Clinical characteristics and prognosis of patients with incidentally discovered chest wall sarcoma compared with those of symptomatic patients.

OBJECTIVE: Sarcomas of the bone and soft tissues are detected after the onset of pain, detectable mass and related symptoms in the absence of a standardized screening examination. However, primary chest wall sarcomas can be incidentally detected upon chest X-ray or computed tomography. Previous studies of incidental primary chest wall sarcomas lack prognosis and disease-specific clinical data. This study aimed to investigate the prognoses of patients with incidental chest wall sarcomas and compare them with those of symptomatic patients. METHODS: This study included 18 patients diagnosed with primary chest wall sarcoma between 2010 and 2023. Patient information such as age, sex, tumour diameter, tumour location, symptoms, treatment, time to treatment initiation, pathological diagnosis and outcome were retrospectively analysed. RESULTS: Among the 18 patients, the sarcomas were incidentally detected in five by chest X-ray and computed tomography in three and two patients, respectively. The pathological diagnoses of the patients were Ewing sarcoma, Chondrosarcoma grade 1, grade 2, periosteal osteosarcoma and malignant peripheral nerve sheath tumour. The patients had no symptoms at the first visit to our hospital, and no lesions in other organs were detected at the time of the initial examination. At the final follow-up, the patients remained disease-free after radical treatment. The tumour sizes of the five patients were significantly smaller than those of patients with symptoms (P = 0.003). CONCLUSIONS: The incidental detection of chest wall sarcomas and consequent early detection and treatment of tumours improves patient prognosis relative to that of symptomatically diagnosed patients.

Surgical Treatment for Primary Chest Wall Sarcoma: A Single-Institution Study.

BACKGROUND: Primary sarcomas of the chest wall are rare aggressive tumors. Surgery is part of the multimodal treatment. We describe our institutional patient cohort and evaluate prognostic factors. METHODS: All patients who had curative intent surgery for primary chest wall sarcoma from 2004 to 2019 were retrospectively reviewed. Impact on survival-calculated from the date of surgery until last follow-up- was assessed for the following variables: age, gender, type of resection, size, grading, stage, completeness of resection, and neoadjuvant and adjuvant therapy. RESULTS: Twenty-three patients (15 males, 65%) with a median age of 54 y (4 to 82) were included. Most common histology was chondrosarcoma (n = 5, 22%). Seven patients (30%) received neoadjuvant and 13 patients (57%) received adjuvant treatment. R0 resection was achieved in 83%. Extended chest wall resection was performed in 14 patients (61%), including lung (n = 13, 57%), diaphragm (n = 2, 9%) and pericardium (n = 1, 4%). Morbidity and 90-day mortality were 23% and 0%, respectively. Three- and 5-year overall survival was 53% and 35%, respectively. R0 resection was predictor of overall survival (P = 0.029). Tumor grade and extended resections were predictors for recurrence (P = 0.034 and P = 0.018, respectively). CONCLUSIONS: Surgical resection of primary chest wall sarcoma is a safe procedure even when extended resection is required.

Predictors of survival after resection of primary sarcomas of the chest wall-A large, single-institution series.

BACKGROUND AND OBJECTIVES: Chest wall sarcomas are rare and may demonstrate heterogeneous features. Surgery remains the mainstay of treatment with chemotherapy and radiotherapy used as adjuncts. Herein, we report outcomes of a large cohort of patients with primary chest wall sarcoma who underwent resection. METHODS: Records of 121 patients who underwent resection for primary chest wall sarcoma between 1998 and 2013 were reviewed. A thoracic pathologist reexamined all tumors and categorized them according to grade. Univariable and multivariable Cox analyses were conducted to identify predictors of overall survival (OS). RESULTS: The median age was 45.0 (range, 11-81) years, and most tumors (63.6%, 77) were high grade. The median tumor size was 7 cm (range, 1-21 cm). Fifty-nine (48.8%) patients received neoadjuvant chemotherapy and 12 (9.9%) received neoadjuvant radiotherapy. A complete resection was achieved in 103 (85.1%) patients. Neoadjuvant chemotherapy (P = 0.532) and radiation ( P = 1.000) were not associated with a complete resection. Five-year OS among patients undergoing R0 and R1 resections was 61.9% and 27.8%, respectively. Multivariable analysis identified high grade (HR, 15.21; CI, 3.57-64.87; P < 0.001), R1 (HR, 3.10; CI, 1.40-6.86; P = 0.005), R2 resection (HR, 5.18; CI, 1.91-14.01; P = 0.001), and age (HR, 1.02; CI, 1.01-1.03; P = 0.002) as predictors of OS. CONCLUSIONS: In this series of resected chest wall sarcomas, complete resection and tumor grade remain the most important survival predictors. Individual decisions are required for the utilization of neoadjuvant therapy.

