|Year : 2019 | Volume
| Issue : 2 | Page : 41-44
Carcinosarcoma of ovary with long-term overall survival
Priyanka Priyadarshini1, Saroj Kumar Das Majumdar2, Dillip Kumar Parida2
1 Department of Obstetrics and Gynaecology, Calcutta National Medical College and Hospital, Kolkata, West Bengal, India
2 Department of Radiotherapy, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
|Date of Web Publication||18-Sep-2019|
Dr. Priyanka Priyadarshini
Department of Obstetrics and Gynaecology, Calcutta National Medical College and Hospital, Kolkata - 700 014, West Bengal
Source of Support: None, Conflict of Interest: None
Carcinosarcoma or mixed Müllerian malignant tumors are a rare entity representing 1% of all the malignant ovarian neoplasms. It has poor prognosis, and the survival outcome is worst. We report a case of carcinosarcoma of the ovary in a 68-year-old female. The patient was treated with cytoreductive surgery followed by platinum- and taxane-based combination chemotherapy regimen. Now, the patient is under regular follow-up with long-term overall survival for 76 months without any disease. Therefore, our case highlights the importance of cytoreductive surgery followed by adjuvant chemotherapy with platinum- and taxane-based combination regimen in the form of improved survival demanding a long-term follow-up for such cases.
Keywords: Carcinosarcoma, ovary, survival
|How to cite this article:|
Priyadarshini P, Majumdar SK, Parida DK. Carcinosarcoma of ovary with long-term overall survival. Oncol J India 2019;3:41-4
| Introduction|| |
Ovarian carcinosarcoma (OCS) is also known as malignant mixed Müllerian tumor. It is a rare gynecological malignancy and accounts for 1% among all ovarian malignancies. The origin of the tumor can occur anywhere along the female genital tract and in the peritoneum, with the most common site being uterus followed by other rare sites such as ovary, vagina, cervix, and Fallopian tube More Detailss. Histologically, OCS is a mixed tumor composed of carcinomatous and sarcomatous components. The carcinomatous components are usually adenocarcinoma and rarely squamous cell carcinoma. However, the sarcomatous components are either homologous, including endometrial stromal sarcoma, fibrosarcoma, and leiomyosarcoma, or heterologous. The clinical course of OCS is aggressive having poor response to treatment and poor overall survival rate. We report a case of carcinosarcoma of the ovary in a 68-year-old female, and the case was successfully treated with surgery followed by chemotherapy having a long-term overall survival.
| Case Report|| |
A 64-year-old postmenopausal female presented with a chief complaint of pain in the lower abdomen without any relevant medical history. Her physical examination showed an ill-defined nontender mass in the left-sided lower abdomen without any distension of the abdomen or fluid thrill. Her routine hematological parameters, liver function tests, and renal function tests were within normal limit. The serum marker CA-125 level was 102 U/ml. The serum carcinoembryonic antigen level was within normal limit.
Ultrasound of the abdomen and pelvis showed a mixed solid and cystic mass in the left adnexa. Contrast-enhanced computed tomography (CECT) scan of the abdomen and pelvis showed a heterogeneous enhancing mass of size approximately 11 cm × 10.5 cm × 8 cm in the left adnexa consisting of both solid and cystic components. Further investigations with CECT scan of the pelvis, abdomen, and thorax ruled out the presence of omental deposits, peritoneal metastasis, and liver and lung metastases.
Optimal cytoreductive debulking surgery was done under general anesthesia. The surgical procedure includes total abdominal hysterectomy, bilateral salpingo-oophorectomy, omental biopsy, appendicectomy, and bilateral pelvic lymph node dissection. Gross examination showed the specimen size of approximately 18 cm × 16 cm × 15.5 cm with normal uterus. There was the presence of a solid mass in the left ovary measuring 10 cm × 8 cm × 6.5 cm, which is brownish-black with a lobulated surface. The cut section is variegated with extensive areas of hemorrhage and necrosis [Figure 1]. Microscopic examination revealed both the carcinomatous and sarcomatous components with the presence of necrosis [Figure 2]. Sarcomatous components consist of highly pleomorphic cells with the presence of bizarre nuclei and also many tumor giant cells. Mitotic index is very high [Figure 3]a and [Figure 3]b. With these findings, a diagnosis of carcinosarcoma of the ovary was made. On immunohistochemical examination, vimentin, pan-cytokeratin (CK), and epithelial membrane antigen (EMA) markers were found to be positive [Figure 4]a, [Figure 4]b, [Figure 4]c.
