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Peduncular hallucinosis is a rare phenomenon characterized by visual hallucination consisting of vivid, colored image of people, animals, scenes or geometric patterns. In most of the cases the hallucination is accompanied by sleeping disorder, and is by the patient recognized as not being real. It can be observed mainly in patients with thalamic/midbrain vascular disease, and is less frequently seen in brain stem tumor. The damage of the ascending reticular activating system by brain stem compression causing dream activity releasing what is normally suppressed during wakefulness is thought to be the mechanism of peduncular hallucinosis. The authors report a 13-year-old female presenting peduncular hallucinosis due to brainstem compression by a pineocytoma. The patient had a 3-month history of complex visual hallucination and slight somnolence. The hallucination mainly consisted of TV game characters, animals, and vegetables which were colorful and vivid. She was well oriented and realized that the hallucination was not real. MRI showed a pineal mass compressing the quadrigeminal plate inferiorly. There was mild obstructive hydrocephalus due to aqueduct stenosis. The tumor was totally removed and was pathologically diagnosed as pineocytoma. After the surgery, the hallucination ameliorated remarkably. Although the administration of benzodiazepine exacerbated the hallucination and sleep disorder, oral carbamazepine was clearly effective and produced nearly complete disappearance of hallucination. To the best of our knowledge, this is the first report of peduncular hallucinosis caused by quadrigeminal plate compression by a pineal tumor.

A 61-year-old woman had suffered from severe headache and nausea over 20 times during the last 43 years. An subarachnoid hemorrhage (SAH) was detected by spinal puncture in some other hospitals, but the source of hemorrhage remained unknown in spite of repeated angiography. At the age of 61, she was diagnosed as having normal pressure hydrocephalus, and received a ventriculo-peritoneal shunt. She suffered from sudden headache 12 days after surgery. A CT scan showed a SAH and enlargement of the pineal mass. The tumor was totally removed via the occipital interhemispheric transtentorial approach and was diagnosed histologically as a pineocytoma. She has been free from SAH for three years since removal of the tumor. Pineal apoplexy should be considered as a cause of SAH.

Purpose: Understanding the molecular mechanisms by which distinct cell fate is determined during organogenesis is a central issue in cell development and disease. Using conditional gene ablation, we show that in mice the transcription factor Otx2 is essential for retinal photoreceptor cell fate determination and the development of the pineal gland., Methods and Results: In the Otx2 knockout mice, differentiating photoreceptor cells were converted to amacrine-like neurons, and the pinealocytes in the pineal gland were totally absent. In addition, promoter assays revealed that Otx2 transactivates Crx, which is required for terminal differentiation and maintenance of photoreceptor cells. Furthermore, retroviral gene transfer of Otx2 steered retinal progenitor cells toward becoming photoreceptors., Conclusion: Thus, Otx2 is a key regulatory gene for the cell fate determination of retinal photoreceptor cells. Our results revealed the key molecular steps required for photoreceptor cell fate determination and pineal gland development.

A 9-year-old female suddenly developed headache and nausea. Computed tomography (CT) revealed pineal mass lesion and obstructive hydrocephalus. One week after the onset, hydrocephalus spontaneously resolved. Magnetic resonance imaging (MRI) revealed that the mass was a subacute hematoma and that hydrocephalus had improved. Sequential MRI 4 months later revealed an enhanced lesion in the pineal region. Total removal of the tumor by the occipital transtentorial approach established the histopathological diagnosis of pineoblastoma.

A case of a primary pineal embryonal carcinoma occurring in a middle aged man is reported. A 42-year-old man suffering from headache and nausea was referred to our department. A neurological examination revealed that he had Parinaud's sign. Head CT and MRI showed a tumor in the pineal region. He was operated on using the occipital trans-tentorial approach. The tumor was partially removed and an intra-operative specimen was used to diagnose a kind of germ cell line tumor. However, the tumor was diagnosed afterwards as a pure embryonal carcinoma. Three courses of PE chemotherapy followed by 30 Gy of whole craniospinal irradiation and 30 Gy of extended local irradiation were completed. An MRI showed the tumor to be in complete remission. Despite careful follow-up with chemotherapy every three months, a re-operation and linac radio-surgery, the tumor recurred, and disseminated. The patient died due to an intra-tumoral hemorrhage. A pure primary pineal embryonal carcinoma occurring in a middle-aged person has never been reported previously in detail.

