scholarly journals Case Report: Identification of Multiple TERT and FGFR2 Gene Fusions in a Pineal Region Glioblastoma Case

2021 ◽  
Vol 11 ◽  
Author(s):  
Xing Guo ◽  
Teng Chen ◽  
Shiming Chen ◽  
Chao Song ◽  
Dezhi Shan ◽  
...  
Keyword(s):  
Tumor Resection ◽  
Residual Tumor ◽  
Pineal Region ◽  
Proliferation Index ◽  
Gene Fusions ◽  
Who Grade ◽  
First Case ◽  
Fgfr2 Gene ◽  
Magnetic Resonance Imaging Mri ◽  
Next Generation Sequencing Ngs

As an oncogenic somatic variant, telomerase reverse transcriptase promoter (TERTp) mutations are frequently observed in adult glioblastoma (GBM). Alternatively, we report the first case of glioblastoma with TERT amplification accompanied by multiple TERT and FGFR2 gene fusions instead of TERTp mutation. A 55-year-old woman presented with dizziness, headache, and diplopia for three weeks. Magnetic resonance imaging (MRI) demonstrated a heterogeneously enhancing lobulated mass centered in the pineal region. Partial tumor resection and ventriculoperitoneal shunt were achieved, and the residual tumor was then treated with standard radiation. The tumor was diagnosed as GBM, IDH-wild type, WHO grade IV, and the Ki67 proliferation index was high (30–40%). Intriguingly, TERT amplification without TERTp mutation was identified via next generation sequencing (NGS). Further analysis revealed multiple TERT (TERT–NUBPL, MARCH6–TERT, and CJD4–TERT) and FGFR2 (CXCL17–FGFR2, SIPA1L3–FGFR2, FGFR2–SIPA1L3, and FGFR2–CEACAM1) gene fusions. After the surgery, the patient’s condition deteriorated rapidly due to the malignant nature of the tumor and she died with an overall survival of 3 months. Our report provides the molecular clue for a novel telomerase activation and maintenance mechanism in GBM.

scholarly journals Recurrent Sphenocavernous Meningioma

2021 ◽  
Author(s):  
Mizuho Inoue ◽  
Mohamed Labib ◽  
Alexander Yang ◽  
A. Samy Youssef
Keyword(s):  
Tumor Resection ◽  
Intensity Modulated Radiotherapy ◽  
Residual Tumor ◽  
World Health ◽  
Neurological Deficits ◽  
Falciform Ligament ◽  
Total Resection ◽  
Anterior Clinoid Process ◽  
Who Grade ◽  
Health Organization

AbstractA case of a recurrent sphenocavernous meningioma is presented. The patient is a 42-year-old male who presented with an episode of transient right-sided numbness. A magnetic resonance imaging (MRI) revealed a large left sphenocavernous meningioma. The patient underwent a frontotemporal craniotomy for tumor resection. Near total resection was achieved with minimal residual in the left cavernous sinus (CS) and orbital apex. The pathology was consistent with meningioma, World Health Organization (WHO) grade I. A follow-up MRI was done 9 months after surgery and showed a growth of the residual tumor, which was treated with intensity modulated radiotherapy. Tumor growth was detected on serial imaging over a 4-year period. Surgical resection was offered. A left frontotemporal craniotomy with pretemporal transcavernous approach was performed. The bone flap was reopened and the dura was opened in a Y-shaped fashion. The roof of the optic canal was drilled off, and the falciform ligament was opened to decompress the optic nerve. The tumor was disconnected from the anterior clinoid region (the anterior clinoid process was eroded by the tumor) and reflected off the wall of the lateral CS. Tumor was adherent to the V2 fascicles (the lateral CS wall was resected in the first surgery) and was sharply dissected off. Gross total resection was achieved. The pathology was consistent with meningioma, WHO grade I. The patient had an unremarkable postoperative course without any new neurological deficits.The link to the video can be found at: https://youtu.be/KVBVw_86JqM.

scholarly journals STMO-06 SMART CYBER OPERATING THEATER REALIZED BY INTERNET OF THINGS - RESULTS OF CLINICAL STUDY FOR 56 CASES

