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.

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.

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.

Intramedullary spinal cord germinoma clinically mimicking multiple sclerosis: A case report.

BACKGROUND: It is important to differentiate intramedullary neoplastic lesions from nonneoplastic diseases such as multiple sclerosis (MS) and other demyelinating or inflammatory diseases. CASE DESCRIPTION: A 26-year-old Japanese male presented with a history of intracranial germinomas and obstructive hydrocephalus, treated with endoscopic surgery, and adjuvant chemotherapy and radiation therapy. Three years later, he developed paresthesias involving the right hand and both lower extremities. The cervical MR scan demonstrated a heterogeneously enhancing intramedullary C1-C2 lesion with surrounding edema. On cytological examination of the cerebrospinal fluid (CSF), there were no neoplastic cells. However, the fluid was positive for oligoclonal immunoglobulin G (IgG) bands. The patient received steroid pulse therapy to address the potential MS diagnosis. The follow-up MR showed reduced edema, but no change in the size of the intramedullary lesion. Therefore, the patient underwent a cervical laminectomy for tumor resection. The pathology was consistent with the same cranial germinoma treated 3 years previously. He subsequently received whole spinal radiation and three courses of chemotherapy. CONCLUSION: Some spinal cord tumors may produce oligoclonal IgG bands in CSF. In this case, an intramedullary C1-C2 spinal cord germinoma was originally misdiagnosed as MS due to the presence of oligoclonal IgG bands in CSF. Differentiating this tumor from MS and initiating appropriate treatment were critical into the care of this patient.

Intermittent oculomotor nerve paresis and hollow appearance on MRI manifested by pituitary adenoma.

A 71-year-old man had sustained intermittent ptosis and double vision for 2 weeks. Neurological examination found unilateral oculomotor nerve (CN III) paresis manifesting as limitations of gaze, ptosis, and mydriasis. Neither headache nor any other cranial neuropathy was noted. Cerebral magnetic resonance imaging revealed a well-circumscribed mass in the pituitary fossa extending laterally into the left cavernous sinus. The segment of the left CN III lying in the oculomotor cistern was considerably compressed by the tumor. The cisternal segments of the left CN III showed an undescribed, "hollow" appearance. The left orbit and brainstem were intact. The patient underwent tumor resection via an endoscopic transsphenoidal approach. The tumor tissue was soft in consistency, involving xanthochromic fluid. The pathological diagnosis was pituitary adenoma accompanied with considerable hemorrhagic changes. The patent's ptosis and limitations of gaze showed remarkable improvements on postoperative day 1, with resolution of the hollow appearance of the affected CN III that was confirmed on day 3. We assumed that the intralesional bleeds and lateral tumor extension into the oculomotor cistern were associated with the intermittent paresis of the CN III as the sole presentation. A hollow appearance identified in the CN III might indicate a reversible dysfunction of the nerve that can anticipate an improvement by prompt surgical intervention.

Orbital Arteriovenous Fistula Coexistent with an Arteriovenous Hemangioma: A Rare Occurrence and Review of Literature.

BACKGROUND: Orbital arteriovenous fistula (AVF) is a rare entity with only 17 cases reported so far. An arteriovenous hemangioma is a distinct entity of an angioma that most frequently affects the skin. However, to our knowledge, there has not been a case arising in the orbit. CASE DESCRIPTION: A previously healthy, 73-year-old man had progressive proptosis for 1 month. At presentation, the patient showed considerable proptosis, hyperemia in the lower eyelid, chemosis, and total ophthalmoplegia on the right side. Computed tomography and magnetic resonance imaging revealed a round retro-orbital mass, 23 mm in maximal dimension, and lying in the inferomedial aspect of the orbit with an irregular-shaped lesion in the orbital apex. Cerebral angiography detected an orbital AVF fed by the ipsilateral maxillary and ophthalmic arteries and draining into the inferior ophthalmic vein. Stain of the retro-orbital mass was not identified. A transvenous coil embolization via the facial and superior ophthalmic veins achieved complete isolation of the AVF with satisfactory outcome. Two months later, the orbital mass, well circumscribed and lacking perilesional hemosiderin deposition, was microsurgically resected via a lateral orbitotomy. Histological diagnosis was consistent with an arteriovenous hemangioma. CONCLUSIONS: An AVF and arteriovenous hemangioma may simultaneously develop in the same orbit. In patients with symptomatic orbital AVF and coexisting other orbital pathology, strategies should be carefully planned before setting about the treatment.

