Müllerian Adenosarcoma with Sarcomatous Overgrowth of the Cervix

Abstract

Müllerian adenosarcoma (MS) is a rare neoplasm of uterine cervix composed of benign epithelial and malignant stromal components. An aggressive variant of adenosarcoma, müllerian adenosarcoma with sarcomatous overgrowth (MASO) is extremely rare. The difference between MS and MASO is the pure high grade sarcoma features in MASO. In this report we present a MASO case, derived from uterine cervix of a 60 year-old-female patient presenting as a cervical polypoid mass, to our knowledge the second case of the English literature. In spite of sarcomatous overgrowth, high mitotic activity and huge tumor size of 12,5 cms, it displayed no myometrial invasion, vascular invasion and heterologous elements. The patient has been clinically free of disease for 14 months of follow up after total abdominal hysterectomy and bilateral salpingo-oopherectomy. The difficulties in diagnosis and treatment of this entity will be evaluated in this report.

Mullerian adenosarcoma (heterologous) of the cervix with sarcomatous overgrowth: a case report with review of literature

“…However, the present case occurred in a young unmarried girl who presented with a friable hemorrhagic tumor replacing the entire uterine cervix. 4,5 On histological examination a clear demarcation was seen in the low grade and high grade sarcomatous areas with pure sarcomatous components constituting approximately 70% of the tumor, suggesting a diagnosis of MASO. The main difference between our case and the previous two reported cases is that the present case occurred in a young unmarried girl with tumor replacing the entire uterine cervix, and histologically showing malignant heterologous elements with no myometrial invasion ( Table 1).…”

“…6,7 Only 2% of adenosarcomas arise in the cervix, with fewer than two dozen cases documented so far in the literature, however only two cases of MASO are reported in the uterine cervix and none of them had heterologous elements. 4,5 Since the proportion of the glandular component to the sarcomatous component in usual adenosarcomas can vary from one area of the tumor to another, and focal areas within an otherwise typical adenosarcoma may contain rare or no glands, the diagnosis of MASO must be rendered only when a pure sarcoma, similar to, or of a higher grade than that of the underlying adenosarcoma, accounts for at least one-quarter of the tumor after thorough histological sampling of the hysterectomy specimen. 3 Although MA is regarded as less aggressive, and of lower malignant potential than other malignant mullerian mixed tumors, some unfavorable prognostic factors have been listed for a MASO, high mitotic rate, heterologous elements, deep myometrial invasion, necrosis, and extrauterine spread.…”

“…MASO in the cervix is an extremely rare entity with only two cases being reported to date in the English literature, which occurred in a middle-aged and an elderly female and clinically presented like a cervical polyp, however in none of these cases were heterologous elements identified. 4,5 In this report, we describe a case of MASO of the uterine cervix with heterologous elements which occurred in a young unmarried girl. To the best of our knowledge such a case has not been reported in the English literature before.…”

Mullerian adenosarcoma of the cervix: case report

“…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 Our patient presented with feeling of a mass in the introitus and irregular bleeding per vaginam.…”

“…These tumours may affect any age group including children. [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.…”