Scholarlink

<p>Poromas are benign sweat gland adnexal neoplasms that include four distinct architectural variants i.e. classical poroma, hidroacanthoma simplex, poroid hidradenoma and dermal duct tumor. Most of the poromas display oncogenic <i>YAP1::MAML2</i> or <i>YAP1::NUTM1</i> fusions and previous studies have suggested a correlation between the <i>YAP1</i>’s fusion partner and the tumor morphology. Recently, two cases of <i>YAP1::NR4A3</i> fused-poromas with distinctive morphology have been described. Herein we described 3 additional cases of <i>YAP1::NR4A3</i> fused-poromas. All specimens harbored a biphasic appearance with a superficial part composed of anastomosing strands of monotonous poroid cells, while the deepest portion was organized as micronodules containing numerous glandular formations with eosinophilic secretory material. Immunohistochemistry revealed loss of YAP1 expression in both tumor parts. Targeted RNA sequencing confirmed the presence of an in frame <i>YAP1::NR4A3</i> fusion transcript in two cases. <i>NR4A3</i> rearrangement was demonstrated by FISH in the last specimen. To conclude, we report three cases of poromas with <i>YAP1::NR4A3</i> fusions, confirming these tumors as a distinctive morphologic variant of poroma and proposed the term “acinar variant” for this latter.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

The acinar variant of poroma: a series of 3 cases with YAP1::NR4A3 fusion

  • 期刊论文

  • 发表日期：

    2026年3月23日

查看全文
  • Jeanne Breton,
  • Daniel Pissaloux,
  • Kerman Zyani,
  • Sebastien Taix,
  • Christelle Zardi,
  • Nicolas Ortonne,
  • Thibault Kervarrec

摘要

Poromas are benign sweat gland adnexal neoplasms that include four distinct architectural variants i.e. classical poroma, hidroacanthoma simplex, poroid hidradenoma and dermal duct tumor. Most of the poromas display oncogenic YAP1::MAML2 or YAP1::NUTM1 fusions and previous studies have suggested a correlation between the YAP1’s fusion partner and the tumor morphology. Recently, two cases of YAP1::NR4A3 fused-poromas with distinctive morphology have been described. Herein we described 3 additional cases of YAP1::NR4A3 fused-poromas. All specimens harbored a biphasic appearance with a superficial part composed of anastomosing strands of monotonous poroid cells, while the deepest portion was organized as micronodules containing numerous glandular formations with eosinophilic secretory material. Immunohistochemistry revealed loss of YAP1 expression in both tumor parts. Targeted RNA sequencing confirmed the presence of an in frame YAP1::NR4A3 fusion transcript in two cases. NR4A3 rearrangement was demonstrated by FISH in the last specimen. To conclude, we report three cases of poromas with YAP1::NR4A3 fusions, confirming these tumors as a distinctive morphologic variant of poroma and proposed the term “acinar variant” for this latter.