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Neuro-oncology Aug 2022This multinational study was conducted to report clinical presentations and treatment strategies in patients with intracranial germinomas across selected Asian centers,...
BACKGROUND
This multinational study was conducted to report clinical presentations and treatment strategies in patients with intracranial germinomas across selected Asian centers, including failure patterns, risk factors, and outcomes.
METHODS
A retrospective data collection and analysis of these patients, treated between 1995 and 2015 from eight healthcare institutions across four countries was undertaken.
RESULTS
From the results, 418 patients were analyzed, with a median follow-up of 8.9 years; 79.9% of the patients were M0, and 87.6% had β-human chorionic gonadotropin values <50 mIU/mL. The 5/10-year overall survival (OS) and recurrence-free survival (RFS) rates were 97.2%/96.2% and 89.9%/86.9%, respectively. RFS was predicted by the radiotherapy (RT) field, with focal RT having the worst outcome, whereas chemotherapy usage had no impact on survival. Among patients who received chemotherapy, response to chemotherapy did not predict survival outcomes. In M0 patients, primary basal ganglia tumors predicted a worse RFS. In patients with bifocal tumors, an extended field RT was associated with better outcomes. In multivariable analysis, only RT fields were associated with RFS. In relapsed patients, salvage rates were high at 85.7%. Additionally, patients who received salvage RT had a better outcome (91.6% vs. 66.7%).
CONCLUSIONS
Survival outcomes of patients with germinoma were excellent. Thus, the focus of treatment for intracranial germinoma should be on survivorship. Further studies are warranted to find the optimal intensity and volume of radiation, including the role of chemotherapy in the survival of patients with intracranial germinomas, considering age, primary tumor location, and extent of disease.
Topics: Brain Neoplasms; Germinoma; Humans; Pineal Gland; Retrospective Studies; Salvage Therapy
PubMed: 34935949
DOI: 10.1093/neuonc/noab295 -
Surgical Neurology International 2022Pineal tumors are uncommon tumors that affect <1% of adults, with 50% of them being germinomas. A combination of endoscopic third ventriculostomy (ETV) and tumor biopsy...
BACKGROUND
Pineal tumors are uncommon tumors that affect <1% of adults, with 50% of them being germinomas. A combination of endoscopic third ventriculostomy (ETV) and tumor biopsy is usually used. Cerebral vasospasm in association with aneurysmal subarachnoid hemorrhage (aSAH) has been extensively studied. However, at least according to the research, this is not the case in intraventricular hemorrhage (IVH) cases. We present a case with two distinct findings: (1) an unexpected large IVH following the removal of an external ventricular drain (EVD) in a patient who had undergone ETV and tumor biopsy, resulting in severe clinical vasospasm and (2) incidental pineal region germinoma regression on follow-up magnetic resonance imaging (MRI) without any prior adjuvant chemoradiation to explain such regression.
CASE DESCRIPTION
The authors describe an 18-year-old male patient who had a routine, uneventful combined ETV and tumor biopsy, as well as the placement of an EVD. Histopathological examination revealed germinoma. His postoperative course was complicated by IVH after EVD removal, which resulted in clinical vasospasm. Without any prior adjuvant chemoradiation, follow-up MRI of the b rain revealed a significant reduction in the size of the germinoma as well as reconstitution of the patency of the previously obstructed aqueduct of Sylvius.
CONCLUSION
The take-home message from this case is that in the case of postoperative clinical deterioration in a patient with concurrent IVH and ETV, a high index of suspicion for vasospasm is required, as this may allow a significant amount of blood to pass down to the basal cisterns. Early detection and management of clinical vasospasm are critical for a better neurological outcome. Furthermore, unexpected tumor size changes can occur due to a variety of factors, so recent preoperative MRI of the brain should be obtained in the lead-up to surgery, and postoperative computed tomography should be used sparingly to avoid radiation-related tumor changes.
PubMed: 35127229
DOI: 10.25259/SNI_944_2021 -
Journal of Clinical Imaging Science 2021We are reporting a case of 16-year-old boy presented with severe headache and blurred vision. MRI showed a pineal region mass that was initially thought to be a...
