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Paroxysmal tonic upgaze of childhood has been described as a benign distinctive syndrome of abnormal ocular movement, with or without concomitant ataxia. After the first observation of four children, a further 29 patients have been reported with a wide spectrum of neurologic abnormalities such as ataxia, unsteady of gait, learning disabilities and mental retardation at follow-up. Electroencephalograms were normal in all the subjects and magnetic resonance imaging showed deficient myelination in only one patient. Recently it has been suggested that paroxysmal tonic upgaze could be a heterogeneous syndrome, ranging from a simply age-dependent manifestation to a clinical appearance of a variety of disorders affecting the corticomesencephalic loop of vertical eye movement. Moreover, it also could be an early sign of more widespread neurologic dysfunction. We describe three patients who presented paroxysmal tonic upgaze; in one, ataxia was present; in the second child, ataxia and language disorder also were observed; and in the third patient paroxysmal tonic upgaze was associated with loss of muscle tone (drop-attack-like events). On magnetic resonance imaging, a pinealoma compressing the dorsal mesencephalic region was detected. On the basis of our observations, we suggest that any insult with periaqueductal mesencephalic gray-matter involvement could be considered the basic condition for this peculiar clinical manifestation.

A 19-year-old male presented with a 4-week history of headache. Neurological examination showed bilateral papilledema. Computed tomography revealed a pineal region mass with remarkable obstructive hydrocephalus. Magnetic resonance imaging showed a pineal region tumor continuously invading through the tectum into the cerebral aqueduct and the fourth ventricle with the preservation of the adjacent structures. The tumor appeared an iso- to hypointense mass on T1-weighted images, a heterogeneous iso- to hyperintense mass on T2-weighted images, and a heterogeneously enhanced mass after administration of contrast medium. Histological examination after endoscopic biopsy confirmed that the tumor was a pineoblastoma. Radiotherapy was given to the whole brain and the spinal cord, and magnetic resonance imaging showed complete remission of the tumor. Pineoblastomas are highly malignant tumors with seeding potential through the neighboring ventricle or along the meninges, and this type of tumor becomes larger with local extension. We found no previous reports of the continuous extension into the fourth ventricle. The present case showed ventricular extension with minimal mass effect to adjacent structures, and did not disturb ventricular configuration. According to the unusual finding of ventricular extension, this rare case of pineoblastoma requires adjuvant chemotherapy.

Tumours occurring in the region of the pineal gland are uncommon in paediatric patients but may manifest with typical signs and symptoms. A 6-year-old boy with precocious puberty caused by a germinoma of the pineal gland detected on skull radiographs and confirmed on MR imaging is reported. The different types of tumours occurring in the region of the pineal gland and their imaging findings are discussed.

Object: To determine the efficacy and accuracy of surgically-assisted systems including endoscopy combined with neuronavigation in the treatment of pineal region tumours through the occipital transtentorial approach, an evaluation of thirty-one patients undergoing surgery was performed over a 10-year period., Method: The study was performed in 2 parts. The surgical approach to the pineal region was the same in the two parts, but in part 2 a smaller craniotomy window was used. Part 1 (from March 1989 to March 1997) included 15 patients who underwent surgical removal of pineal region tumours without using assisted systems; four out of the fifteen patients had surgery-related complications, including seizure and hemianopsia. Part 2 (from April 1997 to February 1999) included 16 patients who underwent surgical treatment by the same surgical team and with assisted systems; all 16 patients had excellent outcomes, with no complications., Conclusions: Although this study was the first specifically to examine the efficacy of endoscopy combined with neuronavigation in the treatment of pineal region tumours, our findings suggest that these systems are very useful, safe, and accurate in evaluating the primary tumour and surrounding anatomy as well as in determining operative strategy, such as the location and size of the scalp incision, craniotomy, and the extent of surgical removal. Therefore, we conclude that the addition of endoscopy combined with neuronavigation to standard surgical procedures can improve the outcome of surgical treatment of pineal region tumours.

