Primary intracranial germ-cell tumors in adults: a practical review

Bromberg, Jacoline E C; Baumert, Brigitta G.; Vos, Filip Y.F. De; Gijtenbeek, J.M.M.; Kurt, Erkan; Westermann, Anneke M.; Wesseling, Pieter

doi:10.1007/s11060-013-1114-6

Cited by 25 publications

(21 citation statements)

References 58 publications

(99 reference statements)

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“…MRI demonstrates soft tissue masses with isointense or slightly hyperintense signals in T1 weighted images, which may be accompanied with calcification or cyst formation in T2 weighted images (22). Definitive diagnosis of GCT is performed through the histopathology approach: The majority of GCTs demonstrate immunohistochemical staining for placenta-like alkaline phophatase and c-Kit, otherwise known as CD117, which is an important mitogen for normal germ cells (11). Unfortunately, a biopsy is not possible for patients where the tumor location is inaccessible.…”

Section: Discussionmentioning

confidence: 99%

β-human chorionic gonadotropin-secreting intracranial germ-cell tumor associated with high testosterone in an adult man: A case report

2017

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Abstract.A 38-year-old male patient presented with general weakness, polydipsia and a body weight loss of 10 kg in two years. Hypopituitarism with central hypothyroidism and central adrenal insufficiency were noted at Taipei City Hospital (Taipei, Taiwan). However, hypogonadotropic hypergonadism was also observed. The patient was diagnosed with an intracranial β-human chorionic gonadotropin (β-hCG) secreting germ-cell tumor, and brain magnetic resonance imaging revealed that the tumor involved the pineal gland, stalk, posterior pituitary gland, right basal ganglion, hypothalamus, corpus callosum and posterior hippocampus. The cerebrospinal fluid (CSF) β-hCG level was 1936 IU/l, while the α-fetoprotein (AFP) level was <0.24 ng/ml. The serum AFP level of the patient was 3.28 ng/ml, and the β-hCG level was 178 IU/l with a CSF:serum β-hCG ratio >2:1. The patient was successfully treated with chemotherapy and radiotherapy, as demonstrated by a marked decrease in size of the tumor and in the serum β-hCG levels. Intracranial β-hCG secreting germ-cell tumors are rare in adults and manifest differently compared with patients of early pubertal age. In contrast with the precocious puberty frequently observed in young patients, the diagnosis of adult patients is often delayed and the symptoms are associated with tumor size and location. The present case report described an adult male with an intracranial β-hCG secreting GCT, demonstrating hypopituitarism and asymptomatic hyperandrogenemia, and reviews and discusses the literature relevant to the case. IntroductionPrimary intracranial germ-cell tumors (GCT) are rare tumors. They account for ~0.5-3% of all pediatric primary central nervous system (CNS) tumors in Western regions, but are observed at higher frequencies among pediatric CNS tumors in Asia, accounting for up to 10% (1). The incidence in the Far East area of Japan is 0.1-0.17 per 100,000 per year, according to the Brain Tumor Registry of Japan (2), slightly higher than the incidence rate of 0.1 per 100,000 per year in the United States (3). Primary intracranial GCT typically occurs in children or young adults, with the majority of patients (60-70%) aged under 20 (4). The peak incidence of intracranial germ-cell tumors is in the early pubertal period with a median age of diagnosis at 10-12 years (5). The disease occurs primarily in males, with the ratio of male to female between 2:1 and 3:1 (6). The World Health Organization had classified intracranial germ-cell tumors into three groups as follows: Germinomas, non-germinomatous germ-cell tumors (NGGCTs) and mixed germ-cell tumors (7). The diseases are heterogeneous in terms of histology, tumor characteristics, treatment response and tumor marker secretion. Among NGGCTs, choriocarcinoma secretes β-human chorionic gonadotropin (β-HCG) into the serum and/or the cerebrospinal fluid (CSF), with high levels detected, while yolk sac tumors secrete α-fetoprotein (AFP). Elevation of AFP levels, combined with characteristic magnetic resonance imaging (MRI) results, is diagnos...

