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  Table of Contents    
CASE REPORT  
Year : 2018  |  Volume : 61  |  Issue : 4  |  Page : 590-592
Primary intrauterine dysgerminoma in a pregnant woman: A rare case report


1 Maternal-Fetal Medicine Research Center, Department of Pathology, Shiraz University of Medical Sciences, Shiraz, Iran
2 Department of Pathology, Shiraz University of Medical Sciences, Shiraz, Iran
3 Department of OB and GYN, Shiraz University of Medical Sciences, Shiraz, Iran

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Date of Web Publication10-Oct-2018
 

   Abstract 


Primary extraovarian dysgerminoma is very rare. Nearly all reported uterine germ cell tumors are nondysgerminoma. Herein, we reported a primary intrauterine dysgerminoma. A 21-year-old pregnant woman G2 L1 with a gestational age of 33 weeks referred to an obstetric ward with a chief complaint of labor pain and membrane rupture. Ultrasonography showed a large hypoechoic lobulated area adjacent to the lower part of her uterus. She underwent an operation and a huge mass was detected in her uterus, which was extended to her pelvic floor. Histopathological and immunohistochemical examinations were consistent with dysgerminoma.

Keywords: Dysgerminoma, extraovarian germ cell tumor, uterus

How to cite this article:
Akbarzadeh-Jahromi M, Aslani FS, Najib F, Hosseini S. Primary intrauterine dysgerminoma in a pregnant woman: A rare case report. Indian J Pathol Microbiol 2018;61:590-2

How to cite this URL:
Akbarzadeh-Jahromi M, Aslani FS, Najib F, Hosseini S. Primary intrauterine dysgerminoma in a pregnant woman: A rare case report. Indian J Pathol Microbiol [serial online] 2018 [cited 2018 Dec 15];61:590-2. Available from: http://www.ijpmonline.org/text.asp?2018/61/4/590/242997





   Introduction Top


Germ cell tumors (GCTs) most commonly originate from gonads. However, some develop in extragonadal sites that account for 1%–5% of all GCTs.[1],[2] Primary extragonadal GCTs (EGCTs) are thought to originate from malignant transformation of the residual primordial germ cells. They are misplaced at different sites in body's midline during embryological migration from the yolk sac into an embryo.[1],[2],[3] The most common sites of EGCTs are intracranial, mediastinal, and abdomen/pleura in males and pelvis/abdomen/mediastinum in females.[4] EGCTs have similar morphology to gonadal germ cells, which is commonly classified into two large groups: dysgerminoma and nondysgerminoma. To the best of our knowledge, most studies have reported uterine GCTs, which were nondysgerminoma.[1],[4] Herein, we discussed the primary uterine dysgerminoma in a pregnant woman.


   Case Report Top


A 21-year-old pregnant woman G2 L1 with a gestational age of 33 weeks referred to an obstetric ward with a chief complaint of labor pain and membrane rupture. She had the history of cesarean section in her the first pregnancy. Ultrasonography showed a large hypoechoic lobulated area measuring about 200 mm × 100 mm adjacent to the lower part of her uterus posterior to urinary bladder. Cesarean section was done due to her previous cesarean operation and breech presentation of the fetus. A large mass was present at the lower segment extending to the cervix and upper part of the vagina. The fetus was delivered with Apgar score = 7. Then, the uterus was stitched, and the patient was referred to our hospital (a third-level OB-GYN hospital). At arrival, the patient was febrile with vaginal bleeding. Thus, she underwent an operation and a huge mass measuring 13 cm × 11 cm × 10 cm was detected in her uterus, which was extended to her pelvic floor [Figure 1]. Total hysterectomy and resection of pelvic mass was done. Both ovaries and fallopian tubes showed normal size and shape. Indeed, the cut surface of the tumor had a multilobular appearance with extensive necrosis and hemorrhagic areas. Histological examination also showed nests and trabecules of uniformed polygonal tumor cells with eosinophilic to clear cytoplasm. The nuclei were large and central with vesicular chromatin and prominent nucleoli. The tumor cells were separated by thin fibrous septa infiltrated by lymphocytes [Figure 2]. Immunohistochemical study of the tumor cells showed immunoreactivity for placental alkaline phosphate (PLAP), CD117, and vimentin. Nonetheless, leukocyte common antigen (LCA), CD20, CD3, CD99 (MIC2, PCB1), S100, desmin, and cytokeratin (AE1/AE3) were negative [Figure 3]. Therefore, diagnosis of dysgerminoma was confirmed. She was treated with four cycles of bleomycin, etoposide, and cisplatin chemotherapy. No abnormal finding was detected in postoperative sonography and follow-up magnetic resonance imaging and computed tomography scan after chemotherapy (6, 12, and 18 months). The patient's serum CA 125 and lactic dehydrogenase (LDH) were also within the normal limits. She also showed no evidence of recurrence or metastases 30 months after surgery.
Figure 1: The uterine mass

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Figure 2: Nests and trabecules of uniformed polygonal tumor cells with eosinophilic to clear cytoplasm. The tumor cells were separated by thin fibrous septa infiltrated by lymphocytes. (a) H and E, ×100 and (b) H and E, ×400

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Figure 3: Immunoreactivity for (a) placental alkaline phosphate, (b) vimentin, and (c) CD117

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   Discussion Top


Malignant ovarian GCTs (MOGCTs) account for 5% of all ovarian cancers and mostly occur in the second decade of life.[5] MOGCTs and also EGCT are commonly classified into two large groups: dysgerminoma and nondysgerminoma.[5] Dysgerminoma makes up 0.6% of all ovarian carcinoma[1] and is the second most common malignant GCT after immature teratoma.[4] It predominantly occurs in young women, mostly (85%) <30 years of age at the time of diagnosis.[6],[7] Gain of isochromosome 12p is an important cytogentic abnormality in both gonadal and extragonadal GCTs.[1]

