Pineal Cyst Research Articles

Missense variant Met119Val in ACTB in a patient with Baraitser-Winter syndrome type 1 and mild intellectual disability.

Here we report on a Brazilian female patient with striking dysmorphic craniofacial features of the Baraitser-Winter syndrome (BRWS), including coarse face with prominent metopic ridge, highly arched eyebrows, ocular hypertelorism, ptosis, long palpebral fissures, broad nasal tip, long philtrum, full cheeks, cleft lip and palate, and low-set ears with abnormal lobe. Other clinical signs included partial agenesis of the corpus callosum, asymptomatic pineal cyst, oligodontia, cervical cystic hygroma, pterygium colli, axillary pterygium, duplicated left hallux and seizures. Sanger sequencing of the ACTB gene showed a heterozygous missense variation NM_001101.5 (ACTB):c.355A>G (p.Met119Val). This variant is located very close to the ACTB variant Thr120Ile, which results in the severe BRWS phenotype with severe intellectual disability and absence of speech, firstly described as Fryns-Aftimos syndrome. Despite this, our patient had mild developmental delay, without impairment of speech, and with moderated learning difficulties.

Comment on - "Pineal cysts without hydrocephalus: microsurgical resection via an infratentorial-supracerebellar approach-surgical strategies, complications, and their avoidance".

Pineal cysts without hydrocephalus present a clinical dilemma, as surgical indications remain controversial. This study evaluates 73 patients who underwent microsurgical resection of pineal cysts via an infratentorial-supracerebellar (SCIT) approach, focusing on surgical strategies, complications, and outcomes. With a follow-up period averaging 26.6 months, 89% of patients reported significant symptom improvement, particularly in headache, visual disturbances, and dizziness. Total cyst resection was achieved in 95% of cases, with minimal complications. The findings suggest that microsurgical resection can provide marked relief in carefully selected patients, even in the absence of ventriculomegaly. Future studies should focus on randomized comparisons between surgical and conservative approaches and explore long-term outcomes.

8267 A Case of Pineal Gland Cyst and Adrenal Adenoma- Is this a New Syndrome?

Abstract Disclosure: M. Siddiqui: None. U. Rafat: None. S. Shaik: None. J.L. Gilden: None. Background: The Pineal Gland receives information through a complex multi-neuronal pathway and is responsible for the secretion of melatonin, a hormone regulated by circadian rhythm and suppressed by light stimuli. Melatonin is involved in biological rhythms and regulates immunomodulation, temperature homeostasis and maturation of hypothalamic-pituitary and gonadal axis, and can also modulate thyroid, prolactin, growth hormone, cortisol, and ACTH activity. Other effects include downregulation of thyroid activity. In addition, studies have shown that pineal body extracts can cause a minor increase in the zona glomerulosa and fasciculata of the adrenal gland. Case: A 54-year-old male with recurrent nephrolithiasis, obstructive sleep apnea and chronic rhinosinusitis, first presented to Endocrinology Clinic in 2018 for evaluation of an incidental adrenal adenoma (CT abdomen -2.8 cm, less than 10 Hounsfield units, left sided lipid rich adrenal adenoma). He had new onset erectile dysfunction, decreased libido, and fatigue. Initial lab work including LH, FSH, TSH, midnight salivary cortisol, ACTH, plasma cortisol AM, free and total testosterone, aldosterone, plasma and urine metanephrines/normetanephrines were all normal. MRI brain-1 cm thin-walled pineal cyst with internal characteristics compatible with proteinaceous benign pineal cyst. He was then lost to follow-up for 5 yrs due to the COVID pandemic. He had tried taking “testosterone protein builders”, but his symptoms remained. In addition, he now has twice weekly dull occipital headaches, relieved by ibuprofen. Lab tests-elevation in 1of 2 MN salivary cortisol samples at 0.58 mcg/dL (nl≤0.09), ACTH 12pg/mL(0-47), IGF1 261 ng/mL(50-317), and low total testosterone 222 ng/dl (250-1100) Free testosterone 36 pg/mL(35-155), borderline low TSH 0.386 µIU/ml(0.55-4.78), Free T3 and Free T4 were normal, thyroid antibodies-negative . Rest of the hormone evaluation was otherwise normal, LH 4.90 mIU/mL(1.5-9.3), FSH 11.10 mIU/mL (1.4-18.1), Prolactin 6.8 ng/mL(2.1-17.7). Thyroid Ultrasound-homogeneous echogenicity of the thyroid gland without discrete thyroid nodules. Repeat MRI brain- benign pineal cyst which was stable with no midline shift or mass effect. Repeat CT adrenal gland-lipid rich adrenal adenoma slightly reduced in size from 2.8 to 2.4 cm. Lab tests returned to normal one month later. Conclusion: Although it is unclear whether there is an exact association between this patient’s pineal cyst and adrenal adenoma, there have been intermittent hormonal abnormalities and symptoms consistent with hypogonadism and/or adrenal, also possibly related to the pineal abnormality. Although more research needs to be conducted explaining the relationship between the pineal and pituitary, and the adrenal gland, this clinical case suggests that there may be a relationship between these two abnormalities and symptoms. Presentation: 6/2/2024

