Homologous Type of Malignant Mixed Mullerian Tumor of the Uterus Presenting As a Cervical Mass

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Homologous Type of Malignant Mixed Mullerian Tumor of the Uterus Presenting as a Cervical Mass

Umur Kuyumcuoğlu, Ahmet Kale* Department of Obstetrics and Gynecology, Dicle University Medical School, Diyarbakir, Turkey.

Malignant mixed Mullerian tumors are composed of a mixture of sarcoma and carcinoma. The carcinomatous element is usually glandular, whereas the sarcomatous element may resemble normal endometrial stroma (homologous or so- called carcinosarcoma). Here, we present a homologous type of malignant mixed Mullerian tumor of the uterus that presented as a cervical mass. We describe a 55-year-old patient who had a cervical mass arising from the uterus. We performed total abdominal hysterectomy and bilateral salpingo-oophorectomy and surgical staging (including (peritoneal washings, suspicious areas or peritoneal surfaces sampled, infracolic omental sampling, pelvic and paraaortic lymph node sampling, and appendectomy). Carcinosarcomas of the uterine cervix are extremely rare, and when a postmenopausal woman with a cervical mass is admitted to the gynecology clinic, the physician should keep in mind that the mass might be a carcinosarcoma. [J Chin Med Assoc 2009;72(10):533–535]

Key Words: carcinosarcoma, cervical mass, malignant mixed Mullerian tumors

Introduction and pelvic/paraaortic lymphadenectomy are optimal therapy for carcinosarcoma.1,2 Uterine sarcoma is a malignant tumor that arises from Here, we describe an interesting case of carcino- the smooth muscle or connective tissue of the uterus. sarcoma (homologous type of malignant mixed tumor Uterine sarcomas are rare neoplasms of the female of the uterus) that presented as a cervical mass. genital tract. They have aggressive clinical behavior and a poor prognosis. The most common histological types are represented by leiomyosarcoma, endometrial Case Report stromal sarcoma, and carcinosarcoma (also known as malignant mixed Mullerian tumor). Carcinosarcomas A 55-year-old postmenopausal woman was referred are unusual forms of a phenotypic presentation of to our clinic with a diagnosis of cervical mass. Her uterine sarcomas. Carcinosarcomas are defined as past medical history included no operation or systemic tumors exhibiting a malignant epithelial component in illness. Her presenting symptom was postmenopausal association with a malignant stromal component; the bleeding. On general examination, she was afebrile, latter may be homologous or heterologous to the native with a blood pressure of 110/72 mmHg and a pulse components of the uterus. Carcinosarcomas account rate of 84 beats/min. Abdominal examination was nor- for approximately 2–5% of all malignancies of the mal. Gynecologic examination revealed a 4 × 4 × 5cm uterine corpus. Carcinosarcomas are rare neoplasms and mass that protruded from the cervix (Figure 1). The usually associated with poor prognosis. Total abdomi- patient’s hematologic workup demonstrated hemo- nal hysterectomy and bilateral salpingooophorectomy globin of 12.0 g/dL, hematocrit of 34.1%, white

*Correspondence to: Dr Ahmet Kale, Department of Obstetrics and Gynecology, Dicle University Medical School, 21280 Diyarbakir, Turkey. E-mail: [email protected] ● Received: July 29, 2008 ● Accepted: July 23, 2009