A 12-year-old boy with primary intracranial choriocarcinoma of the pineal region suffered from repeated intratumoral hemorrhage over a short period. Neuroradiological images demonstrated a pineal-region tumor with intratumoral hemorrhage and obstructive hydrocephalus. HCG-beta subunit concentration of his serum and cerebrospinal fluid was abnormally high. After the 24.2Gy irradiation, the tumor was removed almost totally. After the operation, we treated him with additional radiation and chemotherapy using ifosfamide, cisplatin, and etoposide (namely ICE therapy). No recurrence or metastasis has been noted, and he was in good condition during the 22 months since the operation. At present he receives maintenance ICE therapy every 3 months. Choriocarcinoma involves a high possibility of intratumoral fatal hemorrhage, so a radical operation is recommended as soon as possible.

Primary germinoma of the central nervous system carries a good prognosis because of their radiosensitivity. Recurrence is rare and extraneural metastases are even more unusual. We report a unique case of a primary intracranial germinoma exhibiting complete response to radiotherapy, but recurring as an intra-abdominal yolk sac tumor. The presence of a VP shunt is thought to have facilitated metastatic spread of the intracranial neoplasm. An 21-year-old (corrected) male was admitted with headache and consciousness disturbance. Computed tomography (CT) revealed an enhanced tumor of the pineal region and hydrocephalus. A ventriculo-peritoneal shunt was emplaced immediately. No serum tumor markers such as alpha-fetoprotein or human chorionic gonadotropin were detectable. A test dose of radiotherapy (whole brain 20Gy) was given. The tumor size was remarkably decreased. Clinical diagnosis was germinoma. Additional whole brain radiation (total 45Gy) and whole spine radiation (20Gy) were given. The tumor and the hydrocephalus regressed completely and the patient returned to school. Three years later, he experienced a feeling of abdominal fullness. CT and echotomography of the abdomen showed a large peritoneal and intra-hepatic tumor. But MRI revealed no recurrent tumor of the pineal lesion or of the other areas in the central nervous system. Radiological and clinical findings showed no tumors in the testis, the retroperitoneal cavity, or the thymus. Laboratory investigation demonstrated elevated serum AFP (26,550 ng/ml). AFP level regressed after combined chemotherapy. However, the patient died due to pneumonia and multiple organ failure. Only needle necropsy was performed. The microscopic appearance of the peritoneal tumor was confirmed to be an endodermal sinus tumor. It was suspected to be a metastasis of the pineal tumor through the V-P shunt system.

We analyzed clinically 30 germinomas (22 pure germinomas and 8 germinomas with syncytiotrophoblastic giant cell (STGC) and also investigated the proliferative potential of 18 germinomas immunohistochemically using MIB-1 monoclonal antibody in formalin-fixed paraffin-embedded sections. The majority of patients responded favorably and completely to the treatment. Seven patients (four had germinomas with STGC and the other three had pure germinomas) suffered recurrence after a complete response to radiotherapy and/or chemotherapy. The patients with germinomas with STGC experienced recurrence significantly more often than those with pure germinomas (P < 0.01). Three of the patients died. The MIB-1 indexes of the germinomas ranged from 18% to 80%. The average MIB-1 index of pure germinomas is 58.5 +/- 17.3%, that of germinomas with STGC is 45.3 +/- 17.1%. There was no significant difference between the two groups. The MIB-1 indexes of 3 recurrent cases were 64.3%, 51.9%, and 20.8%, respectively. There were no significant differences in the MIB-1 indexes between nonrecurrent cases and recurrent ones.

We report a simple and easily-applicable method to intraoperatively monitor the oculomotor nerve and oculomotor nucleus in the brainstem. A needle electrode is inserted into the space just beneath the superior root of the orbit. With this superior orbital electrode, evoked compound muscle action potentials from the superior rectus and the levator palpebrae superioris muscles were recorded. The nuclei for these two muscles are located caudally in the midline of the midbrain. The responses from the superior orbital electrode, therefore, give the surgeons useful intraoperative guidance during surgery for pineal region tumors and midbrain gliomas.

