Spinal endodermal cyst undergoing malignant transformation and marked elevation of serum carbohydrate 19-9 level.

Endodermal cyst (EC) is a benign tumor that can arise along the craniospinal axis. Infrequently, ECs undergo malignant transformation. A 43-year-old man presented with numbness in the right arm, leg and occipitalgia. MRI revealed a multicystic, intradural extramedullary tumor at C2 with enhancement along the ventral surface of the spinal cord. Blood test showed an abnormal increase in serum carbohydrate antigen 19-9 (CA 19-9) level. Systemic positron emission tomography-computed tomography was normal. He underwent total tumor resection and was diagnosed with EC. He developed double vision, hearing loss, and swallowing difficulty on postoperative day 70. Cerebral MRI revealed marked extensions of leptomeningeal dissemination. The serum CA 19-9 level increased continuously and finally reached 1515.0 U/ml. He died of respiratory failure on day 108. An autopsy did not reveal abnormalities in the abdominal and chest organs. On microscopic examination, the post-mortem specimen revealed adenocarcinoma. Immunohistochemically, both the surgical and autopsy specimens were positive for CA 19-9. Spinal ECs may lead to malignant transformation with leptomeningeal dissemination that causes abnormal elevation of serum CA 19-9 levels.

Pipeline Flex with Shield Technology Placement for Small- and Medium-sized Aneurysms Achieves Early Complete Occlusion.

The flow diverter has been shown to be a safe and effective device for large cerebral aneurysms in the proximal internal carotid artery (ICA). Recently, its indication has been expanded to small- and medium-sized cerebral aneurysms in the distal segment of the ICA. In this study, we report a single-center, retrospective investigation of the safety and efficacy of the Pipeline Flex device to treat these aneurysms. Of the patients who underwent Pipeline implantation for small- and medium-sized ICA aneurysms (≤12 mm) at our hospital between July 2013 and October 2021, 102 patients with 104 aneurysms were included in this study. The mean age of the patients was 57.7 ± 12.1 years, and 94 (90.4%) were female. The mean aneurysmal dome diameter was 9.2 ± 2.3 mm, the mean neck diameter was 5.3 ± 1.6 mm, and the mean dome-to-neck ratio was 1.8 ± 0.5. Twenty-five patients (24.0%) had incorporated vessels from the aneurysm. Complete occlusion of the aneurysms was obtained in 96 patients (92.3%). There were no cases of parent artery stenosis or major stroke after the procedure. Absence of incorporated vessel from the aneurysm dome and adjunctive coil embolization are statistically significant factors indicating complete occlusion in multivariate analysis. The time to complete occlusion was determined earlier with the use of the Pipeline Shield (p = 0.0386) and with adjunctive coils (p = 0.0025). We showed that Pipeline implantation for small- and medium-sized aneurysms was safe and highly effective.

Preliminary Experience of the Surpass Streamline Flow Diverter for Large and Giant Unruptured Internal Carotid Artery Aneurysms.

As of January 2021, the Surpass Streamline (SS) is the most recently approved flow diverter in Japan. A total of 28 Japanese patients, including 9 clinical trial patients, with 28 large or giant unruptured internal carotid artery (ICA) aneurysms, underwent SS embolization at Juntendo University Hospital. Procedural failure occurred in two patients due to the difficulty to navigate the device in the tortuous parent artery. Therefore, 26 patients with 26 aneurysms were available for clinical and anatomical assessments. Patients' mean age was 62.6 years (range 46-86), and 24 patients (92.3%) were female. Mean aneurysm size and neck width were 15.4 mm and 7.7 mm, respectively, with 20 saccular and 6 fusiform aneurysms. Seven aneurysms were symptomatic due to the aneurysmal mass effect. Twenty patients underwent a 6-month follow-up angiography to evaluate the degree of occlusion. Anatomical outcomes were 12 (60%) complete occlusion (CO), 4 (20%) residual neck (RN), and 4 (20%) residual aneurysm. Favorable aneurysm occlusion consisted of CO, and RN was achieved in 16 (80.0%). There were no significant device stenoses. Aneurysmal mass effect improved in one and was unchanged in eight patients. There were three device-related complications, namely, delayed aneurysm rupture, minor ischemic stroke, and device occlusion (11.5%). One patient with minor ischemic stroke fully recovered before 30 days, and our series showed 7.7% risk of major ipsilateral stroke and neurological death at 30 days. The SS embolization for large and giant unruptured ICA aneurysms offers satisfactory anatomical and clinical outcomes with a low risk of device-related complications.

