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Neuro-Chirurgie 2015Pineal tumor management in pediatric patients must be based on close co-operation between oncologists, surgeons, radiation oncologists, neurologists, ophthalmologists,... (Review)
Review
Pineal tumor management in pediatric patients must be based on close co-operation between oncologists, surgeons, radiation oncologists, neurologists, ophthalmologists, and endocrinologists. Radiation therapy (RT) remains critical in most situations and should be assessed as soon as the diagnosis is made, in order to optimize the radiation technique. This paper will focus on RT modalities, indications, as well as modalities in main pediatric pineal tumors (germ cell tumors and pineal parenchyma tumors). RT modalities are presently being debated and new RT techniques (intensity-modulated RT, proton therapy etc.) that are now available for pineal lesions need to be evaluated. Radiation strategies are also controversial for germ cell tumors: cranio-spinal radiation versus chemotherapy followed by focal radiation, which also requires discussion.
Topics: Brain Neoplasms; Combined Modality Therapy; Humans; Neoplasms, Germ Cell and Embryonal; Pineal Gland; Pinealoma; Treatment Outcome
PubMed: 25612810
DOI: 10.1016/j.neuchi.2014.11.002 -
Child's Nervous System : ChNS :... Dec 2023Pineal 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...
BACKGROUND
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.
METHODS
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.
RESULTS
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.
CONCLUSIONS
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.
Topics: Humans; Child; Pinealoma; Cysts; Retrospective Studies; Central Nervous System Cysts; Brain Neoplasms; Magnetic Resonance Imaging; Pineal Gland; Headache
PubMed: 37898987
DOI: 10.1007/s00381-023-06181-y -
Human Pathology Mar 2014Glial cysts of the pineal gland can frequently be found in adults and children, but only rarely do they enlarge to become clinically relevant. We report a unique...
Glial cysts of the pineal gland can frequently be found in adults and children, but only rarely do they enlarge to become clinically relevant. We report a unique presentation of a pineal cyst in the midbrain tectum of a 16-month-old girl who initially presented with ptosis and strabismus. Preoperative imaging studies and intraoperative findings revealed no continuity between the tectal cyst and the pineal gland proper. We surmise that this tectal pineal cyst may have arisen from duplicated pineal gland tissue.
Topics: Brain Diseases; Cysts; Female; Humans; Infant; Pineal Gland; Tectum Mesencephali; Treatment Outcome
PubMed: 24411061
DOI: 10.1016/j.humpath.2013.10.002 -
Advances in Experimental Medicine and... 2023Pineal region tumors fall into five broad categories: benign pineal region tumors, glial tumors, papillary tumors, pineal parenchymal tumors, and germ cell tumors....
Pineal region tumors fall into five broad categories: benign pineal region tumors, glial tumors, papillary tumors, pineal parenchymal tumors, and germ cell tumors. Genetic and transcriptional studies have identified key chromosomal alterations in germinomas (RUNDC3A, ASAH1, LPL) and in pineocytomas/pineoblastomas (DROSHA/DICER1, RB1). Pineal region tumors generally present with symptoms of hydrocephalus including nausea, vomiting, papilledema, and the classical Parinaud's triad of upgaze paralysis, convergence-retraction nystagmus, and light-near pupillary dissociation. Workup requires neuroimaging and tissue diagnosis via biopsy. In germinoma cases, diagnosis may be made based on serum or CSF studies for alpha-fetoprotein or beta-HCG making the preferred treatment radiosurgery, thereby preventing the need for unnecessary surgeries. Treatment generally involves three steps: CSF diversion in cases of hydrocephalus, biopsy through endoscopic or stereotactic methods, and open surgical resection. Multiple surgical approaches are possible for approach to the pineal region. The original approach to the pineal region was the interhemispheric transcallosal first described by Dandy. The most common approach is the supracerebellar infratentorial approach as it utilizes a natural anatomic corridor for access to the pineal region. The paramedian or lateral supracerebellar infratentorial approach is another improvement that uses a similar anatomic corridor but allows for preservation of midline bridging veins; this minimizes the chance for brainstem or cerebellar venous infarction. Determination of the optimal approach relies on tumor characteristics, namely location of deep venous structures to the tumor along with the lateral eccentricity of the tumor. The immediate post-operative period is important as hemorrhage or swelling can cause obstructive hydrocephalus and lead to rapid deterioration. Adjuvant therapy, whether chemotherapy or radiation, is based on tumor pathology. Improvements within pineal surgery will require improved technology for access to the pineal region along with targeted therapies that can effectively treat and prevent recurrence of malignant pineal region tumors.
