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Highlights • Uterine adenosarcoma appears to have relatively good prognosis, especially in the absence of high-risk factors. • Lymphadenectomy and adjuvant therapy have an unclear benefit for Mullerian adenosarcoma. • Ovarian preservation can be considered in premenopausal patients with presumed uterine-confined disease., Objective To examine clinicopathologic characteristics and oncologic outcomes of patients diagnosed with Mullerian adenosarcoma and to evaluate ovarian preservation as a practical management option in early-stage disease. Methods A retrospective review was performed of 31 patients treated for uterine, ovarian, or cervical adenosarcoma at our institution between 1/2000–3/2020. Recurrence-free survival (RFS) and overall survival (OS) were analyzed with Kaplan-Meier estimates, the log-rank test, and Cox proportional hazards regression. Results Median age was 51 years (IQR: 41–68). Primary sites included uterine corpus (n = 23, 74.2%), uterine cervix (n = 7, 22.6%), and ovary (n = 1, 3.2%). Surgical management primarily consisted of total hysterectomy +/- bilateral adnexectomy +/- lymph node dissection. Fifteen (48.1%) patients underwent lymph node dissection; no patients had positive nodes. Ovaries were preserved in 6 (19.4%). Twenty-two (71.0%) patients received no adjuvant therapy, 4 (12.9%) received chemotherapy, 1 (3.2%) received chemoradiation, and 3 (9.7%) received hormonal therapy. Sarcomatous overgrowth (p = 0.04), high grade histology (p = 0.002), and greater depth of myometrial invasion (p = 0.001) were associated with decreased RFS. None of the 6 patients with ovarian preservation had recurrences. At last follow up, 21 patients (67.7%) had no evidence of disease, 7 (22.6%) were deceased due to disease, and 3 (9.7%) were deceased due to non-cancerous reasons. Conclusions Uterine adenosarcoma appears to have a relatively good prognosis, especially in the absence of risk factors, such as sarcomatous overgrowth, high grade histology, and deep myometrial invasion. Ovarian preservation may be a feasible management option with non-inferior outcomes for premenopausal women with early-stage disease. Future studies including larger patient cohorts are needed for this rare disease.

Highlights • A woman with a history of Stage IA low-risk uterine adenosarcoma presented with shortness of breath and rib pain. • She was found to have recurrent metastatic disease with resultant fatal SVC Syndrome. • Better methods to determine which patients with uterine adenosarcoma are at risk of recurrence and death are needed.

Highlights • DICER1 mutations play a significant role in gynecologic malignancy. • DICER1 may be involved in the sarcomagenesis of endometrial adenosarcoma. • The knowledge of a genetic mutation can help clarify a patient's medical history.

Highlights • Cervical Mullerian adenosarcoma is a tumor that affects reproductively aged women. • Hysterectomy had been the standard of care for these premenopausal women. • This case reports the most minimally invasive approach with no recurrence. • Accurate pathology interpretation is essential to diagnose and treat patients. • This is a rare tumor that if misdiagnosed or mischaracterized could be lethal.