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 »  Abstract
 »  Introduction
 »  Case report
 »  Discussion
 »  Conclusion
 »  References

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Year : 2001  |  Volume : 49  |  Issue : 4  |  Page : 395-7

An unusual presentation of an intra-parenchymatous frontal yolk sac tumour : case report.

Department of Neurosurgery, National Neurosurgical Centre, Khoula Hospital, Muscat, Sultanate of Oman.

Correspondence Address:
Department of Neurosurgery, National Neurosurgical Centre, Khoula Hospital, Muscat, Sultanate of Oman.

  »  Abstract

Yolk sac tumours are rare conditions among the germ cell tumours. Intracerebral germ cell tumours are exceedingly rare. A 15 year old girl presenting with a one week history of raised intracranial pressure is described. She had bilateral papilloedema and a right 6th nerve palsy. CT scan showed an intra-parenchymatous right frontal ring enhancing lesion of 2 cms diameter. The patient underwent microsurgical total excision of the tumour, followed by chemotherapy. She was asymptomatic at three years following surgery.

How to cite this article:
Netalkar A S, Sharma R R, Mahapatra A K, Sousa J, Lad S D, Pawar S J, Mishra G P, Musa M M. An unusual presentation of an intra-parenchymatous frontal yolk sac tumour : case report. Neurol India 2001;49:395

How to cite this URL:
Netalkar A S, Sharma R R, Mahapatra A K, Sousa J, Lad S D, Pawar S J, Mishra G P, Musa M M. An unusual presentation of an intra-parenchymatous frontal yolk sac tumour : case report. Neurol India [serial online] 2001 [cited 2018 Apr 25];49:395. Available from:

   »   Introduction Top

Yolk sac tumours are rare intracranial tumours.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10] Typically, the common location of these tumours is the pineal region or the suprasellar area.[4],[7],[8] Rarely, these have been reported in the cerebellar[10] or cerebral hemispheres.[11] Very rarely germinomas present with intra-lesional haemorrhage.[12] A 15 year old girl with a frontal lobe yolk sac tumour is presented.

   »   Case report Top

A 15 year old girl was admitted at the National Neurosurgical Center, at Khoula Hospital, Oman, with a short history of a headache, vomiting and blurring of vision of one week duration. Except for a right 6th nerve palsy and bilateral gross papilloedema, she had no other neurological deficit and her systemic examination was unremarkable. Her haematological and biochemical parameters were normal. Serum alpha foeto protein levels were elevated (1230 ng/ml) but, the ?HCG was normal. Abdominal and pelvic ultrasound examination was also normal. Plain and contrast CT scans revealed a right frontal lesion with a hypo-attenuated center, and a hyper-attenuated ring, which enhanced on contrast administration. Mild peritumoural oedema was noted. A right frontal craniotomy was performed and a cystic lesion containing dark fluid was noted. The cyst wall was of non-uniform thickness and was infiltrating into the brain parenchyma at many places. Total excision using micro-neurosurgical technique was possible. At the site of parenchymal infiltration, the involved parenchymal tissue had to be excised along with the cyst wall. Post operatively, the patient made an uneventful recovery.
Histological examination showed features of a typical yolk sac tumour with areas of haemorrhage. A tubullopapillary structure with finely vacuolated, cuboid pleomorphic cells, cystic spaces containing eosinophilic hyaline colloid material and intracytoplasmic hyaline droplets was noted (Schiller Duval bodies glomeruloid structures with surrounding cresentric spaces) [Figure. 3]. There were areas of haemorrhage.
Post operatively, the patient received chemotherapy using a combination of cisplatinum, vincristine and bleomycin (PVB) as follows: - cisplatinum-20 mg/m2 of body surface from day 1-5, vinblastin-4 mg/m2 of body surface on days 1 and 8, and bleomycin 10 mg/m2 on days 1,8 and 19. The above regimen was repeated for 3 cycles with a 3 week interval, and thereafter repeated every 4 months for a period of 2 years. She was regularly followed up and was asymptomatic after 3 years. Periodic CT scans in the interim period revealed no recurrence.

