Primary cerebellar endodermal sinus tumor: A case report

Abstract

Endodermal sinus tumors are rare malignant germ cell tumors that usually originate from the gonads and are rarely observed extragonadally. Pure primary endodermal sinus tumors of the cerebellar hemisphere are extremely rare and patients diagnosed with the disease often have a poor prognosis. The symptoms of YSTs are unspecific and associated with the location of tumors. Intracranial YSTs (such as cerebellar hemispheres) always present with symptoms including headache and poor vision. The present study reports the case of a three-year-old male who presented to The First Affiliated Hospital of Nanchang University (Nanchang, China) with a headache that had persisted for one month, and then worsened for the last 10 days. This was accompanied by vomiting and gait disturbance. An abnormal signal mass was identified in the left cerebellar hemisphere on brain magnetic resonance imaging. The case initially presented as a medulloblastoma and the patient was followed up for six months. The final pathology report revealed an endodermal sinus tumor, also known as a yolk sac tumor. Six months following resection of the left cerebellar tumor, the patient succumbed to recurrence of the disease, due to acute vomiting and severe headache.

Primary Yolk Sac Tumor in the Cerebellar Hemisphere: A Case Report and Literature Review of the Rare Tumor

“…YST is typically a well-defined round or oval tumor with isointense T1-weighted images and slightly hyperintense T2-weighted images (2, 3). After gadolinium injection, the tumor has strong homogenous enhancement (2,3). The imaging findings of our patient were consistent with those in previous studies.…”

“…It has been confirmed that the significant increase of AFP level in serum and cerebrospinal fluid is related to YST (3,4). Initially, our case was not considered to be primary YST because it was consistent with medulloblastoma, given the age distribution and due to the rarity of YST in the cerebellar hemisphere.…”

“…Many studies have reported that YST occurs in the vagina, seminal vesicle, pancreas, omentum and stomach, as well as in the intracranial and sacrococcygeal regions (2,3,(16)(17)(18)(19)(20)(21)(22)(23). GCTs originating in the intracranial region almost always occur in the suprasellar regions or pineal gland, but tumors in the cerebellar hemisphere are extremely rare (2).…”

“…Our final diagnosis of YST was confirmed by microscopic pathology and immunohistochemical staining of the tumor. Furthermore, AFP is a useful marker not only for immunohistochemical staining and pathological diagnosis but also for evaluating treatment response and detecting recurrence (2,3).…”

Multidisciplinary treatment of primary intracranial yolk sac tumor

“…The anterior third ventricle Yes No Yes Alive 2014 [14] M 3 Left cerebellar hemisphere Yes No No 6 2013 [15] F 17 Bilateral basal ganglia Yes Yes Yes Alive 2013 [16] M 4 Posterior fossa Yes Yes Yes 60 2013 [17] M 2.5 Posterior fossa Yes Yes Yes Alive 2012 [18] F 15 Pineal region, the right lateral ventricle Yes Yes Yes NR 2011 [19] M 13 Right basal ganglia No Yes Yes 21 2011 [20] M 2 Left temporoparietal lobe Yes No No 36 2011 [5] M recurrence, and it is recommended that patients receive intensive chemotherapy. [33] In our case, the serum AFP level dropped constantly to the normal range during the 8 cycles of EP chemotherapy (Fig.…”