A Case Report of Adult Pineoblastoma Occurring in a Pregnant Woman.

BACKGROUND/AIM: Pineoblastoma of the adult age is an uncommon tumor with only 200 cases reported. A standardized approach for an optimal adjuvant strategy is currently lacking. The case presented herein also deals with the issue of central nervous system tumors in pregnancy. CASE REPORT: A 21-year-old pregnant woman presented with massive hydrocephalus due to a mass in the pineal region detected with MRI. After positioning an urgent ventricular derivation, a cesarean section was performed. During a third ventriculocisternostomy, a biopsy revealed a pineoblastoma. After a maximal safe resection, postoperative craniospinal irradiation for a total dose of 36 Gy plus a sequential boost to the tumor bed to 54 Gy, and adjuvant chemotherapy with CDDP plus CCNU plus vincristine were performed. After one year, the patient is alive with no evidence of disease. CONCLUSION: The use of adjuvant radio-chemotherapy provided excellent outcomes in our case. The advanced gestational age facilitated the choice of the therapeutic strategy.

Pineal Cyst-Related Aqueductal Stenosis as Cause of Intractable Headaches in Nonhydrocephalic Patients.

BACKGROUND: Pineal cysts (PCs) are histologically benign lesions of the pineal gland. Although the majority of PCs are asymptomatic, some cases are ambiguous and accompanied by nonspecific symptoms of variable severity. We suggested that disabling headache in nonhydrocephalic patients with PCs is associated with cerebral aqueduct (CAq) stenosis. METHODS: A retrospective analysis was conducted in patients with PCs suffering from headache without secondary hydrocephalus who underwent surgical resection at Burdenko Neurosurgery Center between 1995 and 2016. All available medical records and radiographic images were retrospectively assessed in these patients. The comparison groups included 22 patients with nonoperated PCs and 25 healthy individuals. Specific magnetic resonance imaging measures were selected to assess the morphometry of the CAq and degree of the stenosis. RESULTS: In 25 patients (82%) we observed clinical improvement after surgery in a follow-up period. Among those with improvement, 10 of them (40%) experienced total relief and 15 of them (60%) had marked headache diminishment. In 5 patients the headache remained persistent. The preoperative rostral CAq diameter appeared to be significantly narrower (P = 0.0011045), and the preoperative rostral/caudal diameter ratio (Rd/Cd) was found to be lower (P = 0.004391) in patients who recovered from headache versus those who did not. CONCLUSION: The results indicate a statistically significant relationship between the changes in the CAq morphometrics and the clinical outcome in postoperative period. Surgical removal of symptomatic pineal cysts in patients without hydrocephalus can be considered as an effective treatment. However, a thorough preoperative examination and patient selection should be conducted in every case.

A 45-year-old woman with reversible bilateral hearing loss.

A 45-year-old woman complained of a progressive 2-month history of bilateral hearing impairment and diplopia on upward gaze. She had a history of a recurrent pineal region ganglioglioma with repeated tumor excision, adjuvant radiotherapy, and a ventriculo-peritoneal shunt performed 12 years prior. Subsequent imaging studies 6 years ago showed a pineal region cyst with progressive increase in size and a Rickham reservoir (Codman; Johnson & Johnson, Raynham, MA) was placed for percutaneous cyst fluid aspiration. The size of the cystic lesion remained static upon follow-up CT scans for several years.

Visual evoked potentials in patients with pineal gland cyst.

