Factors affecting functional outcomes in long-term survivors of intracranial germinomas: a 20-year experience in a single institution

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>Radiation monotherapy—prophylactic craniospinal or whole-brain irradiation paired with a radiation boost to the primary tumor—is the standard treatment for intracranial germinomas at the authors' institution. The authors assessed long-term outcomes of patients with germinoma who underwent therapy and identified factors affecting them.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>The authors retrospectively analyzed data obtained in 46 patients (35 males and 11 females, age 5–43 years at diagnosis) who had been treated for intracranial germinomas between 1990 and 2009 at the authors' institution. Thirty patients had germinomas in localized regions and 16 in multiple regions. Thirty-eight patients (83%) underwent radiotherapy alone (craniospinal irradiation in 32 and whole-brain irradiation in 6). Seven patients underwent radiochemotherapy and 1 underwent chemotherapy alone. The mean radiation doses for the whole brain, spine, and primary tumor site were 26.9, 26.6, and 49.8 Gy, respectively. The median follow-up period was 125 months.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>The 10-year overall and recurrence-free survival rates were 93.3% and 89.3%, respectively. None of the 38 patients who received radiation monotherapy developed a recurrent lesion, whereas 1 of 7 who underwent radiochemotherapy and the 1 patient who underwent chemotherapy had a recurrent lesion. Of the entire population, 26 patients required hormone replacement therapy, 2 had short stature, and 1 developed a radiation-induced meningioma. Seventeen of the 25 childhood- or adolescent-onset patients were 19 years or older at the latest follow-up visit, 15 of whom graduated from senior high school, and only 2 of whom graduated from college. Of 34 patients who were 19 years or older at the latest visit, 4 were students, 18 worked independently, 4 worked in sheltered workplaces, and 8 were unemployed. Of the 34 patients, 4 got married after the initial treatment, 3 of whom had children. There were 8 patients (17%) with low postoperative Karnofsky Performance Scale (KPS) scores that were significantly associated with impaired neurocognitive functions, severe surgical complications, and neurological impairments. In 10 of the 46 patients, KPS scores at the latest visit were lower than their postoperative KPS scores. These decreases in KPS scores were significantly correlated with a delayed decline in neurocognitive functions in childhood-onset patients and a postoperative impairment of neurocognitive functions in patients with adolescent- or adult-onset germinoma.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>No tumor recurrence occurred in germinoma patients treated with the authors' radiation monotherapy, which appears to be effective enough to cure the tumor. Brain damage caused by tumors themselves and surgical complications were found to adversely affect functional outcomes in patients regardless of their age. Although radiotherapy rarely caused late adverse effects in patients with adolescent- or adult-onset, in some childhood-onset lesions, the radiation seems to carry the risk of neurocognitive dysfunctions, which are attributable to late adverse effects. Accordingly, treatments for germinoma patients should be selected according to a patient's age and the extent of the tumor and with particular care to avoid surgical complications.</jats:p></jats:sec>

Management of CNS germinoma

“…MRI's of the spine may be included annually to exclude subclincal relapse in the spine. Radiation therapy can also cause visual field impairments, endocrinopathies, cognitive decline and learning disabilities [28][29][30]. In one study, 17% of patients had low postoperative Karnofsky performance associated with impaired neurocognitive function.…”

“…In one study, 17% of patients had low postoperative Karnofsky performance associated with impaired neurocognitive function. On subsequent followup, ten of 46 patients (21%) had even lower Karnofsky performance [28]. Strojan et al published a review of the long-term survival and late effects in patients with CNS GCT treated with radiotherapy alone [31].…”

Current concepts and future strategies in the management of intracranial germinoma

“…Although RT rarely caused late adverse effects in patients treated as adolescents or adults, the radiation seemed to carry the risk of neurocognitive dysfunctions. However, brain damage caused by tumors themselves and surgical complications were an important additional factor and adversely affected functional outcome in patients regardless of their age [67]. Sands compared quality of life in patients after chemotherapy alone with patients who received additional RT to the neuroaxis.…”

“…Data on quality of survival and neurocognitive function are retrospective in nature, based on heterogeneous cohorts of patients including non-germinoma who received more intensive treatments [13,15,19,22,36,42,63,65,67,68,70,71]. Longitudinal observations are often missing and the instruments used considerably vary between series.…”

The combination of a bifocal germinoma and arteriovenous malformations of the brain in children. Clinical observation and review of the literature

“…Десятилетняя общая выживаемость пациентов с чистой герминомой достигает 90 % [8,10,[17][18][19][20][21], в противоположность пациентам с негерминомами, выживаемость которых варьирует, по данным разных авторов, от 30 до 80 % [22][23][24]. В то же время неко-торые авторы предполагают более плохой прогноз для бифокальных гермином, так как рассматривают их с точки зрения метастатического распространения со-литарного опухолевого узла [15].…”