2019 ◽  
Vol 1 (Supplement_2) ◽  
pp. ii19-ii19
Author(s):  
Muragaki Yoshihiro ◽  
Jun Okamoto ◽  
Taiichi Saito ◽  
Satoshi Usui ◽  
Ushio Yonezawa ◽  
...  
Keyword(s):  
Brain Tumors ◽  
Removal Rate ◽  
Tumor Resection ◽  
Pituitary Tumors ◽  
Residual Tumor ◽  
Clinical Results ◽  
Digital Data ◽  
Clinical Effects ◽  
Operating Theater ◽  
First Case

Abstract PURPOSE Unlike conventional operating rooms that provide a sterilized space, we have developed a Smart Cyber Operating Theater (SCOT) in which the room itself performs treatment as a single medical device. We report the clinical results of 3 types of SCOT. METHODS Basic SCOT packaged with intraoperative MRI (0.4Tesla) was introduced in Hiroshima University in 2016. Standard SCOT networked with middleware OPeLiNK was introduced to Shinshu University in 2018, and Hyper SCOT introduced to Tokyo Women’s Medical University in 2019. RESULTS The average of all 56 patients was 44 years old. There were 38 brain tumors (68%), 11 functional diseases (19%), and 7 orthopedic diseases (13%). Basic SCOT is used for 41 cases (/56; 73%) with 22 gliomas, 10 epilepsies, 7 bone tumors, and 2 benign brain tumors. Standard SCOT with 20 networked devices is used for 14 cases (/56; 25%) with 6 gliomas including brain stem and thalamus, 6 pituitary tumors and 2 benign brain tumors. The strategy desk can display a variety of digital data synchronized in time, and the review and comment functions also operate. It is useful for remote advice through mutual communication via strategy desk. Hyper SCOT was used in February 2019 for the first case (1/56 cases; 2%). MRI images were taken with an average of 1.3 shots with good image quality. For 46/56 neoplastic lesions (82%), additional removal of residual tumor was performed in 31/46 cases (67%), and 26/46 cases (57%) were totally removed, with an average removal rate of 89.2%. There was no reoperation (0%) within 1 month in all cases. CONCLUSIONS Three types of SCOT contributed to planned surgical outcome including maximal tumor resection without serious related complications. We will proceed with verification of clinical effects, and develop robotized devices, and utilize AI for strategy desk at Hyper SCOT.

scholarly journals Supracerebellar infratentorial sitting craniotomy for a pinealoblastoma

2016 ◽  
Vol 40 (videosuppl1) ◽  
pp. 1
Author(s):  
Omar Choudhri ◽  
Steven D. Chang
Keyword(s):  
Third Ventricle ◽  
Tumor Resection ◽  
Standard Of Care ◽  
Surgical Anatomy ◽  
Pineal Region ◽  
Who Grade ◽  
Biopsy Site ◽  
Supracerebellar Infratentorial Approach ◽  
Aggressive Tumors ◽  
Tentorial Incisura

Pinealoblastomas are WHO grade IV tumors of the pineal region and comprise up to 50% of all pineal parenchymal tumors. They are highly aggressive tumors that spread along the craniospinal axis and are most commonly seen in children. The standard of care involves maximal surgical resection and chemoradiation following tissue diagnosis. We present the rare case of a large pinealoblastoma in an 18-year-old girl who presented with headaches and Parinaud's syndrome from tectal compression. An attempt was made at endoscopic transventricular biopsy of the tumor at an outside hospital, but it was aborted given bleeding at the biopsy site. We performed a supracerebellar infratentorial approach in a sitting position to achieve a gross-total resection of the tumor. This video case illustrates techniques for setting up a sitting craniotomy and approaching a previously biopsied hemorrhagic pinealoblastoma. The venous conglomerate at the tentorial incisura was found to be enveloped by the tumor and a thickened arachnoid scar. Surgical anatomy of the third ventricle and the pineal region is illustrated in this case through the process of surgical dissection and tumor resection.The video can be found here: https://youtu.be/CzB0lFQ7AyI.

