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Year : 2013  |  Volume : 56  |  Issue : 2  |  Page : 176-177
Carcinosarcoma in dermoid cyst of ovary: An extremely rare malignant transformation


1 Department of Pathology, S.C.B. Medical College, Cuttack, Odisha, India
2 Associate Professor, Department of O & G, S.C.B.Medical College, Cuttack, India

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Date of Web Publication23-Sep-2013
 

How to cite this article:
Kar A, Kar T, Pattnaik K, Biswal P. Carcinosarcoma in dermoid cyst of ovary: An extremely rare malignant transformation. Indian J Pathol Microbiol 2013;56:176-7

How to cite this URL:
Kar A, Kar T, Pattnaik K, Biswal P. Carcinosarcoma in dermoid cyst of ovary: An extremely rare malignant transformation. Indian J Pathol Microbiol [serial online] 2013 [cited 2020 May 29];56:176-7. Available from: http://www.ijpmonline.org/text.asp?2013/56/2/176/118691


Malignant transformation in a dermoid cyst of ovary is very rare, estimated to be seen in less than 2% of cases. Carcinosarcomatous change is even rarer with only a handful of cases reported till date. [1] We report here a case of carcinosarcoma arising from dermoid cyst of ovary comprising of squamous cell carcinoma and pleomorphic sarcoma with focal myogenic differentiation.

A 52-year-old female presented with left lower abdominal pain and a large pelvic mass. Ultrasonography revealed a large left adnexal cystic mass with fluid level and a small solid nodule [Figure 1]. Patient underwent total hysterectomy with bilateral salpingo-oophorectomy. The ovarian tumor was large measuring 18 12 10 cm. It was unilocular cystic mass filled with hair and yellow colored cheesy material. The thickness of cyst wall was 0.5-1.5 cm and a solid area measuring 3 cm in diameter was seen in one pole [Figure 2]. The left ovary appeared normal. The uterus was atrophic with keratinized cervix. Histopathological examination of the cyst wall showed a stratified squamous epithelial lining with focal dysplastic change and transition to infiltrating carcinoma with underlying dermal appendages. Hematoxylin and eosin stained sections from the solid area showed a malignant tumor with two distinct components. One of the components was epithelial showing diffuse squamous differentiation with extensive keratinization [Figure 3]. There was nuclear anaplasia and atypical mitosis. The other component revealed features of pleomorphic spindle cell sarcoma with marked pleomorphism and hyperchromasia, [Figure 3] frequent mitoses and necrosis. The epithelial and mesenchymal components were closely intermixed without well-demarcated boundaries. On immunohistochemistry, the malignant epithelial cells were reactive for high molecular weight keratin [Figure 4]a and P63 protein [Figure 4]b] while the pleomorphic spindle cell sarcomatous component showed diffuse positivity for vimentin [Figure 4]c and focally for smooth muscle actin [Figure 4]d. Desmin, S100 protein and CD10 were negative in both components.
Figure 1: Ultrasounography showing a large ovarian cyst

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Figure 2: Gross photograph showing cut opened ovarian cyst with a solid nodule

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Figure 3: Malignant epithelial and stromal components of carcinosarcoma, H and E ×100

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Figure 4: Immunohistochemistry in carcinosarcoma: (a) High molecular weight keratin positivity in epithelial component ×10 (b) P63 positivity ×10. (c) Smooth muscle actin positivity in sarcomatous component ×10. (d) Vimentin positivity in sarcomatous tissue ×10

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The present case was a large ovarian cyst with characteristic squamous epithelial lining as described by Arora and Haldane who found adenocarcinoma in combination with sarcoma in dermoid cyst of ovary. [2] This tumor should be differentiated from pure malignant mixed mullerian tumor (MMMT) and immature teratoma. MMMT occurs in still older age group, are usually solid; lack the squamous epithelial lined cyst and also the transition to carcinoma in squamous epithelium and shows mucin expression. The presence of benign epithelium, squamous cell carcinoma and pleomorphic sarcoma in a dermoid cyst in our case rules out the possibility of origin from an immature teratoma. Furthermore, immature teratoma occurs in younger age group, usually shows neural tissue with variety of mesenchymal differentiation and most important, we did not identify any immature element even after thorough examination. [1] According to Allam-Nandyala et al. the distinction between pure MMMTs of the ovary versus collision tumors versus a squamous cell carcinoma with sarcomatous transformation is of academic interest. [1] The coexistence of two malignancies in a benign teratoma in a postmenopausal female confirms our diagnosis. In addition, no immunoreactivity for HMWK was noted in the sarcomatous component. We believe that the histogenesis of this tumor is from the neoplastic totipotential stem cells present in the atypical basal cells of the squamous epithelium. [3]

Malignant transformation in a mature cystic teratoma is a rare event. Risk factors for malignancy include age more than 45 years, tumor diameter greater than 10 cm and rapid growth. [4] Al-Rayyan recommended that a higher suspicion of malignancy should be maintained while examining rapidly growing dermoid cysts larger than 10 cm in females over the age of 45 years. The prognosis of patients with malignant transformation in teratoma is very poor, with most women dying within 1 year. However, the prognosis is better if the tumor is limited to one ovary, with an intact capsule not adhered to adjacent structure and with uniform thickness of cyst wall. In our case, the tumor was limited to left ovary without rupture and no adhesion to surrounding structures. Prognosis of patients with malignant transformation is very poor with life expectancy less than 1 year. Since the role of platinum based chemotherapy in these patients is not well- documented (due to rarity of the lesions), so the extensive surgery like hysterectomy with bilateral salpingo-oophorectomy was all the treatment given to our patient so as to provide her a better palliative life without the complications of chemotherapy.

In conclusion, we report a very rare occurrence in surgical pathology specimens and for the awareness of pathologists that all the dermoid cysts should be examined histologically by giving multiple sections with special attention to any unusual or solid areas.


   Acknowledgment Top


The authors would like to thank Prof. B. K. Mohanty (Retd.) AHRCC, Cuttack for his kind support.

 
   References Top

1.Allam-Nandyala P, Bui MM, Caracciolo JT, Hakam A. Squamous cell carcinoma and osteosarcoma arising from a dermoid cyst - A case report and review of literature. Int J Clin Exp Pathol 2010;3:313-8.  Back to cited text no. 1
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2.Arora DS, Haldane S. Carcinosarcoma arising in a dermoid cyst of the ovary. J Clin Pathol 1996;49:519-21.  Back to cited text no. 2
[PUBMED]    
3.Cabibi D, Martorana A, Cappello F, Barresi E, Di Gangi C, Rodolico V. Carcinosarcoma of monoclonal origin arising in a dermoid cyst of ovary: A case report. BMC Cancer 2006;6:47.  Back to cited text no. 3
[PUBMED]    
4.Al-Rayyan ES, Duqoum WJ, Sawalha MS, Nascimento MC, Pather S, Dalrymple CJ, et al. Secondary malignancies in ovarian dermoid cyst. Saudi Med J 2009;30:524-8.  Back to cited text no. 4
[PUBMED]    

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Correspondence Address:
Asaranti Kar
Department of Pathology, S.C.B. Medical College, Qrs. No.-JO-1, S.C.B. Medical College Campus, Cuttack - 753 007, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.118691

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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