Case Report Primary Juxtaovarian Yolk Sac Tumor Concurrent with an Ipsilateral Ovarian Mature Teratoma in an Adult Woman: a Rare Association

Int J Clin Exp Pathol 2015;8(1):1046-1049 www.ijcep.com /ISSN:1936-2625/IJCEP0003647

Case Report Primary juxtaovarian yolk sac tumor concurrent with an ipsilateral ovarian mature teratoma in an adult woman: a rare association

Haifen Ma, Jihong Yu, Jingjing Tang, Min Wang

Department of Pathology, Beilun People’s Hospital, Ningbo 315800, Zhejiang Province, China Received November 6, 2014; Accepted December 23, 2014; Epub January 1, 2015; Published January 15, 2015

Abstract: Objectives: Yolk sac tumor (YST) is a rare neoplasm that primarily occurs in the ovary in children and young women. Previously, it has been reported that the teratomatous components can be recognized in part of YSTs or appear in the contralateral ovary. Here, we report a rare case of an adult woman with a juxtaovarian YST concurrent with an ipsilateral ovarian mature teratoma. Methods: A 47-year-old woman found a pelvic mass for nine days and subsequently underwent debulking operation. The specimens were evaluated for detailed characterizations through gross examination, microscopy and immunohistochemistry. A literature review was performed and the pathogenesis was briefly discussed about the generation of an extraovarian YST concurrent with a teratoma. Results: The right juxtaovarian tumor showed typical histological patterns of YST. Immunostaining demonstrated the YST nature of Gly-3 and AFP positive tumor cells. The ipsilateral ovarian tumor was a common mature cystic teratoma with chronic fibrotic changes. According to the differences of the origin and the differentiation of the two germ cell tumors, we suspect that the occurrence of the teratoma is earlier than the YST. Conclusions: To our knowledge, this is the first report of an adult woman with a juxtaovarian YST concurrent with an ipsilateral ovarian mature teratoma.

Keywords: Yolk sac tumor, extraovarian, ovarian mature teratoma, ipsilateral, concurrent, adult

Introduction ian malignancy in the right adenexa (Figure 1A, 1B). Yolk sac tumor (YST) is a rare malignancy that primarily occurs in the ovary in children and Explorative laparotomy was performed. The young women. The teratomatous components mass was found near the right ovary and was can be recognized in some YSTs. Benign cystic located between the mesovarium and the mid- teratoma can appear in the contralateral ovary dle of ipsilateral fallopian tube. The right ovary in about 10% of the cases. However, it is appeared slightly enlarge. The frozen section of extremely rare to encounter a juxtaovarian YST the mass was suggestive of YST. Thus, a total concurrent with an ipsilateral ovarian mature abdominal hysterectomy, bilateral salpingo- oophorectomy, omentectomy and pelvic lymph teratoma in an adult woman. We present one nodes dissection were performed. such rare and interesting association of these two diseases. Pathological findings

Case report The uterus, bilateral adnexas, greater omentum and pelvic lymphadenectomy specimens were A 47-year-old woman presented with menstrual sent separately. disorders for six months and a pelvic mass for nine days. The laboratory tests showed an On gross examination, an encapsulated mass extremely high level of alpha-fetoprotein (AFP) measuring about 7 cm × 6 cm × 5 cm was dis- at 1874.1 ng/ml (reference: < 13.4 ng/ml). covered in the right juxtaovarian area focally Ultrasonography and Magnetic resonance attaching to the ipsilateral fallopian tube. The imaging (MRI) findings were suggestive of ovar- sectioned surface was solid, soft, gray and Juxtaovarian YST Concurrent with Ovarian MT

Figure 1. MRI revealed a giant tumor in the right adenexa (A, B).

Figure 2. On gross examination, a mass measuring about 7 cm × 6 cm × 5 cm was discovered in the right juxtaovarian area focally attaching to the ipsilateral fallopian tube. On the right, is the tumor that was capsulated and showed solid, soft, gray and partly presented necrosis and hemorrhage. On the middle left, is the fallopian tube. On the lower left, is the right ovary (A). The malignancy was found objectively besides the ovary by observing pathologic large slice. On the top right, is the extraovarian yolk sac tumor. On the upper left, is the ovarian tissue (teratoma not shown here) (B). partly presented necrosis and hemorrhage. The observed in some areas. The cystic tumor in right ovary measured about 2.5 cm × 2 cm × the right ovary was composed of dermoid cyst 0.8 cm, in which a cystic tumor measuring 1.0 components of a few hairs and small foci of cm in maximum dimension was present (Figure well-differentiated hyaline cartilages (Figure 2A, 2B). The left adnexa and uterus were 3C). In addition, multinucleated giant cells, cal- unremarkable. cification and significant fibrosis were seen in the capsule wall, which suggested that the Microscopically, the right juxtaovarian tumor tumor growth had gone through a long time. showed numerous histological patterns: a typi- The malignant tumor was confined to the right cal papillary, reticular (microcystic), glandular juxtaovarian area. No metastases were found. pattern and partly micropapillary structures with perivascular Schiller-Duval bodies (Figure Immunohistochemistry of the juxtaovarian tu- 3A, 3B). In addition, hyaline globules were mor revealed diffuse expression of AE1/AE3,

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Figure 3. Microscopically, the right juxtaovarian tumor showed reticular (microcystic) pattern and characteristics of Schiller-Duval bodies (A, B). The cystic tumor in the right ovary was composed of dermoid cyst components of a few hairs and small foci of well-differentiated hyaline cartilages and calcification (C). Immunohistochemistry for the juxtaovarian tumor revealed diffuse expression of Gly-3 (D) and scattered expression of AFP (E).

