Proposed System for Selection of Surgical Approaches for Craniopharyngiomas Based on the Optic Recess Displacement Pattern.

OBJECTIVE: Craniopharyngiomas remain surgically challenging because of the strong adhesion to vital neurovascular structures. We propose a system for the selection of surgical approaches based on the optic recess (OR) displacement pattern to facilitate surgical planning and obtain optimum visual and endocrinologic outcomes. METHODS: Craniopharyngiomas were divided into 3 types based on the OR displacement pattern: superior, anterior, and involvement types. Selected surgical approaches and patient outcome were retrospectively reviewed according to these classifications. Visual and endocrinologic outcomes were compared among the groups. RESULTS: This study included 26 patients with primary craniopharyngiomas who underwent surgery at our institution, classified into 11 anterior, 11 superior, and 4 involvement types. The extended endoscopic endonasal approach provided excellent exposure inferodorsal aspect of the chiasm for manipulation of the dissection plane in the anterior and superior types with midline location. A unilateral subfrontal approach was required for tumor of the superior type with lateral extension. An interhemispheric translamina terminalis approach could provide safe dissection under direct vision of strong adhesion at the superior aspect of the chiasm in the involvement type. Visual and endocrinologic outcomes were better in the involvement type compared with the superior and anterior types. Visual outcome was significantly correlated with preoperative visual function. CONCLUSIONS: Craniopharyngiomas with the involvement type are indicated for the translamina terminalis approach to achieve the best visual and endocrinologic outcome. Our classification of the OR displacement pattern is useful to select the optimal surgical approach for craniopharyngiomas more accurately and concisely, especially in cases with third ventricular extension.

Characteristics of Preoperative Visual Disturbance and Visual Outcome After Endoscopic Endonasal Transsphenoidal Surgery for Nonfunctioning Pituitary Adenoma in Elderly Patients.

BACKGROUND: Pituitary adenomas in elderly patients may become more common as the population ages. Surgical benefits, especially for visual outcome, after endoscopic endonasal transsphenoidal surgery for pituitary adenomas remain to be elucidated. This retrospective analysis investigated clinical factors affecting visual outcome. METHODS: The study included 35 patients with nonfunctional pituitary adenomas who underwent surgery and were subdivided into elderly (≥70 years old, n = 12) and younger (<70 years old, n = 23) groups for analysis. Clinical characteristics and preoperative and postoperative visual function evaluated using visual impairment score (VIS) were compared between groups. RESULTS: Mean age at diagnosis was 75.1 ± 1.5 years in the elderly group and 55.5 ± 2.0 years in the younger group. VIS was improved in 91% of elderly patients and 80% of younger patients, but postoperative VIS remained higher in elderly patients. Preoperative VIS and incidence of previous cataract surgery were significantly higher in elderly patients than in younger patients. Preoperative and postoperative VISs were significantly correlated with age. Preoperative VIS was significantly higher in patients with previous cataract surgery and correlated with postoperative VIS. CONCLUSIONS: Visual disturbances were improved postoperatively in most patients in both groups at similar rates, but preoperative and postoperative visual disturbances were more severe in elderly patients because the symptoms may have been masked by the presence of lens opacity. Early diagnosis and intervention may be required in elderly patients for better visual outcome.

[A Case of Malignant Transformation of Epidermoid Cyst with Cerebrospinal Fluid Dissemination].

An epidermoid cyst is a benign tumor, accounting for 0.2-1.8% of all brain tumors. Histologically, an epidermoid cyst has a wall consisting of squamous epithelium with keratinization and without skin components. An epidermoid cyst tends to develop in the cerebellopontine angle(CPA), middle cranial fossa, pituitary fossa, spinal cord, and other locations. We describe a rare case of malignant transformation of an epidermoid cyst with cerebrospinal fluid(CSF)dissemination. A 54-year-old man was referred to our hospital with chief complaints of dysesthesia of the right leg, persisting for 6 months, and dizziness and dysarthria for 2 months. Magnetic resonance(MR)imaging showed multiple neoplastic lesions with lobulated margins in the left frontal and temporal lobes, left cerebellum, right brainstem, and right CPA. Diffusion-weighted imaging showed a hyperintense cord-like lesion in the right CPA. Blood examination revealed squamous cell carcinoma(SCC)antigen positivity;however, systemic examination revealed no neoplastic lesion, except for the head lesion. Intracranial lesions were considered unlikely to be metastatic brain tumors. We performed surgical removal of the tumor in the left CPA via a left lateral suboccipital craniotomy. The histopathological diagnosis was SCC. MR imaging for the headache was taken at another hospital 2 years before the surgery, revealed an epidermoid cyst in the right CPA. The current MR imaging showed enhancement in part of the epidermoid cyst in the right CPA, strongly suggesting that the epidermoid cyst in the right CPA had disseminated to the left frontal and temporal lobes, left cerebellum, and right brainstem via the CSF.

Case of primary diffuse leptomeningeal gliomatosis.

Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare and fatal disease characterized by diffuse infiltration of the leptomeninges by neoplastic glial cells without evidence of tumor in the brain parenchyma or spinal cord. We report a 60-year-old man with PDLG. He suffered transient right hemiparesis and generalized seizures. MRI showed diffuse leptomeningeal thickening and enhancement throughout the brain and spinal cord without any intraaxial involvement. Biopsy resulted in a diagnosis of glioblastoma with methylated MGMT promoter and wild-type IDH1. He underwent craniospinal radiotherapy and temozolomide treatment but despite concomitant adjuvant therapy he died 8 months after initial presentation.