A large cystic meningioma incidentally detected during general examination for breast cancer.

A 57-year-old woman who underwent needle biopsy for a subcutaneous mass in the breast was diagnosed with invasive ductal carcinoma. General examination incidentally revealed an intracranial tumor. At presentation, the patient showed memory disturbance but no focal neurological deficits. Cranial computed tomography (CT) revealed a large, hypodense cyst in the left frontotemporal region, involving a tumor with extensive hyperostotic changes in the left sphenoid and frontal bones. Magnetic resonance imaging showed that the tumor was attached to the dura mater of the pterional region and extensively enhanced, with involvement of the frontal and sphenoid bones. The less vascular tumor was removed en bloc by drilling the affected sphenoid and frontal bones as much as possible. The microscopic findings of the tumor were consistent with meningothelial meningioma with invasion into the dura mater and bone. Cystic meningioma should be considered when encountered with a dural-based cystic tumor, even in patients with cancer. In such circumstances, prompt and preferential resection may be indicated for intracranial tumors for timely initiation of the long-term treatment of cancer.

Vertebral artery compression of the medulla oblongata: A benign radiological finding?

BACKGROUND: To the best of our knowledge, no study has documented the natural history of rostral medullary compression of the vertebral artery (RMCVA) as radiological finding. The aim of this study was to explore it. METHODS: A total of 57 patients with RMCVA and not presenting symptoms of medullary compression syndrome were enrolled. These participants underwent cerebral magnetic resonance imaging with contrast, and 19 of them who were followed for 5.7 ± 1.9 years (range: 3.0-10.3 years) were analyzed in detail. For comparison, clinical courses of two other patients with vertebrobasilar dolichoectasia (VBDE) were presented. RESULTS: RMCVA was well delineated in all 57 patients. In the 19 patients analyzed, RMCVA was found in 17 sides on the right and 15 on the left. Moreover, the ventrolateral medulla was the most frequent compression site, and it was found in 69% of cases, with 84.2% presenting as mild compression and 15.8% as considerable compression. During the follow-up period, no patients showed neurological deterioration or radiological progression. In contrast, the two VBDE patients demonstrated both neurological and radiological progressions during the follow-up period. CONCLUSION: Unlike VBDE, RMCVA seems to be a benign condition without progression, even when with a considerable compression. Degree of the compression in RMCVA may not be relevant to the patient's neurological status.

Simultaneous presentation of subcortical hemorrhage, subdural hemorrhage, and cerebral infarct in a hemiplegic patient.

A 90-year-old, non-hypertensive woman presented gait disturbance followed by falls. She had developed a lacunar infarction in the right frontal lobe 1 month previously that subsequently resulted in subtle motor weakness in the left lower extremity. At presentation, the patient showed motor weakness in the left upper and lower extremities with normal findings on blood test. Cranial computed tomography (CT) revealed a subcortical hemorrhage in the anterior part of the right frontal lobe that was accompanied by perilesional edema. In addition, two small subdural hematomas, apparently in the acute phase, were found. Magnetic resonance imaging performed immediately after the CT revealed hyperacute infarct in the right precentral gyrus adjacent to the previous infarct. It was hyperintense on the diffusion-weighted imaging but indistinct on the fluid-attenuated inversion recovery sequence. In addition, findings suggesting cerebral contusions were not observed. Based on these, we assumed that the patient's symptoms were mainly derived from the infarct and the subdural hemorrhages had developed in association with falls. However, it was unclear whether the infarct had developed before or after the formation of subcortical hemorrhage. Traumatic and non-traumatic intracranial hemorrhage and cerebral infarcts may present simultaneously. When intracranial hemorrhages appearing on CT do not adequately explain the patient's neurological findings, undetected cerebral ischemia should be assumed.

