Thoracic Sarcoma Research Articles

SMARCA4-deficient thoracic sarcoma: a distinctive clinicopathological entity with undifferentiated rhabdoid morphology and aggressive behavior

A distinct subset of thoracic sarcomas with undifferentiated rhabdoid morphology and SMARCA4 inactivation has recently been described, and potential targeted therapy for SMARC-deficient tumors is emerging. We sought to validate the clinicopathological features of SMARCA4-deficient thoracic sarcomas. Clinicopathological information was gathered for 40 undifferentiated thoracic tumors with rhabdoid morphology (mediastinum (n=18), lung (n=14), pleura (n=8)). Thymic carcinomas (n=11) were used as a comparison group. Immunohistochemistry included BRG1 (SMARCA4), BRM (SMARCA2), INI-1 (SMARCB1), pan-cytokeratin, desmin, NUT, S-100 protein, TTF1, CD34, and SOX2. BRG1 loss was present in 12 of 40 rhabdoid thoracic tumors (30%): 7 of 18 in mediastinum (39%), 2 of 8 in pleura (25%), and 3 of 14 in lung (21%). All BRG1-deficient tumors tested for BRM (n=8) showed concomitant loss. All thymic carcinomas showed retained BRG1 and INI-1. Morphologically, tumors with BRG1 loss showed sheets of monotonous ovoid cells with indistinct cell borders, abundant eosinophilic cytoplasm, and prominent nucleoli. Scattered areas with rhabdoid morphology (ie, eccentric nuclei, dense eosinophilic cytoplasm, discohesion) were present in all the cases. SMARCA4/BRG1-deficient sarcomas showed rare cells positive for cytokeratin in 10 cases (83%). One showed rare TTF1-positive cells. All were negative for desmin, NUT, and S-100 protein. CD34 was positive in three of five (60%) BRG1-deficient tumors tested. SOX2 was positive in all four BRG1-deficient tumors tested, and negative in all seven tested cases with retained BRG1. SMARCA4/BRG1-deficient sarcomas occurred at median age of 59 years (range 44–76) with male predominance (9:3) and had worse 2-year survival compared with BRG1-retained tumors (12.5% vs 64.4%, P=0.02). SMARCA4-deficient thoracic sarcomas can be identified based on their distinctive high-grade rhabdoid morphology, and the diagnosis can be confirmed by immunohistochemistry. Identification of these tumors is clinically relevant due to their aggressive behavior, poor prognosis, and potential targeted therapy.

Clinicopathological and molecular characterization of SMARCA4-deficient thoracic sarcomas with comparison to potentially related entities

A growing number of studies suggest critical tumor suppressor roles of the SWI/SNF chromatin remodeling complex in a variety of human cancers. The recent discovery of SMARCA4-deficient thoracic sarcomas has added to the list of tumor groups with the SMARCA4 inactivating mutation. To better characterize these tumors and establish their nosological status, we undertook a clinicopathological and molecular analysis of 12 SMARCA4-deficient thoracic sarcomas and compared them with three potentially related disease entities. Eleven men and one woman with SMARCA4-deficient thoracic sarcomas (aged 27–82 years, median 39 years) were included in the study. Most of the patients had heavy smoking exposure and pulmonary emphysema/bullae. The primary tumors were large and involved the thoracic region in all cases and simultaneously affected the abdominal cavity in 3 cases. The patients followed a rapid course, with a median survival of 7 months. Histologically, all tumors showed diffuse sheets of mildly dyscohesive, relatively monotonous, and undifferentiated epithelioid cells with prominent nucleoli. Immunohistochemically, all tumors demonstrated a complete absence (8 cases) or diffuse severe reduction (4 cases) of SMARCA4 expression. Cytokeratin, CD34, SOX2, SALL4, and p53 were expressed in 6/12, 10/12, 10/12, 10/12, and 7/10 cases, respectively. SMARCA2 expression was deficient in 11/12 cases, and none (0/8) expressed claudin-4. Targeted sequencing was performed in 5 cases and demonstrated the inactivating SMARCA4 mutation in each case and uncovered alterations in TP53 (5/5), NF1 (2/5), CDKN2A (2/5), KRAS (1/5), and KEAP1 (1/5), among others. Comparative analysis supported the distinctiveness of SMARCA4-deficient thoracic sarcomas as they were distinguishable from 13 malignant rhabdoid tumors, 15 epithelioid sarcomas, and 12 SMARCA4-deficient lung carcinomas based on clinicopathological and immunohistochemical grounds. SMARCA4-deficient thoracic sarcomas constitute a unique, highly lethal entity that requires full recognition and differentiation from other epithelioid malignancies involving the thoracic region.