A 14-year-old boy presented with an extraneural metastasis of germinoma with syncytiotrophoblastic giant cells (STGC). The tumor was located in the pineal area, suprasellar cistern, and frontal base, and associated with the slight elevation of the beta-human chorionic gonadotropin (HCG) serum level. Following radiation therapy, the tumor completely disappeared, and the beta-HCG level decreased to normal. Two years after the initial treatment, metastasis of the tumor to the left femur was observed without recurrence of the primary lesion. The biopsy specimen revealed germinoma, the diagnosis was germinoma with STGC because of the associated elevation of HCG level. The metastatic tumor was treated by chemotherapy with cisplatin and etoposide, and the tumor completely disappeared. With adjuvant chemotherapy, he has been recurrence-free for 3 years. Extraneural metastasis occurs more frequently from germinoma with STGC than germinoma. Aggressive chemotherapy combined with radiation therapy in the initial treatment might have to be tried to avoid metastasis from germinoma with STGC.

The primary aim of this study is to report the long-term outcome of pineal and suprasellar germinoma after stereotactic biopsy and whole brain radiotherapy. The second purpose is to report an investigation of the biological features and cell kinetics of this peculiar and enigmatic brain tumour. Of 34 supratentorial germ cell tumours diagnosed and treated between 1980 and 1993, 20 patients were found to be affected by true germinoma localized in the pineal and/or suprasellar regions. The diagnosis was achieved by stereotactic biopsy in all cases. In 14 patients, the potential proliferative activity of the tumour was investigated by (3H)thymidine in vitro binding and labelling index determination. Chorionic gonadotropin, alpha-fetoprotein and embryonal carcinoma antigen were negative in the cerebrospinal fluid of these patients. All but 1 patient underwent whole brain radiotherapy. Clinical and neuroradiological follow-up ranged between 3 and 13 years (mean 8). Complete clinical and neuroradiological recovery was achieved in all patients after treatment. Fatal recurrences owing to neuraxis dissemination occurred in three cases. The labelling index in the whole series ranged between 0.1 and 5% (median 2.5). Only syncytiotrophoblastic cells had proliferative activity, while none of the lymphoid-like cells showed thymidine labelling.

Primary intracranial germ cell tumors are rare neurological diseases. Secreting germ cell tumors that predominantly appear in the pineal region are characterized by elevated levels of beta-HCG and AFP in CSF. Adequate diagnostic and therapeutic regimens of these tumors are still under debate. We present the case of a patient suffering from spinal metastasis of a secreting germ cell tumor with raised levels of beta-HCG in CSF. Six years before, a pineocytoma of intermediate differentiation had been diagnosed by stereotaxic biopsy. This demonstrates the importance of an early detection of a germ cell neoplasm in case of midline-sized brain-tumors by evaluation of beta-HCG and AFP in CSF and serum. Today, different therapeutic strategies depending on the histological diagnosis are available and justify a high priority of a surgical tumor biopsy. However, the value of stereotaxic procedures remains controversial. Recently published data gave evidence of a definite improved prognosis of secreting CNS germ cell tumors following combined treatment with high-dose polychemotherapy, surgical resection and radiation of the tumor area. beta-HCG and AFP may serve as useful markers to enable follow-up of the disease.

Background: A certain diagnosis, as soon as possible, is indispensable in children with intraocular lesions, the presence of retinoblastoma always being a possibility., Patients: From our casuistic of 418 children since 1970 and from the literature, we currently adopt the following attitude. Where the media are clear, a drawing and fundus photographs are made. Echography is performed in all cases. Ultrasound biomicroscopic examination is carried out in all lesions where involvement of the anterior segment is suspected., Results: Where echography shows no calcifications, computed tomography (CT) is indicated. CT may also enable a possible lesion extension, particularly to the optic nerve, to be studied., Conclusions: Magnetic resonance imaging (RMI) is the procedure of choice for evaluating secondary retinal detachment, massive extension of retinoblastoma and for detecting any early involvement of the pineal gland (pinealoblastoma), or any other associated lesion. In case of unilateral unifocal sporadic retinoblastoma, however, no CT or MRI is performed, except where there is a suspected extension into the optic nerve or the orbit. Echography remains the most economical, rapid and safest means of diagnosis even in difficult cases.

