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June Ho Lee, Chae-Yong Kim, Dong Gyu Kim and Hee-Won Jung

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Jung Ho Han, Dong Gyu Kim, Hyun-Tai Chung, Chul-Kee Park, Sun Ha Paek, Jeong Eun Kim, Hee-Won Jung and Dae Hee Han

Object

In this paper the authors analyzed the clinical and neuroimaging outcomes of patients with cerebral arteriovenous malformations (AVMs) after Gamma Knife surgery (GKS), focusing on the analysis of the radiation injury rate depending on the AVM volume.

Methods

Between 1997 and 2004, 277 consecutive patients with cerebral AVMs were treated with GKS. Of these patients, 218 were followed up for ≥ 2 years. The mean age was 31 ± 15 years, the median AVM volume was 3.4 cm3 (range 0.17–35.2 cm3), the median marginal dose was 18.0 Gy (range 10.0–25.0 Gy), and the mean follow-up duration was 44 ± 20 months. The authors reduced the prescription dose by various amounts, depending on the AVM volume and location as prescribed in the classic guideline to avoid irreversible radiation injuries.

Results

The angiographic obliteration rate was 66.4% overall, and it was 81.7, 53.1, and 12.5% for small, medium, and large AVMs, respectively. The overall annual bleeding rate was 1.9%. The annual bleeding rate was 0.44 and 4.64% for small and large AVMs, respectively. Approximately 20% of the patients showed severe postradiosurgery imaging (PRI) changes. The rate of PRI change was 11.4, 33.3, and 9.5% for small, medium, and large AVM volume groups, respectively, and a permanent radiation injury developed in 5.1% of patients.

Conclusions

By using the reduced dose from what is usually prescribed, the authors were able to obtain outcomes in small AVMs that were comparable to the outcomes described in previous reports. However, medium AVMs appear to still be at risk for adverse radiation effects. Last, in large AVMs, the authors were able to attain a tolerable rate of radiation injury; however, the clinical outcomes were quite disappointing following administration of a reduced dose of GKS for large AVMs.

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Yang Kwon, Jae Sung Ahn, Sang Ryong Jeon, Jeong Hoon Kim, Chang Jin Kim, Jung Kyo Lee, Byung Duk Kwun, Do Hee Lee and Sun Young Kim

Object. The authors evaluated whether gamma knife radiosurgery (GKS) could be a causative factor in intratumoral bleeding in meningiomas.

Methods. Gamma knife radiosurgery was used in the treatment of 173 meningiomas during a 10-year period. Four patients suffered post-GKS intratumoral hemorrhage. The course in these patients was reviewed.

Four of 173 patients suffered an intratumoral hemorrhage during a follow-up period of 1 to 8 years. The risk of intratumoral bleeding after GKS for meningioma was 2.3%. Intracystic hemorrhage occurred in two patients 1 and 5 years, respectively, after radiosurgery. In the other two cases intratumoral bleeding occurred 2 and 8 years, respectively, after radiosurgery. Histological examination in three cases found no specific findings related to the postradiosurgical changes.

Conclusions. Because the reported risk of spontaneous intratumoral bleeding in meningiomas is 1.3 to 2.7%, the incidence in this series was not unduly high. Radiosurgery itself could not be shown to be a significant factor in the development of the intratumoral bleeding.

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Myoung Soo Kim, Chang Wan Oh, Dae Hee Han, O-Ki Kwon, Hee-Won Jung and Moon Hee Han

✓ The most common clinical presentations of dural arteriovenous fistulas (DAVFs) are bruit, headache, increased intracranial pressure, and intracranial hemorrhage. In particular locations, such as the cavernous sinus or middle cranial fossa, cranial nerve involvement due to dural arterial steal or venous occlusion may develop. A case in which a DAVF is associated with hearing loss, however, has not previously been reported. The authors report a case in which an intraosseous DAVF and associated hearing loss probably resulted from cochlear nerve or vascular compression caused by the draining vein or nidus of the DAVF.

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Jung-Hee Lee, Ki-Tack Kim, Kyung-Soo Suk, Sang-Hun Lee, Bi-O Jeong, Hyun-Seok Oh, Chul-Hee Lee and Myung-Seo Kim

Intraspinal cystic lesions with different pathogeneses have been reported to cause neurological deficits; however, no one has focused on the intraspinal extradural cysts that develop after osteoporotic compression fracture. The reported case features a 66-year-old woman presenting with progressive neurological deficit, back pain, and no history of additional trauma after undergoing conservative treatment for an osteoporotic fracture of L-1. The authors present serial radiographs and MR images demonstrating an epidural cyst successfully treated via a single posterior approach. This appears to be the first such case reported in the literature.

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Dong Gyu Kim, Jong Soo Kim, Je G. Chi, Sung Hye Park, Hee-Won Jung, Kil Soo Choi and Dae Hee Han

✓ The authors analyzed 13 central neurocytomas diagnosed at Seoul National University Hospital between January 1982 and December 1993 to clarify the proliferative potential and biological behavior of these tumors. The tumor was confined to the lateral and third ventricles in 12 cases and in one case extended from the posterior thalamus to the body and trigone area of the lateral ventricle. In all 13 cases, typical clinical and radiological findings were observed, and histological diagnosis was performed via craniotomy. The diagnosis was made using light microscopic examination, immunohistochemical staining for neuronal markers, and electron microscopic findings of neuronal differentiation. One patient died due to tumor progression with recurrence 26 months after subtotal removal plus radiation therapy. Another patient had a recurrence 18 months after total tumor removal. The remaining 11 patients are free of recurrent tumor after a follow-up period that ranged from 14 to 109 months (median 50 months). To predict the proliferative potential, immunoreactivity to proliferating cell nuclear antigen (PCNA), silver colloid staining for nucleolar organizing regions (AgNORs), and DNA flow cytometry were performed in 10 of the 13 cases. The proportion of PCNA-positive cells was less than 1% in all cases and the AgNORs score ranged from 1.11 to 2.0 (mean 1.67). The DNA flow cytometry revealed diploidy in all cases and the calculated proliferation index ranged from 5.1% to 9.6% (mean 7.8%). The one case of tumor recurrence, in which the authors performed the study of proliferative potential, and another case that demonstrated mild nuclear pleomorphism also showed low percentages of PCNA-positive cells, low AgNORs scores, and diploidy in DNA flow cytometry.

