scholarly journals CS-5 A case of anaplastic pleomorphic xanthoastrocytoma in the pineal region

2021 ◽  
Vol 3 (Supplement_6) ◽  
pp. vi27-vi28
Author(s):  
Noriyuki Nakayama ◽  
Ryuta Yamada ◽  
Tetsuya Yamada ◽  
Toru Iwama
Keyword(s):  
Pineal Gland ◽  
Pleomorphic Xanthoastrocytoma ◽  
Braf V600e ◽  
Subtotal Resection ◽  
Communicating Hydrocephalus ◽  
Pineal Tumor ◽  
Sitting Position ◽  
Jugular Veins ◽  
Pineal Tumors ◽  
Microvascular Proliferation

Abstract Tumors in the pineal gland are rare tumors that account for about 0.3% of all brain tumors and have various histological types of tumors develop with germinoma, pineocytoma, and pineoblastoma in that order. On the other hand, pleomorphic xanthoastrocytoma (PXA) is a rare tumor of less than 0.2% and frequently occurs in supratentorial cerebral surface of children and young adults.A case was a 61-year-old man whose pineal tumor was found due to visual disturbance. MRI showed a 23 mm-sized lesion with cysts and inhomogeneous enhancement in the pineal gland. Partial calcification was observed, but there was no non-communicating hydrocephalus, and no increase in HCG-β and AFP with blood sampling. A midline suboccipital craniotomy was performed in the sitting position, a head-up surgery was performed using a 4K / 3D video microscope system (ORBEYE exoscope, Olympus) by the infratentorial supracerebellar approach (ITSCA). The bridging veins and precentral cerebellar vein were dissected to expose the posterior surface of the tumor, and internal decompression was performed. For the complication of air embolism, artificial cerebrospinal fluid was sprayed, and the bilateral internal jugular veins were compressed to confirm the inflow point. There was strong adhesion to the Rt vein of Rosenthal, and the site was removed intracapsularly. Finally, subtotal resection was performed with remaining the upper part of the tumor, a blind lesion behind the Vein of Galen. Vertical gaze palsy occurred after this operation, but it gradually improved over time. Tumor showed pathologically remarkable polymorphism, poor microvascular proliferation and necrosis, but mitotic figures 4–5 / 10HPF, MIB-1 index 10%, GFAP positive, no BRAF V600E mutation. There are few reports of PXA occurring in the pineal gland, and this case is the sixth case. It is also the first report for pineal tumors using ORBEYE through ITSCA in the sitting position.

Malignant pineal region tumors

1979 ◽  
Vol 51 (5) ◽  
pp. 597-607 ◽  
Author(s):  
Edward A. Neuwelt ◽  
Mark Glasberg ◽  
Eugene Frenkel ◽  
W. Kemp Clark
Keyword(s):  
Metastatic Tumor ◽  
Tumor Burden ◽  
Pineal Region ◽  
Subtotal Resection ◽  
Pineal Tumor ◽  
Pineal Tumors ◽  
Content Type ◽  
Pineal Region Tumors ◽  
Tissue Diagnosis ◽  
Craniospinal Radiation

✓ Eight patients with primary malignant pineal tumors have been seen at this institution over the past 6 years; six of them underwent definitive surgical exploration. Complete gross microsurgical excision of well encapsulated tumors was possible in four of these patients. In two cases of pineal germinomas, a biopsy and a subtotal resection were carried out because of the known radiosensitivity of this tumor. These six surgical patients all received postoperative craniospinal radiation and continue to do well up to 6 years postoperatively. Two nonoperative patients were initially treated at other institutions by ventriculoperitoneal shunt and radiation and were the only ones to develop metastatic disease. One patient had metastasis of her pineoblastoma to her unirradiated spinal canal and the other patient had metastasis of his germinoma to the peritoneum. The former patient was quadriplegic on admission, although her pineal tumor was no longer visible on computerized tomography (CT), and she died of pneumonia. The latter patient's tumor secreted the beta chain of human chorionic gonadotropin (HCG). This patient's massive metastatic tumor burden completely regressed as determined by body CT scan and HCG levels after four courses of chemotherapy with bleomycin, vinblastine, and cis-platinum. In 20 patients with lesions of the pineal region, craniotomy was associated with only one death (a patient with metastatic adenocarcinoma). Thus, microsurgery for pineal tumors provides either a reasonably safe potential for complete tumor extirpation and possible cure, or a tissue diagnosis which is necessary for appropriate therapeutic planning for radiotherapy and/or chemotherapy. The traditional therapeutic approach of empiric radiotherapy without a tissue diagnosis for pineal lesions may no longer be warranted.

