Current Management of Intracranial Germ Cell Tumours

Al-Naeeb, Anna Bowzyk; Murray, Matthew J.; Horan, Gail; Harris, Frances; Kortmann, RD; Nicholson, James C.; Ajithkumar, TV

doi:10.1016/j.clon.2018.01.009

Cited by 44 publications

(33 citation statements)

References 63 publications

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“…NGGCT still have good outcomes, with progression-free survival rates of 72% and 68%, and overall survival rates of 82% and 75%, for localised and metastatic patients, respectively, but require a more intensive combined chemoradiotherapy approach for cure than germinomas 12. While patients with metastatic NGGCT are treated with CSI, the radiotherapy management of localised NGGCT differ globally and varies from focal radiotherapy to CSI 13 14…”

Section: Introductionmentioning

confidence: 99%

Symptom interval and treatment burden for patients with malignant central nervous system germ cell tumours

et al. 2019

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ObjectivePatients with central nervous system germ cell tumours (CNS-GCTs) commonly initially present to primary care or general paediatricians. Prolonged symptom intervals (SI) are frequently seen in CNS-GCTs and have been associated with inferior outcomes in other brain tumours. This study reviewed the clinical presentation of CNS-GCTs and examined the effect of prolonged SI.Design/Setting/Patients/OutcomesInternational multicentre 10-year retrospective study (2002–2011 inclusive), across six international paediatric oncology treatment centres. All newly diagnosed patients with CNS-GCT were included. Main outcome measure was time interval from first symptom to diagnosis.ResultsThe study cohort included 86 (58 males:28 female) patients (59 ‘germinoma’ and 27 ‘non-germinomatous’ GCTs), with tumours being pineal (n=33), suprasellar (n=25), bifocal (pineal+suprasellar; n=24) and ‘other’ site (n=4), of which 16 (19%) were metastatic. Median age at diagnosis was 14 years (0–23 years). The time to diagnosis from first symptom (SI) was 0–69 months (median 3 months, mean 9 months). A prolonged SI (>6 months) was observed in 28/86 patients (33%) and significantly associated with metastatic disease (11/28 (39%) vs 5/58 (9%); p=0.002)) at diagnosis, but not overall survival. With prolonged SI, endocrine symptoms, particularly diabetes insipidus, were more common (21/28 (75%) vs 14/58 (24%) patients; p<0.002), but raised intracranial pressure (RICP) was less frequent (4/28 (14%) vs 43/58 (74%) patients; p<0.001)) at first symptom.ConclusionsOne-third of patients with CNS-GCT have >6 months of symptoms prior to diagnosis. Delayed diagnosis is associated with metastatic disease. Early symptom recognition, particularly related to visual and hormonal disturbances in the absence of RICP, may improve timely diagnosis, reduce metastatic disease frequency and consequently reduce treatment burden and late effects.

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Section: Introductionmentioning

confidence: 99%

Symptom interval and treatment burden for patients with malignant central nervous system germ cell tumours

et al. 2019

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“…In some clinical trials, 50 IU/L of β-HCG was also used as a cut-off for germinoma patient recruitment [ 4 , 10 ]. However, patients with mild elevation of β-HCG only were just considered as “germinoma,” NGGCTs components, which were less sensitive to anti-tumor therapy, could not be ruled out completely [ 9 , 12 ]. As a result, it is problematic if hydrocephalus did not improve satisfactorily after full-dose chemotherapy delivering and surgical intervention was inevitable, especially during the peri-bone-marrow-suppression period.…”

