A systematic review of undifferentiated pleomorphic sarcoma of the chest wall
Review Article

A systematic review of undifferentiated pleomorphic sarcoma of the chest wall

Cade Bennett1^, Sandeep Bharadwaj2^, Andrew Arndt2, Akhil Chawla1,3,4^

1Division of Surgical Oncology, Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, USA; 2Division of Thoracic Surgery, Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, USA; 3Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, IL, USA; 4Northwestern Quality Improvement, Research, & Education in Surgery, Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

Contributions: (I) Conception and design: C Bennett, A Chawla, S Bharadway; (II) Administrative support: A Arndt, A Chawla; (III) Provision of study materials or patients: S Bharadwaj; (IV) Collection and assembly of data: C Bennett; (V) Data analysis and interpretation: C Bennett; (VI) Manuscript writing: All authors; (VII) Final approval of manuscript: All authors.

^ORCID: Cade Bennett, 0009-0001-8195-2622; Sandeep Bharadwaj, 0000-0003-0561-5103; Akhil Chawla, 0000-0003-3122-6873.

Correspondence to: Akhil Chawla, MD. Division of Surgical Oncology, Department of Surgery, Northwestern University Feinberg School of Medicine, 310 E. Superior Street, Chicago, IL 60611, USA; Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, IL, USA; Northwestern Quality Improvement, Research, & Education in Surgery, Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, USA. Email: Akhil.chawla@nm.org.

Background: Undifferentiated pleomorphic sarcoma (UPS) accounts for approximately 15% of all soft-tissue sarcoma (STS) cases and have a 5-year survival prognosis of around 60%. Due to its complexity, tumors are often identified by clinical and pathological exclusion. UPS is commonly found in the extremities, so finding them in the trunk and chest wall is rare. The primary objectives of this systematic review are: (I) identifying patient characteristics with lesion; (II) compiling patient outcomes following surgery; (III) identifying best therapy modalities; (IV) characterizing reported lesion histology; (V) assessing current surgical recommendations for resection; (VI) classifying lesions and their association with radiation.

Methods: The PRISMA framework was utilized to identify case reports and records providing information on UPS in the chest wall. Case reports and articles were screened for relevance, full-text accessibility, and if they contained the terms (“undifferentiated pleomorphic sarcoma”, “breast”, “chest wall”, or “trunk”) in their title or abstract. The PubMed database was the primary database, and the search criteria was “(undifferentiated pleomorphic sarcoma) AND ((breast) OR (trunk) OR (chest) OR (chest wall))” from 01/01/2003 to 05/21/2023. Given that these were case reports, bias risk and heterogeneity was not assessed due to its difficulty. Information from case reports were compiled into a table and a Chi-squared test was performed, but no meta-analysis was completed.

Results: Of 433 studies, 24 case reports and 22 records were selected to inform on UPS in the chest wall. The 24 case reports yielded 32 cases providing information on patient outcomes, tumor characteristics, and treatment. A meta-analysis was not performed, but literature was summarized to inform on treating the condition. Case reports were compiled into a table providing information on patient age, gender, tumor location, treatment modalities, margin distance, and other factors.

Conclusions: Treatment of UPS involving the chest is extremely complex. Unlike typical UPS, it is more often found in women than in men, which is corroborated by the results of this study. This study also notes no difference in recurrence or metastasis between patient who were treated and those who were not treated with other therapies.

Keywords: Undifferentiated pleomorphic sarcoma (UPS); chest wall; soft-tissue sarcomas (STS); torso

Submitted Jul 13, 2023. Accepted for publication Nov 09, 2023. Published online Nov 28, 2023.

doi: 10.21037/cco-23-71

Highlight box

Key findings

• There are more reported cases of undifferentiated pleomorphic sarcoma (UPS) in the chest for women than men.

• Recurrence/metastasis for these patients was not different for patients treated with radiotherapy and/or chemotherapy.

• Secondary development of UPS was associated with radiation in 5/22 patients.

What is known and what is new?

