The current criteria for adenosarcoma is phyllodiform cleft-like or dilated glands (rigid cysts) lined by benign endometrial or ciliated epithelium surrounded by a distinct cuff of neoplastic stroma, which is typically hypercellular relative to nearby benign tissue (cambium layer). Stromal aypia or mitosis can be minimal.
However, in subset of tumor with focal architecture abnormalities but not enough for adenosarcoma, WHO mentioned benign endometrial or cervical polyps with unusual morphology; Dr. Chapel and Dr. Nucci's paper proposed terminology of atypical uterine polyps. Previously called adenofibroma was not recognized anymore and those cases were found to be low grade adenosarcoma
Key finding in their paper:
Sarcomatous overgrowth and aggressive clinical behavior in adenosarcomas are linked to specific genetic alterations, including MYBL1 amplification, CDKN2A deletion, and mutations in BAP1, TP53, and RB1. High-grade features like stromal atypia and sarcomatous differentiation are associated with ATRX, DICER1, and TP53 mutations. Atypical uterine polyps lack these high-grade alterations but share molecular similarities with low- and high-grade adenosarcomas, particularly chr 12q13-15 amplification. This suggests a molecular continuum between these entities, raising the potential for refined risk stratification that could allow conservative management of certain adenosarcomas, benefiting young women seeking fertility preservation.
Prognosis of adenosarcoma in another study:
Among 5,496 patients, 544 (10%) had adenosarcomas. These patients were generally younger and more likely to have early-stage tumors (p<0.0001). Multivariate analysis showed that patients with adenosarcomas had a 65% lower risk of death (HR=0.35; 95% CI, 0.28-0.45) compared to those with carcinosarcomas. Five-year survival rates were higher for adenosarcomas: 79% for stage I (95% CI, 75-84%) and 48% for stage III (95% CI, 29-65%), compared to carcinosarcomas with 51% for stage I (95% CI, 49-54%) and 24% for stage III (95% CI, 20-27%).
Reference:
Chapel, D.B., Howitt, B.E., Sholl, L.M. et al. Atypical uterine polyps show morphologic and molecular overlap with mullerian adenosarcoma but follow a benign clinical course. Mod Pathol 35, 106–116 (2022). https://doi.org/10.1038/s41379-021-00946-z
Arend R, Bagaria M, Lewin SN, Sun X, Deutsch I, Burke WM, Herzog TJ, Wright JD. Long-term outcome and natural history of uterine adenosarcomas. Gynecol Oncol. 2010 Nov;119(2):305-8. doi: 10.1016/j.ygyno.2010.07.001. Epub 2010 Aug 4. PMID: 20688363.
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