The role of MRI biomarkers in evaluation of symptomatic pineal cysts – a retrospective analysis

BackgroundOur aim was to determine whether the Apparent Diffusion Coefficient is able to predict the presence of a symptomatic pineal cyst by detecting cerebral edema.MethodsWe retrospectively analyzed MRIs of 45 patients with pineal cysts before and after resection and 51 patients without pineal cysts, comparing ADC values of thalamus, central, periventricular and subcortical white matter. Furthermore we evaluated cyst size and morphology and analyzed its correlation to ADC values in corresponding patients.ResultsDifferences between patients with symptomatic pineal cyst and control group were not significant (p = 0.200 – 0.968). ADC ratios did not change significantly after resection of the cyst (p = 0.575 – 0.862). Cyst size showed no significant correlation to ADC ratios (p = 0.071 – 0.918). Raw data analyses revealed more significance, especially periventricularly and in central white matter, which resulted in significant interhemispheric differences in ADC ratios in both subgroups (p < 0.001 and p = 0.031). MRI of 1.5T showed consistently higher values than 3T but mostly insignificant.ConclusionOur analysis revealed no evidence that pineal cysts lead to intracerebral edema caused by venous compression. Since variability was higher than the differences seen, ADC sequences do not appear to be an appropriate diagnostic tool for symptomatic pineal cysts.

SNO-EANO-EURACAN consensus on management of pineal parenchymal tumors.

Pineal parenchymal tumors are rare neoplasms for which evidence-based treatment recommendations are lacking. These tumors vary in biology, clinical characteristics, and prognosis, requiring treatment that ranges from surgical resection alone to intensive multimodal antineoplastic therapy. Recently, international collaborative studies have shed light on the genomic landscape of these tumors, leading to refinement in molecular-based disease classification in the 5th edition of the World Health Organization (WHO) classification of tumors of the central nervous system. In this review, we summarize the literature on diagnostic and therapeutic approaches, and suggest pragmatic recommendations for the clinical management of patients presenting with intrinsic pineal region masses including parenchymal tumors (pineocytoma, pineal parenchymal tumor of intermediate differentiation, and pineoblastoma), pineal cyst, and papillary tumors of the pineal region.

Lack of inflammation or immune response in cyst tissue of patients with symptomatic non-hydrocephalic pineal cysts

Pineal cysts are frequently encountered as incidental findings in magnetic resonance imaging, usually devoid of symptoms, yet some patients exhibit symptomatic manifestations possibly associated with the cyst, even in the absence of hydrocephalus. The etiology of these symptoms remains contentious. This study aims to investigate the presence of lymphatic endothelial cell (LEC) markers and indications of inflammation or immune response within the pineal cysts of patients experiencing symptomatic non-hydrocephalic presentations. Eight patients who underwent surgical excision of their cysts were included in the study. Immunohistochemistry was utilized to assess the expression of LYVE-1, PDPN, and VEGFR3 as LEC markers, alongside IL-6 and CD3 for indications of inflammation or immune activity. Our analysis revealed an absence of inflammatory markers or immune response. However, a distinct expression of VEGFR3 was observed, likely localized to neurons within the pineal cyst tissue. We propose that these VEGFR3+ neurons within the pineal cyst may contribute to the headache symptoms reported by these patients. Further investigations are warranted to substantiate this hypothesis.

