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CASE REPORT |
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Year : 2013 | Volume
: 6
| Issue : 3 | Page : 298-301 |
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Mixed germ cell tumors: Report of two cases
Pradhan M Pagaro, Charusheela R Gore, Tushar Patil, Shirish Chandanwale
Department of Pathology, Padmashree Dr. D.Y. Patil Medical College, Hospital and Research Centre, Dr. D.Y. Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India
Date of Web Publication | 5-Jul-2013 |
Correspondence Address: Pradhan M Pagaro Department of Pathology, Padmashree Dr. D.Y. Patil Medical College, Pune, Maharashtra India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0975-2870.114658
Germ cell tumors arise in the ovaries and testis and rarely in other tissues. Mixed germ cell tumors are rare. We report two cases of mixed germ cell tumors, one consisting of seminoma and immature teratoma in the testis of a 30-year-old male and second consisting of a yolk sac tumor and immature teratoma in the ovary of a 17-year-old female. Many combinations of mixed germ cell tumors have been reported but very few cases of the above-mentioned combinations have been reported in literature. Keywords: Immature teratoma, mixed germ cell tumors, pluripotent embryonic stem cells, yolk sac tumor
How to cite this article: Pagaro PM, Gore CR, Patil T, Chandanwale S. Mixed germ cell tumors: Report of two cases. Med J DY Patil Univ 2013;6:298-301 |
Introduction | |  |
Twenty percent of ovarian tumors are germ cell tumors, whereas 90-95% of testicular tumors are of germ cell origin. [1] Mixed germ cell tumors consisting of various combinations are generally rare. [2] Mixed germ cell tumors of ovary generally consist combinations of dysgerminoma with embryonal carcinoma, yolk sac tumor or mature and immature teratoma, but combination of yolk sac tumor with immature teratoma have been very rarely reported. Similarly, testicular mixed germ cell tumor combinations usually consist of yolk sac tumor with embryonal carcinoma, choriocarcinoma and mature teratoma, but combinations of seminoma with immature teratoma are rare. We report two cases of mixed germ cell tumors, one consisting of immature teratoma with seminoma in a 30-year-old male and the other an immature teratoma with yolk sac tumor in a 17-year-old female.
Case Report | |  |
The first case was a 30-year male presenting with mass in the right testis. Sonography confirmed a solid and cystic mass. Hematological investigations, human chorionic gonadotrophin (HCG) and alpha-fetoprotein levels were normal. The mass was removed. Grossly tumor was 10 × 12 × 8 cm in dimensions. External surface was smooth and nodular. Cut surface showed grey-white areas, nodular and small cystic areas 2 × 2 × 7.5 mm. At places yellow areas were noted and the cut surface had variegated appearance. Microscopically the tumor consisted areas of immature teratoma showing immature cartilage, immature fat, gland-like spaces and many areas of immature neuroepithelium and hypercellular spindle cell stroma. Other areas showed second type of tumor consisting of nests of round cells with large round nucleus, prominent nucleoli, moderate amount of clear cytoplasm and distinct cell borders. These nests of cells were separated by thin and at places thick bundle of fibrous tissue infiltrated by lymphocytes, with a sprinkling of the same within tumor nests. This was a rare type of mixed germ cell tumor, immature teratoma with seminoma, with only a few cases reported in literature. The histopathological features are shown in [Figure 1], [Figure 2], [Figure 3], [Figure 4] and [Figure 5]. | Figure 1: Seminoma (upper right), and teratomatous elements (lower left) in a case of mixed GCT of testis
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 | Figure 2: The microphotograph shows nests of round cells with clear cytoplasm consistent with seminoma in mixed GCT of testis
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 | Figure 3: Seminoma in a mixed GCT of testis showing infiltration of lymphocytes in the fibrous bands and within the nests of tumour cells
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 | Figure 4: The microphotograph shows immature cartilage, immature fat and immature neuroepithelium in a slide of mixed germ cell tumor of testis
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 | Figure 5: Immature cartilage, immature fat and immature neuroepithelium in a slide of mixed germ cell tumor of testis
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The other case was that of a 17-year-old with complain of pain in abdomen. Sonography revealed an ovarian mass. The alpha fetoprotein levels were 477 ng/ml (normal range: <8.1 ng/ml). The ovarian mass was removed. Grossly it was 18 × 12 × 16 cm in dimensions. External surface was smooth and multilobated. Cut surface shows predominantly solid and small cystic areas. There were foci of gritty cartilaginous as well as areas of hemorrhage and necrosis. Microscopically the mass showed areas of immature cartilage. There were many areas of immature neuroepithelium with hypercellular stroma. Other areas showed many Shiller-Duvall bodies consisting of a central core of blood vessel surrounded by columnar to cuboidal tumor cells and a second layer of cuboidal tumor cells separated by a cystic space resembling glomeruloid structures suggestive of yolk sac tumor. Papillary pattern resembling vitelline structures and areas of reticular pattern of yolk sac tumor consisting of sieve-like spaces lined by flattened endothelium like layer of cells were also suggestive of yolk sac tumor. This was a rare type of mixed germ cell tumor consisting of yolk sac tumor with immature teratoma. The histopathological features are illustrated in [Figure 6], [Figure 7], [Figure 8], [Figure 9] and [Figure 10]. | Figure 6: Shiller-Duvall bodies in the form of glomeruloid and vitelline structures and presence of reticular pattern of yolk sac tumor in mixed GCT
