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World Neurosurgery May 2023Pineal parenchymal tumors are exceedingly rare, and optimal disease management has yet to be defined. In this study, we aimed to identify prognostic factors and...
BACKGROUND
Pineal parenchymal tumors are exceedingly rare, and optimal disease management has yet to be defined. In this study, we aimed to identify prognostic factors and establish a predictive model for the prognosis of patients with pineal parenchymal tumors.
METHODS
All patients with pineal parenchymal tumors in the Surveillance, Epidemiology and End Results database between 1975 and 2019 were reviewed. Data were summarized, and survival was modeled with Cox regression analyses. In addition, a nomogram predicting 5- and 10-year survival probability for pineal parenchymal tumors was developed and validated.
RESULTS
We found 691 pineal parenchymal and 1961 pineal region neoplasms during 1975 and 2019 resulting in an incidence of 35%. In total, 441 patients were excluded due to incomplete data. The final cohort was subdivided into groups based on tumor histology: pineocytomas, pineoblastomas, and pineal parenchymal tumors of intermediate differentiation. Multivariate Cox analysis identified age and beam radiation as prognostic factors in pineoblastomas. Age, histology, tumor size, extent of resection, radiation, and chemotherapy were selected to build a clinical nomogram. The C-index for the nomogram was 0.795 (95% confidence interval 0.738-0.852). The calibration curves of the 5- and 10-year survival rates showed good agreement between the nomogram predictions and actual observations.
CONCLUSIONS
This nomogram is a convenient and precise tool for clinicians to evaluate prognosis of pineal parenchymal tumors.
Topics: Humans; Pinealoma; Nomograms; Prognosis; SEER Program; Brain Neoplasms; Pineal Gland
PubMed: 36841539
DOI: 10.1016/j.wneu.2023.02.084 -
World Neurosurgery Dec 2022Most pineal cysts are not surgical lesions and should be conservatively managed. Select lesions, notably those that present with hydrocephalus and Parinaud syndrome and...
Most pineal cysts are not surgical lesions and should be conservatively managed. Select lesions, notably those that present with hydrocephalus and Parinaud syndrome and lesions presenting with symptoms consistent with intermittent blockage of cerebrospinal fluid pathways and/or neurovascular compression, however, can be considered for surgical intervention. Two workhorse surgical approaches to the pineal region include the occipital interhemispheric transtentorial and supracerebellar infratentorial approaches. Each approach provides unique benefits and drawbacks and is associated with morbidities. In this patient, we demonstrate the use of a minimally invasive, supine, lateral supracerebellar infratentorial approach to the pineal region (Video 1). The approach makes use of early access to the foramen magnum to release cerebrospinal fluid for cerebellar relaxation, followed by navigation-guided, minimal dissection of the supracerebellar potential space to arrive at the pineal cyst. Opening of the arachnoid membranes ventrolateral to the confluence of the deep cerebral veins allows for direct access to the cyst with minimal manipulation of the venous complex and risk for injury. The cyst is then fenestrated, and the cyst capsule is removed. It should be noted that surgical fenestration does not guarantee relief of headache symptoms, and patients should be consoled about this preoperatively.
Topics: Humans; Pinealoma; Pineal Gland; Brain Neoplasms; Central Nervous System Cysts; Cysts
PubMed: 36174946
DOI: 10.1016/j.wneu.2022.09.084 -
Child's Nervous System : ChNS :... Dec 2023Aggressive surgical resection for pineal region tumors, which is a mainstay of management in almost all cases, remains technically challenging because these tumors are...
PURPOSE
Aggressive surgical resection for pineal region tumors, which is a mainstay of management in almost all cases, remains technically challenging because these tumors are located at the geometric center of the brain and surrounded by critical neurovascular structures. Therefore, any refinement in the surgical procedure is desirable. We have recently introduced an endoscope to the occipital transtentorial approach (OTA), which has been one of the most common procedures for reaching the pineal region, to overcome some of the disadvantages of the procedure, including the necessity of a relatively large skin incision and bone opening, the difficulty in orientation due to the obliqueness of the approach, the possibility of retraction injury on the occipital lobe and the corpus callosum, and blind spots at the ipsilateral wall and the roof of the third ventricle.
