Liposarcoma in children and young adults: a clinicopathologic and molecular study of 23 cases in one of the largest institutions of China

Peng, Ran; Li, Nan; Lan, Ting; Chen, Huijiao; Du, Tianhai; He, Xin; Chen, Min; Xie, You; Zhang, Zhang; Zhao, Wei; Zhang, Hongying

doi:10.1007/s00428-021-03076-8

Cited by 11 publications

(38 citation statements)

References 55 publications

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“…15 Moreover, a subset of myxoid pleomorphic liposarcomas show TP53 alterations, and myxoid pleomorphic liposarcoma has been observed in the setting of Li-Fraumeni syndrome. 16,17 Despite these similarities between APLT and myxoid pleomorphic liposarcoma, the latter is distinguished diagnostically by the presence of both myxoid liposarcoma-like areas, with abundant myxoid stroma and prominent capillary-type blood vessels, and pleomorphic liposarcoma-like areas, with high cellularity and increased mitotic activity. 15 Case 8 is unique, given the finding of a pathogenic PTEN variant in addition to an RB1 mutation in a tumour from a child that was originally classified as ALT/WDLPS.…”

Section: Discussionmentioning

confidence: 99%

Well‐differentiated lipomatous neoplasms with p53 alterations: a clinicopathological and molecular study of eight cases with features of atypical pleomorphic lipomatous tumour

et al. 2022

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Well-differentiated lipomatous neoplasms with p53 alterations: a clinicopathological and molecular study of eight cases with features of atypical pleomorphic lipomatous tumour Aims: Well-differentiated lipomatous neoplasms encompass a broad spectrum of benign and malignant tumours, many of which are characterised by recurrent genetic abnormalities. Although a key regulator of p53 signalling, MDM2, is characteristically amplified in well-differentiated liposarcoma, recurrent abnormalities of p53 itself have not been reported in well-differentiated adipocytic neoplasms. Here, we present a series of well-differentiated lipomatous tumours characterised by p53 alterations and histological features in keeping with atypical pleomorphic lipomatous tumour (APLT). Methods and results: We reviewed the morphological, immunohistochemical and molecular genetic features of eight lipomatous tumours with p53 alterations. Four tumours arose in the thigh, and one case each arose in the shoulder, calf, upper back, and subclavicular regions; six tumours were deep/subfascial and two were subcutaneous. Relevant clinical history included two patients with Li-Fraumeni syndrome. Morphologically, all cases showed well-differentiated adipocytes with prominent nuclear pleomorphism, limited mitotic activity, and no tumour cell necrosis. All cases were negative for MDM2 overexpression and amplification as determined with immunohistochemistry and fluorescence in-situ hybridisation, respectively. Immunohistochemically, p16 was diffusely overexpressed in all cases; seven tumours (88%) showed abnormal loss of Rb and p53. TP53 mutation or deletion was identified in four of six tumours evaluated with exon-targeted hybrid capture-based massively parallel sequencing; RB1 mutation or deletion was present in five of six cases. Conclusions: We present a series of eight welldifferentiated lipomatous neoplasms characterised by p53 alterations in addition to Rb loss and histological features of APLT. These findings suggest that impaired p53 signalling may contribute to the pathogenesis of APLT in a subset of cases.

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Section: Discussionmentioning

confidence: 99%

Well‐differentiated lipomatous neoplasms with p53 alterations: a clinicopathological and molecular study of eight cases with features of atypical pleomorphic lipomatous tumour

et al. 2022

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“…The commonest soft tissue sarcomas, liposarcomas account for ˜20% of all malignant mesenchymal neoplasms, 82 with the vast majority arising in adults, and the small proportion in younger patients occurring mostly in the second decade. Myxoid pleomorphic liposarcoma (MPL), described below, is a newly delineated, highly aggressive subtype that characteristically occurs in young patients (˜13% of paediatric LPS) 83 . LPSs in young patients have tended to occur overwhelmingly in the extremities (69.6%), followed by the head and neck, chest, and waist (each 8.7%), and rarely in the retroperitoneum (4.3%).…”

