Hisato Ishii

Transcranial transsphenoidal approach for tuberculum sellae meningiomas.

Summary Objective. A series of 21 patients with tuberculum sellae meningioma who received surgical treatment is reported. Patients and Methods. All 9 females and 12 males (mean age 49 years) presented visual disturbances of varying degrees in either one or both eyes. Eighteen of the tumours were less than 3 cm in size, and 3 were larger. Tumour resection of uniform surgical technique was performed in all cases. Following a bicoronal scalp incision, bifrontal craniotomy combined with removal of the orbital rim bilaterally was performed. The frontal dura was opened bilaterally, and the most anterior portion of the superior sagittal sinus was transected. Bifrontal retraction and arachnoid dissection along the proximal olfactory tracts brought the tumour into view. Additional dissection of the interhemispheric fissure extended the operative field to the anterior communicating artery. The anterior skull base was drilled out to resect the basal part of the tumour. In all cases, the optic canal and sphenoid sinus, and additionally in some cases the ethmoid sinus were opened. The tumour uniformly extended inferomedially to the optic nerve, and direct visualization of this portion of the tumour was possible with our approach. The opened paranasal sinuses were reconstructed with adipose tissue harvested from the patients abdomen and the pericranial flap. Results. In all patients, total or almost total resection of the tumour was accomplished. Postoperatively, visual function was improved in 11 patients, was unchanged in 8, and worsened in 2. There were no operative deaths. Cerebrospinal fluid leakage was occurred in two patients but could be conservatively managed. In a mean 3-year follow-up, tumour recurrence was observed in only one patient who presented a malignant histology. Conclusions. We are confident that our surgical approach has great clinical value in surgical resection of tuberculum sellae meningioma. The good accessibility to a tumour extending inferomedially to the optic nerve should, in particular, be stressed.

Surgical Experience of 120 Patients with Lumbosacral Lipomas

Summary.The surgical experience of 120 patients with lumbosacral lipomas is described. 47 of 120 patients were preoperatively found to be neurologically intact, the remaining 73 patients presented with various neurological signs including reflex changes, sensory disturbances, muscle weakness and sphincter problems. Neuro-imagings allowed a classification of lumbosacral lipomas into five types: (1) dorsal type; (2) caudal type; (3) combined type; (4) filar type; and (5) lipomyelomeningocele. Although all 120 patients underwent untethering of the spinal cord, the nerve roots passing through the lipoma itself and the neural tissues protruding externally to the spinal canal, respectively, tended to prevent satisfactory surgical removal of the lipoma in combined type lipomas and lipomyelomeningoceles. During 8.96 years of a mean postoperative follow-up period, there was no significant deterioration in most of the patients and some patients even improved in function. However, two patients with combined type lipomas developed neurological deterioration just after surgery, and five (two dorsal, two caudal and one combined type lipomas) did in the fashion of a late-onset. There are two different patient groups of lumbosacral lipomas; one group (caudal and filar type lipomas, and most of dorsal type lipomas) in whom the surgical anatomy is simple and satisfactory untethering surgery could be done without risk, and another (combined type lipomas and lipomyelomeningocele) in whom surgery would be accompanied with some risk and sometimes complete untethering could not be achieved because of the complicated anatomy of the lesion. Surgical difficulty of the latter group can be correlated with the increased frequency of neurological deterioration occurring just after the operation, but not of delayed one. Concerning prophylactic surgery for asymptomatic patients, the former group of patients are obviously good candidates, but the latter group is not.

Phase IIa clinical study of [(18)F]fluciclovine: efficacy and safety of a new PET tracer for brain tumors.

