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Zhuang et al. | Journal of Clinical and Translational Research 2024; 10(1): 62-71 63
1. Introduction its role as a prognostic factor [18]. Elevated levels of SIRT1
and VEGF are linked to unfavorable clinical characteristics and
Retroperitoneal soft-tissue sarcoma (RPS) accounts for prognosis, suggesting SIRT1 as a potential therapeutic target [19].
approximately 15% of all soft-tissue malignancies, whereas Finally, modulation of FGFR signaling and its inhibitors show
myxoid/round cell liposarcoma (MLPS/RCLPS) represents less promise in high-grade liposarcoma treatment, which highlights
than 5% of all RPS [1-4]. MLPS/RCLPS is the most prevalent the potential of developing targeted therapies [20]. Moreover,
lipomatous malignancy in children and adolescents [5,6]. The CXCR4 and AXL are emerging as promising therapeutic targets
onset of MLPS/RCLPS occurs earlier than that of other subtypes in the management of aggressive MLPS behavior.
of liposarcoma and reaches its incidence peak in middle age. Although it has been known for some time that the prognosis
The diagnosis of MLPS/RCLPS is definitive due to its
distinctive morphology, which is rarely mistaken for other of MLPS/RCLPS of different primary sites varies [21,22], there is
currently no prognostic study on MLPS/RCLPS of retroperitoneal
monomorphic soft tissue tumors with myxoid stromal and origin and no prognostic tool. Therefore, we analyzed the
lipomatous differentiation [7]. In addition, specific chromosomal surveillance, epidemiology, and end results (SEER) database, which
translocations were identified, including FUS and CHOP gene provides data from 17 geographically variable cancer registries
fusions [(t12;16)(q13;p11)] and EWS and CHOP gene fusions representing approximately 26% of the U.S. population [21,22],
[(t12;22)(q13;q12)] in 90% of tumors in >5% of tumors. The to investigate the DSS-related prognostic factors in MLPS and to
detection of these translocations with polymerase chain reaction attempt to develop a prognosis nomogram prediction model.
(PCR) techniques enables pathologists to make precise diagnoses
in difficult cases [8,9]. RCLPS refers to MLPS with round 2. Materials and Methods
cells, accounting for more than 5% of all cases [7]. In terms of
aggressiveness, RCLPS has a worse prognosis than MLPS [10]. Using SEER*Stat 8.4.0.1, patients diagnosed with MLPS/
MLPS/RCLPS is distinguished from other soft-tissue sarcomas RCLPS between 2000 and 2019 were identified from the SEER
by a number of characteristics. First, it is more susceptible to database, in which all cases were reported from the United States.
extrapulmonary metastases than other sarcomas [11,12]; second, The following were the criteria for inclusion: (1) the International
it is more sensitive to radiotherapy and chemotherapy than other Classification of Diseases (ICD) code O-3 morphology 8852
liposarcomas [13]; third, the prognosis is favorable, with the or 8853; (2) the primary site recodes of ICD-O-3 was the
5-year disease-specific survival (DSS) rate for local diseases retroperitoneum; and (3) active patient monitoring to ensure a
exceeding 90% [13]. reliable patient status. The following were the criteria for exclusion:
Several crucial prognostic factors impact patient survival, (1) patients with non-primary tumor and (2) patients younger
including both distant and local recurrence. Key factors include than 18 years old. Myxoid/Round cell liposarcoma is diagnosed
the completeness and negative margins of surgical resection, through a combination of histological, immunohistochemical,
histological grade reflecting differentiation in myxoid/round and genetic examinations. Pathologically, it is characterized by
cell liposarcoma patients [14], patient age, and the role of tumor abundant myxoid stroma and a round cell component, with varying
biomarkers for treatment monitoring, prognosis assessment, early degrees of lipogenic differentiation. Immunohistochemically,
diagnosis, and treatment prediction. these tumors typically express S-100 protein, CDK4, and MDM2.
Furthermore, analysis suggests that high FGF-21 expression A critical aspect of the diagnosis is the identification of hallmark
improves prognosis [15]. Multivariate analysis considers genetic alterations, particularly the FUS-CHOP or EWS-CHOP
clinicopathological factors, such as tumor site, round cell (RC) fusion genes, often detected through molecular tests like reverse-
components, high MIB-1 labeling index, and p53 missense transcription PCR or fluorescence in situ hybridization.
mutation as unfavorable indicators. In cases of MLS/RCLS, The primary endpoint of this study was DSS. We collected
reduced p14 protein expression and p53 mutations associate with and analyzed data on gender, age, marital status, race, history of
poor prognosis. In addition, the RC component is identified as a previous tumors, the interval between diagnosis and treatment,
negative prognostic factor, potentially involving the p14ARF/p53 presence of metastatic disease, histologic subtypes, tumor
pathway in its development [16]. differentiation, tumor size, and treatment methods including
Commonly mutated genes such as TP53, NF1, and PIK3CA are radiotherapy, chemotherapy, and surgery. Information regarding
identified in STS through genome studies. PIK3CA mutations, more the interval between diagnosis and treatment and tumor size was
frequent in myxoid/round cell and pleomorphic tumors compared missing for 20 (11.7%) and 21 (12.3%) patients, respectively.
to well-differentiated/dedifferentiated tumors, suggest PIK3CA as Given the rarity of retroperitoneal MLPS/RCLPS, we chose not to
a potential driver gene and therapeutic target. Survival analysis exclude these patients, instead substituting the missing values with
reveals that patients with increased PIK3CA copy numbers have their respective medians (1 month, 20 cm). All the aforementioned
worse prognosis, highlighting its significance [17]. NY-ESO-1’s variables were included in the univariate Cox model analysis.
association with higher tumor grade and shorter survival establishes Variables with P < 0.1 were further included in the multivariate
it as a valuable prognostic marker for myxoid liposarcoma. In analysis. Variables with P < 0.05 in the Cox multivariate regression
addition, PRAME’s high expression is correlated with unfavorable model were selected for the nomogram prediction model. The
prognosis and elevated levels in myxoid liposarcoma, indicating accuracy of the nomogram was subsequently validated using the
DOI: https://doi.org/10.36922/jctr.00113