   »   Discussion Top

Intracranial germ cell tumours (GCT) are rare and constitute less than 2% of all intracranial neoplastic lesions.[1] Among the various germ cell tumours, intracranial intra-parenchymal yolk sac tumours are extremely rare and interesting clinical entities whose pathogenesis is not yet clear.[1],[2],[3],[4],[7],[8] Yolk sac tumours, choriocarcinomas and mixed tumours are all malignant tumours and highly resistant to treatment, with a median survival of less than 2 years.[5],[6] Generally, germ cell tumours are located either in the pineal region or in suprasellar region.[1],[4],[5] However, very rarely these tumours have been reported at other sites as well.[8],[10],[11] Of the five paediatric cases reported by Ustio et al three were in the suprasellar region and the remaining two were in the pineal region.[4] In the present case, the tumour was in the right frontal lobe. We could find one such case in literature.[11]
Generally, patients with intracranial germ cell tumours by virtue of their location in the pineal gland or suprasellar area, present with raised intracranial tension of long duration, giving rise to hydrocephalus.[1],[4],[9] In the present case, the patient had a short history of only 7 days. Rapid symptoms may have been due to haemorrhage into the cyst. Pathologically, germinomas are solid vascular tumours without any cyst formation and necrosis.[4],[8],[9] and they enhance on contrast CT scan. Our patient had a hypo-attenuating lesion with a ring enhancement on contrast, which is a rare CT finding in germinomas.
Histologically, the present case had typical features of a yolk sac tumour,[4],[7],[9] characterised by tubullopapillary structures with vacuolated cuboidal cells, cystic spaces containing eosinophilic hyaline (colloid like) material, and intracytoplasmic hyaline droplets called Schiller Duval bodies. Histology also showed haemorrhagic areas. We could find only one other report mentioning haemorrhage in a yolk sac tumour,[12] emphasising this as a pathological rarity.
Overall outcome of children with intracranial yolk sac tumour is not very encouraging.[1],[4],[6],[9],[13],[14] Median survival is less than 2 years and the 5 year survival rate is less than 25%.[6],[9],[14] Einhorn and Donahue in 1977 introduced the PVB regimen in the treatment of germinomas.[15] Since then, there has been an increased use of chemotherapy as an adjuvant with a moderately increased survival. However, rarely long term survival has been reported.[4],[14],[16] Ustio et al[4] employed combination chemotherapy and concomitant radiotherapy in 5 cases. All the patients survived more than 34 months with an average of 7 years, and at the time of the report all the patients were still surviving. Our patient underwent a combined surgical excision followed by combination chemotherapy (PVB) and is doing well at 36 months after surgery.

   »   Conclusion Top

Intracerebral yolk sac tumour is a rare condition with a relatively poor outcome. With improved imaging facilities and combined radio-chemotherapy, the outcome of these patients has shown improvement.

  »   References Top

1.Kuratsu JI, Ushio E : Epidemiological study of primary intracranial tumours: a regional survey in Kaunomoto Prefecture in the southern part of Japan. J Neurosurg1996; 84 : 946-950.   Back to cited text no. 1    
2.Sano K : So called intracranial germ cell tumours. Are they really germ cell origin? Br J Neurosurg1995; 9 : 391-401.   Back to cited text no. 2    
3.Sano K : Pathogenesis of intracranial germ cell tumours reconsidered. J Neurosurg 1999; 90 : 258-264.   Back to cited text no. 3    
4.Ustio Y, Kochi M, Kuratsu JI et al : Preliminary observations for a new treatment in children with primary intracranial yolk sac tumour or embryonal carcinoma. Report of 5 cases. J Neurosurg 1999; 90 : 133-137.   Back to cited text no. 4    
5.Itomaya Y, Kochi M, Kuratsu J et al : Treatment of intracranial non-germinomatous malignant germ cell tumours producing ?fetoprotein. Neurosurgery 1995; 36 : 459-466.   Back to cited text no. 5    
6.Arita N, Ushio Y, Hayakawa T et al : Role of tumour markers in the management of primary intracranial germ cell tumours. Prog Exp Tumor Res 1987; 30 : 289-295.   Back to cited text no. 6    
7.Takei Y, Pearl GS : Ultrastructure study of the intracranial yolk sac tumours with special reference to the oncologic phylogeny of germ cell tumours. Cancer 1981; 48 : 2038-2046.   Back to cited text no. 7    
8.Fujiwara T, Honjo Y, Nagao S et al : Primary yolk sac tumour: Case report. Surg Neurol 1994; 42 : 121-124.   Back to cited text no. 8    
9.Jennings MT, Gelman R, Hodiberg F : Intracranial germ cell tumour. Natural history and pathogenesis. J Neurosurg 1985; 63 : 155-167.   Back to cited text no. 9    
10.Tsukamoto H, Matsushita T, Shono S et al : Primary yolk sac tumour of the cerebellar vermis: Case report. Surg Neurol 1992; 38 : 50-56.   Back to cited text no. 10    
11.Sugawara T, Tsurumi Y, Kuwahara K et al : Primary intracranial yolk sac tumour developing in the frontal lobe from the inside of the sphenoid ridge. No Shinko Geka1984; 12 : 401-406.   Back to cited text no. 11    
12.Takano S et al : Primary intracranial yolk cell tumour. No Shinko Geka 1993; 21 : 533-537.   Back to cited text no. 12    
13.Rich TA, Cassady JR, Strand RD et al : Radiation therapy for pineal gland and suprasellar germ cell tumours. Cancer 1985; 55 : 932-940.   Back to cited text no. 13    
14.Mitsutani M, Sano K, Takakura K et al : Primary intracranial germ cell tumours: A clinical analysis of 153 histologically verified cases. J Neurosurg 1997; 86 : 446-455.   Back to cited text no. 14    
15.Einhorn LH, Donahue J : Diaminodichloroplatinum, vinblastine and bleomycin combination in disseminated testicular cancer. Ann Int Med 1977; 87 : 293-298.   Back to cited text no. 15    
16.Robertson PL, Da Rosso RC, Allen JC : Improved prognosis of intracranial non-germinous germ cell tumours with multinodal therapy. J Neurooncol 1997; 32 : 71-80.   Back to cited text no. 16    


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