Prognosis and survival of patients diagnosed with well-differentiated and dedifferentiated retroperitoneal liposarcoma

Osuna-Soto, Julio; Cuenca, Teresa Caro; Sanz-Zorrilla, Alicia; Torrecilla-Martínez, Antonio; Salas, Rosa Ortega; Leiva-Cepas, Fernando

doi:10.1016/j.cireng.2022.06.034

Cited by 3 publications

(4 citation statements)

References 24 publications

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“…Surgical series of DDLPSs are scarce, given the rarity of these tumors and the tendency of grouping them with other RPSs. A recent cohort of 32 patients undergoing resection (18 DDLPSs vs. 14 WDLPS) showed a significant difference in recurrence-free survival times at 2 years (26% vs. 59%, respectively; P= 0.005), but could not confirm a difference in OS (40). Local recurrence of DDLPS develops four times more frequently compared with WDLPS (38), especially if the dedifferentiated component is found at the resection margin (15,(35)(36)(37)41).…”

Section: Discussionmentioning

confidence: 94%

R0 surgical resection of giant dedifferentiated retroperitoneal liposarcomas in the COVID era with and without nephrectomy: A case report

Pegoraro

Santangelo

et al. 2023

Oncol Lett

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Retroperitoneal sarcomas (RPSs) are rare findings that can grow into large masses without eliciting severe symptoms. At present, surgical resection is the only radical therapy, whenever it can be performed with the aim to achieve a complete removal of the tumor. The present report describes two consecutive cases of RPSs that resulted in dedifferentiated liposarcomas (DDLPSs) and these patients underwent R0 surgical resection with and without a nephron-sparing procedure. The diagnostic workup, the surgical approach, the impact of late surgical management due to the COVID pandemic and the latest literature on the topic are discussed and analyzed. The patients, who refused to undergo any medical examination during the prior 2 years due to the COVID pandemic, were admitted to Federico II University Hospital (Naples, Italy) complaining about weight loss and general abdominal discomfort. In the first case, a primitive giant abdominal right neoplasm of retroperitoneal origin enveloping and medializing nosis. When possible, surgical management of recurrences or incompletely resected masses must be pursued. Since the COVID pandemic caused limited medicalization of a number of population groups and delayed diagnosis of other oncologic diseases, an increased number of DDLPSs could be expected in the near future.

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Section: Discussionmentioning

confidence: 94%

R0 surgical resection of giant dedifferentiated retroperitoneal liposarcomas in the COVID era with and without nephrectomy: A case report

Pegoraro

Santangelo

et al. 2023

Oncol Lett

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“…Liposarcoma (LPS) tumors can vary extensively based on their grade of differentiation [ 1 , 2 , 9 , 10 , 11 , 12 ]. While there are five described subtypes of liposarcoma, we sought to better understand the underlying molecular differences between the well-differentiated (WDLPS) and dedifferentiated (DDLPS) subtypes that can cause varying degrees of tumor differentiation, which can be a contributing factor to clinical prognosis [ 3 ]. Using transcriptomics data collected from the Gene Expression Omnibus (GEO [ 6 ]) ( Figure 1 A), we evaluated differentially expressed genes (DEGs) between adipose progenitors and mature adipocytes (GSE20697 [ 13 ]) ( Figure 1 B), followed by DEGs upregulated in either WDLPS or DDLPS tumors (GSE30929 [ 14 ]) ( Figure 1 C).…”

Section: Resultsmentioning

confidence: 99%

“…Liposarcomas (LPSs) are soft tissue malignant neoplasms that have the greatest incidence amongst all soft tissue sarcomas [ 1 , 2 , 3 ]. The five histological subtypes of LPS consist of well-differentiated (WDLPS)/atypical lipomatous tumors (ALTs), dedifferentiated (DDLPS), myxoid, pleomorphic, and round cell, with the cell of origin and genetic abnormalities varying extensively across the different subtypes.…”

Section: Introductionmentioning

confidence: 99%

Bioinformatics Screen Reveals Gli-Mediated Hedgehog Signaling as an Associated Pathway to Poor Immune Infiltration of Dedifferentiated Liposarcoma