|Figure 1: Left ovarian mass of size about 9.5 cm × 8 cm × 6 cm having brownish-black with a lobulated surface, and cut section of the tumor showing extensive areas of hemorrhage and necrosis|
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|Figure 2: Microscopic examination (H and E, ×100) showing both the carcinomatous and sarcomatous components with necrosis in the lower field|
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|Figure 3: Histosection showing (a) both the carcinomatous and sarcomatous components in the same field with very high mitotic index and (b) sarcomatous components consisting of highly pleomorphic cells with the presence of bizarre nuclei along with many tumor giant cells (H and E, ×400)|
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|Figure 4: Immunohistochemistry examination (×100) showing (a) vimentin positive in the sarcomatous component of the tumor, (b) pan-cytokeratin positive in the epithelial components of the tumor, and (c) epithelial membrane antigen positive in the epithelial cells|
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In view of aggressive nature of carcinosarcoma histopathology, the patient was received six cycles of adjuvant chemotherapy with paclitaxel and platinum-based combination regimen. At present, the patient was under regular follow-up for the last 76 months. During her last follow-up, serum CA-125 level was within normal limit and CECT scan showed no residual, recurrent, or metastatic lesions.
| Discussion|| |
In the United States, the highest mortality and lowest 5-year survival among all gynecological tumors are seen in ovarian malignancies. Carcinosarcoma is a poorly differentiated epithelial ovarian cancer, i.e., metaplastic carcinoma. It has an older mean age of onset in comparison to epithelial ovarian cancers. The median age of presentation for carcinosarcoma is 65 years, and our case had presented at the age of 64 years. The staging of carcinosarcoma of the ovary is done using the same staging system as that for ovarian cancer.
The clinical presentation for the carcinosarcoma is similar to that of epithelial ovarian cancer, but with worse outcomes. The majority of women (75%–80%) with carcinosarcoma present with Stage III or IV and spread beyond the ovary (>90%) at the time of diagnosis.,
Both the clinical and radiological findings of OCS are similar to other ovarian surface epithelial tumors. Therefore, it is difficult to make preoperative diagnosis of such tumor. Furthermore, CA-125 level is not elevated in all the cases. Menon et al. in their study found that preoperative rise in the serum CA-125 level in 9 of 12 cases of carcinosarcoma. In our case, the preoperative serum marker CA-125 level was elevated, i.e., 66 U/ml. Preoperative serum CA-125 level >75 U/ml is associated with poor outcome. In our case, the serum CA-125 level was 102 U/ml but having a long-term survival.
Immunohistochemical examination detects marker for both the epithelial and mesenchymal components. Anti-CK monoclonal antibodies and anti-EMA markers are used to detect the epithelial component. However, vimentin, CD10, smooth muscle actin, desmin, and myoglobin are used to detect the mesenchymal components. In our case, immunohistochemical examination revealed vimentin, pan-CK, and EMA markers to be positive, supporting the above literature.
Advanced age and stage, suboptimal cytoreduction, stromal predominant tumors, and tumors with serous epithelial component are the different adverse prognostic factors., Carcinosarcoma is a poorly understood disease with aggressive behavior having rapid progression and dismal survival rate. The disease has poor response to both surgery and chemotherapy treatment. Due to paucity of data, the optimal therapeutic strategies for the disease are unclear.
Data show that maximal cytoreduction correlates with better progression-free and overall survival in carcinosarcoma, and complete cytoreduction should be the goal of surgical treatment. Adjuvant chemotherapy should be given in pathological Stage I–IV disease, and the chemotherapy regimens are same as in epithelial ovarian cancer. Platinum-based chemotherapy is the key drug for OCS, and combination chemotherapy such as carboplatin and paclitaxel or ifosfamide exhibits a higher response rate than single-agent platinum chemotherapy. However, carcinosarcoma has poor response to chemotherapy having response rates of about 20%.
The average survival for the carcinosarcoma of the ovary is <2 years. A case–control study by Rauh-Hain et al. showed that the median progression-free survival for carcinosarcoma of the ovary was 11 months, whereas it was 16 months for epithelial ovarian cancer. Furthermore, they reported a median overall survival of 24 months for carcinosarcoma and 41 months for epithelial ovarian cancers. Carcinosarcoma at advanced stage disease has a poor 5-year overall survival rate of 7%–20% and a median survival of 4–27 months.
OCS has a worse progression-free and cause-specific survival in comparison to serous adenocarcinoma of the ovary. Brown et al. in a study reported a median survival of 14.8 months in patients with optimally debulked International Federation of Gynecology and Obstetrics (FIGO) Stage III OCSs as opposed to 3.1 months for suboptimally debulked Stage III disease, with a significantly lower objective response rate to platinum therapy.
Harris et al. in a study found poor overall median survival of 8.7 months for carcinosarcoma with the 1-year survival of 40% and the 5-year survival of 7.5% as compared to approximately 15%–40% 5-year survival in epithelial ovarian carcinoma.
OCS is a rare entity with aggressive clinical behavior. It usually presents in advanced stages having poor prognosis. In our report, the case was diagnosed in Stage I and has a long-term survival.
| Conclusion|| |
Carcinosarcoma of the ovary is a rare entity. Instead of aggressive clinical course, the overall survival status is unclear due to paucity of data. Optimal debulking cytoreductive surgery followed by adjuvant chemotherapy should be the treatments. The present case is noteworthy with a survival of about 76 months and demands further research in the area of its early detection and optimal management for better outcomes.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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