The results are reported of a retrospective review of the presentation and outcome of 43 pineal region tumors treated from 1982 to 1996, including 20 identified tumors: 5 germinomas, 8 teratomas, 2 embryonal carcinomas, 1 endodermal sinus tumor, 2 pineocytomas and 2 pineoblastomas. Of the 43 tumors reviewed, 36 were located in the pineal region, 5 in the suprasellar, and 2 in both the pineal and suprasellar regions. Twenty patients underwent surgical resection: total in 6 and partial in 10, while only a biopsy was taken in 4 cases. Fifteen patients were managed on the basis of serum CSF tumor markers and radiation response. Twenty-three patients with germinomas received radiotherapy (RT) and had a 5-year survival rate of 87%. Fifteen patients with non-germinomatous germ cell tumors received RT and chemotherapy following direct surgery, and 5 died (mortality rate of 33.3%). The overall survival rate of the 43 patients with pineal tumors was 79.1% (34/43) and the death rate was 20.9% (9/43). It is now recognized that the wide variety of tumor types found in the pineal region necessitates different modes of treatment, and improved microsurgical and stereotactic surgical techniques have made mortality and morbidity rates acceptably low. Because the radiation response and CSF cytology are not enough to determine optimum treatment, a tissue diagnosis should be obtained in all patients.

A 58-year-old male suffered a massive intratumoral hemorrhage after placement of a ventriculoperitoneal shunt before treatment for pineocytoma. At admission, he complained of headache, urinary incontinence, gait disturbance, and recent memory disturbance. His neurological examination revealed impaired memory and papilledema from hydrocephalus. Two days after the placement of the ventriculoperitoneal shunt, he suddenly became stuporous and Parinaud's sign appeared. Computed tomography showed a massive intratumoral hemorrhage occupying the third ventricle which had extended into the lateral ventricle. The infratentorial supracerebellar approach was used to remove the hematoma and the tumor. The histological diagnosis was pineocytoma. Intratumoral hemorrhage in pineocytoma after the placement of a ventriculoperitoneal shunt is rare, but should be kept in mind when a ventriculoperitoneal shunt is placed before a craniotomy.

Germinomas arising within the sella turcica are extremely rare. The association of intrasellar and a pineal region tumours is even more unusual. We report a 30-year-old man with germinomas in the sellar and pineal region.

Bilateral superior oblique palsy is an uncommon ocular motility problem, the commonest cause being closed head trauma. Two cases, both adults, are presented in whom bilateral superior oblique palsy occurred as a result of neoplastic infiltration of the dorsal midbrain in the region of the anterior medullary velum. In the absence of a history of head trauma, the presence of an acquired bilateral superior oblique palsy is a definite sign of a single lesion in the region of the decussation of the trochlear nerves and appropriate imaging is indicated.

A patient with a synchronous pineal and suprasellar germinoma and symptomatic hydrocephalus underwent a minimally invasive endoscopic biopsy and third ventriculostomy. We performed multiple biopsies of both tumors and fenestration of the floor of the third ventricle with a flexible fiberoptic endoscope through a single burr hole. Postoperative quantitative cine-mode magnetic resonance imaging (MRI) confirmed the patency of the fenestration. Selected pineal/posterior third ventricle region tumors, especially radiosensitive germinomas, could be treated with this approach thereby obviating the need for either stereotactic or craniotomy/open biopsy and the subsequent placement of a permanent cerebrospinal fluid (CSF) shunt. The endoscopic approach to tumors in the pineal/posterior third ventricle region provides an accurate straightforward, and safe method of obtaining a diagnosis and treating the associated increased intracranial pressure.

Purpose: The aim of this survey was to review the different studies regarding the occurrence of second primary tumours (SPT) among survivors of retinoblastoma., Methods: Ovid (Medline, Current contents life, Psychlit, Embase) was searched for the years 1966-1995 using the mesh headings: 'retinoblastoma', 'second primary neoplasms', and 'multiple primary neoplasms'. The inclusion criteria were: the study should involve 50 patients or more and should not be limited to one specific SPT. A checklist with criteria regarding the study design and the results was applied to each study., Results: Eleven studies were identified which met the inclusion criteria. Thirty-five different types of SPT (Ntotal = 243) were reported. Most of them were osteosarcomas (37.0%), followed by melanomas (7.4%), soft-tissue sarcomas (6.9%), brain tumors (4.5%), fibrosarcomas (3.3%), chondrosarcomas (3.3%), and sarcomas (3.3%). Less frequently reported were leukemias (2:4%), sebaceous cell carcinomas (1.6%), and non-Hodgkin lymphomas (1.6%). Pineoblastoma, which in fact is a trilateral retinoblastoma and not an SPT, was found in 2.4%. Despite the differences, all 11 studies showed a higher incidence of SPT compared to the general population. Only 4 studies were judged to be free from selection bias, reporting a cumulative incidence of SPT of 8.4% 18 years after diagnosis, 15.7% at the age of 20 years, 19% at the age of 35 years, and a relative risk of 15.4 for SPT, respectively., Conclusion: SPT is a serious problem for the survivors of hereditary retinoblastoma and its importance should be recognized in (genetic) counseling of patients.

