Tsukushi, Satoshi MD*; Nishida, Yoshihiro MD*; Sugiura, Hideshi MD†; Nakashima, Hiroatsu MD‡; Ishiguro, Naoki MD*
*Department of Orthopaedic Surgery, Nagoya University Graduate School and School of Medicine; †Department of Orthopaedic Surgery, Aichi Cancer Center Hospital and Research Institute, Nagoya City, Japan; and ‡Department of Orthopaedic Surgery, Aichi Cancer Center Aichi Hospital, Okazaki City, Japan.
Disclosure: No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article.
Address for correspondence: Satoshi Tsukushi, MD, Department of Orthopaedic Surgery, Nagoya University, 65 Tsurumaicho, Showa-ku, Nagoya City 466-8550, Japan. E-mail: firstname.lastname@example.org
Each author certifies that his or her institution has approved the human protocol for this investigation and that all investigations were conducted in conformity with ethical principles of research, and that informed consent was obtained.
Introduction: Soft tissue sarcomas originating from the chest wall include in their definition those originating from the trunk and, in many cases, are analyzed together with retroperitoneal tumors whose clinical results differ, with only a few detailed reports on such tumors strictly limited to the chest wall available. The main purpose of this study was to evaluate the clinical outcomes for patients with chest wall soft tissue sarcomas.
Methods: A retrospective review of 44 surgically treated patients was conducted from 1992 to 2006. The median age of the patients was 51.8 years. The median follow-up time was 56.7 months. Twenty-two sarcomas (50.0%) were high-grade, and 22 (50.0%) were low-grade. Of the 44 patients, 31 (70.1%) had previously undergone unplanned excision elsewhere. Histologic examinations of the surgical margins, and oncological outcome for each patient were collected and analyzed.
Results: Twenty-six (59.1%) patients were continuously free of disease, 12 were alive and currently free of disease, three were alive with metastasis, and three had died. Local recurrence developed in five patients. The overall survival rate at 5 years was 88.5%. Local recurrence-free survival rate at 5 years was 88.5%. Univariate analyses disclosed age (p < 0.05), high-grade malignancy (p < 0.05), and local recurrence (p < 0.05) as independent predictors of death. Histopathologic examination of the specimens showed that two patients had evidence of bone invasion.
Conclusions: The clinical behavior of the chest wall soft tissue sarcomas is similar to that of extremity sarcomas. High-grade tumors are more likely to require a muscle transfer, and it is often very difficult to achieve local control of them in view of their anatomic characteristics. Considering the high local control rate in this study, they are best controlled by adequate wide surgical resection.
Primary soft tissue sarcomas of the chest wall are uncommon. Because of the limited experience accumulated by single institutions with this neoplasm, there are few series of patients described in the literature.
Soft tissue sarcomas originating from the chest wall include in their definition those originating from the trunk and, in many cases, are analyzed together with retroperitoneal tumors whose clinical results differ1–3 with only a few detailed reports on such tumors strictly limited to the chest wall available. Also, their anatomic characteristics sometimes cause particular difficulties in their treatment.
In this study, we investigated the therapeutic results of these tumors, and we also discussed therapeutic strategies and associated problems.
PATIENTS AND METHODS
A retrospective review of 44 patients treated for soft tissue sarcomas originating from the chest wall was conducted by our oncology group from 1992 to 2006. The clinical results were determined, and the pathologic features were evaluated. Computed tomography (CT) scans and magnetic resonance images of the chest and the site of primary tumor were routinely obtained. The maximum tumor diameter was measured on CT scans taken at the time of diagnosis. The follow-up duration was defined from the date of diagnosis to the date of death or the last visit. Patients were followed for at least every 6 months in our outpatient clinic for 10 years postsurgery or until death. Follow-up examination included evaluation of local recurrence and pulmonary metastasis, chest CT, and magnetic resonance imaging (MRI). One pathologist assessed the resected specimens for tumor infiltration of bone (ribs, scapula, and sternum), tumor grade, and microscopic margin.
We define osteosarcoma, Ewing sarcoma, rhabdomyosarcoma, and synovial sarcoma to be absolute indications for neoadjuvant chemotherapy, and malignant fibrous histiocytoma (MFH), liposarcoma, and malignant peripheral nerve sheath tumor to be relative indications in high-grade cases based on factors such as tumor size and site. The surgical margin is planned to be more than 3 cm wide for all soft tissue sarcomas. The indications for thoracotomy including rib or sternum resection were sarcomas located adjacent to the bone, with no intervening normal tissue on MRI and infiltration by the tumor suspected. We use a myocutaneous flap transfer when it is difficult to cover a bone defect or exposed bone. We usually reconstructed the chest wall with polypropylene mesh (Marlex mesh) for defects of more than three ribs. Radical and wide margins were considered adequate, whereas marginal and intralesional margins were considered inadequate. Patients with inadequate surgical margins received postoperative radiotherapy.
The SPSS 11.0 program for Windows was used for statistical analysis. The Kaplan-Meier and log-rank methods were used to draw and evaluate the significance of event-free survival curves. Differences were considered statistically significant when the p value was less than 0.05.
