MEIS1::NCOA1 Primitive Spindle Cell Sarcoma of the Kidney

Primitive sarcomas harboring the MEIS1::NCOA2 gene fusion were originally described in the kidney in 2018, and subsequently reported in other organs. These variably cellular neoplasms feature monomorphic primitive plump spindle cells forming nodules and whorls in addition to nondescript fascicular, solid, and storiform patterns. They lack skeletal muscle differentiation in contrast to the primarily intraosseous rhabdomyosarcomas that harbor the same gene fusion. We describe 7 new primary primitive renal sarcomas with MEIS1::NCOA1 gene fusions. Although their morphology overlaps with that described in MEIS1::NCOA2 renal sarcoma, 3 of the 7 cases contained adipose tissue. The majority had intimately admixed entrapped cystic epithelial elements and demonstrated patchy immunoreactivity for estrogen receptor and nuclear labeling for WT1 protein, leading to the differential diagnosis of malignant mixed epithelial stromal tumor (MEST) in 4 cases and metanephric stromal tumor in one. The neoplasms demonstrate a broad spectrum of clinicopathologic features ranging from a bland low-grade neoplasm that metastasized 9 years after diagnosis to a high-grade sarcoma with multiple recurrences, ultimately leading to patient death in under 1 year. In summary, MEIS1::NCOA1 primitive sarcomas overlap with the previously described MEIS1::NCOA2 primitive renal sarcomas and represent a distinctive renal neoplasm that can be mistaken for malignant MEST. Grade ranges from low to high but even low-grade neoplasms require long-term clinical follow-up.

Authors

Institutions

Publication Details

Journal
The American Journal of Surgical Pathology
Published
2025-03-31
DOI
https://doi.org/10.1097/pas.0000000000002386
Citations
3
Primary Topic
Renal and related cancers
Type
article
Field-Weighted Citation Impact
2.42
Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

MEIS1::NCOA1 Primitive Spindle Cell Sarcoma of the Kidney

Pedram Argani, Maria Tretiakova, Benjamin F. Smith, Tanaya Neff et al.
3 citations
The American Journal of Surgical Pathology
Renal and related cancers
2.42
article

MEIS1::NCOA1 Primitive Spindle Cell Sarcoma of the Kidney

Pedram Argani, Maria Tretiakova, Benjamin F. Smith, Tanaya Neff, Gregory W. Charville, Abbas Agaimy, Christopher L. Corless, John Gross, Kristina Wakeman, Yajuan J. Liu, Katrina Collins, Fadi Brimo, Ankur R. Sangoi, Sara M. Falzarano, Ezra Baraban, Andrés Matoso, Cristina R. Antonescu, Sintawat Wangsiricharoen, Ali Abdel Satir
article en
3 citations

Abstract

Primitive sarcomas harboring the MEIS1::NCOA2 gene fusion were originally described in the kidney in 2018, and subsequently reported in other organs. These variably cellular neoplasms feature monomorphic primitive plump spindle cells forming nodules and whorls in addition to nondescript fascicular, solid, and storiform patterns. They lack skeletal muscle differentiation in contrast to the primarily intraosseous rhabdomyosarcomas that harbor the same gene fusion. We describe 7 new primary primitive renal sarcomas with MEIS1::NCOA1 gene fusions. Although their morphology overlaps with that described in MEIS1::NCOA2 renal sarcoma, 3 of the 7 cases contained adipose tissue. The majority had intimately admixed entrapped cystic epithelial elements and demonstrated patchy immunoreactivity for estrogen receptor and nuclear labeling for WT1 protein, leading to the differential diagnosis of malignant mixed epithelial stromal tumor (MEST) in 4 cases and metanephric stromal tumor in one. The neoplasms demonstrate a broad spectrum of clinicopathologic features ranging from a bland low-grade neoplasm that metastasized 9 years after diagnosis to a high-grade sarcoma with multiple recurrences, ultimately leading to patient death in under 1 year. In summary, MEIS1::NCOA1 primitive sarcomas overlap with the previously described MEIS1::NCOA2 primitive renal sarcomas and represent a distinctive renal neoplasm that can be mistaken for malignant MEST. Grade ranges from low to high but even low-grade neoplasms require long-term clinical follow-up.

The American Journal of Surgical PathologyVol. 49(6)
Memorial Sloan Kettering Cancer Center (US), Johns Hopkins University (US), Oregon Health & Science University (US), Friedrich-Alexander-Universität Erlangen-Nürnberg (DE), University of Washington (US), Johns Hopkins Medicine (US), McGill University Health Centre (CA), University of Florida (US), Universitätsklinikum Erlangen (DE), Comprehensive Cancer Center Erlangen (DE), Indiana University School of Medicine, Indiana University – Purdue University Indianapolis (US), Stanford University (US)
Good health and well-being
Openalex Percentile: Top 12%
Renal and related cancers
2.42
DOIOpen Access PDFOpenAlex
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.