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Year : 2015  |  Volume : 11  |  Issue : 4  |  Page : 1022

Carcinosarcoma of ovary with its various immunohistochemical expression: Report of a rare case

Department of Pathology, Medical College, Kolkata, West Bengal, India

Date of Web Publication15-Feb-2016

Correspondence Address:
Senjuti Dasgupta
Department of Pathology, Medical College, Kolkata - 700 073, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.147390

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 > Abstract 

Ovarian carcinosarcoma is an extremely rare tumor with an incidence of <2%. A report of such a rare case in a 40--year-old multiparous woman is being presented here. The patient complained of abdominal pain and distension. Abdominal computed tomography revealed a variegated mass in the right adnexal region. The CA-125 level was 1635 U/ml. The patient underwent laparotomy and the tumor was removed. Microscopic examination of the tumor showed presence of both carcinomatous and sarcomatous elements. Immunohistochemical analysis revealed that the carcinomatous component was positive for cytokeratin and estrogen receptor and negative for progesterone receptor. The sarcomatous component was positive for vimentin. Ki-67 was positive in 60% cells. A final diagnosis of ovarian carcinosarcoma was rendered. This case is significant owing to extreme rarity of the tumor. Records of similar cases must be maintained for future reference with regard to impact of treatment protocol followed on prognosis.

Keywords: Carcinosarcoma, immunohistochemistry, ovary

How to cite this article:
Dasgupta S, Bose D, Bhattacharyya NK, Biswas PK. Carcinosarcoma of ovary with its various immunohistochemical expression: Report of a rare case. J Can Res Ther 2015;11:1022

How to cite this URL:
Dasgupta S, Bose D, Bhattacharyya NK, Biswas PK. Carcinosarcoma of ovary with its various immunohistochemical expression: Report of a rare case. J Can Res Ther [serial online] 2015 [cited 2020 Feb 19];11:1022. Available from: http://www.cancerjournal.net/text.asp?2015/11/4/1022/147390

 > Introduction Top

Ovarian carcinosarcoma is an extremely rare malignant tumor with a reported incidence of < 2%.[1] It is an aggressive tumor with a dismal prognosis. Most tumors extend beyond ovary at the time of diagnosis. Usually the patient is a postmenopausal woman with a history of low parity.[2] Here, a case of ovarian carcinosarcoma is being reported which is significant due to rarity of occurrence of such tumors.

 > Case Report Top

A 40-year-old female patient presented with abdominal pain and distension for 3 months. She was postmenopausal and multiparous (P3+0).

General examination revealed that the patient had pallor. Abdominal examination revealed a huge non-tender swelling involving the right iliac and hypogastric regions. The approximate dimensions of the swelling were 16 × 10 cm. There was no ascites or hepatosplenomegaly. Per vaginal examination revealed that the uterus was normal in size and mobile. The fornices were clear.

Routine blood tests indicated that the hemoglobin level of the patient was 11 g/dl. Her total and differential counts were within normal limits. Blood biochemical test showed that the patient was non-diabetic.

Abdominal computed tomography (CT) revealed a variegated mass about 22 × 9 cm in dimensions, in the right adnexal region [Figure 1]. On the basis of radiologic findings, the lesion was suspected to be an ovarian neoplasm.
Figure 1: CT scan picture showing a variegated mass in the right adnexal region

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An assay for CA-125 was advised. The level of CA-125 was highly raised (1635 U/ml).

A laparotomy was planned for removal of the mass. When the abdomen was opened, hemorrhagic fluid was seen. The fluid was sent for cytologic examination. A huge mass in relation to the right ovary was found. The mass was grossly adhered to the gut and omentum. The mass was removed and adjoining gut was resected. Anastomosis of jejunum was done approximately 1 m distal to the duodenojejunal flexure. Total abdominal hysterectomy and bilateral salpingo-oophorectomy was performed. All the tissues removed were sent for histopathological examination.

Cytologic examination of the fluid did not reveal any malignant cells. Gross examination of the resected specimen revealed a large tumor mass measuring approximately 21 × 10 × 7 cm. The outer surface was irregular and nodular. Cut surface showed both solid and cystic areas along with large areas of hemorrhage and necrosis. The resected gut and uterus were grossly unremarkable.

Microscopic examination of the mass showed a biphasic tumor with both carcinomatous and sarcomatous elements. The carcinomatous component was of glandular type. The sarcomatous part was composed of spindle-shaped cells with hyperchromatic pleomorphic nuclei. No specific heterologous mesenchymal elements like skeletal muscle, cartilage, bone or adipose tissue could be seen [Figure 2]. Deposits of this tumor were found on the outer wall of small intestine and mesentery. No deposits were found in the adjoining lymph nodes. The tumor was diagnosed as carcinosarcoma of ovary (stage pT3N0MX).
Figure 2: Photomicrograph showing a tumor containing both carcinomatous and sarcomatous elements (H and E, ×400)

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Immunohistochemical analysis was performed, and it revealed that the carcinomatous component was positive for cytokeratin (CK) and estrogen receptor (ER). This element was however negative for progesterone receptor (PR). The sarcomatous component was found to be positive for vimentin. Ki-67 was found to be positive in 60% cells [Figure 3].
Figure 3: Ovarian carcinosarcoma (immunohistochemistry): (a) CK (×400)-positive carcinomatous element, (b) Vimentin (×400)-positive sarcomatous element, (c) ER (×400)-positive carcinomatous element, (d) Ki 67 (×400)-positive tumor cells of carcinosarcoma

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The patient was subsequently treated with platinum-based chemotherapy. She has been on follow-up for the past 6 months which was uneventful.