Management of soft tissue sarcomas of the chest wall: a comprehensive overview.

Sarcomas of the chest wall are rare and their current treatment regimen is diverse and complex due to the heterogeneity of these tumors as well as the variations in tumor location and extent. They only account for 0.04% of newly diagnosed cancers of whom about 45% comprise soft tissue sarcomas. Larger cohort studies are scarce and often focus on one specific treatment item. We therefore aim to provide helicopter view for clinicians treating patients with sarcomas of the chest wall, focusing mainly on soft tissue sarcomas. This overview includes the value of neoadjuvant systemic or radiotherapy, surgical resection, approaches for thoracic wall reconstruction, and the need for follow-up. Provided the heterogeneity and relative rarity, we recommend that treatment decisions in soft tissue sarcoma of the chest wall are discussed in a multidisciplinary tumor board at a reference sarcoma center or within sarcoma networks to ensure personalized, rational decision making. A surgical oncologist specialized in sarcoma surgery is crucial, and for extensive resections involving the thoracic cavity we recommend involvement of a thoracic surgeon. In addition, a specialized medical- and radiation oncologist as well as a plastic surgeon is required to ensure the best multimodality treatment plan to optimize patient outcome.

Chest wall resection and reconstruction for primary chest wall sarcomas: analysis of survival, predictors of outcome and long-term functional status.

OBJECTIVES: We aimed to analyze survival, predictors of outcome and the long-term functional status of patients with a diagnosis of primary chest wall sarcoma who undergo chest wall resection and reconstruction (CWRR). METHODS: We analysed a prospectively maintained database, including all patients operated on between 2008-2021. The primary outcome measures were overall and disease-free survival and analyses were employed to determine the risk factors for poor survival and recurrence. RESULTS: One hundred and thirty-nine patients included, 55% were male. The majority (96%) had an R0 resection and 75.1% had no post-operative complications up to 30 days post procedure; median length of hospital stay was 7 (6-10) days. Median overall and disease-free survival (DFS) was 58.8 and 53.6 months respectively. For those alive, at long-term follow-up, 80% had a Medical Research Council (MRC) dyspnoea score of 0 and Karnofsky index >80%. Survival and mortality rates were better in chondrosarcomas compared to non-chondromatous sarcomas (p<0.05). Previous history of radiotherapy, previous history of cancer, the type of sarcoma (Ewing's or soft tissue), the need for adjuvant treatment and tumour grade were significant predictors of mortality and recurrence on univariate testing. Extended resection, a higher number of ribs removed, and the incidence of post-operative complications were significantly associated with a worse post-operative MRC score. CONCLUSIONS: Careful patient selection and multi-disciplinary decision-making is crucial. This leads to clear resection margins, good overall and disease-free survival and good functional outcomes.

Chest Wall Synovial Sarcoma: A Unique Encounter at the Breast Base.

Synovial sarcomas most commonly arise in the para-articular locations of the extremities, such as the upper limbs, thigh, knee, ankle, and foot. Thoracic synovial sarcomas are a rare entity that can arise in the chest wall, pleura, lung, heart, or mediastinum. We present a case of a 23-year-old female with a complaint of swelling of the left breast. Examination demonstrated an enlarged left breast and a hard-fixed swelling without overlying skin changes or nipple retraction. Ultrasound showed a well-defined, solid-appearing lesion deep in the left breast parenchyma, which was adherent to the underlying left chest wall musculature and seemed to be displacing the breast parenchyma anteriorly. Contrast-enhanced computed tomography (CECT) and magnetic resonance imaging (MRI) confirmed the lesion centered at the left pectoralis major and minor muscles, confirming the chest wall's origin. Histopathology findings favored monophasic synovial sarcoma.