Delayed traumatic intracerebral hemorrhage associated with dolichoectasia of the middle cerebral artery.

A 74-year-old man tripped while walking. He had not been administered antiplatelet or anticoagulation therapy. At presentation, the patient was well-oriented, with a blood pressure of 130/91 mmHg, while present with tetraplegia and numbness in the upper extremities. Blood work revealed normal findings, while magnetic resonance imaging of the cervical spine revealed severe cord compression at the C3/4 and C4/5 levels. Cranial computed tomography (CT) showed elongated masses in the Sylvian fissures without intracranial hemorrhage. CT taken 2 days later revealed an intraparenchymal hemorrhage located mainly in the right putaminal region, while the patient showed no signs of neurological deterioration. Three-dimensional CT angiography (3D CTA) demonstrated marked ectasia and elongation in the right internal carotid, bilateral middle cerebral, and left anterior cerebral arteries. The patient was conservatively managed. Repeat 3D CTA performed 3 months later showed no de novo abnormalities in the ectatic cerebral arteries. It is assumed that the delayed traumatic intracerebral hemorrhage was caused by disruption of the perforating vessels arising from the ipsilateral dolichoectatic middle cerebral artery. Periodical surveillance neuroimaging is recommended for patients with head trauma who are simultaneously diagnosed with incidental dolichoectasia, especially when complicated with cervical cord injury.

Bilateral vertebral artery fenestrations in the craniocervical junction region: the clinical role of 3D computed tomography angiography.

Fenestration of the vertebral artery (FVA) is a rare occurrence that comprises only 2% of fenestrations formed in the intracranial arteries. Herein, we report a unique case of bilateral FVA in the craniocervical junction (CCJ) region using conventional magnetic resonance angiography (MRA) and three-dimensional computed tomography angiography (3D-CTA). Compared to MRA, 3D-CTA was more sensitive in delineating the FVAs located in the region. Furthermore, it effectively depicted the relationships between the VAs and surrounding bony structures. The clinical implications of FVAs can be better understood in relation to the surrounding bony structures. 3D-CTA is mandatory when performing endovascular and open surgical interventions in and around the VA segments in the CCJ region.

Late orbital metastasis from colon cancer complicated by multiple tumors in the breast, lung, liver, and spine.

A 69-year-old woman sustained progressive proptosis for 2 months. The patient had undergone surgery for resection of colon cancer 10 years prior, which was considered to have been completely cured 5 years prior. She was also aware of a hard mass in her left breast, but it had been left untreated. Furthermore, she experienced back pain for a month. Blood examination revealed an elevated level of serum carcinoembryonic antigen, at 17.4 ng/mL (< 5). Computed tomography (CT) revealed a tumor occupying the superolateral part of the right orbit, with intratumoral calcifications and destructive changes in the lateral orbital wall. On magnetic resonance imaging, it appeared as a heterogeneously enhancing, extraconal tumor, 44 mm × 31 mm in maximal dimension, extending into the middle fossa and the adjacent subcutaneous region. Fluorodeoxyglucose positron emission tomography/CT revealed abnormal accumulation in the left breast, T12 vertebra, liver, and lung, in addition to the orbital tumor. The patient underwent total tumor resection through a lateral orbitotomy. Histological examination of the tumor was highly suggestive of a metastatic colon cancer. Late metastasis should be assumed as a differential diagnosis that can be determined only through histological verification.