Changes in the central nervous system in football players: an MRI study.

Football (soccer) is the world's most popular team sport. To comprehensively examine the brain in football (soccer) players, with the use of magnetic resonance imaging (MRI) techniques. The study involved 65 football players and 62 controls. The MR examinations were performed using MR 1.5-T system (Optima MR 360; GE Medical Systems). The examinations were carried out in the 3D Bravo, CUBE, FSEpropeller, and diffusion-weighted imaging (DWI) sequences. The 1HMRS signal was obtained from the volume of interest in the frontal and occipital lobes on both sides. The present study, based on structural MRI, shows some changes in the brains of the group of football players. The findings show asymmetry of the ventricular system in four football players, arachnoid cysts in the parieto-occipital region, and pineal cysts. NAA/Cr concentration in the right frontal lobe was lower in the football players than in the controls, and the Glx/Cr concentration in the right occipital lobe was higher. The apparent diffusion coefficient value is lower in football players in the occipital lobes. Playing football can cause measurable changes in the brain, known to occur in patients diagnosed with traumatic brain injury. The present findings fill the gap in the literature by contributing evidence showing that playing football may lead to changes in the brain, without clinical symptoms of concussion.

Pineal gland cyst-to fight or not to fight?

Since professional boxing is associated with substantial risk of both acute and chronic traumatic brain injury, many athletic (boxing) commissions in the United States and abroad request a neuroimaging study of the brain to determine brain fitness to fight. Either magnetic resonance imaging (MRI) brain or computed tomography (CT) head is needed to obtain a license to compete in professional combat sports and periodically thereafter. The neuroimaging study helps to identify and/or exclude structural brain lesions that may pose a risk of rupture or bleeding from head impact exposures (HIEs) sustained during the course of the bout. Identification of structural brain lesions such as large tumors, vascular malformations such as aneurysms, arteriovenous malformations, and large cavernous malformations results in a denial of licensure to fight. Pineal gland cysts (PC) are usually benign cysts discovered on occasions incidentally in MRI scans of boxers. A 25-year-old asymptomatic boxer was found to have a 2 cm PC with intracystic hemorrhage on MRI scan done for licensure. The medical literature was reviewed to determine whether boxers with PC can safety compete in combat sports.

Pineal Gland Epidermoid cyst, a rare Tumor

Background: Intracranial epidermoid cysts, forming 1% of adult and 3-8% of pediatric brain tumors, are among the rarest of intracranial neoplasms. Originating from ectodermal remnants retained during embryonic development, pineal epidermoid cysts are rare benign tumors that often manifest as obstructive hydrocephalus due to midbrain tectum compression. Objective: To report on the clinical presentation, diagnostic process, and surgical management of a pineal epidermoid cyst in a young adult, emphasizing the role of ventriculoperitoneal shunting and the outcomes of surgical intervention. Methods: A case study of a 20-year-old male presenting with a 4-month history of occipital headaches and vertigo, without seizures, neurological deficits, or visual disturbances. Diagnostic evaluation included brain imaging that revealed hydrocephalus secondary to a pineal space-occupying lesion. A ventriculoperitoneal shunt was placed for obstructive hydrocephalus treatment prior to the surgical excision of the pineal lesion. Results: The patient underwent a ventriculoperitoneal shunting procedure, followed by a suboccipital transtentorial craniotomy 2 months later, leading to the complete resection of the pineal space-occupying lesion. The histopathological analysis confirmed the diagnosis of a pineal epidermoid cyst. Post-surgical follow-up indicated significant symptomatic improvement with no recurrence observed. Conclusion: Pineal epidermoid cysts, while rare, can significantly impact patient well-being through the development of obstructive hydrocephalus. Early intervention with ventriculoperitoneal shunting followed by surgical resection can lead to favorable outcomes. Long-term monitoring is essential to ensure there is no recurrence.