Gly-3, CD117, CD99, β-Catenin, P53 and scat- the urogenital crest during embryogenesis, tered expression of AFP (Figure 3D, 3E), while where they will become the future gonads. CD30, HCG-β, CK7, CK20, WT-1, ER, PR, PAX-8, Ovarian teratomas are the most common germ Inhibin-α, Napsin A were negative. cell tumors generating in the yolk sac and most of them are mature and behave as benign Histopathology confirmed a YST of the right jux- tumors. The teratomatous components can taovarian area concurrent with ipsilateral ovar- also be recognized in part of YSTs. In 10% to ian mature cystic teratoma. 15% cases, germ cells with abnormal migration and trapped along the midline without degrada- Follow-up tion can result in extraovarian YST develop- ment, from the brain to the sacrococcygeal On the thirteenth postoperative day, her AFP region [1-3]. It seems to be the most likely level decreased to 137.3 ng/mL, and on the explanation for the extraovarian YST in our twentieth postoperative day, it was 51.6 ng/ case. mL. More interestingly, benign cystic teratoma can Discussion appear in the contralateral ovary in about 10% of ovarian YSTs in the literature, while the tera- The yolk sac is an embryonal structure and also toma is in the ipsilateral ovary in our case. contains germ cells that migrate bilaterally to According to the differences of the origin and

1048 Int J Clin Exp Pathol 2015;8(1):1046-1049 Juxtaovarian YST Concurrent with Ovarian MT the differentiation of the two germ cell tumors, Disclosure of conflict of interest we suspected that the occurrence of the teratoma is earlier than the YST. Histopathology None. confirmed that the teratoma existed earlier in our case. Address correspondence to: Dr. Haifen Ma, Depart- ment of Pathology, Beilun People’s Hospital, Ningbo There were few reports about the recurrence of 315800, Zhejiang Province, China. Tel: +86-139- mature teratomas as YSTs. In a study by Yoshida 58311234; Fax: +86-574-86776335; E-mail: mh- et al, 13 cases of YSTs were found after sacro- [email protected] coccygeal teratoma (SCT) resection, which accounted for 5.4% of 241 SCTs patients References enrolled [4]. Utsuki et al described a case of the [1] Lim SH, Kim YH, Yim GW, Nam EJ, Kim S. Pri- malignant transformation of an intracranial mary omental yolk sac tumour. Obstet Gynecol mature teratoma into a YST in a 16-year-old boy Sci 2013; 56: 412-415. [5]. D’Antonio et al reported a YST arising in the [2] Subrahmanya NB, Kapadi SN, Junaid TA. Pri- fallopian tube of an elderly woman. They specu- mary yolk sac (endodermal sinus) tumour of lated this YST could originate from a pre-exist- the vulva: a case report. Med Princ Pract 2011; ing (rare) mature teratoma [6]. 20: 90-92. [3] Wang CH, Hsu TR, Yang TY, Wong TT, Chang FC, It is worth noting that YST usually occurs in chil- Ho DM, Chiang KP. Primary yolk sac tumour of dren and young women at the median age of bilateral basal ganglia. Chin Med Assoc 2010; 17.6 years, but the patient in our case is 73: 444-448. 47-year-old. As far as we are aware, this is the [4] Yoshida M, Matsuoka K, Nakazawa A, Yoshida first case of a juxtaovarian YST concurrent with M, Inoue T, Kishimoto H, Nakayama M, Takaba an ipsilateral ovarian mature teratoma in an E, Hamazaki M, Yokoyama S, Horie H, Tanaka adult woman. M, Gomi K, Ohama Y, Kiqasawa H, Kitano Y, Uchida H, Kanamori Y, Iwanaka T, Tanaka Y. YST has the ability to synthesize AFP. The sig- Sacrococcygeal yolk sac tumour developing after teratoma: a clinicopathological study of pe- nificantly increased serum AFP level is of great diatric sacrococcygeal germ cell tumours and significance in diagnosing YST and monitoring a proposal of the pathogenesis of sacrococcy- tumor recurrence in YST patients. Yoshida et al geal yolk sac tumours. J Pediatr Surg 2013; found that intervals of AFP measurement ≤ 4 48: 776-781. months helped to detect subclinical localized [5] Utsuki S, Oka H, Sagiuchi T, Shimizu S, Suzuki YSTs for resection. They recommend that S, Fujii K. Malignant transformation of intracra- patients undergo serum AFP monitoring every 3 nial mature teratoma to yolk sac tumour after months for ≥ 3 years after SCT resection [4]. late relapse. Case report. J Neurosurg 2007; 106: 1067-1069. Surgical operation followed by cisplatin-based [6] D’Antonio A, Sparano L, Addesso M, Anqrisani chemotherapy is always recommended for any B, Cassese S, Anqrisani P. Extraovarian yolk YSTs [7]. sac tumour in an elderly woman with an endo- metrioid-like pattern concurrent with a hepa- Acknowledgements toid component. J Obstet Gynaecol 2010; 30: 530-532. This work was partially supported by grants [7] Rittiluechai K, Wilcox R, Lisle J, Everett E, Wal- from the Medical Scientific Research lace HJ 3rd, Verschraegen CF. Prognosis of Foundation of Zhejiang Province (2011KYB103) hepatoid yolk sac tumour in women: what’s up, Doc? Eur J Obstet Gynecol Reprod Biol 2014; and the Clinical Scientific Research Fund 175: 25-29. Project of Zhejiang Medical Association (2013 zyc-A77).

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