Pineal Tumors Research Articles

Visual outcome after endoscopic third ventriculostomy for hydrocephalus

Hydrocephalus-related symptoms are mostly improved after successful endoscopic third ventriculostomy (ETV). However, visual symptoms can be different. This study was focused on visual symptoms. We analyzed the magnetic resonance images (MRI) of the orbit and visual outcomes. From August 2006 to November 2016, 50 patients with hydrocephalus underwent ETV. The male-to-female ratio was 33:17, and the median age was 61years (range, 5-74years). There were 18 pediatric and 32 adult patients. Abnormal orbital MRI findings included prominent subarachnoid space around the optic nerves and vertical tortuosity of the optic nerves. We retrospectively analyzed clinical symptoms, causes of hydrocephalus, ETV success score (ETVSS), ETV success rate, ETV complications, orbital MRI findings, and visual impairment score (VIS). The median duration of follow-up was 59months (range, 3-113months). The most common symptoms were headache, vomiting, and gait disturbance. Visual symptoms were found in 6 patients (12%). The most common causes of hydrocephalus were posterior fossa tumor in 13 patients, pineal tumor in 12, aqueductal stenosis in 8, thalamic malignant glioma in 7, and tectal glioma in 4. ETVSS was 70 in 3 patients, 80 in 34 patients, and 90 in 13 patients. ETV success rate was 80%. ETVSS 70 showed the trend in short-term survival compared to ETVSS 90 and 80. ETV complications included epidural hematoma requiring operation in one patient, transient hemiparesis in two patients, and infection in two patients. Preoperative abnormal orbital MRI findings were found in 18 patients and postoperative findings in 7 patients. Four of six patients with visual symptoms had abnormal MR findings. Three patients did not show VIS improvement, including two with severe visual symptoms. Patients with severe visual impairment were found to have bad outcomes. The visual symptoms related with increased intracranial pressure should be carefully monitored and controlled to improve outcomes.

Pineal malignant solitary fibrous tumor: report of two cases

Pineal malignant solitary fibrous tumor: report of two cases

Nuclear CRX and FOXJ1 Expression Differentiates Non-Germ Cell Pineal Region Tumors and Supports the Ependymal Differentiation of Papillary Tumor of the Pineal Region.

Papillary tumor of the pineal region (PTPR) is a neuroepithelial neoplasm first described in 2003. Despite the anatomic association of PTPR with the pineal gland, the features of these tumors resemble those of the ependymal circumventricular subcommissural organ (SCO) of the posterior third ventricle. Given the presumed distinct derivation of PTPR and pineal parenchymal tumors, we hypothesized that expression of lineage-specific transcription factors could distinguish these tumors and provide additional insight into the differentiation of PTPR. A broad series of pineal region samples was reviewed, including 7 benign pineal glands, 4 pineal cysts, 13 pineocytomas, 28 pineal parenchymal tumors of intermediate differentiation, 11 pineoblastomas, and 18 PTPR. All samples were evaluated by immunohistochemistry for expression of CRX, a master transcriptional regulator of photoreceptor differentiation expressed in pineal gland and retina and/or FOXJ1, a master transcriptional regulator of ciliogenesis expressed in normal ependymal cells and ependymal neoplasms. Diffuse nuclear CRX expression is present in 100% of pineal samples. FOXJ1 is negative in all pineal samples. CRX staining is present in 53% of PTPR, though expression is nearly always limited to rare cells. Diffuse nuclear FOXJ1 expression is present in 100% of PTPR. Fetal human SCO diffusely expressed FOXJ1 but was negative for CRX. Immunohistochemistry for FOXJ1 and CRX differentiates non-germ cell pineal region tumors with high sensitivity and specificity, including pineal parenchymal tumors and PTPR. Our findings support the hypothesis that PTPR have ependymal differentiation and are phenotypically more similar to SCO than pineal gland.

Tumors of the lateral and third ventricle: surgical management and outcome analysis in 42 cases.