scholarly journals HGG-02. ADOLESCENT AND YOUNG ADULT (AYA) GLIOMA WITH BRAF V600E-MUTANTATION

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii344-iii344
Author(s):  
Yui Kimura ◽  
Yukitomo Ishi ◽  
Yuko Watanabe ◽  
Yoshiko Nakano ◽  
Shigeru Yamaguchi ◽  
...  
Keyword(s):  
Braf Inhibitor ◽  
Clinical Information ◽  
Early Recurrence ◽  
Pleomorphic Xanthoastrocytoma ◽  
Adolescent And Young Adult ◽  
Braf V600e ◽  
Low Grade ◽  
Diffuse Astrocytoma ◽  
Genetic Features ◽  
Epithelioid Glioblastoma

Abstract BACKGROUND Biological features of pediatric glioma differ significantly from those of adult glioma, and limited data are available on those of AYA patients. Here, we focused on AYA patients with glioma, especially those harboring BRAF V600E mutation, and investigated their clinical and genetic features. METHOD: We retrospectively analyzed AYA patients with brain tumors harboring BRAF V600E, who were treated in two hospitals in Japan. RESULTS Clinical information was available for 14 patients. The median age at diagnosis was 25 years (range: 15–38). Five patients were diagnosed with glioblastoma (GBM), including one epithelioid type. These patients were over 25. Although one patient with GBM died of the disease 6.9 years after initial diagnosis, the remaining patients were alive. Two patients were alive without recurrence at 38 and 51 months after the treatment. The patient with epithelioid glioblastoma experienced early recurrence. The remaining nine patients (64%) were diagnosed with low-grade glioma, including ganglioglioma, pilocytic astrocytoma, diffuse astrocytoma, oligodendroglioma, pleomorphic xanthoastrocytoma, and polymorphous low-grade neuroepithelial tumor of the young. No patients died of the disease, and four patients are alive without recurrence after initial operation without adjuvant treatment. Two patients are (epithelioid glioblastoma and ganglioglioma) currently undergoing treatment with a BRAF inhibitor for recurrent tumors. DISCUSSION Although the number of this study is limited, our study suggested that the prognosis of AYA patients with BRAF-V600E positive GBM may not be as dismal as that of children or adults.

Pineal Apoplexy: A Case Series and Review of the Literature

2021 ◽  
Author(s):  
Martin Majovsky ◽  
David Netuka ◽  
Radim Lipina ◽  
Jan Mraček ◽  
Vladimír Beneš
Keyword(s):  
Surgical Treatment ◽  
Pineal Gland ◽  
Clinical Significance ◽  
Obstructive Hydrocephalus ◽  
Case Series ◽  
Surgical Removal ◽  
Pineal Tumor ◽  
Review Of The Literature ◽  
Melatonin Secretion ◽  
Pubmed Database

