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Mullerian adenosarcoma: a clinicopathologic and management review of 5 cases

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ESGO State of the Art 2018 Conference

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Abstract

Introduction Adenosarcomas are mixed Mullerian tumors of the female genital tract. Most of this type of neoplasms arise from the endometrium and with less frequency, endocervix, lower uterine segment or myometrium. However, they can be found in the ovaries or in extrauterine tissues possibly related to endometriosis. They constitute less than 0,5% of uterine malignancies and 5% of uterine sarcomas. They are characterized by a benign epithelium and a sarcomatous stroma being typically low grade. In most cases it is diagnosed in stage I, with a 5-year overall survival of 60-80% . However, adenosarcoma with sarcomatous overgrowth , defined as the presence of pure sarcoma occupying at least 25% of the tumor, show a decreased 5 year overall survival and a increased risk of recurrence. As a rule, this type of tumors affect old postmenopausal women, with a peak of incidence between sixth and seventh decades, although a 30% of the cases appear in premenopausal women. Patients with adenosarcomas usually debut with abnormal uterine bleeding. It can frequently be seen as a polypoid mass protruding through the external cervical os. Limited data of the clinicopathologic features and optimal management is available. Hysterectomy with bilateral salpingo-oophorectomy is recommended. The role of lymphadenectomy is uncertain, and currently is not indicated as the incidence of lymph node metastasis is rare. Regarding adjuvant therapy, it is not clear which adjuvant treatment should be given to improve the outcomes of these patients. Additional data is required to determine the best strategies for these patients. Material and methods: We analyze 5 cases of uterine adenosarcoma diagnosed between 2000 and 2018 in our Hospital. Mean results : The mean age of presentation is 45 (range 33-58). All the patients underwent total hysterectomy with bilateral salpingectomy at the time diagnosis was made. The bilateral oophorectomy was performed in 3 patients. In one case, a 33 year old woman with stage Ia adenosarcoma, the hysterectomy was postponed until the patient fulfilled her reproductive desires, performing surgery fifteen months after diagnosis was made. The 80% of the cases were diagnosed in stage I . The remaining 20% was a case of adenosarcoma with heterologous elements, showing striated muscle differentiation (70% rhabdomyoma and 30% embryonal rhabdomyosarcoma). Only this patient received adjuvant chemotherapy due to presence of recurrence risk factors, presenting local recurrence 6 months after surgery in vaginal vault, treated with radiotherapy. This patient currently has a followed-up period of two years. One patient show complete remission with a followed-up period of 18 years. The other patients have a period of progression-free survival of 2 years, 1 year and one has a recent diagnosis. Conclusion Adenosarcomas are rare tumors. Most cases are diagnosed in early stages. However, the presence of myometrial invasión, heterologous elements including rhabdomyoblastic differentiation , linfovascular invasión, necrosis, sarcomatous overgrowth confer a high risk

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© Copyright 2020 Morressier GmbH.
All rights reserved.