'Mucin'-secreting papillary renal cell carcinoma: clinicopathological, immunohistochemical, and molecular genetic analysis of seven cases
Autor: | Kristyna Pivovarcikova, Milan Hora, Tomáš Pitra, Ondrej Hes, Monika Ulamec, Ana Dunatov, Michal Michal, Faruk Skenderi, Kvetoslava Peckova, Delia Perez Montiel, Tomáš Svoboda, Romuald Curik, Magdalena Dubova, Ondrej Daum, Kristyna Kalusova, Pavla Rotterova, Ondrej Ondič, Petr Martinek |
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Jazyk: | angličtina |
Rok vydání: | 2016 |
Předmět: |
0301 basic medicine
Male Pathology medicine.medical_specialty Vimentin TFE3 Biology Pathology and Forensic Medicine 03 medical and health sciences 0302 clinical medicine Mucicarmine stain Renal cell carcinoma Antigens Neoplasm medicine Biomarkers Tumor Humans Molecular Biology Carcinoma Renal Cell In Situ Hybridization Fluorescence Aged Polysomy Papillary renal cell carcinomas Mucin Mucins Cell Biology General Medicine Middle Aged medicine.disease Immunohistochemistry Carcinoma Papillary Kidney Neoplasms 030104 developmental biology array CGH FISH immunohistochemistry kidney mucin mucin-like secretion papillary renal cell carcinoma 030220 oncology & carcinogenesis biology.protein Female |
Popis: | 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. |
Databáze: | OpenAIRE |
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