Intracranial germ cell tumors make up approximately 0. Refer to the pdq summary on childhood central nervous system germ cell tumors treatment for information about the treatment of intracranial gcts. The pathology showed gcrminoma and yolk sac tumor, respectively. P rimary intracranial germ cell tumors are uncommon malignancies in the central nervous system, making up less than 1% of all intracranial neoplasms. Intracranial germ cell tumors are a heterogeneous group of lesions which occur in children and adults. Adult patients with extragonadal germ cell tumors protocol applies to germ cell tumors located in the mediastinum, sacrococcygeal area, retroperitoneum, neck, and intracranial sites. Primary intracranial germ cell tumors gcts represent 35% of all central nervous system cns tumors.
Germ cell brain tumors are tumors, usually cancerous, that arise in the pineal or suprasellar region of 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. 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 tumours are rare tumours affecting mainly male adolescents, mainly in asia. 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. Treatment and outcomes of uk and german patients with.
Paediatric extracranial germcell tumours sciencedirect. Case report coexistence of intracranial germ cell tumor. Procedure biopsy consider extragonadal germ cell biopsy protocol tumor type testicular germ cell tumors consider the testis protocol. Intracranial germ cell tumors in children with and without. The latter group is particularly heterogeneous and includes the malignant subtypes yolk sac tumor yst and choriocarcinoma chc which secrete the tumor markers alpha. The treatment for each type of germ cell tumor is different. Our patient series included 48 males and females with an age range of 5 to 41 years mean, 15 years.
Thus, an accurate diagnosis is vital for patient counselling and treatment planning. Germinoma, teratoma choriocarcinoma, embryonal carcinoma, yolk sac. Intracranial gcts comprise two distinct histologic groupings. Impact of margin for target volume in lowdose involved field radiotherapy after induction chemotherapy for. Intracranial germ cell tumours gcts are rare tumours of childhood and adolescence with. The diagnosis of an intracranial germ cell tumor usually requires histological information, but a subgroup of tumors will secrete specific tumor. Intracranial germ cell tumor in the molecular era ncbi. Childhood extracranial germ cell tumors treatment pdq. Intracranial germ cell tumors are a heterogeneous group of lesions which arise in patients of all ages. 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. Childhood extracranial germ cell tumors form from germ cells in parts of the body other than the brain. Thirtysix tumors arose in the pineal region, 14 were suprasellar, and 11 in the basal ganglia and thalamus. Approximately twothirds of intracranial gcts are germinomas and the remaining onethird are nongerminomatous germ cell tumors nggcts. Pluripotent embryonic cells mismigrate and give rise to gcts.
Diagnostic imaging of intracranial germ cell tumors. Primary intracranial germ cell tumors are rare icgcts and usually localized in the pineal and suprasellar regions of the brain. Protocol for the examination of resection specimens from patients with extragonadal germ cell tumors. A ventricular drainage tube is inserted into the posterior horn of the right lateral ventricle. While germinoma and teratoma are frequently encountered as pure tumor types, other intracranial germ cell neoplasms are often of mixed histologic composition. A male preponderance was noted in the various pineal tumors, except for pineocytomas. Hoyoux 3 germ cell tumours represent about 3 to 8% of pediatric brain tumours. 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. Teratocarcinoma refers to a germ cell tumor that is a mixture of teratoma with embryonal carcinoma, or with choriocarcinoma, or with both.
Within the classification of intracranial germ cell tumors, there are a variety of different tumor types which carry different prognoses. Patients with germcell tumours are managed by a diverse array of specialists. Most pineal tumors occurred in the first three decades of life, with the exception of pineocytomas, which were seen at a mean age of 34. Primary chemotherapy for intracranial nongerminomatous germ. Understanding the treatment strategies of intracranial germ.
We have collected over 200 fresh frozen tissue samples of igcts through the intracranial germ cell tumor genome analysis consortium in japan. This kind of mixed germ cell tumor may be known simply as a teratoma with elements of embryonal carcinoma or. The majority of intracranial germ cell tumors arise in structures around the third ventricle, most commonly in the. Intracranial germ cell tumors are seen mainly in adolescents and young adults. These tumors start and grow mainly in two areas in the center of the brain. Pdf primary intracranial germ cell tumors are rare icgcts and usually localized in the pineal and suprasellar regions of the brain. Management of paediatric extracranial germ cell tumours carries a unique set of challenges.
Comparative genomic profiling analysis was carried out using microarraycgh method. Felix i, becker le 1990 intracranial germ cell tumors in children. Radiation therapy for intracranial germ cell tumors. Consensus on the management of intracranial germcell.
Treatment of primary intracranial germ cell tumors. Malignant intracranial germ cell tumors gcts are a rare group of predominantly midline neoplasms,1 often classi. Improved prognosis of intracranial nongerminoma germ cell. Pdf treatment of intracranial germ cell tumors suzanne. 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. Germinoma, teratoma choriocarcinoma, embryonal carcinoma, yolk sac tumor, and malignant mixed germ cell tumors.