Section: Discussionmentioning

confidence: 99%

Emergency irradiation of 3.4Gy/2f in pineal gland germinoma patients with symptomatic hydrocephalus

et al. 2019

Chin Neurosurg Jl

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Background: Surgical interventions including ventriculostomy and ventriculo-peritoneal shunt were usually administrated in pineal germ cell tumor patients with symptomatic hydrocephalus. Considering higher sensitivity of germinoma to anti-tumor therapy, we explored emergency irradiation as non-invasive measure in this situation. Methods: Data of 35 germinoma patients with symptomatic hydrocephalus who received emergency irradiation of 3. 4 Gy/2f were studied retrospectively. The maximum width of frontal horn and the minimum width of trunk of corpus callosum (TCC) were measured to evaluate hydrocephalus changing. Besides, mean deviation (MD) of Humphrey perimetry was employed to evaluate visual field defect. Correlations between hydrocephalus changing and clinical factors, including age, percentage of tumor regression, radiographic re-evaluation interval, and serum beta-human chorionic gonadotropin (β-HCG) level, were analyzed. Results: The median maximum diameter and volume of pineal lesions was 27 mm (range 10-55 mm) and 6.5cm 3 (range 0.4-74.1 cm 3), respectively. At median 8 days after irradiation, the median percentage of tumor remission was 55% (range 10-100%). The median maximum width of FN and the median minimum width of TCC were 11.6 mm and 39.0 mm, and 8.0 mm and 31.4 mm, before and after irradiation, respectively. The improvement of both parameters reached significant level (p < 0.001). However, none clinical factor was found to have correlation with their improvement. In 14 patients with paired data of pre-and post-irradiation MD, its change did not reach the significant level for both eyes. All patients successfully received subsequent chemoradiotherapy without surgical intervention. Conclusions: Emergency irradiation of 3.4 Gy/2f was an effective non-invasive measure to relief hydrocephalus in pineal germinoma patients.

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“…CNS germinoma responds well to RT alone. Substantial late effects of irradiation especially in children and recent findings that reduced dosage of RT to primary site without prophylactic use of craniospinal RT (for localized tumors) can be effectively administered had led to attempts to reach an optimal reduced dosage for treating CNS germinoma [ 11 13 14 ]. Some clinical trials investigated the role of neoadjuvant chemotherapy in effectively reducing RT dose and volume, the results showed excellent progression-free survival [ 11 13 14 ].…”

Section: Discussionmentioning

confidence: 99%

Pineal and Suprasellar Germinoma Cooccurence with Vertebra Plana: A Case Report

Toosi

Aminzadeh

Rad

et al. 2018

Brain Tumor Res Treat

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Germinoma is the most common type of intracranial germ cell tumors (GCTs). Pineal gland and suprasellar region are the most frequent sites of central nervous system (CNS) involvement. Intracranial masses caused by Langerhans cell histiocytosis (LCH) mimics features of CNS GCTs. LCH frequently involve spine and is the most common cause of vertebra plana in children. A 15-year-old boy presented with progressing symptoms of polydipsia, polyuria, general headache, nausea and severe back pain. Brain MRI showed brain tumor with simultaneous involvement of suprasellar region and pineal gland. An excisional biopsy of suprasellar mass was done. The pathologic assessment confirmed the diagnosis of germinoma. Patient's treatment continued accordingly. A spine MRI, done due to persistent backache, showed a vertebra plana. We reevaluated the primary diagnosis suspecting LCH. Germinoma of CNS was confirmed and a biopsy of vertebral lesion resulted in hemangioma. Thus we report a case of CNS germinoma with co-occurrence of vertebra plana. We emphasized the importance of histopathologic diagnosis of pineal/suprasellar masses and primary investigation of other CNS regions including spine for possible metastasis or comorbidities.

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Current Management of Intracranial Germ Cell Tumours

Cited by 44 publications

References 63 publications

Symptom interval and treatment burden for patients with malignant central nervous system germ cell tumours

Symptom interval and treatment burden for patients with malignant central nervous system germ cell tumours

Emergency irradiation of 3.4Gy/2f in pineal gland germinoma patients with symptomatic hydrocephalus

Pineal and Suprasellar Germinoma Cooccurence with Vertebra Plana: A Case Report

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