• Title, Summary, Keyword: Intracranial germ cell tumor

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Cytologic Features of Intracranial Germ Cell Tumors in Crush Preparation (두개내 악성 생식세포종양의 압착도말 세포학적 소견)

  • Yim, Hyun-Ee;Kim, Jung-Sun;Shim, Chul
    • The Korean Journal of Cytopathology
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    • v.7 no.2
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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.

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Understanding the Treatment Strategies of Intracranial Germ Cell Tumors : Focusing on Radiotherapy

  • Kim, Joo-Young;Park, Jeonghoon
    • Journal of Korean Neurosurgical Society
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    • v.57 no.5
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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.

Intracranial Endodermal Sinus Tumor (두개강내 내배엽성 동종양(Endodermal Sinus Tumor))

  • Lim, Yong Chull;Cho, Kyung Gi;Lee, Seong Un;Park, Han Jun;Shin, Yong Sam;Yun, Soo Han;Cho, Ki Hong
    • Journal of Korean Neurosurgical Society
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    • v.30 no.12
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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.

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Intracranial Germ Cell Tumor in the Molecular Era

  • Phi, Ji Hoon;Wang, Kyu-Chang;Kim, Seung-Ki
    • Journal of Korean Neurosurgical Society
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    • v.61 no.3
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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.

Failure Pattern of Pineal and Ectopic Pineal Germ Cell Tumor after Gamma Knife Radiosurgery (송과선 및 이소성 송과선 생식세포종의 감마 나이프 수술 후 재발 양상)

  • Cho, Heung-Lae;Sohn, Seung-Chang
    • Radiation Oncology Journal
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    • v.18 no.2
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    • pp.92-100
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    • 2000
  • Purpose :This study was performed to determine the optimal treatment velum of Patients treating with radiation therapy for intracranial germ cell tumor. Materials and Methods : From 1993 to 1998, 19 patients with intracranial germ cell tumors treated by gamma knife radiosurgery were analyzed. The location of tumor was as follows; 9 cases on pineal region, 1 case on suprasellar region, and 9 cases of multiple lesion. 7 patients were pathologically verified; 5 cases of germ cell tumor and 2 cases of non germinomatous germ cell tumor. Tumor volume was ranged from 2.4 cm$^{3}$ to 74 cm$^{3}$. Irradiation dose was 10 Gy to 20 Gy with 50% isodose curve. Follow up period was 10 months to 54 months. Results : Recurrences were observed in 14 cases among 19 (74%) patients. Complete remission and partial remission were achieved in 2 (11%) and 10 (53%) respectively. No response was observed in 7 (36%). 2 cases were recurred within original tumor bed. 6 cases were recurred beyond but contiguous with tumor bed. Ventricular relapses separated from pretreatment tumor bed were 3. Spinal recurrences were 4. Among 8 recurred cases of which tumor volume is smaller than 20 cm$^{3}$, 2 were recurred within original tumor bed, 4 were recurred beyond but contiguous with tumor bed, and 1 spinal recurrence. Meanwhile, 6 cases of which tumor volume larger than 20 cm3, 1 case was recurred beyond but contiguous with tumorbed, 2 ventricular recurrences separated with original tumor bed, and 3 spinal recurrences. 5 cases which did not show any recurrence sign showed characteristics of single lesion, tumor volume smaller than 20 cm$^{3}$ and normal tumor marker. All of 4 cases of spinal recurrences happened in the case having ventricular invasion or lesion. Among 9 cases having multiple lesion, only 3 cases recurred within original tumor bed or around tumor bed, the other 6 cases recurred separated from pretreatment tumor bed. Conclusion : Gamma knife radiosurgery is not recommended for the treatment of intracranial germ cell tumor. It is because of small treatment volume and inadequate radiation dose that are characteristics of gamma knife radiosurgery. Tumor volume, ventricular invasion or ventricular lesion in multiple lesion are important factors to be considered for the wide field radiation therapy Tumor volume smaller than 20 cm$^{3}$, single lesion, no ventricular lesion or invasion, and normal tumor marker are ideal indications for small involved field radiation therapy. Prophylactic spinal irradiation seems to be necessary when there is ventricular lesion, ventricular invasion, and multiple lesions. When the tumor volume is larger than 20 cm$^{3}$, multiple lesions, abnormal tumor marker, and whole ventricular irradiation or partial brain irradiation would be possible and neoadjuvant chemotherapy would be most beneficial in these group.