Asbestos-related lung cancers: A retrospective clinical and pathological study.

Exposure to asbestos results in serious risks of developing mesothelioma and lung cancer. The link between asbestos exposure and lung carcinoma is well established. Nevertheless, precise histopathological data are poorly considered when investigating the asbestos-cancer link in a compensatory approach. In the present study, we aim to describe the features of individuals with compensated lung cancer who were referred to an occupational disease center, regarding occupational exposure to asbestos, smoking history and pathological data. We led a retrospective study of compensated ARLC cases seen in our occupational disease center between 2003 and 2013. A total of 146 men were included (mean age at diagnosis, 63.2 years) of whom approximately 90% were heavy current or former smokers (mean value, 30.4 packs/year). The major industries associated with the lung cancer cases were shipbuilding (69.9%), and building construction (7.5%) in this harbor region. The results of the present study showed that lung upper lobe was most prevalent (61.6%) and an excess of adenocarcinoma was found (45.9%), followed by squamous cell carcinoma (38.4%) as well as thoracic sarcomas (2.1%). Neoplasm was not histologically proven in 6.8% of the cases. Subsequent pathology examinations also reclassified 2 tumors as metastases from esophageal and laryngeal origins. In conclusion, smoking prevention should be encouraged in asbestos-exposed workers as reflected by the number of smokers with asbestos-related lung cancer. Thus, histological data should be considered further to evaluate the potent relationship between asbestos exposure and lung malignancy, especially in a compensatory approach.

P3.04-037 Rare Giant Malignant Tumors of the Chest: Reconstructive Challenge: Topic: Miscellaneous II

In this article, we would like to present an unusual primary malignant tumors of the thorax. Case history and radiological studies of 4 patients with histologic diagnosis of thoracic sarcomas were interpreted retrospectively. Tumors originated from the chest wall, mediastinum, and pulmonary parenchyma. Histopathologic diagnoses were: thymo-liposarcoma, leiomyosarcoma, malignant Schwannoma and malignant mesothelioma – sarcomatoid type. In order to evaluate thoracic sarcomas, cross-sectional methods such as CT and MRI can be useful in demonstrating the origin of the mass, relationship with and involvement of adjacent structures. Chest wall resections are associated with significant morbidity, with respiratory failure in as many as 27% of patients. We hypothesized that our selective use of Dual-mesh prosthesis and STRATOS System (Strasbourg Thoracic Osteosyntheses) for chest wall reconstruction reduces respiratory complications. The records of all patients with giant tumors undergoing chest wall resection and reconstruction were reviewed. Patient demographics, use of preoperative therapy, the location and size of the chest wall defect, performance of lung resection if any, the type of prosthesis, and postoperative complications were recorded. From February 2009 to January 2014, 4 patients underwent surgical treatment and chest wall resection for giant chest tumor. The median defect size was 80 cm2 and the median number of ribs resected was 3. lung resection was performed in 3. Prosthetic reconstruction was done with use of dual and polypropylene mesh and STRATOS system. Postoperatively, 1 patients died (had pneumonectomy plus chest wall resection. Respiratory failure did not occurred. Sarcomas are rare tumors of the thorax. They can originate from the lung parenchyma, mediastinum, pleura, or the chest wall. Angiosarcoma, leiomyosarcoma, rhabdomyosarcoma, and mesothelioma (sarcomatoid variant) are the most commonly encountered histopathological types. The diagnosis is established after making the differential diagnosis for histopathologically sarcoma-like malignancies (sarcomatoid carcinomas) and metastatic disease. Radiologically, these lesions usually appear as large heterogeneous masses. The use of malleable Titanium bars to restore anatomic rib continuity helps to preserve the mechanics of ventilation better than a soft patch repair. We have no incidence of respiratory failure. Pneumonectomy plus chest wall resection should be performed only in highly selected patients.