A 6-month-old girl was admitted to another hospital because of consciousness disturbance, preceded by 2 weeks of decreased activity and vomiting. She was referred to our hospital after ventricular drainage had been instituted for hydrocephalus and the tumor in the pineal region. The patient was noted to have conjugate upward gaze palsy and papilledema. CT scan and MRI revealed a large tumor in the pineal region with tumoral hemorrhage and a small mass in the right frontal lobe. At surgery, the pineal region tumor was removed subtotally. Histological examination showed the tumor to be composed of sheets of large polyhedra or round cells with an eccentric round nuclei, prominent nucleoli, and cytoplasmic inclusions. Immunohistochemical studies were positive for GFAP, vimentin, S-100, CK, EMA, and SMA, but negative for AFP, HCG, PLAP, and CEA. Following surgery, she received three 5-day cycles of chemotherapy, consisting of intravenous administration of cisplatin 20 mg/m2/day and etoposide 60mg/m2/day. After these therapies, MRI showed a decrease in the area of high intensity in the pineal region, but almost no change in the right frontal mass lesion. Follow-up radiological examination showed that the tumor had grown rapidly one month after chemotherapy and the patient died 5 months after her first hospitalization. Malignant rhabdoid tumor of the CNS is rare and remarkably malignant. This tumor should be treated using multidisciplinary management with surgery, intensive chemotherapy, and radiotherapy depending on the patient's age.

We report a case of "functional aqueductal stenosis" which reveals dilatation of the lateral and 3rd ventricles without stenosis at the aqueduct in MRI. This case shows a pineal teratoma which presents one year later with symptoms of hydrocephalus caused by "functional aqueductal stenosis". A seven-year-old boy was admitted to our department owing to headache and vomiting. CT and MRI showed hydrocephalus. The lateral and 3rd ventricles were dilated while the 4th ventricle was normal. Furthermore, tumoral obstruction of the aqueduct was not found. After a ventriculoperitoneal shunt, he recovered well without neurological deficits. One year later, symptoms of precocious puberty, that is the appearance of public hair and deepening of his voice, were found. A follow-up MRI demonstrated a pineal region tumor. Although human chorionic gonadotropin level in the serum and urine was transiently elevated, it normalized before surgery. The operation was performed by the occipital transtentorial approach and the tumor was totally removed. Histological examination proved this tumor to be a mature teratoma, showing three germ cell layers. About two weeks later, he was discharged without any neurological deficit. In this case, although hydrocephalus occurred, MRI didn't demonstrate aqueductal obstruction caused by the tumor. However, one year later, a pineal region tumor was confirmed by MRI. This suggests that hydrocephalus might have some association with the appearance of the pineal region tumor. Therefore, it is necessary to be aware of the possibility of the occurrence of tumors whenever we encounter "functional aqueductal obstruction", when MRI doesn't demonstrate aqueductal obstruction caused by a tumor.

A 40-year-old woman experienced three attacks of aseptic meningitis in 19 months. Each attack was characterized by sudden onset of meningeal irritation followed by spontaneous remission in several days. The patient was free of neurological symptoms between the attacks. In the acute phase of each attack, her cerebrospinal fluid (CSF) showed mononuclear pleocytosis and increased protein concentration. Epithelial cell clusters in the CSF were evident during the third episode. No pathogenic microorganisms were identified in CSF or blood cultures. Brain imaging revealed a pineal cyst, 1 cm in diameter, and we suspected that the epithelial cell clusters represented the ruptured cystic wall and that the recurrent aseptic meningitis was triggered by spontaneous rupture of the cyst. Recently several new etiologies for the benign recurrent aseptic meningitis (Mollaret's meningitis) have been reported, but its existence as a clinical entity is still in dispute. Our case appears to support "spontaneous rupture of central nervous system epidermoid cysts" as one of the etiologies of recurrent aseptic meningitis.

