Primary soft-tissue sarcomas (STSs) of the chest wall are uncommon. Complete surgical resection remains the mainstay of treatment being sternal resection ocassionally required. We present a 25-year-old man with an anterior chest wall STS. The patient underwent complete oncological resection with reconstruction using titanium bars combined with a free vascularised anterolateral thigh flap. STSs of the chest wall are very rare and they comprise a surgical challenge for both resection and reconstruction.
- cancer intervention
- general surgery
- plastic and reconstructive surgery
- surgical oncology
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Adult soft-tissue sarcomas (STSs) are rare, with an average incidence of 4–5/100 000/year in Europe.1 Wide excision surgery with negative margins is the standard of care and must be carried out by a surgical team trained in this pathology.2 Primary bone/STSs arising in or around the sternum are uncommon and its treatment represents a surgical challenge for both resection and reconstruction owing to the complex anatomy and functional importance of the anterior chest wall.3
We present a case of a STS of the anterior chest wall resected en-bloc together with the manubrium sterni and partial resection of both clavicles and first–second costal arches.
A 25-year-old triathlete man was referred to our surgical department because of a presternal mass recurrence. His medical history was significant for resection of a presternal tumour 12 months before in another centre with histological diagnosis of desmoid tumour. Patient’s physical examination revealed a non-mobile and painless mass in the anterior chest wall (figure 1A and B) with normal neurologic and functional tests. CT scan showed a well-defined hypodense soft-tissue mass measuring 120×90×66 mm. The tumour was in intimate contact with the manubrium sterni and showed extension up to the yugular fossa. The structures of the mediastinum were spared (figure 2A and B). PET-TC was also performed showing a hypermetabolic mass (SUV 7.6) with no systemic disease.
After discussing the case in a multidisciplinary tumour board, multiple CT-guided core biopsies were performed with a 14G needle. Histopathology study of the samples revealed a low-grade sarcoma. Based on this result, the decision was made for complete surgical resection with thoracic reconstruction. The patient underwent en-bloc resection of the tumour together with partial sternectomy and partial resection of both clavicles, partial resection of both pectoralis major muscles and first and second anterior costal arches. Great vessels and other mediastinum structures were not compromised intraoperatively. Finally, the bony chest wall reconstruction was performed using a rigid fixation with titanium bars and were directly covered with a free anterolateral thigh flap using the left internal mammary pedicle (figure 3A and C) also used to reconstruct the soft tissue defect of the thoracic wall. After surgery patient was discharged on postoperative day 7. He developed a left vocal cord paresis (that resolved spontaneously after 3 weeks without any sequel). Final histopathological examination confirmed a low-grade (FNCLCC Grade 1) myofibroblastic sarcoma with deep marginal status (due to close proximity to great chest wall vessels) and a new multidisciplinary evaluation recommended close follow-up without adjuvant treatment. He developed a right supraclavicular recurrence of 1 cm diameter near the surgical scar at postoperative month 18 that was completely resected with wide free margins. After more than 5 years of follow-up, the patient remains alive without evidence of local recurrence or metastatic disease, with no pain and optimal chest wall stability. He has a good quality of life and has returned to competition.
Due to the impossibility to establish a definitive diagnosis on an imaging basis only, pathological assessment remains the gold standard of diagnosis. A percutaneous CT-guided core-needle biopsy was performed and final diagnosis was made with the complete surgical specimen.
Complete surgical resection with negative margins continues to be the cornerstone of the treatment of STSs.
Outcome and follow-up
After 7 years of follow-up, the patient remains alive without evidence of disease recurrence.
Sarcomas represent a rare and heterogeneous group of malignant tumours of mesenchymal origin that comprise only 1% of adult malignancies.4 Approximately 10%–13% of them occur in the trunk, including the chest wall.5 6 For this reason, sarcomas of the chest wall are rare between a rare group of tumours. Moreover, the complexity of their anatomic location and the functional importance of the chest wall pose special surgical challenges for both resection and reconstruction.7 8 Most reports are limited by heterogeneous patients’ cohorts and small sample sizes. They are usually managed using an algorithm similar to that used for extremity sarcomas.
