Germ cell tumours of the central nervous system in children—controversies in radiotherapy

Borg, Martin

doi:10.1002/mpo.10285

Cited by 31 publications

(18 citation statements)

References 56 publications

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“…There is good evidence in the literature describing the progressive linear decline in IQ points among patients receiving whole brain and craniospinal irradiation, ranging from 2 to 5 points per year after completion of radiotherapy . In recent years, significant effort has been focused on therapeutic strategies that limit cranial irradiation, especially in localized germinomas …”

Section: Discussionmentioning

confidence: 99%

Clinical and neuropsychological outcome of pediatric non‐midline central nervous system germinoma treated with chemotherapy and reduced dose/volume irradiation: The Children's Hospital Los Angeles experience

Yeo

Kayser

Margol

et al. 2019

Pediatric Blood & Cancer

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Background Germ cell tumors (GCT) arising from non‐midline structures (basal ganglia, thalamus, and posterior fossa) are rare. Although patients with midline (pineal and suprasellar) germinoma have excellent survival with chemotherapy and whole ventricular irradiation (WVI), germinoma in non‐midline locations have traditionally been treated with craniospinal irradiation (CSI) or whole brain irradiation (WBI) to achieve similar outcomes. However, CSI and WBI are associated with significant long‐term neuropsychological sequelae. Methods We describe the clinical and neuropsychological outcomes of patients with non‐midline germinoma treated at the Children's Hospital Los Angeles between 1990 and 2015. Results Nine patients had basal ganglia/thalamic germinoma and one patient had a cerebellar primary. Eight patients received chemotherapy followed by reduced dose/volume irradiation, whereas two patients received chemotherapy alone as upfront therapy. One patient in the chemotherapy alone group relapsed after 4.3 years and was salvaged with CSI plus boost. The overall survival for the entire cohort was 100% at a median follow‐up of 8.5 years. Neuropsychological data were available for six patients at a median of five months (baseline) and 4.2 years (follow‐up) post‐diagnosis. At four‐year follow‐up, data available revealed intact overall cognitive ability, verbal memory, and executive functioning, but persistent deficits in fine motor function. Comparison of baseline to follow‐up suggests a downward trend in working memory, planning/problem‐solving, verbal memory, and visuospatial integration. Conclusion Chemotherapy followed by reduced dose/volume of irradiation is an effective strategy resulting in long‐term survival in patients with non‐midline germinoma. Neuropsychological data suggest relatively minimal morbidity over time.

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Section: Discussionmentioning

confidence: 99%

Clinical and neuropsychological outcome of pediatric non‐midline central nervous system germinoma treated with chemotherapy and reduced dose/volume irradiation: The Children's Hospital Los Angeles experience

Yeo

Kayser

Margol

et al. 2019

Pediatric Blood & Cancer

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“…Pure germinomas are extremely radiosensitive and are successfully cured in over 90% of cases by irradiation alone . Recent studies, however, have demonstrated the utility of using chemotherapy in conjunction with lower doses and limited fields of irradiation with equally successful overall and event‐free survival …”

Section: Discussionmentioning

confidence: 99%

“…Patients with MMGCT have an overall poorer prognosis than those with pure germinomas as MMGCT are not particularly radiosensitive. In studies where these patients were treated with irradiation alone, the overall 5‐year survival was only between 20 and 40% . The best outcomes result from treatment with chemotherapy and targeted irradiation in combination, leading to a 5‐year survival between 50 and 70% …”

Section: Discussionmentioning

confidence: 99%

Relapse and outcome patterns of patients with central nervous system mixed malignant germ cell tumors treated without irradiation: Findings from the Third International Central Nervous System (CNS) Germ Cell Tumor (GCT) Study

Pruitt

DaSilva

Cappellano

et al. 2015

Pediatric Blood & Cancer

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Patients with CNS MMGCT relapsing following chemotherapy alone display two distinct patterns of recurrence and outcome; patients relapsing early possess MMGCT elements and have a dismal prognosis, while patients relapsing late do so with pure germinomatous elements and have an excellent outcome. Current cooperative group studies utilizing more localized fields of irradiation should monitor closely the patterns of relapse and outcome; late recurrences with germinomatous elements might be avoided by initial use of low-dose larger field irradiation in select patients.

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“…With combination cisplatin chemotherapy, 5-year survival rates are now 67-74% [9,13]. A therapeutic impact of radiotherapy has not yet been established [14], and both indications and extent of the surgery are still unclear [15]. Some studies suggest a therapeutic benefit from tumor debulking [16,17,18], whereas other reports emphasize that malignant GCTs should be treated with radiation therapy and chemotherapy without surgery [19,20].…”

Section: Discussionmentioning

confidence: 99%

Primary Pure and Nonsecreting Embryonal Carcinoma of the Anterior Third Ventricle: A Case Report

Nsir

Darmoul²,

Hadhri³

et al. 2015

Pediatr Neurosurg

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Central nervous system germ cell tumors (GCTs) account for less than 5% of primary brain tumors in children and adolescents but continue to attract much attention. To the best of our knowledge, a primary pure and nonsecreting embryonal carcinoma of the anterior third ventricle has never been previously reported. A 15-year-old boy presented with signs of increased intracranial pressure for the past 2 weeks complicated by 2 episodes of generalized tonic-clonic seizures 1 day before admission. Neurological examination was normal, and funduscopic examination disclosed a grade II papilledema bilaterally. CT and MRI revealed a well-demarcated and enhancing mass within the anterior third ventricle associated with a left lateral ventricle hydrocephalus. There was no evidence of tumor within the pineal or suprasellar region, and systemic and cerebrospinal fluid evaluation demonstrated normal levels of α-fetoprotein and human chorionic gonadotropin. Radical surgery was advised, and total tumor resection was achieved via a transcallosal transforaminal approach. The postoperative course was uneventful, and the final histological diagnosis was a pure embryonal carcinoma. Further screening showed no other location, and adjunctive high-dose chemotherapy was administered. The patient has been symptom free with no clinical or radiological sign of progression at the most recent follow-up examination 2 years after surgery. Primary pure and nonsecreting embryonal carcinoma can develop within the anterior third ventricle and should be considered in the differential diagnosis of anterior third ventricular masses especially in young patients. Accurate identification, radical surgery and high-dose chemotherapy can result in better tumor control and improve the postoperative outcome.

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Germ cell tumours of the central nervous system in children—controversies in radiotherapy

Cited by 31 publications

References 56 publications

Clinical and neuropsychological outcome of pediatric non‐midline central nervous system germinoma treated with chemotherapy and reduced dose/volume irradiation: The Children's Hospital Los Angeles experience

Clinical and neuropsychological outcome of pediatric non‐midline central nervous system germinoma treated with chemotherapy and reduced dose/volume irradiation: The Children's Hospital Los Angeles experience

Relapse and outcome patterns of patients with central nervous system mixed malignant germ cell tumors treated without irradiation: Findings from the Third International Central Nervous System (CNS) Germ Cell Tumor (GCT) Study

Primary Pure and Nonsecreting Embryonal Carcinoma of the Anterior Third Ventricle: A Case Report

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