We are reporting a case of 16-year-old boy presented with severe headache and blurred vision. MRI showed a pineal region mass that was initially thought to be a germinoma. Endoscopic biopsy revealed tuberculoma which is an exceedingly rare and unusual location for CNS tuberculoma. The patient was treated successfully with anti-tuberculous therapy.
PubMed: 34221644
DOI: 10.25259/JCIS_84_2021 -
Surgical Neurology International 2018Germ cell tumors comprise a heterogeneous group of neoplasms, classified as germinomas and nongerminomatous germ cell tumors based on clinicopathological features. The...
BACKGROUND
Germ cell tumors comprise a heterogeneous group of neoplasms, classified as germinomas and nongerminomatous germ cell tumors based on clinicopathological features. The nongerminomatous group of tumors includes embryonal carcinoma, endodermal sinus tumor (yolk sac tumor), choriocarcinoma, mature and immature teratoma, and mixed germ cell tumors with more than one element. While germinomas are radiation-sensitive tumors, all other tumors have less response to radiotherapy, and it is suggested that gross total resection improves their overall survival and tumor-free survival rates. Herein, we present the microsurgical management of a histologically confirmed mixed-germ cell of the pineal region.
CASE DESCRIPTION
A patient with a mixed germ cell tumor underwent sitting praying position and midline supracerebellar infratentorial approach. After opening of the dura, a midline cerebellar vein was coagulated and cut, and the pineal region was accessed over the superior cerebellar surface. A tight reactive dorsal membrane of the quadrigeminal cistern was widely opened with subsequent evaluation of the neurovascular structures by intraoperative angiography. Under high microsurgical magnification between both basal veins, the dorsal wall of the fibrotic and solid tumor was coagulated and opened aiming an internal debulking of the lesion. Water dissection and cotton dissection were useful tools to separate the lateral borders of the tumor from the surroundings. Bipolar coagulation was helpful shrinking the tumor as well. The superior borders of the lesion, firmly attached to the roof of the third ventricle, required a careful evaluation. Ring microforceps in the right hand and thumb-regulated suction tube in the left one allowed us to pull out the tumor in a piece under soft and continuous traction with dissection of the cleavage plane. The superior attachment of the tumor was coagulated and cut. Finally, bipolar coagulation and small pieces of surgicel ensured a proper hemostasis. Postoperatively, the patient had a partial gaze palsy that improved gradually. The patient underwent adjuvant radiochemotherapy and currently is alive, free of tumor recurrence >12 years after surgery.
CONCLUSION
This unedited video offers all detailed aspects that a neurosurgeon as the senior author JH considers essential when performing an efficient and safe surgery for a mixed germ cell tumor.
VIDEOLINK
http://surgicalneurologyint.com/videogallery/pineal-tumor-5.
PubMed: 30687573
DOI: 10.4103/sni.sni_357_18 -
Radiation Oncology (London, England) Jun 2015We performed dosimetric comparisons between proton beam therapy and intensity modulated radiotherapy (IMRT) of intracranial germ cell tumors (ICGCTs) arising in various... (Clinical Trial)
Clinical Trial Comparative Study
BACKGROUND
We performed dosimetric comparisons between proton beam therapy and intensity modulated radiotherapy (IMRT) of intracranial germ cell tumors (ICGCTs) arising in various locations of the brain.
MATERIALS
IMRT, passively scattered proton therapy (PSPT), and spot scanning proton therapy (SSPT) plans were performed for four different target volumes: the whole ventricle (WV), pineal gland (PG), suprasellar (SS), and basal ganglia (BG). Five consecutive clinical cases were selected from the patients treated between 2011 and 2014 for each target volume. Total 20 cases from the 17 patients were included in the analyses with three overlap cases which were used in plan comparison both for the whole ventricle and boost targets. The conformity index, homogeneity index, gradient index, plan quality index (PQI), and doses applied to the normal substructures of the brain were calculated for each treatment plan.