Posterior Cervical Laminoplasty for Resection Intradural Extramedullary Spinal Meningioma: 2-Dimensional Operative Video

2018 ◽  
Vol 16 (3) ◽  
pp. 392-392 ◽  
Author(s):  
Simone E Dekker ◽  
Thomas A Ostergard ◽  
Chad A Glenn ◽  
Efrem Cox ◽  
Nicholas C Bambakidis
Keyword(s):  
High Speed ◽  
Treatment Options ◽  
Tumor Resection ◽  
Cervical Laminoplasty ◽  
En Bloc ◽  
Who Grade ◽  
Left Hand ◽  
Postoperative Mri ◽  
Intradural Extramedullary ◽  
Magnetic Resonance Imaging Mri

Abstract This operative video demonstrates a posterior cervical laminoplasty for the resection of a cervical intradural extramedullary meningioma. In addition, the natural history, treatment options, and potential complications are discussed. The patient is a 68-yr-old male who presented with left-hand grip weakness and paresthesias. Magnetic resonance imaging (MRI) demonstrated an enhancing mass that displacing the spinal cord anteriorly and causing severe flattening of the cord at C4 and C5. The patient underwent a posterior cervical laminoplasty for tumor resection. Removal of the dorsal elements with a high-speed drill was performed at C3, C4, and C5. A midline durotomy was performed and a large extra-axial intradural tumor was encountered. The tumor was resected en bloc and specimens were sent for permanent pathological analysis. The dura was closed in a watertight fashion using 6-0 Prolene sutures. The laminoplasty was performed by using titanium miniplates and screws to reconstruct the dorsal bony elements, and the wound was closed in layers using sutures. There were no complications. Final pathology was consistent with a WHO grade I meningioma. Postoperative MRI demonstrated gross total resection. The patient's perioperative course was uncomplicated and his preoperative weakness completely resolved by time of discharge.

scholarly journals Complete Resection of a Large Mediastinal Calcifying Fibrous Tumor

2020 ◽  
Vol 08 (01) ◽  
pp. e48-e51
Author(s):  
Géraldine L. P. Bono ◽  
Markus Lehner ◽  
Freimut H. Schilling ◽  
Nikolai Stahr ◽  
Miriam Nowack ◽  
...  
Keyword(s):  
Tumor Resection ◽  
Residual Tumor ◽  
Anterior Mediastinum ◽  
Complete Resection ◽  
Venous Stasis ◽  
Surgical Removal ◽  
Large Tumor ◽  
Calcifying Fibrous Tumor ◽  
Magnetic Resonance Imaging Mri ◽  
Fibrous Tumor

AbstractCalcifying fibrous tumor (CFT) is a benign tumor entity which can present in a variety of different sites. Till date, eight cases with a mediastinal manifestation have been published in literature. Surgical removal is the treatment of choice for this often incidentally detected tumor. Surgery of thoracic CFT may be challenging due to its localization within the mediastinum. A 10-year old boy with a right-sided thoracic pectus carinatum-like deformity was referred for further evaluation, incidentally, revealing a mediastinal mass in computed tomography (CT). Laboratory results were all within normal range. Magnetic resonance imaging (MRI) showed a large tumor in the upper anterior mediastinum suggesting expansive but not infiltrative character. The tumor was displacing surrounding structures like the heart and the diaphragm. Lower venous stasis with dilation of the inferior cava vein could be demonstrated. The tumor was considered to be of benign dignity and surgical removal was indicated. Complete tumor resection could be achieved through a sternotomy approach, along with thymectomy. A partial resection of both the pericardium and diaphragm was required due to adhesion with soft tissue at those sites. The specimen's size was 320 mm × 145 mm × 100 mm, histologically confirmed as CFT. The patient showed no residual tumor at 3- and 9-month follow-up. This case is a report on a large mediastinal CFT which underwent successful complete surgical removal. Following tumor resection, prognosis is considered to be good; however, key issue is complete resection to avoid local tumor recurrence.

Magnetic Resonance Imaging in the Diagnosis and Treatment of Lhermitte-Duclos Disease (Dysplastic Gangliocytoma of the Cerebellum)

Neurosurgery ◽  
1988 ◽  
Vol 23 (2) ◽  
pp. 240-245 ◽  
Author(s):  
Ralph F. Reeder ◽  
Richard L. Saunders ◽  
David W. Roberts ◽  
Jonathan D. Fratkin ◽  
Laurence D. Cromwell
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
High Probability ◽  
Residual Tumor ◽  
Resonance Imaging ◽  
Dysplastic Gangliocytoma ◽  
First Case ◽  
Clinically Significant ◽  
Magnetic Resonance Imaging Mri ◽  
Significant Mass