The treatment strategy and prognosis of pineal cell tumors are still subjects of debate because of their rarity and the mixture of pineoblastoma and pineocytoma as components. Pineoblastoma is believed to be the more malignant tumor and total gross resection of this tumor is very difficult because of its invasive tendency and location. Although the effectiveness of external irradiation and chemotherapy has been reported, the outlook for patients with this tumor is extremely poor. We treated a case of pineoblastoma with a single total resection nine months after interstitial irradiation and chemotherapy. In this case, brachytherapy was successful as neoadjuvant therapy for decreasing the tumor's volume and clarifying its boundary. The patient was a 36-year-old woman who had complained of occipital headache for about a month. On admission, neurological examination revealed bilateral papilloedema but, otherwise, there were no deficits. A magnetic resonance image (MRI) showed a homogeneously enhanced tumor in the pineal region and obstructive hydrocephalus. Two weeks after ventriculo-peritoneal shunting, a stereotactic needle biopsy was performed using a BRW MRI-guided stereotactic apparatus, and three catheters for interstitial brachytherapy were implanted into the tumor through the biopsy tract. Iridium-192 seeds were inserted through the catheters and kept there for 8 days to irradiate 40 Gy at the tumor periphery. Four courses of chemotherapy with carboplatin (400 mg/m2) and VP-16 (400 mg/m2) were administered after brachytherapy. Repeat MRI scans every month showed a gradual regression of tumor volume. However, the tumor did not disappear and no further decrease in tumor volume was observed 8 months after brachytherapy. A suboccipital craniotomy was performed 9 months after brachytherapy and en bloc tumor resection was achieved readily via an infratentorial supracerebellar approach. After receiving another course of chemotherapy and external irradiation at 40 Gy, the patient was discharged without any symptoms and no residual tumor or recurrence was observed for 24 months after brachytherapy and 15 months after surgery. The clinical course of this case suggests that a combination of stereotactic biopsy and brachytherapy with chemotherapy followed by surgery may be a good strategy for the treatment of pineal cell tumors regardless of their histopathological features.

A 5-year-old girl was admitted to another clinic because of vomiting and convulsions. She was brought to our clinic after a ventriculoperitoneal shunt was inserted. CT scan on admission in our clinic showed a tumor in the pineal region with tumoral hemorrhage. Tumor markers such as HCG, AFP, CEA, P-LAP were within normal range. A biopsy of the tumor was performed and the histological diagnosis was pineoblastoma. Her recovery was excellent and disseminated metastasis was not recognized. A subtotal removal of the tumor was performed through the occipital transtentorial approach. She had no neurological deficits after surgery. She then received two 5-day cycles of chemotherapy, consisting of intravenous administration of 20 mg/m2/day cisplatin and 60 mg/m2/day etoposide, and craniospinal radiotherapy. After these therapies, the tumor responded and disappeared completely. Follow-up radiographic investigations also demonstrated no abnormal evidence except for brain atrophy. She is attending a primary school without any problems. Pineoblastoma is quite rare and remarkably malignant. Hence, aggressive therapies including surgery, radiotherapy and chemotherapy is indicated for this tumor.

Patients who have a pineal germinoma usually present with symptoms of intracranial hypertension or disturbances in vertical ocular motion but rarely present with amnesia. We recently encountered a case of pineal germinoma accompanied by severe amnesia, but not by hydrocephalus. A 32-year-old, right-handed man was admitted with severe memory disturbance which had gradually worsened in the preceding three months. On admission, he was alert and cooperative. His speech was well preserved and not fabricated. He had neither symptoms of intracranial hypertension or visual disturbance. There was no manifestation of interhemispheric disconnection symptoms. His immediate and semantic memory was preserved, however, his recent and antegrade episodic memory was severely impaired. The amnesia involved both verbal and visual modalities. He could recall only 1 out of 5 objects in 5 minutes. He was orientated to people, but not to time and place. And his motivation was severely affected. Magnetic resonance imaging (MRI) showed a heterogeneously enhanced tumor in the splenium and pineal body that extended into the bilateral ventricular trigon through major forceps and was accompanied by edema in the retrosplenial region. The bilateral crura of the fornix was obscured by the tumor. The enlarged pineal body was slightly compressing the upper colliculus but hydrocephalus was not observed. The tumor, which was partially excised through a right parietal corticotomy, had features typical of a germinoma.(ABSTRACT TRUNCATED AT 250 WORDS)

Four cases of pineal cyst were presented and their features on magnetic resonance (MR) imaging were discussed while reviewing the literature relevant to this clinical entity. Pineal cyst is a normal developmental variant and in most of the cases its diagnosis is not difficult, but in a limited number of cases, pineal cyst needs to be differentiated from cystic tumors which have developed in the region of the pineal gland, such as pineocytoma or astrocytoma. Most pineal cysts are found incidentally during MR screening studies and no surgical interventions are needed. Pineal cysts should be considered for surgery only when the diameter of the cyst is more than 1.0cm, or when obstructive hydrocephalus is verified on MRI or CT, and/or when definitive neurological signs such as Parinaud's syndrome are present. It was also stressed that embryological considerations are indispensable for evaluating pineal cysts on MR images.