Mucoepidermoid carcinoma of the lacrimal gland in a patient with the CRTC1-MAML2 fusion gene.

Mucoepidermoid carcinoma (MEC) of the lacrimal gland (LG) is a rare entity. A 47-year-old woman was aware of periorbital swelling for 3 months. At presentation, the patient showed periorbital swelling in the right eye. CT scan showed an isodense mass in the anterior superolateral part of the orbit. MRI delineated the mass as enhancing, extra-conal tumor appearing isointense on T1-weighted sequences, and to be of mixed intensity on T2-weighted sequences. The tumor was totally resected. Microscopically, the tumor tissue was comprised of squamous, epithelioid cells, and cells with plump and clear cytoplasm. Necrosis, neural invasion, or mitotic figures were not observed. Immunohistochemical examination revealed intense staining for cytokeratin 7. A subset of the cells was positively stained with periodic acid-Schiff and mucicarmine stains. Genetic analysis revealed the presence of the CRTC1-MAML2 fusion. The CRTC1-MAML2 fusion may be a useful indicator for the prognosis and planning of adjuvant therapy.

An infantile case of posterior fossa arachnoid cyst presenting with marked engorgement of contralateral supraorbital vein.

A 6-month-old, previously healthy boy presented with poor feeding and vomiting for 2 weeks. At presentation, his left supraorbital vein was found to engorge considerably. A cranial computed tomography (CT) scan revealed a large cyst in the posterior fossa with a prominent left superior ophthalmic vein (SOV). Magnetic resonance imaging delineated the well-demarcated cyst markedly compressing the right cerebellar hemisphere. The patient underwent microscopic fenestration of the cyst wall through lateral suboccipital craniotomy. The cyst wall, comprising multi-layered, opacified membranes, was extensively resected and communicated with the prepontine cistern. The histological appearance of the cyst wall was consistent with an arachnoid cyst. Engorgement of the left supraorbital vein resolved immediately after surgery. A postoperative CT scan did not identify the SOV in the left orbit. In the present case, engorgement of the supraorbital vein might reflect the increased venous pressure of the SOV caused by compression of the contralateral cerebellar hemisphere.

Traumatic cervical vertebral artery aneurysm associated with suicidal stabs.

BACKGROUND: Cervical vertebral artery (VA) aneurysm occasionally develops in association with penetrating injury. However, its treatment strategy is not yet determined. CASE DESCRIPTION: A 50-year-old woman with bipolar disorder attempted suicide by stabbing herself in the lateral neck. At presentation, focal neurological deficits were not observed. Spinal computed tomography (CT) showed unclear delineation of the VA in the right C4/5 intervertebral foramen. CT performed 7 days later identified an aneurysm of the right VA at C4/5, with abnormal arteriovenous shunts between the aneurysm and paravertebral venous plexus. The patient underwent coil embolization of the VA segment involving the aneurysm on the same day that was complicated by cerebellar ataxia due to procedure-associated infarction. CONCLUSION: Traumatic VA aneurysms associated with penetrating injuries should be carefully managed with a detailed presurgical evaluation of the relevant cranial and spinal structures.

An unprecedented case of penetrating head trauma caused by shoji (a Japanese-style paper sliding door).

Penetrating head trauma (PHT) includes any traumatic injury where an object pierces the skull and breaches the dural membrane surrounding the brain. PHTs are less prevalent than blunt head injuries. However, they often have more complex damage, worse prognosis, and higher rates of morbidity and mortality. An 83-year-old man fell at his home and hit his head on the right side toward a shoji (a Japanese-style paper sliding door). He reported to the emergency room the following day with his family. He had a small wound before the right ear, which was sutured in the emergency room. A CT scan demonstrated tiny pieces of bone fragments inside the brain, as well as right temporal subcortical hemorrhage and pneumocephalus. He was admitted to the hospital and received intensive prophylaxis with antibiotics. He developed life-threatening skin disease and subsequent acute kidney disease requiring hemodialysis. He fully recovered from his life-threatening condition. Here, we report an unprecedented case of a penetrating head injury of an older adult caused by a shoji.