Objectives:To discuss the clinical presentation, pathological diagnosis, and surgical outcome for a series of 42 consecutive patients treated for lateral and third ventricular tumors.Methods:This is a retrospective series study conducted between 2001 and 2015 and included 42 patients (mean age: 25 years; range: 2 months-65 years) with lateral and third ventricle tumors surgically treated at King Khaled University Hospital, Riyadh, Kingdom of Saudi Arabia. Demographic, clinical, radiological, surgical, histopathological, and follow up data were analyzed.Results:The most common symptoms at presentation included headache (69%), nausea/vomiting (38%), visual deficits (24%), and seizures (17%). Lesions were located in the lateral ventricle in 15 patients, third ventricle in 20 patients, and involved both the lateral and third ventricles in 7 patients. The most common tumor types in the overall cohort were colloid cysts (n=6) and pineal tumors (n=6). The postoperative complication rate was 36%. The most common postoperative complications were seizure and hydrocephalus (n=5 each, 12%). Surgical mortality was 5%.Conclusion:The selection of the surgical approach for intraventricular tumor resection is fundamentally dependent on the surgeon’s experience and preference. We recommend that this decision be based on the anatomic considerations that provide the best and safest access to the mass, rather than on the risk of seizure following transcortical approach.

“Your body, your choices”: Peer and professional support for breastfeeding

We aimed to identify the factors that can predict the risk of occipital lobe damage preoperatively when resecting tumors located at the tentorial or pineal regions with the occipital-transtentorial approach (Poppen approach).In 27 consecutive patients who underwent tumor resection with the Poppen approach for tentorial or pineal region meningiomas, the following morphologic parameters were assessed on a preoperative magnetic resonance imaging: (1) tentorial angle, (2) tentorial length, and (3) the shortest distance from the confluence of the sinus to the tumor. These parameters, together with tumor size, texture, and resection extent, were correlated with occipital lobe damage by using the one-way analysis of variance, χ2, or Fisher's exact tests.The mean value was 55.3° ± 5.6° (range, 45°–66°) for the tentorial angle, which was significantly associated with the occipital lobe damage grades (P = 0.008), but this was not the case for the tentorial length (P = 0.802) and the shortest distance from the confluence of the sinus to the tumor (P = 0.695). Interestingly, age was also strongly associated with occipital lobe damage risk (P = 0.020). The patients in the subgroup with no occipital damage (grade 4) were the youngest (aged 47.3 years), compared with other grades, with age of 58.0 years for grade 1, 54.3 years for grade 2, and 58.6 years for grade 3. These 2 parameters were also significant after multivariate analysis. No correlation was observed between either tumor nature or the extent of resection and damage grades.The risk of occipital lobe damage increases in the presence of a steep tentorial angle during the Poppen approach for tentorial or pineal area tumors. Awareness of such anatomic features preoperatively is important for minimizing operative complications.

A Prospective, Multicenter Trial for Intracranial Germinomas (KSPNO G051/G081 Protocol): 5-year Follow-up Results of Upfront Chemotherapy Followed by Response Adaptive Radiation Therapy in Korean Patients

There is no definite consensus in the optimal treatment for intracranial germinomas. The Korean Society of Pediatric Neuro-Oncology (KSPNO) had launched a prospective randomized trial of intracranial germinomas (KNSPNO G051) and the protocol had been modified with a single arm study (KSPNO G081) comprising of upfront chemotherapy followed by response adaptive radiotherapy (RT). Herein, we report the five-year follow-up results of KSPNO G051/081 protocols. Between April 2005 and April 2011, a total of 91 patients from five institutions were registered in the KSPNO G051/G081 protocols. The protocol schema will be shown in our presentation. In brief, germinomas were classified into solitary and multiple/disseminated diseases and response adaptive RT was conducted according to the protocols. The primary study end-points were overall survival (OS) and event-free survival (EFS). The median age of study cohorts was 14 (range, 3-30) years and 68 (74.7%) patients were male. The majority of enrolled patients initially presented with solitary lesion (n = 65, 71.4%). The common solitary sites were pineal (n = 24, 26.4%), sellar/suprasellar (n = 24, 26.4%) and basal ganglia (n = 15, 16.5%). A total of 75 (82.4%) patients completed four cycles of planned chemotherapy. In the entire study population, the median CSI dose was 19.8 (range, 18-36) Gy and the median total tumor bed dose was 39.3 (range, 27-54) Gy. The median tumor bed doses for complete responses (CRs) and partial responses (PRs) after chemotherapy were 30.6 Gy and 40.2 Gy, respectively. The median follow-up duration was 67.9 (range, 6.6-119.3) months. The 5-year and 7-year OS rates were 98.8% and 98.8%, and the 5-year and 7-year EFS rates were 96.6% and 93.8 %, respectively. During follow-up periods, relapses occurred in four (4.4%) patients, 5.9-77 months after completion of planned treatment with CRs states. The primary origin of all these four patients was basal ganglia and they were treated with CSI (one patient) or local RT (three patients). All patients with basal ganglia germinomas and treated with local RT recurred outside of RT field. There was no relapse in 48 patients with solitary pineal or suprasellar lesions and 23 (48%) patients of them including seven PRs had received whole ventricle RT. Current upfront chemotherapy followed by reduced-dose, reduced-volume RT is acceptable and there is a room to reduce relapses by modification of RT volumes. RT volumes for localized tumors should include whole ventricle for pineal or suprasellar tumors and at least whole brain for basal ganglia/thalamus lesions. For disseminated diseases, CSI alone would be appropriate. This combined modality strategy should be compared with reduced-dose, reduced-volume RT alone in the future clinical trials.

