Yasuo Sasagawa

Gamma knife radiosurgery-induced cavernous hemangioma: case report.

OBJECTIVEWe report a rare case of gamma knife radiation–induced cavernous hemangioma. CLINICAL PRESENTATIONA 35-year-old man underwent resection of a left vestibular schwannoma and gamma knife radiosurgery (maximal dose, 24 Gy; marginal dose, 12 Gy) for the residual schwannoma. Follow-up magnetic resonance images showed no tumor progression. Ten years later, he developed right hemihyperesthesia and mild hemiparesis. Magnetic resonance imaging revealed a size reduction at the resected tumor site and a newly developed lesion in the adjacent pons. No connection was observed between the new mass and the previous tumor. The T2-weighted image showed the new mass as heterogeneous and “popcorn-like” with a mixed signal intensity core and a hypointense hemosiderin rim. Two years after its appearance, the new lesion appeared hypointense on the T2-weighted image, with a hyperintense core on the T1-weighted image. These findings were compatible with cavernous hemangioma. INTERVENTIONWe diagnosed the new lesion as a radiation-induced cavernous hemangioma. Ten days after admission, symptoms improved without surgical intervention. CONCLUSIONGamma knife radiosurgery induced this rare case of cavernous hemangioma in an area that received a low dose of irradiation and was distant from the primary tumor. Because patients undergoing radiosurgery face the possibility, although small, that such neoplasms may occur, they should be followed for many years.

Undifferentiated sarcoma of the cavernous sinus after gamma knife radiosurgery for pituitary adenoma

We report a rare case of gamma knife radiation-induced undifferentiated sarcoma in the cavernous sinus. A 24-year-old woman underwent resection of a growth hormone-secreting pituitary adenoma and gamma knife radiosurgery (maximal dose 24 Gray (Gy); marginal dose 16 Gy) for residual adenoma in the right cavernous sinus. Follow-up MRI showed the disappearance of the adenoma. Fifteen years later, she developed right oculomotor nerve palsy. MRI revealed a new tumor in the right cavernous sinus. Partial removal of the tumor was performed via a transsphenoidal approach. Histological diagnosis revealed undifferentiated sarcoma. The patient received three cycles of chemotherapy containing doxorubicin and ifosfamide, then carbon ion radiotherapy (65 GyE in 26 fractions). Subsequent MRI showed tumor regression for five months. To our knowledge, this is the first report of undifferentiated sarcoma following gamma knife radiosurgery for pituitary adenoma. As patients undergoing radiosurgery face the possibility of such neoplasms developing, long-term follow-up is required.

Pituitary apoplexy following gonadotropin-releasing hormone agonist administration with gonadotropin-secreting pituitary adenoma

Gonadotropin-releasing hormone (GnRH) agonists are widely used in hormone therapy for prostate cancer. We report a patient with pituitary apoplexy following this therapy as a rare complication and review the related literature. A 62-year-old man presented with elevated prostate specific antigen. Transrectal ultrasound guided biopsy of the prostate gland revealed adenocarcinoma. Whole-body (18)F-fluorodeoxyglucose (FDG) positron emission tomography/CT scan showed FDG-uptake in the pituitary region. MRI also demonstrated a pituitary tumor, diagnosed as an incidental non-functioning adenoma. The patient received his first dose of GnRH agonist (leuprolide 11.25mg) against prostate cancer. He complained of a severe headache 10 minutes after leuprolide administration and suffered from right third nerve palsy in the next 48 hours. MRI demonstrated a high intensity area on T1-weighted images, diagnosed as pituitary apoplexy. The patient underwent transsphenoidal surgery. Pathology revealed predominantly necrotic tissue and a gonadotropin secreting pituitary adenoma. Overall, 15 patients, including ours, have been reported with pituitary apoplexy after GnRH agonists with pathologic gonadotropin secreting adenoma. Fourteen of 15 patients were male. Pituitary apoplexy developed within 4 hours after administration of the agents in 8/15 patients. The combined data suggest that GnRH agonists have the potential to precipitate pituitary apoplexy in men with gonadotropin secreting adenoma. Therefore, prior to GnRH agonist therapy for prostate cancer, a known pituitary adenoma should be treated. Otherwise, the patients should be cautiously observed for any symptomatic change following drug administration.