A rare case of mature pineal teratoma with interesting radiological findings in a 16-month-old infant is reported. The patient was referred to our clinic because of generalized convulsions. A CT scan showed marked hydrocephalus and a low density mass lesion without contrast enhancement in the pineal region. A CT cisternography demonstrated the lesion as a filling defect area on the image produced immediately after the emergent V-P shunt, and a filling area 24 hours later. The lesion was of signal intensity on T-1 weighted MR image and of high signal intensity on T-2 weighted MR image, equal to CSF intensity. These radiological findings were compatible with an arachnoid cyst in the quadrigeminal cistern, so we performed the excision of both anterior and posterior cyst walls using the occipital tentorial approach. However, the histology of the cyst wall was not compatible with that of an arachnoid cyst, showing neuroepithelial-like cell lining with positive staining for cytokeratine. The postoperative follow-up on MRI was continued for 31 months. An MRI performed 9 days after the first operation showed, compared with the gray matter, iso-signal intensity area and high signal intensity area on T-1 weighted image. At first, we thought this was because of bleeding of the pineal gland brought on by the operative maneuver. However, the mixed intensity lesion shown on T-1 weighted images gradually expanded and distorted, and finally showed the typical MR images for a teratoma. The operative findings using the occipital transtentorial approach were typical for a teratoma.(ABSTRACT TRUNCATED AT 250 WORDS)

Although radiation therapy has been universally accepted as treatment for primary intracranial germinomas, the optimal technique for both diagnosis and treatment continues to be a matter of controversy. We report a case of germinoma in the pineal region treated by interstitial brachytherapy. A 23-year-old female was admitted with diplopia and consciousness disturbance. Magnetic resonance imagings (MRI) revealed an enhanced tumor of the pineal region and hydrocephalus. No serum and CSF tumor markers such as alpha-fetoprotein (AFP), human chorionic gonadotropin (HCG) or placental alkaline phosphatase (PLAP) were detectable. Stereotactic needle biopsy was performed using BRW MRI-guided stereotactic apparatus. After the histological diagnosis confirmed the presence of germinoma, three catheters for interstitial brachytherapy were implanted into the tumor through the biopsy tract, and iridium-192 seeds were inserted into the catheters and kept there for 5 days to give 35 Gy of irradiation at the tumor periphery. Subsequent MRI showed marked tumor regression and the clinical symptoms were gradually improved, and Gd-enhanced tumor on MRI completely disappeared 2 months after brachytherapy. Histological diagnosis is essential before initiation of treatment because a germinoma is commonly thought to be radiosensitive. However, pineal region tumor is dangerous to remove surgically because of high morbidity and mortality rates. Image-guided stereotactic technique provides a safe method to obtain an accurate diagnosis, and subsequent interstitial brachytherapy enables selective irradiation of the tumor, and does not give rise to complications caused by external irradiation such as mental disturbance, hypopituitarism, visual disturbance and brain necrosis. Interstitial brachytherapy following image-guided biopsy can be a useful tool for patients with germinoma.

A 13-year-old boy complained of frequent headaches and diplopia on rightward gaze. A CT scan demonstrated hydrocephalus and a non-homogeneous enhancing mass in the pineal area, leading to a diagnosis of pineal tumor. The physical examination revealed no abnormalities. On neurological examination, there were a right homonymous hemianopsia and slight choked disc in both optic fundi. His right eye was slightly adducted on primary gaze. On right lateral gaze, his right eye could not move beyond the midline and showed gaze paretic nystagmus, whereas his left eye could move fully in all directions. The abducens palsy could be overcome by the oculocephalic maneuver or caloric test. Interestingly, his right eye could abduct when his left eye was covered. From these finding, this was labelled a supranuclear abducens palsy. The convergent nystagmus was observed. Rightward OKN (quick phase to right) of the right eye was abolished with and without the left eye covered, while leftward OKN of the right eye was preserved. Pursuit to right was disturbed. After removal of the tumor, the gaze palsies disappeared. It is postulated that the supranuclear lateral gaze palsy was caused by impairment of supranuclear control by involvement of lateral gaze pathways to gaze center coursing near the oculomotor nucleus.

