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Gonadoblastoma Overgrown by Dysgerminoma in Women with 46,XX Karyotype -A Report of Two Cases

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Gonadoblastoma Overgrown by Dysgerminoma in Women with 46,XX Karyotype -A Report of Two Cases 대한병리학회지: 제 37 권제1 호 2003 The Korean Journal of Pathology. 2003; 37: 66-70 Gonadoblastoma Overgrown by Dysgerminoma in Women with 46,XX Karyotype -A Report of Two Cases- Mi-Jung Kim∙Hee-Jeong Ahn1 Gonadoblastoma is a neoplasm containing an intimate mixture of germ cells and elements Ji-Young Kim1∙ Kyu-Rae Kim resembling immature granulosa or Sertoli cells. It has been considered as in situ germ cell malignancy that can be overgrown by more malignant germ cell neoplasms. The tumor has been reported to almost exclusively develop in various types of gonadal maldevelopment syn- Department of Pathology, University of dromes containing the Y chromosome, such as in pure or mixed gonadal dysgenesis and, less Ulsan College of Medicine, Asan commonly, in male hermaphroditism. However, occurrences in phenotypically and chromoso- Medical Center, Seoul, Korea; mally normal, menstruating women are exceptionally rare. We report two cases of gonadoblas- 1Department of Pathology, Bundang Hospital, College of Medicine Pochon toma overgrown by dysgerminoma occurring in the ovaries of phenotypically and cytogeneti- Cha University, Seongnam, Korea cally normal menstruating women. One of the two cases showed an area composed of granu- losa cell tumor-like elements. This type of combination has been very rarely described, and exemplified that gonadoblastoma may progress to sex cord-stromal tumors as well as to the malignant germ cell tumors. Received : June 28, 2002 Accepted : November 6, 2002 Corresponding Author Kyu-Rae Kim, M.D. Department of Pathology, University of Ulsan College of Medicine, Asan Medical Center, 388-1 Pungnap-dong, Songpa-gu, Seoul 138-736, Korea Tel: 02-3010-4514 Fax: 02-472-7898 Key Words : Gonadoblastoma-Dysgerminoma-Sex Cord-Stromal Tumor-Gonadal Dysgen- E-mail: [email protected] esis, 46,XX In 1953, Scully described gonadoblastoma as a separate enti- gonadoblastomas in the phenotypically and cytogenetically nor- ty. It was defined histologically by the occurrence of discrete mal menstruating women and a rare case in which the gonadoblas- aggregates composed of germ cells and sex cord elements resem- toma, the malignant germ cell neoplasia and the sex cord-stromal bling immature Sertoli or granulosa cells.1,2 The incidence of tumor coexist in a same tumor. gonadoblastoma is high in patients with various gonadal malde- velopment syndromes containing the Y chromosome.3,4 It is found in 25-30% of patients with XY gonadal dysgenesis, in CASE REPORT 15-20% of 45,X/46,XY individuals,5,6 and rarely in 46,XY male hermaphroditism patients.7,8 A small fraction of gonadoblas- Case 1 tomas, however, develops in 46,XX females with no evidence of Y chromosomal DNA,9,10 in patients with histories of normal A 33-year-old unmarried woman was admitted to the hospi- pregnancies or menstruation,2,11 and in phenotypically normal men tal for evaluation of a low abdominal mass. The patient had with undescended testes.12,13 We report two cases of gonadoblas- received an evaluation for secondary amenorrhea one year before tomas associated with dysgerminoma that occurred in phenotypi- and was found to have bilateral cystic ovarian masses with mul- cally and cytogenetically normal menstruating women. The tiple leiomyomas of the uterus. Her family history and past significance of this report is that it describes the rare cases of medical history were unremarkable. On physical examination, 66 Gonadoblastoma in 46,XX 67 A B Fig. 1. (A) The cut surface of the ovary shows an ill-defined 1.8× 1.1×1 cm sized solid nodule in the cystic wall. The oligolocular cyst shows a hemorrhagic inner surface. (solid arrows: gonadoblas- tomatous area). (B) The gonadoblastomatous area (lower half) is overgrown by a typical dysgerminomatous area (upper half). The gonadoblastomatous area is composed of germ cells and small- er epithelial cells resembling immature Sertoli or granulosa cells forming Call-Exner like hyaline bodies. (C) Sex cord-stromal ele- ments in the connective tissue stroma have a trabecular or cord- like arrangement. The cells have angulated nuclei with inconspic- C uous nuclear grooves. the patient’s height was 160 cm and her weight was 90 kg. were multiple small subserosal leiomyomas in the uterus. The Breast development, pubic hair, and external genitalia were right ovary, the left ovarian cyst and the uterine myomas were normal. A pelvic examination revealed an enlarged uterus with removed. The right ovary was composed of an oligolocular cyst. bilateral pelvic masses. She had had normal menstruation until The inner surface of the right ovarian cyst was hemorrhagic and one year prior to the examination revealing the pelvic masses. shaggy, and the thickness of the wall ranged from 0.2 to 0.7 Her menstruations were moderate in amount, and they occurred cm. There was an ill-defined firm, solid grayish yellow