P3.01-025 Primary Pulmonary Sarcomas: An Entity Lost in Misdiagnosis: Topic: Morphology

Primary pulmonary sarcomas are very rare with an incidence rate of <0.5% of all lung malignancies. Their low incidence has impeded comprehensive evaluation of their association with smoking, definitive diagnostic and treatment-regimes. They are often misdiagnosed, both on radiology as well as on fine-needle-aspirate/small-biopsies. We present a series of primary pulmonary sarcomas diagnosed over the last two and a half years. All cases of primary pulmonary sarcomas (2014-2016) were retrieved and reviewed. A total of 21 sarcomas were identified. The most common was synovial sarcoma. Four exceptionally rare cases included pulmonary-artery intimal sarcoma, primary pulmonary myxoid sarcoma, malignant peripheral nerve-sheath tumor and follicular dendritic-cell sarcoma. The clinical and pathology details of which are provided in table1. The patients were distributed over a wide-age range (range: 9-65 years, median: 34 years) with a male-preponderance (M:F=2.2:1). Radiological features were non-specific except in case1 (table1). Histopathology revealed spindle-cell tumor in all cases (figure1) and an extensive immunohistochemical-panel and cytogenetic testing was required to clinch the diagnosis.View Large Image Figure ViewerDownload Hi-res image Download (PPT) This is a series of primary thoracic sarcomas with a highlight on four extremely rare cases which bring to light their unique clinical, radiological, histopathological and immunohistochemical findings. Awareness of such entities is essential for proper diagnosis, appropriate molecular-testing and treatment.

Rare giant malignant tumors of the chest - reconstructive challenge

In this article, we would like to present an unusual primary malignant tumors of the thorax. Case history and radiological studies of 4 patients with histologic diagnosis of thoracic sarcomas were interpreted retrospectively.

SMARCA4 inactivation defines a group of undifferentiated thoracic malignancies transcriptionally related to BAF-deficient sarcomas

While investigating cohorts of unclassified sarcomas by RNA sequencing, we identified 19 cases with inactivation of SMARCA4, which encodes an ATPase subunit of BAF chromatin-remodeling complexes. Clinically, the cases were all strikingly similar, presenting as compressive mediastino-pulmonary masses in 30- to 35-year-old adults with a median survival time of 7 months. To help define the nosological relationships of these tumors, we compared their transcriptomic profiles with those of SMARCA4-mutated small-cell carcinomas of the ovary, hypercalcemic type (SCCOHTs), SMARCB1-inactivated malignant rhabdoid tumors (MRTs) and lung carcinomas (of which 10% display SMARCA4 mutations). Gene profiling analyses demonstrated that these tumors were distinct from lung carcinomas but related to MRTs and SCCOHTs. Transcriptome analyses, further validated by immunohistochemistry, highlighted strong expression of SOX2, a marker that supports the differential diagnosis of these tumors from SMARCA4-deficient lung carcinomas. The prospective recruitment of cases confirmed this new category of 'SMARCA4-deficient thoracic sarcomas' as readily recognizable in clinical practice, providing opportunities to tailor their therapeutic management.

Thoracopleuropneumonectomy with Riblike Reconstruction for Recurrent Thoracic Sarcomas

Large intrathoracic tumors may occasionally present with massive infiltration of the lung and chest wall that would require pneumonectomy and total removal of the rib cage to obtain radical surgical excision, but this operation carries a prohibitive risk of death for cardiopulmonary failure in the absence of adequate chest wall reconstruction. We report here four consecutive cases of thoracopleuropneumonectomy (TPP) with en-bloc resection of the entire lung, chest wall, and diaphragm and immediate riblike reconstruction for recurrent thoracic sarcomas. Patients had undergone the initial thoracic surgical resection with curative intent 2-14 years before TPP. There was no postoperative mortality, and all patients were alive and free of disease 8-21 months after TPP. To our knowledge, this is the first report in the medical literature of such an extensive operation, demonstrating technical feasibility, tolerability, and efficacy of one-stage resection and reconstruction by a semirigid three-dimensional riblike prosthesis modeled on a human-derived aluminum cast. Selected patients with advanced low-intermediate thoracic sarcomas are the ideal candidates for this extreme procedure, to maximize the chance of long-term tumor control and possibly cure.