A case with abnormal eye movement induced by eyelid closure and selective paralysis of downward gaze was reported. CT scan and MRI imaging revealed a pineal tumor with bilateral involvement of the thalamomesenchephalic junctions without obvious damage to the posterior commissure. Histopathologically, the tumor was a germinoma. The pupillary light reflex was absent and near reflex was reduced. Lightning eye movement, skew deviation and convergence-retraction nystagmus were observed. The horizontal eye movement was normal in both eyes. The eye did not move downward below the level of direct forward gaze spontaneously or on attempted downward gaze. Downward vestibulo-ocular reflex was absent. Bilateral abnormal vergence eye movement with alternating convergence and divergence was observed during eyelid closure. The cycle of the abnormal eye movement was about 3Hz and there was no fast phase. The abnormal eye movement was not observed in the darkened room with both eyes open. After subtotal removal and radiation therapy of the pineal tumor, the abnormal eye movement disappeared.

Presented is a retrospective study of 20 patients with intracranial germ cell tumors who the authors have treated during past 10 years. The cases among these patients included thirteen germinomas, three teratomas, two embryonal carcinomas, and two malignant teratomas. A ventriculoperitoneal shunt was installed in 15 cass and the tumor was surgically removed in four cases. All cases were irradiated with 33 to 61 Gy; in 18 cases, more than half of the total dose was delivered to the whole brain, and in 9 cases, spinal irradiation was given because of a verified CSF dissemination. Chemotherapy was the regimen for four cases. The tumor responded to therapy in 16 (84%) of 19 evaluable cases. Overall, the median and five year survival were 102 months and 83%, respectively. The survival rate was found to vary between the benign group (germinoma, teratoma) and the malignant group (embryonal carcinoma, malignant teratoma, germinoma with STGC) (p less than 0.01).

A 58-year-old man was admitted to our hospital upon his request for an operation on a hemifacial spasm that had developed twelve years ago. The hemifacial spasm on the left side was the only positive neurological findings at the time of admission. However, a pre-operative neuroradiological examination revealed a meningioma originating in the falco-tentorial junction. Left neurovascular decompression was initially performed and was followed by the excision of the tumor through a right parietooccipital approach. The pathological diagnosis was meningothelial meningioma. The patient was discharged with no neurological deficit. There have been only a few reports on pineal meningioma and even now this type of tumor is considered to be rare. Some similar cases reported in the literature are reviewed. The clinical aspects, clinical features and the pathological entity are discussed.

A case of pineal epidermoid cyst with interesting findings of magnetic resonance imaging (MRI) is reported referring to the literature. In our case, homogeneous high density area is seen on CT and high signal intensity is recognized on T1-weighted MRI and low signal intensity on T2-weighted MRI.

A case of pineal gangliocytoma in a 51 year-old man is presented. He was admitted to the hospital on February 2, 1982, with complaints of headache, nuchal pain, blurred vision, nausea and vomiting of three years' duration. Neurological examination did not show any neurological deficits but bilateral choked disc. Preoperative CT scan disclosed a sharp by circumscribed high density lesion in the pineal region with moderate hydrocephalus. Preoperative 99m Tc-DTPA brain scan revealed a warm activity in the pineal region, and changes of its activity were only little in the course of time. Preoperative Amipaque ventriculogram showed dilation of the lateral ventricles and a shadow of the tumor bulging into the posterior half of the distended third ventricle. A diagnosis of tumor of the pineal region was made and removal of the tumor was performed by biparieto-occipital interhemispheric approach in "sea lion" position. The tumor was a dark reddish solid mass which replaced the pineal body and extended under the cerebellar tentorium. The patient made an uneventful recovery without any neurological deficits. By light microscopy, the neoplasm was composed mostly of mature and immature ganglion cells and small round cells with moderate cellularity and multiform cytologic features. Ganglion cells with large nuclei and prominent nucleoli had characteristic Nissl substance in various amounts. Oligodendrocytes and astrocytes appeared around the tumor but did not show neoplastic growth. GFAP stain did not show glial component in the tumor. According these findings, the tumor was diagnosed as gangliocytoma originated from the pineal body, and this was the first case in Japan.

