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  • Author or Editor: Chang Kyu Park x
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Ji Hoon Phi, Seung-Ki Kim, Sung-Hye Park, Seok Ho Hong, Kyu-Chang Wang and Byung-Kyu Cho

Object

Immature teratomas of the central nervous system (CNS) are rare neoplasms. Although adjuvant therapy is generally recommended after resection, the exact role of each therapeutic modality is not yet established. The purpose of this study was to analyze the clinicopathological correlation and the role of resection to define the optimal treatment modalities for immature teratomas of the CNS.

Methods

Between 1987 and 2002, eight patients underwent radical surgery for a lesion diagnosed as a CNS immature teratoma at the authors' institution. The clinical courses of these patients and the pathological features of their tumors were retrospectively reviewed.

Gross-total resection (GTR) was achieved in six patients at the initial operation. The mean follow-up period was 75 months. Two patients received postoperative adjuvant therapies and two patients did not, against medical advice. None of the four patients experienced recurrence after long-term follow up. Another four patients, all of whom underwent GTR of the tumor, did not receive adjuvant therapy as part of a prospective treatment scheme. One of them exhibited early recurrence and metastasis. The tumor had pathological features denoting a high-grade (Norris Grade III) lesion and neurocytomatous differentiation.

Conclusions

Aggressive resection seems to be of utmost importance in the treatment of immature teratomas of the CNS. Adjuvant chemotherapy and radiotherapy can be deferred if GTR is achieved in low-grade, immature teratomas, but adjuvant therapies may be warranted for high-grade ones.

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Kyung Sun Song, Sung-Hye Park, Byung-Kyu Cho, Kyu-Chang Wang, Ji Hoon Phi and Seung-Ki Kim

Meningiomas are rare in children. Chordoid meningioma is a very rare variant, as only 16 cases in children have been reported. The authors report the first case of a chordoid meningioma in the third ventricle.

A 12-year-old boy presented with headache, abnormal behaviors, and ataxia. Brain MR imaging revealed a 2-cm, well-enhanced mass in the third ventricle and hydrocephalus. Positron emission tomography with [18F]fluorodeoxyglucose showed that the mass was hypermetabolic. Gross-total removal of the mass was performed using a left frontal transcortical and transventricular approach. The mass originated from the left caudate head and was connected to the choroid plexus. A chordoid meningioma was diagnosed on the basis of the histological characteristics of the tumor, which was composed of cords and nests of eosinophilic vacuolated cells with an abundant myxoid matrix, similar to the features of a chordoma. A typical focal meningiomatous pattern was observed. The tumor cells were immunoreactive for vimentin and epithelial membrane antigen. The patient's headache and gait disturbance improved after the tumor was removed. The tumor showed no signs of recurrence during 12 months of follow-up.

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Byung Chan Lim, Jong Hee Chae, Seung-Ki Kim, Sung-Hye Park, Kyu-Chang Wang, Ji Yeoun Lee and Ji Hoon Phi

Brainstem glioma is a highly devastating disease, and any mass-like lesion in the brainstem can raise suspicion of this diagnosis. However, other inflammatory, demyelinating, or degenerative diseases can mimic brainstem glioma in clinical presentation and imaging features. Therefore, diagnosis based solely on imaging is often insufficient for brainstem lesions and may lead to incorrect diagnosis and treatment.

This case report is the first description of central nervous system aquaporin-4 (AQP4) autoimmunity confined mainly to the brainstem. It demonstrates the wide spectrum of neuroinflammatory diseases in children and highlights the utility of surgical biopsy for suspicious brainstem lesions with atypical imaging features for glioma.

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Do-Hun Lee, Eun Young Kim, Sepill Park, Ji Hoon Phi, Seung-Ki Kim, Byung-Kyu Cho, Jinho Lim and Kyu-Chang Wang

Object

The authors previously reported that human embryonic stem cells (hESCs) injected into the amniotic cavity of chick embryos immediately after neural tube incision in a surgically induced spinal open neural tube defect (ONTD) model promote the reclosure capacity of neural tubes. To simulate more closely the clinical situation of human ONTDs, in which a ssubstantial time period elapses before the prenatal diagnosis of spinal ONTDs, the authors investigated whether this reclosure capacity remains enhanced by the intraamniotic injection of hESCs at 24 hours after ONTD induction.

Methods

One hundred twenty-two chick embryos with ONTDs were randomly assigned to two groups: the control group (59 embryos) and the hESC-injection group (hESC group, 63 embryos). After the neural tubes of both groups had been opened, the hESC group underwent direct intraamniotic injection with hESCs marked with an enhanced green fluorescent protein at 24 hours after ONTD induction. The lengths of the remnant ONTDs were measured and the presence of hESCs was determined at 4, 6, and 8 days after ONTD induction. No difference in survival rates was observed between the two groups. The mean length of the ONTDs, adjusted for body length at the time of death and initial lesion length, was significantly shorter in the hESC group than in the control group (p < 0.001). No hESCs were found within reclosed neural tubes; rather, they covered the defect area during the reclosure process.

