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“Mucin”-secreting papillary renal cell carcinoma: clinicopathological, immunohistochemical, and molecular genetic analysis of seven cases

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Abstract

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.

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Acknowledgments

The authors thank Dr. Ivan Damjanov for his help with the editing of our paper and for the constructive discussion.

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Authors and Affiliations

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

    Kristyna Pivovarcikova, Kvetoslava Peckova, Petr Martinek, Ondrej Daum, Pavla Rotterova, Ondrej Ondic, Magdalena Dubova, Romuald Curik, Michal Michal & Ondrej Hes

  2. Department of Pathology, Instituto Nacional de Cancerologia, Mexico City, Mexico

    Delia Perez Montiel

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

    Kristyna Kalusova, Tomas Pitra & Milan Hora

  4. Department of Pathology, University Hospital Sarajevo, Sarajevo, Bosna and Hercegovina

    Faruk Skenderi

  5. Pathology Department, Clinical Hospital Center, Sestre milosrdnice, Zagreb, Croatia

    Monika Ulamec

  6. Department of Pathology, University Hospital Split, Split, Croatia

    Ana Dunatov

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

    Tomas Svoboda

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

    Ondrej Hes

Authors
  1. Kristyna Pivovarcikova
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  2. Kvetoslava Peckova
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  3. Petr Martinek
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  4. Delia Perez Montiel
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  6. Tomas Pitra
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  7. Milan Hora
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  10. Ondrej Daum
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  11. Pavla Rotterova
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  12. Ondrej Ondic
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  13. Magdalena Dubova
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  14. Romuald Curik
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  15. Ana Dunatov
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  16. Tomas Svoboda
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  17. Michal Michal
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  18. Ondrej Hes
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Corresponding author

Correspondence to Ondrej Hes.

Ethics declarations

The study was approved by local Ethics Committee (Medical Teaching Hospital and Medical School of Charles University in Plzen).

Funding

The study was supported by the Charles University Research Fund (project number P36), by the project CZ.1.05/2.1.00/03.0076 from European Regional Development Fund, and by SVV 260283.

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The authors declare no conflict of interest.

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Pivovarcikova, K., Peckova, K., Martinek, P. et al. “Mucin”-secreting papillary renal cell carcinoma: clinicopathological, immunohistochemical, and molecular genetic analysis of seven cases. Virchows Arch 469, 71–80 (2016). https://doi.org/10.1007/s00428-016-1936-7

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  • DOI: https://doi.org/10.1007/s00428-016-1936-7

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