• 1.5 cm margin distance is likely sufficient in preventing recurrence.

• Relapse-free survival is better in patients receiving some forms of radiotherapy, whether adjuvant or neoadjuvant.

• Surgical resection is the primary mode of treatment and is often supplemented with radiotherapy and chemotherapy.

• Prognosis is worse in patients with deeper and larger tumors of the chest wall.

What is the implication, and what should change now?

• Future case reports need to provide information on margin distance to limit complications without compromising patient outcomes.

• Future research needs to elucidate the necessity of other therapies in the context of margin distance, tumor size, and tumor location.

Introduction

Sarcomas are a heterogenous group of malignancies that originate from mesenchymal stem cells with a yearly incidence of 5 cases per 100 thousand individuals (1,2). They may be broadly grouped into osteosarcomas, originating from the bone, and soft-tissue sarcomas (STS).

STS account for around 1% of all adult malignancies and encompass over 70 subtypes (3,4). The specific subtype defines treatment options and prognosis (5,6). Subtype diagnosis, however, may be challenging, and undifferentiated pleomorphic sarcoma (UPS) accounts for around 11–17% of STS (7). UPS commonly originates in the lower extremity muscles and deep fascia, so breast and chest wall presentation are atypical (1,8). Originally termed “malignant fibrous histiocytomas”, UPS is staged using tumor, node, and metastasis (TNM) criteria and histologic grade criteria—as determined by a tumor’s mitotic count, necrosis extension, and differentiation—from the French Federation of Cancer Centers Sarcoma Group (FNCLCC) (9,10). During the period of time in which UPS were classified as malignant fibrous histiocytomas, attempts were made at subclassifying these cases into distinct histological subtypes: pleomorphic/storiform, giant cell, inflammatory, angiomatoid, and myxoid (11). However, this system is no longer utilized due to the reclassification of these lesions to UPS and the relative difficulty in defining a lesion as a specific subtype. Given the changes in diagnostic classification and the growing number of reports on UPS involving the chest wall and breast, this review aims to characterize the outcomes, treatment modalities, prognoses, and histology of the current case reports.

The following review has several objectives that we want to address: (I) patient characteristics with the lesion; (II) patient outcomes following surgical resection or resection attempt; (III) best treatment modalities outside of surgical resection; (IV) common histological characteristics of these lesions; (V) current recommendations for UPS resection when considering localization to the thorax; (VI) classification of lesions as primary or secondary and association with radiation. We present this article in accordance with the PRISMA reporting checklist (available at https://cco.amegroups.com/article/view/10.21037/cco-23-71/rc).

Methods

Literature search and search criteria

A literature search was conducted using the PubMed database to identify case reports of UPS localized to the chest wall or breast tissue. Studies were considered within the date range of 01/01/2003 to 05/21/2023. Search criteria was placed into PubMed as follows: (undifferentiated pleomorphic sarcoma) AND ((breast) OR (trunk) OR (chest) or (chest wall)).

Of note, search terms did not include “malignant fibrous histiocytoma” because of possible over-classification and misclassification prior to 2002 (12). Increasing adoption by pathologists of tumor classification using immunohistochemistry and electron microscopy resulted in the World Health Organization’s reclassification of malignant fibrous histiocytoma to UPS in 2002 (13).

Inclusion and exclusion criteria

Studies were initially excluded if they did not contain at least one of the following terms in their title or abstract: “undifferentiated pleomorphic sarcoma”, “breast”, “chest wall”, or “trunk”. Included case reports were screened for relevance, if they contained “undifferentiated pleomorphic sarcoma” in the text, and full-text accessibility. Further, only case reports that considered or used surgical resection as a treatment modality were analyzed. All case reports were screened by one reviewer, and no automation tools were used. The PRISMA framework was utilized to identify possible studies to be included in the final tabulation.

