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Journal of Neuro-oncology Apr 2023Pineal parenchymal tumors of intermediate differentiation (PPTIDs), which were recognized in the 2007 World Health Organization (WHO) classification, are rare,...
PURPOSE
Pineal parenchymal tumors of intermediate differentiation (PPTIDs), which were recognized in the 2007 World Health Organization (WHO) classification, are rare, accounting for less than 1% of all central nervous system tumors. This rarity and novelty complicate the diagnosis and treatments of PPTID. We therefore aimed to evaluate the clinicopathological significance of this tumor.
METHODS
At 11 institutions participating in the Kyushu Neuro-Oncology Study Group, data for patients diagnosed with PPTID were collected. Central pathology review and KBTBD4 mutation analysis were applied to attain the diagnostically accurate cohort.
RESULTS
PPTID was officially diagnosed in 28 patients: 11 (39%) with WHO grade 2 and 17 (61%) with WHO grade 3 tumors. Median age was 49 years, and the male:female ratio was 1:2.1. Surgery was attempted in all 28 patients, and gross total resection (GTR) was achieved in 46% (13/28). Adjuvant radiotherapy and chemotherapy were administered to, respectively, 82% (23/28) and 46% (13/28). The 5-year progression-free survival (PFS) and overall survival rates were 64.9% and 70.4% respectively. Female sex (p = 0.018) and GTR (p < 0.01) were found to be independent prognostic factors for PFS and female sex (p = 0.019) was that for OS. Initial and second recurrences were most often leptomeningeal (67% and 100% respectively). 80% (20/25) of patients harbored a KBTBD4 mutation.
CONCLUSIONS
Female sex and GTR were independent prognostic factors in our patients with PPTID. Leptomeningeal recurrence was observed to be particularly characteristic of this tumor. The rate of KBTBD4 mutation observed in our cohort was acceptable and this could prove the accuracy of our PPTID cohort.
Topics: Humans; Male; Female; Middle Aged; Pinealoma; Brain Neoplasms; Cohort Studies; Progression-Free Survival; Pineal Gland; Retrospective Studies
PubMed: 37052748
DOI: 10.1007/s11060-023-04310-w -
Rozhledy V Chirurgii : Mesicnik... 2016The pineal region is a deep-seated part of the brain surrounded by highly eloquent structures. Differential diagnosis of space-occupying lesions in this region... (Review)
Review
INTRODUCTION
The pineal region is a deep-seated part of the brain surrounded by highly eloquent structures. Differential diagnosis of space-occupying lesions in this region encompasses pineal gland cysts, pineal gland tumours, metastases, germ cell tumours, meningiomas, gliomas, hemangioblastomas and neuroectodermal tumours. A treatment strategy is based mainly on tumour anatomical characteristics and histological type. Except germinatous tumours, a surgical excision is the treatment of choice.
METHODS
Microsurgical approaches: The microsurgical supracerebellar-infratentorial approach is an essential approach to the pineal region. Despite certain risks, it allows a straightforward and completely extracerebral approach with a minimal cerebellar retraction. The other basic approach is the microsurgical occipital-transtentorial approach that is advantageous in patients with a supratentorial tumour extension or a steep tentorium. The interhemispheric-transcallosal approach and the transcortical-transventricular approach are possible options in selected cases.Endoscopic approaches: The neuroendoscopy provides a minimally invasive method to perform a tumour biopsy and to treat hydrocephalus in one session. Stereotactic biopsy: The stereotactic needle biopsy represents an alternative to the endoscopic biopsy in patients without hydrocephalus and in patients with dorsally located lesions inaccessible from the third ventricle.
CONCLUSION
Modern neurosurgery offers a rich variety of surgical approaches to the pineal region. The complexity of space-occupying lesions in this region requires an individualised treatment, a prudent preoperative planning and a meticulous surgical technique.
