this case, the adipose tissue around the pulmonary vessels and pericardium was successfully removed with CUSA.
Interestingly, surgical resection plus radiation therapy was suggested in a previous case report to be effective for treatment of a subset of diffusely infiltrating nonencapsulated mediastinal liposarcomas [6]. Another interesting point was that long-term survival was achieved despite incomplete surgical resection. In our case, radiation therapy was an optional postoperative adjuvant treatment. However, tissue destruction and adhesion caused by radiation therapy generally hinders the surgical procedure. Considering possible future surgical treatment, we did not select adjuvant radiation therapy in this case, although the effectiveness and utility of adjuvant radiation therapy for dense infiltrating mediastinal liposarcoma warrant further study. We believe that this case report provides further evidence that aggressive surgical intervention, if possible, can improve the quality of life and prolong the survival of patients with mediastinal liposarcoma.
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References
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Abstract
Introduction