AIMS: The functional effect of the pineal gland cyst is difficult to evaluate with visual field examination. The aim of this study is to investigate the usefulness of visual evoked potentials (VEP) in patients with pineal gland cyst due to the possible compression on the visual pathway. SUBJECTS AND METHODS: Black-and-white pattern-reversal checkerboard VEP were recorded in 75 patients (50 females and 25 males, mean age 26.3 ± 15.7 and 25.6 ± 17.6 years, respectively) with pineal gland cyst detected on magnetic resonance of the brain (subject group) and 75 age and sex-matched control subjects (control group). Amplitudes and P100 latencies were collected and later grouped as: (1) normal finding; (2) prechiasmal; (3) prechiasmal and postchiasmal; and (4) postchiasmal dysfunction. RESULTS: P100 latencies differed significantly between subject (110.26 ± 13.23 ms) and control group (101.01 ± 5.36 ms) (p < 0.01). Findings of the VEP differed significantly (p < 0.01) between subject and control group, mainly due to the postchiasmal dysfunction frequency in subject group. Findings of the VEP differed significantly according to the pineal gland cyst volume (p = 0.006) with more frequent postchiasmal dysfunctions among subjects with larger cysts. Postchiasmal changes were significantly more frequent in patients with described compression of the cyst on surrounding brain structures (p = 0.016). CONCLUSIONS: Postchiasmal dysfunction on VEP can be seen in patients with pineal gland cyst, mostly with larger cysts and with compression of the cyst on surrounding brain structures. VEP serve as a useful method to determine functional impairment of the visual pathway in patients with pineal gland cyst.

[Videooculography in evaluation of oculomotor function in patients with mass lesions of pineal region, midbrain and pons].

Aim of this study was to assess feasibility of videooculography in evaluation of oculomotor function and its dynamics in patients with mass lesions of pineal region, midbrain and pons. Computed videooculography was performed in 48 patients with tumors of pineal region and midbrain, 9 patients with pontine hematomas and 10 healthy volunteers. 25 patients with midbrain and pineal neoplasms were followed after tumor resection or open biopsy. We evaluated amplitude of eye movements in normal and impaired oculomotor function. 4 grades decreases of amplitude upwards, downwards, medially and laterally were emphasized. In early postoperative period statistically significant decrease of amplitude in patients with clinical deterioration was observed. Application of videooculography allows reliable quantitative assessment of oculomotor function, registration and storage of examination data and follow-up of oculomotor function for observation of tendencies in course of disease.

Pineal region tumors in children: a single center experience.

Pineal region tumors are a relatively uncommon, deep-seated heterogeneous group of mass lesions of the brain. Their management is much more complicated in children with cancer, both in terms of survival and sequelae, due to primary location of the tumor and treatment modality. The goal of this retrospective study was to report the presentation, treatment, and outcome of tumors that arose from this region in 24 children treated at our institution between March 1975 and May 2006. In all, 15 (62.5%) of the 24 children were initially treated with partial or complete resection, adjuvant radiotherapy was given to 18 (75%) patients, and chemotherapy was given to 15 (62.5%) of the patients. Overall survival was 44.5%. Although statistically insignificant, the most favorable outcome were obtained in patients with grossly resected tumors (66%) and in children >10 years of age (80%). Long-term sequelae occurred at a high rate in this study due to the primary location of the tumors and treatment modalities, which warrants further investigation.

Quality of life of extremely long-time germinoma survivors mainly treated with radiotherapy.

PURPOSE: To assess the quality of life (QOL) of extremely long-time survivors with germinoma mainly treated with radiotherapy. PATIENTS AND METHODS: We enrolled 52 of 68 patients who received radiotherapy between 1968 and 1995 at our hospital. They were 41 males and 11 females; the tumor location was pineal in 20, neurohypophyseal in 15, pineal and neurohypophyseal in 11 patients; in 6 it was located in another region. All underwent radiotherapy; the median dose was 48.2 (range 40.0-60.2) Gy. The median follow-up period was 226 (range 0-448) months. The clinical outcome and QOL were evaluated retrospectively. RESULTS: In 6 patients, the tumor recurred; 6 other patients developed second tumors while in complete remission from the first tumor. The main cause of 12 deaths was complications due to primary tumor invasion, the initial treatment, or tumor recurrence rather than tumor progression. The 10-, 20-, and 30-year actuarial survival rate was 83.6, 77.5, and 64.2%, respectively. Of 44 patients, 6 were married and 3 males with solitary pineal tumors were fathers. Among 32 patients, 14 had, or had not, graduated from high school; the other 18 went on to higher education. Twenty-one patients had no occupation; 7 of 11 formerly employed patients had left their jobs. CONCLUSION: Radiotherapy delivered between 1968 and 1995 to patients with germinoma yielded satisfactory outcomes but a decline in the QOL.