RESULTS
The PQI was significantly superior for PSPT and SSPT than IMRT for ICGCTs in all locations (median; WV: 2.89 and 2.37 vs 4.06, PG: 3.38 and 2.70 vs 4.39, SS: 3.92 and 2.49 vs 4.46, BG: 3.01 and 2.49 vs 4.45). PSPT and SSPT significantly reduced the mean dose, and the 10 and 15 Gy dose volumes applied to the normal brain compared with IMRT (p ≤ 0.05). PSPT and SSPT saved significantly greater volumes of the temporal lobes and hippocampi (p < 0.05) in the SS and PG targets than IMRT. For tumors arising in the BG, PSPT and SSPT also saved greater volumes of the contralateral temporal lobes.
CONCLUSIONS
PSPT and SSPT provide superior target volume coverage and saved more normal tissue compared with IMRT for ICGCTs in various locations. Future studies should assess whether the extent of normal tissue saved has clinical benefits in children with ICGCTs.
Topics: Adolescent; Antineoplastic Combined Chemotherapy Protocols; Basal Ganglia; Brain Neoplasms; Child; Child, Preschool; Combined Modality Therapy; Female; Germinoma; Humans; Infant; Male; Neoplasms, Germ Cell and Embryonal; Organ Sparing Treatments; Pineal Gland; Pituitary Neoplasms; Proton Therapy; Radiation Injuries; Radiometry; Radiotherapy Dosage; Radiotherapy Planning, Computer-Assisted; Radiotherapy, Image-Guided; Radiotherapy, Intensity-Modulated; Treatment Outcome
PubMed: 26112360
DOI: 10.1186/s13014-015-0441-5 -
Neuro-oncology Nov 2017Following promising results to increase survival and reduce treatment burden in intracranial non-germinomatous germ cell tumors (NGGCTs), we conducted a European study...
BACKGROUND
Following promising results to increase survival and reduce treatment burden in intracranial non-germinomatous germ cell tumors (NGGCTs), we conducted a European study using dose-intense chemotherapy followed by risk-adapted radiotherapy.
METHODS
All patients received 4 courses of cisplatin/etoposide/ifosfamide. Non-metastatic patients then received focal radiotherapy only (54 Gy); metastatic patients received 30 Gy craniospinal radiotherapy with 24 Gy boost to primary tumor and macroscopic metastatic sites.
RESULTS
Patients with localized malignant NGGCT (n = 116) demonstrated 5-year progression-free survival (PFS) and overall survival (OS) of 0.72 ± 0.04 and 0.82 ± 0.04, respectively. Primary tumor sites were: 67 pineal, 35 suprasellar, 5 bifocal, 9 others. One patient died postsurgery in clinical remission; 3 patients progressed during treatment and 27 (23%) relapsed afterward. Fourteen were local, 6 combined, and 7 distant relapses (outside radiation field). Seventeen of the 27 relapsed patients died of disease. Patients with metastatic disease (n = 33) demonstrated 5-year PFS and OS of 0.68 ± 0.09 and 0.75 ± 0.08, respectively; 1 patient died following progression on treatment and 9 (27%) relapsed afterward (5 local, 1 combined, 3 distant). Only one metastatic patient with recurrence was salvaged. Multivariate analysis identified diagnostic alpha-fetoprotein level (serum and/or cerebrospinal fluid level >1000 ng/mL, 19/149 patients, of whom 11 relapsed; P < 0.0003) and residual disease following treatment, including after second-look surgery (n = 52/145 evaluable patients, 26 relapsed; P = 0.0002) as significant prognostic indicators in this cohort.
CONCLUSION
In localized malignant NGGCT, craniospinal radiotherapy could be avoided without increased relapses outside the radiotherapy field. Chemotherapy and craniospinal radiotherapy remain the gold standard for metastatic disease.
