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Type 1 PRCC, also known as a renal tumor caused by a genetic predisposition of hereditary papillary renal cancer syndrome, compromises approximately 25% of all PRCCs. [14] [15] In the perspective of immunochemistry, it has a profile of strong CK7 and alpha-methyl acyl-CoA racemase (AMACR) expression at most focal CA-IX expression. [16]
Psammoma bodies are commonly seen in certain tumors such as: Papillary thyroid carcinoma [2]; Papillary renal cell carcinoma [3]; Ovarian papillary serous cystadenoma and cystadenocarcinoma [4]
The renal cell carcinoma tends to be of the papillary (type 2) form and tends to occur more commonly in women than men with this syndrome. These cancers present earlier than is usual for renal cell carcinomas (typically in the twenties and thirties) and tend to be at relatively advanced stages at presentation.
Several morphological variants have been described, as the ‘‘mucin-poor variants’’, showing a predominance of tubular or spindle cell components and only minimal pale mucinous background. Focal papillations or papillary cores and foamy histiocytes can also be seen, creating confusion with papillary RCC. Helpful features for diagnosis ...
Metanephric adenoma (MA) is a rare, benign tumour of the kidney, that can have a microscopic appearance similar to a nephroblastoma (Wilms tumours), [1] or a papillary renal cell carcinoma. It should not be confused with the pathologically unrelated, yet similar sounding, mesonephric adenoma .
Hereditary bilateral, multifocal kidney tumors similar to those seen in BHD can occur with von Hippel–Lindau disease (clear cell renal cell carcinoma), hereditary papillary renal cancer (papillary renal cell carcinoma), and hereditary leiomyomatosis and renal cell cancer syndrome. They are differentiated with examination of the tumors' histology.
Clear cell papillary renal cell carcinoma (CCPRCC) is a rare subtype of renal cell carcinoma (RCC) that has microscopic morphologic features of papillary renal cell carcinoma and clear cell renal cell carcinoma, yet is pathologically distinct based on molecular changes and immunohistochemistry.
Bilateral kidney tumors, concurrent birth defects, and/or metastatic disease at presentation favor a diagnosis of Wilms tumor. [ 6 ] congenital infantile sarcoma is a rare aggressive sarcoma typically presenting in the lower extremities, head, or neck of infants during their first year of life.
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