OVARIAN MIXED GERM CELL TUMOR IN A PATIENT WITH 45,X/46,X,+mar MOSAIC TURNER'S SYNDROME

Hae-Kyung Kim; Mi-Hyun Song; Byul-Nim Ahn; Jong-Yoon Choi; Hoon-Kyu Oh; Tae-Sung Lee

doi:10.5468/KJOG.2011.54.8.468

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Kim, Song, Ahn, Choi, Oh, and Lee: OVARIAN MIXED GERM CELL TUMOR IN A PATIENT WITH 45,X/46,X,+mar MOSAIC TURNER'S SYNDROME

Case Report

Korean J Obstet Gynecol 2011;54(8):468-473.

Published online: 21 January 2011

DOI: https://doi.org/10.5468/KJOG.2011.54.8.468

OVARIAN MIXED GERM CELL TUMOR IN A PATIENT WITH 45,X/46,X,+mar MOSAIC TURNER'S SYNDROME

Hae-Kyung Kim, MD¹, Mi-Hyun Song, MD¹, Byul-Nim Ahn, MD¹, Jong-Yoon Choi, MD¹, Hoon-Kyu Oh, MD², Tae-Sung Lee, MD¹

¹Department of Obstetrics and Gynecology, Catholic University of Daegu School of Medicine, Daegu, Korea

²Department of Pathology, Catholic University of Daegu School of Medicine, Daegu, Korea

Corresponding author: Tae-Sung Lee, MD Department of Obstetrics and Gynecology, Catholic University of Daegu School of Medicine 3056-6, Daemyeong 4-dong, Nam-gu, Daegu 705-718, Korea Tel: +82-53-650-4075 Fax: +82-53-650-4078 E-mail: leets@cu.ac.kr

Received 23 March 2011 Revised 11 May 2011 Accepted 26 June 2011

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

We report a case that mixed germ cell tumor developed in an 18-year-old girl with 45,X/46,X,+mar mosaic Turner's syndrome. Molecular biological studies showed that the patient's DNA contained a fragment of Y chromosome. The patient underwent an operation and chemotherapy, and until now there is no evidence of recurrence. A presence of Y chromosome component should be evaluated in case a marker chromosome is found in a woman with Turner's syndrome.

Keywords: Turner syndrome, Marker chromosome, Germ cell tumor

REFERENCES

1. Kim SC, Park SS, Kim KH, Park DY, Yoon MS. A case of coincidental gonadoblastoma and dysgerminoma in 45X/47XYY mosaic Turner variant. Korean J Obstet Gynecol. 2005; 48:1805–10.

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Fig. 1.

Patient's axillary and pubic hair was absent, but her breast development was normal. (A) Tanner stage III.

Fig. 2.

Chromosomal analysis showed mosaic Turner's syndrome [46,X,+mar] (A) and SRY gene of marker chromosome was identified (B).

Fig. 3.

The representatives of MRI imaging showed large multiseptated cystic mass with solid component and thick septal wall, small and immature uterus, and no visible normal ovaries (A) horizontal view, (B) sagittal view.

Fig. 4.

The resected right ovary shows solid and partially cystic tumor with areas of necrosis and hemorrhage (gross view).

Fig. 5.

Representative microphotographs of right (A to E) and left (F) ovary. (A) Varying sized and shaped immature cartilage island embedded in immature mesenchymal stroma seen in immature teratoma portion (H&E, ×40). (B) Scattered primitive germ cells and lymphoplasma cells in dysgerminoma portion (H&E, ×100). (C) Abortive glands and papillae of primitive germ cells seen in embryonal carcinoma portion (H&E, ×40). (D) Reticular and microcystic structures in loose connective tissue of the yolk sac tumor portion (H&E, ×100), (E) Scattered multinucleated syncytial giant cells seen in the choriocarcinoma portion (H&E, ×100), (F) Multiple mineralized cartilage islands and nests of primitive germ cells in left ovary (H&E, ×40). (G) Positive immunohistochemical stain of alpha-fetoprotein in yolk-sac tumor component (×100). (H) Positive immunohistochemical stain of beta human chorionic gonadotropin in syncytial giant cells in choriocarcinoma component (×100). (I) Positive immunohistochemical stain of CD30 in primitive epithelial cells of embryonal carcinoma component (×100).

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