Data synthesis and analysis

All data was collected manually by one reviewer. Information from the case reports were compiled into a table for greater understanding of current treatment practices and treatment effectiveness. The topics that were extracted from these cases are as follows: authors, year published, patient age, patient sex, histology of tumor, tumor location, lesion size, if neoadjuvant or adjuvant therapy was given, surgical treatment protocol, patient follow-up, if tumor was primary or secondary, association with radiation, tumor grade, and margin distance following surgery. Any information that was not given in these studies were indicated as not specified, except in the case of adjuvant and neoadjuvant therapy. For this, it was assumed that the patients did not receive this treatment as this would likely be reported in the case.

Data synthesis occurred in two parts. Our current understanding of UPS of the chest and its treatment modalities were synthesized from guidelines, studies, or other forms of literature with information, such as margin distance, patient characteristics, or treatment modality. Furthermore, the case reports were used to compile information into an accessible table for clinicians and researchers to identify areas of improvement in future research.

Meta-analysis and other statistical methods analyzing heterogeneity and outcome certainty were not performed with this study for a few key reasons. First, the rarity of non-extremity UPS and the lack of controlled trials comparing treatment modalities make it difficult to draw conclusions and assess heterogeneity accurately. Secondly, this study compiles information from unique case reports, so there is no control patient cohort to compare patient presentations, making it difficult to draw conclusive statistical metrics. Third, an intended goal of this review was not to draw statistical conclusions but to provide researchers with cogent topics to analyze by systematically selecting literature.

A Chi-squared test of independence was performed comparing patients who received therapy versus patients who did not receive therapy to see if there was any association with recurrence or metastasis. The programming language, R, was utilized to perform these assessments.

Bias assessment

Given that the studies analyzed were all unique case reports or series, there is risk of inherent bias in each report. However, it is difficult to quantify this bias without a control cohort of patients. Moreover, this study is intended to supplement current surgical practices by providing an overview of current treatment approaches and identify unique characteristics that may further our current understanding of this non-extremity UPS.

Results

Included studies

Initial screening identified 433 records in the PubMed database (Figure 1). Of these, 88 reports were further screened, resulting in 24 case reports with 32 cases total and 22 other studies being included in the final review. The cases are summarized in Table 1 along with further details on tumor origin/grade and treatment modalities employed.

Figure 1 PRISMA framework for included studies from PubMed. Reports were excluded based on relevance, failing to contain “undifferentiated pleomorphic sarcoma”, lack of full-text availability, and no plan or completion for surgery. Forty-six reports were included in the review in which 24 of these were case reports and 22 were relevant studies and reviews that were not case reports.

Table 1

Information on case studies with patients who had an undifferentiated pleomorphic sarcoma involving the trunk