 > Discussion Top

Carcinosarcomas, also called malignant mixed mullerian tumors, occur commonly in the uterus. Their occurrence in the ovary is an extremely rare event. These tumors account for < 2% of all the ovarian malignancies.[1]

The pathogenesis of ovarian carcinosarcoma is a topic of debate. The combination theory suggests that the two components of the tumor originate from a common epithelial stem cell, whereas the collision theory states that they are of different origin.[3] An association between ovarian carcinosarcomas and pelvic irradiation has been suggested by Wei, et al.[4]

Usually the patient is a postmenopausal woman of low parity.[2] Lamb MR et al. reported a case of carcinosarcoma of ovary in a 54-year-old patient.[5] The common symptoms include abdominal pain, distension, nausea, vomiting, and weight loss.[3] In the present case, the patient was a 40-year-old multiparous woman, who presented with abdominal pain and distension.

Menon, et al. stated that the radiological features of these tumors are non-specific, being similar to ovarian epithelial tumors.[6] Dai, et al. reported a mean CA-125 level of 696.54 ± 314.06 U/ml in patients of carcinosarcoma.[1] In our patient, the CA-125 level was 1635 U/ml.

Microscopically, ovarian carcinosarcomas are subclassified into homologous and heterologous types based on the presence or absence of a stromal element containing heterologous tissue not normally present at the primary site of the tumor. The carcinomatous component may be of serous, endometrioid, clear cell, or squamous types. The sarcomatous element may either be non-specific or may simulate chondrosarcoma, osteosarcoma, rhabdomyosarcoma, or angiosarcoma.[2] Athavale R stated that tumors with stromal predominance and serous epithelial component have worse prognosis.[7]

Various authors are in unanimous agreement that the prognostic criterion of utmost importance is the initial tumor stage. Usually, the tumor is diagnosed at a late stage so that the prognosis in most cases is extremely poor.[2]

For ER-positive tumors, coexpression of PR is most likely for endometrioid carcinoma and least likely for high-grade serous carcinoma and clear-cell carcinoma.[8] The carcinomatous component, in the present case, was CK-positive, ER-positive and PR-negative, favoring a diagnosis of high-grade serous carcinoma. The sarcomatous component was vimentin positive. Ki-67 was positive in approximately 60% cells. Comparable findings were noted by Kondi-Pafiti, et al. in their study.[9]

Treatment of ovarian carcinosarcoma consists of optimal debulking surgery and chemotherapy, either neoadjuvant or adjuvant.[6] However, it has not been possible to establish an optimal chemotherapeutic regimen for this tumor, owing to its rarity.[10]

We conclude that ovarian carcinosarcoma is an extremely uncommon tumor with an aggressive course and dismal prognosis. Such cases must be meticulously recorded for future reference with regard to the treatment provided and outcome achieved.

 > References Top

Dai Y, Shen K, Lang JH, Huang HF, Pan LY, Wu M, et al. Primary sarcoma of the ovary: Clinicopathological characteristics, prognostic factors and evaluation of therapy. Chin Med J (Engl) 2011;124:1316-21.  Back to cited text no. 1
Rosai J, Ackerman. Ovary. In: Rosai J, Ackerman, editors. Rosai and Ackerman's Surgical Pathology. 10th ed. New Delhi: Elsevier; 2011. p. 1553-635.  Back to cited text no. 2
Brown E, Stewart M, Rye T, Al-Nafussi A, Williams AR, Bradburn M, et al. Carcinosarcoma of the ovary: 19 years of prospective data from a single center. Cancer 2004;100:2148-53.  Back to cited text no. 3
Wei LH, Huang CY, Cheng SP, Chen CA, Hsieh CY. Carcinosarcoma of ovary associated with previous radiotherapy. Int J Gynecol Cancer 2001;11:81-4.  Back to cited text no. 4
Lamb MR, Gertsen E, Middlemas E. Carcinosarcoma of the ovary: Case report and literature review. Tenn Med 2012;105:41-2.  Back to cited text no. 5
Menon S, Deodhar K, Rekhi B, Dhake R, Gupta S, Ghosh J, et al. Clinico-pathological spectrum of primary ovarian malignant mixed mullerian tumors (OMMMT) from a tertiary cancer institute: A series of 27 cases. Indian J Pathol Microbiol 2013;56:365-71.  Back to cited text no. 6
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Athavale R, Thomakos N, Godfrey K, Kew F, Cross P, de Barros Lopes A, et al. The effect of epithelial and stromal tumor components on FIGO stages III and IV ovarian carcinosarcomas treated with primary surgery and chemotherapy. Int J Gynecol Cancer 2007;17:1025-30.  Back to cited text no. 7
Sieh W, Köbel M, Longacre TA, Bowtell DD, deFazio A, Goodman MT, et al. Hormone-receptor expression and ovarian cancer survival: An ovarian tumor tissue analysis consortium study. Lancet Oncol 2013;14:853-62.  Back to cited text no. 8
Kondi-Pafiti A, Grapsa D, Hasiakos D, Gennatas K, Fotiou S. Carcinosarcomas of the uterus and ovary: A clinicopathologic and immunohistochemical study of 11 cases. Eur J Gynaecol Oncol 2009;30:93-7.  Back to cited text no. 9
Otsuka I, Takaya H, Takagi K, Tanaka A, Kaseki H, Izuta C, et al. Carcinosarcoma of the ovary treated with paclitaxel and carboplatin chemotherapy-a report of 4 cases. Gan To Kagaku Ryoho 2013;40:1249-53.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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