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 | Figure 7: Vitelline structures and reticular pattern of yolk sac tumour in a case of mixed GCT
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 | Figure 8: Vitelline structures and reticular pattern of yolk sac tumour in a case of mixed GCT
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 | Figure 9: Immature cartilage and immature neuroepithelium in a mixed germ cell tumour of ovary
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 | Figure 10: Gland like spaces, immature cartilage, and immature neuroepithelium consistent with immature teratoma in mixed GCT of ovary
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Discussion | |  |
Germ cell tumors are not uncommon. But some mixed germ cell tumors are uncommon with very few such cases reported in literature. Mixed germ cell tumor reported in ovary are dysgerminoma with choriocarcinoma, embryonal carcinoma, yolk sac tumor and mature teratoma, but very few cases consisting of yolk sac tumor and immature teratoma have been reported in literature. [3],[4] Douglas reported an 18-year-old primi with ovarian tumor consisting of immature teratoma and yolk sac tumor [3] Blake reported two cases of mixed germ cell tumor in two siblings with normal karyotype which was unusual. [5] Talerman reported 13 patients of ovarian germ cell tumors, in which 8 were mixed germ cell tumors consisting of yolk sac tumor with dysgerminoma, embryonal carcinoma and choriocarcinoma. [3] Zuntova reviewed mixed germ cell tumors between 1979 and 2002 and found them to be rare. All were yolk sac tumors with dysgerminoma, embryonal carcinoma and choriocarcinoma, but not immature teratoma with yolk sac tumor. [6] Lovri described an unusual mixed GCT consisting of yolk sac tumor and teratoma with rhabdomyosarcomatous element. [7] Terrier Lacombe has also described embryonal rhabdomyosarcoma arising in a mature teratoma of testis. [8] Kaw YT has described metastasis of rhabdomyosarcoma in testicular mixed germ cell tumor consisting of seminoma, teratoma, and rhabdomyosarcoma by doing cytology of pleural effusion. This may be due to deviation of tumor cells from pleuripotent germ cells and malignant transformation from teratomatous elements. [9] Gonzalez-vela JL has reported poor prognosis of germ cell tumors with sarcomatous component. [10] Koshy and Vijayanathan found mixed germ cell tumors to be rare with a common combination of dysgerminoma with yolk sac tumor. [11]
The pathogenesis of mixed germ cell tumors includes chromosomal aberrations in the form of isochromosome formation and deletion of chromosome 12. The pluripotency of homeobox genes NANOG is expressed in human germ cell tumors. [12] OCT3/4 and SOX2 genes are transcription factors expressed in embryonic stem cells. Through a co-operative interaction of these two there occurs pleuripotent expression of genes like NANOG that drives the cells into a feedback autoregulatory loop. Increased levels of these transcription factors SOX2 and OCT3/4 maintains the pleuripotent state of the cells of these mixed germ cell tumors. [13]
The purpose of reporting these two cases is the rarity of these types of mixed germ cell tumors.
References | |  |
1. | Rosai J. Female reproductive system. In: Rosai J, editor. Rosai and ackermans surgical pathology. 9 th ed. Vol. 2. New Delhi, India: Elsevier, a division of Reed Elsevier; 2004. p. 1636-761.  |
2. | Oosterhius JW, Looijenga LH, van Echten J, de Jong B. Chromosomal constitution and developmental potential of human GCTs and teratomas. Cancer Genet Cytogenet 1997;95:96-102.  |
3. | Talerman A, Haije WG, Baggerman L. Serum alphafetoprotein (AFP) in diagnosis and management of endodermal sinus (yolk sac) tumor and mixed germ cell tumor of the ovary. Cancer. 1978;41:272-8.  |
4. | Ulbright TM. Germ cell neoplasms of the testis. Am J Surg Pathol 1993;17:1075-91.  |
5. | Blake KL, Getrard MP. Malignant GCTs in two siblings. Med Pediatr Oncol 1993;21:299-300.  |
6. | Zuntova A, Sameraeur D, Teslík L, Kabícková E, Koutecký J. Mixed GCTs of ovary in childhood and adolescence. Cesk Patol 2004;40:92-101.  |
7. | Lovric E, Hizak DB, Balja MP, Lenicek T, Kruslin B. An unusual mixed germ cell tumor of the testes consisting of rhabdomyosarcoma, mature teratoma and yolk sac tumor. Asian Journal of Andrology 2010;12:451-2.  |
8. | Terrier Lacombe MJ, Martinez-Madrigal F, Porta W, Rahal J, Droz JP. Embryonal rhabdomyosarcoma arising in a mature teratoma of testis. J Urol 1990;143:1232-4.  |
9. | Kaw YT, Crame HM. Cytological diagnosis of rhabdomyosacroma in a patient with germ cell tumor. A case report. Acta Cytol 1995;39;249-51.  |
10. | Gonzalez-vela JL. Poor prognosis of germ cell tumours with sarcomatous elements. Cancer 1990;66:1114-6.  |
11. | Koshy M, Vijayanathan A, Vadiveloo V. Malignant ovarian mixed germ cell tumour: A rare combination. Biomed Imaging Interv J 2005;1:e10.  |
12. | Tai MH, Chang CC, Kiupel M, Webster JD, Olson LK, Trosko JE. OCT4 in adult human cell, evidence in support of stem cell theory on carcinogenesis. Carcinogenesis 2005;26:495-502.  |
13. | Hart AH, Hartley L, Parker K, Ibrahim M, Looijenga LH, Pauchnik M, et al. The pleuripotency of homeobox gene NANOG expressed in human mixed GCTs. Cancer 2005;104:2092-8.  |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]
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