Outcome Analysis of Treatment Modalities for Thoracic Sarcomas.

BACKGROUND: Primary chest wall sarcomas are a rare and heterogeneous group of chest wall tumors that require multimodal oncologic and surgical therapy. The aim of this study was to review our experience regarding the surgical treatment of chest wall sarcomas, evaluating the short- and long-term results. METHODS: In this retrospective single-center study, patients who underwent surgery for soft tissue and bone sarcoma of the chest wall between 1999 and 2018 were included. We analyzed the oncologic and surgical outcomes of chest wall resections and reconstructions, assessing overall and recurrence-free survival and the associated clinical factors. RESULTS: In total, 44 patients underwent chest wall resection for primary chest wall sarcoma, of which 18 (41%) received surgery only, 10 (23%) received additional chemoradiotherapy, 7% (3) received surgery with chemotherapy, and 30% (13) received radiotherapy in addition to surgery. No perioperative mortality occurred. Five-year overall survival was 51.5% (CI 95%: 36.1-73.4%), and median overall survival was 1973 days (CI 95% 1461; -). As determined in the univariate analysis, the presence of metastasis upon admission and tumor grade were significantly associated with shorter survival (p = 0.037 and p < 0.01, respectively). Five-year recurrence-free survival was 71.5% (95% CI 57.6%; 88.7%). Tumor resection margins and metastatic disease upon diagnosis were significantly associated with recurrence-free survival (p < 0.01 and p < 0.01, respectively). CONCLUSION: Surgical therapy is the cornerstone of the treatment of chest wall sarcomas and can be performed safely. Metastasis and high tumor grade have a negative influence on overall survival, while tumor margins and metastasis have a negative influence on local recurrence.

Chest wall resection and reconstruction for primary and metastatic sarcomas: an 11-year retrospective cohort study.

OBJECTIVES: Chest wall sarcomas are rare, aggressive malignancies, the management of which mainly revolves around surgery. Radical tumour excision with free margins represents the optimal treatment for loco-regional clinically resectable disease. The objective of this study was to review our 11-year experience with chest wall resection for primary and metastatic sarcomas, focusing on surgical techniques and strategies for reconstruction. METHODS: Retrospective analysis of a comprehensive database of patients who underwent chest wall resection for primary or secondary sarcoma at our Institute from January 2009 to December 2019. RESULTS: Out of 26 patients, 21 (81%) suffered from primary chest wall sarcoma, while 5 (19%) had recurring disease. The median number of resected ribs was 3. Sternal resection was performed in 6 cases (23%). Prosthetic thoracic reconstruction was deemed necessary in 24 cases (92%). Tumour recurrence was observed in 15 patients (58%). The median overall survival was 73.6 months. Primary and secondary tumours showed comparable survival (P = 0.49). At univariate analysis, disease recurrence and infiltrated margins on pathological specimens were associated with poorer survival (P = 0.014 and 0.022, respectively). In patients with primary sarcoma, the median progression-free survival was 13.3 months. Associated visceral resections were significantly associated to postoperative complications (P = 0.02). CONCLUSIONS: Chest wall resection followed by prosthetic reconstruction is feasible in carefully selected patients and should be performed by experienced surgeons with the aim of achieving free resection margins, resulting in improved long-term outcomes.

Case Report: Surgical Reconstruction of a Massive Thoracic Wall Defect After the Resection of an Undifferentiated Radiation-Induced Sarcoma of the Breast.