Abstract Background Pineal apoplexy is a rare condition, with unknown incidence and clinical significance. To elucidate this clinical condition, we analyzed our own case series and performed a review of the literature. Methods We enrolled all patients with a hemorrhagic pineal apoplexy who were referred to our department between January 2000 and January 2020. Hemorrhagic pineal apoplexy was defined as the presence of fluid–fluid levels inside the pineal cyst (PC) on an axial or sagittal magnetic resonance scan. In one patient, after PC apoplexy, we performed a circadian melatonin sampling from peripheral blood to determine the function of the pineal gland. The PubMed database was searched for publications using the terms “pineal” and “apoplexy.” Results Eight patients were enrolled, of which three patients underwent surgical treatment and five patients were managed conservatively. One patient was tested for circadian melatonin secretion. Results confirmed melatonin secretion with preserved physiologic circadian rhythm.Our search of the literature led us to 31 studies that comprised 30 patients with apoplectic PC, 9 with apoplectic pineal tumor, and 1 with bleeding into the normal pineal gland. Most patients presented with headache, nausea, and vomiting, less frequently with acute hydrocephalus and gaze palsy. Twenty patients with a PC underwent resection or aspiration. Two patients underwent shunt placement as the only procedure and five received both shunt and surgical removal. Six patients with a PC were observed without surgical treatment. All the nine patients with a pineal tumor were operated on. In indicated cases, four patients received radiation therapy and one received chemotherapy. Conclusion Clinical significance of hemorrhagic pineal apoplexy ranges from an asymptomatic course to rapid deterioration and death. In patients with mild symptoms, observation is indicated, whereas surgical treatment is reserved for severe cases presenting with obstructive hydrocephalus and includes cerebrospinal fluid diversion, resection of apoplectic pineal lesions, or both.

scholarly journals Extracranial invasion of a recurrent, transformed anaplastic pleomorphic xanthoastrocytoma: a case report

2018 ◽  
Vol 45 (S1) ◽  
pp. S2-S3
Author(s):  
K.D. Langdon ◽  
D. Krivosheya ◽  
M.O. Hebb ◽  
B. Wehrli ◽  
L.C. Ang
Keyword(s):  
Posterior Fossa ◽  
Jugular Foramen ◽  
Jugular Bulb ◽  
Neck Mass ◽  
Pleomorphic Xanthoastrocytoma ◽  
Braf V600e ◽  
Rare Tumour ◽  
Who Grade ◽  
Original Tumour ◽  
The Right

Pleomorphic xanthoastrocytoma (PXA) is a rare tumour comprising <1% of all primary central nervous system tumours and the majority (~98%) occur supratentorially. We report on a 40-year-old female with a past medical history of a rare posterior fossa/cerebellar PXA who presented with a right-sided neck mass, decreased shoulder power and longstanding right tongue deviation with right-sided hemi-atrophy. The patient had prior tumour debulking. Recent MRI demonstrated an enhancing posterior fossa mass extending to the skull base at the jugular foramen and another mass in the upper neck along the jugular bulb with displacement and encasement of the right common carotid artery down to C5. Resection of the neck mass reveals an anaplastic PXA. The tumour has close approximation with adjacent peripheral nerves and is positive in 2 lymph nodes. Comparison with the original tumour molecular and immunohistochemical profiles reveals a conserved BRAF V600E mutation but the transformed malignant glioma now expresses dot-like EMA positivity and ATRX is completely lost (mutated). Transformation of a PXA (WHO Grade II) into an anaplastic PXA (WHO Grade III) has been well documented, but extracranial extension is extraordinarily rare. We report herein the first documented case of a posterior fossa PXA that underwent malignant transformation and extracranial invasion to the parapharyngeal space.

Combined “Infiltrating Astrocytoma/Pleomorphic Xanthoastrocytoma” Harboring IDH1 R132H and BRAF V600E Mutations

2015 ◽  
pp. 1 ◽  
Author(s):  
Seiji Yamada ◽  
Benjamin R. Kipp ◽  
Jesse S. Voss ◽  
Caterina Giannini ◽  
Aditya Raghunathan
Keyword(s):  
Pleomorphic Xanthoastrocytoma ◽  
Braf V600e ◽  
Idh1 R132h

Effects of positive end-expiratory pressure ventilation on cerebral venous pressure with head elevation in dogs