It is suggested that most central neurocytomas follow a benign clinical course with low proliferative potential assessed by PCNA, AgNORs, and DNA flow cytometry; however, recurrence is possible within a relatively short time period.

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Dong Gyu Kim, Chi Heon Kim, Hyun-Tai Chung, Sun Ha Paek, Sang Soon Jeong, Dae Hee Han and Hee-Won Jung

Object. The authors analyzed tumor control rates and complications in patients with superficially located meningiomas after gamma knife surgery (GKS).

Methods. Between 1998 and 2003, GKS was performed in 23 patients with 26 lesions in whom follow-up imaging for 1 year or more was available. The male/female ratio was 1:22. The mean age was 59 years. The median tumor volume was 4.7 cm3, and the mean margin dose was 16 Gy at the 50% isodose line. Peritumoral edema was revealed on magnetic resonance (MR) imaging in four patients before GKS. Magnetic resonance imaging and clinical examinations were performed every 6 months after GKS. The mean follow-up duration was 32 months.

The tumor shrank in eight cases, was stable in 17, and enlarged in one; thus 25 (95%) of 26 tumors were controlled. A peritumoral high signal on T2-weighted MR images was found in eight lesions and preexisting edema was aggravated in three lesions after GKS. Ten of these 11 patients complained of severe headache, and three patients experienced neurological deficits at the same time after a mean latency of 3 months; however, high signal was not demonstrated on imaging before 6 months on average. Steroid agents, when required, gave relief to all patients. The complication rate was 43% (10 of 23 cases). High signal disappeared in nine patients and decreased in the remaining two. High signal was associated with a high integral dose and a large tumor volume. Tumor shrinkage at the last follow-up examination was more prominent in the patients with symptomatic high signal (p = 0.03).

Conclusions. There was a good tumor control rate with a high complication rate. Longer follow up of more patients is needed. Adjusting the dose—volume relationship should be considered to reduce complications.

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Dae Hee Han, Dong Gyu Kim, Je G. Chi, Sung Hye Park, Hee-Won Jung and Young Gyu Kim

✓ The authors present the clinical, radiological, pathological features, and autopsy findings of a patient with malignant triton tumor of the acoustic nerve, which probably arose from a pre-existing acoustic schwannoma. The term “malignant triton tumor” is applied to malignant schwannomas with rhabdomyoblastic differentiation. A cerebellopontine angle tumor with spinal drop metastasis occurred in this patient 10 months after near-total removal of the original tumor.

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Dae Kyu Lee, Dong Gyu Kim, Gheeyoung Choe, Je G. Chi and Hee-Won Jung

✓ The authors present a case of chordoid meningioma in a 55-year-old woman who manifested headache and personality change. Magnetic resonance imaging of the brain and cerebral angiography demonstrated a mass in the right frontal lobe that resembled a typical convexity meningioma. However, the pathological diagnosis was chordoid meningioma, a rare subtype of this tumor that usually occurs in adolescence and is known to be associated with Castleman syndrome. A meningothelial meningiomatous pattern suggestive of a meningothelial origin was focally present, and cytokeratin-positive squamoid cells were noted in the tumor. The lesion lacked dense infiltration of lymphocytes and plasma cells. Polyclonal gammopathy was the only sign of Castleman syndrome and hypochromic microcytic anemia was absent in this case. Polyclonal gammopathy resolved completely 6 months after total removal of the mass.

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So-Hyang Im, Chang Wan Oh, O-Ki Kwon, Jung Eun Kim and Dae Hee Han

Object. In moyamoya disease (MMD), ischemic events are usually precipitated by activities associated with hyperventilation or physical strain. The authors report on four patients with a rare combination of Graves disease—associated thyrotoxicosis and MMD, whose cerebrovascular ischemic events occurred while in a thyrotoxic state. The clinical correlation between MMD and Graves' thyrotoxicosis, and outcome after surgical intervention are described.

Methods. Four young women, ages 22 to 25 years, presented with profound cerebrovascular ischemic accidents. They had clinical and radiological features consistent with the diagnosis of MMD and were in the active thyrotoxic state of Graves disease. To prevent a future ischemic event, patients underwent superficial temporal artery—middle cerebral artery anastomosis combined with encephalomyosynangiosis or encephaloduroarteriosynangiosis after normalization of their hormonal conditions. All patients have been neurologically stable since revascularization procedures and lead a normal daily life.

In patients with MMD, cerebrovascular ischemic events may be precipitated by thyrotoxicosis. One possible pathomechanism of cerebrovascular ischemic aggravation in the thyrotoxic state may be a hemodynamic compromise induced by an excessive increase in the cerebral metabolism and oxygen demand over the compensation of the cerebral blood flow deficit through collateral supply in MMD.

Conclusions. Surgical revascularization after optimal control of thyrotoxicosis is thought to be an appropriate treatment in patients with MMD concurrent with Graves disease for the prevention of further ischemic events, especially in those with impaired cerebral perfusion and cerebral ischemic symptoms.