The authors analyze otoneurological symptoms in 120 patients with pineal tumors. A marked cochleovestibular syndrome was found characterized by bilateral neurosensory hypoacusis with vocal-tonal dissociation, head noises, vertigo, multiple spontaneous nystagmus including a converging one, impaired optokinetic nystagmus, enhanced vestibulovegetative reactions. 39 cases are reported with pineal tumors removed microsurgically. Otoneurological symptoms were followed up in 32 patients early after surgery (within 20 postoperative days) and in 6 patients 1 to 5 years after radiotherapy. As evident from otoneurological examination, electronystagmography, tonal and speech audiometry, positive postoperative changes in auditory and vestibular functions occurred in most of the patients. The response persisted long in 5 patients.

In the Pediatric Neurosurgical Service of the Veterans General Hospital, Taipei, flexible endoscopic III ventriculostomy has been applied in children with noncommunicating hydrocephalus and periaqueductal/pineal tumor. We modified the technique to allow enlargement of the opening of the III ventricular floor. The use of an Atlas Wire Stone Extractor for this purpose is described.

The paper presents the follow-up data on 39 patients who underwent microsurgical removal of pineal tumors. Computerized and magnetic resonance tomographies were performed prior to and following the removal of pineal tumor. Otoneurological symptoms were analyzed in their dynamics in 32 patients in the early postoperative period (up to 20 days) and in 6 patients in the late period (1 to 5 years) after radiation therapy. Seven patients died from intracranial complications in the early postoperative period. Acoustic and vestibular functions were evaluated from otoneurological, electronystagmographic, pure-tone and verbal audiometric findings. The postoperative positive dynamics of acoustic and vestibular functions was observed in most patients. A steady-state positive effect also remained in 5 patients in the long-term period. The paper gives 2 cases with clear-cut positive cochlear dynamics after removing pineal tumors.

The author has treated 30 cases of pinealoma by the infratentorial supracerebellar approach with the patient in the sitting position. Long-term outcome has been excellent in 26 cases, poor in 1, and death in 3 cases. Postoperative deaths were due to: (i) high venous pressure in the jugular vein caused by a tourniquet placed around the neck to prevent air-embolism, making hemostasis more difficulty, (ii) the absence of retraction resulting in a narrow surgical field when acute brain swelling developed, and (iii) high coagulation and ablation of the precentral cerebellar vein causing diffusion thrombosis of deep veins. The details of these cases are presented in this article.

A variety of tumours may arise in the pineal region; accurate diagnosis is important in the selection of treatment and prognosis. A retrospective analysis of the MRI studies of 25 patients with pathologically proven pineal region tumours was performed, focused on the relationship between the tumour and neighbouring structures. Compression of the tectal plate was classified as expansive or invasive, and compression of the corpus callosum as inferior, anterior or posterior. In 10 of the 14 patients (71%) with germ cell tumours tectal compression was of the invasive type; 8 patients (57%) had multiple tumours and in 13 (93%) the tumor margins were irregular. Teratomas were readily diagnosed because of characteristic heterogeneous signal intensity. Pineal cell tumours were differentiated from germ cell tumours by their rounded shape, solid nature, sharp margins, and expansive type of tectal compression. Meningiomas were characterised by their falcotentorial attachments, posterior callosal compression, and a low-intensity rim on T2-weighted images. Gd-DTPA injection enabled clear demonstration of the site and extent of tumour spread and was useful in differentiating cystic and solid components. The appearances described, while not pathognomonic, are helpful in the differential diagnosis of pineal region tumours, and valuable in planning appropriate treatment.

Background: Stereotactic biopsy followed by cytoreductive surgery and/or radiation are the standard treatment for pineoblastoma. We are reporting a patient with a relatively large pineoblastoma, who was treated with a combination of nonresective treatment modalities., Method: The patient is a 20-year-old woman who presented with signs and symptoms of raised intracranial pressure. Magnetic resonance imaging (MRI) showed a large tumor in the pineal region and hydrocephalus. She was emergently treated by placement of a ventriculoperitoneal shunt. Three days later she underwent a stereotactic biopsy, which showed the tumor to be pineoblastoma. She was then further treated with the following treatment modalities: permanent implantation of high activity I125 seeds, external radiation, and chemotherapy., Results: At 10 months' follow-up, MRI showed complete disappearance of the tumor. At 18 months' follow-up, the patient is asymptomatic, neurologically intact, and her MRI scans show no evidence of intracranial or intraspinal tumor., Conclusions: Though the follow-up is relatively short, the results of the nonresective approach in this patient have been excellent.

