Table 7 - uploaded by Antonio Perez-Martinez
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Therapeutic classification of intracranial germ cell tumours. Group with good prognosis Pure germinoma Mature teratoma
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Introduction
Intracranial germ cell tumours are rare in children. They are a heterogeneous group of neoplasms that show different clinical manifestations despite having a common origin.
Patients and methods
A retrospective analysis was carried out on the epidemiological and histological characteristics, clinical manifestations, and outcomes of 20...
Context in source publication
Context 1
... This distinction is very relevant in clinical practice, as histological subtype is the most significant isolated prog- nostic factor in IC GCTs. 7,14,16 Different authors have proposed the therapeutic classifi- cation of IC GCTs based on histology 15,25 categorising IC GCTs as having a good, intermediate, or poor prognosis ( Table 7). Germinomas are extremely sensitive to CTX and radia- tion. ...
Citations
... To our knowledge, this is the first study that assessed the difference in the OS and tumour size between groups of patients with early and delayed diagnosis of intracranial germinoma. It also appears to be the first study involving a large group of patients, in whom the first neurological and endocrinological symptoms occurred before the tumour could be diagnosed by the MRI [27,28]. ...
Background:
Intracranial germinoma is a rare malignant neoplasm of the central nervous system (CNS) that occurs in children and young adults. The aim of our study was to assess the initial manifestation of the disease, and to find differences in outcomes dependent on time of diagnosis.
Methods:
The study group consisted of 35 consecutive patients (adults and children) who were treated for intracranial germinoma with radiotherapy at a tertiary centre, and their data were retrospectively collected. We evaluated time from the first symptoms to diagnosis and divided patients into early and delayed diagnosis groups. Delayed diagnosis has been defined as the time from initial presentation to final diagnosis longer than six months.
Results:
A total of 17 (48.6%) of the patients had delayed diagnoses. Patient survival data spanned a median of six (interquartile range 3-12) years. At the time of the diagnosis, patients presented exclusively neurological symptoms in 16 (45.7%) cases, exclusively endocrinological symptoms in five (14.3%) cases, and mixed symptoms in the remaining cases (n = 14; 40.0%). Patients with neurological symptoms had shorter time (p < 0.001) from first symptoms to the final diagnosis (5.91 months) than in patients without them (19.44 months). The delayed diagnosis group presented significantly smaller tumour size (mean maximal dimension 2.35 cm) compared to early diagnosis group (3.1 cm). The 5-year and 10-year survival rates of our patients were 94.3% and 83.4%, respectively. Patients with a delayed diagnosis (n = 17) had a significantly worse (p = 0.02) 10-year OS (63%) compared to the early diagnosis group (n = 18; OS = 100%). Importantly, in five patients (14.29%), initial manifestation occurred before radiological signs of the disease.
Conclusion:
Our study stresses the need for timely diagnosis in intracranial germinoma, as a delay has a significant impact on the prognosis. In particular, if the tumour is small or causes exclusively endocrinological symptoms, the diagnosis may be difficult and delayed.
... Oligodendroglioma and low-grade astrocytoma are the most common pathological types of CNS tumors known as the low-grade gliomas [2,3]. CNS germ cell tumor is the third most common CNS tumor diagnosed primarily during childhood and adolescence [4] with intracranial germinoma (IG) as its most common histological subtype [3,5,6]. Although IG, oligodendroglioma, and low-grade astrocytoma all belong to the CNS tumor category and have similar cellular morphology, their therapeutic schemes and prognoses vary greatly. ...
