What’s new in adipocytic neoplasia?

Thway, Khin

doi:10.1111/his.14548

Cited by 13 publications

(10 citation statements)

References 187 publications

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“…Keratin expression is seen focally in ~20%. Epithelial membrane antigen, keratin, and melan A are more common in the epithelioid variant 21,23,32 . An entity that should be mentioned is myxoid PLPS, a rare and highly aggressive LPS initially reported as being most common in children, adolescents, and young adults that has been added in the most recent World Health Organization classification 33 .…”

Section: Discussionmentioning

confidence: 99%

“…Epithelial membrane antigen, keratin, and melan A are more common in the epithelioid variant. 21,23,32 An entity that should be mentioned is myxoid PLPS, a rare and highly aggressive LPS initially reported as being most common in children, adolescents, and young adults that has been added in the most recent World Health Organization classification. 33 Nonetheless, a median age of 35 years in a recent study by Creytens et al 34 showed that myxoid PLPS may occur in patients older than previously appreciated.…”

Section: Discussionmentioning

confidence: 99%

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Pleomorphic liposarcoma: A clinicopathologic study of 20 FNA cases

Wakely

Wangsiricharoen

Ali

2022

Cancer Cytopathology

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BACKGROUND Pleomorphic liposarcoma (PLPS) is the least common but most aggressive of all forms of liposarcoma (LPS). Its diagnosis relies on the recognition of pleomorphic lipoblasts (PLBs), whose numbers vary considerably. Because few large fine‐needle aspiration (FNA) biopsy studies exist, the authors review their experience with PLPS. METHODS The authors’ cytopathology files were searched for PLPS with histopathologic verification. FNA biopsy smears were performed via standard techniques. RESULTS Twenty cases from 20 patients (male/female ratio, 2.3/1; age range, 22‐77 years; mean age, 58 years) met the inclusion criteria. All had tissue confirmation. Biopsy sites included the following: thigh (11 [55%]), upper extremity (4 [20%]), axilla (2 [10%]), neck (1 [5%]), chest wall (1 [5%]), and mediastinum (1 [5%]). Aspirates were from primary (17 [85%]), locally recurrent (2 [10%]), and metastatic neoplasms (1 [5%]). The FNA diagnoses were PLPS (10 [50%]), myxofibrosarcoma (4 [20%]), LPS (2 [10%]), sarcoma (2 [10%]), and high‐grade malignant neoplasm (2 [10%]). Smears showed thick cell clusters and dissociated single forms. Pleomorphic, epithelioid, and bizarre cell/nuclear shapes were common. PLBs were absent, rare, or unnoticed in 45%. In 25%, smears dominated by myxoid stroma were diagnosed as high‐grade myxofibrosarcoma or myxoid LPS. Ancillary testing performed in 5 cases had limited diagnostic efficacy. CONCLUSIONS FNA biopsy of PLPS, although able to successfully recognize malignancy, suffers from a sampling bias due to an inability to capture or recognize PLBs in a significant proportion of cases secondary to the heterogeneous composition of this neoplasm.;

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Pleomorphic liposarcoma: A clinicopathologic study of 20 FNA cases

Wakely

Wangsiricharoen

Ali

2022

Cancer Cytopathology

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“…ASPLTs show molecular differences from SCL and PL, with typically more complex deletions of the 13q region and further deletions/losses of genes flanking RB1. However, there is a possibility that these features may represent a disease continuum [ 29 , 31 , 32 , 33 ]. ASPLT remains an evolving entity with a need for further understanding.…”

Section: Adipocytic Tumorsmentioning

confidence: 99%

The Recent Advances in Molecular Diagnosis of Soft Tissue Tumors

Choi

2023

IJMS

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Soft tissue tumors are rare mesenchymal tumors with divergent differentiation. The diagnosis of soft tissue tumors is challenging for pathologists owing to the diversity of tumor types and histological overlap among the tumor entities. Present-day understanding of the molecular pathogenesis of soft tissue tumors has rapidly increased with the development of molecular genetic techniques (e.g., next-generation sequencing). Additionally, immunohistochemical markers that serve as surrogate markers for recurrent translocations in soft tissue tumors have been developed. This review aims to provide an update on recently described molecular findings and relevant novel immunohistochemical markers in selected soft tissue tumors.

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“…More than 90% of MLPS tumours contain fusion-genes (FUS-DDIT3 and EWSR1-DDIT3) that drive tumorigenesis [1,2,16]. Moreover, MLPS tumours frequently have upregulation of the IGF1R/PI3K/Akt signaling pathway, and targeting these pathway alterations with PI3K and mTOR inhibitors is being investigated [1,[17][18][19][20][21]. The highly aggressive PLPS is the rarest subtype and accounts for approximately 5% of all LPS.…”

Section: Introductionmentioning

confidence: 99%

Therapeutic opportunity of phosphodiesterase 3A modulators in myxoid liposarcomas

Toivanen

Kilpinen

Ojala

et al. 2022

Preprint

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Liposarcoma is a common soft tissue sarcoma, and is classified into four histological subtypes, each with distinct disease outcome, morphology, and genotype. Typical primary treatment in advanced liposarcoma consists of surgery, combined with chemotherapy, radiotherapy, or both. There are currently no approved targeted therapeutics or precision medicine opportunities in liposarcoma. Here we investigated 131 liposarcoma tumour samples by transcriptional profiling and 543 sarcomas by immunoprofiling and compared these data with high-throughput drug sensitivity testing in liposarcoma cell lines in vitro. The data showed that i) myxoid liposarcomas, specifically the high-grade phenotype, display high levels of PDE3A gene expression and that ii) PDE3A modulators are effective inhibitors in PDE3A and SLFN12 co-expressing soft tissue sarcoma cell lines. iii) In addition, PDE3A modulators synergize with many emerging targeted therapies and sensitize sarcoma cell lines to Bcl-2 family inhibitors. We suggest that overexpression of PDE3A and SLFN12 genes is common in myxoid liposarcomas and that this leads to sensitivity to PDE3A modulators and synergy with Bcl-2 family inhibition. Further studies are warranted to translate these observations into precision liposarcoma therapy.

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What’s new in adipocytic neoplasia?

Cited by 13 publications

References 187 publications

Pleomorphic liposarcoma: A clinicopathologic study of 20 FNA cases

Pleomorphic liposarcoma: A clinicopathologic study of 20 FNA cases

The Recent Advances in Molecular Diagnosis of Soft Tissue Tumors

Therapeutic opportunity of phosphodiesterase 3A modulators in myxoid liposarcomas

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