2000 ◽  
Vol 88 (2) ◽  
pp. 655-661 ◽  
Author(s):  
Thomas J. K. Toung ◽  
H. Aizawa ◽  
Richard J. Traystman
Keyword(s):  
Prone Position ◽  
Venous Pressure ◽  
Air Embolism ◽  
Right Atrial ◽  
Sitting Position ◽  
Positive End Expiratory Pressure ◽  
Jugular Veins ◽  
Venous Air Embolism ◽  
Head Elevation ◽  
Cerebral Venous Pressure

Mechanical ventilation with positive end-expiratory pressure (PEEP) may prevent venous air embolism in the sitting position because cerebral venous pressure (Pcev) could be increased by the PEEP-induced increase in right atrial pressure (Pra). Whereas it is clear that there is a linear transmission of the PEEP-induced increase in Pra to Pcev while the dog is in the prone position, the mechanism of the transmission with the dog in the head-elevated position is unclear. We tested the hypothesis that a Starling resistor-type mechanism exists in the jugular veins when the head is elevated. In one group of dogs, increasing PEEP linearly increased Pcev with the dog in the prone position (head at heart level, slope = 0.851) but did not increase Pcev when the head was elevated. In another group of dogs, an external chest binder was used to produce a larger PEEP-induced increase in Pra. Further increasing Pra increased Pcev only after Pra exceeded a pressure of 19 mmHg (break pressure). This sharp inflection in the upstream (Pcev)-downstream (Pra) relationship suggests that this may be caused by a Starling resistor-type mechanism. We conclude that jugular venous collapse serves as a significant resistance in the transmission of Pra to Pcev in the head-elevated position.

scholarly journals RARE-31. TREATMENT OUTCOME OF CHILDREN WITH PLEOMORPHIC XANTHOASTROCYTOMA: RETROSPECTIVE ANALYSIS – A SINGLE INSTITUTION EXPERIENCE

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi228-vi228
Author(s):  
Ossama Maher ◽  
Toba Niazi ◽  
Ziad Khatib ◽  
John Ragheb
Keyword(s):  
Local Recurrence ◽  
Retrospective Analysis ◽  
Malignant Transformation ◽  
Cranial Nerve ◽  
Pleomorphic Xanthoastrocytoma ◽  
Braf V600e ◽  
Biological Behavior ◽  
High Local Recurrence Rate ◽  
Cranial Nerve Palsies

Abstract BACKGROUND Pleomorphic xanthoastrocytoma (PXA) and anaplastic pleomorphic xanthoastrocytoma (APXA) are two types of rare astrocytomas in pediatrics. There is limited literature reporting their outcomes. METHODS A retrospective analysis of pediatric patients with diagnosis of PXA and APXA treated at Nicklaus Children’s Hospital was conducted using descriptive measures. RESULTS A chart review of patients with brain tumors from 2001 to 2019 revealed that 13 patients (median age 6 years, range 2–17 years) were diagnosed with non-metastatic PXA (n=12) and APXA (n=1). Six patients (46%) were male. Clinical presentation included seizures (n=8), headaches (n=2) cranial nerve palsies (n=2). Diagnostic imaging showed tumor in the temporal lobe (n=5), parietal lobe (n=2), temporoparietal lobe (n=2), frontal lobe (n=1), occipital lobe (n=1), others (n=2). BRAF V600E mutation were identified in three of five analyzed tumors including PXA (n=4) and APXA (n=1). Surgical intervention consisted of gross total resection (n=8) and near/subtotal resection (n=5). Ten patients (76%) required a second surgery (median 2; range 1–4) due to local recurrence. The median time to recurrence was 3 years (range 6 months - 7 years). Three patients (23%) had malignant transformation of PXA, which occurred three to eight years from the initial surgery; one of them also had extensive spinal metastasis. Focal radiation was given to six patients (46%) due to multiple recurrence (n=2), malignant transformation (n=3), APXA (n=1). At a median follow up of 8 years (range 3 to 20 years), eight patients remain alive, three patients had lost to follow up, two patients died from progressive disease. Long-term sequelae varied as follows; seizures (n=5), motor weakness (n=4), cranial nerve palsies (n=2), and learning disability (n=1). CONCLUSION PXA is associated with high local recurrence rate and uncommonly malignant transformation to APXA in pediatrics, which requires close follow-up due to unpredictable biological behavior of these tumors.

