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  Table of Contents    
CASE REPORT  
Year : 2014  |  Volume : 57  |  Issue : 2  |  Page : 278-280
Primary yolk sac tumor of the cerebellar vermis: A case report


1 Department of Pathology, Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, India
2 Department of Neurosurgery, Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, India

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Date of Web Publication19-Jun-2014
 

   Abstract 

Extragonadal germ cell tumors are rare and comprise 2-5% of all germ cell tumors. Seminoma/dysgerminoma, teratoma, yolk sac tumor (YST) and mixed germ cell tumors are few of the extragonadal germ cell tumors arising within the brain. Of these, primary pure YSTs arising in the brain are extremely rare and arise in the pineal (62%) and suprasellar (31%) regions. Primary YST occurring in the cerebellum is exceedingly rare, associated with a very poor prognosis and has been reported in less than 10 cases in literature. We herein report a case of primary pure yolk sac tumor in the cerebellar vermis in a 2-year-old child.

Keywords: Cerebellum, vermis, primary, yolk sac tumor

How to cite this article:
Shenoy AS, Desai HM, Tyagi DK, Savant HV, Kavishwar VS, Balasubramaniam M. Primary yolk sac tumor of the cerebellar vermis: A case report. Indian J Pathol Microbiol 2014;57:278-80

How to cite this URL:
Shenoy AS, Desai HM, Tyagi DK, Savant HV, Kavishwar VS, Balasubramaniam M. Primary yolk sac tumor of the cerebellar vermis: A case report. Indian J Pathol Microbiol [serial online] 2014 [cited 2020 May 28];57:278-80. Available from: http://www.ijpmonline.org/text.asp?2014/57/2/278/134711



   Introduction Top


Extragonadal germ cell tumors are rare and comprise 2-5% of all germ cell tumors. [1] The common sites of extragonadal germ cell tumors are usually along the midline of the body with very few reported outside the midline. Few of the extragonadal germ cell tumors arising within the brain include germinoma (seminoma/dysgerminoma), teratoma, yolk sac tumor (YST) and mixed germ cell tumors. [2] Of these, primary YST arising in the brain is extremely rare and commonly arises in the pineal (62%) and suprasellar (31%) regions. [1] Primary YST occurring in the cerebellum is exceedingly rare and is reported in less than 10 cases. It is associated with a very poor prognosis. We herein report a case of primary pure YST in the cerebellar vermis in a 2-year-old child.


   Case report Top


A 2-year-old male child came with complaints of generalized weakness, irritability and vomiting since 1 week. Magnetic resonance imaging scan showed a 1.9 cm × 1.7 cm × 1.5 cm well-circumscribed hyper intense mass arising from the cerebellar vermis in the midline and indenting on the quadrigeminal cistern with a probable diagnosis of medulloblastoma [Figure 1]. There was associated vasogenic edema in the adjacent cerebellar hemisphere that extended along the fourth ventricle. The tumor was excised and the mass was sent for histopathological examination, which showed a tumor composed of cells arranged in a tubulo-papillary and glandular pattern against a myxoid reticular background [Figure 2]. The individual cells were tall cuboidal to columnar with vesicular nuclei and prominent nucleoli. Few multinucleate giant cells were seen. Schiller Duval bodies that are diagnostic of YST were also seen. Few of the tumor cells also showed hyaline eosinophilic bodies that were Per-iodic acid Schiff positive and resistant to diastase. The histopathological features were suggestive of pure YST of the cerebellar vermis.
Figure 1: Magnetic resonance imaging images show a 1.9 cm × 1.7 cm × 1.5 cm well-circumscribed hyper intense mass arising from the cerebellar vermis in the midline with associated vasogenic edema in the adjacent cerebellar hemisphere extending along the fourth ventricle

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Figure 2: Histological sections showing a tumor composed of cells in a tubulo-papillary pattern against a myxoid reticular background. (Hematoxylin and eosin [H&E], 100×). Inset: Schiller-Duval body (H&E,
400×)


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Immunohistochemical (IHC) staining showed diffuse staining for alfa-feto protein (AFP) [Figure 3] and cytokeratin (CK-AE1/AE3) [Figure 4] and negative staining for Glial fibrillary acid protein (GFAP) and Vimentin which confirmed the diagnosis of YST. Blood levels of AFP were 1600 ng/mL done only post-operatively as the diagnosis was not clinched pre-operatively.
Figure 3: Tumor cells showing diffuse positivity for alfa-feto protein immunohistochemistry stains

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Figure 4: Tumor cells showing positivity for CKA E1/AE3 immunohistochemistry stains

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A thorough search was made to identify the primary lesion. However, no lesion was identified in the testis or elsewhere. Hence, it was labeled as primary YST of the cerebellar vermis.

Post-operative recovery was uneventful. In view of the high post-operative levels of AFP, a repeat imaging scan was asked for which showed a 2.7 cm × 2.4 cm × 1.7 cm heterogeneously enhancing hyperdense lesion in the midline at the superior aspect of the cerebellum and vermis suggestive of a residual tumor within a bed of blood [Figure 5].
Figure 5: Post-operative magnetic resonance imaging scan shows a 2.7 cm × 2.4 cm × 1.7 cm heterogeneously enhancing hyperdense lesion in the midline at the superior aspect of the cerebellum and vermis suggestive of a residual tumor within a bed of blood

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The child was referred for chemotherapy and was subsequently lost to follow-up.