Pdf primary intracranial germcell tumors are rare tumors primarily of adolescence, and literature on this disease in adults is scarce. Paediatric extracranial germcell tumours the lancet. 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 tumors mainly composed of choriocarcinoma, yolk sac tumor, or embryonal carcinoma classi. 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. There are two main types of intracranial germ cell tumour. Of the intracranial germ cell tumors, germinoma is the most common and is cured the most readily by radiotherapy, with 5. Intracranial germ cell tumors icgct are rare, biologically diversified, and diagnostically challenging tumors. 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. 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. Pdf primary intracranial germ cell tumors researchgate. Comparative genomic profiling analysis was carried out. Intracranial germ cell tumors igcts are a group of brain tumors with extraordinary characteristics.
Extragonadal germ cell college of american pathologists. Novel somatic and germline mutations in intracranial germ. The diagnosis of an intracranial germ cell tumor usually requires histological information, but a subgroup of tumors will secrete specific tumor markers. Ct of pineal tumors and intracranial germcell tumors. The management of intracranial germ cell tumours is complex because of varied clinical presentations, tumour sites, treatments and outcomes, and the need for multidisciplinary input. Primordial germ cells mismigrate during embryonic development and subsequently undergo malignant transformation. Thus, staging, risk stratification, and treatment approaches for. 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.
Primary chemotherapy for intracranial germ cell tumors. Pdf primary intracranial germ cell tumors shagufta. Intracranial germ cell tumors in children with and without d. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. Enlarge extracranial germ cell tumors form in parts of the body other than the brain. Different types of these germ cells cause different types of tumors, including the following. Patients with germ cell tumours are managed by a diverse array of specialists. A ct scan shows a slightly highdensity mass in the pineal region, together with a cyst and some calcifications. 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. 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. 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. Management of paediatric extracranial germcell tumours carries a unique set of challenges. Results one patient survived 3 years without radiologic. Malignant intracranial germ cell tumors gcts are a rare group of predominantly midline neoplasms, 1 often classified into germinoma or non.
Germ cell tumor, germinoma, incidence, management, surgery. A germ cell is a type of cell that forms as a fetus unborn baby develops. Apr 30, 2017 intracranial germ cell tumors gcts are a rare, heterogeneous cancer group and first. Nongerminomatous germ cell tumors secrete chemicals into the spinal fluid and bloodstream and require more intensive treatment than pure germ cell tumors. The patient was transferred to the endocrinology department of the same hospital in order to test the efficiency of the pituitary hormones.
Among these, a common form is teratoma with endodermal sinus tumor. Incidence of intracranial germ cell tumors by race in the united states, 19922010. Germ cell tumours are a heterogeneous group of neoplasms that present across a wide age range and vary in site, histology, and clinical behaviour. After that the patient was diagnosed with primary germ cell intracranial tumor. Coexistence, intracranial germ cell tumor, craniopharyngioma 214 int j clin exp med 20. Intracranial germ cell tumors igct are rare malignancies that account for only 3 % of brain tumors in children in the united states. Childhood gcts are rare in children younger than 15 years, accounting for approximately 3% of cancers in this age group. Germ cells normally occur inside the gonads ovary and testis. Apr 27, 2012 the nongerminomatous germ cell tumours nggct. Primary intracranial germ cell tumor originating from. Intracranial germ cell tumors gct represent 311% of pediatric and 1% of adult brain tumors.
Protocol for the examination of resection specimens from. Pineal and suprasellar regions are the most frequent intracranial sites. There are several subtypes of nongerminomatous germ cell tumor, including teratomas, choriocarcinomas, endodermal sinus tumors yolk sac tumors, embryonal carcinomas and mixed tumors. The classification of gcts includes germinoma, teratoma, yolk sac tumor, embryonal carcinoma, and choriocarcinoma. A germ cell tumor gct is a neoplasm derived from germ cells. The management of intracranial germcell tumours is complex because of varied clinical presentations, tumour sites, treatments and outcomes, and the need for multidisciplinary input. Thus, staging, risk stratification, and treatment approaches for germ cell. However, a germ cell brain tumor can spread to other parts of.
Treatment and prognosis of patients with intracranial. They can spread to the cerebrospinal fluid csf surrounding the brain and spine. Understanding the treatment strategies of intracranial. Primary chemotherapy for intracranial nongerminomatous. 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. Primary intracranial germ cell tumors pubmed central pmc. These were treated by radical surgical resection and chemotherapy with cisplatin. 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 constitute nearly 24,5 to 3% of pediatric brain tumors, and germinomas alone account for approximately two. Intracranial germ cell tumour the royal marsden nhs. 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. 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.
Another type of germ cell tumour which can be either benign or malignant is known as a teratoma. Intracranial germ cell tumors usually present as discrete enhancing masses, often containing calcification, and usually are associated with hydrocephalus. Primary intracranial germ cell tumor originating from septum. 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. Germinoma, teratoma choriocarcinoma, embryonal carcinoma, yolk sac tumor, and malignant mixed germ cell tumors mmgcts. 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. Consensus on the management of intracranial germcell tumours.
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. The intracranial group consisted of three germinomas, two mature teratomas and one mixed germ cell tumor. 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. Most pineal tumors occurred in the first three decades of life, with exception pineocytomas which were seen at a mean age of 34. Fifth international central nervous system germ cell tumor.
1048 569 1333 778 185 961 535 61 431 450 1155 1367 420 137 655 663 988 865 1343 1129 859 1268 814 618 641 179 31 1128 1222 805 274 744 103 589 1055 878 375 521 441 48 628 306