MR showed an enhancing mass in the pineal region and hydrocephalus and leptomeningeal enhancement, thought to indicate pinealoblastoma with leptomeningeal spread. During resection there was no evidence of spread, and repeat MR showed no residual tumor or meningeal enhancement, so the patient was not treated for metastasis. Because there were no signs of leptomeningeal tumor 4 months after surgery, the meningeal enhancement is thought to have been related to venous stasis secondary to obstructive hydrocephalus.

This is the first report of a series of adults (> 16 years of age) with pineoblastomas who had their entire neuraxis staged at the time of diagnosis. Between 1975 and 1992, seven men and four women with histologically proven pineoblastomas were evaluated at the University of California, San Francisco. The median age at diagnosis was 36 years (range, 17-59 yr). All patients presented with symptomatic hydrocephalus. One patient had a complete surgical resection, eight had subtotal resections, and two had biopsies only. One patient refused any treatment or follow-up review and died 6 months after diagnosis. The five patients with positively staged disease had progression either focally or in the spine 8 to 49 months (median, 10 mo) after initial diagnosis and died 1 to 20 months after recurrence; the median overall survival time from the date of surgery was 30 months. In contrast, all five patients with negatively staged disease were alive without disease progression after a median of 26 months of follow-up. Our retrospective review shows that the extent of disease at diagnosis seems to be an important prognostic factor for pineoblastomas, as is true for medulloblastomas and other primitive neuroectodermal tumors. Initial staging should include examination of the cerebrospinal fluid and magnetic resonance imaging of the spine. Although patients with pineoblastomas are often treated with adjuvant systemic chemotherapy after craniospinal irradiation, the benefits of this approach are unclear.

Objective: Our goal was to characterize the CT, MR, and pathologic features of pineal parenchymal tumors (PPTs)., Materials and Methods: We retrospectively reviewed 19 cases of resected PPTs with pathologic confirmation [10 pineocytomas (PCs), 1 mixed pineocytoma-pineoblastoma (PC-PB), and 8 pineoblastomas (PBs)], noting clinical, CT, and MRI features., Results: Six male and four female patients presented with PC, one female patient with mixed PC-PB, and seven female and one male patient with PB. The CT imaging revealed 6 of 10 round and 6 of 10 small- to medium-sized masses; 7 of 9 were homogeneous, 1 of which appeared cystic. The mixed PC-PB was round and homogeneous. Eight precontrast CT scans of PCs and the PC-PB demonstrated calcifications, either peripheral (four of eight) or central (four of eight, including the mixed tumor). Pineocytomas were hypodense (five of seven) tumors, with heterogeneous postcontrast enhancement (five of seven). Most PBs (six of eight) were lobulated, and five of eight were homogeneous. Four of eight PBs imaged with CT were noncalcified hyperdense masses, which enhanced homogeneously. Pineocytomas and the PC-PB had variable signal intensity on MRI. Most PBs were hypo- to isointense on T1-weighted images, and six of six enhanced heterogeneously postgadolinium., Conclusion: Pineocytomas are predominantly smaller, round, hypodense, homogeneous masses with calcifications, particularly peripheral. They enhance heterogeneously on CT and present with a lesser degree of hydrocephalus in older patients. Pineoblastomas, on the other hand, are larger, lobulated, homogeneous tumors, are infrequently calcified, and present with a greater degree of hydrocephalus, often in young females. They tend to be hyperdense on CT and hypo- to isointense on T1-weighted images and enhance homogeneously on CT and heterogeneously on MRI.

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)

We reviewed MRI findings in proven intracranial germ cell tumours in 22 cases, 12 of whom received Gd-DTPA. On T1-weighted images, the signal intensity of the tumour parenchyma was moderately low in 19 cases and isointense in 3; on T2-weighted images, it was high in all cases. Regions of different intensity thought to be cysts were found in 17 (77%): 7 of 12 patients with germinoma (58%) and in all other cases. Of the 13 patients with pineal lesions T1-weighted sagittal images showed the aqueduct to be obstructed in 5, stenotic in 7 and normal in 1. Strong contrast enhancement was observed in all 12 cases. Of the 14 patients with suprasellar lesions, 5 were found to have an intrasellar extension, and in 3 of these, the normal pituitary gland, which could be distinguished from the tumour, was displaced anteriorly. Ten patients (45%) had multiple lesions.

