• 대한한방내과학회지
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• 제44권5호
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• pp.979-986
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• 2023

Objective: The purpose of this study is to report the case of intracranial germ cell tumor-induced central diabetes insipidus (CDI) in a child treated with Korean herbal medicine. Methods: A nine-year-old female patient diagnosed with intracranial germ cell tumor-induced CDI suffering from polyuria, polydipsia, and headache was hospitalized. Nocturia frequency and 24-hour urine volume were assessed. Results: The patient was treated with Korean medicine, including Nokyong-hwan. As a result of the 6-day inpatient treatment, nocturia frequency was decreased 3-4 times to 0-1 time, and 24-hour urine volume was decreased. Conclusion: Korean medicine, including Nokyong-hwan, may be considered an optional treatment for releasing the symptoms of intracranial germ cell tumor-induced CDI. Further studies are needed to confirm this finding.

• 대한세포병리학회지
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• 제7권2호
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• pp.177-184
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• 1996

Intraoperative cytologic examination of intracranial tumors using crush preparation provides useful information in operative decision making. The diminutive nature of many biopsy specimens, particularly those obtained by stereotactic neurosurgical procedures emphasizes the importance of combining the cytologic smear method with conventional frozen section interpretation. The great advantage of the cytologic smear method resides in its suitability for the study of minute fragments of tissue, allowing retention of the majority of the specimen for optimal processing. We present the cytologic features of 3 cases of intracranial germ cell tumors(2 germinomas and 1 endodermal sinus tumor), using crush preparation during intraoperative diagnosis and compare them with histologic findings. The cytologic features of the germ cell tumors were similar to those of the respective gonadal counterparts. The cytologic differential diagnosis of both types of germ cell tumors is described.

• Journal of Korean Neurosurgical Society
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• 제57권5호
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• pp.315-322
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• 2015

Intracranial germ cell tumors (ICGCT) occur in 2-11% of children with brain tumors between 0-19 years of age. For treatment of germinoma, relatively low radiation doses with or without chemotherapy show excellent 10 year survival rate of 80-100%. Past studies showed that neoadjuvant chemotherapy combined with focal radiotherapy resulted in unacceptably high rates of periventricular tumor recurrence. The use of generous radiation volume which covers the whole ventricular space with later boost treatment to primary site is considered as standard treatment of intracranial germinomas. For non-germinomatous germ cell tumors (NGGCT), 10-year overall survival rate is still much inferior than that of intracranial germinoma despite intensive chemotherapy and high-dose radiotherapy. Craniospinal radiotherapy combined with cisplatin-based chemotherapy provides the best treatment outcome for NGGCT; 60-70% of overall survival rate. There is a debate on the surgical role whether surgery can contribute to improved treatment outcome of NGGCT when added to combined chemoradiotherapy. Because higher dose of radiotherapy is required for treatment of NGGCT than for germinoma, it is tested whether whole ventricular irradiation can replace craniospinal irradiation in intermediate risk group of NGGCT to minimize radiation-related late toxicity in the recent studies. To minimize the treatment-related neural deficit and late sequelae while maintaining long-term survival rate of ICGCT patients, optimized administration of chemotherapy and radiotherapy should be selected. Use of technically upgraded radiotherapy modalities such as intensity-modulated radiotherapy or proton beam therapy is expected to bring an improved neurocognitive outcome with longitudinal assessment of the patients.

• Journal of Korean Neurosurgical Society
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• 제30권12호
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• pp.1381-1387
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• 2001

Objective : Endodermal sinus tumor or yolk sac tumor is an uncommon malignant germ-cell neoplasm. This tumor was originally described as a germ cell tumor of the ovary or the testis. Intracranial endodermal sinus tumor is extremely rare and usually develop in the pineal or suprasellar regions. The authors evaluated the effect of adjuvant therapy(chemotherapy combined with radiotherapy) and radical removal of intracranial endodermal sinus tumors. Material and Methods : Between 1996 and 2001, four patients of intracranial endodermal sinus tumor were diagnosed with tumor marker(AFP) and biopsy. Three patients were treated with surgical removal and chemotherapy with cisplatin($20mg/m^2$), etoposide($100mg/m^2$) and bleomycin($15mg/m^2$) as well as external beam radiation therapy. We compared the management problems for these tumors. Result : In all three patients the tumor size and the level of tumor marker decresed during initial adjuvant therapy. However, Tumors showed regrowth with elevated AFP of serum and CSF possibly related to delayed chemotherapeutic treatment or inadequate administration of chemotherapeutic drugs due to severe bone marrow suppression. An additional chemotherapy and external radiation therapy were given, but tumors could not be controlled with leptomeningeal seeding. Conclusion : Radiotherapy is considered to be less effective. The combination chemotherapy with PVB(cisplatin, vinblastine, bleomycine) or PE(cisplatin, etoposide) is considered to be value in prolongation of the survival rate. But the role of chemotherapy in this tumor has not yet been clarified due to bone marrow suppression and drug resistance. Further study with large series of this tumor is necessary to establish the optimal management.