A 7-year-old boy was admitted to Hokkaido University Hospital complaining of headache and vomiting. On admission he was slightly confused and presented Parinaud's sign. CT scan revealed abnormal high density mass with contrast enhancement effect at the pineal region and obstructive hydrocephalus. Laboratory studies showed the normal value of human chorionic gonadotropin and no trace of alpha-fetoprotein. Germinoma was most suspected based on the findings of CT scan and laboratory studies. The radiation therapy was carried out for a month and CT scan taken after the radiation therapy revealed marked reduction of the size of the tumor at the pineal region, and he was discharged. But he was re-admitted 3 months after the discharge complaining of headache and vomitting again. CT scan showed the recurrence of the tumor and laboratory studies showed abnormal high value of A.F.P. After the ventriculo-peritoneal shunt, the sub-occipital craniectomy was performed by the Stein's approach, and the tumor was removed. Pathologically the tumor was a typical yolk sac tumor. This case is a very interesting case because it suggests an alternation of the element of the germ cell tumor by the radiation therapy. At first admission, germinoma was the main element of the tumor judging from the effectiveness of the radiation therapy and laboratory studies. But the main element of the tumor seemed to have changed to yolk sac tumor after the radiation therapy. The relation between the tumor markers and the types of the germ cell tumor and histopathological characters of the intracranial germ cell tumor were discussed.

The authors report a rare case of tuberous sclerosis associated with pineal region mixed glioma. A 38-year-old woman with tuberous sclerosis, who had a past history of left nephrectomy and tumorectomy of the right kidney for bilateral renal angiomyolipomas, was admitted because of headache and ataxic gait. CT scan and cerebral angiography suggested a pineal region tumor. Suboccipital craniectomy and partial removal of the tumor was performed via infratentorial supracerebellar approach. Microscopic examination revealed mixed oligodendroglioma and astrocytoma. It is well known that tuberous sclerosis is occasionally associated with brain tumors. However, with few exceptions, these have been subependymal giant cell astrocytomas in the lateral ventricles. They seem to originate from subependymal nodules of hamartomatous nature. As far as we are aware, association of pineal region tumor with tuberous sclerosis as in this case has not been reported.

A pineocytoma in an old man, whose initial symptoms resembled a normal pressure hydrocephalus, is reported. This 67-year-old man gradually became uncommunicative and difficult to walk alone in three months. Just before visiting our clinic, his family also noticed his nocturnal urinary incontinence. CT scan on admission disclosed tumor in the posterior wall of the third ventricle, and subsequent hydrocephalus. This oval, isodense tumor was homogeneously enhanced after the injection of contrast medium on CT scan. The vertebral angiography showed a mass in the pineal region with no vascular staining. Ventricular drainage, the opening pressure of which was 100 mmH2O, could not offer cytological verification of the tumor. By the infratentorial supracerebellar approach, the tumor was successfully extirpated, and the post-operative course was uneventful. The microscopic study revealed the nature of this tumor to be well compatible with that of a pineocytoma.

A 44-year-old man came to our clinic, complaining of slowly progressive disturbance of visual acuity and of ocular movement. This patient suffered from headache, narrowing of visual field and polyuria about 20 years ago, and received surgical and radiation therapy under the diagnosis of pituitary adenoma. Clinical symptoms and signs of this patient, except for bitemporal hemianopsia, almost completely disappeared after these treatment. The detailed information about the histology and radiation dose are not available at the present time. CT scan in our clinic revealed a round low-density area at the suprasellar region and a high density area at the left quadrigeminal cistern. Pineal calcification was compressed to the right about 2-3 mm from midline. This high density mass were not enhanced with contrast medium. Vertebral angiography showed a slight lateral displacement of the left medial posterior choroidal artery. Specimen of tissue removed 20 years ago was reexamined but definitive diagnosis could not be established. Presumptive diagnosis of an ectopic pinealoma in the suprasellar region treated successfully 20 years ago, and its recurrence in the pineal region was made. On May 24, the patient underwent a posterior fossa craniectomy and the pineal region was explored via the infratentorial supracerebellar approach. On sectioning the precentral cerebellar vein, yellowish mass was seen in the quadrigeminal cistern. Aspiration of this mass yielded dark red liquid hematoma. Incising into the capsule, a dark brownish mass of about 4g was removed en bloc.(ABSTRACT TRUNCATED AT 250 WORDS)