Secondary malignant giant cell tumor of the clivus: Case report

Giant cell tumors (GCTs) of bone are rare primary bone neolasms, representing approximately 5% of skeletal tumors. The piphyses of the long bones, particularly the distal femur, proxial tibia, and distal radius, account for 75–90% of GCTs [1]. GCTs arely occur in the skull, and comprise less than 1% of all reported hese tumors of bone GCTs and also preferentially involve the spheoid and temporal bones [2]. Malignancy arising in a GCT can be xpected in less than 2% of GCTs. Malignant transformation of GCT n the skull is exceptionally rare [3]. We report a case of GCT of the livus, which underwent malignant transformation 10 years after nitial treatment. We discuss the diagnosis and therapeutic considrations of GCT of the sphenoid, including the clivus, based on a eview of the pertinent literature.

Multiple intraosseous inflammatory myofibroblastic tumors presenting with an aggressive clinical course: case report.

BACKGROUND AND IMPORTANCE:The authors report a rare case of multiple intraosseous inflammatory myofibroblastic tumors presenting with an aggressive clinical course. CLINICAL PRESENTATION:A 60-year-old man presented with a 3-month history of headache and 2 weeks of jaw pain. Magnetic resonance imaging showed a homogeneously enhancing mass in the right parietal bone with subcutaneous and intracranial invasion. Bone scintigraphy revealed 4 intraosseous lesions involving the cranium, mandible, ischium, and calcaneum. After admission, the patient showed left hemiparesis and seizures caused by rapid intracranial tumor extension. The cranial and mandible tumors were resected. Histopathological examinations of both specimens revealed myofibroblastic spindle cell proliferation with inflammatory cell infiltration, and a diagnosis of inflammatory myofibroblastic tumor was made. Two days postoperatively, the patient presented with a high fever and disturbance of consciousness with swelling of the subcutaneous tissues of the head and mandibular lesions. Magnetic resonance imaging revealed a massive intracranial extension of the tumor. Corticosteroid therapy induced remarkable shrinkage of all lesions, and relief from symptoms was obtained. Radiotherapy was then performed for residual tumors. CONCLUSION:Multiple intraosseous inflammatory myofibroblastic tumors of the bone are very uncommon and may mimic malignant tumors. It is important to recognize that this entity can occur in the cranium and as multiple bony lesions. The recommended treatment is complete surgical resection with adjuvant steroid treatment. Considering the aggressive nature of this entity, additional chemo- and/or radiotherapy may be warranted.

Significant Improvement in Chronic Persistent Headaches Caused by Small Rathke Cleft Cysts After Transsphenoidal Surgery

PURPOSE Rathke cleft cysts (RCC) usually are asymptomatic and can be observed via the use of conservative methods. Some patients with RCCs, however, have severe headaches even if they are small enough to be confined to the sella, and these small RCCs seldom have been discussed. This study presents an investigation into clinical characteristics of small RCCs associated with severe headaches, demonstrating efficacy and safety of endoscopic transsphenoidal surgery (ETSS) to relieve headaches. METHODS In this study, 13 patients with small RCCs (maximum diameter <10 mm) who presented with headaches and were treated by ETSS at our institute from 2009 to 2014 were recruited. These RCCs were treated Headache Impact Test-6 (HIT-6) score was calculated both pre- and postoperatively to evaluate headache severity. RESULTS All patients complained of severe headaches, which disturbed their daily life. Most headaches were nonpulsating and localized in the frontal area. Characteristically, 6 patients (46%) experienced severe headaches with sudden onset that continued chronically. HIT-6 score was 64 on average, meaning headaches affected daily life severely. After surgical decompression of the cyst, headache in all of the patients improved dramatically and HIT-6 score decreased significantly to 37, suggesting that headaches were diminished. No newly developed deficiencies of the anterior pituitary lobe function were detected. Postoperative occurrence of diabetes insipidus was found in 2 patients, both of which were transient. No recurring cysts were found. CONCLUSIONS Severe headaches can develop from small RCCs. In the present study, ETSS was performed on such patients effectively and safely to relieve their headaches.

Narrow band imaging-guided endoscopic biopsy for intraventricular and paraventricular brain tumors: clinical experience with 14 cases