Pineal Parenchymal Tumor with Intermediate Differentiation Treated with Radio-Chemotherapy: First Case Report in Our Institution

Pineal parenchymal tumor with intermediate differentiation (PPTID) is an uncommonlesion of the brain with a controversial management strategy. The aim of this study wasto analyse the role of chemo-radiotherapy for a PPTID patient at the institution with onecase report treated by multidisciplinary team.

Trilateral retinoblastoma: A systematic review of 211 cases.

We conducted a systematic review of 72 studies to characterize trilateral retinoblastomas. Kaplan-Meier analysis was used to estimate survival, and statistical significance was assessed by using a log-rank test. We analyzed 211 cases of trilateral retinoblastomas. The average age of onset of retinoblastoma was 0.79±1.38years, and the average latency period between the onset of retinoblastomas and trilateral retinoblastomas was 1.49±1.76years. The brain tumors were found before the retinoblastoma diagnosis in 6 cases (3.1%), concurrently in 61 cases (32.1%), and after the retinoblastoma diagnosis in 123 cases (64.7%). Pineal tumors were found in 155 cases (73.4%) and sellar tumors in 46 cases (21.8%). The overall median survival was 10.3months (95% CI, 8.5-13) and the 5-year survival rate was 15.7%. Central nervous system symptoms were variable and associated with shorter survival in univariate and multivariate analyses. The survival time in patients who received high-dose chemotherapy with stem cell transplant was significantly longer (p=0.0067) than that of with or without conventional chemotherapy. Twelve long-term survivors were reported, and of these, six patients were treated with high-dose chemotherapy with stem cell transplant and six patients were treated with conventional chemotherapy. It is important that survivors continue to undergo regular medical surveillance in order to detect trilateral retinoblastoma at a potentially curative stage. Trilateral retinoblastoma patients with an irradiation history had shorter survival than those without irradiation history for retinoblastoma. High-dose chemotherapy should be considered as a potential treatment option for trilateral retinoblastomas.

Significance of the Tentorial Alignment in Protecting the Occipital Lobe with the Poppen Approach for Tentorial or Pineal Area Meningiomas.

We aimed to identify the factors that can predict the risk of occipital lobe damage preoperatively when resecting tumors located at the tentorial or pineal regions with the occipital-transtentorial approach (Poppen approach). In 27 consecutive patients who underwent tumor resection with the Poppen approach for tentorial or pineal region meningiomas, the following morphologic parameters were assessed on a preoperative magnetic resonance imaging: (1) tentorial angle, (2) tentorial length, and (3) the shortest distance from the confluence of the sinus to the tumor. These parameters, together with tumor size, texture, and resection extent, were correlated with occipital lobe damage by using the one-way analysis of variance, χ2, or Fisher's exact tests. The mean value was 55.3° ± 5.6° (range, 45°-66°) for the tentorial angle, which was significantly associated with the occipital lobe damage grades (P= 0.008), but this was not the case for the tentorial length (P= 0.802) and the shortest distance from the confluence of the sinus to the tumor (P= 0.695). Interestingly, age was also strongly associated with occipital lobe damage risk (P= 0.020). The patients in the subgroup with no occipital damage (grade 4) were the youngest (aged 47.3 years), compared with other grades, with age of 58.0 years for grade 1, 54.3 years for grade 2, and 58.6 years for grade 3. These 2 parameters were also significant after multivariate analysis. No correlation was observed between either tumor nature or the extent of resection and damage grades. The risk of occipital lobe damage increases in the presence of a steep tentorial angle during the Poppen approach for tentorial or pineal area tumors. Awareness of such anatomic features preoperatively is important for minimizing operative complications.

