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Tumors of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Tumors arise from totipotential germ cells, capable of producing several different cell types; tumors can be composed of more than one tumor cell type. Tumors are classified by cell of origin into two basic types: undifferentiated germinoma and nongerminoma (NGGCT).
Central Nervous System
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
The outcome following these approaches is varied. In pure germinoma, 5-year disease-free survivals of 85%–100% can be expected. Radiotherapy alone for NGGCT produces only around 20% long-term survivors, but the introduction of platinum-based chemotherapy has improved this to around 50% or better, although radiation remains an important element of treatment in all patients. As in gonadal germ cell tumors, it is not uncommon to image residual abnormality in the tumor site following treatment. In the absence of evidence of progression, this may just need observation. However, if there is doubt concerning the completeness of treatment, surgery may be indicated. The treatment options for relapsed disease include chemotherapy for germinoma and high-dose chemotherapy with stem cell rescue for NGGCT.
Germ Cell Tumors of the Central Nervous System
Published in David A. Walker, Giorgio Perilongo, Roger E. Taylor, Ian F. Pollack, Brain and Spinal Tumors of Childhood, 2020
Matthew J. Murray, Ute Bartels, James C. Nicholson
Presentation is frequently delayed in intracranial GCTs, in comparison with other types of brain tumor.58,59 Germinoma, in particular, can be associated with indolent presentation and may manifest radiologically after several years of surveillance scanning following the development of DI. The diagnosis of germinoma should therefore be borne in mind in cases presenting with new onset of DI, which should lead to diagnostic workup including magnetic resonance imaging (MRI). Initial MRI may appear normal, or show only slight infundibular thickening, but in 15–25% of affected individuals follow-up imaging will demonstrate progression of findings, which ultimately requires biopsy and in most cases histological confirmation of the presence of germinoma.60,61
Pure endoscopic resection of pineal region tumors through supracerebellar infratentorial approach with ‘head-up’ park-bench position
Published in Neurological Research, 2023
Wei Hua, Hao Xu, Xin Zhang, Guo Yu, Xiaowen Wang, Jinsen Zhang, Zhiguang Pan, Wei Zhu
In our study, the mean preoperative tumor and cyst volumes was 19.2 ± 17.2 cm3. Two tumors (50%) were larger than 3 cm, and one was tenacious, and all of them underwent GTR. Pathological examination confirmed the diagnosis of two mixed germinomas, one glioblastoma multiforme, and one hemangioblastomas (recurrent). All patients experienced symptom resolution after surgery. In one patient, headache was only partially resolved. As his hydrocephalus was relieved and stable, no more intervention was needed. Three tumors invading the third ventricle, including one tenacious tumor, underwent GTR without complications. The follow-up period was 16.53 ± 1.06 months. One germinoma patient and one GBM patient underwent chemotherapy combined with radiotherapy, and the other germinoma patient underwent radiotherapy only. No signs of recurrence were reported during follow-up. The progression-free survival of the GBM patients was 18 months.
Radiological Latency in Pineal Germinoma: A Case Report and Literature Review
Published in Neuro-Ophthalmology, 2022
Ivan Chi Hin Siu, Noel Ching Yan Chan, Xian Lun Zhu, Ryan Pak to Yuen, Zhexi He, Danny Tat Ming Chan
The phenomenon of radiological latency is well documented in patients with suprasellar GCTs, but comparatively sparse in patients with GCTs of the pineal region. Our patient characterised this phenomenon; from presenting with isolated visual symptoms despite normal imaging findings, to progression of symptoms, development of radiological evidence 30 months later, and finally having a histologically proven germinoma. We believe that a high index of suspicion for a pineal GCT should be adopted in patients present with dorsal midbrain syndrome in spite of the absence of initial radiological evidence. It is also important for clinicians to note that this radiological latency can vary from months to years. A follow-up MRI protocol is mandatory, such as every 6 months for 2 years then yearly for 3 or more years for suspected cases, to facilitate earlier detection, and minimise the time to treatment in cases of pineal GCT.
Could Aberrant Migration Explain Metachronous Germ Cell Tumors?
Published in Cancer Investigation, 2021
Pierre Kubicek, Tanguy Fenouil, Julien Jacquemus, Olivia Chapuis, Aude Fléchon, Cécile Dumesnil, Cécile Faure-Conter
A man with no particular personal history and no family history of GCT was diagnosed at 14 years of age with a primary mediastinal pure seminoma. He underwent macroscopically complete removal of the tumor and received two and a half cycles of adjuvant chemotherapy according to the SFCE (Societé française des cancers et des leucémies de l’enfant)-TGM90 protocol (vinblastine 3 mg/m2 days 1-2; bleomycin 15 mg/m2 days 1–2; carboplatin 400 mg/m2 day 3; actinomycin 15 µg/kg days 22, 23, and 24; and cyclophosphamide 500 mg/m2 days 22, 23, and 24). Nineteen years later, he presented with binocular diplopia, with a tumor located in the pineal gland. Tumor markers (alpha-fetoprotein AFP and human chorionic gonadotrophin hCG) were negative in both the cerebrospinal fluid (CSF) and the serum. The CSF cytology and spinal MRI were also negative. The biopsy was consistent with the diagnosis of a pure germinoma. The seminoma cells exhibited strong positivity for CD117 and cytokeratin by immunohistochemistry (IHC). The treatment consisted of 24 Gy craniospinal irradiation with a 16 Gy focal boost to the pineal region, as per the SIOP GCT96 protocol (3). Seven years after the second tumor event, he is still in complete remission.