|Year : 1994 | Volume
| Issue : 2 | Page : 85-87
Suprasellar germinoma : A case report.
Apjit Kaur, Kartikeya Sharma, Piyush Mittal, Vijendra K Jain
Department of Neurosurgery, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
Department of Neurosurgery, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Raibareily Road, P.B. No. 375, Lucknow 226 002
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Kaur A, Sharma K, Mittal P, Jain VK. Suprasellar germinoma : A case report. Indian J Ophthalmol 1994;42:85-7
The incidence of intracranial germinomas is less than 1 % of all intracranial neoplasms.  Germinomas do not commonly occur in a suprasellar location. The presence of diabetes insipidus as an early symptom has been reported in almost all patients of suprasellar germinomas and may help in distinguishing these tumours from other suprasellar lesions, especially optic nerve gliomas from which they may be even peroperatively indistinguishable. Although radiological studies including MRI and biochemical markers may be indicative of germinoma, histological verification is considered essential.  Germinomas are extremely radiosensitive, but chemotherapeutic agents offer an alternate therapeutic modality which is particularly useful in younger children to avoid post-irradiation growth retardation.
| Case report|| |
A 10-year-old girl presented with complaints of dull lethargic behaviour since 1 year and increased appetite of 1 month duration associated with headache, vomiting, frequent bed wetting, generalized seizures, and progressive painless diminution of vision in both eyes.
Physical examination revealed a thin built anaemic girl (body weight, 18 kg; height, 50 inches), with no abdominal, cardiovascular, or respiratory system abnormalities. Higher mental functions were normal. All cranial nerves except optic nerve were normal. Fundus showed bilateral primary optic atrophy. Her visual acuity was light perception in the right eye and 6/18 in the left eye. Visual field charting of the right eye was not possible, while the left eye showed temporal hemianopsia. Motor and sensory system examinations were normal.
Investigations revealed Hb, 10.7 gm%; random blood sugar, 168 mg%; serum urea, 29 mg%; serum Na, 136 mEq/l; Serum K, 3.8 mEq/l; urine osmol, 149 mosmols; Plasma osmol 284 mosmols. Tumour markers like alpha-fetoprotein (AFP) and human chorionic gonadotropin (HCG) could not be assessed preoperatively.
Plain X-ray skull lateral view showed demineralization of dorsum sellae, silver beaten appearance and sutural diastasis [Figure - 1]. Cranial contrast CT scan revealed a suprasellar, midline, well defined, homogeneously enhancing mass with intrasellar extension [Figure - 2][Figure - 3]. Small specks of calcification were also seen. The mass was extending into the third ventricle and both the lateral ventricles were dilated.
A ventriculoperitoneal shunt with biventricular connection was performed. A week later, a right pterional craniotomy was done under general anaesthesia. Peroperatively, a white fusiform swelling in the right optic nerve involving the chiasm was identified. The right optic nerve with adjacent chiasm was excised. Peroperative impression was of optic glioma.
In the postoperative period she developed diabetes insipidus which was managed by inj. aqueous pitressin. The postoperative visual acuity and field remained unchanged. Histopathological examination of the tumour showed groups and nests of large cells separated by thin fibrous septa infiltrated by lymphocytes [Figure - 4]. The tumour cells had abundant granular to vacuolated and clear cytoplasm containing glycogen. Nuclei were pleomorphic and contained prominent nucleoli. Six to seven mitotic figures per high power field were seen. These findings were consistent with the diagnosis of germinoma. Chemotherapy was started one month later with IV inj. cisplatin 20 mg/sqm and IV inj. etoposide 80 mg/ sqm given on the same day for five successive days. Four such cycles were given at an interval of four to six weeks. In order to avoid impairment of renal functions, prior hydration and IV mannitol were given. Hearing was monitored throughout the course of chemotherapy. At the end of the second cycle, the CT scan showed no evidence of the tumour mass. Her haemogram at the end of the fourth cycle was Hb, 10.5 gm%; TLC, 5.5 X 10 cumm/l; platelet, 106 X 10 cumm/l; serum creatinine, 0.09 mg/dl (Normal, 0.5 to 1.6 mg/dl).
| Discussion|| |
Suprasellar germinomas, apart from their rarity have variable clinical presentations. They have been grouped into three types : (1) primary tumour arising in the pineal region; (2) primary tumour arising in the third ventricle; and (3) primary tumour as an ectopic pinealome outside the brain.
The third type of suprasellar germinoma may appear as an enlarged grey or greyish-red chiasm and may be mistaken for an optic glioma 
The histological picture of germinomas is distinctive, regardless of their intracranial location .  Our patient falls into the third category of suprasellar germinomas. The initial diagnosis was optic glioma which was revised after histological examination.
Suprasellar germinomas are extremely radiosensitive tumours.  However, recent reports have highlighted the adverse effects of irradiation like myelosuppression, psychomotor retardation, etc., with brain atrophy, hypothalamic and endocrinal dysfunction. A CDDP-based combination chemotherapy with etoposide has been tried for germinoma. The therapeutic synergism has been suggested from experimental and clinical studies. 
In a 1-year follow-up, her visual acuity in the left eye improved to 6/6 but temporal hemianopsia persisted. Fundus examination of the right eye revealed primary optic atrophy and the left disc showed temporal pallor. Her height was 51 inches and weight was 25 kg on the last follow-up visit.
We present this case with the view of emphasizing the inclusion of this rare entity in the differential diagnosis of a child presenting with visual loss and endocrine dysfunction. The tumour in this case is radiosensitive and also responds to chemotherapy, thus avoiding extensive surgery in childhood. In the presence of definitively raised tumour markers, histological verification is, however, no longer essential. A peroperative frozen section study may avoid extensive surgery in patients with normal tumour markers. Combination chemotherapy with CDDP and etoposide is the recommended modality of treatment.
| References|| |
Alberchesten R, Klee JG, Moller JE. Primary intracranial germ cell tumors including five cases of endodermal sinus tumor. Acta Pathol Microbiol. Scand. 80(suppl 233):32-38, 1972.
Kilgore DP, Strother CM, Starshak RJ, et al. Pineal germinoma: MR Imaging. Radiology. 158:435-438, 1986.
Kageyama N, Belsky R. Ectopic pinealoma in the chiasmal region. Neurology. 11:318-327, 1961.
Cohen DN, Stein Berg M, Buchwald ' R. Suprasellar germinoma:diagnostic confusion with optic gliomas. J Neurosurg. 41:490-498, 1974.
Andrew J, Kendall BE, Brook CD. Germinoma of the pituitary gland: a report of 2 cases: Short report. J Neurol, Neurosurg, Psychiatry. 48:589-592, 1985.
Kageyama N. Ectopic pinealoma in the region of the optic chiasm: report of five cases J Neurosurg. 35:755759, 1971.
Miller NR. Tumors of maldevelopmental origin and related lesions. In: Walsh and Hoyt's Clinical NeuroOphthalmology. Vol.3, 4th ed. William & Wilkins, Baltimore, 1988.
Leibel SA, Sheline GE. Radiation therapy for neoplasms of the brain. J Neurosurg. 66:1-22, 1987.
Kobayashi T, Yoshida J, Ishiyama J. Combination chemotherapy with cisplatin and etoposide for malignant intracranial germ cell tumor. An experimental and clinical-study. J Neurosurg. 1989.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]