-
Something wrong with this record ?
Sclerosing epithelioid fibrosarcoma of bone: morphological, immunophenotypical, and molecular findings of 9 cases
K. Kosemehmetoglu, F. Ardic, SE. Kilpatrick, U. Aydingoz, VP. Sumathi, M. Michal
Language English Country Germany
Document type Journal Article
NLK
ProQuest Central
from 2003-01-01 to 1 year ago
Medline Complete (EBSCOhost)
from 2011-01-01 to 1 year ago
Nursing & Allied Health Database (ProQuest)
from 2003-01-01 to 1 year ago
Health & Medicine (ProQuest)
from 2003-01-01 to 1 year ago
- MeSH
- Adult MeSH
- Phenotype MeSH
- Fibrosarcoma diagnosis genetics metabolism pathology MeSH
- Gene Rearrangement MeSH
- Immunohistochemistry MeSH
- Middle Aged MeSH
- Humans MeSH
- Neoplasm Recurrence, Local diagnosis genetics metabolism pathology MeSH
- Neoplasm Metastasis MeSH
- Adolescent MeSH
- Young Adult MeSH
- Biomarkers, Tumor genetics metabolism MeSH
- Bone Neoplasms diagnosis genetics metabolism pathology MeSH
- Follow-Up Studies MeSH
- Aged MeSH
- Transcription Factors metabolism MeSH
- Matrix Attachment Region Binding Proteins metabolism MeSH
- High-Throughput Nucleotide Sequencing MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
Primary sclerosing epithelioid fibrosarcoma (SEF) of bone is a rare and scarcely reported neoplasm. We document clinicopathological and molecular features of 9 additional cases. Five males and 4 females had a mean age of 39 years (14-71 years). Most tumors affected flat/irregular bones; only 3 cases involved a long bone. By radiology, it has characteristic radiographic features of a predominantly lytic expansile lesion with a sclerotic rim. Referring diagnoses were SEF (n = 2), low-grade osteosarcoma (n = 2), chondrosarcoma (n = 1), and chondromyxoid fibroma (n = 1). Histologically, five cases revealed classical morphology of SEF of soft tissue. Remaining cases were classified as hybrid SEF/low-grade fibromyxoid sarcoma, characterized by spindle or stellate cells, prominent stroma, and giant hyalinized areas. Various morphological deviations such as prominent vasculature (n = 3), osteoid-like material (n = 4), or parallel bone trabeculae (n = 2) were observed. Immunohistochemically, all cases showed diffuse and strong MUC4 expression. SATB2 was observed in 5/8 cases. Using FISH, EWSR1, and FUS rearrangements were detected in 4 cases and 1 case, respectively. EWSR1-CREB3L1 fusion was identified in 1 additional case by next-generation sequencing. Recurrence and metastasis were observed in 1 case and 2 cases, respectively. All but one patient were alive with disease for a mean interval of 31 months. SEF of bone is a relatively indolent sarcoma of adults, most commonly located in the flat/irregular bones. Due to overlapping histological features, it is often misdiagnosed as osteosarcoma or a chondroid tumor. Most SEF of bone exhibit EWSR1 rearrangements, but rare cases may harbor a FUS gene fusion.
Abdurrahman Yurtaslan Oncology Research and Training Hospital Ankara Turkey
Bioptical Laboratory Ltd Plzen Czech Republic
Cleveland Clinic Cleveland OH USA
Department of Pathology Faculty of Medicine in Plzen Charles University Plzen Czech Republic
Department of Pathology Hacettepe University Ankara Turkey
References provided by Crossref.org
- 000
- 00000naa a2200000 a 4500
- 001
- bmc21018974
- 003
- CZ-PrNML
- 005
- 20210830100532.0
- 007
- ta
- 008
- 210728s2021 gw f 000 0|eng||
- 009
- AR
- 024 7_
- $a 10.1007/s00428-020-02953-y $2 doi
- 035 __
- $a (PubMed)33084978
- 040 __
- $a ABA008 $b cze $d ABA008 $e AACR2
- 041 0_
- $a eng
- 044 __
- $a gw
- 100 1_
- $a Kosemehmetoglu, Kemal $u Department of Pathology, Hacettepe University, Ankara, Turkey. dokemal@hotmail.com
- 245 10
- $a Sclerosing epithelioid fibrosarcoma of bone: morphological, immunophenotypical, and molecular findings of 9 cases / $c K. Kosemehmetoglu, F. Ardic, SE. Kilpatrick, U. Aydingoz, VP. Sumathi, M. Michal
- 520 9_
