Biphasic papillary (biphasic squamoid alveolar) renal cell carcinoma: a clinicopathologic and molecular study of 17 renal cell carcinomas including 10 papillary adenomas.

Autor: Nova-Camacho LM; Department of Pathology, Donostia University Hospital, San Sebastian, Spain. luismi_15_16@hotmail.com.; Gainesville, USA. luismi_15_16@hotmail.com., Acosta AM; Department of Pathology, Indiana University, Indianapolis, IN, USA., Akgul M; Department of Pathology and Laboratory Medicine, Albany Medical Center, Albany, NY, USA., Panizo A; Department of Pathology, University Hospital of Navarra, Pamplona, Spain., Galea LA; Department of Anatomical Pathology, Sonic Healthcare, Melbourne PathologyVictoria, Australia., Val-Carreres A; Department of Pathology, Donostia University Hospital, San Sebastian, Spain., Talavera JA; Department of Internal Medicine, Diagnósticos da America DASA, Sao Paulo, Brazil., Guerrero-Setas D; Department of Pathology, University Hospital of Navarra, Pamplona, Spain., Martin-Arruti M; Department of Pathology, Donostia University Hospital, San Sebastian, Spain.; Laboratory of Molecular Pathology and Therapeutic Targets, Donostia University Hospital, San Sebastian, Spain., Ruiz I; Department of Pathology, Donostia University Hospital, San Sebastian, Spain.; Laboratory of Molecular Pathology and Therapeutic Targets, Donostia University Hospital, San Sebastian, Spain., García-Martos M; Department of Pathology, Gregorio Marañon University Hospital, Madrid, Spain., Sangoi AR; Department of Pathology, Stanford University, Stanford, CA, USA.
Jazyk: angličtina
Zdroj: Virchows Archiv : an international journal of pathology [Virchows Arch] 2024 Mar; Vol. 484 (3), pp. 441-449. Date of Electronic Publication: 2024 Feb 22.
DOI: 10.1007/s00428-024-03768-x
Abstrakt: Biphasic papillary renal cell carcinoma (synonymous with biphasic squamoid alveolar renal cell carcinoma) is considered within the spectrum of papillary renal cell carcinoma (PRCC). With < 70 reported cases of biphasic PRCC, there is limited data on the pathologic spectrum and clinical course. Seventeen biphasic PRCC cases and 10 papillary adenomas with similar biphasic morphology were assessed. The mean age of the biphasic PRCC patients was 62 years (male to female ratio of 1.8:1), from 10 partial nephrectomies, 6 radical nephrectomies, and 1 biopsy. The mean tumor size was 3.6 cm (range 1.6-8 cm), with 24% showing multifocality. Fifteen out of 17 cases were limited to the kidney (one of which was staged as pT2a but had lung metastases at diagnosis) and 2/17 cases were staged as T3a. All tumors showed typical biphasic morphology with an extent of squamoid foci widely variable from 10 to 95%. Emperipolesis was identified in 88% of cases. All biphasic PRCC tested exhibited positivity for PAX8 (16/16), keratin 7 (17/17), EMA (15/15), AMACR (17/17), and vimentin (12/12) in both large and small cells; cyclin D1 was only expressed in the large cells (16/16). The 10 papillary adenomas showed a similar immunoprofile to biphasic PRCC. NGS testing performed on 13 biphasic PRCC revealed 4 (31%) harboring MET SNVs. In 1/5 (20%) papillary adenomas, a pathogenic MET SNV was identified. Biphasic PRCC is rare with a generally similar immunoprofile to "type 1" PRCC but with notable strong positivity for cyclin D1 in the large cell component. Although most of the biphasic PRCC cases were of small size, low stage, and with an indolent behavior, one patient had metastatic disease and one patient died of the disease.
(© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
Databáze: MEDLINE
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