May 31, 2015 intracranial germ cell tumors icgct are rare in north america and europe, occurring in 24% of children with brain tumors between birth and 19 years of age 6,44,65. Patients with germcell tumours are managed by a diverse array of specialists. The natural history of primary intracranial germcell tumors gcts is defined from 389 previously published cases, of which 65% were germinomas, 18% teratomas, 5% embryonal carcinomas, 7% endodermal sinus tumors, and 5% choriocarcinomas. Firstly, we analyzed dna methylation status in 83 igcts, 3 seminomas and 6 normal control samples using infinium human methylation 450k beadchip array illumina, ca. Primary intracranial germ cell tumor originating from. Intracranial germ cell tumours are rare tumours affecting mainly male adolescents, mainly in asia. Germ cell brain tumors are tumors, usually cancerous, that arise in the pineal or suprasellar region of the brain. The management of intracranial germcell tumours is complex because of varied clinical presentations, tumour sites, treatments and outcomes, and the need for multidisciplinary input.
A germ cell tumor gct is a neoplasm derived from germ cells. This kind of mixed germ cell tumor may be known simply as a teratoma with elements of embryonal carcinoma or. Pdf primary intracranial germcell tumors in adults. Singlecenter experience with 42 clinically diagnosed cases qun ying yang, cheng cheng guo, mei ling deng, jian wang, jing wang, fu hua lin, ji zhang, xiao bing jiang, yong gao mou, zhong ping chen. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. Impact of margin for target volume in lowdose involved field radiotherapy after induction chemotherapy for. Patients with germ cell tumours are managed by a diverse array of specialists. Extragonadal germ cell college of american pathologists. However, a germ cell brain tumor can spread to other parts of. We have collected over 200 fresh frozen tissue samples of igcts through the intracranial germ cell tumor genome analysis consortium in japan. Refer to the pdq summary on childhood central nervous system germ cell tumors treatment for information about the treatment of intracranial gcts. Thus, staging, risk stratification, and treatment approaches for.
The patient was transferred to the endocrinology department of the same hospital in order to test the efficiency of the pituitary hormones. Felix i, becker le 1990 intracranial germ cell tumors in children. After that the patient was diagnosed with primary germ cell intracranial tumor. Teratocarcinoma refers to a germ cell tumor that is a mixture of teratoma with embryonal carcinoma, or with choriocarcinoma, or with both. Intracranial germ cell tumors are seen mainly in adolescents and young adults.
Fifth international central nervous system germ cell tumor. Nongerminomatous germ cell tumors nggct make up one third of intracranial germ cell tumor and consist of several different subtypes endodermal sinus tumor yolk sac tumor, embryonal carcinoma,choriocarcinoma, teratoma and germ cell tumors of. Primary intracranial germ cell tumor originating from septum. Coexistence, intracranial germ cell tumor, craniopharyngioma 214 int j clin exp med 20. Consensus on the management of intracranial germcell.
The intracranial group consisted of three germinomas, two mature teratomas and one mixed germ cell tumor. Improved prognosis of intracranial nongerminoma germ cell. A ct scan shows a slightly highdensity mass in the pineal region, together with a cyst and some calcifications. By contrast, icgct is the most common supratentorial brain tumor in japan, taiwan, and korea, accounting for 1115% of brain tumors in children and adolescents 14, 30, 49, 67. Pdf treatment of intracranial germ cell tumors suzanne. The diagnosis of an intracranial germ cell tumor usually requires histological information, but a subgroup of tumors will secrete specific tumor markers, including fetoprotein and human chorionic. Thus, an accurate diagnosis is vital for patient counselling and treatment planning. Primary chemotherapy for intracranial germ cell tumors.
These cells later become sperm in the testicles or eggs in the ovaries this summary is about germ cell tumors that form in parts of the body that are extracranial outside the brain. Thirtysix tumors arose in the pineal region, 14 were suprasellar, and 11 in the basal ganglia and thalamus. The treatment for each type of germ cell tumor is different. Pineal region tumor germ cell tumor most common pineoblastoma pineocytoma pineal parenchymal tumor of intermediate differentiation meningioma ependymoma central neurocytoma metastasis benign cyst suprasellar region tumor pituitary adenoma most common germ cell tumor craniopharyngioma. Ct of pineal tumors and intracranial germcell tumors. Pdf primary intracranial germ cell tumors researchgate. Paediatric extracranial germcell tumours sciencedirect. Thus, staging, risk stratification, and treatment approaches for germ cell. Germ cell tumor, germinoma, incidence, management, surgery. Within the classification of intracranial germ cell tumors, there are a variety of different tumor types which carry different prognoses. Hoyoux 3 germ cell tumours represent about 3 to 8% of pediatric brain tumours. Primary mediastinal germ cell tumor the mediastinum is the most common site of extragonadal germ cell tumors 11, constituting 5070% of all extragonadal germ cell tumors.