Serum Melatonin Levels: A New Neurodiagnostic Tool in Pineal Region Tumors?

Neurosurgery ◽  
1987 ◽  
Vol 21 (6) ◽  
pp. 817-824 ◽  
Author(s):  
Peter Vorkapic ◽  
Franz Waldhauser ◽  
Robert Bruckner ◽  
Christian Biegelmayer ◽  
Manfred Schmidbauer ◽  
...  
Keyword(s):  
Pineal Gland ◽  
Limit Of Detection ◽  
Tumor Resection ◽  
Serum Levels ◽  
Pineal Region ◽  
Computer Assisted ◽  
Surgical Removal ◽  
Pineal Tumors ◽  
Pineal Region Tumors ◽  
Computer Assisted Tomography

Abstract The pineal hormone melatonin (MLT) is secreted in a circadian rhythm with high serum levels during nighttime and low serum levels during daytime. Several authors have reported an altered secretion pattern of MLT in patients with pineal tumors and have proposed that MLT may be used as a tumor marker. In nine patients, a pineal region tumor was diagnosed by computer-assisted tomography. Before and after surgical removal of the tumor, several day- and nighttime serum samples were collected and MLT concentrations were estimated by radioimmunoassay. Before operation, five patients presented a normal circadian pattern of MLT secretion. In the remaining four subjects, MLT levels were undetectable or at the limit of detection, with no signs of a circadian secretion pattern. Eight patients were reexamined after tumor resection, when all but one had undetectable or very low MLT levels. The remaining subject, with a pineomesencephalic pilocytic astrocytoma, dislocating but not involving the pineal gland, presented a normal circadian secretion pattern of MLT after operation; in this case, tumor resection was possible without damaging the pineal gland. Thus, before operation, MLT deficiency rather than exaggerated serum levels may be used as a marker for pineal tumors that destroy the pineal gland. After tumor resection, serum MLT may serve to demonstrate complete pinealectomy.

BRAF V600E mutation: A treatable driver mutation in pleomorphic xanthoastrocytoma (PXA)

2015 ◽  
Vol 55 (1) ◽  
pp. 122-123 ◽  
Author(s):  
Silvia Hofer ◽  
Grégoire Berthod ◽  
Christian Riklin ◽  
Elisabeth Rushing ◽  
Jonas Feilchenfeldt
Keyword(s):  
Pleomorphic Xanthoastrocytoma ◽  
Braf V600e Mutation ◽  
Driver Mutation ◽  
Braf V600e

scholarly journals Combined pleomorphic xanthoastrocytoma-ganglioglioma with BRAF V600E mutation: case report

2016 ◽  
Vol 18 (1) ◽  
pp. 53-57 ◽  
Author(s):  
Marta Cicuendez ◽  
Elena Martinez-Saez ◽  
Francisco Martinez-Ricarte ◽  
Esteban Cordero Asanza ◽  
Juan Sahuquillo
Keyword(s):  
Case Report ◽  
Tumor Recurrence ◽  
Pleomorphic Xanthoastrocytoma ◽  
Braf V600e Mutation ◽  
Braf V600e ◽  
Rare Tumor ◽  
Total Resection ◽  
Cell Lineages ◽  
Cellular Origin ◽  
History Of

Combined pleomorphic xanthoastrocytoma (PXA) and ganglioglioma (GG) is an extremely rare tumor, with fewer than 20 cases reported. The authors report a case of combined PXA-GG in an 18-year-old man with a history of seizures. The tumor showed necrosis and the BRAF V600E mutation on histological examination, with no evidence of tumor recurrence 1 year after gross-total resection. The BRAF V600E mutation was present, which suggests that both cell lineages may share a common cellular origin.

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