A 54-year-old lady was referred to our institute because of a massive thoracic neoplasm arising from the thoracic wall which infiltrated and dislocated the left breast. Twenty years before, the patient had undergone a quadrantectomy with axillary dissection for an infiltrating ductal carcinoma of the left breast, followed by adjuvant radiotherapy and chemotherapy. A true-cut biopsy of the mass showed a low differentiated malignant neoplasm with spindle-shaped cells. The patient underwent a total-body CT scan which showed a 16 × 15 × 10 cm largely necrotic mass with irregular and undefined margins, with little homolateral round-shaped cervical and mesenteric lymph nodes but no distant metastases. After a multidisciplinary discussion, we proposed surgery as the first therapeutic option. The planned treatment was a wide excision of the mass with the underlying ribs (II-VI) followed by the reconstruction of the thoracic wall using titanium bars covered by the acellular porcine dermis, latissimus dorsi flap, and finally, skin grafts from the thighs.

Primary chest wall sarcoma; a single institution experience of 3 years.

INTRODUCTION: Primary chest wall sarcoma is a rare entity. It can be classified based on its origin, as bone sarcomas or soft tissue sarcomas. Various prognostic factors have been studied in different case series like age, sex, tumor histology, grade, resection margin status, adjuvant treatment, and others. The present study aimed to analyze common histological types, their management by resection and reconstruction and prognosis, in cases presenting at a regional cancer center in western India. MATERIAL AND METHOD: This was an observational study from a prospectively maintained database. 57 patients with chest wall sarcoma treated with curative intent between January 2016 till January 2019 with a minimum follow-up of 3 months were included in the study. The goals of surgical treatment were to obtain a wide resection margin of 3-4 cm, preserve the function of the chest wall and provide stability and rigidity to protect intrathoracic organs. RESULTS: The median follow-up of the present patient's cohort was for 20.2 months. Overall two-year survival was 74.7%. Two-year OS and DFS of bone sarcoma were 62.3% and 35% and soft tissue sarcomas were 91% and 71.3%. Ewing's sarcoma had the worst two-year overall survival of 50.6% and chondrosarcoma and fibromatosis had 100% two-year overall survival. CONCLUSION: Chest wall sarcoma forms a heterogeneous group of tumors. In the present study, Ewing's sarcoma was the most common histology with the worst survival, since they presented in advanced stages. Management should be multidisciplinary and surgical resection should be aggressive to achieve an R0 resection. Reconstruction of chest wall should aim to provide structural and functional stability with minimal morbidity. Frozen section assessment should be utilized whenever in doubt.

11-year experience with Chest Wall resection and reconstruction for primary Chest Wall sarcomas.

BACKGROUND & OBJECTIVES: Primary chest wall sarcomas are rare and therapeutically challenging tumors. Herein we report the outcomes of a surgery-based multimodality therapy for these pathologies over an 11-year period. In addition, we present a case that illustrates the surgical challenges that extensive chest wall resection may pose. METHODS: Using the Society of Thoracic Surgeons general thoracic surgery database, we have prospectively collected data in our institute on all patients undergoing chest wall resection and reconstruction for primary chest wall sarcomas between June 2008-October 2019. RESULTS: We performed 28 surgical procedures on 25 patients aged 5 to 91 years (median age 33). Eleven tumors were bone- and cartilage-derived and 14 tumors originated from soft tissue elements. Seven patients (7/25, 28%) received neo-adjuvant therapy and 14 patients (14/25, 56%) received adjuvant therapy. The median number of ribs that were resected was 2.5 (range 0 to 6). In 18/28 (64%) of surgeries, additional skeletal or visceral organs were removed, including: diaphragm [1], scapula [2], sternum [2], lung [2], vertebra [1], clavicle [1] and colon [1]. Chest wall reconstruction was deemed necessary in 16/28 (57%) of cases, polytetrafluoroethylene (PTFE) Gore-Tex patches was used in 13/28 (46%) of cases and biological flaps where used in 4/28 (14%) of cases. R0, R1 and R2 resection margins were achieved in 19/28 (68%), 9/28 (32%) and 0/28 (0%) of cases, respectively. The median follow up time was 33 months (range 2 to 138). During the study period, disease recurred in 8/25 (32%) of patients. Of these, 3 were re-operated on and are free of disease. At date of last follow up, 5/25 (20%) of patients have died due to their disease and in contrast, 20/25 (80%) were alive with no evidence of disease. CONCLUSIONS: Surgery-based multimodality therapy is an effective treatment approach for primary chest wall sarcomas. Resection of additional skeletal or visceral organs and reconstruction with synthetic and/or biological flaps is often required in order to obtain R0 resection margins. Ultimately, long-term survival in this clinical scenario is an achievable goal.