Authors Year Age, year Sex Histologic type Location Size of lesion Adjuvant or neoadjuvant therapy? Surgical treatment Follow-up Primary or secondary? Radiation-induced? Tumor grade Margin distance?
Cariboni et al. (14) 2021 62 Male NS Paravertebral, infiltrating the aortic wall and the 9th thoracic vertebra 25 mm × 19 mm No therapy Aortic resection with bypass grafting and triple en bloc vertebrectomy with tumor excision No evidence of disease (22 months) Primary NA Grade 3 (FNCLCC standards) NS
Cozzolino et al. (15) 2018 65 Female NS Left breast >5 cm Adjuvant radiotherapy Mastectomy NS Primary NA High-grade (NCI criteria) NS
Hoshi et al. (16) 2020 44 Male NS Right chest wall 10 cm × 7 cm × 9 cm Neoadjuvant chemotherapy and Mohs’ chemosurgery Wide resection Lung metastasis (6 months), death (18 months) NS NS High-grade (NCI criteria) NS
Kong et al. (17) 2020 75 Female NS Right breast 6 cm Adjuvant radiotherapy Wide local resection without invasion of the chest wall No evidence of disease (15 months) Secondary Yes Grade 2 0.1 cm posterior margin distance
Patel et al. (18) 2019 58 Male Spindle cells Left chest wall/lung mass protruding through first and second rib 9 cm × 7 cm × 4 cm Neoadjuvant chemotherapy and Mohs’ chemosurgery Left chest wall resection and left upper lobectomy NS Secondary Yes Grade 2 (FNCLCC criteria) NS
Qorbani and Nelson (19) 2019 66 Male Epithelioid Right chest wall/lung mass protruding through the 8th and 9th intercostal spaces 15 cm × 13.6 cm × 6.2 cm Adjuvant radiotherapy and chemotherapy Wedge resection of right lower and middle lobes and adjacent right chest wall No evidence of disease (4 months) Primary NA High-grade NS
Sahu et al. (20) 2021 31 Male Primarily spindle with giant cells Superior, anterior aspect of right chest wall 5.1 cm × 2.4 cm × 4.2 cm Adjuvant radiotherapy Wide local excision No evidence of disease (6 months) Primary NA Grade 2 (FNCLCC criteria) NS
Singh et al. (21) 2021 52 Male Spindle cells Right chest wall infiltrating underlying skeletal muscle 4.7 cm × 3.6 cm × 3.1 cm No therapy Right mastectomy Systemic metastasis (6 months) Secondary No NS NS
Komaei et al. (22) 2019 63 Female Spindle-shaped, fibroblast-like cells; multinucleated giant cells Left breast 3.0 cm diameter No therapy Wide local excision NS Secondary Yes NS NS
Chakrabarti et al. (23) 2013 60 Female Spindle cells with multinucleated giant cells Left breast 6 cm × 4 cm No therapy Wide local excision DOD (3 weeks) Primary NA High-grade NS
Kocama et al. (24) 2021 71 Male Spindle cell with focal myxoid change; high cellularity with nuclear pleomorphism Right scapula 16 cm diameter No therapy Wide local excision Local recurrence (6 months); non-progression of local recurrence (15 months) Primary NA High-grade 3 cm skin margin
Prakash et al. (25) 2022 77 Female Osteoblastic-like, multinucleated giant cells and spindle cells Right posterior shoulder 11.2 cm × 14.2 cm × 8.8 cm No therapy En bloc, wide local excision NS Primary NA High-grade (FNCLCC criteria) NS
Srinivasamurthy et al. (26) 2016 29 Female Spindle cells with hyperchromatic nuclei and eosinophilic cytoplasm, bizarre cells, osteoclast-like giant cells Left breast 7 cm × 4 cm × 3 cm No therapy Total mastectomy NS Primary NA High-grade NS
Qiu et al. (27) 2013 68 Female It should be noted that they did not provide which patients had the specific types Left breast 7.9 cm diameter No therapy Modified radical mastectomy Alive, NED Primary NA NS NS
58 Female Left breast 5 cm diameter No therapy Modified radical mastectomy Lung metastasis, DOD (6 months) Primary NA NS NS
63 Female 1 case had xanthoma cells, atypical spindle cells, and inflammatory cells Left breast 15 cm diameter Adjuvant chemotherapy Modified radical mastectomy Liver metastasis, DOD (7 months) Primary NA NS NS
24 Female Left breast 5 cm diameter Adjuvant chemotherapy Modified radical mastectomy Alive, NED Primary NA NS NS
52 Female 1 case had osteoclast-like giant cells Right breast 13 cm diameter Adjuvant chemotherapy Modified radical mastectomy Alive, NED Primary NA NS NS
20 Female Left breast 3 cm diameter No therapy Lumpectomy Alive, NED Primary NA NS NS
73 Female 7 cases had mixed heteromorphic giant cells, spindle cells, and histiocytic-like cells Left breast 4 cm diameter No therapy Modified radical mastectomy Chest wall recurrence (19 months), DOD (26 months) Primary NA NS NS
51 Female Left breast 4 cm diameter Adjuvant chemotherapy Modified radical mastectomy Chest wall recurrence (3 months), DOD (8 months) Primary NA NS NS
48 Female Left breast 17 cm diameter Adjuvant chemotherapy Radical mastectomy Dead, NED Primary NA NS NS
Quadros et al. (28) 2006 44 Female Pleomorphic bizarre giant tumor cells with multinuclear spindle cells Left breast 9.5 cm × 9.0 cm × 8.5 cm Neoadjuvant chemotherapy Total radical mastectomy with chest wall resection NED (44 months) Secondary Yes High-grade >2 cm
Yam (29) 2022 53 Female Spindle cells Right breast 17.4 cm × 10.2 cm × 18 cm Neoadjuvant and adjuvant CRT Total radical mastectomy NED (12 months) Primary NA High-grade NS
Sang et al. (30) 2021 51 Female Atypical spindle cells Left breast 8 cm × 4 cm × 9 cm Neoadjuvant and adjuvant CRT Radical mastectomy Brain and lung metastasis (8 months) Primary NA High-grade NS
Bertucci et al. (31) 2015 61 Female Fibroblast-like spindle cells Right breast 2 cm diameter None Radical mastectomy with wide chest wall en bloc resection Local recurrence (4 months), DOD (25 months) Secondary Yes High-grade NS
Noh et al. (32) 2012 70 Female NS Left axillary region 8 cm diameter None Wide local excision NS Secondary Yes Grade 3 (FNCLCC criteria) NS
Balbi et al. (33) 2013 50 Female Atypical spindle-shaped and ovoid cells with multinuclear giant cells and epithelioid cells Right breast 10 cm diameter None Total radical mastectomy NED (15 months) Primary NA NS NS
Yamazaki et al. (34) 2018 55 Female Spindle-shaped cells with heteromorphic strong nuclei Right breast >5 cm diameter Neoadjuvant chemotherapy Simple mastectomy Lung metastasis, DOD (4 months) Primary NA NS NS
Gambichler et al. (35) 2023 58 Female Giant and atypical spindle-shaped tumor cells with nuclear pleomorphism Left breast NS Adjuvant immunotherapy and radiotherapy Total radical mastectomy Local recurrence (3 months), distal metastasis, DOD (15 months) Primary NA High-grade NS
Jeong et al. (36) 2011 76 Male Spindle cells with eosinophilic infiltrates and lymphoplasma cells. Atypical cells were noted Left breast 3.8 cm diameter None Wide local en bloc resection NS Primary No High-grade NS
Anzali et al. (37) 2023 58 Female NS Left breast Extremely large based on image (>5 cm, size not given) Neoadjuvant chemotherapy and radiotherapy None DOD (time not specified) Primary No High-grade NA