- $a Primary sclerosing epithelioid fibrosarcoma (SEF) of bone is a rare and scarcely reported neoplasm. We document clinicopathological and molecular features of 9 additional cases. Five males and 4 females had a mean age of 39 years (14-71 years). Most tumors affected flat/irregular bones; only 3 cases involved a long bone. By radiology, it has characteristic radiographic features of a predominantly lytic expansile lesion with a sclerotic rim. Referring diagnoses were SEF (n = 2), low-grade osteosarcoma (n = 2), chondrosarcoma (n = 1), and chondromyxoid fibroma (n = 1). Histologically, five cases revealed classical morphology of SEF of soft tissue. Remaining cases were classified as hybrid SEF/low-grade fibromyxoid sarcoma, characterized by spindle or stellate cells, prominent stroma, and giant hyalinized areas. Various morphological deviations such as prominent vasculature (n = 3), osteoid-like material (n = 4), or parallel bone trabeculae (n = 2) were observed. Immunohistochemically, all cases showed diffuse and strong MUC4 expression. SATB2 was observed in 5/8 cases. Using FISH, EWSR1, and FUS rearrangements were detected in 4 cases and 1 case, respectively. EWSR1-CREB3L1 fusion was identified in 1 additional case by next-generation sequencing. Recurrence and metastasis were observed in 1 case and 2 cases, respectively. All but one patient were alive with disease for a mean interval of 31 months. SEF of bone is a relatively indolent sarcoma of adults, most commonly located in the flat/irregular bones. Due to overlapping histological features, it is often misdiagnosed as osteosarcoma or a chondroid tumor. Most SEF of bone exhibit EWSR1 rearrangements, but rare cases may harbor a FUS gene fusion.
- 650 _2
- $a mladiství $7 D000293
- 650 _2
- $a dospělí $7 D000328
- 650 _2
- $a senioři $7 D000368
- 650 _2
- $a nádorové biomarkery $x genetika $x metabolismus $7 D014408
- 650 _2
- $a nádory kostí $x diagnóza $x genetika $x metabolismus $x patologie $7 D001859
- 650 _2
- $a ženské pohlaví $7 D005260
- 650 _2
- $a fibrosarkom $x diagnóza $x genetika $x metabolismus $x patologie $7 D005354
- 650 _2
- $a následné studie $7 D005500
- 650 _2
- $a genová přestavba $7 D015321
- 650 _2
- $a vysoce účinné nukleotidové sekvenování $7 D059014
- 650 _2
- $a lidé $7 D006801
- 650 _2
- $a imunohistochemie $7 D007150
- 650 _2
- $a mužské pohlaví $7 D008297
- 650 _2
- $a vazebné proteiny DNA v oblastech připojení k matrix $x metabolismus $7 D036961
- 650 _2
- $a lidé středního věku $7 D008875
- 650 _2
- $a metastázy nádorů $7 D009362
- 650 _2
- $a lokální recidiva nádoru $x diagnóza $x genetika $x metabolismus $x patologie $7 D009364
- 650 _2
- $a fenotyp $7 D010641
- 650 _2
- $a transkripční faktory $x metabolismus $7 D014157
- 650 _2
- $a mladý dospělý $7 D055815
- 655 _2
- $a časopisecké články $7 D016428
- 700 1_
- $a Ardic, Fisun $u Abdurrahman Yurtaslan Oncology Research and Training Hospital, Ankara, Turkey
- 700 1_
- $a Kilpatrick, Scott E $u Cleveland Clinic, Cleveland, OH, USA
- 700 1_
- $a Aydingoz, Ustun $u Department of Radiology, Hacettepe University, Ankara, Turkey
- 700 1_
- $a Sumathi, Vaiyapuri P $u Royal Orthopaedic Hospital, Birmingham, West Midlands, UK
- 700 1_
- $a Michal, Michael $u Department of Pathology, Faculty of Medicine in Plzen, Charles University, Plzen, Czech Republic $u Bioptical Laboratory, Ltd., Plzen, Czech Republic
- 773 0_
- $w MED00004660 $t Virchows Archiv : an international journal of pathology $x 1432-2307 $g Roč. 478, č. 4 (2021), s. 767-777
- 856 41
- $u https://pubmed.ncbi.nlm.nih.gov/33084978 $y Pubmed
- 910 __
- $a ABA008 $b sig $c sign $y p $z 0
- 990 __
- $a 20210728 $b ABA008
- 991 __
- $a 20210830100533 $b ABA008
- 999 __
- $a ok $b bmc $g 1689913 $s 1139420
- BAS __
- $a 3
- BAS __
- $a PreBMC
- BMC __
- $a 2021 $b 478 $c 4 $d 767-777 $e 20201021 $i 1432-2307 $m Virchows Archiv $n Virchows Arch $x MED00004660
- LZP __
- $a Pubmed-20210728
