Evidence map›Paper›PMID 42747556›Full record

ArticleSkeletal radiology2026

Perivascular epithelioid cell (PEComa) tumors of the musculoskeletal system: a case series with MR imaging and histopathologic review.

Bryan W Buckley, Lawrence M White, Elizabeth G Demicco, Brendan C Dickson, Ali Naraghi, Angela Atinga, Anthony M Griffin, Abdulazeez Salawu, Aaron M Gazendam, Kim M Tsoi

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Article in Skeletal radiology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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2 · The registry

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4 · The record

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5 · Who and what money

Authors and funding

10 authors.

Bryan W BuckleyDepartment of Medical Imaging, Division of Musculoskeletal Imaging, Temerty Faculty of Medicine, University of Toronto, 4 Floor, 263 McCaul Street, Toronto, ON, M5T 1W7, Canada.
Lawrence M WhiteDepartment of Medical Imaging, Division of Musculoskeletal Imaging, Temerty Faculty of Medicine, University of Toronto, 4 Floor, 263 McCaul Street, Toronto, ON, M5T 1W7, Canada. lawrence.white@uhn.ca.ORCID http://orcid.org/0000-0001-6670-1756
Elizabeth G DemiccoDepartment of Laboratory Medicine and Pathobiology, Temerty Faculty of Medicine, University of Toronto, 6th Floor, 1 King's College Circle, Toronto, ON, M5S 1A8, Canada.
Brendan C DicksonDepartment of Laboratory Medicine and Pathobiology, Temerty Faculty of Medicine, University of Toronto, 6th Floor, 1 King's College Circle, Toronto, ON, M5S 1A8, Canada.
Ali NaraghiDepartment of Medical Imaging, Division of Musculoskeletal Imaging, Temerty Faculty of Medicine, University of Toronto, 4 Floor, 263 McCaul Street, Toronto, ON, M5T 1W7, Canada.
Angela AtingaDepartment of Medical Imaging, Division of Musculoskeletal Imaging, Temerty Faculty of Medicine, University of Toronto, 4 Floor, 263 McCaul Street, Toronto, ON, M5T 1W7, Canada.
Anthony M GriffinDepartment of Surgery, Division of Orthopedics, Mount Sinai Hospital, Sinai Health System, 600 University Avenue, Toronto, ON, M5G 1X5, Canada.
Abdulazeez SalawuDepartment of Medicine, Division of Medical Oncology, Temerty Faculty of Medicine, University of Toronto, 3 Floor, 6 Queen's Park Crescent West, Toronto, ON, M5S 3H, Canada.
Aaron M GazendamDepartment of Surgery, Division of Orthopedic Surgery, Temerty Faculty of Medicine, University of Toronto, 5th Floor, 149 College Street, Toronto, ON, M5T 1P5, Canada.
Kim M TsoiDepartment of Surgery, Division of Orthopedic Surgery, Temerty Faculty of Medicine, University of Toronto, 5th Floor, 149 College Street, Toronto, ON, M5T 1P5, Canada.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectiveTo describe the spectrum of MRI features, histopathological findings, and clinical outcomes of musculoskeletal PEComas at a single tertiary sarcoma center. MATERIALS AND

methodsFollowing institutional ethics approval, orthopedic oncology and pathology databases were searched for histopathologically confirmed musculoskeletal soft tissue PEComas managed between 2000 and 2025. Inclusion required pretreatment MRI. Pulmonary, intra-abdominal/pelvic, and cutaneous lesions were excluded. Demographic and clinical features were reviewed for all cases. MRI examinations were reviewed in consensus by two fellowship-trained musculoskeletal radiologists and archival histopathology by a dedicated musculoskeletal pathologist.

resultsTwelve patients met inclusion criteria (7/12 male), mean age 64.3 years (range 16-89 years). One tuberous sclerosis complex (TSC)-associated fibroma-like PEComa appeared as a well-defined periarticular mass with homogeneously low T1 and T2 signal and no significant enhancement. The remaining 11 non-TSC tumors had a mean diameter of 13.0 cm; 10 of 11 were deep to deep fascia, and 6 of 11 showed trans-compartmental extension. Intratumoral nonenhancement consistent with necrosis was present in all 10 contrast-enhanced cases. Neurovascular encasement was identified in 4 of 11 (36%) and osseous invasion in 2 of 11 (18%). All nine histologically evaluable non-TSC cases showed malignant morphology. The overall metastatic rate was 91%, with disease-specific mortality of 64% at a median follow-up of 19 months.

conclusionMusculoskeletal PEComas demonstrate large size, deep location, macroscopic intratumoral necrosis, as well as frequent trans-compartmental extension on MRI. Clinical outcomes reflect aggressive biologic behavior, with high metastatic rates and disease-specific mortality in non-TSC-related PEComas of the musculoskeletal system.

Indexed as

HistopathologyImmunohistochemistryMagnetic Resonance ImagingMolecularMRIMusculoskeletalPEComaPerivascular Epithelioid Cell TumorSarcomaSoft Tissue

Identifiers

PMID42747556

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