NS, not specified; NA, not Applicable; NCI, National Cancer Institute; FNCLCC, Fédération Nationale des Centres de Lutte Contre le Cancer; DOD, died of disease; NED, no evidence of disease; CRT, chemoradiotherapy.

Findings

Table 1 reveals some metrics that may be interesting to consider given our current understanding of UPS. First, 8/32 (25%) of the patients identified in our review of trunk UPS were male. Additionally, 24/32 patients (75%) had tumor diameters greater than 5 cm in one dimension.

In terms of treatment, 17/32 (53.13%) received some form of adjuvant or neoadjuvant therapy. Of these 17 individuals, 6 (35.3%) still developed distal metastasis or local recurrence. Comparatively, 6/10 (60%) that had follow-up information and no adjuvant therapy reported metastasis or recurrence. The Chi-squared test results revealed that there is no connection between therapy application and the occurrence of metastasis or local recurrence (χ2=1.56, df=1, P=0.2122).

For patients who received adjuvant or neoadjuvant chemotherapy, 5/13 (38.5%) experienced some form of recurrence or distal metastasis, with the liver and lungs being the primary locations. For patients who received neoadjuvant or adjuvant radiotherapy (RT), 2/8 (25%) had local recurrence or metastasis. Furthermore, 6/16 (37.5%) of surviving patients with tumor diameter greater than 5 cm in one dimension had local recurrence or metastasis, and 2 were noted as dying of their disease; 5/22 (22.7%) with a UPS of the breast had a radiation-associated malignancy as well.

