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Year : 2016  |  Volume : 59  |  Issue : 1  |  Page : 122-123
Ovarian malignant mixed germ cell tumor: A rare combination with five germ cell components


Department of Pathology, Shrimati Kashibai Navale Medical College and General Hospital, Pune, Maharashtra, India

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Date of Web Publication9-Mar-2016
 

How to cite this article:
Gaurish SK, Avinash J, Hardas S, Kulkarni MM, Barpande CP. Ovarian malignant mixed germ cell tumor: A rare combination with five germ cell components. Indian J Pathol Microbiol 2016;59:122-3

How to cite this URL:
Gaurish SK, Avinash J, Hardas S, Kulkarni MM, Barpande CP. Ovarian malignant mixed germ cell tumor: A rare combination with five germ cell components. Indian J Pathol Microbiol [serial online] 2016 [cited 2019 Nov 13];59:122-3. Available from: http://www.ijpmonline.org/text.asp?2016/59/1/122/174876


A 14 years, postpubertal female presented to the gynecology outpatient department with a brief history of lower abdominal pain. On examination, a firm, palpable mass was noted in the right iliac fossa. Laboratory investigations revealed elevated alpha-fetoprotein (AFP) (1729.97 ng/ml) and β-human chorionic gonadotropin (β-HCG) (11331.33 mIU/ml) levels. Computed tomography (CT) scan showed a large, well-defined mixed density lesion measuring 20 cm 17 cm 9 cm in the lower abdomen, and pelvis suggesting ovarian neoplasm. CT of the thorax showed a well-defined, pleura based soft tissue lesion along the anterior wall of the left upper hemithorax. In view of the extensive disease (stage IV as per the FIGO classification), debulking of the tumor was attempted with right salpingo-oophorectomy and omentectomy.

On gross examination, the external surface of the tumor was smooth, glistening with vascular markings. Cut surface revealed a variegated appearance. Microscopic examination showed features indicative of the following components: Dysgerminoma (25%), yolk sac tumor (YST) (30%), embryonal carcinoma (EC) (5%), choriocarcinoma (5%), and immature teratoma (IT) (35%) [Figure 1] and [Figure 2].
Figure 1: (a) Macroscopic features of tumor: Cut surface shows a greyish white tumor with variegated appearance having microcystic, glistening, hemorrhagic, and necrotic areas, (b) dysgerminoma showing tumor cells having clear cytoplasm and central nuclei with prominent nucleoli. There are few lymphocytes seen, (c) yolk sac tumor showing Schiller-Duval bodies, (d) immature teratoma showing immature neuroepithelium, (e) embryonal carcinoma showing glands and papillae lined by large cells with vesicular nuclei, prominent nucleoli, and eosinophilic cytoplasm with numerous mitotic figures, and (f) choriocarcinoma showing areas of necrosis and hemorrhage, syncytiotrophoblasts, and cytotrophoblasts

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Figure 2: Immunohistochemical findings: (a) Dysgerminoma showing strong membrane immunoreactivity for placental alkaline phosphatase (×400) and (b) embryonal carcinoma showing membrane immunoreactivity for cluster of differentiation 30 (×400)

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Histopathological diagnosis of malignant mixed germ cell tumor (MMGCT) was made and the representative sections were submitted for relevant immunohistochemical (IHC) study. IHC markers done such as placental alkaline phosphatase (PLAP) showed membrane positivity in dysgerminoma and cluster of differentiation (CD) 30 showed membrane positivity in EC component [Figure 2].

Postoperative AFP levels returned to normal (9 ng/ml) whereas β-HCG levels reduced marginally (3102.15 mIU/ml) confirming the presence of tumor metastasis to the lung. The patient was referred to a private health institute for receiving four cycles of platinum based chemotherapy (bleomycin, etoposide, and cisplatin). However, within a month she presented to the emergency department with breathlessness. Repeat abdominal and thorax CT scan showed the presence of lung and liver metastasis. She succumbed to the illness in 2 days.