• Radiation Oncology Journal
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• 제18권2호
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• pp.92-100
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• 2000

목적 :본 연구의 목적은 뇌 생식 세포종 환자들의 방사선치료 시 가장 적절한 조사 영역을 알아보고자 시행하였다. 대상 및 방법 : 1993년부터 1998년까지 뇌 생식세포종으로 진단되거나 또는 추정되어 감마 나이프를 시행 받은 환자 19 명을 대상으로 분석하였다. 송과선 9예, 안상(suprasellar) 1예, 그 외 2군데 이상 다발성 병소가 9예였다. 조직이 확인이 된 예는 7예이었고 배아종(germ ceil tumor)이 5명, 내배엽동종(endodermal sinus tumor)이 2명이었다. 종양의 부피는 2.4 cm$^{3}$부터 74 cm$^{3}$ 까지 이었다. 감마나이프 방사선 치료는 50% 등선량 곡선을 중심으로 10 Gy부터 20 Gy에 걸쳐 조사되었다. 추적 기간은 10개월에서부터 54개월까지였다. 결과 : 총 19명 중 14명(74%)에서 재발을 하였다. 완전 관해와 부분 관해는 각각 2명(11%), 10명(53%)이었다. 무반응은 7명(36%)이었다. 원발 병소가 있었던 자리에서 재발한 경우가 2예, 치료 조사영역을 벗어났으나 원발 병소와 연결되어서 그 주위로 재발한 경우가 6예 이었다. 원발 병소와 떨어져서 뇌실 재발이 된 경우 3예, 척수 전이가 된 경우가 4예 이었다. 종양의 부피가 20 cm$^{3}$ 이하인 경우는 8예이었으며 이중 2예는 치료 조사영역 내에서만 재발한 경우, 4예는 원발 병소와 연이어져서 치료 부위 주위로 재발한 경우, 1예는 척수 전이된 경우이었다. 종양의 부피가 20 cm$^{3}$ 보다 큰 경우는 6예 이었으며 그 중 원발 병소와 연이어져서 치료 부위 주위에 재발한 경우 1예, 원발 병소와 떨어져서 뇌실 전이가 된 경우가 2예, 척수 전이를 일으킨 경우가 3예였다. 재발을 하지 않은 5예는 종양의 부피가 20 cm$^{3}$ 이하인 경우이고 모두 단일 병소이며 종양기표가 모두 정상이었다. 척수 전이는 4예(21%)에서 발생하였으며 모두 뇌실 침범이 있는 경우에 발생하였다. 총 9명의 다발성 병소 중 국소 재발만을 한 경우는 3경우이었고 나머지는 모두 치료 조사영역을 벗어나 원발 병소와 떨어져서 재발하였다. 결론 : 감마 나이프 치료가 뇌 생식세포종에 대한 치료로서는 부적절한 치료이며 이것은 감마 나이프의 특성인 작은 치료 용적과 조사 선량의 부적절함에서 기인하는 것으로 판단된다. 뇌 생식 세포종에서 병소 부위 만을 치료하는 경은 종양의 부피와 다발성 병소의 뇌실 침범 유무가 치료 성공의 열쇠이다. 20 cm$^{3}$ 이하, 단일 병소, 뇌실 침범이 혀는 경우, 정상적인 종양지표, 등이 가장 이상적인 적응증이 될 수 있다. 다발성 병소에서 뇌실 침범이나 뇌실 병소가 있을 경우는 예방적 뇌 척수 조사를 고려해야 할 것으로 생각된다. 병소의 크기가 cm$^{3}$ 보다 클 경우 다발성 병소인 경우, 종양지표의 증가가 있는 경우에는 확정적인 제안을 하기는 어렵지만 전 뇌실 조사 또는 부분방사선 조사가 시도될 수 있을 것으로 생각되며 이 경우가 선행 화학 요법과 함께 치료할 수 있는 대상이며, 앞으로 이 부분에 대한 연구가 계속 이루어질 것으로 생각된다.

• Journal of Korean Neurosurgical Society
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• 제61권3호
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• pp.333-342
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• 2018

Intracranial germ cell tumors (iGCTs) are a heterogeneous group of tumors with peculiar characteristics clearly distinguished from other brain tumors of neuroepithelial origin. Diverse histology, similarity to gonadal GCT, predilection to one sex, and geographic difference in incidence all present enigmas and fascinating challenges. The treatment of iGCT has advanced for germinoma to date; thus, clinical attention has shifted from survival to long-term quality of life. However, for non-germinomatous GCT, current protocols provide only modest improvement and more innovative therapies are needed. Recently, next-generation sequencing studies have revealed the genomic landscape of iGCT. Novel mutations in the KIT-RAS-MAPK and AKT-MTOR pathways were identified. More importantly, methylation profiling revealed a new method to assess the pathogenesis of iGCT. Molecular research will unleash new knowledge on the origin of iGCT and solve the many mysteries that have lingered on this peculiar neoplasm for a long time.