Discussion

Historically termed malignant fibrous histiocytoma by Ozzello et al. in 1963, UPS accounts for approximately 15% of all adult soft tissue sarcomas and primarily arises in the limbs (38-42). UPS lesions are found less frequently in the trunk (~15% of all cases), specifically near the chest wall and breasts (24,40). The five-year overall survival rate typically ranges from 55% to 65%, and patients with higher grades of UPS exhibit worse long-term survival as compared with other STS counterparts (1).

In the breast, UPS is characterized by a local recurrence rate of 44% and distal metastasis of 42% (43). A retrospective review of 192 patients with resection of STS of the chest wall found that 32/192 (17%) patients’ sarcomas were classified as UPS, second to desmoid tumors (44). The most common histological subtype of recurrence in this cohort was UPS as well (44). Multiple studies have demonstrated that high tumor grade and large maximal tumor diameter, typically >5 cm, is associated with higher risk for recurrence (2,27,45,46).

Histologic characteristics for undifferentiated STS are remarkably diverse. These sarcomas are commonly divided into pleomorphic, spindle cell, round cell, epithelioid, and unspecified types (1,40,47). Pleomorphic lesions are often patternless but are defined by variation in nuclear size, hyperchromasia, and necrosis surrounding the lesion (1,40). These lesions involve a variety of cells, including fibroblast-like spindle and giant cells with multiple nuclei (20,22,23,48).

Treatment depends on staging. Metastatic UPS is typically treated with systemic therapy. Treatment of localized non-metastatic UPS involves surgical resection, when technically feasible, occasionally coupled with neoadjuvant or adjuvant therapies, which may include radiation therapy and chemotherapy. With regards to surgical resection, adequate margin distance is a crucial to limit, and studies typically indicate that 4 cm is an adequate size (27,49,50). However, a recent retrospective study of 41 patients demonstrated increased rates of recurrence when margin distance was less than 1.5 cm as compared with patients with margin distances greater than 1.5 cm (46). Larger tumor size as well as proximity to important structures may limit accessible margin distances, leading to poorer prognoses and outcomes (46). Inadequacy of margins with large tumors are often secondary to proximity to deeper, vital structures located within the thoracic cavity and mediastinum. Furthermore, deeper, and larger (>5 cm diameter) tumors are more likely to have local recurrence, metastasis, and mortality (27,51).

Neoadjuvant or adjuvant chemotherapy and RT, though debated, are often used to supplement surgical resection of UPS. The National Cancer Comprehensive indicates that neoadjuvant RT is more effective than its counterpart in treating UPS in the trunk. Some studies have demonstrated that RT reduces local recurrence, and Issakov et al. [2005] found that patients who received adjuvant RT had a 10-year relapse free survival of 62% (15,50,52,53). Comparatively, a 5-year relapse free survival rate of 55% was determined for 100 UPS patients who did not receive RT in a long-term follow-up (54). Cozzolino et al. [2018] described a necessary dosage of at least 60 GyRBE to adequately treat the tumor bed, but it will ultimately need to consider margin distance, tumor size, and grade to best balance risks and benefits (15). According to the National Comprehensive Cancer Network (NCCN), adjuvant RT should only be applied with R1 and R2 resections, since R1/2 resections have demonstrated worse outcomes compared to R0 (55,56). Since margin distance is crucial for limiting recurrence, RT benefits may outweigh the risks when patients do not achieve a margin distance of at least 1.5 cm. As always, a multidisciplinary approach should be applied to determine ideal treatment options for each case.

Broadly speaking, chemotherapy is commonly applied to help treat higher grade (intermediate and high grade) UPS (10). The typical chemotherapy regimens for UPS include anthracycline-based medications, such epirubicin, and ifosfamide (9,40). In comparison to treatment with gemcitabine plus docetaxel, multiple studies have demonstrated greater efficacy with the standard chemotherapy than the histotype-specific regimen with gemcitabine (57-59). Like RT, the use of chemotherapy is debated, but growing evidence suggests that adjuvant chemotherapy may have some benefit in reducing distant recurrence (48).

