Immunohistochemical characterization of renal tumors in patients with Birt-Hogg-Dubé Syndrome

Autor: Yoshiaki Imamura, Naoto Kuroda, Yukio Nakatani, Yoji Nagashima, Midori Ando, Masahiro Yao, Reiko Tanaka, Yasushi Nakamura, Akinobu Araki, Yasuhiro Iribe, Hiroko Gotoda, Mitsuko Furuya, Jun Matsushima, Fumi Kawakami
Rok vydání: 2015
Předmět:
Zdroj: Pathology International. 65:126-132
ISSN: 1320-5463
DOI: 10.1111/pin.12254
Popis: Birt-Hogg-Dube syndrome (BHD) is an autosomal dominant disorder associated with a germline mutation of folliculin (FLCN). The affected families are at a high risk for developing multiple renal cell carcinomas (RCC). Little is known about the immunostaining patterns of mutant FLCN-associated RCCs. We investigated 32 RCCs obtained from 17 BHD patients. The studied tumors included chromophobe RCCs (n = 15), hybrid oncocytic/chromophobe tumors (HOCT) (n = 14) and clear cell RCCs (n = 3). Almost all chromophobe RCCs and HOCTs revealed positive staining for S100A1, Ksp-cadherin and CD82. They stained either focally or diffusely for CK7, and were negative for CA-IX. All clear cell RCCs were positively stained for CA-IX and negative for CK7. These data confirmed that mutant FLCN-associated oncocytic and clear cell RCCs exhibited generally similar immunostaining patterns compared to their sporadic counterparts. Frequent positive staining for S100A1, Ksp-cadherin and CD82 in chromophobe RCCs and HOCTs indicated that these two types were relatively similar rather than distinctively different in their patterns of immunoreactivity. Characteristic peri-nuclear halos and polygonal cells with clear cytoplasm, which often misleads pathologists into the diagnosis of clear cell RCC, should be carefully examined using an immunohistochemical panel including CA-IX, Ksp-cadherin, CD82 and CK7.
Databáze: OpenAIRE
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