Spinal Dural Arteriovenous Fistula Assumed to be Symptomatic after Placement of Lumbar Cerebrospinal Fluid Drain.

A 69-year-old man presented with severe headache. Cranial computed tomography revealed diffuse subarachnoid hemorrhage. An anterior communicating artery aneurysm was identified and successfully obliterated by open microsurgery on the same day. Following placement of a continuous lumbar cerebrospinal fluid drain on hospitalization day 7, the patient developed a severe paraplegia and sensory loss below T6. Cerebral magnetic resonance imaging did not identify a responsible lesion. Spinal magnetic resonance imaging, however, showed extensive intramedullary hyperintensity on T2-weighted sequences. Spinal angiography identified a dural arteriovenous fistula fed by the segmental artery branching with the 12th intercostal artery. It was successfully embolized and the patient's sensorimotor disturbances remarkably improved. A spinal dural arteriovenous fistula may better be considered as one of the underlying etiologies when patients exhibit new neurological deficits after placement of a continuous lumbar cerebrospinal fluid drain.

Ossified extradural en-plaque meningioma of the cervical spine.

A 57-year-old woman sustained gradually progressive sensorimotor disturbance in the left upper extremity for one year. Neurological examination found a diminished sensation below the left C7 dermatome and reduced strength in the left interosseous muscles. Computed tomography of the cervical spine revealed a high density mass at C7, in the left dorsal part of the spinal canal. Magnetic resonance imaging found an enhancing, en-plaque tumor at C6-T1, involving a non-enhancing part, and considerable compression of the spinal cord. The patient underwent tumor resection through hemilaminectomy of C5-C7. The tumor was located epidurally, highly fibrous including bony-hard parts, and severely adhered to the dura mater that necessitated drilling for debulking. A subtotal resection was achieved and histological diagnosis was a fibrous meningioma with metaplastic ossification. Ossification may be a pathognomonic appearance of spinal extradural meningiomas that makes resection maneuvers difficult.

Osteochondroma Presenting as a Calcified Mass in the Sellar Region and Review of the Literature.

Objective Osteochondroma (OC) is the most common benign bone neoplasm. It infrequently occurs in the cranial cavity as a calcified lesion and very rarely presents in the sellar region. The present study summarizes the knowledge about OCs of the sellar region. Methods We searched the literature search for the clinical appearance of OCs and other calcified pathologies occurring in the sellar region. Results A total of 21 English-language articles published from 1961 to 2015 documented cases of calcified lesions in the sellar region including cerebral aneurysm, chondroid chordoma, chondroma, craniopharyngioma, OC, odontome, osteoma, pituitary adenoma, pituitary stone, Rathke cleft cyst, retinoblastoma, schwannoma, and xanthogranuloma. Among them, six were OC cases: three in the parasellar region and three in the sellar-suprasellar region. Patients with sellar-suprasellar OCs presented with visual loss and hypopituitarism; patients with parasellar OCs did not show these symptoms. OCs appeared as irregular and multilobulated calcifications on X-ray and computed tomography. On magnetic resonance imaging, OCs showed variable intensity on T1-weighted sequences and consistently heterogenous intensity on T2. Four patients underwent transcranial tumor resection, and the transsphenoidal route was selected for one. Five of the six resulted in a partial resection or internal decompression with a satisfactory outcome. Conclusions Calcified tumors occurring in the sellar region may be OCs, especially if they appear as irregular multilobulated calcification.

Skull base chondroid chordoma: atypical case manifesting as intratumoral hemorrhage and literature review.