Histology-Stratified Tumor Control and Patient Survival After Stereotactic Radiosurgery for Pineal Region Tumors: A Report From the International Gamma Knife Research Foundation.

Pineal region tumors represent a rare and histologically diverse group of lesions. Few studies are available to guide management and the outcomes after stereotactic radiosurgery (SRS). Patients who underwent SRS for a pineal region tumor and for whom at least 6 months of imaging follow-up was available were retrospectively assessed in 5 centers. Data were collected from the medical record and histology level analyses were performed, including actuarial tumor control and survival analyses. A total of 70 patients were treated between 1989 and 2014 with a median follow-up of 47 months. Diagnoses were pineocytoma (37%), pineoblastoma (19%), pineal parenchymal tumor of intermediate differentiation (10%), papillary tumor of the pineal region (9%), germinoma (7%), teratoma (3%), embryonal carcinoma (1%), and unknown (14%). Median prescription dose was 15 Gy at the 50% isodose line. Actuarial local control and survival rates were 81% and 76% at 20 years for pineocytoma, 50% and 56% at 5 years for pineal parenchymal tumor of intermediate differentiation, 27% and 48% at 5 years for pineoblastoma, 33% and 100% at 5 years for papillary tumor of the pineal region, 80% and 80% at 20 years for germinoma, and 61% and 67% at 5 years for tumors of unknown histology. New focal neurological deficit, Parinaud syndrome, and hydrocephalus occurred in 9%, 7%, and 3% of cases, respectively. SRS is a safe modality for the management of pineal region tumors. Its specific role is highly dependent on tumor histology. As such, all efforts should be made to obtain a reliable histologic diagnosis.

Descartes Comes Out of the Closet

While “Descartes Comes Out of the Closet” is ostensibly about a young woman’s journey to Paris, the descriptive detail borrows language and images from Cartesian coordinate geometry, dualistic philosophy, neuroanatomy (the pineal), and projections of three dimensions onto planes. This mathematical universe is counterpointed in the natural language of the suppressed love story that locates the real in the human. Thus, at the heart of the story is the tension between competing notions of mathematics, i.e., as either an independent realm apart from history or as a culturally produced and historical set of practices. Of course, the central character proves to suffer from a pineal gland tumor, so the mechanisms of reconciliation aren’t functioning very well.

Reduced-volume radiotherapy for patients with localized intracranial nongerminoma germ cell tumors.

Craniospinal irradiation is standard radiotherapy (RT) for localized intracranial nongerminoma germ cell tumors (NGGCT). Given its toxicity, there is interest in using smaller fields. We examined outcomes of NGGCT patients receiving reduced-volume RT at a single institution. Records of 16 patients who received reduced-volume RT as part of definitive treatment between 1996 and 2016 were reviewed. Median age at presentation was 10.8 years (range 4.6-41.0 years). Ten patients had pineal tumors and 6 had suprasellar tumors. All received chemotherapy and 9 patients received second-look surgery thereafter. RT volume was tumor-only to a median of 54Gy (range 50.4-54Gy) in 3 patients and whole-ventricle irradiation to a median of 30.6Gy (range 30.6-36Gy) with a boost to 54Gy in 13 patients. Median follow-up was 4.1 years (range 1.9-19.3 years). Three patients recurred locally at a median 9.9 months (range 9.6-10.6 months) after diagnosis, and one of these developed leptomeningeal relapse after 30 months. One patient expired from disease 2.6 years post-diagnosis and another due to stroke 19.3 years post-diagnosis. Fourteen patients are alive with no evidence of disease. Kaplan-Meier estimates of the 4-year overall survival and failure-free survival are 92% (95% confidence interval [CI], 57-99%) and 81% (95% CI 53-94%), respectively. Excellent disease control was observed in these patients with no initial relapses outside of these RT fields. The results of ACNS1123 may better delineate patterns of failure and identify subgroups likely to benefit from this approach.

Parinaud syndrome: Any clinicoradiological correlation?