Objective[18F]Fluciclovine (anti-[18F]FACBC) has demonstrated diagnostic efficacy for cancers of the brain where [18F]fludeoxyglucose has limitations. We conducted a phase IIa study of anti-[18F]FACBC to assess its accumulation pattern and safety in patients with malignant glioma.MethodsFive patients with glioma scheduled for brain tumor resection received anti-[18F]FACBC. Brain positron emission tomography (PET) was performed following intravenous administration of anti-[18F]FACBC, and subsequently, preoperative gadolinium contrast-enhanced T1-weighted (CE-T1W) magnetic resonance imaging (MRI) was performed for surgery. Specimens for histopathological evaluation were collected during surgery, and their location was precisely determined on CE-T1W MRI and anti-[18F]FACBC PET/CT images. In addition, tumor extent defined on the MRI and PET/CT images was compared. To determine time–activity curves for anti-[18F]FACBC uptake in brain tumor and normal tissues, regions of interest were set in the brain tumor, contralateral normal tissue and the cerebellum, and their standardized uptake values (SUV) were calculated. The safety of anti-[18F]FACBC was assessed based on subjective symptoms and objective findings, electrocardiograms, vital signs, laboratory results, and the incidence of adverse events.ResultsAnti-[18F]FACBC accumulated in the malignant gliomas of all patients. CE-T1W MRI detected gliomas in all patients, but anti-[18F]FACBC PET/CT generally delineated wider regions of tumor extent than CE-T1W MRI. Two of the histopathologically confirmed tumors were located in regions that were defined using anti-[18F]FACBC PET/CT, but not using CE-T1W MRI. Two patients experienced three mild adverse events: one complained of a dull headache and later a mild headache, and the other showed general malaise. These symptoms resolved spontaneously without treatment. Only the mild headache could not be ruled out from having a causal relationship with anti-[18F]FACBC. Favorable T/N ratios regarding anti-[18F]FACBC uptake between tumors and normal control tissues were demonstrated in this trial.ConclusionsIt is suggested that anti-[18F]FACBC PET/CT has the ability to delineate glioma spread that is undetectable using CE-T1W MRI. Anti-[18F]FACBC is safe in patients with malignant glioma.This study was registered in the Japan Pharmaceutical Information Center Clinical Trials Information, which is one of the World Health Organization registries (registration number: JapicCTI-111387).

Effectiveness of interferon-beta and temozolomide combination therapy against temozolomide-refractory recurrent anaplastic astrocytoma

BackgroundMalignant gliomas recur even after extensive surgery and chemo-radiotherapy. Although a relatively novel chemotherapeutic agent, temozolomide (TMZ), has demonstrated promising activity against recurrent glioma, the effects last only a few months and drug resistance develops thereafter in most cases. Induction of O6-methylguanine-DNA methyltransferase (MGMT) in tumors is considered to be responsible for resistance to TMZ. Interferon-beta has been reported to suppress MGMT in an experimental glioma model. Here we report a patient with TMZ-refractory anaplastic astrocytoma (AA) who was treated successfully with a combination of interferon-beta and TMZ.Case presentationA patient with recurrent AA after radiation-chemotherapy and stereotactic radiotherapy was treated with TMZ. After 6 cycles, the tumor became refractory to TMZ, and the patient was treated with interferon-beta at 3 × 106 international units/body, followed by 5 consecutive days of 200 mg/m2 TMZ in cycles of 28 days. After the second cycle the tumor decreased in size by 50% (PR). The tumor showed further shrinkage after 8 months and the patients KPS improved from 70% to 100%. The immunohistochemical study of the initial tumor specimen confirmed positive MGMT protein expression.ConclusionIt is considered that interferon-beta pre-administration increased the TMZ sensitivity of the glioma, which had been refractory to TMZ monotherapy.

Early experience with poly L-lactic acid bioabsorbable fixation system for paediatric craniosynostosis surgery. Report of 3 cases.

Summary¶ Objective. The authors describe early experience in the use of bioabsorbable fixation devices for cranial reconstruction of paediatric craniosynostosis patients. Methods. Three patients, individually respectively presenting sagittal synostosis, metopic synostosis, and clover leaf skull deformity, underwent cranial reconstruction using poly L-lactic acid (PLLA) plates and screws. The patients ranged in age from 2 to 10 months at the time of surgery. The postoperative clinical follow-up ranged from 16 to 18 months. All patients showed satisfactory wound healing without signs of infection or local inflammation. The plates provided satisfactory fixation and were not palpable through the skin after 16 to 18 postoperative months. Conclusion. Our experience demonstrated the efficacy of PLLA bioabsorbable plates and screws for cranial reconstruction in cases of infant craniosynostosis. Prospective studies and longer follow-up of a larger number of patients is desirable for confirmation of these findings.