Pineal gland cyst and ADHD in a juvenile patient: a case report

IntroductionThe role of the pineal gland in psychiatric disorders is poorly investigated. There are studies, primarily on adult patients, that indicate a higher presence of pineal gland abnormalities in patients suffering from depression, schizophrenia and attention-deficit/hyperactivity disorder (ADHD). When it comes to ADHD, there is speculation about the role of melatonin and the influence of the pineal gland on the dopaminergic system. Data on the association between pineal gland cysts and ADHD in juvenile patients are particularly scarce.ObjectivesDue to all of the above, our goal is to present the case of a nine-year-old male patient who has a confirmed cyst of the pineal gland and is being treated for ADHD. MethodsThe patient was examined by a neuropediatrician, EEG and brain MRI were performed. He was also examined by a psychologist and by a psychiatrist. Endocrinological, hematological, rheumatological, pulmonological treatment and karyotyping were performed.ResultsMRI of the brain revealed a cyst of the pineal gland with an anteroposterior diameter of 1 cm without significant compression. The EEG was mildly slowed and paroxysmally dysrhythmic for the age, ie. paroxysms of high-voltage delta waves were described. The EEG findings after sleep deprivation were paroxysmally altered with rare focal changes in the right temporoparietal region. Through psychological analysis, it was determined that specific deficits persist in the area of verbal understanding, perceptual organization and visual processing, information processing speed, numerical reasoning, attention and short-term memory. On the level of visuomotor perception and coordination, deviations are observed by organic type. He is motorically more active, impulsive, emotionally immature, easily distractible.ConclusionsThe etiology of ADHD is poorly researched, and so is the role of the pineal gland, its cyst and melatonin. There is scant knowledge for other psychiatric disorders, but primarily from researches on adult psychiatric patients. Additional researches are definitely needed on this topic, especially in the field of child and adolescent psychiatry.Disclosure of InterestNone Declared

Pineal cysts may promote pubertal development in girls with central precocious puberty: a single-center study from China.

Pineal cysts have long been considered a benign intracranial variation. However, in our clinical practice, it has been observed that some children with central precocious puberty (CPP) who have pineal cysts experience rapid progression in adolescent development. In recent years, there has been a significant increase in the prevalence of CPP in girls, leading to more diagnoses of CPP among children with pineal cysts. Despite this, there is no consensus regarding whether pineal cysts contribute to CPP as one of its organic factors. This study aimed to analyze the clinical characteristics of pineal cysts in children with CPP and explore the potential effects of pineal cysts on puberty development. This single-center study retrospectively analyzed clinical data from girls aged 3 to 10 years who underwent head/pituitary magnetic resonance imaging at the Children's Hospital Affiliated to Zhengzhou University between 2019 and 2022. The study categorized the detection rates of pineal cysts based on systematic disease classification and compared the rates of cyst detection between girls diagnosed with CPP and those without CPP. Subsequently, CPP-diagnosed girls with pineal cysts were examined. Among CPP-diagnosed girls meeting the study's criteria, those with pineal cysts formed the 'cyst group,' while those without cysts were matched in a 1:1 ratio based on age and body mass index to form the 'non-cyst group.' Comparative analyses were conducted to assess the clinical characteristics between these two groups. CPP-diagnosed girls with cysts were further subdivided into three groups according to cyst size (≤5 mm, 5.1-9.9 mm, and ≥10 mm) to investigate potential differences in clinical characteristics among these subgroups. The study involved an analysis of clinical data from girls diagnosed with CPP and included imaging follow-ups to explore the progression of pineal cysts over time. Among the 23,245 girls who underwent head/pituitary magnetic resonance imaging scans, the detection rate of pineal cysts was 3.6% (837/23,245), with most cases being associated with endocrine diseases. The detection rate of pineal cysts in CPP patients was 6.4% (262/4099), which was significantly higher than the 3.0% (575/19,146) in patients without CPP. In comparison to the non-cyst group, the cyst group exhibited statistically significant increases in estradiol levels, peak luteinizing hormone (LH) levels, peak LH/follicle-stimulating hormone (FSH) ratios, uterine body length, and cervix length (P < 0.001). As cyst size increased, there were significant rises in LH peak, peak LH/FSH ratio, uterine body length, and cervical length (P < 0.01). Estradiol levels and left ovarian volume also showed an increasing trend (P < 0.05). Among girls who underwent follow-up imaging, 26.3% (5/19) exhibited an increase in cyst size. Pineal cysts are relatively common in children with CPP. They may affect the pubertal development process, with larger cysts correlating to faster pubertal development. Therefore, the authors hypothesize that pineal cysts may trigger CPP in some cases, especially when the cysts are larger than 5mm in size, as indicated by our data.