Pineoblastomas, which originate from the pineal gland, are extremely malignant tumors that grow quickly and aggressively. Because they spread so fast and the probability of longterm survival is poor, it is important to diagnose these tumors early. This article discusses magnetic resonance's role in imaging pineoblastomas. MR is particularly effective for this task due to its ability to visualize the pineoblastoma in three different planes, both before and after the administration of gadolinium.

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 pineal mass in a patient with acquired immunodeficiency syndrome (AIDS) is reported. Computed tomography (CT) scan revealed a nodular mass in the pineal region with foci of calcification and obstruction of the aqueduct mimicking a pineal tumor. At autopsy, the brain revealed a well-circumscribed lesion with central necrosis in the pineal region suggestive of toxoplasma and involving the periaqueductal area. Susceptibility of a patient with AIDS to opportunistic infections should be considered.

Small asymptomatic cysts of the pineal gland represent a common incidental finding in adults undergoing computerized tomography or magnetic resonance (MR) imaging or at postmortem examination. In contrast, large symptomatic pineal cysts are rare, being limited to individual case reports or small series. The authors have reviewed 24 cases of large pineal cysts. The mean patient age at presentation was 28.7 years (range 15 to 46 years); 18 were female and six male. Presenting features in 20 symptomatic cases included: headache in 19; nausea and/or vomiting in seven; papilledema in five; visual disturbances in five (diplopia in three, "blurred vision" in two, and unilateral partial oculomotor nerve palsy in one); Parinaud's syndrome in two; hemiparesis in one; hemisensory aberration in one; and seizures in one. Four lesions were discovered incidentally. Magnetic resonance imaging typically demonstrated a 0.8- to 3.0-cm diameter mass (mean 1.7 cm) with homogeneous decreased signal intensity on T1-weighted images, increased signal intensity on T2-weighted images, and a distinct margin. Hydrocephalus was present in eight cases. The cysts were surgically excised via an infratentorial/supracerebellar approach (23 cases) or stereotactically biopsied (one case). Histological examination revealed a cyst wall 0.5 to 2.0 mm thick comprised of three layers: an outer fibrous layer, a middle layer of pineal parenchymal cells with variable calcification, and an inner layer of hypocellular glial tissue often exhibiting Rosenthal fibers and/or granular bodies. Evidence of prior hemorrhage, mild astrocytic degenerative atypia, and disorganization of pineal parenchyma were often present. Postoperative follow-up review in all 24 cases (range 3 months to 10 years) revealed no complications in 21, mild ocular movement deficit in one, gradually resolving Parinaud's syndrome in one, and radiographic evidence of a postoperative venous infarct of the superior cerebellum with ataxia of 1 week's duration in one. Of the patients referred for study, the cysts were most often initially misdiagnosed as a pineocytoma in eight and a pilocytic astrocytoma in three. Only two patients were correctly diagnosed as having pineal cysts. This stresses the importance of recognizing the histopathological spectrum of pineal cysts, as well as correlation with radiographic findings, if a correct diagnosis is to be attained.

Purpose: To describe the spectrum of MR and CT findings in clinically symptomatic pineal cysts and to determine whether there are certain diagnostic imaging features that allow one to distinguish a benign pineal cyst from other neoplasms of the pineal region., Methods: MR and CT scans of 19 patients with clinically symptomatic pineal cysts were retrospectively reviewed. Age range was 15 to 46 years with a mean age of 28 years. There were five male and 14 female patients., Results: Presenting features included headache (15 patients), diplopia (four), nausea and vomiting (four), papilledema (four), seizure (three), Parinaud syndrome (two), ataxia (one), and hemiparesis (one). All cysts were resected or biopsied to provide histopathologic confirmation of the diagnosis. Preoperative diagnoses included pineal neoplasm (14 of 19), pineal cyst (3 of 19), and dermoid cyst (2 of 19). The lesions ranged from 0.8 to 3.0 cm, with a mean diameter of 1.6 cm. Three cysts showed fluid/fluid levels consistent with hemorrhage. Slightly less than half (9 of 19) had evidence of hydrocephalus. The MR signal changes were variable but typically demonstrated low signal on T1-weighted images and high signal on T2-weighted images. More than half (7 of 12) demonstrated enhancement with gadolinium. Calcification of the cyst wall was observed in only four of nine patients who had CT studies but identified histologically in all cases., Conclusion: The MR appearance of benign pineal cysts is variable, ranging from that of an uncomplicated cystic mass to a mass associated with hemorrhage, enhancement, or hydrocephalus. This variability may make them indistinguishable from other pineal-region tumors.