Germinoma, teratoma choriocarcinoma, embryonal carcinoma, yolk sac tumor, and malignant mixed germ cell tumors mmgcts. The diagnosis of an intracranial germ cell tumor usually requires histological information, but a subgroup of tumors will secrete specific tumor markers. Nongerminomatous germ cell tumors secrete chemicals into the spinal fluid and bloodstream and require more intensive treatment than pure germ cell tumors. Intracranial germ cell tumors are a heterogeneous group of lesions which arise in patients of all ages. Primary intracranial germ cell tumors gcts represent 35% of all central nervous system cns tumors. The majority of intracranial germ cell tumors arise in structures around the third ventricle, most commonly in the. Primary intracranial germ cell tumors pubmed central pmc. Pdf primary intracranial germcell tumors are rare tumors primarily of adolescence, and literature on this disease in adults is scarce. Among these, a common form is teratoma with endodermal sinus tumor. Intracranial germ cell tumors are a heterogeneous group of lesions which occur in children and adults.
Intracranial germ cell tumors icgct are rare in north america and europe, occurring in 24% of children with brain tumors between birth and 19 years of age 6,44,65. It is common for several of these elements to be found within a single tumor specimen, and these mixed tumors are more common than pure forms 4,5. Intracranial germ cell tumors gct represent 311% of pediatric and 1% of adult brain tumors. Nongerminomatous germ cell tumors nggct make up one third of intracranial germ cell tumor and consist of several different subtypes endodermal sinus tumor yolk sac tumor, embryonal carcinoma,choriocarcinoma, teratoma and germ cell tumors of mixed origin termed mixed germ cell tumors. Intracranial germ cell tumors in children with and without. Procedure biopsy consider extragonadal germ cell biopsy protocol tumor type testicular germ cell tumors consider the testis protocol. Management of paediatric extracranial germ cell tumours carries a unique set of challenges. Treatment of primary intracranial germ cell tumors.
Results one patient survived 3 years without radiologic. Novel somatic and germline mutations in intracranial germ. The classification of gcts includes germinoma, teratoma, yolk sac tumor, embryonal carcinoma, and choriocarcinoma. The latter group is particularly heterogeneous and includes the malignant subtypes yolk sac tumor yst and choriocarcinoma chc which secrete the tumor markers alpha. Apr 30, 2017 intracranial germ cell tumors gcts are a rare, heterogeneous cancer group and first.
Primary intracranial germ cell tumor originating from septum pellucidum that mimics central neurocytoma intracranial germ cell tumors gcts, representing 2% to 3% of primary intracranial tumors, are rare neoplasms affecting children and young adults. Paediatric extracranial germcell tumours the lancet. Treatment and outcomes of uk and german patients with. Intracranial germ cell tumours gcts are rare tumours of childhood and adolescence with. Mixed tumors mainly composed of choriocarcinoma, yolk sac tumor, or embryonal carcinoma classi. Intracranial germ cell tumors make up approximately 0. Of the intracranial germ cell tumors, germinoma is the most common and is cured the most readily by radiotherapy, with 5. Protocol for the examination of resection specimens from. These were treated by radical surgical resection and chemotherapy with cisplatin. Diagnostic imaging of intracranial germ cell tumors.
Intracranial germ cell tumors igcts are a group of brain tumors with extraordinary characteristics. Germ cell tumours are a heterogeneous group of neoplasms that present across a wide age range and vary in site, histology, and clinical behaviour. A ventricular drainage tube is inserted into the posterior horn of the right lateral ventricle. Germ cell tumors includes pediatric and adult patients with germ cell tumors located in the mediastinum, sacrococcygeal area, retroperitoneum, and neck the following should not be reported using this protocol. Childhood extracranial germ cell tumors form from germ cells in parts of the body other than the brain. This includes the testicles, ovaries, sacrum lower part of the spine, coccyx tailbone, mediastinum area between the lungs, retroperitoneum the back wall of the abdomen, and the head and neck. Intracranial germ cell tumors icgct are rare, biologically diversified, and diagnostically challenging tumors. Most pineal tumors occurred in the first three decades of life, with exception pineocytomas which were seen at a mean age of 34. Comparative genomic profiling analysis was carried out using microarraycgh method. Germ cells normally occur inside the gonads ovary and testis.