OBJECTIVE: Chondroid chordoma (CC) is a rare but commonest subtype of chordoma with little reported clinical information. The present study summarizes and updates present knowledge of CC. METHODS: Literature search for demographic data and clinical appearance of cranial CCs except for those entirely confined to the sinonasal region. RESULTS: A total of 48 English language papers published from 1968-2013 were retrieved describing 132 CCs as skull base tumors. The male-to-female ratio was 1:1. The mean age at diagnosis was 43 years, predisposing to the third to fifth decades of life. The clival (34%) and spheno-occipital (29%) regions were the most frequent sites of origin followed by the sellar (12%) and sphenoid (5%) regions. Intratumoral calcification and bony erosion were identified as the characteristic neuroimaging findings. Surgical resection by the transcranial, transsphenoidal, transnasal, transpharyngeal, or transpalatal route with or without adjuvant radiotherapy was the main treatment option. The initial treatment outcome was satisfactory in 82% of cases with considerably better prognosis compared with typical chordomas. CONCLUSION: CC is a distinct entity to be discriminated from the typical type of chordoma. There are no distinguishing features on magnetic resonance imaging between CC and typical chordoma. Intratumoral calcification and concurrent bony erosion on neuroimaging should suggest the possibility of CC. Extensive surgical resection and adjuvant radiotherapy can achieve satisfactory outcome.

A ruptured aneurysm located at a collateral artery that extended from the proximal A2 segment to the M1 segment, associated with an anomalous branch of the anterior choroidal artery and middle cerebral artery hypoplasia: case report.

We here describe the first case of a ruptured aneurysm located at a collateral artery that extended from the proximal A2 segment to the M1 segment, which was associated with an anomalous branch of the anterior choroidal artery and middle cerebral artery (MCA) hypoplasia. The aneurysm was revealed by angiograms and intraoperative findings. No previous accounts have been published of such an extremely rare vessel anomaly. In practice, this case highlights the urgent need to preoperatively recognize such vascular anomalies, as well as to better understand the collateral blood supply in cerebral ischemia associated with these MCA anomalies. Such knowledge will be helpful for planning optimal surgical procedures.

Adiponectin and traumatic brain injury.

Adiponectin, a circulating adipose-derived hormone regulating inflammation and energy metabolism, has beneficial actions on cardiovascular disorders. Recent studies have suggested that adiponectin might be a potential molecular target for ischemic stroke therapy; however, little is known about the effects of adiponectin on traumatic brain injury. The present study examined the immunoactivity of adiponectin.Adult male Sprague-Dawley rats were subjected to lateral fluid percussion injury using the Dragonfly device. Immuno-histochemical studies showed that the adiponectin expression was increased in the cerebral cortex at 24 h after injury and in the hippocampus at 72 h after injury. Our findings suggest that adiponectin might participate in the pathophysiological process occurring after traumatic brain injury.

Cerebrospinal fluid congestion in the perioptic space.

Recent attention has been paid to the -cerebrospinal fluid (CSF) dynamics between the intracranial subarachnoid space (SAS) and the SAS around the optic nerve (ON-SAS). We experienced three patients who had an expanded ON-SAS associated with mass lesions extending into the optic canal, and studied their MRI findings after decompressive surgery. In all three patients, decompressive surgery of the optic canal resulted not only in the disappearance of the expanded ON-SAS, but also in improvement of the visual function. The present study may indicate that normalization of the ON-SAS can be considered to be the achievement of "effective" decompression. Therefore, we suggest that, in patients with an expanded ON-SAS associated with mass lesions, the state of the ON-SAS should be evaluated by pre- and postoperative MRI, in addition to the degree of tumor resection.

Surgical results after primary decompressive craniectomy in poor-grade aneurysmal subarachnoid hemorrhage.

It is well known that patients with poor-grade aneurysmal subarachnoid hemorrhage (SAH) have poor outcomes owing to significant mass effect and brain stem compression. On the other hand, decompressive craniectomy (DC) has shown efficacy in reducing morbidity and mortality in patients with intracranial hypertension. Here, we study the efficacy of DC in poor-grade SAH with attention to surgical outcome. A total of 38 consecutive patients with poor-grade SAH was treated in our hospital between 1 August 2005 and 30 July 2010. Among these 38 patients, we involved 15 patients with DC in the present study. We retrospectively reviewed medical charts and radiological findings. Glasgow Outcome Scale score on discharge showed good response in 1 (6.7 %), moderate disability in 6 (40.0 %), severe disability in 4 (28.1 %), vegetative state in 2 (1.3 %), and death in 2 (13.3 %). In particular, 3 grade IV patients (50.0 %) had a favorable outcome. Recent several experimental studies also indicated that DC significantly improves outcome owing to increased perfusion pressure or reduced intracranial pressure. We suggest that the DC provided the efficacy in reducing mortality in poor-grade SAH patients.