Invited Commentary on “Thoraco-pleuro-pneumonectomy (TPP) with Riblike Reconstruction for Recurrent Thoracic Sarcomas” by A. Gronchi et al., (ASO-2013-09-1533.R1)

The authors’ solution for recurrent advanced low-intermediate grade thoracic sarcomas is interesting, promising, and unique. As stated, there are no other reported interventions of this scale. The organic shape and fluid kinetics of the thorax are critical to its function. Previous attempts at lesser resections of the chest wall have been successful when combined with reconstruction. For the reconstruction, surgeons have used muscle, autologous bone, biologic mesh, synthetic mesh, and synthetic sternal shields to try and attain the perfect balance of rigidity and pliability to most accurately reflect normal chest physiology. This paper increases the definition of resectability and expands the methods or reconstruction and hence the boundaries of potential cure for large low-grade chest wall sarcomas. Soft tissue sarcomas are rare malignant tumors whose benign counterparts are 100 times more common. They occur in every anatomic location, but are predominantly found in extremities and the retroperitoneum. Approximately 10 % of these tumors are found in the trunk, making the chest wall one of the least common locations. As shown in the NCCN guidelines, the main decision point in the treatment algorithm for sarcomas is resectable versus nonresectable. The resection of the hemithorax in this paper was extensive and thorough, but the resection would have been in vain if not for the unique reconstruction that followed. Chest wall reconstruction is performed for four reasons: malignancy, radiation injuries, infection, and trauma. Reconstruction should always be functional and has been described in the literature. There are biologic materials and synthetic meshes on the market today of all sizes and pliability, yet these are all less than ideal for such a largescale resection. The authors used riblike reconstruction, which mimics the 3-dimensional attributes of the human rib cage. This may provide more functionality than its mesh predecessors, and it aesthetically looks natural as the authors have demonstrated. As with any large resection with foreign material implantation, there are some concerns: the scope of the resection is extensive, the technology of reconstruction is not common, and a risk of infection is real. In spite of this, there exists no good alternative therapy. The large, lowgrade sarcomas are unlikely to be cured by chemotherapy and/or radiation therapy. The authors have demonstrated that immediate riblike reconstruction is a valid option for this select group of individuals within an experienced center. While this procedure is currently being performed in one center, it would seem reasonable to expand its use to experienced hands in high-volume tertiary thoracic centers. Currently, its effectiveness is only reaching a small fraction of the patient population who may benefit. With any multistep, innovative operation there will be a steep learning curve. With appropriate mentoring and a structured program of education, it is possible to effectively reduce the learning curve of complex thoracic operations. The follow-up by this group does show promising results in terms of short-term survival, infection rate, and graft durability. With further investigation, this operation may become a more common option for the patient with a first time diagnosis of large low-grade chest wall sarcoma. Recurrence is treated with a similar algorithm as the initial tumor as seen again in the NCCN guidelines. It is Society of Surgical Oncology 2014

EComment. Soft-tissue sarcomas in a ‘nutshell’

