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Clinical Neurology and Neurosurgery Jun 2024Pure germinoma typically originates from the midline and is usually found in the pineal and suprasellar regions in 76-90 % of cases. When it is in both regions, it is... (Review)
Review
BACKGROUND
Pure germinoma typically originates from the midline and is usually found in the pineal and suprasellar regions in 76-90 % of cases. When it is in both regions, it is considered bifocal (10 % at diagnosis). If pure germinoma is located outside of the midline, it is considered ectopic, with a global incidence of about 0.7 %. The study aims to describe the clinical and surgical approach to patients with atypical intracranial ectopic germinoma (IEG) and bifocal germinoma (BG) through a literature review with the goal to delineate the correct diagnostic and therapeutic pathway, to reduce the diagnostic delay and improve the prognosis of these patients.
METHODS
A systematic review of the literature in most common electronic database (PubMed, Ovid MEDLINE and Ovid EMBASE) on IEG and BG, in according with the "PRISMA statement" criteria, from January 1990 to September 2022 was done. In addition, two rare cases of IEG and BG were reported.
RESULTS
This systematic review included 16 papers (20 patients) with a final diagnosis of IEG and 30 papers (121 patients) with a final diagnosis of BG. IEGs seems to involve primary basal ganglia (40 %) and corpus callosum (40 %). For IEGs, biopsy (70 %, 14 cases out of 20) was the most common surgical approach: open approach (35 %), stereotactic minimally invasive approach (30 %) or endoscopic trans-sphenoidal approach (5 %). Partial resection was performed in 10 % of cases, whereas a total resection was performed in 20 % of cases. Also for BGs, biopsy was the most common surgical approach in 80 % of patients, whereas surgical resection (partial or total) was performed in 5.3 % of patients.
CONCLUSION
IEG and BG are rare type of primary intracranial germ cell tumor, whose unusual location often can cause delays in diagnosis, which can have a significant impact on the patient's prognosis and requiring a multidisciplinary and timely approach.
PubMed: 38954867
DOI: 10.1016/j.clineuro.2024.108408 -
World Neurosurgery Dec 2017Tumors arising from the pineal region account for approximately 1% of intracranial neoplasms. We present a case of a previously healthy 5-year-old boy with an acute... (Review)
Review
BACKGROUND
Tumors arising from the pineal region account for approximately 1% of intracranial neoplasms. We present a case of a previously healthy 5-year-old boy with an acute onset of headache. A magnetic resonance imaging (MRI) scan showed a pineal mass with aqueduct compression. The patient was scheduled for tumor resection. An endoscopic third ventriculostomy was performed in advance for the treatment of hydrocephalus. Afterwards, MRI showed a relevant regression of the pineal mass without specific treatment. Consequently, surgery was cancelled and further MRI follow-up showed a regression of the mass and a constant tumor mass over a period of 30 months. Spontaneous regression of malignant tumors is a rare phenomenon with an incidence of 1 of 60,000-100,000 cases. Only a few cases with spontaneous regression of pineal tumors have been reported.
METHODS
We performed a systematic literature review according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) guidelines on spontaneously regressing pineal lesions and found 13 cases in the literature.
RESULTS
Six hypotheses for explaining tumor regression were found, comprising treatment with steroids, effects of diagnostic irradiation, treatment of hydrocephalus, pineal apoplexy, surgical trauma, and immunologic mechanisms. None of these mechanisms was evidentiary. However, in all reported cases, some kind of treatment (e.g. treatment of hydrocephalus, application of steroids, and so on) has been performed before tumor regression.
CONCLUSIONS
The clinician has to bear in mind that regression of pineal tumors might be triggered by use of steroids, for example, and in cases of improvement of the patient's presenting symptoms, new MRI scans should be performed.
Topics: Brain Neoplasms; Cerebral Aqueduct; Child, Preschool; Consciousness Disorders; Diffusion Magnetic Resonance Imaging; Headache; Humans; Hydrocephalus; Magnetic Resonance Imaging; Male; Neoplasm Regression, Spontaneous; Pineal Gland; Pinealoma; Ventriculostomy; Vomiting
PubMed: 28844909
DOI: 10.1016/j.wneu.2017.08.106 -
International Journal of Radiation... May 2016Because it spares many normal tissues and reduces the integral dose, proton therapy (PT) is the preferred tumor irradiation technique for treating childhood cancer.... (Review)
Review
Because it spares many normal tissues and reduces the integral dose, proton therapy (PT) is the preferred tumor irradiation technique for treating childhood cancer. However, to the best of our knowledge, no systematic review of the clinical effectiveness of PT in children has been reported in the scientific literature. A systematic search for clinical outcome studies on PT published between 2007 and 2015 was performed in Medline (through OVID), EMBASE, and the Cochrane Library. Twenty-three primary studies were identified, including approximately 650 patients overall. The median/mean follow-up times were limited (range, 19-91 months). None of the studies were randomized, 2 were comparative, and 20 were retrospective. Most suffered from serious methodologic limitations, yielding a very low level of clinical evidence for the outcomes in all indications. For example, for retinoblastoma, very low-level evidence was found that PT might decrease the incidence of second malignancies. For chondrosarcoma, chordoma, craniopharyngioma, ependymoma, esthesioneuroblastoma, Ewing sarcoma, central nervous system germinoma, glioma, medulloblastoma, osteosarcoma, and rhabdomyosarcoma, there was insufficient evidence to either support or refute PT in children. For pelvic sarcoma (ie, nonrhabdomyosarcoma and non-Ewing sarcoma), pineal parenchymal tumor, primitive neuroectodermal tumor, and "adult-type" soft tissue sarcoma, no studies were identified that fulfilled the inclusion criteria. Although there is no doubt that PT reduces the radiation dose to normal tissues and organs, to date the critical clinical data on the long-term effectiveness and harm associated with the use of PT in the 15 pediatric cancers under investigation are lacking. High-quality clinical research in this area is needed.
Topics: Adolescent; Child; Child, Preschool; Follow-Up Studies; Humans; Infant; Infant, Newborn; Neoplasms; Organ Sparing Treatments; Organs at Risk; Proton Therapy; Radiation Injuries; Retrospective Studies; Time Factors; Young Adult
PubMed: 27084646
DOI: 10.1016/j.ijrobp.2015.10.025