The significance of MRI findings of patients with Parinaud syndrome (PS) with respect to clinical characteristics is poorly defined. Over the past decades, all patients with PS undergo magnetic resonance imaging which allows a better identification of the lesion localization. We compared the neuro-ophthalmological findings of patients with PS caused by intrinsic (intra-axial) vs extrinsic (pineal gland tumor) brainstem lesions. Medical records of patients with PS evaluated between 2000 and 2016 were retrospectively reviewed. Twenty-six patients with PS were included. Eight patients had pineal gland tumors and hydrocephalus. Two patients had hydrocephalus due to aqueduct stenosis and fourth ventricle tumor. Sixteen patients suffered from an intrinsic brainstem lesion and seven associated with hydrocephalus. The neuro-ophthalmological findings did not differ between patients with extrinsic and intrinsic brainstem lesions. No correlation was found between the grade of hydrocephalus and number of clinical findings except for more findings in low-grade hydrocephalus in intrinsic (40%) vs extrinsic (0%) lesions (P=.003). Patients with moderate brainstem lesions and hydrocephalus had more clinical findings (65%) than patients with the same grade of brainstem involvement without hydrocephalus (29%) (P=.03). The resolution rate of ophthalmological findings was comparable in all groups of patients. Our results did not show differences in neuro-ophthalmological findings between intra- and extra-axial lesions causing PS. However, the presence of hydrocephalus was an important factor influencing clinical findings. The prognosis of PS was less favorable than generally reported.

Five-year Review of Pineal Region Tumors at the Philippine General Hospital

Rationale: Pineal region tumors are rare neoplasms with a reportedly higher incidence in Asian countries; however, local Philippine data is lacking. Methods: A retrospective chart review was conducted on all newly diagnosed adult and pediatric patients with pineal region tumors admitted at the Philippine General Hospital between 2011 and 2015. Data about demographic profile, biochemical markers, imaging findings, histopathology, and treatment were collected. Results: Forty-two patients (36 males, 6 females; Sex Ratio = 6:1) were included in the study, with a mean age of 16.5 years. On imaging, solitary pineal area tumors were seen in 34 (81%) patients, while 8 (19%) presented with synchronous tumors in the pineal and suprasellar areas. Hydrocephalus was present in 41 (98%). Tumor marker (serum +/- CSF αFP and βhCG) determination was performed in 33(79%) patients. Thirty-eight (90%) patients underwent surgical intervention for tumor biopsy and/or CSF diversion. Combining the tumor marker levels and histopathology results, there were 20(48%) germ cell tumors, 4(9%) pineal parenchymal tumors, 1(2%) meningioma, 1(2%) epidermoid tumor, and 16(38%) tumors with incomplete diagnosis. Regarding adjuvant treatment, 5 patients underwent chemotherapy, 6 underwent radiotherapy, and 1 patient received both. Follow-up data were available in only 16 patients, with a mean follow-up of 12 months (range: 1-33 months). Conclusion: The demographic profile and histologic subtypes of patients with pineal region tumors in this series were comparable with other series in the literature. However, due to limited resources leading to suboptimal medical care and poor follow-up, a reliable treatment outcome could not be determined. Key words: pineal gland, brain neoplasms, germinoma, biomarkers tumor

Microsurgical Resection After Failed Gamma Knife Radiosurgery of Papillary Tumor of the Pineal Gland.

Abstract Primary papillary tumors of the pineal region (PTPR) are rare entities with distinct histopathological characteristics. Most common surgical approaches include the supra-cerebellar infratentorial corridor or the occipital interhemispheric approach. Gamma knife radiosurgery (GKRS) is an acceptable treatment modality for parenchymal tumors of the pineal gland, and often used as a primary treatment modality for asymptomatic and indolent lesions, and as an adjunct to surgical resection. In this presentation, we describe the surgical nuances of posterior interhemispheric approach in an elderly male with a previously failed empirical GKRS. Intraoperatively, the tumor was mostly avascular, presumed to be due to prior GKRS; however, a good tumor-brain interface allowed a gross total resection of the mass. Histopathological examination of the mass was consistent with PTPR. Post-operative course was uneventful and long-term follow up showed no signs of tumor recurrence. The video discusses radiological findings, clinic-pathological correlates, and emphasizes on key microsurgical techniques including the significance of patient positioning and incision type for meticulous resection of pineal tumors.