The pathological diagnosis of intracranial germinoma (IG), oligodendroglioma, and low-grade astrocytoma on intraoperative frozen section (IFS) and hematoxylin-eosin (HE)-staining section directly determines patients' treatment options, but it is a difficult task for pathologists. We aimed to investigate whether whole-slide imaging (WSI)-based deep learning can contribute new precision to the diagnosis of IG, oligodendroglioma, and low-grade astrocytoma. Two types of WSIs (500 IFSs and 832 HE-staining sections) were collected from 379 patients at multiple medical centers. Patients at Center 1 were split into the training, testing, and internal validation sets (3:1:1), while the other centers were the external validation sets. First, we subdivided WSIs into small tiles and selected tissue tiles using a tissue tile selection model. Then a tile-level classification model was established, and the majority voting method was used to determine the final diagnoses. Color jitter was applied to the tiles so that the deep learning (DL) models could adapt to the variations in the staining. Last, we investigated the effectiveness of model assistance. The internal validation accuracies of the IFS and HE models were 93.9% and 95.3%, respectively. The external validation accuracies of the IFS and HE models were 82.0% and 76.9%, respectively. Furthermore, the IFS and HE models can predict Ki-67 positive cell areas with R2 of 0.81 and 0.86, respectively. With model assistance, the IFS and HE diagnosis accuracy of pathologists improved from 54.6%-69.7% and 53.5%-83.7% to 87.9%-93.9% and 86.0%-90.7%, respectively. Both the IFS model and the HE model can differentiate the three tumors, predict the expression of Ki-67, and improve the diagnostic accuracy of pathologists. The use of our model can assist clinicians in providing patients with optimal and timely treatment options.
... In this study, the proportion of multifocal tumors was comparable to that of the North American cohort, whereas that of the basal ganglia and bifocal tumors was similar to that in the Japanese cohort. Although the primary tumor site did not have a bearing on the survival of patients in their study, multiple other studies have shown that ICGCTs located in the pineal region fare better than those in non-pineal locations [4,[23][24][25][26]. Surprisingly, in this study, patients with tumors in non-pineal locations had significantly better PFS (p = 0.043) than those with pineal tumors, although no difference was observed in OS. ...
... The survival outcome of this study population was comparable to published outcomes of various study groups (Table 4) [4,6,11,[15][16][17][22][23][24][25][29][30][31][32]. In this study, administration of chemotherapy prior to RT has been shown to translate into an OS benefit. ...
Background: Primary intracranial germ cell tumors (ICGCT) are often diagnosed with tumor markers and imaging, which may avoid the need for a biopsy. An intracranial germ cell tumor with mild elevation of markers is seldom stratified as a distinct entity. Methods: Fifty-nine patients were stratified into three groups: pure germinoma (PG), secreting germinoma (SG) and non-germinomatous germ cell tumors (NGGCTs). Results: At 5 years, progression-free survival and overall survival of the three groups (PG vs SG vs NGGCT) were 91% versus 81% versus 59%, and 100% versus 82% versus 68%, respectively. There was no statistically significant difference in outcome among histologically and clinically diagnosed germinomas. Conclusion: A criterion for clinical diagnosis when a biopsy is not feasible is elucidated, and comparable outcomes were demonstrated with histologically diagnosed germinomas.
... Tres pacientes tenían lesiones pineales y todos cursaron con aumento de la presión intracraneal seguido de déficit visual. De la misma manera, Cormenzana Carpio et al. 4 llevó a cabo un análisis retrospectivo de veinte pacientes diagnosticados con TGIC entre 1994 a 2014, de los cuales, 45% tenían tumores pineales, y de estos, el 77.7% presentó cefalea y diplopía al momento del diagnóstico, y 44.4% presentaron alteraciones del campo visual. ...
Introducción: Los tumores de células germinales intracraneales constituyen un grupo infrecuente de neoplasias que afectan principalmente a la población pediátrica. Su presentación clínica varía en función de la localización y el tamaño del tumor. Las lesiones localizadas en la región pineal a menudo causan hidrocefalia obstructiva, mientras que las lesiones de localización supraselar, usualmente se asocian a disfunción del eje hipotálamo-hipofisario. Un tumor germinal de localización pineal que curse con endocrinopatías es una entidad extremadamente rara. Por tanto, reportamos el caso de un paciente masculino de 16 años de edad, con panhipopituitarismo secundario a un germinoma pineal.
Material y métodos: Este paciente fue referido al Departamento de Emergencias tras presentar hipernatremia en un análisis sanguíneo de rutina. Se presentó con polidipsia, poliuria y fatiga de un año de evolución. Las evaluaciones clínicas y pruebas de laboratorio reportaron diabetes insípida, panhipopituitarismo y hemianopsia bitemporal. El paciente recibió terapia de sustitución hormonal y tres meses después, la resonancia magnética de seguimiento reveló una masa en el área pineal que, a través de una posterior evaluación, fue diagnosticada como germinoma.