A case of teratoma in the pineal region which recurred 4 years after the first tumor removal was reported in this paper. When the patient was 5 years old, she, complained of headache and vomiting, and visited our hospital. As a heterogeneous mass with no enhancement effect was found in the pineal region by CT scan, she was admitted on November 9, 1976. There was no abnormalities on physical examination but neurological examination revealed slight disturbance of conjugate upward gaze (Parinaud's sign). Left vertebral angiogram demonstrated posterior superior displacement of posterior choroidal artery and downward displacement of Rosenthal vein, but early venous filling and tumor stain were not seen. Under preoperative diagnosis of a teratoma in the pineal region, the first operation (left occipital craniotomy and total removal of the tumor) was performed on November 24, 1975. Microscopic examinations revealed that the removed tumor was a mature teratoma in the pineal region. Postoperative course was uneventful and discharged on December 20, 1975. The follow-up study was continued at outside clinic after discharge. There was no signs of recurrence until 3 years after the first operation, but on January, 1981 (4 years after the first operation), she suffered from severe headache and vomiting again and re-admitted to our hospital on February 3, 1981. There was no remarkable neurological deficits except for the mild intracranial hypertensive sign and no changes of findings on angiogram. But CT findings were markedly characteristic. It revealed a heterogenous mass with remarkable enhancement effect in the pineal region and ventricular enlargement. Because a mixed type (teratomatous and germinomatous) of pineal tumors was suspected from the CT findings, irradiation was done after V-P shunt. The tumor was reduced to half size after the first course of 2000 rads irradiation, but there is no more reduction of the size of the tumor following the second course of 2000 rads (total 4000 rads) irradiation. Against the residual tumor, tumor removal was performed on June 2, 1981. Microscopically, the most part of the resected tumor showed fibrous changes caused by irradiation and partially teratomatous compartment. From this result (radiosensitivity and histology) the authors assumed that the recurred tumor could be a mixed type (germinoma and teratoma) of pineal tumor. Postoperative course was uneventful except for a transient disturbance of conjugate upward gaze and she was discharged on June 25, 1981. And now, there is no signs of recurrence 12 months after the second operation. Conclusively, it will be stressed that we should continue follow-up study the case even after total removal of teratoma, especially in the pineal region. Moreover, it was considered that there is a possibility of the changes of the histological features on recurrence of the pineal teratoma. When germinomatous compartment is suspected, irradiation is the first choice and then microsurgical operation should be done against residual tumor.

A case of primary choriocarcinoma in the pineal region is reported. A six-year-old boy showing precocious puberty, suddenly developed coma by massive ventricular hemorrhage. He recovered gradually up to akinetic mutism, but whose clinical, hormonal and neuroradiological studies failed to demonstrate any pathological lesions. Four years later a small tumor was detected by CT in the pineal region. At the same time increased serum-HCG level was shown. Subtotal removal of the tumor was carried out by Stein's supracerebellar infratentorial approach. The pathology of the tumor showed the characteristics of choriocarcinoma, consisting of major necrotic area and of tumor cells, syncytiotrophoblast and cellular trophoblast. The former revealed strong activity of HCG by immunoperoxidase study. Postoperative chemotherapy with actinomycin-D and methotrexate was given for a month. In the course of these treatments, serum-HCG recovered within normal range. This patient shows no signs and symptoms of the recurrence 6 months after operation. Early diagnosis with aids of neuroradiology as well as hormonal studies and the early initiation of the treatment may prevent major complications like massive tumoral bleeding, also might achieve the favorable outcome of this tumor.

Detection of an intraventricular or intratumoral fat-fluid level on the plain craniograms has been known as a characteristic sign indicating the presence of intracranial teratomatous tumors. On CT scans, however, only thirteen cases have been previously reported to be found an intraventricular and/or subarachnoid free fat associated with spontaneous ruptures of these tumors. We reported a case of pineal teratoma with intraventricular free-fat seen on CT scans. A nine-year-old male with precocious puberty was admitted to our hospital complaining a moderate nonpulsatile headache. Neurological examinations were normal without signs of meningeal irritation. The serum and CSF titer of HCG were raised markedly. The laboratory data of the CSF were normal and there were no pathological cells in the CSF. The CT scans revealed a large heterogeneous mass containing multiple areas of negative density in the pineal region. There were negative density droplets in the bilateral frontal horn on the same CT scans indicating a presence of free fats. At surgery, an yellowish oily material was drained from the tumor, but there was no sign of meningitis over the cortical surface of the occipital lobe. An intraventricular free fat on CT scan have been reported in fourteen cases including ours following the first case described by Fawcitt in 1976. Although most of the cases presented headache, only two cases was diagnosed clinically as chemical meningitis. Pathological changes indicating granulomatous meningitis, however, were noted in five cases, all of them presenting seizure attacks.