Связь кисты эпифиза с особенностями строения головного мозга и наличием определенных черт личности

Aim. To study the psychological characteristics and microstructural changes of the brain, according to magnetic resonance imaging (MRI), in healthy volunteers with pineal cyst to determine their clinical significance. Design. A prospective case — control study. Materials and methods. The study involved conditionally healthy volunteers. Based on brain MRI data, study groups were formed: the main group consisted of 33 people with pineal cyst, the control group consisted of 30 people with normal epiphysis structure. The groups were comparable in gender and age. We conducted neurological examination, experimental psychological and neuropsychological examinations, MRI of the brain with post-processing of the data obtained by MR Voxel-based morphometry, determination of the level of melatonin in saliva using enzyme immunoassay. Results. The results of experimental psychological and neuropsychological examinations have shown the tendency of people with a pineal cyst to be absorbed in worries, thoughts about something, as well as to express anxiety. According to MR Voxel-based morphometry, it was found that during cystic transformation of the pineal gland, there are zones of increased cortical thickness in both parietal lobes, each of which is located in the central sections of the postcentral gyrus on the left and the superior parietal gyrus on the right. Conclusion. Psychological features in the presence of a pineal cyst indicate the need to include an experimental psychological examination in the protocol of patient management to identify risk groups for affective disorders. The data obtained by us on microstructural changes in the cortex of the parietal lobes during cystic transformation of the epiphysis indicate the variability of the normal structure of the brain substance and require further clarification. Keywords: brain, pineal cyst, anxiety, depression, melatonin, magnetic resonance imaging, magnetic resonance voxel-based morphometry.

Prospective investigation of the relationship between preoperative melatonin levels and typical symptoms in patients with pineal cysts

Prospective investigation of the relationship between preoperative melatonin levels and typical symptoms in patients with pineal cysts

Purely Endoscopic Supracerebellar Infratentorial Approach to the Pineal Region in Pediatric Population.

Pineal lesions represent less than 1% of all brain tumors (Villani et al., Clin Neurol Neurosurg 109:1-6, 2007). The abysmal location and critical neurovascular structures remain a surgical challenge, despite the advent of microneurosurgery. The classical wide surgical suboccipital craniotomy with the supracerebellar infratentorial approach, described by Sir Victor Horsley (Victor, Proc R Soc Med 3:77-78, 1910), is infamous for its considerable surgical morbidity and mortality. This was later upgraded microneurosurgically by Stein to improve surgical outcomes (Stein, J Neurosurg 35:197-202, 1971).Ruge et al. reported the first purely endoscopic fenestration of quadrigeminal arachnoid cysts via this corridor (Ruge et al., Neurosurgery 38:830-7, 1996). A cadaver-based anatomical study by Cardia et al. demonstrated the viability for endoscope-assisted techniques (Cardia et al., J Neurosurg 2006;104(6 Suppl):409-14). However, the first purely endoscopic supracerebellar infratentorial (eSCIT) approach to a pineal cyst was performed in 2008 by Gore et al. (Gore PA et al., Neurosurgery 62:108-9, 2008).Unlike transventricular endoscopy, eSCIT approach poses no mechanical risk to the fornices and can be utilized irrespective of ventricular size. More vascular control and resultant reduction in uncontrolled hemorrhage improve the feasibility of attaining complete resection, especially around corners (Zaidi et al,, World Neurosurg 84, 2015). Gravity-dependent positioning and cerebrospinal fluid (CSF) diversion aid cerebellar relaxation, creating the ideal anatomical pathway. Also, angle of the straight sinus, tentorium, and tectal adherence can often influence the choice of approach; thus direct endoscopic visualization not only counteracts access to the engorged Galenic complex but also encourages sharp dissection of the arachnoid (Cardia et al., J Neurosurg 104:409-14, 2006). These tactics help provide excellent illumination with magnification, making it less fatiguing for the surgeon (Broggi et al., Neurosurgery 67:159-65, 2010).The purely endoscopic approach thwarts the dreaded risk of air embolisms, via simple copious irrigation from a small burr hole (Shahinian and Ra, J Neurol Surg B Skull Base 74:114-7, 2013). The tiny opening and closure are rapid to create, and the smaller wound decreases postoperative pain and morbidity. Recent literature supports its numerous advantages and favorable outcomes, making it a tough contender to traditional open methods.