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Section: Discussionmentioning

confidence: 99%

β-human chorionic gonadotropin-secreting intracranial germ-cell tumor associated with high testosterone in an adult man: A case report

2017

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“…[1] However, the immunohistochemical expression of HCG-β had been detected in various tumors other than GCTs, including cystic pituitary adenomas, Rathke's cleft cysts, and craniopharyngiomas. [2310] In this case, we could not distinguish a craniopharyngioma from a GCT by the detection of HCG-β in the CSF.…”

Section: Discussionmentioning

confidence: 99%

A craniopharyngioma with spontaneous involution of a gadolinium-enhanced region on magnetic resonance imaging

et al. 2014

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Background:It has not been reported previously that the solid enhancing portion of a craniopharyngioma has involuted without a change in cyst size.Case Description:We herein report a case of a craniopharyngioma with spontaneous involution of a solid gadolinium (Gd)-enhanced region on magnetic resonance imaging (MRI). A 44-year-old female was referred to our department with a mass on MRI associated with headaches and polyuria. The images showed a suprasellar cystic lesion progressing from the posterior pituitary lobe to the right hypothalamus along the pituitary stalk. Examinations of the cerebrospinal fluid showed aseptic meningitis and a positive titer of beta-human chorionic gonadotropin (HCG-β) preoperatively. The hypothalamic lesion became enlarged over the following 3 weeks, and a biopsy of the posterior pituitary lobe was performed via the endonasal transsphenoidal approach under a preoperative diagnosis of a germ cell tumor (GCT). The histological diagnosis was a craniopharyngioma, and the patient's postoperative findings on MRI were atypical: The solid Gd-enhanced region in the hypothalamus had spontaneously decreased in size and the peritumoral edema had improved, although the biopsy site in the posterior pituitary lobe was distant from the area of shrinkage. We speculated that the involutional portion on MRI mimicking a tumor was actually the normal hypothalamus, which was abnormally enhanced due to a disruption of the blood-brain barrier caused by the craniopharyngioma.Conclusion:Gd-enhanced regions of parenchyma neighboring the cysts of craniopharyngioma should be carefully managed, taking into consideration the possibility of the enhancement of normal tissue.

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“…Studies of intensive chemotherapy for malignant intracranial germ cell tumor have shown that NGGCT is highly responsive to chemotherapy and demonstrate a 5-year overall survival rate (OS) of nearly 60%-75% for patients treated with chemotherapy without irradiation at initial therapy (most of the patients were subsequently treated with radiotherapy); nevertheless, chemotherapy alone results in a higher relapse rate than chemotherapy combined with radiotherapy, indicating that irradiation should be included in treatment regimens. 4,6 Bromberg et al 3 reviewed the recent literature for intracranial germ cell tumor and reported that radiotherapy combined with platinum-based chemotherapy was effective and should represent the current standard for intracranial NGGCT. Neoadjuvant chemoradiotherapy is also an effective strategy, as Kochi et al 8 treated 11 patients with NGGCT using neoadjuvant chemotherapy with platinum-based agents (CE, PE, or ICE) and radiotherapy and reported that the 5-year progression-free survival rate and 5-year OS were both 90.9%.…”

Section: Chemotherapymentioning

confidence: 99%

Successful treatment of mixed yolk sac tumor and mature teratoma in the spinal cord: case report

Mukasa¹,

Yanagisawa²,

Tanaka³

et al. 2017

SPI

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Primary spinal germ cell tumors are rare, and spinal nongerminomatous germ cell tumors represent an even rarer subset for which no standard therapy has been established. The authors report the case of a 24-year-old woman with multifocal primary spinal germ cell tumors scattered from T-12 to L-5 that consisted of yolk sac tumor and mature teratoma. After diagnostic partial resection, the patient was treated with 30 Gy of craniospinal irradiation and 30 Gy of local spinal irradiation, followed by 8 courses of chemotherapy based on ifosfamide, cisplatin, and etoposide (ICE). Salvage surgery was also performed for residual mature teratoma components after the third course of ICE chemotherapy. Chemotherapy was continued after the operation, but ifosfamide was entirely eliminated from the ICE regimen because severe myelosuppression was observed after previous courses. The patient remains recurrence free as of more than 5 years after the completion of chemotherapy. This case suggests that this treatment strategy is an effective option for primary spinal yolk sac tumor.

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Primary intracranial germ-cell tumors in adults: a practical review

Cited by 25 publications

References 58 publications

β-human chorionic gonadotropin-secreting intracranial germ-cell tumor associated with high testosterone in an adult man: A case report

β-human chorionic gonadotropin-secreting intracranial germ-cell tumor associated with high testosterone in an adult man: A case report

A craniopharyngioma with spontaneous involution of a gadolinium-enhanced region on magnetic resonance imaging

Successful treatment of mixed yolk sac tumor and mature teratoma in the spinal cord: case report

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