Cervical Mullerian Adenosarcoma with heterologous sarcomatous overgrowth: a fourth case and review of literature

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.

Assessment of tumor size as a useful marker for the surgical staging of endometrial cancer

“…Endometrial cancer, different from non-epithelial cancer of corpus uteri (19,20), is a gynecological malignancy with an elevated prevalence and a low mortality rate in the initial stages, especially in developed countries. These data are a result of the greater number of diagnostic instruments, increased average life expectancy and diagnosis in older patients.…”

Mullerian adenosarcoma of the cervix: case report

“…23 The ages in which cervical MA occur have shown a wide variation, with the youngest reported case being in an 10 year old girl and oldest being 72 years of age. 6,24 Mean age of cases reported worldwide has been 33±18 years. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]24 Extensive literature review shows that the most common modes of clinical presentation are irregular menstrual bleeding (41%), painless mass in the introitus (41%), postmenstrual bleeding (7%), urinary retention (7%) and postcoital bleeding and vagina discharge (3% each).…”

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]24 Extensive literature review shows that the most common modes of clinical presentation are irregular menstrual bleeding (41%), painless mass in the introitus (41%), postmenstrual bleeding (7%), urinary retention (7%) and postcoital bleeding and vagina discharge (3% each). [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]24 Our patient presented with feeling of a mass in the introitus and irregular bleeding per vaginam.…”

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] Mullerian adenosarcomas (MA) account for about 8% of all uterine sarcomas. 6 In Nigeria, even though Omoniyi-Esan et al 21 have reported 2 cases of pure sarcomas over a ten year period, our report, from available literature, is the first for MA in the country. While most commonly seen in the corpus uteri they may also be seen in other parts of the female genital tract including peritoneum.…”

Identification of multiple pathways involved in the malignant transformation of endometriosis (Review)

“…Müllerian adenosarcoma is an uncommon, but not rare, tumor which is characterized by an admixture of benign, but occasionally atypical, epithelial and a malignant, usually lowgrade, stromal component (49). The most common site is the uterine corpus endometrium (50).…”