Conclusions

The authors demonstrate that hESCs injected into the amniotic cavity at 24 hours after ONTD induction enhance reclosure ability in chick embryos.

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Eun Jung Koh, Ji Hoon Phi, Sung-Hye Park, In-One Kim, Jung-Eun Cheon, Kyu-Chang Wang, Byung-Kyu Cho and Seung-Ki Kim

This 14-year-old boy presented with left hemiparesis, gait disturbance, and multiple cranial nerve palsies. Magnetic resonance imaging revealed a multicystic mass with hemorrhagic fluid–fluid levels in the right midbrain, suggesting the presence of a cavernous malformation. Diffusion tensor imaging showed the mass to be close to the right corticospinal tract and ipsilateral medial lemniscus. Subtotal removal of the mass was performed via a right subtemporal approach. The histopathological diagnosis was of a mixed germ cell tumor (GCT) comprising mature teratoma and germinoma cells with syncytiotrophoblastic giant cells. The patient underwent postoperative chemotherapy and radiotherapy, and no tumor progression was found during 1 year of follow-up.

Intracranial GCTs arise mainly in the pineal and the suprasellar area. Germ cell tumors in the brainstem are rare, with only 12 reported cases. Among these, 4 were in the midbrain and histologically were pure germinomas. To the authors' knowledge, this is the first reported case of a mixed GCT in the midbrain combining mature teratoma and germinoma cells.

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Seung-Yeob Yang, Kyu-Chang Wang, Byung-Kyu Cho, Young-Yim Kim, Su-Young Lim, Sung-Hye Park, Il Han Kim and Seung-Ki Kim

✓Radiation-induced glioblastoma multiforme (GBM) is a rare complication of radiotherapy. The authors report such a case occurring 10 years after treatment of cerebellar medulloblastoma. The patient was a 15-year-old boy who had undergone a gross-total removal of a medulloblastoma and received radiation therapy at the age of 5 years. He had experienced no tumor recurrences for 10 years until a new enhancing mass was found at the original site of the medulloblastoma. Following its resection the new lesion was found to be a GBM and there was no evidence of a medulloblastoma. The second tumor developed at the same site as the previous one after a sufficient latent period and fulfilled the criteria for a radiation-induced neoplasm. The original tumor cells expressed synaptophysin without p53 overexpression, a characteristic feature of medulloblastomas. In contrast, cells from the later tumor expressed glial fibrillary acidic protein and p53 but not synaptophysin. A sequence analysis of the p53 gene showed deletion at codon 233 and a C to G transition at codon 278 in the GBM but no mutation in the medulloblastoma. A GBM specimen revealed no amplification of the epidermal growth factor receptor compared with a normal control specimen. In conclusion, the clinical features of a radiation-induced GBM are similar to that of the primary GBM, whereas its genetic alterations render it a secondary GBM. These findings indicate that radiation-induced GBM should be considered a distinct clinical entity.

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Ji Hoon Phi, Jin Hyun Kim, Kyoung Mi Eun, Kyu-Chang Wang, Ki Ho Park, Seung Ah Choi, Young Yim Kim, Sung-Hye Park, Byung-Kyu Cho and Seung-Ki Kim

Object

Supratentorial primitive neuroectodermal tumor (PNET) and medulloblastoma are highly malignant embryonal brain tumors. They share morphological similarities, but differ in their differentiation patterns and global gene expression. The authors compared the expression of specific genes involved in neuroglial differentiation in supratentorial PNETs and medulloblastomas to define the distinct characters of these tumors.

Methods

The mRNA expression of 8 genes (SOX2, NOTCH1, ID1, ASCL-1, NEUROD1, NEUROG1, NEUROG2, and NRG1) was evaluated in 25 embryonal tumors (12 supratentorial PNETs and 13 medulloblastomas) by quantitative real-time polymerase chain reaction. The expression levels of the transcripts of these genes were compared between the tumor groups. Activation of the JAK/STAT3 pathway was assessed by immunoblotting. Relative expression levels of STAT3 and phosphorylated STAT3 proteins were compared.

Results

Supratentorial PNETs expressed significantly higher levels of SOX2, NOTCH1, ID1, and ASCL-1 transcripts, whereas the transcription of proneural basic helix-loop-helix factors, NEUROD1, NEUROG1 (significantly), and NEUROG2 (not significantly) was upregulated in medulloblastomas. The proportion of phosphorylated STAT3α relative to STAT3α was significantly greater in supratentorial PNETs than in medulloblastomas, indicating activation of the JAK/STAT3 pathway in supratentorial PNETs.