We read this interesting retrospective study by Luo et al. [1] and we would like to add a comment on soft-tissue sarcomas. Soft-tissue sarcomas are a group of malignant neoplasms originating from mesenchymal tissue and deriving etymologically from the Greek word 'σάρξ' (flesh) and the suffix 'ώμα' (tumour). They make up less than 2% and 10–15% of all adult and paediatric malignant neoplasms respectively and their overall incidence is approximately 6 per 100 000 persons/year [2]. Histological typing (according to WHO) is based on the tissue giving origin to the tumour, and histological grading, which is a good predictor of distant metastasis and overall survival, is based on a three-grade system (1–3) and depends on the mitotic index and the extent of tumour necrosis [3]. Staging of these tumours is clinically important and imparts additional prognostic information. The TNM system is employed, which combines histological grade, tumour size and depth, regional lymph node involvement and distant metastases [3]. Histological diagnosis should be based on the WHO classification for the grade and stage to be determined. Five-year survival has been reported to be 95%, 75%, and 45% in patients with grade 1, 2, and 3 sarcomas respectively [2]. Thoracic sarcomas account for 10% of all sarcomas and the rest are found on upper (15%) and lower (35%) limbs, visceral organs (15%), abdomen and retroperitoneum (15%), followed by head and neck (10%) [2]. Metastasis may either occur locally to nearby tissues or distally through the circulatory system predominantly to the lung and less frequently to the liver and bones [2]. Good prognosis is associated with young age, female gender, low histological grade, stage 1 tumours and complete surgical resection, while poor prognosis is related to high-grade tumours, stage 2 sarcomas, incomplete surgical removal, metastatic disease and relapses as well as aggressive histological subtypes [2]. For non-metastatic disease, the treatment of choice is surgical removal, which, depending on its outcomes, may be combined with radiotherapy. Each case should be approached on an individual basis and assessed by a multi-disciplinary team considering the need for neoadjuvant or adjuvant chemotherapy and its medications [2]. Even though there is inadequate evidence to dictate specific policies for follow-up, risk assessment should be conducted based on tumour grade, size and site with primary focus on the lungs (for metastatic disease) for routine follow-up. Standard follow-up should consist of clinical history, clinical examination for local recurrence (followed by ultrasound scan or MRI if needed), and chest X-ray with subsequent CT scan for suspicious lesions. The European Society of Medical Oncology (ESMO) recommends follow-up every 3–4 months in surgically treated intermediate/high-risk patients in the first 2 years, later on twice a year until the fifth year and then yearly afterwards. Those with low-grade sarcomas may be investigated for local relapse every 4–6 months, with chest X-rays or CT scan at larger intervals in the first 3–5 years, and then once a year [4,5]. In summary, the role of a multidisciplinary team is essential in the management of these challenging tumours and further well-conducted research is necessary for evidence-based guidelines to be devised [5]. Conflict of interest: none declared.

Intensity‐modulated radiation therapy with dose‐painting for pediatric sarcomas with pulmonary metastases

We examined patterns of failure in pediatric patients with thoracic sarcoma and pulmonary metastases treated with intensity-modulated radiation therapy with dose-painting (DP-IMRT). Eleven pediatric patients, five with Ewing sarcoma family tumors (ESFT) and six with rhabdomyosarcoma (RMS), with primary thoracic tumors and pulmonary metastases underwent DP-IMRT with chemotherapy for definitive treatment. Eight patients also underwent surgery. Median time to RT was 21 (15-31) weeks. Nine patients received 45-50.4-Gy in 1.8 Gy fractions to the primary tumor (n = 3) or post-operative tumor bed (n = 6). Two patients ≤4 years received 12 Gy intraoperative radiation therapy and 30.6-36 Gy IMRT postoperatively to the tumor bed. All patients received 14-16.8 Gy in 0.54-0.88 Gy fractions to the whole lungs (n = 6) or hemithorax (n = 5) using dose-painting technique. A representative case was re-planned with IMRT plus standard AP/PA whole lung irradiation (WLI) for dosimetric comparison. With 27-month median follow-up, 3-year pulmonary relapse-free survival in all patients was 61%: 80% for RMS and 40% for ESFT. Five patients (4 ESFT and 1 RMS) experienced pulmonary relapse at median 16 (9-41) months. There were no local failures. Our representative case demonstrated more homogeneous target volume coverage of the whole lungs and decreased mean dose to esophagus (15%), heart (31%), spinal cord (15%), and liver (19%) with DP-IMRT. The treatment of children with a primary thoracic tumor and pulmonary metastases poses a significant challenge. DP-IMRT is one solution to this technical problem. Initial data from this small series suggest DP-IMRT is feasible and produces superior sparing of critical normal tissues.

Localised thoracic sarcomas: Outcome improvement over time at a single institution

PurposeTo assess changes in survival over time in patients affected by thoracic soft tissue sarcomas treated at a single institution. Patients and MethodsPatients with localised adult-type deep thoracic soft tissue sarcoma surgically treated at our institution between 1980 and 2012 were retrospectively reviewed. Patients were categorised into two groups according to timing of their first operation, i.e. surgery done before or after 31st December 2001 (so called ‘early years’ and ‘recent years’ groups, respectively), since a more extended surgery was used in the second interval. Overall survival (OS) and crude cumulative incidence (CCI) of local recurrence (LR) and distant metastases (DM) were calculated for each time period. ResultsThree-hundred-thirty-seven patients were identified. Median follow-up was 4.7years. Tumour size and rate of critical site involvement were larger in ‘recent years’, while the distribution of all other tumour- and patient-related factors was identical in the two periods. Despite this, OS and CCI of LR were significantly better in ‘recent years’ as compared to ‘early’ ones, the 5-year OS increasing from 58% to 72% and the CCI of LR dropping from 22% to 11%. CCI of DM was equal in the two periods. ConclusionReference institutions for sarcomas may have improved their outcome in the last years. Although biases of retrospective analyses need to be discounted, it is possible that optimal exploitation of a series of subtle improvements in sarcoma treatment may make a difference in results achievable today.