Of 440 consecutive children with retinoblastoma whom we treated between April 1972 and April 1992, 13 (3%) developed a midline intracranial neoplasm. Four of these 13 patients had bilateral familial retinoblastoma, 7 had bilateral sporadic retinoblastoma, and 1 patient had unilateral familial retinoblastoma. The twin brother of the latter patient developed pinealoblastoma without retinoblastoma. In this series, the incidence of trilateral retinoblastoma was 8% of all bilateral familial retinoblastoma and 5% of all bilateral sporadic retinoblastoma. The retinoblastoma was diagnosed at a mean age of 4.5 months and the midline intracranial tumor was diagnosed at a mean age of 23 months. The mean time interval between the diagnosis of retinoblastoma and the intracranial tumor was 22 months (range, 0 to 48 months). Seven patients (55%) were asymptomatic and their intracranial neoplasm was found on routine brain-imaging studies. The primary intracranial neoplasm had a pineal location in 10 cases and parasellar location in three cases. Despite aggressive combined treatment, 12 patients (92%) have died at a mean interval of 11 months after the diagnosis of intracranial malignancy. The mean survival of patients with midline intracranial neoplasm who were asymptomatic was significantly longer than that of symptomatic patients (P = .05).(ABSTRACT TRUNCATED AT 250 WORDS)

A 58-year-old female with pineocytoma developed intratumoral hemorrhage after ventriculoperitoneal shunting for hydrocephalus. Neurological examination revealed Parinaud's sign and papilledema. Computed tomography and magnetic resonance (MR) imaging revealed a pineal neoplasm and obstructive hydrocephalus. Serum and cerebrospinal fluid levels of human chorionic gonadotropin, alpha-fetoprotein, carcinoembryonic antigen, and placental alkaline phosphatase were negative or within normal limits. MR images after the ventriculoperitoneal shunting disclosed intratumoral hemorrhage and markedly smaller ventricles. The tumor was totally removed via the occipital transtentorial approach and was diagnosed histologically as a pineocytoma with astrocytic differentiation. The tumor probably shifted away from the surrounding structures following the marked reduction in ventricular size after ventriculoperitoneal shunting, resulting in changed venous circulation in the tumor and the formation of intratumoral hematoma.

Evaluation of 158 patients younger than 25 years-of-age who had been hospitalized in a specialized adult endocrine department during an 11 year-period, brought to light specific endocrine problems connected both to pediatrics and internal medicine. There is need for close collaboration between pediatricians, as experts on disturbances in growth and pubertal development, and internists, with experience of diagnosing rare endocrinological disorders. Such collaboration might also improve the follow-up of young patients into adulthood.

Review of 500 consecutive MRI studies was undertaken to assess the frequency and the appearances of cystic pineal glands. Cysts were encountered in 2.4% of cases. Follow-up examination demonstrated no change in these cysts and they were considered to be a normal variant. Size, MRI appearances and signs associated with this condition are reported in order to establish criteria of normality.

A 24-year-old male presented with a metastatic germinoma in the thoracic vertebra 7 years after irradiation of a pineal germinoma. Combination chemotherapy with cisplatin and etoposide was highly effective. No recurrence has appeared in the 2 years since chemotherapy.

The cytogenetic findings based on G-banding in a pineocytoma detected in a 29-year-old woman are reported. The chromosomal study showed numerical alterations involving chromosomes X, 5, 8, 11, 14, and 22, structural alterations of chromosomes 1, 3, 12, and 22, as well as various markers. Tumors of the pineal region are infrequent, and this is the first report of a pineocytoma studied cytogenetically.