Topics: Humans; Biopsy; Brain Neoplasms; Diagnosis, Differential; Microsurgery; Neuroendoscopy; Neurosurgical Procedures; Patient Care Planning; Pineal Gland; Pinealoma; Risk Adjustment; Stereotaxic Techniques
PubMed: 27650561
DOI: No ID Found -
Journal of Neurosurgery. Pediatrics Aug 2023Pineal region tumors account for 2.7%-11% of all CNS tumors in children. In this series, the authors present their surgical results and long-term outcomes from a...
OBJECTIVE
Pineal region tumors account for 2.7%-11% of all CNS tumors in children. In this series, the authors present their surgical results and long-term outcomes from a pediatric pineal region tumor cohort.
METHODS
A total of 151 children aged 0-18 years were treated from 1991 to 2020. Tumor markers were collected in all patients; if positive, chemotherapy was performed, and if negative, biopsy was performed, preferably endoscopically. Resection was performed when there was a residual germ cell tumor (GCT) lesion after chemotherapy.
RESULTS
The distribution based on histological type, as verified by markers, biopsy, or surgery, was germinoma (33.1%), nongerminomatous GCT (NGGCT) (27.2%), pineoblastoma (22.5%), glioma (12.6%), and embryonal tumor (atypical teratoid rhabdoid tumor) (3.3%). A total of 97 patients underwent resection, and gross-total resection (GTR) was achieved in 64%; the highest GTR rate (76.6%) was found in patients with GCTs, and the lowest (30.8%) was found in those with gliomas. The supracerebellar infratentorial approach (SCITA) was the most common, performed in 53.6% of patients, followed by the occipital transtentorial approach (OTA), performed in 24.7% of patients. Lesions were biopsied in 70 patients, and the diagnostic accuracy was 91.4. The overall survival (OS) rates at 12, 24, and 60 months as stratified by histological type were 93.7%, 93.7%, and 88% for patients with germinomas; 84.5%, 63.5%, and 40.7% for patients with pineoblastomas; 89.4%, 80.8%, and 67.2% for patients with NGGCTs; 89.4%, 78.2%, and 72.6% for patients with gliomas; and 40%, 20%, and 0% for patients with embryonal tumors, respectively (p < 0001). The OS at 60 months was significantly higher in the group with GTR (69.7%) than in the group with subtotal resection (40.8%) (p = 0.04). The 5-year progression-free survival was 77% for patients with germinomas, 72.6% for patients with gliomas, 50.8% for patients with NGGCTs, and 38.9% for patients with pineoblastomas.
CONCLUSIONS
The efficacy of resection varies by histological type, and complete resection is associated with higher OS rates. Endoscopic biopsy is the method of choice for patients presenting with negative tumor markers and hydrocephalus. For tumors restricted to the midline and with extension to the third ventricle, a SCITA is preferred, whereas for lesions with extension toward the fourth ventricle, an OTA is preferred.
Topics: Male; Child; Humans; Pinealoma; Pineal Gland; Glioma; Germinoma; Brain Neoplasms
PubMed: 37148224
DOI: 10.3171/2023.3.PEDS22468 -
British Journal of Neurosurgery 2016Pineal region lesions are uncommon, and pineal parenchymal tumours (PPT) account for 20-30% of tumours in this area of which pineocytomas (PCs) and pineoblastomas (PBs)... (Review)
Review
Pineal region lesions are uncommon, and pineal parenchymal tumours (PPT) account for 20-30% of tumours in this area of which pineocytomas (PCs) and pineoblastomas (PBs) are more prevalent. In 2007, the World Health Organisation (WHO) reclassified PPT from two subgroups (PC and PB) into four, including pineal parenchymal tumours of intermediate differentiation (PPTID). PPTID have been further divided into low- and high-grade lesions (WHO II and III), but due to their rarity have proven difficult lesions to diagnose and a paucity of literature means their optimal treatment options are a challenge to define. This article is a review of the literature of PPTID highlighting diagnostic criteria, a discussion on the role of surgery and radiotherapy, including treatment paradigms and reported outcomes for these problematic neoplasms.
