Mucin and mucin-like material are features of mucinous tubular and spindle renal cell carcinoma (MTS RCC) but are rarely seen in papillary renal cell carcinoma (PRCC). We reviewed 1311 PRCC and identified 7 tumors containing extracellular and/or intracellular mucinous/mucin-like material (labeled as PRCCM). We analyzed these using morphological, histochemical, immunohistochemical, and molecular genetic methods (arrayCGH, FISH). Clinical data were available for six of the seven patients (five males and one female, age range 61-78 years). Follow-up was available for four patients (2-4 years); one patient died of widespread metastases. Tumor size ranged from 3 to 5 cm (mean 3.8). Of all cases, histological architecture showed a predominantly papillary pattern. Mucin or mucin-like was extracellular in one, intracellular in three, and both intra/extracellular in three cases. All tumors were positive for AMACR, vimentin, and OSCAR, while CK7 was positive in four. Mucicarmine stain was positive in all cases, PAS in six and Alcian blue in three cases. Five tumors were positive for MUC 1, but none were positive for MUC 2, MUC 4, or MUC 6. In only four cases, genetic analysis could be performed. Gain of chromosomes 7 and 17 was found in two cases; gain of 17 only was found in one case. Loss of heterozygosity of 3p was found in one case together with polysomy of chromosomes 7 and 17. No abnormalities of VHL, fumarate dehydrogenase, and TFE3 genes were detected. We conclude that PRCCM is a rare but challenging subtype of RCC that deserves to be further studied. In all the tumors, the mucin-like material was found in those stained with mucicarmin, but other conventional and immunohistochemical stains did not reveal consistent features of a single mucin. The molecular-genetic profile of these tumors was most consistent with that of typical papillary RCC, although one case had mixed genetic features of papillary and clear RCC. PRCCM has metastatic potential, as evidenced by one case with widespread metastases. It remains to be determined whether PRCCM represents a unique tumor subtype, deserving to be distinguished from other subtypes of PRCC.

Biomedical Centre Faculty of Medicine in Plzen Charles University Prague Plzen Czech Republic

Department of Oncology Medical Faculty Charles University and Charles University Hospital Plzen Prague Czech Republic

Department of Pathology Instituto Nacional de Cancerologia Mexico City Mexico

Department of Pathology Medical Faculty Charles University and Charles University Hospital Plzen Alej Svobody 80 304 60 Pilsen Czech Republic

Department of Pathology University Hospital Sarajevo Sarajevo Bosna and Hercegovina

Department of Pathology University Hospital Split Split Croatia

Department of Urology Medical Faculty Charles University and Charles University Hospital Plzen Prague Czech Republic

Pathology Department Clinical Hospital Center Sestre milosrdnice Zagreb Croatia

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Am J Surg Pathol. 2012 Mar;36(3):432-42 PubMed

Am J Surg Pathol. 1998 Aug;22(8):1037-40 PubMed

Hum Pathol. 2002 Jan;33(1):60-7 PubMed

Histol Histopathol. 2001 Apr;16(2):387-92 PubMed

Virchows Arch. 2013 Oct;463(4):563-73 PubMed

Br J Urol. 1971 Feb;43(1):4-15 PubMed

Am J Surg Pathol. 2013 Oct;37(10):1469-89 PubMed

Virchows Arch. 2000 Feb;436(2):119-26 PubMed

Front Biosci. 2001 Oct 01;6:D1207-15 PubMed

Pathol Res Pract. 1999;195(1):11-7 PubMed

Urol Oncol. 2009 Jan-Feb;27(1):3-7 PubMed

Am J Surg Pathol. 2006 Dec;30(12):1554-60 PubMed

Hum Pathol. 2006 Nov;37(11):1371-88 PubMed

Mod Pathol. 1997 Jun;10(6):537-44 PubMed

Ann Diagn Pathol. 2013 Apr;17(2):226-9 PubMed

Ann Diagn Pathol. 2015 Aug;19(4):226-31 PubMed

Diagn Pathol. 2008 Sep 03;3:36 PubMed

Mod Pathol. 2009 Jun;22 Suppl 2:S96-S118 PubMed

Cancer Res. 1995 Jun 15;55(12):2681-90 PubMed

Mod Pathol. 2002 Nov;15(11):1126-30 PubMed

Mod Pathol. 2008 Dec;21(12):1533-41 PubMed

Hum Pathol. 2002 Feb;33(2):236-41 PubMed

Arch Pathol Lab Med. 1988 Aug;112(8):847-9 PubMed

Leukemia. 2003 Dec;17(12):2257-317 PubMed

J Biol Chem. 1994 Jan 28;269(4):2440-6 PubMed

Am J Surg Pathol. 2009 May;33(5):659-68 PubMed

Pathol Res Pract. 2011 Apr 15;207(4):271-4 PubMed

Histopathology. 2002 Dec;41(6):549-55 PubMed

Histopathology. 2002 May;40(5):450-7 PubMed

Biochimie. 2010 Jan;92(1):1-11 PubMed

Acta Histochem. 1969;34(2):263-72 PubMed

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