Conclusions

These results indicate that supratentorial PNET predominantly has glial features and medulloblastoma largely follows a neuronal differentiation pattern. These divergent differentiation patterns may be related to the location and origin of each tumor.

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Jin Wook Kim, Sung-Hye Park, Sung-Sup Park, Kyu-Chang Wang, Byung-Kyu Cho, So Yeon Kim, Eun Kyung Ra, Chae-Yong Kim and Seung-Ki Kim

✓Fetus-in-fetu is a very rare condition in which one fetus is contained within another. About 100 cases have been reported, and in most of these the fetus was located in the retroperitoneum. The authors describe an extremely rare case of an intracranial fetus-in-fetu in an extraaxial location. This is the eighth intracranial fetus-in-fetu to be reported, the first intracranial extraaxial case, and involves the oldest documented patient with this condition.

Histopathological analysis of the mass revealed a degenerated amnionic membranelike tissue, well-differentiated extremities (including fingerlike structures), skin, matured lungs, well-formed intestines, cerebellar and cerebral tissue, and a notochord with ganglion cells. DNA analysis using short tandem repeat polymorphisms confirmed that the fetus-in-fetu mass and the host infant had heterozygous alleles and were of identical sex and genotype.

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Hyun-Seung Kang, Youn-Joo Moon, Young-Yim Kim, Woong-Yang Park, Ae Kyung Park, Kyu-Chang Wang, Jeong Eun Kim, Ji Hoon Phi, Ji Yeoun Lee and Seung-Ki Kim

Object

Moyamoya disease (MMD) is a cerebrovascular occlusive disease affecting bilateral internal carotid termini. Smooth-muscle cells are one of the major cell types involved in this disease process. The characteristics of circulating smooth-muscle progenitor cells (SPCs) in MMD are poorly understood. The authors purified SPCs from the peripheral blood of patients with MMD and sought to identify differentially expressed genes (DEGs) in SPCs from these patients.

Methods

The authors cultured and isolated SPCs from the peripheral blood of patients with MMD (n = 25) and healthy control volunteers (n = 22). After confirmation of the cellular phenotype, RNA was extracted from the cells and DEGs were identified using a commercially available gene chip. Real-time quantitative reverse transcription polymerase chain reaction was performed to confirm the putative pathogenetic DEGs.

Results

The SPC-type outgrowth cells in patients with MMD invariably showed a hill-and-valley appearance under microscopic examination, and demonstrated high α–smooth muscle actin, myosin heavy chain, and calponin expression (96.5% ± 2.1%, 42.8% ± 18.6%, and 87.1% ± 8.2%, respectively), and minimal CD31 expression (less than 1%) on fluorescence-activated cell sorter analysis. The SPCs in the MMD group tended to make more irregularly arranged and thickened tubules on the tube formation assay. In the SPCs from patients with MMD, 286 genes (124 upregulated and 162 downregulated) were differentially expressed; they were related to cell adhesion, cell migration, immune response, and vascular development.

Conclusions

With adequate culture conditions, SPCs could be established from the peripheral blood of patients with MMD. These cells showed specific DEGs compared with healthy control volunteers. This study provides a novel experimental cell model for further research of MMD.

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Kyung Sun Song, Ji Hoon Phi, Byung-Kyu Cho, Kyu-Chang Wang, Ji Yeoun Lee, Dong Gyu Kim, Il Han Kim, Hyo Seop Ahn, Sung-Hye Park and Seung-Ki Kim

Object

Glioblastoma is the most common primary malignant brain tumor; however, glioblastoma in children is less common than in adults, and little is known about its clinical outcome in children. The authors evaluated the long-term outcome of glioblastoma in children.

Methods

Twenty-seven children were confirmed to have harbored a glioblastoma between 1985 and 2007. The clinical features and treatment outcomes were reviewed retrospectively. All patients underwent resection; complete resection was performed in 12 patients (44%), subtotal resection in 12 patients (44%), and biopsy in 3 patients (11%). Twenty-four patients (89%) had radiation therapy, and 14 (52%) patients received chemotherapy plus radiation therapy. Among the latter, 5 patients had radiation therapy concurrent with temozolomide chemotherapy. Four patients with small-size recurrent glioblastoma received stereotactic radiosurgery.

Results

The median overall survival (OS) was 43 months, and the median progression-free survival was 12 months. The OS rate was 67% at 1 year, 52% at 2 years, and 40% at 5 years. The median OS was significantly associated with tumor location (52 months for superficially located tumors vs 7 months for deeply located tumors; p = 0.017) and extent of removal (106 months for completely resected tumors vs 11 months for incompletely resected tumors; p < 0.0001).

Conclusions

The prognosis of glioblastoma is better in children than in adults. Radical resection followed by concurrent chemoradiation therapy may be the initial treatment of choice.