Advanced uterine adenosarcoma with sarcomatous overgrowth in a young woman

Abstract

Rationale: Uterine adenosarcoma (UA) with sarcomatous overgrowth (ASSO) is a rare and aggressive disease. Herein, wereported the case of a young patient with advanced uterine ASSO. Patients concerns: A 29-year-old woman with the diagnoses of endometrial polyp and adenomyosis underwent hysteroscopic endometrial polypectomy for the giant endometrial polyp. Postoperative regular ultrasound scan indicated thickened endometriumand an ill-defined mass with continuous enlargement in the myometrium of the posterior wall of the uterus, which was considered as an adenomyoma. Two years after hysteroscopy, she was re-admitted due to lower abdominal distension and large pelvic mass. At that time, she had taken oral short-acting contraceptives for 2.5 years. Diagnoses: Magnetic resonance imaging (MRI) of the pelvis revealed an irregular mass with the size of 12∗56∗107 mm3 in the right annex area, without distinct border with the rectum, moreover, an uneven intrauterine echo that has no obvious boundary with uterine wall. Right ovarian cancer and adenomyoma were initially considered. Interventions: The patient received transperitoneal retroperitoneal pelvic combined with total viscera resection, including uterus, bilateral appendages and rectum, omentectomy, appendectomy, lymphadenectomy, and ileostomy. Postoperative pathology confirmed ASSO in the uterine cavity and muscular layer, the whole cervical duct and the right adnexal. She underwent 2 systemic chemotherapy sessions after the surgery. The chemotherapy regimen was ifosfamide 2.5 g day 1 to 3, with liposomal doxorubicin 40 mg day 1. Outcomes: The final diagnosis was uterine ASSO, International Federation of Gynecology and Obstetrics stage IVa. The patient has been following-up so far, with no progression. Lessons: Review of the case indicated that history of long-term oral short-acting contraceptives and giant endometrial polyps may be the high-risk factors for UA. For patients with high-risk factors, the follow-up ultrasound scan should be more frequently conducted. Moreover, 3D-ultrasound, MRI and outpatient hysteroscopy are recommended for routine screening. Placement of levonorgestrel-releasing intra-uterine system after hysteroscopy may be an effective intervention for patients with a high risk of giant polyps. Cluster of Differentiation 10, Estrogen receptor, Progesterone receptor, and nuclear antigen may be predictors for prognosis and selection of individualized treatment program.

Case Report: Uterine Adenosarcoma With Sarcomatous Overgrowth and Malignant Heterologous Elements

“…Occasionally other types of the epithelium (mucinous, squamous, secretory, or clear cells) may be found. Glands are often seen in the myometrial invasion areas of uterine adenocarcinoma, suggesting that, despite its inactive glandular appearance, this component is an active part of the neoplasm ( 1 , 2 , 4 ). The stromal component is the most striking feature of these tumors, in which a low-grade sarcoma with variable cytological atypia (generally low to moderate) is seen.…”

“…Areas of heterologous differentiation ( 1 , 8 , 10 ) (skeletal muscle, lipoblasts, cartilage, etc. ), or of stromal overgrowth (which is defined as a sarcomatous component that occupies 25% or more of the total tumor volume) ( 2 , 10 ). Heterologous components can be observed in the last cases, especially rhabdomyosarcoma, and this represents a more aggressive clinical course.…”

“…It is the sixth most diagnosed cancer in women and the second most exclusively diagnosed female genital tract cancer ( 1 ). Uterine adenosarcoma (UA) is a mixed tumor of the uterus consisting of a benign glandular epithelium and a malignant mesenchymal component, which was reported as müllerian adenosarcoma by Clement and Scully in 1974 ( 2 , 3 ). It is a rare tumor that corresponds to 8% of all uterine sarcomas and <0.5% of uterine malignant tumors ( 4 , 5 ).…”

Uterine adenosarcoma with sarcomatous overgrowth and rhabdoid features: A rare case

“…Extrauterine adenosarcoma has been reported in younger women and is considered more aggressive than its uterine counterpart. About 33-50% of such cases have shown the presence of sarcomatous overgrowth,[ 2 ] which imparts poor prognosis with a high risk of recurrence. [ 3 ] Heterologous elements, i.e., rhabdomyosarcoma and leiomyosarcoma, have also been identified in the sarcomatous overgrowth.…”

Imaging findings of uterine adenosarcoma with sarcomatous overgrowth: two case reports, emphasizing restricted diffusion on diffusion weighted imaging

“…Although MRI findings of adenosarcoma without SO have been described in the literature [ 4 , 5 ], the imaging findings in adenosarcoma with SO may be poorly defined. [ 6 ].…”