Surgical corridor formation by minimally invasive lateral occipital infracortical supra-/transtentorial (OICST) approach in pineal region tumor surgery: A review of 11 cases

IntroductionThe pineal region is a hard-to-reach part of the brain. There is no unequivocal opinion on the choice of a surgical approach to the pineal region. The surgical approaches described differ in both trajectory (infra- and supratentorial, interhemispheric) and size of craniotomy. They have advantages and disadvantages. The minimally invasive lateral occipital infracortical supra-/transtentorial (OICST) approach we have described has all the advantages of the standard supratentorial approach and minimizes its disadvantages, namely, compression and contusion of the occipital lobe. The minimally invasive craniotomy and small surgical corridor facilitate that. MethodsWe describe 11 consecutive patients with various pineal region tumors (7 cases of pineal cysts, 2 cases of pinealocytoma, 1 case of medulloblastoma, and 1 case of meningioma) who were operated on in our hospital using the lateral OICST approach. Preoperative planning was performed using Surgical Theater®. The surgical corridor was formed using a retractor made from half of a syringe shortened according to the length of the surgical corridor. Preoperative lumbar drain was used. ResultsThe pineal region tumors were completely resected in all cases. The mean craniotomy size was 2.22 × 1.79 cm. No long-term neurological deficits were reported. ConclusionsThe use of semicircular retractors and intraoperative CSF drainage via a lumbar drain allows to form a small surgical corridor to the pineal region via minimally invasive craniotomy. This reduces traction and traumatization of the occipital lobe, as well as minimizes intra- and postoperative risks.

Abstract 103: Acute Onset Rhythmic Tremor In Acute Frontal Cortical Infarction: A Case Report

Introduction Acute ischemic stroke varies in presentation, and it is crucial to quickly identify patients presenting with stroke for timely intervention. Secondary movement disorders occurring after a delayed period following ischemic stroke have been well‐documented, though rare. Holmes tremor specifically has been described as a delayed result of ischemic infarction involving subcortical structures such as mollaret triangle, thalamus, and basal ganglia. However, it is novel for frontal cortical infarcts to present with acute‐onset contralateral limb tremor, especially as an isolated symptom. Our case suggests a “secondary insult” involving the frontal cortex can disinhibit the motor pathway that leads to tremor in preexisting midbrain and thalamic lesions. We present a 67‐year‐old male with acute onset, constant, rhythmic tremor in the setting of acute ischemic frontal cortical infarction. Methods n/a, case report Results A 67‐year‐old male with medical history of pineal gland cyst status‐post shunt placement and hypertension who presented for evaluation of acute onset right upper extremity rhythmic tremor that started 24 hours prior. He did not have any associated weakness, numbness, vision changes, nausea, or vomiting. He had never had this tremor before. Neurologic examination was significant for a 4.0 Hz rhythmic resting tremor that was present throughout exam and was non‐distractible. This tremor remained consistent even with kinetic movements and postures. Electroencephalogram (EEG) was performed STAT during this tremor and was not based in seizure. Non‐contrast head computerized tomography (CT) was negative for any acute hemorrhage or intracranial pathology. CT angiogram head and neck showed left internal carotid artery (ICA) long segment tapered occlusion from the proximal cervical ICA to the distal petrous segment, likely dissection of undetermined chronicity. Magnetic resonance imaging (MRI) Brain with and without contrast was significant for acute punctate foci involving the precentral cortex of the left frontal lobe consistent with acute ischemic infarction. Encephalomalacia was also present involving the bilateral posterior thalami and left midbrain chronic ischemic infarctions. This suggests the tremor was caused by disinhibition of the motor pathway from the ischemic insult to the frontal cortex, as he had pre‐existing asymptomatic ischemic insult to the typical structures known to cause this tremor. He was outside of the Tenecteplase window and was given a load of Aspirin followed by aspirin 81 mg daily. He was trialed on propranolol for treatment of symptomatic tremor however this was discontinued before discharge as it was ineffective and patient preferred to hold off on other treatments. He is now awaiting outpatient Neurology follow up. Conclusion This case suggests that acute onset Holmes tremor can be the sole presenting sign in acute ischemic frontal cortical infarction. The pre‐existing asymptomatic infarctions in the midbrain and thalamus also support that a “second hit” located in the frontal cortex can disinhibit the motor pathway between the cortex and rubral tract. This is important as Holmes tremor is more commonly known to be from a mechanism of damage to the red nucleus or thalamic structures in a delayed manner, typically at least two weeks after stroke. This knowledge will help identify rare strokes in a timely manner.