Beadle

Bennett

Rhoades

2023

Cancers

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Liposarcomas are the most diagnosed soft tissue sarcoma, with most cases consisting of well-differentiated (WDLPS) or dedifferentiated (DDLPS) histological subtypes. While both tumor subtypes can have clinical recurrence due to incomplete resections, DDLPS often has worse prognosis due to a higher likelihood of metastasis compared to its well-differentiated counterpart. Unfortunately, targeted therapeutic interventions have lagged in sarcoma oncology, making the need for molecular targeted therapies a promising future area of research for this family of malignancies. In this work, previously published data were analyzed to identify differential pathways that may contribute to the dedifferentiation process in liposarcoma. Interestingly, Gli-mediated Hedgehog signaling appeared to be enriched in dedifferentiated adipose progenitor cells and DDLPS tumors, and coincidentally Gli1 is often co-amplified with MDM2 and CDK4, given its genomic proximity along chromosome 12q13-12q15. However, we find that Gli2, but not Gli1, is differentially expressed between WDLPS and DDLPS, with a noticeable co-expression signature between Gli2 and genes involved in ECM remodeling. Additionally, Gli2 co-expression had a noticeable transcriptional signature that could suggest Gli-mediated Hedgehog signaling as an associated pathway contributing to poor immune infiltration in these tumors.

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“…Surgical series of DDLPSs are scarce, given the rarity of these tumors and the tendency of grouping them with other RPSs. A recent cohort of 32 patients undergoing resection (18 DDLPSs vs. 14 WDLPS) showed a signi cant difference in recurrence-free survival times at 2 years (26% vs. 59%, respectively; p=0.005), but could not con rm a difference in OS (37). Local recurrence of DDLPS develops four times more frequently compared with WDLPS (35), especially if the dedifferentiated component is found at the resection margin (15,(32)(33)(34)38).…”

Section: Discussionmentioning

confidence: 96%

R0 Surgical Resection of Giant Dedifferentiated Retroperitoneal Liposarcomas in the COVID era: Nephrectomy or Not Nephrectomy?

Pegoraro

Santangelo

Alfonso

et al. 2022

Preprint

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Background: Retroperitoneal sarcomas (RPS) are rare findings that can grow into large masses without eliciting severe symptoms. Nowadays surgical resection is the only radical therapy, whenever possible. We present two consecutive cases of RPSs that resulted in dedifferentiated liposarcomas (DDLPS) and underwent R0 surgical resection with and without a nephron-sparing procedure. The diagnostic workup, the surgical approach, the impact of late surgical management due to the COVID pandemic, and the latest literature on the topic will be discussed and analyzed. Case Presentation: The patients, who refused to undergo any medical examination during the prior two years due to the COVID pandemic, were admitted to our Institution complaining about weight loss and general abdominal discomfort. In the first case, we observed a primitive giant abdominal right neoplasm of retroperitoneal origin enveloping and medializing the right kidney. The second patient had a similar primitive retroperitoneal giant left neoplasm which didn’t affect the kidney. Given the masses’ characteristics and the absence of distant metastases, after a multidisciplinary discussion, radical surgical removal was carried out for both patients. The lesions appeared well-defined from the surrounding tissues, and significantly compressed all the adjacent organs, without signs of infiltration. In the first patient, the right kidney was surrounded and undetachable from the tumor and it was removed en bloc with the mass. The second patient benefited from a nephron-sparing resection, thanks to the existence of a clear cleavage plane. The postoperative courses were uneventful. Both the histological examinations were oriented towards a DDLPS. And both patients were addressed with adjuvant chemotherapy. Conclusions: The treatment of giant RPS is still challenging and requires multidisciplinary treatment as well as, when possible, radical surgical removal. The lack of tissue infiltration and the avoidance of major organs’ excision or reconstruction (including the kidney) could lead to an easier postoperative course and a better prognosis. When possible, surgical management of recurrences or incompletely resected masses must be pursued. As the COVID pandemic caused limited medicalization of many population groups and delayed diagnosis of other oncologic diseases, an increased number of DDLPSs could be expected in the near future.

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Prognosis and survival of patients diagnosed with well-differentiated and dedifferentiated retroperitoneal liposarcoma

Cited by 3 publications

References 24 publications

R0 surgical resection of giant dedifferentiated retroperitoneal liposarcomas in the COVID era with and without nephrectomy: A case report

R0 surgical resection of giant dedifferentiated retroperitoneal liposarcomas in the COVID era with and without nephrectomy: A case report

Bioinformatics Screen Reveals Gli-Mediated Hedgehog Signaling as an Associated Pathway to Poor Immune Infiltration of Dedifferentiated Liposarcoma

R0 Surgical Resection of Giant Dedifferentiated Retroperitoneal Liposarcomas in the COVID era: Nephrectomy or Not Nephrectomy?

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