The most common histological subtype of EGCT in males is seminoma followed by teratoma or teratocarcinoma and embryonal carcinoma. In females, more than half of EGCTs are teratoma or teratocarcinoma and 20% are dysgerminoma.[4]

Primary extraovarian dysgerminoma is very rare. A study on 10 women with EGCTs indicated that four participants had dysgerminoma, but the tumor site was not distinguished among the pineal region, mediastinum, sacrum, and pharynx.[2] Moreover, Su Hyun Yoo reported a primary retroperitoneal dysgerminoma presented as an adrenal tumor in a 17-year-old girl.[2]

Up to now, nearly all reported uterine GCTs were nondysgerminoma,[1],[4] and only a few cases of primary uterine dysgerminoma were reported.

Generally, the main differential diagnosis of dysgerminoma is lymphoma/leukemia. Both macroscopic and microscopic features of dysgerminoma can be confused with lymphoma. Other differential diagnoses include solid type of yolk sac tumor, embryonal carcinoma, and sertoli cell tumor, which were all excluded by morphology and immunohistochemistry.[8] Other differential diagnoses in the uterus, especially in pregnant women, are placental site trophoblastic tumor and epithelioid trophoblastic tumor. Placental site trophoblastic tumor shows diffuse reactivity for human placental lactogen (hPL), CD146, and cytokeratin and is focally positive for human chorionic gonadotropin (HCG) and PLAP. On the other hand, epithelioid trophoblastic tumor is positive for p63 and negative for hPL. Both of them are negative for c-kit.[9]

In our case, vimentin, c-kit, and PLAP were positive, and LCA, CK, CD20, CD99, S100, hPL, and p63 were negative. These results confirmed GCT and excluded carcinoma, rhabdomyosarcoma, lymphoma, ewing sarcoma/primitive neuroectodermal tumor, placental-site trophoblastic tumor, and epithelioid trophoblastic tumor. There was no evidence of ovarian lesions in the patient's follow-up.

Some tumor markers (alpha-fetoprotein, HCG, and LDH) are produced by specific histological subtypes of GCTs. These specific molecules are used for preoperative diagnosis, monitoring response to treatment, and follow-up.[10]

Most of MOGCTs have very favorable prognoses and are responsive to chemotherapy. The long-term outcome of patients with dysgerminoma is also excellent. Yet, the 5-year survival rate depends on lymph node status, being significantly worse in patients with positive nodes (98% vs. 90.9%).[6] The majority of recurrences occur within 2 years of diagnosis.[6] Late recurrence is expected over 2 years after diagnosis, which is very rare.[6] In our case, there was no evidence of recurrence within 30 months of follow-up.

Acknowledgment

The authors would like to thank Ms. A. Keivanshekouh at the Research Improvement Center of Shiraz University of Medical Sciences for improving the use of English in the manuscript.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Stang A, Trabert B, Wentzensen N, Cook MB, Rusner C, Oosterhuis JW, et al. Gonadal and extragonadal germ cell tumours in the united states, 1973-2007. Int J Androl 2012;35:616-25.  Back to cited text no. 1
    
2.
Yoo SH, Kim KR, Hong SJ, Cho KJ. Primary retroperitoneal dysgerminoma presenting as an adrenal tumor: A case report and literature review. Pathol Int 2011;61:248-51.  Back to cited text no. 2
    
3.
Rusner C, Trabert B, Katalinic A, Kieschke J, Emrich K, Stang A, et al. Incidence patterns and trends of malignant gonadal and extragonadal germ cell tumors in germany, 1998-2008. Cancer Epidemiol 2013;37:370-3.  Back to cited text no. 3
    
4.
Pauniaho SL, Salonen J, Helminen M, Vettenranta K, Heikinheimo M, Heikinheimo O, et al. The incidences of malignant gonadal and extragonadal germ cell tumors in males and females: A population-based study covering over 40 years in Finland. Cancer Causes Control 2012;23:1921-7.  Back to cited text no. 4
    
5.
Gadducci A, Lanfredini N, Tana R. Menstrual function and childbearing potential after fertility-sparing surgery and platinum-based chemotherapy for malignant ovarian germ cell tumours. Gynecol Endocrinol 2014;30:467-71.  Back to cited text no. 5
    
6.
Vicus D, Beiner ME, Klachook S, Le LW, Laframboise S, Mackay H, et al. Pure dysgerminoma of the ovary 35 years on: A single institutional experience. Gynecol Oncol 2010;117:23-6.  Back to cited text no. 6
    
7.
Lopes da Silva J, Luiz Renna N Jr., Paulino E, de Melo AC. Outcomes of ovarian germ cell tumors: Ten years of experience at the brazilian national cancer institute. Int J Gynecol Cancer 2015;25:786-91.  Back to cited text no. 7
    
8.
Adekunle OO, Zayyan M, Kolawole AO. Case report: A rare case of dysgerminoma presenting with skin and breast metastasis. Case Rep Clin Med 2013;2:170.  Back to cited text no. 8
    
9.
Shih IM, Kurman RJ. The pathology of intermediate trophoblastic tumors and tumor-like lesions. Int J Gynecol Pathol 2001;20:31-47.  Back to cited text no. 9
    
10.
von Eyben FE. Laboratory markers and germ cell tumors. Crit Rev Clin Lab Sci 2003;40:377-427.  Back to cited text no. 10
    

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Correspondence Address:
Mojgan Akbarzadeh-Jahromi
Department of Pathology, School of Medicine, Maternal-Fetal Medicine Research Center, Shiraz University of Medical Sciences, Zand Street, Shiraz
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_598_17

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