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

Abstract

Mullerian adenosarcoma is a rare biphasic malignant neoplasm of the cervix characterized by an admixture of benign epithelial elements and a malignant sarcomatous stromal component, which may be either homologous or heterologous. An aggressive variant of adenosarcoma, mullerian adenosarcoma with sarcomatous overgrowth (MASO) is extremely rare, with only two such cases being reported in the English literature to date. In this report we present a case of MASO of uterine cervix with heterologous elements in a 15-year-old unmarried girl presenting with foul smelling menstrual bleeding and passage of fleshy masses. Because MASO with heterologous elements seems to appear at the earliest stages of reproductive lifespan in women, and have an uncertain malignant potential, gynecologists and pathologists should be aware and think about the possibility of this tumor.

Embryonal rhabdomyosarcoma of the uterine cervix: a report of 14 cases and a discussion of its unusual clinicopathological associations

“…8,23 Duggal et al 23 reported the case of a 15-year-old female patient with a cervical neoplasm composed of benign endocervical glands with a ‘low-grade stromal sarcoma’ and a ‘high-grade sarcoma’ constituting 70% of the tumor area; as described, this tumor is the archetype of an adenosarcoma with sarcomatous overgrowth. By contrast, the second case, also a 15-year-old female patient, presented with a friable cervical mass with benign glands, cartilage and embryonal rhabdomyosarcoma with features of sarcoma botryoides.…”

Mullerian adenosarcoma of the cervix: case report

“…The occurrence of heterologous differentiation, as seen in our case, has been about 69% and mostly as cartilage, bone and skeletal muscle. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] Such heterologous elements, usually cartilaginous, as seen in this case, may also be seen in adenofibroma, carcinosarcoma and embryonal rhabdomyosarcoma (ERMS). Like ERMS MAs may show cambium-like disposition but phyllodes-like histology seen in MA is not present in ERMS.…”

“…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.…”

“…Treatments given have included hysterectomy, with or without bilateral salpingo-oophorectomy, with or without pelvic lymph node clearance and with or without adjuvant chemo-and/ or radiotherapy. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] However, the underlying principle of management is to prevent recurrence and/or metastasis. In staging the disease it is our opinion that there may be need to carry out endometrial curettage, vaginal biopsy as well as exploratory laparotomy to exclude spread to other tissues because of their anatomical contiguity.…”

“…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.…”