ABSTRACT Lhermitte-Duclos disease is a rare disorder characterized by a slowly enlarging mass lesion in the cerebellum. The pathophysiology of the disease is poorly understood, but recent reports have suggested that a clinically significant mass may recur many years after total gross removal of the tumor. Computed tomography and angiography have been relatively insensitive in imaging the tumor either at presentation or during the postoperative period. The authors present three patients with histologically proven Lhermitte-Duclos disease who have undergone magnetic resonance imaging (MRI). In the first case, preoperative T1- and T2-weighted images clearly demonstrated the location and extent of the lesion, which was verified at operation. In the other two cases, only postoperative images were obtained. These demonstrated the extent of residual tumor and indicate a high probability of lesion expansion during periods of 4 and 10 years, respectively. We conclude that MRI is an important tool in both the diagnosis and the treatment of patients with Lhermitte-Duclos disease.

scholarly journals Midline Suboccipital Subtonsillar Approach with C1 Laminectomy for Resection of Foramen Magnum Meningioma: 2-Dimensional Operative Video

2019 ◽  
Vol 80 (S 04) ◽  
pp. S365-S367
Author(s):  
Stefan Lieber ◽  
Maximiliano Nunez ◽  
Rocio Evangelista-Zamora ◽  
Marcos Tatagiba
Keyword(s):  
Cranial Nerves ◽  
Inpatient Rehabilitation ◽  
Craniocervical Junction ◽  
Foramen Magnum ◽  
Proliferation Index ◽  
Surgical Approaches ◽  
Who Grade ◽  
Magnetic Resonance Imaging Mri ◽  
The Right ◽  
Lower Cranial Nerves

AbstractWe present a case of a medium-sized foramen magnum meningioma that was resected through a midline suboccipital subtonsillar approach with C1 laminectomy in prone (Concorde) position. The patient is a 77-year-old woman with a 6-month history of intermittent vertigo, moderate gait instability, and slight decline of memory.On magnetic resonance imaging (MRI) an extra-axial intradural lesion was discovered that originated from the right epicondylar region just inferior to the jugular tubercle and occupied the anterolateral aspect of the foramen magnum. There was moderate transposition and compression of the medulla at the level of the cerebellar tonsils.On physical examination the patient was ambulatory independently without motor weakness but exhibited some gait instability. The function of the lower cranial nerves was preserved.A gross-total resection was achieved, histopathology confirmed a WHO grade-I meningothelial meningioma with a low-proliferation index. The patient was discharged home 5 days after surgery, her gait instability improved significantly immediately after surgery and had resolved completely after 2 weeks of inpatient rehabilitation. There was no other neurological deficit. At 3-month follow-up MRI, there was no indication of meningioma residual or recurrence.In summary, the midline suboccipital subtonsillar approach is a powerful tool with limited morbidity in the armamentarium for the microsurgical management of a variety of pathologies residing in the posterior cranial fossa and the craniocervical junction. Oftentimes the space created by the pathology opens up corridors that can be exploited for microsurgical access to avoid more extensive surgical approaches.The link to the video can be found at: https://youtu.be/0uUxs13ze7w.

scholarly journals Anterior Clinoid Metastasis Removed Extradurally: First Case Report

2018 ◽  
Vol 79 (02) ◽  
pp. e55-e62 ◽  
Author(s):  
Mirza Pojskić ◽  
Blazej Zbytek ◽  
Kenan Arnautović
Keyword(s):  
Case Report ◽  
Malignant Tumors ◽  
Tumor Resection ◽  
Disease Recurrence ◽  
Double Vision ◽  
Anterior Clinoid Process ◽  
First Case ◽  
Magnetic Resonance Imaging Mri ◽  
The Right

Background We report a case of isolated metastasis on the anterior clinoid process (ACP) mimicking meningioma. Clinical Presentation A 58-year-old male presented with headaches, right-sided visual disturbances, and blurred and double vision. The cause of double vision was partial weakness of the right III nerve, resulting from compression of the nerve by “hypertrophied” tumor-involved right anterior clinoid. Medical history revealed two primary malignant tumors—male breast cancer and prostate cancer (diagnosed 6 and 18 months prior, respectively). The patient was treated with chemotherapy and showed no signs of active disease, recurrence, or metastasis. Postcontrast head magnetic resonance imaging (MRI) showed extra-axial well-bordered enhancing mass measuring 1.6 × 1.1 × 1 × 1 cm (anteroposterior, transverse, and craniocaudal dimensions) on the ACP, resembling a clinoidal meningioma. Extradural clinoidectomy with tumor resection was performed via right orbitozygomatic pretemporal skull base approach. Visual symptoms improved. Follow-up MRI showed no signs of tumor residual or recurrence. Conclusion This is the first case report of a metastasis of any kind on ACP. Metastasis should be included as a part of the differential diagnosis of lesions of the anterior clinoid. Extradural clinoidectomy is a safe and effective method in the treatment of these tumors.