Topics: Brain Neoplasms; Humans; Pineal Gland; Pinealoma; Radiotherapy; Treatment Outcome; World Health Organization
PubMed: 26571134
DOI: 10.3109/02688697.2015.1096912 -
Neuro-Chirurgie 2015The management of pineal tumors is a model for multidisciplinarity. Apart from an emergency situation that requires immediate shunting of cerebrospinal fluid (CSF), the... (Review)
Review
The management of pineal tumors is a model for multidisciplinarity. Apart from an emergency situation that requires immediate shunting of cerebrospinal fluid (CSF), the initial discussion should involve at least a radiologist, a surgeon, a neurologist and an oncologist. The initial decision is whether obtaining a histological proof is obligatory. It depends on age and ethnicity, site (mono- or bifocality), presence of markers in serum as well as CSF, and/or of malignant cells in the CSF. In cases of marker elevation indicating a germ cell tumor, front line chemotherapy can avoid dangerous immediate surgery. When histological proof is required, the extent of surgery should be discussed, aiming either only at obtaining tissue or removal. If a germ cell tumor is detected, treatment will include a cisplatin-containing chemotherapy followed by focal or ventricular irradiation. Tumors of the pineal parenchyma will be treated according to grade, either by surgery alone (pinealocytoma) or chemo-radiotherapy (pinealoblastomas). Similarly, gliomas will be treated depending on their grade with several different possible lines in low grade, and usually radio-chemotherapy in high grade. A careful balance between improved survival rates and decreased long-term side effects will guide the decisions of all these specialists.
Topics: Brain Neoplasms; Disease Management; Glioma; Humans; Neoplasms, Germ Cell and Embryonal; Pinealoma; Survival Rate
PubMed: 24863688
DOI: 10.1016/j.neuchi.2014.03.003 -
Turkish Neurosurgery 2015Primary pineal melanoma is a rare tumor. We herein review the histogenesis, pathology, radiology and therapeutic options of this rare tumor. (Review)
Review
AIM
Primary pineal melanoma is a rare tumor. We herein review the histogenesis, pathology, radiology and therapeutic options of this rare tumor.
MATERIAL AND METHODS
We conducted a PUBMED search using a combination of keywords such as "primary pineal melanoma", "CNS melanoma", and "pineal tumor" and identified 16 cases of primary pineal melanoma. Clinical features, pathologic characteristics and treatment details of these patients were noted from respective case reports. We also describe a case of a 45-year-old Indian woman with primary pineal melanoma treated with a combination of surgery and post-op radiation.
RESULTS
The median age at presentation is 50 years. Median duration of symptoms is 6 weeks. Common symptoms at presentation include headache (58.8%), personality changes (41.2%), gait disturbance (35.3%) and Parinaud's syndrome (29.4%). Surgery, radiotherapy and chemotherapy have been used in 29.4%, 47.1% and 23.5% of patients respectively. Median overall survival is 56 weeks. Leptomeningeal dissemination and ventricular ependymal spread were noted in 70.6% and 35.3% patients, respectively.
CONCLUSION
Combined modality treatment comprising maximal safe surgery and post-operative radiation should be preferred in patients with localized pineal melanoma without leptomeningeal dissemination. Taking a cue from other subsites of melanoma, chemotherapy can perhaps be deferred until recurrence.
Topics: Female; Humans; Melanoma; Middle Aged; Pinealoma
PubMed: 26014001
DOI: 10.5137/1019-5149.JTN.6568-12.1 -
Der Radiologe Aug 2014The (supra) sellar and pineal regions are relatively common sites for pediatric brain tumors; however, the distinct spectrum of space-occupying lesions is in marked... (Review)
Review
The (supra) sellar and pineal regions are relatively common sites for pediatric brain tumors; however, the distinct spectrum of space-occupying lesions is in marked contrast to that of the adult population. Craniopharyngeoma represents the most common intrasellar and/or suprasellar lesion in children, whereas the pituitary adenoma typical for adults- is generally rare. The pineal region is a predilection site for germ cell tumors in pediatric patients, while in adults this cancer category is largely confined to the gonads. The role of imaging is in the early detection of lesions, adequate demonstration of the characteristics and delineation of the complex anatomical relationship to adjacent vital structures.