Surgical Management of Chest Wall Sarcoma.

Chest wall sarcoma is a rare and challenging pathology best managed by a multidisciplinary team experienced in the management of a multiple different pathologies. Knowledge of the management sequence is important for each sarcoma type in order to provide optimal treatment. Surgical resection of chest wall resections remains the primary treatment of disease isolated to the chest wall. Optimal margins of resection and reconstruction techniques have yet to be determined.

The impact of racial/ethnic disparities on survival for children and young adults with chest wall sarcoma: A population-based study.

BACKGROUND: To determine whether there are racial/ethnic disparities in disease presentation, treatment and survival outcomes among children and young adults with chest wall sarcomas. METHODS: The Surveillance, Epidemiology and End Results (SEER) database was analyzed for patients 21 years old and younger with chest wall sarcoma. We performed multivariate logistic regression to investigate the association of race/ethnicity with advanced stage of disease at presentation and likelihood of undergoing surgical resection. Overall survival (OS) was evaluated using Cox regression modeling to calculate hazard ratios with 95% confidence intervals. RESULTS: A total of 669 patients were identified: 393 non-Hispanic whites (NHW) (59%), 151 Hispanics (23%), 64 non-Hispanic blacks (NHB) (11%), and 64 other race/ethnicity (9%). The 5- and 10-year OS rates for the entire cohort were 69% and 64%, respectively. NHB had significantly worse 5-year and 10-year OS compared to NHW based on the log rank test (61% versus 70%, 52% versus 66%, respectively; p = 0.037).). Most patients (80%) underwent surgical resection. However, NHB were less likely than NHW to undergo surgical resection by multivariate analysis (OR 0.43, 95% CI 0.22-0.82). CONCLUSIONS: NHB children and young adults with chest wall sarcoma have decreased overall survival. In addition, NHB are less likely to undergo surgical resection which may contribute to survival disparities. It is paramount that health care providers work to close the treatment gap between racial/ethnic groups to improve survival in children and young adults with chest wall sarcoma. LEVEL OF EVIDENCE: Level III Treatment Study.

R0 resection and reconstruction for a large, rapidly progressive chest wall sarcoma.

BACKGROUND: Chest wall sarcomas are a rare group of soft tissue malignancies with variable presentations. Here we describe the definitive management of a large, rapidly progressing chest wall sarcoma arising from the pectoralis major muscle. CASE REPORT: An obese 42-year-old African American male with multiple medical comorbidities presented with new onset right-sided chest pain and a palpable right chest mass. Initial CT chest demonstrated a 9x9x9cm necrotic mass arising from the pectoralis major. CT-guided core biopsy was positive for high-grade spindle cell neoplasm (positive for smooth muscle actin, desmin, S100, and CD31; negative for CD34, PAX8, and beta-catenin). Staging imaging 2 months later demonstrated growth of the mass to 21.4 × 17.8 × 13.7 cm. The patient underwent neoadjuvant chemoradiation with surveillance CT imaging demonstrating a stable tumor. Then he underwent wide local excision of the mass followed by delayed local myocutaneous flap reconstruction and skin grafting. Final pathology was R0 resection, 38x20x18 cm tumor with 70% gross necrosis. Microscopic examination confirmed high-grade sarcoma with smooth muscle differentiation. Final pathologic staging was Stage III G3 pT2bNxMx. CONCLUSIONS: This patient presented with a rare, rapidly enlarging high-grade leiomyosarcoma of the chest wall without metastases or violation of the thorax. We describe the definitive management including a multidisciplinary team to manage a complex and rapidly progressive sarcoma of the chest wall.