Topics: Adolescent; Adult; Antineoplastic Combined Chemotherapy Protocols; Biomarkers, Tumor; Brain Neoplasms; Chemoradiotherapy; Child; Child, Preschool; Cisplatin; Cranial Irradiation; Etoposide; Female; Follow-Up Studies; Humans; Ifosfamide; International Agencies; Lymphatic Metastasis; Male; Neoplasm Recurrence, Local; Neoplasms, Germ Cell and Embryonal; Prognosis; Prospective Studies; Survival Rate; Testicular Neoplasms; Young Adult
PubMed: 29048505
DOI: 10.1093/neuonc/nox122 -
Cancer Research and Treatment Oct 2020Whether craniospinal irradiation (CSI) could be replaced by limited-field radiation in non-metastatic bifocal germinoma remains controversial. We addressed the issue... (Comparative Study)
Comparative Study
PURPOSE
Whether craniospinal irradiation (CSI) could be replaced by limited-field radiation in non-metastatic bifocal germinoma remains controversial. We addressed the issue based on the data from our series and the literature.
MATERIALS AND METHODS
Data from 49 patients diagnosed with non-metastatic bifocal germinoma at our hospital during the last 10 years were collected. The Pediatric Quality of Life Inventory 4.0 was used to evaluate health-related quality of life (HRQOL). Additionally, 81 patients identified from the literature were also analyzed independently.
RESULTS
In our cohort, 34 patients had tumors in the sellar/suprasellar (S/SS) plus pineal gland (PG) regions and 15 in the S/SS plus basal ganglia/thalamus (BG/T) regions. The median follow-up period was 52 months (range, 10 to 134 months). Our survival analysis showed that patients treated with CSI (n=12) or whole-brain radiotherapy (WBRT; n=34) had comparable disease-free survival (DFS; p=0.540), but better DFS than those treated with focal radiotherapy (FR; n=3, p=0.016). All 81 patients from the literature had tumors in the S/SS+PG regions. Relapses were documented in 4/45 patients treated with FR, 2/17 treated with whole-ventricle irradiation, 0/4 treated with WBRT, and 1/15 treated with CSI. Survival analysis did not reveal DFS differences between the types of radiation field (p=0.785). HRQOL analysis (n=44) in our cohort found that, compared with S/SS+PG germinoma, patients with BG/T involvement had significantly lower scores in social and school domains. However, HRQOL difference between patients treated with CSI and those not treated with CSI was not significant.
CONCLUSION
In patients with non-metastatic bifocal germinoma, it is rational that CSI could be replaced by limited-field radiation. HRQOL in patients with BG/T involvement was poorer.
Topics: Adolescent; Adult; Antineoplastic Combined Chemotherapy Protocols; Brain Neoplasms; Chemoradiotherapy; Child; Child, Preschool; Cranial Irradiation; Craniospinal Irradiation; Disease-Free Survival; Female; Follow-Up Studies; Germinoma; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Neoplasm Recurrence, Local; Pineal Gland; Quality of Life; Radiotherapy Dosage; Young Adult
PubMed: 32646203
DOI: 10.4143/crt.2020.437 -
Cureus Aug 2022Tumors involving the pineal gland include germinomas, non-germinomatous, and parenchymal tumors. Sometimes these tumors can be differentiated into rhabdomyosarcoma,...
Tumors involving the pineal gland include germinomas, non-germinomatous, and parenchymal tumors. Sometimes these tumors can be differentiated into rhabdomyosarcoma, which is an aggressive and rapidly recurring sarcoma but is a rare event. We present the case of a 23-year-old male, with an eight-year-long history of a non-treated brain tumor compatible with a teratoma. Chemotherapy and radiotherapy were offered, and two years later, malignant transformation to astrocytoma, rhabdomyosarcoma, neural cell carcinoma, ganglioglioma, and low-grade chondrosarcoma was noted. Immunohistochemistry was valuable in differentiating these entities that confirmed the diagnosis. Malignant transformations may be secondary to the normal transformation of multipotent embryonic cells into more developed tissues after radiotherapy of teratoma and malignant ectomesenchymoma transformation.
PubMed: 36081970
DOI: 10.7759/cureus.27711 -
Neuro-oncology May 2022Central nervous system (CNS) germ cell tumors (GCTs) are neoplasms predominantly arising in pediatric and young adult populations. While germinomas generally respond to...
