24th EUPSA CONGRESS, İzmir, Türkiye, 7 - 10 Haziran 2023, ss.48
Aim: Mixed germ cell tumors (MGCT) of ovary are rare
aggressive cancers affecting young adolescent girls. Most MGCT consist of
dysgerminoma accompanied by endodermal sinus tumors, immature teratoma or
choriocarcinoma. Besides radiologic imaging, tumor markers such as alpha
feto-protein (AFP), lactate dehydrogenate (LDH) and CA-125, contribute to the
diagnosis, prognosis, and follow-up of the disease. Modality of treatment is
usually fertility sparing surgery followed by chemotherapy. Herein, we report a
unique case of giant ovarian MGCT and its pathological findings.
Case description: A 7-year-old girl with no prior history
admitted to the emergency room with an abdominal mass. On physical examination
abdominal distention without pain was noted. Computed tomography (CT) showed a
23 cm long mass containing heterogeneous cystic lesions, including areas of
contrast and thick septations. Additional magnetic resonance imaging could not
determine its origin. Only CA-125 was found to be high (170 U/mL), AFP, LDH and
other tumor markers were negative. An explorative laparotomy was performed. A
multicystic, grape-like mass surrounded by omentum covering the entire abdomen
was found (Figure 1). Left ovary originated tumor was carefully dissected
without any rupture in addition to salphingo-oopherectomy. Pathology was
reported as MGCT with yolk sac dominancy. However, both tissue and serum AFP
levels were negative. DICER-1 mutation was found. Patient received chemotherapy
postoperatively.
Conclusion: Tumor markers can be helpful for diagnosis of MGCT. However,
this patient is the first case which shows negative AFP on tissue while being
yolk sac dominancy with an unusual appearance.