title: Cystic and necrotic papillary renal cell carcinoma: prognosis, morphology, immunohistochemical, and molecular-genetic profile of 10 cases.
abstract: Conflicting data have been published on the prognostic significance of tumor necrosis in papillary renal cell carcinoma (PRCC). Although the presence of necrosis is generally considered an adverse prognostic feature in PRCC, we report a cohort of 10 morphologically distinct cystic and extensively necrotic PRCC with favorable biological behavior. Ten cases of type 1 PRCC with a uniform morphologic pattern were selected from the 19 500 renal tumors, of which 1311 were PRCCs in our registry. We focused on precise morphologic diagnosis supported by immunohistochemical and molecular-genetic analysis. Patients included 8 men and 2 women with an age range of 32-85 years (mean, 62.6 years). Tumor size ranged from 6 to 14 cm (mean, 9.4 cm). Follow-up data were available in 7 patients, ranging from 0.5 to 14 years (mean, 4 years). All tumors were spherical, cystic, and circumscribed by a thick fibrous capsule, filled with hemorrhagic/necrotic contents. Limited viable neoplastic tissue was present only as a thin rim in the inner surface of the cyst wall, consistent with type 1 PRCC. All cases were positive for AMACR, OSCAR, CAM 5.2, HIF-2, and vimentin. Chromosome 7 and 17 polysomy was found in 5 of 9 analyzable cases, 2 cases demonstrated chromosome 7 and 17 disomy, and 1 case showed only chromosome 17 polysomy. Loss of chromosome Y was found in 5 cases, including 1 case with disomic chromosomes 7 and 17. No VHL gene abnormalities were found. Papillary renal cell carcinoma type 1 can present as a large hemorrhagic/necrotic unicystic lesion with a thick fibroleiomyomatous capsule. Most cases showed a chromosomal numerical aberration pattern characteristic of PRCC. All tumors followed a nonaggressive clinical course. Large liquefactive necrosis should not necessarily be considered an adverse prognostic feature, particularly in a subset of type 1 PRCC with unilocular cysts filled with necrotic/hemorrhagic material.
authors.affiliation: Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic.
authors.forename: Kvetoslava
authors.initials: K
authors.lastname: Peckova
authors.affiliation: Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic.
authors.forename: Petr
authors.initials: P
authors.lastname: Martinek
authors.affiliation: Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic.
authors.forename: Kristyna
authors.initials: K
authors.lastname: Pivovarcikova
authors.affiliation: Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic.
authors.forename: Tomas
authors.initials: T
authors.lastname: Vanecek
authors.affiliation: Department of Pathology, University of British Columbia, Royal Columbian Hospital, Vancouver, Canada.
authors.forename: Reza
authors.initials: R
authors.lastname: Alaghehbandan
authors.affiliation: Department of Urology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic.
authors.forename: Kristyna
authors.initials: K
authors.lastname: Prochazkova
authors.affiliation: Department of Pathology, Instituto Nacional de Cancerologia, Mexico City, Mexico.
authors.forename: Delia Perez
authors.initials: DP
authors.lastname: Montiel
authors.affiliation: Department of Urology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic.
authors.forename: Milan
authors.initials: M
authors.lastname: Hora
authors.affiliation: Department of Pathology, University Clinical Center, Sarajevo, Bosnia and Herzegovina.
authors.forename: Faruk
authors.initials: F
authors.lastname: Skenderi
authors.affiliation: "Ljudevit Jurak" Pathology Department, Clinical Hospital Center "Sestre milosrdnice,", Zagreb, Croatia.
authors.forename: Monika
authors.initials: M
authors.lastname: Ulamec
authors.affiliation: Biopticka Laborator, Plzen, Czech Republic.
authors.forename: Pavla
authors.initials: P
authors.lastname: Rotterova
authors.affiliation: Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic.
authors.forename: Ondrej
authors.initials: O
authors.lastname: Daum
authors.affiliation: Department of Radiodiology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic.
authors.forename: Jiri
authors.initials: J
authors.lastname: Ferda
authors.affiliation: Department of Pathology, The University of Kansas School of Medicine, Kansas City, KS.
authors.forename: Whitney
authors.initials: W
authors.lastname: Davidson
authors.affiliation: Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic.
authors.forename: Ondrej
authors.initials: O
authors.lastname: Ondic
authors.affiliation: Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic.
authors.forename: Magdalena
authors.initials: M
authors.lastname: Dubova
authors.affiliation: Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic.
authors.forename: Michal
authors.initials: M
authors.lastname: Michal
authors.affiliation: Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic. Electronic address: hes@medima.cz.
authors.forename: Ondrej
authors.initials: O
authors.lastname: Hes
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis; D002291:Carcinoma, Papillary / Q000175:diagnosis* / Q000473:pathology*; D002292:Carcinoma, Renal Cell / Q000175:diagnosis* / Q000473:pathology*; D002869:Chromosome Aberrations; D005260:Female; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D017404:In Situ Hybridization, Fluorescence / Q000379:methods; D007680:Kidney Neoplasms / Q000175:diagnosis* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D057089:Pathology, Molecular / Q000379:methods; D011379:Prognosis
chemical_list: D014408:Biomarkers, Tumor
keywords: Cystic; Kidney; Molecular genetics; Necrosis; Necrotics; Papillary renal cell carcinoma