BACKGROUND
Central nervous system (CNS) germ cell tumors (GCTs) are neoplasms predominantly arising in pediatric and young adult populations. While germinomas generally respond to chemotherapy and radiation, non-germinomatous GCTs (NGGCTs) require more intensive treatment. This study aimed to determine whether 12p gain could predict the prognosis of CNS GCTs.
METHODS
Eighty-two CNS GCTs were included in this study. The 12p gain was defined by an additional 12p in the background of potential polyploidy or polysomy. Cases were analyzed using an Illumina methylation 450K array for copy number investigations and validated by fluorescence in situ hybridization (FISH).
RESULTS
A 12p gain was found in 25-out-of-82 cases (30%) and was more frequent in NGGCTs (12% of germinoma cases and 50% of NGGCT cases), particularly in cases with malignant components, such as immature teratoma, yolk sac tumor, choriocarcinoma, and embryonal carcinoma. 12p gain and KIT mutation were mutually exclusive events. The presence of 12p gain correlated with shorter progression-free (PFS) and overall survival (OS) (10-year OS: 59% vs. 94%, with and without 12p gain, respectively, P = 0.0002), even with histology and tumor markers incorporated in the multivariate analysis. Among NGGCTs, 12p gain still had prognostic significance for PFS and OS (10-year OS: 47% vs. 90%, respectively, P = 0.02). The 12p copy number status was shared among histological components in mixed GCTs.
CONCLUSIONS
12p gain may predict the presence of malignant components of NGGCTs, and poor prognosis of the patients. It may be associated with early tumorigenesis of CNS GCT.
Topics: Brain Neoplasms; Central Nervous System Neoplasms; Child; Germinoma; Humans; In Situ Hybridization, Fluorescence; Male; Neoplasms, Germ Cell and Embryonal; Pineal Gland; Testicular Neoplasms
PubMed: 34698864
DOI: 10.1093/neuonc/noab246 -
Cancer Research and Treatment Apr 2017Malignant central nervous system (CNS) germ cell tumors (GCTs), although rare, are thought to occur more frequently among Asians. However, a recent population-based...
PURPOSE
Malignant central nervous system (CNS) germ cell tumors (GCTs), although rare, are thought to occur more frequently among Asians. However, a recent population-based study revealed no differences in GCT incidence between Asians and Caucasians. Therefore, this study was conducted to determine the incidence and survival rates of CNS GCTs using the national cancer incidence database, and to compare these rates to those in the United States and Japan.
MATERIALS AND METHODS
We extracted CNS GCT patients diagnosed between 2005 and 2012 from the Korea Central Cancer Registry database. Age-standardized rates (ASRs), annual percentage change, and the male-female incidence rate ratios (IRRs) were calculated. To estimate the survival rate, we used data for patients diagnosed between 2005 and 2010 and followed their cases until December 31, 2013.
RESULTS
The ASR for CNS GCT between 2005 and 2012 was 0.179 per 100,000 (95% confidence interval, 0.166 to 0.193), with an overall male-to-female (M:F) IRR of 2.95:1. However, when stratified by site, the M:F IRR was 13.62:1 for tumors of the pineal region and 1.87:1 for those located in nonpineal regions. The most frequent histologic type was germinoma (76.0%), and the most frequent location was the suprasellar region (48.5%). The 5-year survival rate of germinoma patients was 95.3%.
CONCLUSION
The incidence rate of CNS GCTs in Korea during 2005-2012 was 0.179 per 100,000, which was similar to that of the Asian/Pacific Islander subpopulation in the United States. Moreover, the CNS GCT survival rate in Korea was similar to rates in Japan and the United States.
Topics: Adolescent; Adult; Central Nervous System Neoplasms; Child; Child, Preschool; Female; History, 21st Century; Humans; Incidence; Infant; Infant, Newborn; Male; Middle Aged; Neoplasms, Germ Cell and Embryonal; Population Surveillance; Registries; Republic of Korea; Sex Factors; Young Adult
PubMed: 27554477
DOI: 10.4143/crt.2016.129