In regards to the results, there are a few limitations that are important when considering these results. As stated prior, these are case reports and case series of a rare phenomenon, so there is likely some selection bias. As such, there were more reported cases of females with UPS of the breast than males. This outcome could be explained by a few factors with greater selection of female patients, sex-related differences in tumor location, or difficulty identifying tumors early in women due to the proximity to breast tissue. Furthermore, 75% of the patients had a tumor size greater than 5 cm in 1 dimension. Given that 24 of the patients were female, difficulty in noticing small masses in the chest and a lack of notable symptoms could allow tumors to grow >5 cm without being detected.

The Chi-squared test of independence noted no association with recurrence or metastasis and whether the patient received other therapies. However, these are case reports and not random controlled trials, so it is difficult to draw conclusions relative to current literature without proper experimental methods.

Current literature suggests that radiation-associated UPS occurs in 5.2% of UPS cases, but these case reports demonstrate an occurrence of 5/22 for secondary malignancies (22.7%) (60). Consequently, this may further support current evidence that radiation-associated UPS is more commonly found in the chest than other parts of the body, but this data needs to be taken likely given the nature of the reports.

For the purpose of this study, a systematic review was performed with the intention of qualitatively selecting literature for the purpose of providing a cogent direction for future researchers to focus on when considering this entity. Given its rarity and the minimal empirical work done to draw strong conclusions regarding the surgical treatment of this type of tumor, this work was supplemented with information from case reports and series to help fill in some of these gaps. We elected to follow a systematic approach to help keep cases relatively consistent in terms of the presented information in those studies.

It is important to note our review demonstrates characteristics of a limited number of patients. Additionally, we note a possible bias towards primary UPS in our review as many reports only documented primary malignancies, such as Qiu et al. [2013] (27). Beyond the limited number of patients, we utilized case reports given the lack of other study types assessing surgical treatment for these cases. As such, it should be noted there was likely bias and heterogeneity within these publications. We do not intend to draw significant conclusions on better treatment modalities or outcomes, but we do intend to provide possible areas of interest for future research and some of the characteristics we are seeing in patients from our current understanding.

UPS of the trunk and chest wall is increasingly reported in the literature. Margin size, use of chemoradiation, and prior radiation exposure are all areas of ongoing study. These areas should be explored in greater depth in future research to optimize patient outcomes. We also suggest that case reports provide greater details on this information to help researchers draw more effective conclusions. Consultation with a multidisciplinary team is crucial in the care of patients with UPS to review all treatment options and provide individualized care to each patient (61).

Conclusions

Overall, the study aims to enlighten researchers on the current best practices for UPS, specifically involving the chest wall. This study illustrates that are more case reports of women with UPS of the chest wall despite men tending to develop UPS more often in general. Further, there is some discrepancy in proper margin distance, but 1.5 cm may be sufficient in preventing recurrence. However, this study was unable to acquire sufficient information on margin size in these cases to make a definitive conclusion. Additionally, RT and chemotherapy are often recommended in the treatment UPS, but this study found no difference in the recurrence or metastasis of UPS in the chest wall between patients receiving therapy and those who did not. A final point to note is that this study also corroborates previous reports that radiation-associated UPS is more commonly found in the trunk as well.

Acknowledgments

Funding: None.

Footnote

Reporting Checklist: The authors have completed the PRISMA reporting checklist. Available at https://cco.amegroups.com/article/view/10.21037/cco-23-71/rc

Peer Review File: Available at https://cco.amegroups.com/article/view/10.21037/cco-23-71/prf

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://cco.amegroups.com/article/view/10.21037/cco-23-71/coif). A.C. serves as an unpaid editorial board member of Chinese Clinical Oncology from April 2022 to March 2024. The other authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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Cite this article as: Bennett C, Bharadwaj S, Arndt A, Chawla A. A systematic review of undifferentiated pleomorphic sarcoma of the chest wall. Chin Clin Oncol 2023;12(6):66. doi: 10.21037/cco-23-71

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