BackgroundNarrow-band imaging (NBI) has been confirmed as a useful endoscopic technique to distinguish neoplasm from normal tissue, on the basis of the enhanced neovascularity of tumor tissue. NBI-guided tissue biopsy for laryngopharyngeal and digestive lesions is a novel methodology, but the feasibility for central nervous system tumors remains unclear. The aim of our study was to evaluate the feasibility of NBI-guided biopsy for intraventricular and paraventricular tumor.MethodsFourteen patients with intraventricular or paraventricular tumors underwent neuroendoscopic biopsy using a videoscope with NBI. Ventricular walls and tumors were observed using conventional imaging, followed by NBI. Colors of ventricle walls and tumors visualized using NBI were compared to those visualized under conventional imaging. Extracted specimens were stained using CD31 antibody and numbers of microvessels in each specimen were counted for analyzing vascular density.ResultsNormal ventricle walls were a similar color under conventional imaging and NBI. Tumor surfaces appeared to be cyan in color under NBI. Vessels on the tumor were more clearly visualized with NBI than with conventional imaging. NBI was able to identify tumor surfaces that were not perceptible on conventional imaging. All specimens in the lesion surfaces from cyan-colored areas under NBI contained tumor cells. Specimens extracted from regions that appeared cyan in color under NBI (51.0 vessels/mm2) had significantly greater vascular density than regions that appeared a normal color (17.4 vessels/mm2; p = 0.039).ConclusionNBI-guided biopsy of intraventricular and paraventricular tumors is feasible for visualizing tumor surface-enhancing neovascularities. NBI would contribute to accurate histological diagnosis while minimizing injury to surrounding structures.

Troubleshooting distraction osteogenesis for craniosynostosis.

Purpose: We previously reported that distraction osteogenesis is less invasive and gives greater skull advancement compared to conventional cranioplasty [Akai et al: Pediatr Neurosurg 2006;42:288-292]. In this study, we analyzed the distraction osteogenesis process and tried to identify and solve various technical problems. Patients and Results: We operated on 22 patients, 5 syndromic and 17 nonsyndromic. During treatment, we encountered several problems: (i) dural laceration during craniotomy (2 cases), solution: repair by suturing with fascia; (ii) skull fracture at sphenofrontal or coronal sutures (2 cases), solution: completed distraction; (iii) device dislocation during distraction (1 case), solution: the device was secured to the skull with stainless wire; (iv) wound issues around shaft and device (3 cases), solution: treated with antibiotic ointment. Discussion: (1) Extra caution is needed to avoid dural damage at frontal bottom burr holes. (2) Completion of craniotomy should be confirmed by checking if the bone flap moves in sync with brain pulsation. The craniotomy line should be placed forward of coronal sutures. (3) For patients younger than 2 years, employ clamp-type devices. (4) Shafts should be cut short enough to prevent their tips from exerting pressure or puncturing the scalp from beneath. Conclusions: The distraction osteogenesis technique has complications that may not occur in conventional one-stage cranioplasty. In this study, neither age at operation nor distraction distance were significant causes of complications during distraction osteogenesis.

Oncocytic meningioma presenting with intratumoral hemorrhage

Oncocytic meningiomas have been recently reported as a rare variant of meningiomas. Immunohistochemical analysis shows that neoplastic cells are positive for antimitochondrial antibodies. We report our first patient with oncocytic meningioma, presenting with intratumoral bleeding. A 72-year-old woman suffered from a disturbance of consciousness. A CT scan showed a tumor with intratumoral hemorrhage. An emergency craniotomy was performed and the tumor and hematoma were removed. Examination of the tumor revealed meningothelial cells with oncocytic change. We discuss the clinicopathological considerations of this uncommon variant and review the pertinent literature.

Radiation-induced gliomas: a report of four cases and analysis of molecular biomarkers

Radiation-induced glioma (RIG) is a rare secondary glioma. The tumors morphologically resemble their sporadically arising counterparts. Recently, the WHO classification of tumors of the central nervous system was revised to incorporate molecular biomarkers together with classic histological features. The status of molecular biomarkers in RIG, however, remains unclear. The objective of this study was to investigate if commonly accepted glioma-specific biomarkers are relevant in RIGs. Among 269 gliomas diagnosed as WHO grade 2, 3 and 4 in our institution, four were diagnosed as RIGs. Immunohistochemical (IHC) staining for isocitrate dehydrogenase 1 (IDH1), p53, alpha thalassemia/mental retardation syndrome X-linked (ATRX), and H3K27M, and direct DNA sequencing of IDH1/2, telomerase reverse transcriptase (TERT) promoter, Histone H3.3 (H3F3A) and B-Raf (BRAF) genes was performed. All tumor specimens were IDH1-, p53- and H3K27M-negative. The nuclei of tumor cells in all cases exhibited positive staining for ATRX. In direct DNA sequencing analysis, no IDH1, IDH2, TERT promoter, H3F3A or BRAF mutations were found in any of the cases. Our findings suggest that these characteristic glioma-associated molecular mutations may be rare events in RIGs. More RIGs need to be tested for analysis of molecular biomarkers to clarify the clinical and histopathological spectra of this tumor.