   Discussion Top


YST is a rare germ cell tumor that occurs mainly in the gonads. In the testis, it is frequently seen as a part of mixed germ cell tumor and in the ovary it is the third most common germ cell tumor. [3]

The pathogenesis of YST is still unknown with few theories suggesting the origin from transformation of misplaced primordial germ cells. These germ cells migrate through the dorsal mesentery during the 4 th -6 th weeks of embryogenesis. If any remnant of tissue remains along this course of migration, a germ cell tumor can develop ectopically. [4] The primordial germ cells can give rise to either seminoma/dysgerminoma, teratoma, YST or choriocarcinoma depending on whether they differentiate toward embryonal or extra-embryonal cells.

The occurrence of YST in the extragonadal sites is extremely rare. The extragonadal YSTs have a midline distribution and are described in the sacrococcygeal region, mediastinum, central nervous system (CNS) and retroperitoneal sites. [1] Cases of YST have also been reported in the pelvis, female genital tract, prostate, bile ducts, omentum and hernia sac. [1] Within the CNS, primary YST occurs within the pineal (62%) and suprasellar (31%) regions and both pineal and suprasellar regions (7%) with very few cases reported to occur in the cerebellum. [1] On review of the literature, less than 10 cases of primary pure YST in the cerebellum have been reported so far. Although cerebellar vermis is also a midline structure, only two cases of cerebellar vermian YST, excluding our case, have been reported so far. [5],[6]

Most cases of YST occur in children between 7 months and 3 years of age, although cases have been reported in post-menopausal females as well. [7] Our case was a 2-year-old male child with cerebellar vermian lesion.

The common presenting symptoms of primary YST include gait disturbances, headaches, vomiting, limb ataxia and occasional visual disturbances.

There are no definite radiological criteria to diagnose a YST. Most case reports of YST have shown iso/low-intensity masses with good heterogenous enhancement and perifocal edema. [8] However, cyto-histopathological confirmation is required for the final diagnosis.

IHC stains for germ cell tumors vary depending on the type of tumor. All germ cell tumors show placental alkaline phosphatase (PLAP) positivity which makes it a non-specific marker. Specific markers include CD30 which shows positivity only in embryonal carcinoma. CKAE1/AE3 is positive in both YST and embryonal carcinoma, epithelial membrane antigen (EMA) is positive in teratoma and β-human chorionic gonadotropin is positive in choriocarcinoma. AFP is strongly positive in YST. However, it has a very low sensitivity. Hence, serum and cerebrospinal fluid measurements of AFP are taken as a more accurate predictor for the diagnosis of YST. [9] In our case, high serum levels of AFP and AFP positivity on IHC confirmed the diagnosis of YST.

Treatment for cerebellar YST includes radical removal of the tumor with adjuvant chemotherapy with etoposide, bleomycin and carboplatin. [10] Tumor markers should be regularly monitored post-operatively to check for evidence of completeness of surgery, residual tumor and recurrence.

Early diagnosis in patients with YST is desirable as they are associated with a very poor prognosis. In our case, tumor markers were not performed pre-operatively as YST was not suspected. The patient was lost to follow-up. We report this case because of its rarity as less than 10 cases of cerebellar YST, including only two cases of cerebellar vermian YST having been reported in the literature so far.

 
   References Top

1.Lazzareschi I, Furfaro IF, Coccia P, Puma N, Riccardi R. Extragonadal yolk sac tumor outside of the midline of the body: A case report of a child with a yolk sac tumor of the pontocerebellar angle. Tumori 2009;95:840-2.  Back to cited text no. 1
    
2.Gabrovski S. Germ cell tumors of the brain-their course and management. Khirurgiia (Sofiia) 1997;50:8-15.  Back to cited text no. 2
[PUBMED]    
3.Ulbright TM. Germ cell tumors of the gonads: A selective review emphasizing problems in differential diagnosis, newly appreciated and controversial issues. Mod Pathol. 2005;18:S61-79.  Back to cited text no. 3
[PUBMED]    
4.Dede M, Pabuccu R, Yagci G, Yenen MC, Goktolga U, Gunhan O. Extragonadal yolk sac tumor in pelvic localization. A case report and literature review. Gynecol Oncol 2004;92:989-91.  Back to cited text no. 4
    
5.Nakamura T, Kanamori M, Sonoda Y, Watanabe M, Kumabe T, Tominaga T. Primary yolk sac tumor in the cerebellar vermis. Case report. No Shinkei Geka 2009;37:173-7.  Back to cited text no. 5
    
6.Tsukamoto H, Matsushima T, Shono S, Miyazono M, Fukui M, Kondo A. Primary yolk sac tumor of the cerebellar vermis: Case report. Surg Neurol 1992;38:50-6.  Back to cited text no. 6
    
7.D'Antonio A, Sparano L, Addesso M, Angrisani B, Cassese S, Angrisani P. Extraovarian yolk sac tumor in an elderly woman with an endometrioid-like pattern concurrent with a hepatoid component. J Obstet Gynaecol 2010;30:530-2.  Back to cited text no. 7
    
8.Fujimaki T, Matsutani M, Funada N, Kirino T, Takakura K, Nakamura O et al. CT and MRI features of intracranial germ cell tumors. J Neurooncol 1994;19:217-26.  Back to cited text no. 8
    
9.Ramalingam P, Malpica A, Silva EG, Gershenson DM, Liu JL, Deavers MT. The use of cytokeratin 7 and EMA in differentiating ovarian yolk sac tumors from endometrioid and clear cell carcinomas. Am J Surg Pathol 2004;28:1499-505.  Back to cited text no. 9
    
10.Yang SH, Hong YK, Kang JK, Kim MC. Primary yolk sac tumor of the cerebellar hemisphere: Case report. J Korean Neurosurg Soc 2003;33:419-21.  Back to cited text no. 10
    

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Correspondence Address:
Heena M. Desai
Topiwala National Medical College and BYL Nair Charitable Hospital, Dr. AL Nair Road, Mumbai Central, Mumbai - 400 008, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.134711

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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