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.

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 Medical Faculty Charles University and Charles University Hospital Plzen Alej Svobody 80 304 60 Pilsen Czech Republic Biomedical Centre Faculty of Medicine in Plzen Charles University Prague Plzen 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

000      

00000naa a2200000 a 4500

001      

bmc17013955

003      

CZ-PrNML

005      

20170418105601.0

007      

ta

008      

170413s2016 gw f 000 0|eng||

009      

AR

024    7_

$a 10.1007/s00428-016-1936-7 $2 doi

035    __

$a (PubMed)27072821

040    __

$a ABA008 $b cze $d ABA008 $e AACR2

041    0_

$a eng

044    __

$a gw

100    1_

$a Pivovarcikova, Kristyna $u Department of Pathology, Medical Faculty, Charles University and Charles University Hospital Plzen, Alej Svobody 80, 304 60, Pilsen, Czech Republic.

245    10

$a "Mucin"-secreting papillary renal cell carcinoma: clinicopathological, immunohistochemical, and molecular genetic analysis of seven cases / $c K. Pivovarcikova, K. Peckova, P. Martinek, DP. Montiel, K. Kalusova, T. Pitra, M. Hora, F. Skenderi, M. Ulamec, O. Daum, P. Rotterova, O. Ondic, M. Dubova, R. Curik, A. Dunatov, T. Svoboda, M. Michal, O. Hes,

520    9_

$a 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.

650    _2

$a senioři $7 D000368

650    _2

$a antigeny nádorové $x metabolismus $7 D000951

650    _2

$a nádorové biomarkery $x analýza $7 D014408

650    _2

$a papilární karcinom $x patologie $7 D002291

650    _2

$a karcinom z renálních buněk $x genetika $x metabolismus $x patologie $7 D002292

650    _2

$a ženské pohlaví $7 D005260

650    _2

$a lidé $7 D006801

650    _2

$a imunohistochemie $x metody $7 D007150

650    _2

$a hybridizace in situ fluorescenční $x metody $7 D017404

650    _2

$a nádory ledvin $x genetika $x metabolismus $x patologie $7 D007680

650    _2

$a mužské pohlaví $7 D008297

650    _2

$a lidé středního věku $7 D008875

650    _2

$a muciny $x analýza $7 D009077

655    _2

$a časopisecké články $7 D016428

700    1_

$a Peckova, Kvetoslava $u Department of Pathology, Medical Faculty, Charles University and Charles University Hospital Plzen, Alej Svobody 80, 304 60, Pilsen, Czech Republic.

700    1_

$a Martinek, Petr $u Department of Pathology, Medical Faculty, Charles University and Charles University Hospital Plzen, Alej Svobody 80, 304 60, Pilsen, Czech Republic.

700    1_

$a Montiel, Delia Perez $u Department of Pathology, Instituto Nacional de Cancerologia, Mexico City, Mexico.

700    1_

$a Kalusova, Kristyna $u Department of Urology, Medical Faculty, Charles University and Charles University Hospital Plzen, Prague, Czech Republic.

700    1_

$a Pitra, Tomas $u Department of Urology, Medical Faculty, Charles University and Charles University Hospital Plzen, Prague, Czech Republic.

700    1_

$a Hora, Milan $u Department of Urology, Medical Faculty, Charles University and Charles University Hospital Plzen, Prague, Czech Republic.

700    1_

$a Skenderi, Faruk $u Department of Pathology, University Hospital Sarajevo, Sarajevo, Bosna and Hercegovina.

700    1_

$a Ulamec, Monika $u Pathology Department, Clinical Hospital Center, Sestre milosrdnice, Zagreb, Croatia.

700    1_

$a Daum, Ondrej $u Department of Pathology, Medical Faculty, Charles University and Charles University Hospital Plzen, Alej Svobody 80, 304 60, Pilsen, Czech Republic.

700    1_

$a Rotterova, Pavla $u Department of Pathology, Medical Faculty, Charles University and Charles University Hospital Plzen, Alej Svobody 80, 304 60, Pilsen, Czech Republic.

700    1_

$a Ondic, Ondrej $u Department of Pathology, Medical Faculty, Charles University and Charles University Hospital Plzen, Alej Svobody 80, 304 60, Pilsen, Czech Republic.

700    1_

$a Dubova, Magdalena $u Department of Pathology, Medical Faculty, Charles University and Charles University Hospital Plzen, Alej Svobody 80, 304 60, Pilsen, Czech Republic.

700    1_

$a Curik, Romuald $u Department of Pathology, Medical Faculty, Charles University and Charles University Hospital Plzen, Alej Svobody 80, 304 60, Pilsen, Czech Republic.

700    1_

$a Dunatov, Ana $u Department of Pathology, University Hospital Split, Split, Croatia.

700    1_

$a Svoboda, Tomas $u Department of Oncology, Medical Faculty, Charles University and Charles University Hospital Plzen, Prague, Czech Republic.

700    1_

$a Michal, Michal $u Department of Pathology, Medical Faculty, Charles University and Charles University Hospital Plzen, Alej Svobody 80, 304 60, Pilsen, Czech Republic.

700    1_

$a Hes, Ondrej $u Department of Pathology, Medical Faculty, Charles University and Charles University Hospital Plzen, Alej Svobody 80, 304 60, Pilsen, Czech Republic. hes@medima.cz. Biomedical Centre, Faculty of Medicine in Plzen, Charles University in Prague, Plzen, Czech Republic. hes@medima.cz.

773    0_

$w MED00004660 $t Virchows Archiv an international journal of pathology $x 1432-2307 $g Roč. 469, č. 1 (2016), s. 71-80

856    41

$u https://pubmed.ncbi.nlm.nih.gov/27072821 $y Pubmed

910    __

$a ABA008 $b sig $c sign $y a $z 0

990    __

$a 20170413 $b ABA008

991    __

$a 20170418105909 $b ABA008

999    __

$a ok $b bmc $g 1200420 $s 974733

BAS    __

$a 3

BAS    __

$a PreBMC

BMC    __

$a 2016 $b 469 $c 1 $d 71-80 $e 20160412 $i 1432-2307 $m Virchows Archiv $n Virchows Arch $x MED00004660

LZP    __

$a Pubmed-20170413