A review on uterine sarcomas and a brief report on the role of the notch pathway

Background: Uterine sarcomas represent a rare type of tumor of the uterus (3-7%) with a poor prognosis. Many of the patients will be diagnosed at an advanced stage due to lack or insufficient symptoms. The majority of these tumors, despite common origin, will require a different approach for every patient and for every different form of cancer. Objective: To conduct a review of the literature providing an evidence-based summary of the available literature regarding uterine sarcomas, with a brief report to the Notch pathway that, according to the authors, should be further investigated as therapeutic anti-angiogenic factor. Materials and Methods: Medline was searched for uterine sarcoma related articles from about 1976 to 2015 including terms such as “uterine sarcoma”, “leiomyosarcoma”, “Müllerian tumors”, and others. Results: Leiomyosarcomas (LMS) are considered the most common uterine sarcoma type and has a high risk of recurrence especially when diagnosed late. In addition to LMS, endometrial stromal sarcoma (ESS), arising from the connective tissues (stroma) of the endometrium, and adenosarcomas (mixed epithelial-nonepithelial neoplasms arising from endometrium) represent the major types of uterine sarcomas. Therapists will not easily suspect uterine sarcomas due to the absence of specific symptoms and diagnosis will be set only after hysterectomy. Conclusion: Since carcinosarcomas are more epithelial than stromal neoplasms, they are no longer categorized as uterine sarcomas. There are no sufficient symptoms for the disease. Surgery is the mainstay of therapy in early stages, while recent studies question the undefined role of adjuvant chemo/radiotherapy. The role of Notch pathway in uterine sarcoma needs to be further investigated for possible anti-angiogenic therapeutic role.

Adenosarcoma; Endometrial stromal sarcoma; Leiomyosarcoma; Müllerian tumors; Notch pathway; Uterine sarcoma

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