Conclusión: Luego de tratamiento con quimioterapia y radiación, hubo una resolución completa de la lesión. Sin embargo, no hubo mejoría clínica significativa y el paciente continúo necesitando terapia de reemplazo hormonal.
Pineal germinoma is rare with high cure rates following craniospinal radiotherapy. Efforts to reduce the radiotherapy dose and field via combination with chemotherapy suggest comparable disease control and reduced neurocognitive impairments, while the efficacy of immunotherapy in pineal germinoma remains undetermined. This report aimed to review clinical outcomes in patients treated for pineal germinoma in Queensland, Australia, and assess for Programmed Death-Ligand1 (PD-L1) expression. Patients who commenced radiation and/or chemotherapy for pineal germinoma from 2005 to 2017 were retrospectively identified using Queensland Oncology Online database. Demographic, diagnostic, treatment, and outcome data was obtained from electronic medical records. PD-L1 immuno-histochemistry was performed on available specimens. Eighteen patients with long-term follow-up data were identified. Median age at diagnosis was 16.8 years (range 9–46 years). Diagnosis was made histologically in fifteen patients, and radiologically in three. All patients underwent radiotherapy (median 36 Gy (range 21–54 Gy)) with lower median dose delivered with whole ventricle irradiation (12/18patients) than craniospinal irradiation (5/18patients). Sixteen patients received chemotherapy preceding radiotherapy. All patients are alive at median 7.25 years from primary treatment completion (range 2.03–13.1 years). Relapse occurred in three patients (16.67%) following treatment response, all of whom achieved remission following high-dose chemotherapy with stem-cell support and craniospinal radiotherapy. Post-treatment functional outcomes were similarly excellent. PD-L1 expression was low (1–49% cells) or negative in 87% of tumours tested but results were confounded by specimen quality and availability. Reduced-dose radiotherapy with chemotherapy does not compromise outcome and is standard of care at this institution. Immunotherapy is unlikely to become standard treatment in the near future.
OBJECTIVE
Central nervous system (CNS) germ cell tumors (GCTs) are rare malignant neoplasms that arise predominantly in adolescents and young adults. CNS GCTs demonstrate characteristic trends in national associations, with implications for both tumor incidence and genetics. Although the incidence of CNS GCTs is markedly higher in East Asia than Western countries, direct comparative analyses between these CNS GCT populations are limited.
METHODS
In Japan, to facilitate the genomic analyses of CNS GCTs, the Intracranial Germ Cell Tumor Genome Analysis Consortium was established in 2011, and more than 200 cases of GCTs are available for both tumor tissue and clinical data, which is organized by the National Cancer Center (NCC) Japan. At the Mayo Clinic, there have been 98 cases of intracranial GCTs treated by the Department of Neurologic Surgery since 1988. In this paper, the authors compared the epidemiology, clinical presentation including location and histology, and prognosis between cases treated in the US and Japan.
RESULTS
There was no significant difference in age and sex distributions between the databases. However, there was a significant difference in the tumor locations; specifically, the frequency of basal ganglia was higher in the NCC database compared with the Mayo Clinic (8.4% vs 0%, p = 0.008), and bifocal location (neurohypophysis and pineal gland) was higher at the Mayo Clinic than at the NCC (18.8% vs 5.8%, p = 0.002). There was no difference in histological subdivisions between the databases. There was no difference in progression-free survival (PFS) and overall survival (OS) of germinoma cases and OS of nongerminomatous GCT (NGGCT) cases treated with chemotherapy and radiation therapy covering whole ventricles. However, PFS of NGGCTs differed significantly, and was better in the NCC cohorts (p = 0.04).
CONCLUSIONS
There appears to be a differential distribution of GCTs by neuroanatomical location between major geographic and national groups. Further study is warranted to better characterize any underlying genomic, epigenetic, or environmental factors that may be driving the phenotypic differences.