• Brain Tumor Research and Treatment
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• 제6권2호
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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.

• Clinical and Experimental Pediatrics
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• 제54권3호
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• pp.128-132
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• 2011

Purpose: Cisplatin-based chemotherapy has been commonly used for the treatment of intracranial germ cell tumors (IC-GCTs). However, this treatment exhibits some adverse effects such as renal problems and hearing difficulty. Carboplatin-based chemotherapy was administered to pediatric patients with IC-GCTs from August 2004 at the Samsung Medical Center. In this study, we assessed the responses and adverse effects of carboplatin-based chemotherapy in pediatric IC-GCTs patients according to the risk group, and compared the results with those of the previous cisplatin-based chemotherapy. Methods: We examined 35 patients (27 men and 8 women) diagnosed with IC-GCTs between August 2004 and April 2008 and received risk-adapted carboplatin-based chemotherapy at the Samsung Medical Center. Patients were divided into either low-risk (LR) or high-risk (HR) groups and a retrospective analysis was performed using information from the medical records. Results: Although hematological complications were common, hearing difficulties or grade 3 or 4 creatinine level elevation were not observed in patients who underwent carboplatin-based chemotherapy. The frequency of febrile neutropenia did not differ between the risk groups. The overall survival was 100% and event-free survival (EFS) was 95.7%. The EFS rate was 100% in the LR group and 90% in the HR group, respectively. Conclusion: Despite their common occurrence in high-risk patients, no lethal hematological complications were associated with carboplatin-based treatment. The current carboplatin-based chemotherapy protocol is safe and effective for the treatment of pediatric patients with IC-GCTs.

• Clinical and Experimental Pediatrics
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• 제48권2호
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• pp.186-190
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• 2005

목 적 : 두개 내 생식 세포종 환자를 진단 시 저위험군 및 고 위험군으로 분류하여 화학요법의 강도에 차이를 두어 치료 반응 및 독성을 관찰하고자 하였다. 방 법 : 2002년 10월부터 2003년 12월까지 삼성서울병원에서 두개 내 생식 세포종으로 진단되고 화학요법으로 먼저 치료한 소아 환자 14례를 대상으로 하였다. 배아 세포종이면서 AFP가 정상이고 beta-hCG가 50 mIU/mL 이하이면 저위험군, 그 외에는 고위험군으로 분류하였다. 화학요법은 저위험군의 경우(cisplatin $20mg/m^2/day$ on days 0-4, 42-46; VP-16 $100mg/m^2/day$ on days 0-4, 42-46; cyclophosphamide $1g/m^2/day$ on days 21-22, 63-64; vincristine $1.5mg/m^2/day$ on day 21, 28, 35, 63, 70, 77)에 비해 고위험군에서는 cisplatin과 cyclophosphamide의 용량을 두 배로 사용하였다. 화학요법 종결 후 방사선 치료를 시행하였다. 결 과 : 진단 방법으로 생검 10례, 아전절제 2례, 부분절제 1례, 종양 호르몬의 상승으로만 진단한 경우가 1례였다. 진단 시 중앙연령은 11.6세(1.2-18.7세)였고 고위험군 9례, 저위험군 5례였다. 7례(50%)에서 요붕증이 동반되었다. 종양 호르몬의 상승을 보였던 10례 모두 화학요법 후 정상화되었으며, 저위험군 중4례(80%)와 고위험군 중 7례(77.8%)가 화학요법에 완전반응을 보였다. 화학요법 후 부분반응 상태였던 3례 중 2례는 방사선 치료 후 완전관해에 도달하였고 고위험군 1례는 방사선 치료 전이차 수술을 시행하여 원발 종양은 제거되었으나 척수에 재발하여 구제요법을 시행 중이다. 저위험군 4례(80%)와 고위험군 전예에서 감염증으로 인하여 2-4회의 입원이 필요하였다. 고위험군 9례 중 4례(44.4%)에서 이명과 청력장애 증상이 발생하였고 치료 종결 후에도 지속되었다. 이 중 3례에서는 감각신경성 난청으로 진행되었다. 청력장애를 보인 4례 중 3례는 요붕증이 동반된 환자였다. 1례를 제외한 13례가 정중 추적 기간 13.9개월(8.1-22.3개월)간 무사건 생존 중이다. 결 론 : 고용량의 cisplatin을 근간으로 한 치료 방법으로 고위험군에서도 고무적인 치료 반응을 유도할 수 있었다. 그러나 치료 독성의 빈도가 높아 치료 방침의 수정이 필요할 것으로 사료된다.

• Journal of Korean Neurosurgical Society
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• 제30권5호
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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.