Giant Subclavian Artery Pseudoaneurysm After Pleural Drainage Puncture

A 59-year-old man with thoracic sarcoma was referred for evaluation of swelling, pain, and functional impairment of his right arm. Six weeks prior, a right thoracic catheter was placed for drainage of pleural effusion, where now a pulsatile tumor (Figure 1) was noted. Duplex ultrasound of …

TLE1 is expressed in the majority of primary pleuropulmonary synovial sarcomas

Pleuropulmonary synovial sarcoma (PPSS) is a rare entity, similar to synovial sarcoma of soft tissue (STSS). There are 120 published cases of PPSS, but no studies have explored the expression of TLE1. In soft tissues, it has been proven a useful marker, but in tumors of other sites, its expression has not been explored. The main objective was to study the expression and diagnostic sensitivity and specificity of TLE1 in a group of PPSS, of which the diagnosis was corroborated by fluorescence in situ hybridization confirming t(X;18) in a tissue microarray. Immunohistochemistry including TLE1, vimentin, CD99, CD56, bcl-2, AE1-AE3, EMA, CD34, CK7, CK19, calponin, and S-100 was performed on all PPSS and on 25 control cases (five carcinomas, ten mesotheliomas, and ten thoracic sarcomas). TLE1 was positive in 11 cases (73.3%); bcl-2 and vimentin in 100%; calponin and CD56 in 26.6%; CD99, CK AE1-AE3, CK19, CK7, and EMA in 80%; and S100 negative in all. The only biphasic PPSS was positive for epithelial markers only in the epithelial component. TLE1 was negative in all control cases. TLE1 is expressed in 73% of PPSS, a value inferior to that reported in STSS, but is highly specific for PPSS. TLE1 may therefore be of value in the differential diagnosis of PPSS, but should be used in a panel of antibodies.

Imaging of Thoracic Sarcomas of the Chest Wall, Pleura, and Lung

Primary sarcomas of the thorax are uncommon. The purpose of this review is to describe the radiologic findings of sarcomas affecting the thorax, in particular the chest wall, pleura, and lungs. Most primary sarcomas affecting the thorax arise in the chest wall, and the most common sarcomas of the chest wall are chondrosarcoma, osteosarcoma, Ewing's sarcoma/primitive neuroectodermal tumor, malignant fibrous histiocytoma, and fibrosarcoma. Primary pleural and pulmonary sarcomas are rare. Although histologic analysis is almost always required for accurate diagnosis, imaging is important for staging of these tumors, and several of these tumors have distinctive radiologic features, allowing the radiologist to narrow the differential diagnosis.

Surgical Palliation of Previously Resected Thoracic Sarcoma

Surgical palliation can improve quality of life, provide local control, and extend survival. However, oncologists often view surgical intervention as a drastic measure with prohibitive morbidity. During the past decade, surgical palliation has played a role in the management of many metastatic malignancies, including those of the colon, breast, prostate, and kidney. In concert with this, the morbidity and mortality associated with surgical interventions has continued to decline. We present 2 patients with thoracic sarcoma recurrences after pneumonectomy that were associated with symptomatic compression of pulmonary vessels and airway. Both patients underwent surgical resection, with immediate relief of symptoms, improved quality of life, and extended survival.

Difficult Diagnosis and Differentiated Treatment in Synchronous Thoracic Hemangiomas

Thoracic hemangiomas are rare vascular neoplasia among adults. Even with advanced imaging, diagnosis is sometimes impossible, thereby mandating histopathological examination. The differential diagnosis of thoracic hemangiomas can include arteriovenous malformations, thoracic sarcomas (angiosarcoma, paravertebral mesothelioma), neurinoma, and neurofibroma. We herein present a case which was intitially suggestive of a malignancy that ultimately proved to be a capillary cavernous paravertebral and vertebral hemangioma. Given the unique topography of this lesion, a correct preoperative diagnosis was not possible and a interdisciplinary surgical approach was required.