Diurnally fluctuating frontal dysequilibrium secondary to a pineal pilocytic astrocytoma: is this symptom associated with a check-valve mechanism in a pineal region tumor?

INTRODUCTION: The clinical manifestations of obstructive hydrocephalus vary with the age of the patient, duration of the condition, and etiology of the hydrocephalus. CASE REPORT: A 14-year-old boy presented with diurnally fluctuating dysarthria, dysequilibrium of the gait, and retropulsion, probably due to transient obstruction by a tumor at the entrance of the cerebral aqueduct of the pineal region. This fluctuation was ascertained clinically by assessing the ICARS score 5 h after the patient was in the supine and erect positions. CONCLUSION: This result suggests that the symptoms of hydrocephalus are possibly associated with a check-valve mechanism of the pineal tumor and may be related to transient compression of the basal ganglia and frontal lobes.

Neurofiberscopic biopsy of tumors of the pineal region and posterior third ventricle: indications, technique, complications, and results.

OBJECTIVE: Evaluation of results of the neurofiberscopic biopsy of tumors of the pineal region and posterior third ventricle. METHODS: From 2001 to 2004, 23 patients (mean age, 30.6 yr) with tumors located in the pineal region or posterior third ventricle underwent neurofiberscopic biopsy with simultaneous third ventriculostomy. The procedure was indicated for verification of the histological diagnosis of the neoplasm, which was planned to be treated by radiotherapy and/or chemotherapy without open surgery (eight patients), establishment of the pathological diagnosis for further choice of the most appropriate treatment strategy (11 patients), differentiation of the recurrent neoplasm and radiation necrosis (two patients), and decompression of the large tumor-associated cyst (two patients). In six previously shunted patients, substitution of the ventriculoperitoneal shunt on the third ventricle stoma was performed. RESULTS: There was no postoperative mortality or permanent morbidity. In all cases, the obtained tissue sample was sufficient for pathological diagnosis. Transient postoperative complications included fever (15 patients), nausea and vomiting (three patients), and diplopia (one patient). On the long-term follow-up, delayed third ventricular stoma failure caused by tumor regrowth and scar formation was found in one patient, and dissemination of the malignant glioma through the subarachnoid space was found in another patient. CONCLUSION: Neurofiberscopic biopsy represents a useful method for sampling of tumors of the pineal region and posterior third ventricle, which can be effectively used in both previously shunted and shunt-free patients.

Pineal cysts in childhood.

INTRODUCTION: Little is known about the incidence and symptomatology of pineal cysts in children. Until now, the proper management of this group of patients has not been established. PURPOSE: The purpose of this study was to evaluate the epidemiological and clinical features of pineal cysts in children and adolescents and to try to find guidelines for their management. METHODS AND RESULTS: We analyzed 24 patients (17 girls, mean age 9, and 7 boys, mean age 14) with pineal cysts found as the only pathology on MRI. Six patients were treated surgically (excision of the cysts via a supracerebellar-infratentorial approach) because of the progression of neurological symptoms or the enlargement of the cyst at follow-up. In this group of patients, no surgery-related complications were noted, nor was residual cyst observed on postoperative MRI. In 4 cases, histological examination revealed simple cysts, but in 2 cases pineocytomas were diagnosed. Preoperative symptoms disappeared except light headache in 2 cases and in 1 case no improvement was obtained. The remaining 18 patients had a mean follow-up of 38 months (range 24-60 months). None of the cysts diminished or collapsed. We also measured the circadian pattern of melatonin secretion as well as beta-HCG and AFP levels in serum before surgery. We found very high night levels of melatonin in both of the patients with pineocytomas, while the patients with pineal cysts showed normal or depressed melatonin secretion profile. CONCLUSION: We concluded that though most pineal cysts were clinically benign they should be followed up for many years. If the cyst grows larger in follow-up MRI study and neurological symptoms are progressive, surgical treatment should be performed. In the authors' opinion, one of the markers discriminating benign and neoplastic lesions may be melatonin.

Peduncular hallucinosis due to a pineal meningioma. Case report.

