Clinical management of primary central nervous system germ cell tumors
Section snippets
Histological classification
According to the World Health Organization (WHO)3 classification of tumors of the nervous system, germ cell tumors are categorized into five basic types (germinomas, teratomas, choriocarcinomas, yolk sac or endodermal sinus tumors, and embryonal carcinomas) and their mixture (mixed germ cell tumor).
Germinomas are composed of large polygonal cells with a pale eosinophilic or clear cytoplasms, and small lymphocytes. Their cytoplasm stains positive for placental alkaline phosphatase (PLAP), and
Germinoma
As germinoma may disseminate throughout the CSF pathways, this tumor has traditionally been treated with surgical biopsy followed by prophylactic craniospinal irradiation with a boost to local disease, resulting in excellent 10-year survival rates (>80%). However, with modern imaging procedures, the proportion of patients presenting with metastatic disease at the time of diagnosis is low, and the risk of secondary seeding outside the irradiated volume does not exceed 12% in histologically
Ongoing phase II or phase III studies for intracranial germ cell tumors
Despite excellent long-term results for patients with germinoma treated with radiation therapy, the potential for late effects makes the treatment controversial. On the other hand, most patients with non-germinomatous tumors treated by conventional treatment with surgery and radiation therapy failed to survive longer than 3 years. Today several prospective phase II or phase III studies are being investigated to assess the effect of combination chemotherapy and radiation therapy for germ cell
Summary of treatment results and future prospects
Based on our analysis of treatment outcomes obtained in the Tokyo University series,4, 27 we divided patients with intracranial germ cell tumors into three therapeutic groups with a good, intermediate, and poor prognosis (Table 4). The first step should be histological verification of surgical specimens and, where possible, extensive reduction of tumor mass. In Japan, due to advances in microsurgical techniques and radioimaging techniques, surgery for pineal tumors is now much safer, and the
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Cited by (52)
Socio-behavioral dysfunction in disorders of hypothalamic-pituitary involvement: The potential role of disease-induced oxytocin and vasopressin signaling deficits
2022, Neuroscience and Biobehavioral ReviewsCitation Excerpt :Tumor localization plays a large role in the likelihood of a patient developing DI. One study showed that while 86% of patients with neurohypophyseal tumors developed DI, only three percent of those with pineal lesions developed the condition (Matsutani, 2004). Surgical intervention may also cause alterations in AVP signaling.
Multimodality treatment for Central Nervous System Germ Cell Tumors: Disease spectrum and management strategies - A tertiary care center experience from India
2021, Clinical Neurology and NeurosurgeryCitation Excerpt :In present study, headache, visual abnormalities, and endocrine abnormalities were the presenting symptoms in 64.3 %, 53.6 %, and 17.3 % of patients, respectively. Pineal region followed by suprasellar region are the most common sites of CNS GCT [14,21], as also seen in present study. Other rare locations are hypothalamus, thalamus, brain stem, basal ganglia, and periventricular area around 3rd/4th ventricle [21].
A comparative study of the extent of cerebral microvascular injury following whole-brain irradiation versus reduced-field irradiation in long-term survivors of intracranial germ cell tumors
2015, Radiotherapy and OncologyCitation Excerpt :A radiation field encompassing the whole brain is still widely used [4–6,29] because local irradiation alone to the tumor has been associated with an unacceptable relapse rate of about 15–40% [6,8,30]; most of these reported relapses developed in periventricular areas outside the radiation field [8]. To balance the trade-off between optimal treatment outcome and long-term adverse effects, chemotherapy followed by reduced-dose (24-Gy) reduced-field (whole-ventricle) irradiation was proposed by the Japanese Pediatric Brain Tumor Study Group [25,31]. With this approach, most relapses were avoided [7,8] and no difference was found in the 10-year overall survival rate following whole-ventricle irradiation as compared with whole-brain irradiation [7].
Primary intramedullary spinal germ cell tumors
2011, World NeurosurgeryPrecocious puberty due to secreting chorionepithelioma (Teratoma) of the brain
2011, Journal of PediatricsLong-term follow-up of dose-adapted and reduced-field radiotherapy with or without chemotherapy for central nervous system germinoma
2010, International Journal of Radiation Oncology Biology PhysicsCitation Excerpt :Patients with multifocal intracranial germinoma or tumor invasion of brain parenchyma appear to have higher risk of out-of-field recurrence, although our sample size was too small to show a statistically significant correlation. Other reports have given mixed results about the risk of locoregional failure for such patients (31–33). Current COG protocol requires that CSA RT be used for patient subsets deemed to be at increased risk: those with multifocal tumors, pineal tumors with endocrinopathic findings consistent with suprasellar involvement, or thalamic or basal ganglia primary tumors (16).