Résection large suivie de reconstruction des sarcomes primitifs de la paroi thoracique : une prise en charge multidisciplinaire de 11 patients avec un suivi minimum de deux ans

Thoracic sarcomas are rare, and resection can leave behind defects that require significant reconstruction by the multidisciplinary surgical team. The aim of this study is to review the experience of our regional referral centre with primary thoracic tumor resection and thoracic reconstruction. We have reviewed the treatment of all chest wall tumors resected at Sir Charles Gairdner Hospital in Western Australia over a 5-year period. There were 11 cases in total that involved removal of deep muscle, ribs and/or sternum. In the six cases that required bony resection, the surgical team utilized a Gore-Tex (e-PTFE) mesh prosthesis to allow immediate closure of the defect, whilst five other closures were achievable using primary layered closure alone. Four patients had postoperative complications, including one who required prosthesis removal. Mean length of hospital stay was 5 days. No 30-day or 6-month mortality was recorded. All patients were followed up for a minimum of 24 months, and all patients were alive and free of disease at their most recent follow-up. This study concurs with previous literature indicating that thoracic tumor resection and immediate reconstruction often involving use of prosthetic mesh is a safe and effective one stage surgical procedure for a variety of chest wall defects with low postoperative morbidity.

Large resection and reconstruction of primary parietal thoracic sarcoma: A multidisciplinary approach on 11 patients at minimum 2-years follow-up

Thoracic sarcomas are rare, and resection can leave behind defects that require significant reconstruction by the multidisciplinary surgical team. The aim of this study is to review the experience of our regional referral centre with primary thoracic tumor resection and thoracic reconstruction. We have reviewed the treatment of all chest wall tumors resected at Sir Charles Gairdner Hospital in Western Australia over a 5-year period. There were 11 cases in total that involved removal of deep muscle, ribs and/or sternum. In the six cases that required bony resection, the surgical team utilized a Gore-Tex (e-PTFE) mesh prosthesis to allow immediate closure of the defect, whilst five other closures were achievable using primary layered closure alone. Four patients had postoperative complications, including one who required prosthesis removal. Mean length of hospital stay was 5 days. No 30-day or 6-month mortality was recorded. All patients were followed-up for a minimum of 24 months, and all patients were alive and free of disease at their most recent follow-up. This study concurs with previous literature indicating that thoracic tumor resection and immediate reconstruction often involving use of prosthetic mesh is a safe and effective one stage surgical procedure for a variety of chest wall defects with low postoperative morbidity.

Sarcomatoid Neoplasms of the Lung and Pleura

Sarcomatoid neoplasms of the lung and pleura are rare tumors that present a complex differential diagnosis, making them challenging for surgical pathologists. In the lung, the main tumors are the sarcomatoid carcinomas, including pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma, and pulmonary blastoma. They are characterized by histologic heterogeneity; molecular data support their origin from a pluripotent stem cell that undergoes neoplastic transformation with divergent epithelial and sarcomatous differentiation. Diagnosis is difficult in small biopsy specimens and typically requires a resection specimen. Despite the presence of sarcomatoid features, these tumors are classified as lung carcinomas. Pulmonary blastomas must be distinguished from pleuropulmonary blastomas, which are a unique type of thoracic sarcoma typically occurring in young children. In the pleura, the main tumors to consider are the sarcomatoid and desmoplastic types of malignant mesothelioma, solitary fibrous tumor, and desmoid tumor. While light microscopy is sufficient to diagnose most of these tumors, immunohistochemistry can be useful in selected settings. In particular, it can aid to confirm epithelial differentiation in spindle cell carcinomas and the presence of rhabdomyosarcoma in sarcomatoid carcinomas, mesotheliomas, or pleuropulmonary blastomas. For sarcomatoid and desmoplastic mesothelioma, keratin is the most useful stain because it can highlight invasive growth and mesothelial markers are positive in only the minority of cases. Clinical and radiologic correlation is needed to separate some pleomorphic carcinomas with pleural involvement from sarcomatoid malignant mesothelioma, since these poorly differentiated tumors may not express the usual immunohistochemical markers for carcinoma or mesothelioma.