nodule, at regular intervals. She had not been pregnant and denied hav- measuring 1.8×1.1×1 cm within the ovarian parenchyme ing had any gynecological problems. An ultrasonography revealed (Fig. 1A). The left ovary was cystic and had been ruptured pre- septated multilocular cystic ovarian masses on both adnexa. viously. The inner surface of the left ovarian cyst showed red to Multiple small uterine leiomyomas were also identified. brown patches without solid area. An exploratory laparotomy revealed a 9.5×5×2 cm cystic Microscopically, bilateral ovaries showed the typical features right ovarian mass and a 5×4×4 cm left ovarian mass. There of endometriotic cysts composed of an endometrial-type epithe- 68 Mi-Jung Kim∙Hee-Jeong Ahn∙Ji-Young Kim, et al. A B Fig. 2. (A) The cut surface of the ovary, measuring 8.5×7×5 cm, is mostly replaced by a solid pink gray colored and focally hem- orrhagic tumor. Normal ovarian tissue (arrows) remains as a thin rim beneath the surface. (B) The gonadoblastomatous area is com- posed of cellular nests showing a mixture of germ cells and sex cord-like elements. (C) The dysgerminomatous area is composed of sheets or nests of germ cells and mature lymphocytes in fibrous C septa. lium, stroma, and hemosiderin-laden macrophages. The solid cytoplasm. A minor focus of sex cord-stromal components was nodule in the right ovary was composed of three different histo- seen at the periphery of the nodule, and the cells formed a tra- logical features. There were gonadoblastomatous and dysger- becular or cord-like arrangement (Fig. 1C). The nuclei of these minomatous areas (1.4 cm and 1.3 cm in greatest dimension, cells were oval or angulated; however, the grooves were incon- respectively) and a minor focus of a sex cord-stromal compo- spicuous, and significant pleomorphism and mitosis were absent. nent (Fig. 1B). In the gonadoblastomatous area, discrete cellu- These cells surrounded circular spaces filled with eosinophilic lar nests were composed of intimately admixed germ cells and hyaline materials and formed Call-Exner bodies. smaller epithelial cells resembling immature Sertoli or granu- Immunohistochemical staining for placental alkaline phos- losa cells. The latter formed Call-Exner-like hyaline bodies. The phatase (PLAP: 1:200, DAKO, Glostrup, Denmark) showed a germ cell components within the nests were similar to dysger- strong positive reaction in the dysgerminomatous component minoma which have large, round and vesicular nuclei, often with and in the germ cell components of the gonadoblastomatous prominent nucleoli as well as abundant and clear, or eosinophilic, area. The sex cord-stromal elements resembling Sertoli or gran- Gonadoblastoma in 46,XX 69 ulosa cells in the gonadoblastoma were immunonegative for attend her regular follow-up visits. PLAP, but positive for inhibin (1:50, Serotec, Oxford, U.K.). The Chromosomal analyses were performed with peripheral lym- Ki-67 (1:200, DAKO) proliferation index was high in the germ phocytes using conventional G-banding techniques. Both cases cell components, but not in the sex-cord stromal elements. revealed normal 46, XX karyotypes (Fig. 3). The patient was treated with combination chemotherapy. Eighteen months later, she is well without evidence of recur- rence or metastasis. DISCUSSION Case 2 Neoplasms composed of germ cells and sex cord derivatives consist of two distinctive types: the gonadoblastoma and the A 30-year-old woman visited the emergency room because of unclassified type of mixed germ cell sex cord-stromal tumor. abdominal pain. She had experienced normal menstruation with These two types have different histopathologic, genetic and regular intervals, normal durations, and amount of blood. Her endocrine features and different clinical and biological behaviors. family history and past medical history were unremarkable. She Gonadoblastoma arises almost exclusively in the dysgenetic gonads had borne a healthy male baby two years before admission, and of patients with a Y chromosome. The most common karyotypes she had received dilatation and evacuation for artificial abortion of these patients are 46,XY and 45,X/46,XYmosaicism. It has several times after the last delivery. She was found to have a right been considered as in situ germ cell malginancy that commonly ovarian mass. A right salpingo-oophorectomy was performed. progresses to invasive germinoma or another type of malignant The ovarian mass measured 8.5×7×5 cm and the pedicle was germ cell tumor, or rarely regresses with hyalinization and cal- distorted, resulting in a partly hemorrhagic parenchyme. The cification. On the other hand, the mixed germ cell sex cord-stro- cut surface was mostly occupied by a well demarcated, pinkish mal tumor usually occurs in phenotypically and cytogenetically gray colored solid mass, and there was a thin layer of normal normal females without any evidence of developmental abnor- appearing ovarian tissue beneath the surface (Fig. 2A). The fal- malities affecting the gonads, the external genitalia, or body flu- lopian tube was essentially normal.
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