A huge intraventricular congenital anaplastic astrocytoma: case report with histopathological and genetic consideration.

Congenital malignant gliomas are rare brain tumors about which few reports have been published. We present the clinical course and genetic alterations in an infant with a congenital malignant glioma detected incidentally by ultrasonography at 36 weeks. The tumor occupied the right temporoparietal region, extended to the posterior fossa, and significantly compressed surrounding structures. The female infant was entirely normal without macrocrania, tense fontanel, or sucking difficulties. The tumor was subtotally resected by two-stage surgery; pathological diagnosis was anaplastic astrocytoma. Immunohistochemical staining was positive for p53 and negative for epidermal growth factor receptor. There was no O(6)-methylguanine-DNA methyltransferase (MGMT) gene promoter methylation, no 1p/19q loss of heterozygosity, and no isocitrate dehydrogenase 1 (IDH1) mutation. She underwent postoperative chemotherapy and is alive and well 12 months after surgery.

Granulomatous amoebic encephalitis caused by Balamuthia mandrillaris.

A 51-year-old immunocompetent Japanese woman presented with a rare case of granulomatous amoebic encephalitis (GAE) caused by Balamuthia mandrillaris. She was brought to our hospital with epilepsy. Magnetic resonance imaging of the brain revealed a homogeneously enhanced solitary mass in the left frontal lobe. Histological diagnosis was made by a biopsy, which suggested lymphomatoid granulomatosis. After that, her neurological condition got worse. New masses were found and had spread across the whole brain. She died 2 months later of cerebral hernia. Autopsy revealed that the patient had GAE caused by Balamuthia mandrillaris. GAE is usually fatal, and is difficult to diagnose except at autopsy. Therefore, awareness of this disease is important, and earlier diagnosis and the development of a better therapeutic strategy will improve clinical outcome.

Oligodendroglial ganglioglioma.

Gangliogliomas are rare tumors of the central nervous system, usually containing neoplastic ganglion cells and astrocytic components. Few cases of ganglioglioma containing only oligodendrocytic tissue have been reported to date. We present a case of a 40-year-old woman with ganglioglioma consisting mostly of oligodendroglial components. Magnetic resonance imaging showed a well-demarcated cystic lesion with slight perifocal edema in the right parietal lobe. The wall of the cyst was not enhanced after administration of Gd-DTPA contrast media. The mass was totally resected. Histological examination showed a mixture of two distinct components: oligodendroglioma and dysplastic ganglions. The first component was diffusely proliferated cells with round nuclei and perinuclear halo; the second showed marked nucleoli and basophilic cytoplasm containing Nissl bodies. Immunohistochemical study of the oligodendroglial component was positive for OLIG 2 and NKX2.2 but negative for synaptophysin. In addition, LOH of 1p/19q was detected by FISH. Although no adjuvant therapy was carried out, follow-up MRI showed no recurrence of the tumor 41 months after the operation.

Intraneural synovial sarcoma originating from the median nerve.

A 33-year-old female presented with a rare synovial sarcoma manifesting as a painful 12 x 15 mm tumor in the median palmar carpus. Preoperative neurological examination detected only radiating spontaneous pain in her right radial palm and decreased right grasping power. Magnetic resonance (MR) imaging confirmed the presence of the tumor. The preoperative diagnosis was schwannoma originating from the right median nerve. Subcapsular removal of the tumor was performed for preservation of the nerve function. However, postoperative histological and immunohistochemical studies suggested synovial sarcoma originating from the median nerve. No systemic metastasis was detected and the residual tumor capsule was totally removed. Local radiation therapy of 40 Gy, 2 Gy per day, was administered. Fourteen months later, local recurrence was detected on MR imaging. Total removal of the recurrent tumor was performed. Synovial sarcoma was finally diagnosed by the identification of SYT-SSX1 fusion gene transcripts using reverse transcriptase-polymerase chain reaction with a frozen tumor tissue sample.

Osteoma of the frontal sinus complicated by intracranial mucocele.

We present a rare case of intracranial mucocele associated with frontal sinus osteoma in a patient suffering from generalized convulsion. The intracranial mucocele occurs as a complication of obstruction of sinus drainage caused by osteoma, but it is often diagnosed preoperatively as an intracranial or intracerebral cyst because of the rarity of these combined lesions in neurosurgical practice. However, once the mucocele extends intracranially, several other complications, including infections and/or a convulsion, can occur, indicating the necessity for surgical treatment. Moreover, the differentiation of the mucocele from the intracranial endodermal cyst predominantly depends upon its continuity with the intracranial osteoma portion or the sinus. Thus, knowledge of this rare lesion is important for accurate diagnosis and clinical management.

Basilar artery aneurysm treated with coil embolization via persistent primitive trigeminal artery.

A saccular aneurysm of the right basilar-superior cerebellar artery associated with a persistent primitive trigeminal artery (PPTA) was successfully treated by endovascular occlusion with Guglielmi detachable coils. Since both vertebral arteries and the basilar artery proximal to the junction with the PPTA were hypoplastic, a microcatheter was advanced via the PPTA. To our knowledge, this is the first case report describing the treatment of the aneurysm through the PPTA.