PAEDIATRIC-07 IMAGING OF PINEAL REGION TUMOURS IN CHILDREN

Abstract Pineal tumors comprise between 3% and 8% of all pediatric brain tumors. Pineal region tumors can be divided into four categories: Germ cell tumors, pineal cysts, pineal parenchymal tumors, and tumors of the supporting tissues of the pineal gland or adjacent structures (such as pineal gliomas, dermoids, and epidermoids). The most common pineal region tumors are germ cell tumors, most are pure germinomas. Other variants such as nongerminomatous germ cell tumors, mixed germ cell tumors, teratomas, embryonal cell carcinoma, yolk sac tumor, and choriocarcinoma constitute the remainder. Germinomas are hyperdense on CT and enhance homogenously. The typical appearance on CT is a calcified pineal gland surrounded by noncalcified tumor. On MRI, these lesions have homogenous signal isointense to gray matter on all sequences with intense contrast enhancement. These tumors may infiltrate the thalami and midbrain. Spinal dissemination is seen in up to 36% of cases. Pineocytomas are well-differentiated tumors that retain morphologic features of the pineal parenchymal cells. They are slow-growing, circumscribed, but noncapsulated tumors. They are more commonly solid tumors, although cystic variants have been described. Solid tumors are either hypointense or isointense to gray matter and T2-hyperintense. Enhancement is homogenous and calcification is common. Cystic variants are often indistinguishable from pineal cysts. Pineoblastomas are malignant, unencapsulated tumors that resemble embryonal tumors NOS, but are distinct from such tumors in other locations due to their photosensory differentiation. On MRI, these lesions are hypointense to isointense on T1-weighted images and variable low, high, or mixed signal on T2-weighted images. They have lobulated contours and enhance homogenously. They calcify less often than pineocytomas. The “exploded pattern” of calcification with peripheral displacement of pineal gland calcification is considered more typical of pineal parenchymal tumors, differentiating them from germ cell tumors.

Pineal cysts in children: a paediatric series treated over the last twenty years in Lyon.

Pineal cysts are a rare lesion of the pineal gland. Pineal cysts are benign lesions, generally asymptomatic, and are usually an incidental discovery on MRI performed for other problems. The management of pineal cysts in children remains a matter for debate. Here, we report our own retrospective paediatric cases that have been surgically treated and review the paediatric literature on this topic. This is a retrospective monocentric study. All patients operated by the senior author (CM) for a benign pineal cyst from 2000 to 2021 were included. All other pineal region cystic lesions were excluded. Medical and surgical data were extracted from the hospital medical database. Twelve patients were included. The clinical symptomatology was characterized by headaches in seven patients, visual troubles in two patients, precocious puberty in one patient, signs of intracranial hypertension in two patients, seizures associated with headache in one patient, and headaches associated with behavioural troubles in another patient. No major post-operative complications were observed in this series. It is to noted that surgery was performed because a suspicion of a true pineal parenchymal tumour has been made. Histopathological study came back with the diagnosis of pineal cyst. Pineal cyst is rare. If the radiological diagnosis is clear, no surgery is advocated except in cases associated with hydrocephalus and rapid growth. In case of a suspicion of a true pineal parenchymal tumour, a surgery may be needed to confirm the diagnosis. Lastly, we stress that only cystic lesions of the pineal gland itself should be considered as pineal cyst.