There were 28 men and 16 women ranging in age from 14 to 78 years (mean 52), and the observation period was 3 to 180 months (mean 57). The site of origin was the anterior chest in 25 cases, back in 11, and lateral chest in eight. The maximum tumor diameter measured 1 to 25 cm (mean 7). The pathology diagnosis was MFH in 12 cases, dermatofibrosarcoma protuberance in 12, liposarcoma in nine, synovial sarcoma in two, fibrosarcoma in two, Ewing/primitive neuroectodermal tumor sarcoma in two, extraskeletal osteosarcoma in two, malignant peripheral nerve sheath tumor in two, and leiomyosarcoma in one. Twenty-two cases (50%) had high-grade malignant tumors and 22 (50%) low-grade malignant ones. The most common presenting symptom was chest wall mass without pain in 36 patients (81.8%). Four cases (9.1%) received chemotherapy and 4 (9.1%) postoperative radiotherapy. Flap transfer was required in 20 cases (45%) consisting of latissimus dorsi pedicle flaps in eight cases, rectus abdominis pedicle flaps in two, free lateral thigh flaps in four, free rectus abdominis flaps in two, free latissimus dorsi flap in one, and local flaps in three (Table 1). In eight cases (18%), thoracotomy including rib or sternum resection was required. In 31 cases (70%), unplanned excision had been performed by the previous treating physicians (multiple resections in four cases and recurrence in 10 cases).
Postoperative pathologic evaluation of the resection margin revealed a wide margin in 38 cases, marginal margin in six, and intralesional margin in one. At the final follow-up, the results were as follows: patients continuously free of disease in 26 cases, patients alive with disease in three, patients alive with currently no evidence of disease in 12, and patients dead of disease in three. One-year and 5-year overall survival rates were 95.1% and 88.5%, respectively. Histopathologically, tumor infiltration of bone (ribs, scapula, and sternum) was noted in two cases (4.5%), both of which were recurrent cases. Local recurrence was found in five cases (11%). One-year and 5-year local recurrence-free survival rates were 93.2% and 88.5%, respectively. In four of the 12 MFH cases (33%), local recurrence developed despite the acquisition of an adequate resection margin. Factors significantly affecting life prognosis were age (p < 0.05), high-grade malignancy (p < 0.05), and local recurrence (p < 0.05; Figures 1A, B). Gender, tumor location, tumor size, unplanned excision, and surgical margin were not found to be significant (Table 2).
The chest wall is not a clearly defined anatomic site of origin, and numerous investigators use the definition of “trunk” when analyzing their data on tumors in this region and include the results of deeply situated retroperitoneal tumors, despite the fact that these have clearly different clinical results.1–3 Furthermore, even in studies limited to tumors originating from the chest wall, very often primary bone tumors and metastatic tumors to the chest wall are included and confuse the analysis,4–8 whereas very few reports focusing exclusively on soft tissue tumors originating from the chest wall alone are available.9–12 Most soft tissue sarcomas originating from the chest wall are superficial, and even when highly malignant, their prognosis is favorable, as compared with retroperitoneal tumors, being similar to that of soft tissue sarcomas of the lower extremities. In this study as well, comparable results were obtained, attributable to the influence of local control on outcome.
Reflecting the distinctive limitations of studies on rare cancers, this investigation was retrospective in nature, lacked a control group, and focused on a very heterogeneous group of lesions comprising both high-grade and low-grade malignancies. However, as an analysis of soft tissue sarcomas limited to the chest wall it is a large study and it is of significance in investigating the anatomic characteristics of this site.
The chest wall is a site of predilection for various benign lesions including atheroma and lipoma. Most of these are superficially located, facilitating resection. For this reason, inappropriate resection is frequently resorted to without input from diagnostic imaging.
In cases of low-grade malignancy, the tumor tends to infiltrate limitedly, and appropriate additional wide resection or wide resection of recurrences can provide good local control as well as a favorable life prognosis. Conversely, the development of high-grade malignant tumors such as MFH is not rare either, and cases are occasionally encountered in which repeated recurrences make treatment very difficult.
Some of the relevant anatomic characteristics of the chest wall are listed below. (1) Soft tissue sarcomas originating from the chest wall are superficial and are easily palpable on the body surface, and thus lend themselves to unplanned excision by inexperienced physicians (Figure 2A). (2) Unlike the upper and lower extremities, even when deeply situated, there is no concept of compartment, with the tumor readily infiltrating the intramuscular and subcutaneous layers (Figure 2B). (3) Primary tumors rarely infiltrate the ribs or other bones, whereas in recurrent cases, even in the absence of infiltration, the tumor extends close to the bone in many cases precluding the acquisition of an adequate margin and making chest wall resection necessary. (4) At the time of preoperative planning, artifacts on MRI caused by respiratory movement and beating of the heart make grasping the extent of tumor infiltration difficult (Figure 2C). All these characteristics interfere with the establishment of the resection margin in cases of wide resection. To achieve local control, an adequate resection margin is indispensable, and therefore thoracotomy associated with rib resection or flap transfer for extensive skin defect may be required in such cases.
In conclusion, because the majority of soft tissue sarcomas originating from the chest wall are superficial and their resection is easily achieved, unplanned excision is frequently performed. Due to the anatomic characteristics of the chest wall, it is often difficult to achieve an adequate resection margin in cases of wide resection, but to ensure local control, it is necessary to secure an adequate resection margin and resort to a flap. Especially, in MFH, some cases show repeated recurrence, making therapy very difficult and mandating a well-planned biopsy and ensuring of a safe resection margin at presentation. Moreover, the possible development of a high-grade sarcoma must be recognized and a musculoskeletal oncologist consulted.
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