Intracranial germ cell tumors usually present as discrete enhancing masses, often containing calcification, and usually are associated with hydrocephalus. Comparative genomic profiling analysis was carried out. Primary chemotherapy for intracranial nongerminomatous germ. While germinoma and teratoma are frequently encountered as pure tumor types, other intracranial germ cell neoplasms are often of mixed histologic composition. Germcell tumours are a heterogeneous group of neoplasms that present across a wide age range and vary in site, histology, and clinical behaviour.
Intracranial germ cell tumour the royal marsden nhs. Pdf primary intracranial germ cell tumors shagufta. The diagnosis of an intracranial germ cell tumor usually requires histological information, but a subgroup of tumors will secrete specific tumor. Understanding the treatment strategies of intracranial germ. Due to the morbidity associated with surgery of pineal region tumors and the variability in classification schemas utilized to characterize such lesions, the exact incidences of specific types of germ cell tumors are difficult to determine. Case report coexistence of intracranial germ cell tumor. There are two main types of intracranial germ cell tumour. Treatment and prognosis of patients with intracranial. The natural history of primary intracranial germ cell tumors gcts is defined from 389 previously published cases, of which 65% were germinomas, 18% teratomas, 5% embryonal carcinomas, 7% endodermal sinus tumors, and 5% choriocarcinomas.
They can spread to the cerebrospinal fluid csf surrounding the brain and spine. Malignant intracranial germ cell tumors gcts are a rare group of predominantly midline neoplasms, 1 often classified into germinoma or non. Different types of these germ cells cause different types of tumors, including the following. These tumors start and grow mainly in two areas in the center of the brain. Intracranial gcts comprise two distinct histologic groupings. Germinoma, teratoma choriocarcinoma, embryonal carcinoma, yolk sac. Germinoma, teratoma choriocarcinoma, embryonal carcinoma, yolk sac tumor, and malignant mixed germ cell tumors. Childhood gcts are rare in children younger than 15 years, accounting for approximately 3% of cancers in this age group. Intracranial germ cell tumors igct are rare malignancies that account for only 3 % of brain tumors in children in the united states. Intracranial germ cell tumor in the molecular era ncbi. Childhood extracranial germ cell tumors treatment pdq.
Approximately twothirds of intracranial gcts are germinomas and the remaining onethird are nongerminomatous germ cell tumors nggcts. A germ cell is a type of cell that forms as a fetus unborn baby develops. Protocol for the examination of resection specimens from patients with extragonadal germ cell tumors. Apr 27, 2012 the nongerminomatous germ cell tumours nggct.
Radiation therapy for intracranial germ cell tumors. P rimary intracranial germ cell tumors are uncommon malignancies in the central nervous system, making up less than 1% of all intracranial neoplasms. Our patient series included 48 males and females with an age range of 5 to 41 years mean, 15 years. Intracranial germ cell tumors in children with and without d. By contrast, icgct is the most common supratentorial brain tumor in japan, taiwan, and korea, accounting for 1115% of brain tumors in children and adolescents 14,30,49,67. Pluripotent embryonic cells mismigrate and give rise to gcts. Participants of the 20 third international cns germ cell tumour symposium cambridge, uk agreed to undertake a multidisciplinary delphi process to identify consensus in the clinical management of intracranial. Another type of germ cell tumour which can be either benign or malignant is known as a teratoma. Pdf primary intracranial germ cell tumors are rare icgcts and usually localized in the pineal and suprasellar regions of the brain. Participants of the 20 third international cns germ cell tumour symposium cambridge, uk agreed to undertake a multidisciplinary delphi process to identify consensus in the clinical.
Incidence of intracranial germ cell tumors by race in the united states, 19922010. Enlarge extracranial germ cell tumors form in parts of the body other than the brain. A male preponderance was noted in the various pineal tumors, except for pineocytomas. Primary chemotherapy for intracranial nongerminomatous. Adult patients with extragonadal germ cell tumors protocol applies to germ cell tumors located in the mediastinum, sacrococcygeal area, retroperitoneum, neck, and intracranial sites.
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