Figure 2. Histopathology shows a carcinosarcoma. Figure 1. A 4 × 4 × 5 cm mass protruding from the cervix. blood cell count of 2.17/mm3, and platelet count of the most frequent symptom being postmenopausal 122,000/mm3. These findings suggested the possi- bleeding—occurring in 90% of cases. Carcinosarcoma bility of cervical myoma. Surgical management was grows as a large, soft, polypoid mass filling and dis- planned. tending the uterine cavity. On physical examination, The patient was taken to the operating room where uterine enlargement is present in 50–95% of patients, the mass was excised by wide local excision under and a polypoid mass may be seen within or protrud- general anesthesia in the lithotomy position. Frozen ing from the endocervical canal in approximately 50% section revealed that the mass was malignant. The of patients.1–3 Our patient’s gynecologic examination decision was made to continue the operation, and we revealed a 4 × 4 × 5 cm mass protruding from the cervix. performed total abdominal hysterectomy, bilateral Concerning etiologic factors, radiation exposure salpingo-oophorectomy and surgical staging (includ- to the pelvic area, infection with human papilloma ing peritoneal washings, suspicious areas or peritoneal virus 16, obesity, nulliparity, and exogenous estrogen surfaces sampled, infracolic omental sampling, pelvic and use might have an effect on the development of carci- paraaortic lymph node sampling, and appendectomy). nosarcoma.4 Spread of carcinosarcomas is primarily The patient was transferred to the intensive care via the lymphatic system. The most frequent areas of unit after surgery. She made a good recovery within spread are to the pelvis, lymph nodes, lungs and liver. 48 hours and was transferred to the gynecology unit. This metastatic route suggests that carcinosarcomas She was discharged home in good health 10 days after spread by local extension and regional lymph node the operation. Histopathology showed a carcinosarcoma metastasis, as with endometrial adenocarcinoma.2,3 (Figure 2). FIGO stage was IIA, pelvic and paraaortic Bulky tumors, vaginal and parametrial metastases have lymph nodes were negative, peritoneal washing cytol- been observed in carcinosarcomas of the cervix more ogy was negative, sampling biopsies from the staging frequently than in other epithelial malignancies. Spread surgery were negative, and the tumor was extended of the disease at diagnosis can explain the poor prog- to the endocervical glands. nosis related to carcinosarcomas.2 Abell and Ramirez The patient was referred to the oncology unit for reported a clinicopathologic review of 26 sarcomas adjuvant postoperative radiotherapy. She remained of the uterine cervix, and found that the median without evidence of recurrent disease 18 months after length of survival of the patients was just 11 months.5 surgical resection and pelvic radiation treatment. Another study found that in patients with carcinosarcoma confined to the uterine corpus (stage 1), the 2-year survival rate was 53%, whereas the survival rate Discussion dropped to 8.5% when the disease had extended to the cervix, vagina or parametria (stages II and III).6 Carcinosarcomas of the cervix are rare neoplasms, Treatment of uterine sarcomas should include hys- and only a few cases have been reported in the litera- terectomy, bilateral salpingo-oophorectomy, and treat- ture. Most of these cases were postmenopausal, with ment of the pelvic lymphatics by irradiation or surgery.3

534 J Chin Med Assoc • October 2009 • Vol 72 • No 10 Uterine carcinosarcoma

Pelvic and/or paraaortic lymphadenectomy is indi- When a postmenopausal woman is admitted to the cated for carcinosarcoma, which has a relatively high gynecology department with a diagnosis of a cervical incidence of lymph node metastasis.1–3 Lymph- mass, the clinician should keep in mind that the mass adenectomy appears to be important for this malig- may be a carcinosarcoma, and preoperative work-up nancy, in order to discover occult metastatic disease and surgical management should be performed as and to provide patients with a therapeutic benefit. appropriate. Carcinosarcoma also requires comprehensive peritoneal surgical staging, including peritoneal cytology, omen- tectomy, peritoneal biopsies, and, when appropriate, References tumor debulking.1–6 We performed total abdominal hysterectomy and bilateral salpingo-oophorectomy and 1. Gadducci A, Cosio S, Romanini A, Genazzani AR. The man- surgical staging (including peritoneal washings, suspi- agement of patients with uterine sarcoma: a debated clinical challenge. Crit Rev Oncol Hematol 2008;65:129–42. cious areas or peritoneal surfaces sampled, infracolic 2. Conference report. Carcinosarcoma of the uterine cervix: case omental sampling, pelvic and paraaortic lymph node report and disussion. Gynecol Oncol 2007;107:98–100. sampling, and appendectomy) in our patient. She was 3. Thomas Stovall G. Uterine cancer. In: Berek JS. Berek & referred to the oncology unit for adjuvant postopera- Novak’s Gynecology. Philadelphia: Lippincott Williams and Wilkins, 2007:1343–401. tive radiotherapy. She remained without evidence of 4. McCluggage WG. Uterine carcinosarcomas (malignant mixed recurrent disease 18 months after surgical resection Mullerian tumors) are metaplastic carcinomas. Int J Gynecol and pelvic radiation treatment. Cancer 2002;12:687–90. In conclusion, we have described a rare case of car- 5. Abell M, Ramirez JA. Sarcomas and carcinosarcomas of the uterine cervix. Cancer 1973;31:1176–92. cinosarcoma (homologous type of malignant Mullerian 6. Disaia PJ, Castro JR, Rutledge FN. Mixed mesodermal sar- tumor) of the uterus that presented as a cervical mass. coma of the uterus. AJR Am J Roentgenol 1973;117:632–6.

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