Phenotypic similarities within the morphologic spectrum of DICER1‐associated sarcomas and pleuropulmonary blastoma: Histopathologic features guide diagnosis in the LMIC setting

Extrapulmonary DICER1‐associated sarcomas (DS) can harbor morphological features overlapping with pleuropulmonary blastoma. We report three children with intracranial and genital tract sarcomas, suspected to have DS based on a heterogeneous yet defining combination of spindle‐cell sarcomatous and blastemal morphology, with rhabdomyomatous differentiation. Foci of immature cartilage at diagnosis (n = 2/3) and increased neuroepithelial differentiation at recurrence (n = 1) were noted. Morphological suspicion prompted somatic testing at reference centers, confirming likely biallelic, loss‐of‐function, and “hotspot” missense DICER1 variants in all three tumors. This can serve as a model for this diagnosis in resource‐limited settings and has implications for germline testing, surveillance, and tumor management.