Is decompressive craniectomy a risk factor for ventriculomegaly?

OBJECTIVE: Decompressive craniectomy (DC) is an established therapeutic option following severe traumatic brain injury (TBI). However, several delayed complications of DC have been reported, including ventriculomegaly, which can lead to poor patient outcomes. Nevertheless, ventriculomegaly can occur after TBI even without DC. The aim of the present study was to investigate the influence of DC on ventriculomegaly. MATERIAL AND METHODS: Adult male Sprague-Dawley rats (300-400 g) were subjected to lateral fluid percussion injury using a fluid percussion device. Rats were randomly divided into four groups: sham, craniectomized without trauma (D group), traumatized without DC (FPI group), and craniectomized immediately after trauma (FPI + D group). On day 28 of recovery, ventricular volumes were measured by image analysis. RESULTS: There was no significant difference in ventricular size between the sham group and the D group animals or between the FPI group and the FPI + D group animals. CONCLUSION: These data suggest that DC may not be a risk factor for ventriculomegaly after TBI.

A suitable formula for estimating the volume gained by decompressive craniectomy in malignant hemispheric infarction.

Decompressive craniectomy (DC) improves the survivability and functional outcome in patients with malignant hemispheric infarction (MHI). The decompressive effect of DC depends on the decompressive volume (DV). The value of the formulas for estimating DV has not been reported to date. We have investigated the value of the formulas to estimate DV in patients with MHI. We analyzed the head CTs of six patients who underwent DC for MHI. We examined 1/2ABC, 1/3ABC, π/6ABC, and 2/3Sh formulas to determine the formula that gives the closest estimation of DV compared with computer-assisted volumetric analysis (gold standard). The mean volume values of the gold standard, 1/2ABC, 1/3ABC, π/6ABC, and 2/3Sh formulas were 100.2, 102.4, 68.3, 105.2, and 109.2 mL respectively. Spearman's correlation coefficient was assessed for DV obtained by each of the four different formulas relative to the gold standard. These were as follows: 1/2ABC = 0.8095 (p < 0.05), 1/3ABC = 0.8095 (p < 0.05), π/6ABC = 0.7381 (p < 0.05), and 2/3Sh = 0.4524 (p > 0.05). In conclusion, the 1/2ABC formula is the most useful and the closest estimation of DV in patients with MHI after DC.

Cerebral infarction along the distribution of perforating arteries during aneurysm surgery in a patient with pheochromocytoma--case report.

A 58-year-old woman with refractory hypertension presented with subarachnoid hemorrhage. Digital subtraction angiography and three-dimensional computed tomography (CT) angiography revealed a ruptured left vertebral artery (VA) aneurysm and an unruptured left middle cerebral artery (MCA) aneurysm. The patient successfully underwent neck clipping of the left VA aneurysm. However, CT obtained just after the operation showed an asymptomatic cerebral infarction along the distribution of medial striate arteries of the right anterior cerebral artery in the caudate nucleus. The pathogenesis of the infarction was unknown. Before clipping surgery of the left MCA aneurysm, detailed examinations to find the cause of her refractory hypertension were performed. Laboratory tests revealed plasma serum level of norepinephrine at 15,521 pg/ml (normal range 100-450 pg/ml). Abdominal magnetic resonance imaging revealed a pheochromocytoma in the right adrenal gland. After preoperative management of the pheochromocytoma, the neck of the left MCA aneurysm was successfully clipped. When the patient awakened from anesthesia, she noticed right hemiparesis and motor aphasia. CT showed cerebral infarction along the distribution of lenticulostriate arteries of the left MCA in the putamen. Her symptoms gradually improved, and the pheochromocytoma was removed by laparoscopic surgery. Sustained severe hypertension and depletion of blood volume resulting from excess catecholamine release from the pheochromocytoma may have caused the complications. Hypervolemic fluid infusion and maintenance of normotensive blood pressure during surgery may avoid such ischemic events.