Abstract
BackgroundUterine sarcomas are relatively rare tumors that account for approximately 1-3% of female genital tract malignancies and between 4-9% of uterine cancers. Less than 8% of all cases are Mullerian adenosarcoma, a distinctive uterine neoplasm characterized by a benign, but occasionally atypical, epithelial and a malignant, usually low-grade, stromal component, both of which should be integral and neoplastic constituents of the tumor. Mullerian adenosarcoma with sarcomatous overgrowth (MASO) is a very aggressive variant, associated with post-operative recurrence, metastases, even when diagnosed in early stage.Case PresentationWe present a fourth MASO case derived from uterine cervix in a 72-year-old woman with metrorrhagia and a polypoid mass protruding through the cervical ostium. Total abdominal hysterectomy, bilateral salpingo-oophorectomy, systematic pelvic lymph node dissection, omental biopsy and appendectomy were performed. Surgery treatment was associated with adjuvant whole-pelvis radiation (45 Gy) and adjuvant chemotherapy (cisplatin/ifosfamide). After nine months of follow up, the patient was free of tumor.ConclusionsThe rarity of MASO of the cervix involves a management difficult. Most authors recommend total abdominal hysterectomy, usually accompanied by bilateral salpingo-oophorectomy. There is no common agreement on staging by lymphadenectomy during primary surgery and adjuvant chemo-radio therapy.
Highlights
Uterine sarcomas are relatively rare tumors that account for approximately 1-3% of female genital tract malignancies and between 4-9% of uterine cancers
Three new classifications have been developed: staging for leiomyosarcomas and endometrial stromal sarcomas, staging for adenosarcomas, staging for carcinosarcomas. Particular interest in this treatment is covered by Mullerian adenosarcoma, whose term was introduced by Clement and Scully [6] in 1974 for a distinctive uterine neoplasm characterized by a benign, but occasionally atypical, epithelial and a malignant, albeit usually low
Uterine adenosarcomas are relatively rare tumors, whose incidence appears increased in the last years
Summary
Uterine adenosarcomas are relatively rare tumors, whose incidence appears increased in the last years. MA can be distinguished from adenofibroma (both epithelial and stromal components benign) using the criteria defined as unique to adenosarcoma such as, a marked degree of atypia of mesenchymal cells, a histological malignant element, the presence of myometrial invasion, and two or more mitotic figure per 10 HPF[7,25] These features are not always present; they are less applicable in the discrimination of malignant degree. Some cases presented with recurrent cervical polypoid lesions that were initially considered benign form, probably due to the irregular appearance of the stromal component, with normal areas adjacent to others with higher stromal density, mitotic activity, and atypia In those cases, most patients subsequently return for recurrent endometrial and endocervical polyps, which are reinterpreted as adenosarcoma[7,19,20,21,22,23].
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