The authors report the case of a 53-year-old woman who experienced visual hallucinations diagnosed as peduncular hallucinosis (PH). The cause of the PH was compression of the quadrigeminal plate and/or the splenium due to a meningioma originating from the falcotentorial junction (pineal meningioma). The nature of the visual hallucinations was depicted in drawings created by the patient herself. This is the first report of PH caused by a tumor located in the pineal region.

Pineal parenchymal tumors: a correlation of histological features with prognosis in 66 cases.

The WHO classification of CNS tumors divides pineal parenchymal tumors (PPT) into pineocytoma (PC), pineoblastoma (PB) and mixed pineocytoma-pineoblastoma or PPT with intermediate differentiation. The reported incidence of mixed/intermediate PPT varies and this may reflect the difficulty in classifying tumors of this type. In an attempt to overcome the problem of the classification of PPT with intermediate differentiation, we describe the relationship between histological features and patient survival in a large cooperative series of 66 PPT from 12 neurosurgical centres. All tumors were studied with both light microscopy and immunohistochemically using antibodies against glial markers or neural/neuroendocrine markers. Our series included 11 PC, 39 mixed/intermediate PPT and 16 PB. A number of mitoses greater than 6 and the presence of necrosis were associated with a poorer outcome, while positive immunostaining for neurofilaments was associated with a better survival. We propose a new prognostic grading of 4 grades, grade I for PC, grade II for PPT with fewer than 6 mitoses and positive immunolabelling for neurofilaments, grade III for PPT with either 6 or more than 6 mitoses or fewer than 6 mitoses but without immunostaining for neurofilaments and grade IV for PB.

Paroxysmal tonic upgaze: physiopathological considerations in three additional cases.

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.

Longitudinal neuropsychological evaluation of a case of pineal tumor occurring in an adolescent girl.

There has been a relative absence of studies that have longitudinally examined the neuropsychological profiles of patients suffering from pineal tumors. A case is reported of an adolescent girl with a pineoblastoma and spinal metastases who received extensive chemotherapy and cranio-spinal irradiation. Neuropsychological assessments conducted approximately 5 months and 2.5 years posttumor diagnosis revealed a diversity of impairments indicative of mild to moderate neuropsychological dysfunction. By the 2nd evaluation (2 years postbaseline) there was evidence of increased neurocognitive impairment suggestive of greater dysfunction of the patient's right, versus left, cerebral hemisphere. Overall, the patient's neuropsychological profile coincided with the Syndrome of Nonverbal Learning Disabilities as proposed by Rourke and his colleagues (Rourke, 1987, 1988, 1995; Rourke & Tsatsanis, 1996). These findings are discussed in light of the Syndrome of Nonverbal Learning Disabilities (and the related white matter model) and the possible negative impact of the patient's pineal tumor and subsequent chemotherapy and cranio-spinal irradiation on her neuropsychological functioning.

Surgical management of pineal region tumors.

The authors advocate an aggressive surgical approach to pineal region tumors to provide a definitive histological diagnosis and to facilitate extensive tumor removal. This strategy is based on their surgical experience in 160 operations for pineal region tumors in which operative mortality was 4% with 3% permanent major morbidity. One-third of pineal tumors were benign for which surgery alone was usually curative. A gross total removal was possible in 31 of 107 malignant tumors. The supracerebellar infratentorial approach was preferred in 86% of patients. The tumors displayed considerable histological diversity with germ cell tumors most common (37%), followed by glial cell tumors (28%) and pineal cell tumors (23%). Mixed tumors occurred 15% of the time. Spinal metastases were rare, occurring in less than 10% of patients with malignant tumors. These results with a large series of pineal region tumors demonstrate the safety and efficacy of aggressive pineal tumor surgery.

The VEP in evaluation of pituitary tumors.

12 patients with pituitary tumors were studied with VEP, to demonstrate involvement of the optic chiasma. In 3 patients VEP was recorded pre- and post-surgery to evaluate a modification in the shape and latencies of the potentials. 2 patients were studied only pre-surgery, the other 7 post-surgery. The utility of this test is discussed.