Topics: Child; Child, Preschool; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Magnetic Resonance Spectroscopy; Male; Pineal Gland; Pinealoma; Pituitary Neoplasms
PubMed: 25081918
DOI: 10.1007/s00117-014-2668-9 -
Acta Neurologica Scandinavica Dec 2017The significance of MRI findings of patients with Parinaud syndrome (PS) with respect to clinical characteristics is poorly defined. Over the past decades, all patients...
INTRODUCTION
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.
METHODS
Medical records of patients with PS evaluated between 2000 and 2016 were retrospectively reviewed.
RESULTS
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.
CONCLUSIONS
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.
Topics: Adult; Brain Stem; Female; Fourth Ventricle; Humans; Hydrocephalus; Magnetic Resonance Imaging; Male; Middle Aged; Ocular Motility Disorders; Pinealoma
PubMed: 28653396
DOI: 10.1111/ane.12795 -
Neuro-Chirurgie 2015In cases of pineal tumors, a germ cell tumor should always be suspected. As some of them are secreting tumors, tumoral markers (AFP and/or hGC) are an important part of... (Review)
Review
In cases of pineal tumors, a germ cell tumor should always be suspected. As some of them are secreting tumors, tumoral markers (AFP and/or hGC) are an important part of the diagnostic process. Their positivity either in the serum and/or in the cerebrospinal fluid may lead to an accurate diagnosis, avoiding a potentially dangerous surgical biopsy. Follow-up of tumoral markers is useful during and after treatment in order to monitor response to chemotherapy or a remission status.
Topics: Biomarkers, Tumor; Biopsy; Brain Neoplasms; Humans; Neoplasms, Germ Cell and Embryonal; Pineal Gland; Pinealoma
PubMed: 24856314
DOI: 10.1016/j.neuchi.2013.12.006 -
World Neurosurgery Jun 2016The natural history of incidental pineal cysts is poorly understood. Neurosurgeons and neuroradiologists are more frequently faced with this disease in the advent of...
OBJECTIVE
The natural history of incidental pineal cysts is poorly understood. Neurosurgeons and neuroradiologists are more frequently faced with this disease in the advent of higher-resolution magnetic resonance imaging (MRI) scanning. We aim to suggest a suitable surveillance strategy for these patients.
METHODS
All patients who had MRI of the brain between June 2007 and January 2014 (n = 42,099) at The Walton Centre for Neurology and Neurosurgery were included. Radiologic reports containing the terms "pineal" and "cyst" were reviewed to identify patients.
RESULTS
A total of 281 patients were identified with pineal cysts. The principal indication for head MRI was headache (50.2%), although no symptoms were deemed attributable to pineal disease. A total of 178 patients (63.3%) were female, and the age at diagnosis ranged from 16 to 84 years. The median size of pineal cyst at diagnosis was 10 mm. A total of 181 patients had subsequent follow-up at a median time of 6 months (range, 1-68). Eleven pineal cysts (6%) changed size during the follow-up period. Four patients had a reduction in cyst size; the median change was 2.5 mm. A further 7 pineal cysts increased in cyst size; the median change was 2 mm. No patients developed complications.
CONCLUSIONS
Incidental pineal cysts typically show a benign course. In the adult population, they do not require long-term neurosurgical follow-up, because pineal cysts tend to remain a stable size. In asymptomatic patients, we recommend a single follow-up MRI scan at 12 months to confirm diagnosis. The patient should then be discharged if the cyst remains stable.
Topics: Adolescent; Adult; Age Distribution; Aged; Central Nervous System Cysts; Child; Child, Preschool; Disease Progression; Female; Humans; Incidental Findings; Infant; Magnetic Resonance Imaging; Male; Middle Aged; Pinealoma; Prevalence; Risk Factors; Sensitivity and Specificity; Sentinel Surveillance; Sex Distribution; United Kingdom; Young Adult
PubMed: 26944882
DOI: 10.1016/j.wneu.2016.02.092