Topics: Humans; Pinealoma; Pineal Gland; Brain Neoplasms; Glioma; Hydrocephalus; Ribonuclease III; DEAD-box RNA Helicases
PubMed: 37452938
DOI: 10.1007/978-3-031-23705-8_6 -
Journal of Neuro-oncology Sep 2001The pineal region is a heterogeneous area that includes the pineal gland and several parapineal structures. Pineal region masses include germ cell tumors (GCTs), pineal... (Review)
Review
The pineal region is a heterogeneous area that includes the pineal gland and several parapineal structures. Pineal region masses include germ cell tumors (GCTs), pineal parenchymal cell tumors, glioma, meningioma, metastases, and non-neoplastic masses such as pineal cysts, lipoma, epidermoid, vascular malformations. Although MRI has allowed an improvement in distinguishing true pineal masses from parapineal masses impinging into the region of the gland, it is still difficult to differentiate the pineal GCTs from pineal parenchymal tumors with only images, and the clinical informations such as age, sex, and the tumor markers are very important.
Topics: Brain Diseases; Brain Neoplasms; Cysts; Germinoma; Humans; Magnetic Resonance Imaging; Pineal Gland; Pinealoma
PubMed: 11767291
DOI: 10.1023/a:1012773727022 -
Journal of Neurosurgery Dec 1992The authors present a series of six patients with large symptomatic benign pineal cysts and review the 27 patients previously reported in the literature. Patients with... (Review)
Review
The authors present a series of six patients with large symptomatic benign pineal cysts and review the 27 patients previously reported in the literature. Patients with symptomatic pineal cysts most often present with one of three syndromes: 1) paroxysmal headache with gaze paresis; 2) chronic headache, gaze paresis, papilledema, and hydrocephalus; or 3) pineal apoplexy with acute hydrocephalus. Surgical intervention with radical cyst removal is the treatment of choice for all symptomatic pineal cysts. Complete cyst removal is desirable; however, radical subtotal resection is appropriate if the cyst cannot be easily separated from the quadrigeminal plate. Ventricular shunting should be reserved for patients with persistent hydrocephalus after cyst resection.
Topics: Adolescent; Adult; Brain Diseases; Child, Preschool; Cysts; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Microsurgery; Pineal Gland; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 1432132
DOI: 10.3171/jns.1992.77.6.0896 -
World Neurosurgery Dec 2020A 37-year-old man with no medical history, apart from a lifelong horizontal diplopia, underwent brain magnetic resonance imaging, which revealed a curvilinear posterior...
A 37-year-old man with no medical history, apart from a lifelong horizontal diplopia, underwent brain magnetic resonance imaging, which revealed a curvilinear posterior pericallosal lipoma and a pineal cyst. Corpus callosum lipomas, with an incidence of 0.1%-0.5%, do not require neurosurgical treatment, except in rare cases of malformations causing epilepsy. The indications for surgery of pineal cysts, whose incidence is 0.6%, are controversial, except in case of hydrocephalus. In this case, ophthalmologic examination and magnetic resonance imaging were not consistent for a tectal plate compression and the findings were considered incidental. The association of these 2 abnormalities, which has not been described before, might reflect an interrelated embryologic development for pineal gland and midline lipomas, which derive from aberrant mesenchymal primitive meninx.