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Neurocognitive and Psychological Functioning of Children with an Intracranial Germ Cell Tumor

  • Park, Younghee;Yu, Eun-Seung;Ha, Boram;Park, Hyeon-Jin;Kim, Jong-Heun;Kim, Joo-Young
    • Cancer Research and Treatment
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    • v.49 no.4
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    • pp.960-969
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    • 2017
  • Purpose This study was conducted to investigate the neurocognitive functioning of children with intracranial germ cell tumor (IGCT) prior to receiving proton beam therapy (PBT), and to identify differential characteristics of their neurocognitive functioning depending on tumor location. As a secondary object of this study, neurocognitive functions were followed up at 1-2 years after PBT to examine early post-treatment changes. Materials and Methods Between 2008 and 2014, 34 children with IGCT treated who received PBT at National Cancer Center, Korea were enrolled in this study. Standardized neurocognitive tests of intelligence, memory, and executive functioning were performed with baseline psychological assessments using the Child Behavior Checklist (CBCL). Follow-up assessments after PBT were conducted in 20 patients (T2). The results were analyzed based on the locations of tumors, which included the suprasellar, pineal gland, basal ganglia, and bifocal regions. Results The neurocognitive function of IGCT patients was significantly lower than that of the normal population in performance intelligence quotient (p=0.041), processing speed (p=0.007), memory (p < 0.001), and executive functioning (p=0.010). Patients with basal ganglia tumors had significantly lower scores for most domains of neurocognitive functioning and higher scores for CBCL than both the normal population and patients with IGCT in other locations. There was no significant change in neurocognitive function between T1 and T2 for all types of IGCT patients in first 1-2 years after PBT. Conclusion Tumor location significantly affects the neuropsychological functioning in patients with IGCT. Neuropsychological functioning should be closely monitored from the time of diagnosis in IGCT patients.

Responses and adverse effects of carboplatin-based chemotherapy for pediatric intracranial germ cell tumors

  • Ji, Sun-Tae;Chueh, Hee-Won;Kim, Ju-Youn;Lim, Su-Jin;Cho, Eun-Joo;Lee, Soo-Hyun;Yoo, Keon-Hee;Sung, Ki-Woong;Koo, Hong-Hoe
    • Clinical and Experimental Pediatrics
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    • v.54 no.3
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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.

Pineal and Suprasellar Germinoma Cooccurence with Vertebra Plana: A Case Report

  • Toosi, Farrokh Seilanian;Aminzadeh, Behzad;Rad, Mohammad Faraji;Nekooei, Sirous;Nahidi, Mahsa;Keykhosravi, Ehsan
    • 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.

Responses and Toxicities of Risk-adapted Chemotherapy in Pediatric Intracranial Germ Cell Tumors (소아 두개 내 생식 세포종에서 위험군에 따른 화학요법의 치료 반응 및 독성)

  • You, Dong Kil;Lee, Soo Hyun;Yoo, Keon Hee;Sung, Ki Woong;Lim, Do Hoon;Shin, Hyung Jin;Koo, Hong Hoe
    • Clinical and Experimental Pediatrics
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    • v.48 no.2
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    • pp.186-190
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    • 2005
  • Purpose : The purpose of this study was to evaluate the responses and toxicities of risk-adapted chemotherapy in pediatric intracranial germ cell tumors(IC-GCT). Methods : Fourteen patients who were diagnosed as IC-GCT from October 2002 to December 2003 received chemotherapy as an initial treatment modality. The low risk(LR) group was defined as follows : Pure germinoma and normal AFP level. Beta-hCG level 50 mIU/mL or less. The others belonged to the high risk(HR) group. Chemotherapy was composed of cisplatin, cyclophosphamide, etoposide and vincristine. Double doses of cisplatin and cyclophosphamide was used in HR patients. Results : Pathologic confirmation was done in all but one. Median age at diagnosis was 11.6 yr (1.2-18.7 yr), and nine patients belonged to the HR group. Tumor markers were normalized after chemotherapy in all patients whose tumor markers had been elevated. Four LR patients(80 percent) and seven HR patients(77.8 percent) showed complete response(CR) at the end of chemotherapy. An additional two of the three patients with partial response(PR) achieved CR after radiation therapy (RT), and the remaining one relapsed before RT. Four LR and all HR patients experienced infectious episodes that required hospitalization. Four of the nine HR patients(44.4 percent) suffered from tinnitus, three of whom developed sensorineural hearing loss. All but one are surviving, event-free, with a median follow-up of 13.9 mo(8.1-22.3 mo). Conclusion : Risk-adapted cisplatin-based chemotherapy was effective even in HR patients, but regimen modification seems to be necessary to avoid an unacceptably high toxicity rate.

Endoscopic Management of Pineal Region Tumors with Associated Hydrocephalus (수두증을 동반한 송과체 부위 종양에 대한 내시경적 치료)

  • Kim, Jeong Hoon;Ra, Young Shin;Kim, Joon Soo;Ahn, Jae Sung;Kim, Chang Jin;Kwun, Byung Duk
    • 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.

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