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Aims: Juvenile fibroadenomas (JFA) are biphasic fibroepithelial lesions (FEL) usually occurring in adolescent female patients. Giant (G) JFA, like other FEL, may exhibit prominent pseudoangiomatous stromal hyperplasia (PASH)‐like change. We sought to determine clinicopathological and molecular characteristics of GJFA with and without PASH. Methods and results: Archives were searched for cases of GJFA (1985–2020). All were stained for androgen receptor (AR), beta‐catenin, CD34 and progesterone receptor (PR). Cases were sequenced using a custom 16‐gene panel – MED12 (exons 1 and 2), TERT promoter (–124C>T and –146Ctable>T), SETD2, KMT2D, RARA (exons 5–9), FLNA, NF1, PIK3CA (exons 10, 11 and 21), EGFR, RB1, BCOR, TP53, PTEN, ERBB4, IGF1R and MAP3K1. Twenty‐seven GJFA from 21 female patients aged 10.1–25.2 years were identified. Size ranged from 5.2 to 21 cm. Two patients had multiple, bilateral and later recurrent GJFA. Thirteen (48%) cases showed prominent PASH‐like stroma. All were positive for stromal CD34, negative for AR and beta‐catenin and one case showed focal PR expression. Sequencing showed MAP3K1 and SETD2 mutations in 17 samples, with KMT2D, TP53 and BCOR aberrations in 10 (45%), 10 (45%) and seven (32%) cases, respectively. Tumours with a PASH‐like pattern had higher prevalence of SETD2 (P = 0.004) and TP53 (P = 0.029) mutations, while those without PASH had more RB1 mutations (P = 0.043). MED12 mutation was identified in one case. TERT promoter mutation was observed in four (18%), including two recurrences. Conclusions: Gene mutations along more advanced phases of the proposed FEL pathogenetic pathway in GJFA are unusual, and suggest a mechanism for more aggressive growth in these tumours. [ABSTRACT FROM AUTHOR]

Background: Few data are available regarding the management and outcomes among patients with sarcomatoid hepatocellular carcinoma (HCC) due to its rarity.Methods: Patients diagnosed with sarcomatoid HCC from 2004 through 2015 were identified in the National Cancer Data Base. Overall survival (OS) was calculated among patients with sarcomatoid versus conventional HCC using a 1:3 propensity score matching based on sex, age, and American Joint Committee on Cancer (AJCC) stage of disease.Results: The final analytic cohort included 104 patients with sarcomatoid HCC and 312 patients with conventional HCC. Patients with sarcomatoid HCC more often had a larger median tumor size (8.5 cm vs 5.4 cm; P < .001) and poorly or undifferentiated tumors (52.9% vs 13.8%; P < .001) compared with patients who had conventional HCC. 5-year OS was worse among patients with sarcomatoid versus conventional HCC (5.7% vs 30.1%; P < .001). Subgroup analysis demonstrated worse 5-year OS among patients with sarcomatoid versus conventional HCC among patients treated with either curative-intent or palliative therapies. Stage-specific subgroup analysis indicated a worse OS among patients with AJCC stage I, stage II, or stage III sarcomatoid HCC. On multivariable analysis, uninsured status, advanced AJCC stage (stage III/stage IV), and histological sarcomatoid subtype were independently associated with worse outcomes (all P < .05).Conclusions: Sarcomatoid HCC is a very rare variant of HCC, which was associated with larger tumor size and worse tumor grade on presentation. On propensity score matched analyses that controlled for known confounding factors, patients with sarcomatoid HCC had a worse stage-for-stage long-term survival compared with patients who had conventional HCC. [ABSTRACT FROM AUTHOR]