METHODS
We performed endoscopic OTA with corresponding advantages, including less invasiveness by virtue of a small entrance limiting the retraction of the occipital lobe, the elimination of blind spots, and the facilitation of fine manipulation based on the bright endoscopic view even at substantial depth, which is panoramic and can be magnified as needed. This procedure was substantially different from a conventional microscopic OTA, though both use a similar corridor. To maximize maneuverability within the limited space in endoscopic OTA, it was necessary to thoroughly understand each step of the procedure, as well as optimal sequence in the procedure.
RESULTS AND CONCLUSION
We found endoscopic OTA to be a promising technique for resectioning pineal region tumors, with the potential for extensive and routine application for surgeons familiar with endoscopic surgery.
Topics: Humans; Brain Neoplasms; Neurosurgical Procedures; Pinealoma; Pineal Gland; Endoscopy
PubMed: 36094603
DOI: 10.1007/s00381-022-05658-6 -
Asian Journal of Neurosurgery 2021Primary tumors of the pineal gland occur infrequently with a preponderance of either parenchymal tumors or germ cells tumors. Papillary tumor of the pineal region is a...
Primary tumors of the pineal gland occur infrequently with a preponderance of either parenchymal tumors or germ cells tumors. Papillary tumor of the pineal region is a rare neuroepithelial lesion that arises exclusively in the pineal region. They have been designated as either Grade II or Grade III lesions as per the 2016 WHO classification of central nervous system tumors. Clinically, they usually present with obstructive hydrocephalus and visual disturbance. On imaging, these tumors are solid-cystic, heterogeneously enhancing, and show T2 hyperintensity. Pathologically, they can closely resemble a Grade I pineocytoma and immunohistochemistry is essential to differentiate the two. No definite guidelines exist to confirm the ideal protocol of treatment. Evidence regarding the role of radiation after surgery is limited to case reports and series. Adjuvant therapy is usually recommended for tumors with subtotal excision, high proliferative/mitotic index, or proven metastasis. We describe a case of a 29-year-old male with a recurrent papillary tumor of the pineal region, 9 years after primary surgery where it was misdiagnosed as a pineocytoma. The tumor was effectively controlled with surgical excision, cerebrospinal fluid diversion, and adjuvant radiation for 8 years before showing two recurrences within a span of 6 months with a rising proliferation index.
PubMed: 34268174
DOI: 10.4103/ajns.AJNS_439_20 -
No Shinkei Geka. Neurological Surgery May 2024This article describes the concept and technical aspects of the occipital transtentorial approach(OTA)for tumor extraction in the pineal region, based on the author's... (Review)
Review
This article describes the concept and technical aspects of the occipital transtentorial approach(OTA)for tumor extraction in the pineal region, based on the author's experience and literature review. Awareness of the successful completion of each surgical step is essential. Preoperative preparation and imaging evaluations, with particular attention to the veins and venous sinuses, are especially important. It is also helpful to perform a complete dura incision and inversion up to the edge of confluence, superior sagittal sinus, and transverse sinus. Subsequently, it is necessary to understand the usefulness of adequate dissection in the vicinity of the corpus callosum and internal occipital vein(IOV)so that the occipital lobe can be moved without difficulty. Furthermore, development of the IOV with adequate tentoriotomy facilitates contralateral work. Finally, complete understanding of each step during the bilateral, ambient cistern and cerebellomesencephalic fissure dissection process, where the cerebellar vermis can be moved without difficulty, is necessary for a safe OTA to pineal region tumor extraction.
Topics: Humans; Neurosurgical Procedures; Pinealoma; Pineal Gland; Brain Neoplasms
PubMed: 38783507
DOI: 10.11477/mf.1436204958 -
Neurosurgery Jun 2022Limited retrospective data exist on malignant pineal parenchymal tumors (PPTs) in adults, and there are no large previous studies that review clinical outcomes across...
BACKGROUND
Limited retrospective data exist on malignant pineal parenchymal tumors (PPTs) in adults, and there are no large previous studies that review clinical outcomes across the 3 treatment arms of surgery, radiotherapy, and chemotherapy. As a result, optimal disease management has yet to be defined.
OBJECTIVE
To evaluate treatment trends and perform survival analysis in adult PPT.
METHODS
The National Cancer Database was queried for histologically confirmed PPT diagnosed from 2007 to 2016. Univariate and multivariate Cox regressions were used to evaluate the prognostic impact of covariates. Kaplan-Meier survival curves were generated for comparative subanalyses.