Topics: Adult; Brain Neoplasms; Central Nervous System Cysts; Corpus Callosum; Humans; Lipoma; Magnetic Resonance Imaging; Male; Pineal Gland; Strabismus
PubMed: 32805468
DOI: 10.1016/j.wneu.2020.08.071 -
Radiographics : a Review Publication of... May 1992Most pineal region masses are malignant germ cell neoplasms that occur in young male patients. The most common is a germinoma, which is a homogeneous mass with signal... (Review)
Review
Most pineal region masses are malignant germ cell neoplasms that occur in young male patients. The most common is a germinoma, which is a homogeneous mass with signal intensity and attenuation similar to those of gray matter; the mass engulfs a densely calcified pineal gland. Teratomas are multilocular heterogeneous masses containing lipid areas. Other types of pineal region masses include choriocarcinoma, endodermal sinus tumor, and embryonal carcinoma. Pineal parenchymal neoplasms are usually either pineocytomas or pineoblastomas, which may "explode" preexisting pineal calcifications. Unlike the germinomas, they have no sexual predilection and may be seen in patients who are 20 years of age or older. Although the correct histologic diagnosis may be suggested with a careful evaluation of the morphologic features, attenuation, and signal intensity characteristics, very few of these tumors have a truly pathognomonic imaging pattern. Thus, histologic verification is necessary for most pineal region masses that appear to be neoplastic.
Topics: Brain Diseases; Brain Neoplasms; Diagnosis, Differential; Humans; Pineal Gland; Tomography, X-Ray Computed
PubMed: 1609147
DOI: 10.1148/radiographics.12.3.1609147 -
Headache Mar 2008Pineal cysts have been suggested to be linked to headache pathogenesis. However, no systematic studies on this topic have been performed so far.
BACKGROUND
Pineal cysts have been suggested to be linked to headache pathogenesis. However, no systematic studies on this topic have been performed so far.
OBJECTIVE
The aim of this study was to investigate the relationship between pineal cysts and headache and to determine the relevance of the cyst size in this context.
METHODS
We conducted a case-control study of patients who consulted the neurological department between 1999 and 2006 to investigate the relationship between pineal cysts and headache and to determine the relevance of the cyst size.
RESULTS
A total of 51 patients with pineal cyst were identified, thereby 51% were suffering from headache (half of them had migraine), whereas in the control group, only 25% had headache (thereof 31% suffered from migraine). There was a significant relationship between headache (as well as migraine) and pineal cysts (chi-square test), but no significant difference in pineal cyst diameter between the patient group with headache versus without headache (t-test).
CONCLUSIONS
Our data provide evidence for a causal relationship between pineal cysts and headache, respectively, migraine in this patient cohort.
Topics: Adult; Case-Control Studies; Cysts; Female; Headache; Humans; Male; Pineal Gland
PubMed: 18005138
DOI: 10.1111/j.1526-4610.2007.00965.x -
Molecular Genetics & Genomic Medicine Jun 2021Pineal cyst is a benign lesion commonly occurring in people of any age. Until now, the underlying molecular alterations have not been explored.
BACKGROUND
Pineal cyst is a benign lesion commonly occurring in people of any age. Until now, the underlying molecular alterations have not been explored.
METHODS
We performed whole exome sequencing of 93 germline samples and 21 pineal cyst tissue samples to illustrate its genetic architecture and somatic mutations. The dominant and recessive inheritance modes were considered, and a probability was calculated to evaluate the significance of variant overrepresentation.
RESULTS
By analyzing pineal cyst as a Mendelian disease with a dominant inheritance pattern, we identified 42,325 rare germline variants, and NM_001004711.1:c.476A>G was highly enriched (FDR<0.2). By analyzing it as a recessive disorder, we identified 753 homozygous rare variants detected in at least one pineal cyst sample each. One STIM2 rare variant, NM_001169117.1:c.1652C>T, was overrepresented (FDR<0.05). Analyzing at a gene-based level, we identified a list of the most commonlymutated germline genes, including POP4, GNGT2 and TMEM254. A somatic mutation analysis of 21 samples identified 16 variants in 15 genes, which mainly participated in the biological processes of gene expression and epigenetic regulation, immune response modulation, and transferase activity.
CONCLUSION
These molecular profiles are novel for this condition and provide data for investigators interested in pineal cysts.
Topics: Adolescent; Adult; Cysts; Female; GTP-Binding Protein gamma Subunits; Germ-Line Mutation; Heterozygote; Homozygote; Humans; Male; Middle Aged; Phenotype; Pineal Gland; Ribonucleases; Ribonucleoproteins; Stromal Interaction Molecule 2; Exome Sequencing
PubMed: 33943042
DOI: 10.1002/mgg3.1691