• Journal of Korean Neurosurgical Society
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• v.38 no.3
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• pp.238-241
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• 2005

The main causes of pineal apoplexy are hemorrhage associated with pineal region tumors, vascular malformations, and pineal cysts. Cavernous malformations rarely occur in the pineal region, with only fifteen cases reported previously. Hemorrhage associated with cavernous malformation causes apoplectic event in the pineal region. We report two surgically treated cases of pineal hemorrhage associated with cavernous malformation and discuss the consideration in management of the pineal apoplexy.

• Journal of Korean Neurosurgical Society
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• v.40 no.5
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• pp.342-345
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• 2006

Objective : Gamma Knife Surgery[GKS] for the management of pineal region tumors is challengeable strategy as direct access to this area is not easy. The experiences of pineal region tumor patients treated with GKS were analyzed to evaluate the effectiveness. Methods : Seven patients with tumors in the pineal region were treated with GKS between September 1998 and May 2005. The histological diagnosis were pineal parenchymal tumor [2 patients], low-grade astrocytoma [2 patients], immature teratoma [1 patient], and choriocarninoma [1 patient]. One patient was diagnosed as metastatic brain tumor based on histological diagnosis for primary site and brain imaging study. The median marginal dose was 15Gy [range; $11{\sim}20$] at the 50% isodose line. The median target volume was $2.5cm^3$ [range; $0.8{\sim}12.5$]. The median clinical follow up period was 29 months [range; $13{\sim}93$] and the median radiological follow up period was 18 months [range; $6{\sim}73$]. Results : Tumor volume measured in follow-up images showed reduction in six patients, disappearance in one. No adverse effect due to GKS was found during the follow-up period. The performance status was preserved in all patients except one who died due to progression of primary cancer in spite of controlled metastatic brain lesion. Conclusion : Gamma Knife Surgery can be applied to pineal region tumors irrespective of their histology whenever surgery is not indicated.

• Journal of Korean Neurosurgical Society
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• v.30 no.5
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• pp.575-580
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• 2001

Purpose : In general, pineal region tumors are managed by using microsurgical approach or stereoctactic biopsy. However, in selected cases endoscopic approach to pineal lesions might prove to be as effective as microsurgery and less invasive. We report an alternative surgical strategy for managing certain patients with pineal neoplasms that allows treatment of the symptomatic hydrocephalus as well as tumor biopsy under direct vision in the same sitting. Materials and Methods : Twenty-two patients with pineal region tumors with associated hydrocephalus were treated in one session by endoscopic third ventriculostomy and endoscopic tumor biopsy at our institution from October 1996 to January 2000. All patients were retrospectively evaluated. Results : There was no operative mortality. There was one cause of significant bleeding during biopsy, but was controlled endoscopically, and the patient recovered completely without neurologic deficit resulting from intra-operative bleeding. The symptoms related to increased intracranial pressure(ICP) have resolved in all patients, and the need for a shunt is completely eliminated. Histological diagnosis was achieved in 21 of the 22 patients by this procedure. A biopsy was not obtained in one patient. Although this pineal region tumor was seen endoscopically, this could not be biopsied because of technical difficulties in working around an enlarged massa intermedia. The lesions included fourteen germinomas, three mixed germ cell tumors, and one each of the followings: pineocytoma, pineoblastoma, pineocytoma/pineoblastoma(intermediate type), meningioma, and low grade glioma. Five of the 22 patients subsequently underwent formal microsurgical tumor removal. Additional chemotherapy or radiotherapy could then be initiated according to the histological diagnosis. Conclusion : We consider that endoscopy affords a minimally invasive way of reaching three objectives by one-step surgery in the management of pineal region tumors with associated hydrocephalus : 1) cerebrospinal fluid(CSF) sample for analysis of tumour markers and cytology, 2) treatment of hydrocephalus by third ventriculostomy, and 3) several biopsy specimens can be obtained identifying tumors which will require further open surgery or adjuvant radiation and/or chemotherapy. However, complications and morbidities should be emphasized so as to be avoided with further technical experience.