Giant Cell Tumor of the Temporal Bone with Direct Invasion into the Middle Ear and Skull Base: A Case Report

Giant cell tumor (GCT) is classified as a benign bone tumor, and it is frequently identified at the epiphysis of long bones and relatively rare in the temporal bone. For orthopedists expert at recognizing bone and soft tissue tumors, the diagnosis of GCT is relatively easy; however, since head and neck surgeons experience few cases of GCT, it may be difficult to diagnose when it occurs in the temporal bone. A 32-year-old man complained of left hearing loss, aural fullness, and tinnitus. Examination of the ear revealed a bulging tumor. Audiologic examination demonstrated conductive hearing loss of the left ear. Computer tomograph of the temporal bone showed a soft-tissue-density specification indicating bone destruction at the left temporal bone. The tumor invaded the skull base. Imaging examinations using magnetic resonance imaging revealed a nonhomogenous isosignal intensity area on T1 at the left temporal bone. After intravenous gadolinium, the mass showed unequal enhancement. This patient subsequently underwent surgery to remove the lesion using transmastoid and middle fossa approach. Pathological examinations from specimens of the tumor revealed characteristic of GCT. No clinical or radiological evidence of tumor recurrence was detected for 4 years.

Posterior Subthalamic Area Deep Brain Stimulation for Fragile X–Associated Tremor/Ataxia Syndrome

To the Editor: Fragile X–associated tremor/ataxia syndrome (FXTAS) is an adultonset hereditary neurodegenerative disease underpinned by expansion of the premutation in the CGG trinucleotide repeats in the fragile mental retardation gene 1 (FMR1) (1). The prevalence of the FMR1 premutation is approximately 1/250 in women and 1/800 in men. Therefore, while the prevalence of FXTAS in men is estimated to be approximately 1 in 3000 to 6000 in general population, the prevalence in women is thought to be much lower (1,2). The syndrome clinically presents with postural and action tremors, cerebellar ataxia, cognitive deficits, parkinsonism, dysautonomia, and neuropathy (3), although exact manifestations can be variable among sufferers. Although there is not enough evidence of treatment for FXTAS, tremor in FXTAS can be treated with medications such as primidone, beta blockers, benzodiazepines, and memantine (4). Hall et al. reported that 50% of patients had mild to moderate improvement on primidone, that 37.5% had moderate improvement on beta blockers, and that 25% had moderate improvement on benzodiazepines (4). In patients with medication refractory FXTAS-associated tremor, ventral intermediate (Vim) nucleus of the thalamus deep brain stimulation (DBS) has been applied (5–10). Out of eight cases of Vim DBS, two have resulted in the worsening of balance and ataxia symptoms (5,10). Recently, posterior subthalamic area (PSA) stimulation has been suggested as an alternative option for control of severe tremor beyond essential tremor (ET) (11). Although there is no consensus to date regarding the best DBS target for patients with FXTAS, there has been a concern that the traditional Vim target would provide insufficient benefit, especially in cases of proximal tremor. We conducted a retrospective chart review of a single case of PSA DBS for a patient with the FXTAS mutation. This study was approved by the Institutional Review Board of Juntendo University School of Medicine.

Subthalamic Deep Brain Stimulation for a Parkinson's Disease Patient With Duplication of SNCA

To the Editor: Deep brain stimulation (DBS) of the bilateral subthalamic nucleus (STN) is a widely accepted treatment for cardinal motor symptoms in advanced Parkinson’s disease (PD). Although the effect of STNDBS on cognitive function or psychiatric symptoms is still debated (1), many previous reported studies excluded patients with apparent dementia or psychiatric problems before the surgery; therefore, we should carefully screen for psychiatric or cognitive problems before introducing this therapy. Multiplication of SNCA is a known causative factor of autosomal dominant familial PD, which has a very similar phenotype to sporadic PD. A recent study indicated that patients with duplication of SNCA frequently showed psychiatric problems (2). However, there is only one report about STN-DBS of a patient with SNCA multiplication followed a short time after the surgery (3). Here, we report on a patient genetically proven to have SNCA duplication who received STN-DBS to improve motor fluctuation and to reduce the use of antiparkinsonian drugs for a longer period (four years) after the surgery.

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.

Troubleshooting in hospitalized Parkinson's disease patients with a history of deep brain stimulation of the subthalamic nucleus

Deep brain stimulation is a treatment option for patients with Parkinsons disease who have motor complications, such as wearing off and dyskinesia. However, in some cases, the benefits of deep brain stimulation seem to diminish over time.