The initial work-up in any case of a superficial truncal sarcoma should be a percutaneous core-needle biopsy to diagnose histology and grade. Additionally, a correct staging of the disease to determine extension of local disease should be carried out. MRI is the preferred image in extremities, pelvis and superficial trunk STS.2 9
Surgical resection with negative margins is the gold standard of treatment in STSs regardless of location.2 9 To achieve this main objective it is often necessary to remove soft tissue and bony chest wall en bloc with the tumour and, occasionally, an extended resection to the clavicle or scapula may be necessary. Hence, this resection often requires complex bony and soft tissue reconstruction. The main purpose of chest wall reconstruction is to re-establish a rigid structure for protection and restore chest wall mechanics in order to avoid paradoxical movement and ensure a normal respiratory function.10 11 The bony component can be reconstructed with a customised prosthesis of polymethyl methacrylate between two layers of polypropylene mesh, also called the ‘sandwich technique’, or by placing a titanium mesh and/or titanium bars.11 12 The use of meshes (preferable non-absorbable) for reconstruction is usually easy to manipulate for the surgeon and well tolerated by the patient giving a right rigidity to abolish paradoxical movement and allowing in-growth of fibrous tissue.11 13 Furthermore, recent articles have shown sternal replacement with cadaveric allografts to be safe and effective, providing optimal rigidity and protection of the chest organs.14 15 Many authors agree that titanium bar system is a better solution in the reconstruction of full thickness defects but is not exempt of complications such as bar fractures or dislocation or pain.12 Bar fractures varied between 0% and 11% in some series.13 For neoplastic diseases, the titanium system should be preferably associated with the use of meshes and/or muscle flaps. Moreover, the soft tissue defect after surgical resection sometimes makes difficult to perform a primary closure. In a recent report by Crowley et al pedicled flaps were required in 53% and free flaps in 17% of patients treated to ensure adequate reconstruction.8 In one of the largest series published, Salo and Tukiainen reported the use of pedicled or free flaps in 83 (70%) of 118 patients who required chest wall reconstruction after tumour resection.16 Although in the majority of cases the reconstruction can be safely performed with pedicled flaps, in the case presented here the use of a free flap was considered the best option because of a partial resection of both internal mammary pedicles and the partial resection of both pectoralis major muscles.
Several retrospective studies have identified prognostic factors that influence survival in the chest wall of patients with STSs. Shewale et al17 in one of the largest series published, stated that complete resection and tumour grade are the most important survival predictors. They achieved a complete resection in 85% of patients and the majority presented with high-grade tumours (63.6%). Nakahashi et al,18 found tumour size (>7 cm) and margin status to be independent predictors of OS. Multidisciplinary experience and training in large chest wall STS resections and reconstructions is crucial in this scenario to give the patient the best chances of recovery and survival.19
The use of neoadjuvant or adjuvant chemotherapy is still controversial in adult STS. Conversely, the use of radiotherapy either in the preoperative or postoperative setting has demonstrated an improvement in local control in patients with extremity STS. Extrapolating these data, radiotherapy is sometimes recommended for patients with large (>5 cm) or high-grade STS.8 Moreover, based on studies in extremity STS, neoadjuvant radiation could be a better strategy by delivering lower doses and hence protecting the underlying viscera or spinal cord despite associated with a higher risk of wound complication.20 Finally, there is no doubt that local recurrence relates to the status of surgical margin after excision. But in addition to that, factors intrinsic to the biology of the tumour and type of excision (ie, marginal resection due to close proximity to great vessels or heart such as the case presented) influence in the likelihood of local recurrence. Marginal margins in certain STSs subtypes are acceptable because of the low risk of recurrence.2 21 But this does not means there is no risk of recurrence and only points out the importance of follow-up.
Management of extremities and superficial trunk STS has evolved with emphasis in limb-preserving or function-preserving surgery. Adopting this multimodality approach as a standard of care, together with consideration of tumour biology as primary determinant of long-term survival, is of utmost importance.
Oncologist and surgeon meet with me every year for my follow-up as a multidisciplinary team to evaluate the complementary tests. The follow-up is very organized and strict and I could return to my daily activities with no problem since the surgery.
Soft-tissue sarcoma (STS) of the chest wall are very rare and they comprise a surgical challenge for both resection and reconstruction.
Surgical planning is critical in STSs surgery; all cases should be discussed in a specialised multidisciplinary tumour board meeting.
The quality of the initial surgery is a major prognostic factor.
Contributors WSN and SDQ involved in study concept and design, acquisition of data and drafting of the manuscript. SDQ, WSN, AB and LP critically revised the manuscript for important intellectual content. SDQ contributed to final version and final approval for publication. All authors read and approved the final version.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.