• Journal of Korean Neurosurgical Society
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• v.58 no.5
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• pp.479-482
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• 2015

We report a case of a 31-year-old woman with glioblastoma multiforme (GBM) in the pineal region with associated leptomeningeal dissemination and lumbar metastasis. The patient presented with severe headache and vomiting. Magnetic resonance imaging (MRI) of the brain showed a heterogeneously enhanced tumor in the pineal region with obstructive hydrocephalus. After an urgent ventricular-peritoneal shunt, she was treated by subtotal resection and chemotherapy concomitant with radiotherapy. Two months after surgery, MRI showed no changes in the residual tumor but leptomeningeal dissemination surrounding the brainstem. One month later, she exhibited severe lumbago and bilateral leg pain. Thoracico-lumbar MRI showed drop like metastasis in the lumbar region. Finally she died five months after the initial diagnosis. Neurosurgeons should pay attention to GBM in the pineal region, not only as an important differential diagnosis among the pineal tumors, but due to the aggressive features of leptomeningeal dissemination and spinal metastasis.

• Radiation Oncology Journal
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• v.20 no.2
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• pp.100-107
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• 2002

Purpose : The aim of this retrospective study was to assess the treatment results of 30 patients with pineal region tumors who were underwent radiation therapy under the diagnosis by either CT or MRI. There was no histological verification. We analyzed the prognostic factors that have a significant effect on the overall survival (OS) and disease free survival (DFS) rates. Materials and Methods : A total 30 patients with pineal region tumors were treated between March 1983 and August 1995. After a trial radiation therapy of $20\~30\;Gy/2\~3$ weeks, the patients were evaluated for their clinical response and radiological response by either CT or MRI and the final treatment direction was then decided. According to their response to the trial radiation therapy and the involved site, radiation treatment was given in various fields i.e., local, ventricle, whole brain and craniospinal field. The radiation dose ranged from 40.8 to 59.4 Gy (Median 50.4 Gy). The median follow up was 36.5 months $(4\~172\;months)$. Results : An improvement or stability in the clinical symptoms was observed in 28 patients $(93.3\%)$ after the trial RT. Nineteen patients $(63.3\%)$ showed a partial or complete response by CT or MRI. The two-year and five-year survival rates of the patients were $66.7\%$ and $55.1\%$, respectively. No significant difference in the survival rates according to the degree of the radiological response was abserved after the trial RT. The results of univariate analysis showed that age, the primary site, the performance status $(KPS\geq70)$, the degree of response after completing RT and the RT field were significant prognostic factors affecting the survival and disease free survival rates (p<0.05). Conclusion : The clinical and histological characteristics of pineal region tumors are quite complex and diverse. Therefore, it is difficult to predict the histological diagnosis and the possibility of radiocurability only with the initial response to RT. We think that the development of less invasive histological diagnostic techniques and tailored treatment to the histological type of each tumor are needed.

• Journal of Korean Neurosurgical Society
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• v.30 no.10
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• pp.1245-1249
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• 2001

Germinomas of the central nervous system are rare embryonal tumors(accounting for less than 1% of intracranial neoplasms) that may be located in the pineal region, in the floor of the third ventricle, or in the suprasellar area. We report a case of germinoma developed in periventricular deep white matter without pineal region tumors or suprasellar masses. The 19-year-old male patient presented with slowly progressing headache, dizziness, photophobia, and dysarthria. Initial brain MRI revealed a irregular and dense enhancement from lateral ventricles to 4th ventricle. The stereotactic biopsy of tumor and histologic examination revealed the germinoma. Craniospinal axis radiation therapy was performed. After radiation therapy patient was improved and no neurologic sequelae was seen at discharge. Periventricular germinomas without pineal or suprasellar lesion are very rare. The radiation therapy, as in our case, is beneficial as with other intracranial germinomas. Stereotactic biopsy of periventricular germinoma provides precise pathologic diagnosis and thus allows more specific management.

• Journal of Korean Neurosurgical Society
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• v.42 no.1
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• pp.56-58
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• 2007

A 64-year-old man was admitted to our hospital in semicomatous consciousness. Brain computed tomography scans demonstrated $2.6{\times}2.5\;cm$ sized hyperdense mass in the pineal region with multiple punctate calcifications and hydrocephalus. Brain magnetic resonance imaging demonstrated a pineal mass which was heterogeneously enhanced with gadolinium. After external ventricular drainage, the patient regained consciousness. The mass was totally removed via occipital transtentorial approach. No consequent ventricular shunt was needed and the patient recovered without any neurological deficit. Final pathologic report of the tumor was ependymoma.