scholarly journals Adult sacrococcygeal teratoma excised by endoscopic surgery with a transsacral approach: a case report

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Ryosuke Machi ◽  
Chikashi Hiranuma ◽  
Hayato Suzuki ◽  
Masakazu Hattori ◽  
Kenji Doden ◽  
...  
Keyword(s):  
Histopathological Examination ◽  
Mature Teratoma ◽  
Tumor Resection ◽  
Residual Tumor ◽  
Cystic Mass ◽  
Cystic Tumor ◽  
Sacrococcygeal Teratoma ◽  
Transsacral Approach ◽  
Surgical Field ◽  
Magnetic Resonance Imaging Mri

Abstract Background Although sacrococcygeal teratoma (SCT) is relatively common in children, it is rare in adults. The prognosis for malignant cases is poor, so prompt surgical resection is required. Transabdominal and transsacral approaches are common approaches for tumor resection. In recent years, there have been reports of tumor removal with laparoscopic assistance, but all have applied transabdominal approaches. Case presentation A 27-year-old woman visited our gynecology department because of abdominal pain and genital bleeding. Magnetic resonance imaging (MRI) revealed a 3-cm-sized cystic mass in the left retrorectal area, and she was referred to our department for detailed examinations and treatment. She was diagnosed with a presacral cystic tumor and decided to undergo surgery. We used a transsacral approach to perform tumor excision. Since it was difficult to confirm the deep part of the tumor through direct visualization, we used GelPOINT® Path (a transanal access platform) and AirSEAL® System (insufflation device) to remove the tumor endoscopically. The postoperative course was uneventful with no bladder or rectal dysfunction. Histopathological examination revealed a mature teratoma. Conclusions When the tumor is relatively small and located in the lower sacrum, the endoscopically assisted transsacral approach can establish a stable field of view by expanding the depth of the surgical field. This method is useful considering its ability to perform excision without leaving residual tumor tissue and satisfactory safety and cosmetic results.

scholarly journals Transcavernous Approach for Gross Total Resection of a Dumbbell-Shaped Giant Trigeminal Schwannoma

2021 ◽  
Author(s):  
Sima Sayyahmelli ◽  
Emel Avci ◽  
Burak Ozaydin ◽  
Mustafa K. Başkaya
Keyword(s):  
Skull Base ◽  
Cavernous Sinus ◽  
Tumor Resection ◽  
Mass Effect ◽  
World Health ◽  
Gross Total Resection ◽  
Petrous Apex ◽  
Total Resection ◽  
Who Grade ◽  
Trigeminal Schwannoma

AbstractTrigeminal schwannomas are rare nerve sheet tumors that represent the second most common intracranial site of occurrence after vestibular nerve origins. Microsurgical resection of giant dumbbell-shaped trigeminal schwannomas often requires complex skull base approaches. The extradural transcavernous approach is effective for the resection of these giant tumors involving the cavernous sinus.The patient is a 72-year-old man with headache, dizziness, imbalance, and cognitive decline. Neurological examination revealed left-sided sixth nerve palsy, a diminished corneal reflex, and wasting of temporalis muscle. Magnetic resonance imaging (MRI) showed a giant homogeneously enhancing dumbbell-shaped extra-axial mass centered within the left cavernous sinus, Meckel's cave, and the petrous apex, with extension to the cerebellopontine angle. There was a significant mass effect on the brain stem causing hydrocephalus. Computed tomography (CT) scan showed erosion of the petrous apex resulting in partial anterior autopetrosectomy (Figs. 1 and 2).The decision was made to proceed with tumor resection using a transcavernous approach. Gross total resection was achieved. The surgery and postoperative course were uneventful, and the patient woke up the same as in the preoperative period. MRI confirmed gross total resection of the tumor. The histopathology was a trigeminal schwannoma, World Health Organization (WHO) grade I. The patient continues to do well without any recurrence at 15-month follow-up.This video demonstrates important steps of the microsurgical skull base techniques for resection of these challenging tumors.The link to the video can be found at https://youtu.be/TMK5363836M

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