Histiocyte-Rich Renal Tumor Morphology Linked to FLCN Mutations

A study examined 11 previously unreported cases of histiocyte-rich renal epithelial neoplasms using immunohistochemistry and next-generation sequencing. The study found that 5 of 11 cases (45%) carried FLCN mutations, including at least 3 with evidence of occult Birt-Hogg-Dube syndrome, and that all FLCN-mutated neoplasms were diffusely positive for GPNMB, though this marker was also seen in some FLCN-negative cases. The authors state that recognizing this morphologic pattern may provide the first clue to the presence of occult Birt-Hogg-Dube syndrome.

Most relevant for: Pathologists evaluating renal epithelial neoplasms with oncocytic and histiocyte-rich morphology, particularly when occult Birt-Hogg-Dube syndrome is a diagnostic consideration.

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