• Journal of Korean Neurosurgical Society
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• v.56 no.6
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• pp.504-508
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• 2014

A 59-year-old male patient had 5-month history of gait disturbance and memory impairment. His initial brain computed tomography scan showed $3.5{\times}2.8cm$ sized mass with high density in the pineal region. The tumor was hypointense on T2 weighted magnetic resonance images and hyperintense on T1 weighted magnetic resonance images with heterogenous enhancement of central portion. The tumor was totally removed via the occipital transtentorial approach. Black mass was observed in the operation field, and after surgery, histopathological examination confirmed the diagnosis of malignant melanoma. Whole spine magnetic resonance images and whole body 18-fluoro-deoxyglucose positron emission tomography could not demonstrate the primary site of this melanoma. Scrupulous physical examination of his skin and mucosa was done and dark pigmented lesion on his left leg was found, but additional studies including magnetic resonance images and skin biopsy showed negative finding. As a result, final diagnosis of primary pineal malignant melanoma was made. He underwent treatment with the whole brain radiotherapy and extended local boost irradiation without chemotherapy. His preoperative symptoms were disappeared, and no other specific neurological deficits were founded. His follow-up image studies showed no recurrence or distant metastasis until 26 weeks after surgery. Primary pineal malignant melanomas are extremely rare intracranial tumors, and only 17 cases have been reported since 1899. The most recent case report showed favorable outcome by subtotal tumor resection followed by whole brain and extended local irradiation without chemotherapy. Our case is another result to prove that total tumor resection with radiotherapy can be the current optimal treatment for primary malignant melanoma in the pineal region.

• Brain Tumor Research and Treatment
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• v.6 no.2
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• pp.73-77
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• 2018

Germinoma is the most common type of intracranial germ cell tumors (GCTs). Pineal gland and suprasellar region are the most frequent sites of central nervous system (CNS) involvement. Intracranial masses caused by Langerhans cell histiocytosis (LCH) mimics features of CNS GCTs. LCH frequently involve spine and is the most common cause of vertebra plana in children. A 15-year-old boy presented with progressing symptoms of polydipsia, polyuria, general headache, nausea and severe back pain. Brain MRI showed brain tumor with simultaneous involvement of suprasellar region and pineal gland. An excisional biopsy of suprasellar mass was done. The pathologic assessment confirmed the diagnosis of germinoma. Patient's treatment continued accordingly. A spine MRI, done due to persistent backache, showed a vertebra plana. We reevaluated the primary diagnosis suspecting LCH. Germinoma of CNS was confirmed and a biopsy of vertebral lesion resulted in hemangioma. Thus we report a case of CNS germinoma with co-occurrence of vertebra plana. We emphasized the importance of histopathologic diagnosis of pineal/suprasellar masses and primary investigation of other CNS regions including spine for possible metastasis or comorbidities.

• Radiation Oncology Journal
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• v.9 no.2
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• pp.177-184
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• 1991

From December 1984 to February 1990, 16 patients with tumors of pineal and suprasellar location were treated with radiation therapy. Tissue diagnoses were obtained before radiation therapy in 5 patients and 11 were irradiated without histologic confirmation. Initial treatments for these patients were craniospinal plus boost primary irradiation(six), whole brain plus boost primary irradiation(nine), primary tumor site irradiation(one). The 5 year actuarial survival rate is $71\%$. Three cases with elevated beta-human chorionic gonadotropin(HCG) responded favorably to radiation, but pineal tumors with elevated alpha-fetoprotein(AFP) did not respond well. Spinal metastasis developed in 2 cases(2/15) with elevated AFP : one received prophylactic spinal irradiation, another did not. Our studies suggest that more aggressive treatment would be necessary in patient with elevated AFP and in this patient, radiation therapy may be initiated without pathologic confirmation. From the result of our study, routine use of prophylactic spinal irrdiation for all patients with pineal region tumor is not indicated and use of prophylactic spinal irradiation is considered for the patients with positive craniospinal fluid cytology, meningeal seeding, disease extension along the ventricular wall and biopsy proven germinoma.