Infantile acute subdural hemorrhage probably caused by injury to the diploic channels.

A 6-month-old, previously healthy boy hit the right frontal region of his head against the corner of a plastic toy box. At presentation, a small area with scalp discoloration was noted in the right frontal region. Head computed tomography at the level of discoloration revealed an intracranial hematoma, 5 mm in maximal thickness, below the coronal suture. In addition, there were bony bridges connecting the frontal and parietal bones. Furthermore, a linear crack was found in the diploe of the frontal bone in contact with the coronal suture. Cerebral MRI confirmed linear hyperintensity between the inner tables. We assumed that a kind of ping-pong ball fracture was caused by the head blow, centering on the coronal suture, resulting in rupture of contacting diploic channels and tear of the dura mater, and forming a subdural hematoma. Acute subdural hemorrhage may be caused by an insignificant blow to the coronal suture in infantile patients with underdeveloped cranial bones and sutures, accompanied by subtle external findings.

Supratentorial extraventricular ependymoma presenting calvarial erosion: A report of 3 cases.

Calvarial erosion is an infrequent manifestation associated with oligodendrogliomas, astrocytomas, dysembryoplastic neuroepithelial tumors, astroblastomas, glioblastomas, and meningiomas. Anaplastic ependymoma (AE), a rare malignant form of ependymoma, commonly results in poor prognosis. During the last 12 years, six patients were diagnosed with supratentorial ependymomas. All of them were AEs with extraventricular location identified in the right parietal, left parietal, and left frontal lobes, respectively. Three of them, 7-, 15, and 17-year-old male patients, presented focal calvarial erosion with smooth contour. Calvarial erosion may be a diagnostic hallmark of supratentorial AEs.

Quadrigeminal cistern arachnoid cyst as a probable cause of hemifacial spasm.

Arachnoid cysts arising in the quadrigeminal cistern (ACQCs) are uncommon. A 68-year-old woman presented with an unsteady gait, facial spasm, and cerebellar ataxia. Non-contrast head computed tomography showed a cystic mass centered in the quadrigeminal cistern accompanying ventriculomegaly. On MRI, the cyst appeared hypointense on T1- and hyperintense on T2-weighted sequence. There was no restricted diffusion on diffusion-weighted imaging. The cerebral aqueduct was obstructed and the prepontine cistern was narrowed. The left vertebral artery (VA) coursed adjacent to the facial nerve at its origin. The patient underwent neuroendoscopic fenestration of the posterior wall of the third ventricle and ventral wall of the ACQC. Postoperatively, the patient's symptoms resolved. MRI showed a considerable reduction in the ACQC and expansion of the prepontine cistern, whereas the relationship between the left VA and the proximal segment of the facial nerve did not change. We assumed that the pre-existing close relationship between the VA and facial nerve might have been aggravated by the anterior displacement of the brainstem, thus causing the facial spasm.

Dural arteriovenous fistula with crista galli erosion in a patient with suspected sinusitis.

Dural arteriovenous fistula (dAVF) of the anterior cranial fossa is an aggressive entity with a high risk of intracranial hemorrhage. A 38-year-old woman presented to our institution with nasal obstruction and discharge. Computed tomography (CT) scans performed for suspected sinusitis revealed erosion of the crista galli predominantly on the left side. The frontal and ethmoidal sinuses adjacent to the crista galli were clear and did not communicate with the crista galli. Contrast computed tomography scans revealed ectatic vessels adjacent to the crista galli, coursing on the cortical surface. Cerebral angiography identified an anterior fossa dAVF supplied by the bilateral anterior and posterior ethmoidal arteries and drained by the dilated cortical veins. Based on these findings, we assumed that long-term compression by the pulsatile draining veins caused the erosion. The patient underwent successful microsurgical disconnection procedure of the dAVF with an uneventful postoperative course. We concluded that external erosion of the crista galli could be a sign of aggressive dAVF and requires careful examination.