RESULTS
Of the 251 patients who met inclusion criteria, 172 had PPTs of intermediate differentiation (PPTID) and 79 had pineoblastoma. A plurality of patients with pineoblastoma were treated with trimodal therapy (39.1%), whereas patients with PPTID were commonly treated with either surgery alone or surgery and radiation (33.7% each). Factors independently associated with improved overall survival include younger patient age, female sex, lower comorbidity score, lower tumor grade, and treatment with surgery or radiation (each P < .05). Subanalyses confirm the effect of radiation on survival in patients with grade III PPTID with subtotal resection; however, no survival benefit of adjuvant radiation is demonstrated in patients with grade II PPTID with subtotal resection.
CONCLUSION
Although radiotherapy and surgery were found to increase survival in all patients with PPT, there was no demonstrable survival benefit of adjuvant radiation in surgically treated patients with grade II PPTID. This suggests that adjuvant radiotherapy may not add significant survival benefit in many adult patients with grade II PPTID.
Topics: Adult; Brain Neoplasms; Female; Humans; Pineal Gland; Pinealoma; Prognosis; Radiotherapy, Adjuvant; Retrospective Studies
PubMed: 35311743
DOI: 10.1227/neu.0000000000001915 -
Child's Nervous System : ChNS :... Apr 2024Pallister-Killian syndrome (PKS; OMIM #601803) is a rare genetic disorder typically characterized by developmental delay, seizures, sparse temporal hair, and facial...
Pallister-Killian syndrome (PKS; OMIM #601803) is a rare genetic disorder typically characterized by developmental delay, seizures, sparse temporal hair, and facial dysmorphisms. PKS is most frequently caused by mosaic supernumerary isochromosome 12p. Here, we report a 27-month-old girl with a prenatal diagnosis of PKS and a histopathological diagnosis of pineocytoma.
PubMed: 38689102
DOI: 10.1007/s00381-024-06426-4 -
Cancer Investigation Feb 2021Extragonadal germ cell tumors (GCTs) are thought to arise as a result of local transformation of primordial gonadal cells (PGCs) that become misplaced during... (Review)
Review
BACKGROUND
Extragonadal germ cell tumors (GCTs) are thought to arise as a result of local transformation of primordial gonadal cells (PGCs) that become misplaced during embryogenesis. With the exception of bilateral testis tumors, metachronous GCT (i.e., occurring at a site classically described for primary GCTs) are rare events.
PATIENTS AND METHODS
The clinical, radiological, and molecular data (if available) of patients with metachronous GCT were analyzed.
RESULTS
Three Caucasian males were identified: case 1 presented with a pineal germinoma 19 years after a mediastinal seminoma that had been treated with chemotherapy, case 2 presented with a pineal non-seminomatous GCT (NSGCT) that occurred three years after a mediastinal seminoma treated with chemotherapy, and case 3 presented with a mediastinal seminoma concomitant with a suprasellar germinoma that occurred two years after a stage I testicular NSGCT treated exclusively with surgery. None of these patients had a positive family history or disorder of sex development. Molecular data were available for cases 2 and 3. In case 2, a gene biallelic inactivation in the second tumor suggested chemoresistance to cisplatin. This was further confirmed by tumor progression during second-line treatment. In case 3, the molecular analysis revealed different profiles in the three tumors, thus suggesting distinct tumor cell origins.
CONCLUSION
These rare cases should alert clinicians of the possibility of multiple GCTs that should not be considered to be relapses. The underlying physiopathology is unknown, but multiple PGC mismigrations is a likely cause. Initial treatment with cisplatin may select chemo-resistant clones, thereby making the subsequent treatment more of a challenge.
Topics: Adolescent; Adult; Checkpoint Kinase 2; Combined Modality Therapy; Disease Management; Drug Resistance, Neoplasm; Humans; Male; Mediastinal Neoplasms; MutL Protein Homolog 1; Neoplasms, Germ Cell and Embryonal; Neoplasms, Second Primary; Pinealoma; Seminoma; Young Adult
PubMed: 33017201
DOI: 10.1080/07357907.2020.1828447 -
Child's Nervous System : ChNS :... Sep 2023Pineal tumors are rare and their incidence is of 1% among all pediatric tumors of the central nervous system. Patient survival depends on the histology, the extension...