Ovarian MMGCT is a rare neoplasm containing combinations of two or more types of germ cell elements accounting for 8% of ovarian malignancies in children and adolescents. MMGCTs with four or more components are an unusual occurrence carrying bad prognosis. [1] To the best of our knowledge, MMGCT comprising of five components is not yet documented in the literature.

Most MMGCTs show a combination of two germ cell components. Their incidence varies from 10% to 81%. The relatively common components documented are that of dysgerminoma with YST. [1] The most common type of nondysgerminomatous tumors is YST and IT. [2] Tumors showing a combination of three or four germ cell components is also reported, their incidence being 14-31% and 2-10%, respectively. [2]

The age at presentation is the second decade. The majority of patients present with unilaterally enlarged ovary. The presenting symptoms include abdominal pain, abdominal distension, or a pelvic mass. [1] Tumor markers such as β-HCG and AFP contribute to the diagnosis, prognosis, and follow-up of the disease. [1]

The diagnosis although suspected on clinical, radiological, and hormonal studies, necessitates thorough immunohistomorphological examination to carefully find out all the types of malignant germ cell elements which may determine the patient's prognosis. [1]

IHC facilitates diagnosis of ovarian GCTs. The main markers include SALL4 and PLAP for malignant germ cell differentiation; OCT4, CD117, and D2-40 for dysgerminoma; AFP, and glypican-3 for YST; OCT4, CD30, and SOX2 for EC. [3] The present case showed CD30 positivity in EC and PLAP positivity in dysgerminoma.

Microscopically dysgerminoma is present in 80%, YST in 70%, IT in 53%, choriocarcinoma in 20%, and EC in 16% of MMGCT. EC of the ovary is an extremely rare tumor and represents only about 4% of malignant ovarian GCTs. [2] It is worth mentioning that the present case had EC as one of the components.

Some studies have found that size and histology were the major factors determining the prognosis for patients with ovarian MMGCT. The prognosis was poor for patients with large tumors when more than one-third of the tumor was composed of YST, choriocarcinoma, grade 3 IT, or EC. When the tumor was smaller than 10 cm in diameter, the prognosis was good regardless of the composition of the tumor. [4]

For patients with stage IV GCTs other than pure dysgerminoma, total abdominal hysterectomy, and bilateral salpingo-oophorectomy is recommended. Patients wishing to preserve fertility are treated with unilateral salpingo-oophorectomy. Following maximal surgical debulking, three to four courses of cisplatin-containing combination chemotherapy are indicated. [5]

The present case puts on record the rare combination of five germ cell components in a MMGCT of ovary and the need for adequate sampling for microscopic study, since the presence and quantity of the various components determines the patient's ultimate prognosis.

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Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Munemane A, Munemane M. Malignant mixed germ cell tumours of ovary. A report of two cases. Int J Med Sci Public Health 2014;3:105-7.  Back to cited text no. 1
    
2.
Moniaga NC, Randall LM. Malignant mixed ovarian germ cell tumor with embryonal component. J Pediatr Adolesc Gynecol 2011;24:e1-3.  Back to cited text no. 2
    
3.
Rabban JT, Zaloudek CJ. A practical approach to immunohistochemical diagnosis of ovarian germ cell tumours and sex cord-stromal tumours. Histopathology 2013;62:71-88.  Back to cited text no. 3
    
4.
Kurman RJ, Norris HJ. Malignant mixed germ cell tumors of the ovary. A clinical and pathologic analysis of 30 cases. Obstet Gynecol 1976;48:579-89.  Back to cited text no. 4
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5.
Gershenson DM, Morris M, Cangir A, Kavanagh JJ, Stringer CA, Edwards CL, et al. Treatment of malignant germ cell tumors of the ovary with bleomycin, etoposide, and cisplatin. J Clin Oncol 1990;8:715-20.  Back to cited text no. 5
    

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Correspondence Address:
Sinai Khandeparkar Siddhi Gaurish
E-517, The Island, Wakad, Pune - 411 057, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.174876

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