Microvascular proliferation in the clots: The key finding of acute subdural hematoma transforming into chronic subdural hematoma?

BACKGROUND: Despite extensive investigations, the exact etiology of chronic subdural hematoma (CSDH) remains elusive. Organized CSDHs are a distinct but less-understood type of CSDH. CASE DESCRIPTION: A 50-year-old hypertensive woman experienced headache without any previous head injury. At presentation, the patient showed no focal neurological deficits. Cranial computed tomography (CT) revealed a slightly compressive subdural hematoma that spontaneously regressed and no intracranial vascular lesions. Cerebral magnetic resonance imaging identified a non-enhancing nodular lesion in the subdural hematoma. After the patient presented disorientation and aphasia on post hospitalization day 14, CT showed a considerable enlargement of the subdural hematoma. Partial removal of the bi-layered hematoma was performed through a parietal craniotomy. Histological examination revealed microvascular proliferation in both the outer membrane and the nodular lesion. On postoperative day 35, CT demonstrated a remarkable resolution of the residual hematoma. CONCLUSION: Development of microvascular proliferation in the clots of an acute subdural hematoma may lead to its rapid enlargement as an organized CSDH. Organized CSDH can be managed by partial removal of the outer membrane and hematoma through a craniotomy.

Atypical meningeal hemangiopericytoma presenting with punched-out calvarial erosion.

Meningeal hemangiopericytoma (HPC) is an infrequent but distinct entity affecting the craniospinal axis. A previously healthy 48-year-old man sustained a gradually progressing motor weakness in the left lower extremity. CT showed a hyperdense mass in the right frontal lobe. On MRI, it was 29 × 30 × 36 mm in dimension, appeared isointense on T1 and hyperintense on T2, and was intensely enhanced with erosive changes in the inner table adjacent to the tumor. The patient underwent tumor resection. Reflection of the bone flap revealed a punched-out erosion in the inner table with a defect of the dura over the upper part of the tumor. Microscopic findings were consistent with grade III HPC with dural invasion. A punched-out calvarial erosion and dural defect caused by an extra-axial tumor may be a high-grade HPC that requires extensive surgical resection.

Endovascular Therapy for Aneurysmal Subarachnoid Hemorrhage Complicated by Neurogenic Pulmonary Edema and Takotsubo-Like Cardiomyopathy: A Report of Ten Cases.

OBJECTIVE: Patients sustaining aneurysmal subarachnoid hemorrhage (aSAH) can be further complicated by neurogenic pulmonary edema (NPE) and Takotsubo-like cardiomyopathy (TCM) with dismal outcomes. The present study aimed to validate the efficacy of endovascular therapy for patients with aSAH complicated by NPE and TCM. MATERIALS AND METHODS: Patients who were diagnosed with aSAH complicated by NPE and TCM and treated by endovascular therapy were retrospectively evaluated. RESULTS: In the past 5 years, a total of ten female patients with aSAH were also diagnosed with NPE and TCM. Six of the ten were cases with high-grade aSAH (Hunt and Hess Grades IV and V), whereas four were low-grade aSAH (Grades II and III). The locations of ruptured aneurysms were the internal carotid-posterior communicating artery junctional site in five patients, the anterior communicating artery in two, the vertebral artery in two, and the middle cerebral artery in one. These aneurysms were successfully embolized by endovascular therapy without any procedure-associated complications. The clinical outcome measure at 6 months after discharge on the Modified Rankin Scale was found to be 0 in four patients, 1 in two, 3 in one, and 5 in three. CONCLUSIONS: Endovascular therapy can be a feasible, alternative measure for the treatment of patients with high-grade aSAH who also have NPE and TCM.