INTRODUCTION
Pineal tumors are rare and their incidence is of 1% among all pediatric tumors of the central nervous system. Patient survival depends on the histology, the extension of the surgical removal, and the efficacy of the complementary treatment (chemotherapy and cranio-spinal irradiation), as well as the age of the patient.
MATERIALS AND METHODS
In this study, we analyzed 151 pediatric patients treated for pineal tumors from 1997 to 2020 in Lyon, France. All patients were recorded in the French Register of Pineal tumors, which has been centralized and maintained in Lyon since 2010.
RESULTS
Our analysis shows that benign tumors have an overall positive prognosis with total surgical removal. Concerning pineal parenchymal tumors, pinealoblastomas have a poor prognosis , especially in children less than three years old. A new pathological classification system allows for a better stratification of patient risk within different groups of patients with pineal tumors. It is also important to note that the identification of DICER 1 syndrome in families with pinealoblastomas warrant further medical investigation. Patients with Germ Cell Tumors have more favorable outcomes, with a global survival rate of 87 % and a pure germinoma survival rate of almost 97%. When analyzing the prognosis of pineal gland gliomas, otherwise known as tectal plate gliomas, pilocytic astrocytomas had a promising prognosis. Otherwise, prognosis of other tectal plate gliomas are related to the grade of malignancy and the efficacy of complementary treatment. Lastly, papillary tumors need a complete removal for the best chance of survival, and Atypical teratoid/ rabdoid tumors (AT/TR) still have a bad prognosis, regardless of surgical resection.
CONCLUSIONS
Our results show that, with regards to pediatric pineal region tumors, there are still areas in prognostic indicators that need to be improved. Similarly, these pathologies need to be treated via a multidisciplinary approach to improve a patient's survival rate and their quality of life.
Topics: Humans; Child; Child, Preschool; Pinealoma; Quality of Life; Brain Neoplasms; Pineal Gland; Glioma
PubMed: 36242638
DOI: 10.1007/s00381-022-05649-7 -
Acta Neurologica Belgica Dec 2021Pineal region tumors have different pathological tumors and their optimal management remains controversial. Advancements in neuroendoscopy have led to the ability to...
Pineal region tumors have different pathological tumors and their optimal management remains controversial. Advancements in neuroendoscopy have led to the ability to simultaneously treat the hydrocephalus and obtain a tissue diagnosis. A retrospective review of 34 patients with pineal region tumors in Beijing Tiantan hospital from the year 2016 to 2018 was undertaken. A single bur hole for both procedures was used successfully in all patients. Once pathologic diagnosis is made, the subsequent management of different tumors is dependent on response to therapy, the tumor markers and original pathology. Follow-up period was 4-26 months. All 34 cases presented with hydrocephalus and increased intracranial pressure manifestations. Elevated blood tumor markers were found in seven cases. The neuroendoscopic biopsy was diagnostic in 32 samples (94.1%) and nondiagnostic (gliosis) in two patients. 21 cases were germinomas, five cases were tectal astrocytomas, two cases were pineoblastomas, two cases were non-germinomatous germ-cell tumours (NG-GCTs) and 1 case immature teratoma and glioblastoma respectively. During the follow-up period, all germinomas but one case with elevated blood α-fetoprotein received craniotomy with a final diagnosis of NG-GCT received radiotherapy and chemotherapy. Four tectal astocytomas, two pineoblastomas and two NG-GCTs received subsequent open surgery due to progressive development, the pathological data was concordant with the initial endoscopic biopsy sample. An additional VP shunt was inserted in one tectal astrocytoma who have hydrocephalus after craniotomy. Except for 18 cases of transient fever and a case with intratumoral hemorrhage, there was no other significant complications, cognitive disorder and no death. The simultaneous single-trajectory endoscopic technique permits not only to control hydrocephalus but also to obtain histological diagnosis with a low incidence of complication and higher safety. Providing meaningful pathological data, endoscopic biopsies could lead to an appropriate management decision. Especially, it is favored as an early step in the management of patients with marker-negative tumors.
Topics: Adolescent; Biopsy; Brain Neoplasms; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Infant; Male; Neuroendoscopy; Pineal Gland; Pinealoma; Retrospective Studies; Third Ventricle; Ventriculostomy
PubMed: 32506355
DOI: 10.1007/s13760-020-01387-2