P16.26 Surgical Treatment of Pineal Region Tumors

Objective: To analyse short-term results and assess long-term follow-up of patients with pineal region tumors which were operated in Uzhhorod Regional Centre of Neurosurgery and Neurology. Methods: 24 patients with pineal region tumors were operated in Uzhhorod Regional Centre of Neurosurgery and Neurology since 2011. Male to female ratio was 1:1. Mean age of the patients was 25.2 years (10 were of paediatric age). Patients were divided into two groups according to tumor type: low-grade - 12 cases (meningioma - 3, pylocytic astrocytoma - 3, pineocytoma - 2, ependymoma - 1, choroid glioma - 1, papillary tumor - 1, xantogranulema - 1) and high-grade - 12 patients (pineoblastoma - 5, anaplastic astrocytoma - 2, glioblastoma -2, teratoma -1, germinoma - 1, choriocarcinoma - 1). The extent of resection was evaluated on early postoperative MRI. Short-term results were determined based on neurological examination one month after surgery in all patients. Long-term results were assessed based on overall survival in patients with high-grade tumors and Karnofsky Performance Scale (KPS) on last follow-up in patients with low-grade tumors. Mean follow up was 24 months (in low-grade group - 34 months). Results: Gross total resection was achieved in 10 cases (41.7%) and in 6 patients near total resection was performed (25%). 4 patients underwent a subtotal resection of the tumor (16.7%) and in 4 cases only partial resection was done (16.7%). Majority of the patients (83.3%) were neurologically stable or improved compared to preoperative status on day 30 follow-up. 2 patients (8.3%) deteriorated after resection of the tumor. Postoperative mortality was 8.3% (large venous infarction and postoperative meningitis). All patients with high-grade tumors underwent radio- and chemotherapy after surgery according to tumor histology. Mean overall survival in this group of patients was 11.6 months. 7 out of 12 patients (58.3%) with low-grade pineal tumors underwent gross total resection. On last follow-up 10 patients (83.3%) with low-grade tumors were KPS 100 and 2 (16.7%) patients were KPS 70. Conclusions: Resection of pineal region tumors is safe (morbidity/mortality - 16.6%). In patients with high-grade pineal tumors surgical strategy should consist of simple debunking as overall survival is poor (11.6 months). Excellent long-term outcome (KPS 100 in 83.3%) in patients with low-grade tumors was achieved with aggressive surgical strategy (gross total resection in 58.3%).

P03.04 Histological spectrum of primary pineal tumours - A tertiary cancer centre experience

P03.04 Histological spectrum of primary pineal tumours - A tertiary cancer centre experience

Imaging Spectrum of Unusual Pineal Region Masses: A Short Series of Four Cases

AbstractPineal tumors are rare intracranial neoplasm with germ cell tumors and pineal parenchymal tumors being the predominant type, seen in 80 to 90% of cases. Only a few cases of glioblastomas have been reported. The incidence of astrocytoma and meningioma is also low in this region. In this article, we will discuss the key imaging features and diagnostic dilemmas in four rare types of pineal region tumors including pineal parenchymal tumor of intermediate differentiation, pineal astrocytoma, falcotentorial junction meningioma, and glioblastoma with subependymal spread.

Pineal Region Masses in Pediatric Patients.

A review of pediatric pineal region tumors is provided with emphasis on advanced imaging techniques. The 3 major categories of pineal region tumors include germ cell tumors, pineal parenchymal tumors, and tumors arising from adjacent structures such as tectal astrocytomas. The clinical presentation, biochemical markers, and imaging of these types of tumors are reviewed.

Children with dorsal midbrain syndrome as a result of pineal tumors

Dorsal midbrain syndrome (also known as Parinaud syndrome and pretectal syndrome) is a well-known complication of tumors of the pineal region. However, there are few reports regarding outcomes, especially in children. The purpose of this study was to report the ophthalmic outcomes in a group of children with pineal tumors treated at a single institution. The medical records of pediatric patients diagnosed with pineal region tumors and evaluated at our ophthalmology clinic were studied retrospectively. Descriptive statistics were used to assess rate of dorsal midbrain syndrome, defined as one or more of the following: limitation of upgaze, pupillary light-near dissociation, and convergence retraction nystagmus. Treatment outcomes were recorded. A total of 35 subjects (age range, 5months to 20years) were included, 18 (51%) of whom were found to have dorsal midbrain syndrome. Of those 18, 16 patients (89%) had limitation of upgaze, 15 (83%) had pupillary light-near dissociation, and 9 (50%) had convergence-retraction nystagmus. Convergence insufficiency was noted in 5 patients (28%); exotropia (either intermittent or constant), in 9 (50%). Improvement in dorsal midbrain syndrome findings following treatment was seen in 7 of 17 patients (41%), but only 2 (12%) experienced complete resolution. Treatment consisted of surgery, radiation, and/or chemotherapy. In our study cohort of children with pineal tumors have a high incidence of dorsal midbrain syndrome. Most cases had residual findings after treatment.