Tissue diagnosis is necessary for optimal treatment of pineal region tumors in children. Preoperative staging should include craniospinal MR imaging with and without gadolinium DTPA enhancement, CSF sampling for cytology, and measurement of biologic tumor markers in serum and CSF. Surgical approach is determined by results of preoperative MR imaging and the extent of resection by the results of staging and intraoperative frozen-section histopathologic evaluation. There is no longer a role for the radiation test dose (2000 cGy) in the management of these tumors. Postoperative treatment is based on histopathology and extent of disease. Benign tumors are treated with surgery only, and nondisseminating focal tumors with surgery and focal radiation therapy. Non-germinoma malignant germ cell tumors are best treated with neoadjuvant chemotherapy followed by radiation therapy given focally for focal disease; craniospinal radiation therapy is reserved for patients with evidence of disseminated disease at the completion of induction chemotherapy.

Two cases of intracranial germinoma with different clinical expression, are described. The clinical symptomatology, the diagnosis and the treatment of this tumor are discussed. The symptoms depend on the localization of the tumor: in the suprasellar germinoma endocrinologic manifestations prevail while the symptoms in germinomas which are located in the pineal region, are mainly due to increased intracranial pressure. The diagnosis is suggested by the findings on CT-scan and MRI of the brain, but for the definitive diagnosis, pathologic examination of the tumor remains necessary. Blood HCG and alpha-fetoprotein are useful markers for follow-up; the value of angiotensin converting enzyme (ACE) as a marker, is still unclear. The ideal treatment of germinoma consists of surgical removal, postoperative chemotherapy and, afterwards, local radiotherapy. On the whole, the prognosis of this tumor is good.

An extremely rare case of a left cerebello-pontine angle (CP angle) pineoblastoma has been reported. The patient was a 32-year-old male whose initial manifestations were those of increased intracranial pressure. CT scan showed a large enhancing mass located at the left CP angle, associated with a moderate occlusive hydrocephalus. Left suboccipital craniectomy was performed. The mass was an extramedullary tumor which had compressed the left cerebellar hemisphere, and was easily separable from the adjacent tissue. The tumor was totally resected, and the patient had a temporary release from the symptoms. Recurrence and spinal dissemination were found within the ensuing few months. The tumor had invaded deeply through the left CP angle into the cerebellar parenchyme, and showed no anatomical connection with the pineal body. The tumor dissemination was also observed widely in the spinal subarachnoid space. No abnormalities at the pineal region were able to be confirmed using CT and MRI studies. Irradiation to the whole brain, to the localized left CP angle and to the spinal cord with additional chemotherapy was given. The patient died half a year after the first operation. Autopsy was not performed. Histopathologically, the tumor was delineated into lobular structures by reticulin fibrils and vimentin-positive interstitial tissue. Tumor cells were small in size, and had irregularly shaped hyperchromatic nuclei with increased mitotic figures, and formed various types of rosettes; pineocytomatous, Flexner-Wintersteiner, Homer-Wright and perivascular. Fine argyrophilic cell processes with club-shaped expansions were demonstrated inside the pineocytomatous rosettes.(ABSTRACT TRUNCATED AT 250 WORDS)

Clinicopathological evaluation of pineocytoma was performed in 4 patients. The subjects, 2 males and 2 females, ranged in age from 17 to 40. All the patients were clinically found to have the symptom of increased intracranial pressure on a monthly basis, but none of them were found to have dorsal midbrain dysfunction symptoms such as Parinaud's sign or Argyll Robertson pupil. Diagnostic imaging produced heterogeneous pictures indicating calcifications and cyst in 2 patients and homogeneous pictures of the tumor parenchyma in the other 2 patients. Histologically, the former cases were found to have many pineal-sand-like calcifications. Median sagittal MR images demonstrated expansive growth of pineocytoma. Quadrigeminal plates which kept their shapes were observed in 2 patients. Craniotomy was performed in all cases, removing the tumor totally in 2 patients. Radiation therapy was given to 3 patients, resulting in complete remission, but radiosensitivity varied according to cases. During the follow-up period of 12 to 42 months, one patient died of peritonitis caused by shunt infection. No recurrence of the tumor was seen in any of the patients. The incidence of pineocytoma was very low. Further evaluation of the tumor involving many cases is advisable.

Pineocytoma and pineoblastoma, originating from pineal parenchyma, are rare and usually solid. An unusual case of totally cystic pineocytoma in a 37-year-old female is reported. The tumor showed neuronal differentiation and had a good outcome. Prominent calcification associated with pineocytoma and pineoblastoma is an useful finding to differentiate these from benign pineal cysts.