Section: Lipoma Variantsmentioning

confidence: 99%

“…LPSs in young patients have tended to occur overwhelmingly in the extremities (69.6%), followed by the head and neck, chest, and waist (each 8.7%), and rarely in the retroperitoneum (4.3%). This group is predictably dominated by myxoid LPS with DDIT3 rearrangements, and typically has an excellent prognosis, 84 as high‐grade (>5% hypercellularity) MLPS appears to be extremely rare (approximately 2–8% of paediatric MLPSs 84–86 in contrast to >40% of adult MLPS) 83,84,87–89 . WDL/DDL and MPL remain rare, 90 and pleomorphic LPS is extremely infrequent (2–4% of paediatric LPSs) 83,91–93 .…”

Section: Lipoma Variantsmentioning

confidence: 99%

“…This group is predictably dominated by myxoid LPS with DDIT3 rearrangements, and typically has an excellent prognosis, 84 as high‐grade (>5% hypercellularity) MLPS appears to be extremely rare (approximately 2–8% of paediatric MLPSs 84–86 in contrast to >40% of adult MLPS) 83,84,87–89 . WDL/DDL and MPL remain rare, 90 and pleomorphic LPS is extremely infrequent (2–4% of paediatric LPSs) 83,91–93 . LPSs are biologically and morphologically highly heterogeneous, and the concept of spindle cell LPS has been previously discussed, with genetic and further clinicopathological studies enabling many of these to be more precisely characterised as WDL, DDL, or ASPLT.…”

Section: Lipoma Variantsmentioning

confidence: 99%

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What’s new in adipocytic neoplasia?

Thway

2021

Histopathology

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Adipocytic tumours are among the most common mesenchymal neoplasms, and constitute a clinically, biologically and pathologically diverse group. Their wide histological spectrum and frequent morphological overlap have made classification and diagnosis challenging, with accurate classification being critical because of the considerable differences in prognosis and management between morphologically overlapping neoplasms. Ongoing advances in molecular genetics have aided significantly to our understanding of these neoplasms, with continuing evolution in classification. This review summarises the new developments in benign and malignant adipocytic neoplasms, with discussion of new entities and genetic findings, updates on the clinical and morphological spectrum, and the use of diagnostic immunohistochemistry and molecular markers in the differential diagnosis.

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“…MDM2 and CKD4 amplifications were seen in WDLPS and DDLPS. One patient with PLPS 20 and one with MPLPS had somatic Rb1 mutations. 24 Germline p53 mutations were noted in two patients with MPLPS, 21,32 a single patient with PLPS, and two patients with WDLPS met family history criteria for Li-Fraumeni syndrome.…”

Section: Literature Reviewmentioning

confidence: 99%

Pediatric liposarcoma: A case series and literature review

Baday

Navai

Hicks

et al. 2021

Pediatric Blood & Cancer

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Liposarcoma is arare soft tissue sarcoma in children. While prognosis, clinical behavior, and response to therapy among the various histologic subtypes are well described in adults, data in children are limited. Here, we describe our experience treating 14 children with liposarcoma at a large, academic pediatric center and review the available pediatric literature. This comprehensive report adds treatment, survival, and genomic data to pediatric liposarcoma literature.

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Liposarcoma in children and young adults: a clinicopathologic and molecular study of 23 cases in one of the largest institutions of China

Cited by 11 publications

References 55 publications

Well‐differentiated lipomatous neoplasms with p53 alterations: a clinicopathological and molecular study of eight cases with features of atypical pleomorphic lipomatous tumour

Well‐differentiated lipomatous neoplasms with p53 alterations: a clinicopathological and molecular study of eight cases with features of atypical pleomorphic lipomatous tumour

What’s new in adipocytic neoplasia?

Pediatric liposarcoma: A case series and literature review

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