Solitary fibrous tumor of kidney and renal hilus: a multi-institutional clinicopathologic analysis of 43 cases

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11140%2F25%3A10502666" target="_blank" >RIV/00216208:11140/25:10502666 - isvavai.cz</a>

Result on the web

<a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=9mNJkg.vXo" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=9mNJkg.vXo</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1007/s00428-025-04264-6" target="_blank" >10.1007/s00428-025-04264-6</a>

Result language

angličtina

Original language name

Solitary fibrous tumor of kidney and renal hilus: a multi-institutional clinicopathologic analysis of 43 cases

Original language description

Solitary fibrous tumor (SFT) is a fibroblastic neoplasm characterized by prominent, staghorn, or delicate &quot;hemangiopericytoma (HPC)-like vasculature&quot;, NAB2::STAT6 gene fusion, and its surrogate STAT6 expression. SFT may exhibit an unpredictable clinical course, necessitating risk assessment based on tumor characteristics. Renal SFTs are rare and have not been well characterized. Forty-three primary kidney SFT cases are reviewed for clinical, morphological, and immunohistochemical (STAT6, BCL2, CD34, and PAX8) features. A four-variable risk stratification by Demicco was applied based on patient age, tumor size, mitotic activity, and tumor necrosis. The mean age was 49 years (range 11-83 years) with a slight female predominance (male:female = 20:23). The mean tumor size was 7.8 cm (1.6-32 cm). Tumors were mainly located at the hilus (22/32, 68%) and had well-demarcated borders (31/42, 74%). Morphologically, tumors were categorized as 1) Fibrous (&quot;SFT-like&quot;, 19/43, 44%), characterized by hypocellularity and prominent fine-reticular or keloidal collagen; 2) Cellular (&quot;HPC-like&quot;, 8/43, 19%), with hypercellularity, short spindle to small cell-like proliferation, and lacking a collagenous background; 3) mixed fibrous/cellular (16/43, 37%) displaying both components. Four cases featured a lipomatous component. BCL2 was positive in all tested cases (28/28), CD34 in all but 3 cases (93%), and STAT6 in all but one case (39/40, 97.5%). PAX8 was positive in 6/34 (18%) cases. Most cases (29/43, 68%) were classified as low-risk, followed by intermediate (12/43, 27%) and high-risk (2/43, 5%) groups. Five of 29 (18%) patients had metastatic disease, and two patients with high-risk and one with intermediate-risk tumors died from the disease, while 25 patients with low- or intermediate-risk tumors were alive for an average of about 36 months. We emphasize the usefulness of the risk stratification system in predicting prognosis. PAX8 expression in a subset of renal SFT represents a potential diagnostic pitfall.

Czech name

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Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

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OECD FORD branch

30109 - Pathology

Project

—

Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2025

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Virchows Archiv

ISSN

0945-6317

e-ISSN

1432-2307

Volume of the periodical

487

Issue of the periodical within the volume

6

Country of publishing house

DE - GERMANY

Number of pages

14

Pages from-to

1265-1278

UT code for WoS article

001573786500001

EID of the result in the Scopus database

2-s2.0-105016692932