THU177 Brain Magnetic Resonance Imaging Abnormalities And Effect On Growth Response In Children With Growth Hormone Deficiency

Abstract Disclosure: J. Choe: None. J. Lee: None. J. Kim: None. Purpose: There have been several studies on the association between structural abnormalities of the pituitary gland and its function. However, no studies have yet been conducted on children with idiopathic growth hormone deficiency (GHD) in Korea. We investigated clinical and laboratory findings of Korean children of idiopathic GHD by the presence or absence of pathologies in the pituitary gland. Material/Methods: A total of 479 patients were included from the LG growth study database. Participants had idiopathic GHD with available pituitary magnetic resonance imaging (MRI) and were treated with GH for at least 1 year between Feb, 2001 and Feb, 2020. Clinical characteristics were retrieved from the LG growth study database. Patients were divided into 3 groups according to pituitary MRI findings: (1) pathological findings in the pituitary gland; (2) pathological findings outside the pituitary gland; (3) without pathological findings with MRI. Results: Of the 479 patients, 297 (62.0%) were male and 198 (83.5%) were prepubertal. Their mean age was 7.56±2.95 years (2-16 years) and mean age of GH treatment was 7.56±2.96 years. Pituitary MR images of 354 (73.9%) patients were normal. Of the patients with detected pathologies (26.1%), 77 (61.6%) had pituitary hypoplasia, 15 (12.0%) had Rathke’s cleft cyst, 6 (0.05%) had partial empty sella, 6 (0.05%) had an arachnoid cyst, 3 (0.02%) had ectopic neurohypophysis, and 3 (0.02%) had a pineal cyst. There was no statistically significant difference in the height velocity after one year of GH treatment between groups with or without pituitary pathology (height SDS 0.71±0.34 with pituitary pathology and 0.66±0.35 with pituitary pathology; p=0.09). Conclusions: Among Korean children with GHD, approximately one-quarter of patients had abnormal pituitary MRI findings. There were no significant differences in predicting GH treatment response regardless of pituitary MRI abnormalities. Further long-term studies are needed. Presentation: Thursday, June 15, 2023

THU192 Prevalence Of Brain Abnormalities In Children With Central Precocious Puberty

Abstract Disclosure: A.A. Bastos: None. A.P. Machado Canton: None. L.B. Macena: None. L.F. Pedrosa: None. B.B. Mendonca: None. V.N. Brito: None. A. Latronico: None. Introduction: Brain magnetic resonance imaging (MRI) is the main tool used to investigate the etiology of central precocious puberty (CPP) in children. A systematic review study showed an overall prevalence of 9% of brain lesions in girls with CPP. Aim: To assess the frequency brain lesions in children with CPP. Patients and Methods: In this retrospective study, we recruited data of 163 brain MRI of children with CPP (140 girls,86%; and 23 boys, 14%) followed in a pediatric endocrinology unit from 1990 to 2022. Brain lesions were classified as congenital or acquired, and causative of CPP or incidental. Results: In girls, normal brain MRI was found in 82/140 (59%). Abnormal brain MRI was identified in the remaining 58 girls (41%), with 26 presenting congenital lesions causative of CPP (11 hypothalamic hamartoma, 7 hydrocephalus, 3 optic pathway gliomas, 2 corpus callosum agenesia, 1 pinealoma, 1 tuberous sclerosis, 1 choroid plexus papilloma), and 32 with incidental findings (microadenomas, pituitary stalk deviation, pituitary hyperplasia, rathke cleft cyst, hippocampal alterations). The overall frequency of incidental findings was 20.8%. In addition, the frequency of brain MRI pathological abnormalities in girls older than 6 years without neuro ophthalmological symptoms was 12%(n=7). In boys, normal brain MRI was found in 6/23 (26%). Abnormal brain MRI was identified in the remaining 17 boys, with 14 presenting congenital lesions causative of CPP (9 hypothalamic hamartoma, 4 hydrocephalus, 1 pineal teratoma) and 1 with acquired lesions (1 pilocytic astrocytoma). Incidental findings were detected in only 2 brain MRI (1 pineal cyst and 1 microadenoma). Incidentalomes were more frequent in girls and lesions that can be associated with CPP were more common in boys. Conclusion: The frequency of pathological brain lesions was higher in CPP boys. Hypothalamic hamartoma was the most frequent congenital lesion identified in both sexes. Based on the frequency of pathological brain alterations in girls older than 6 years we recommend to perform brain MRI in all CPP girls. Presentation: Thursday, June 15, 2023