The authors report a rare case involving the occurrence of metachronous pure germinomas long after treatment of a mixed germ cell tumor (GCT) categorized as having a poor prognosis. A neurohypophysial germinoma occurred 4 years and 6 months after the initial treatment of a mixed pineal GCT containing a yolk sac tumor and a germinoma. Furthermore, intramedullary germinomas occurred 21 years after the initial treatment of the mixed GCT and 15 years after the second treatment of the neurohypophysial germinoma. The neurohypophysial germinoma was not confirmed histopathologically, but the intramedullary germinoma was histopathologically diagnosed as a pure germinoma. Serum α-fetoprotein levels at the second neurohypophysial and third intramedullary occurrences of the germinomas were less than 10 ng/ml. Therefore, no yolk sac components seemed to be contained in the tumors. The second neurohypophysial and third intramedullary germinomas might be recurrences of the germinoma component of the pineal mixed GCT, which consisted of a yolk sac tumor and a germinoma. However, it seems very unlikely that only the germinoma, categorized in the good prognosis group, would be the only one to recur. Hence, it seems plausible that both the second and the third occurrences of pure germinoma were de novo metachronous GCTs arising after the pineal mixed GCT was cured. The authors' case indicates the possibility of multicentric GCTs in the CNS.
Shinya Jinguji, Kouichirou Okamoto, Junichi Yoshimura, Yuichiro Yoneoka, Ryousuke Ogura, Akihiko Saito and Yukihiko Fujii
Shinya Jinguji, Junichi Yoshimura, Kenichi Nishiyama, Hiroshi Aoki, Keisuke Nagasaki, Manabu Natsumeda, Yuichiro Yoneoka, Masafumi Fukuda and Yukihiko Fujii
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.
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.
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.
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.
Atsuko Harada, Yukihiko Fujii, Yuichiro Yoneoka, Shigekazu Takeuchi, Ryuichi Tanaka and Tsutomu Nakada
Object. The purpose of this study was to assess the utility of high-field magnetic resonance (MR) imaging as a quantitative tool for estimating cerebral circulation in patients with moyamoya disease.
Methods. Eighteen patients with moyamoya disease who were scheduled to undergo revascularization surgery and 100 healthy volunteers were examined using T2-reversed MR imaging performed using a 3-tesla system. Ten of the 18 patients underwent a second study between 1 year and 3 years after revascularization. Magnetic resonance images obtained in the patients with moyamoya disease were statistically analyzed and compared with those obtained in healthy volunteers. The MR imaging findings were also correlated with results of single-photon emission computerized tomography and conventional cerebral angiography studies.
Transverse lines in the white matter (medullary streaks) were observed in almost all persons. In healthy volunteers, the diameter sizes of the medullary streaks increased significantly with age (p < 0.001). Multiple logistic regression analysis revealed that age-adjusted medullary streak diameters were significantly larger in patients with moyamoya disease (p < 0.001). Diameter sizes also increased significantly with the increased severity of cerebral hypoperfusion (p < 0.001) and a higher angiographically determined stage of the disease (p < 0.001). Diameter sizes decreased significantly after surgery (p < 0.001).
Conclusions. The increases in medullary streak diameters observed in patients with moyamoya disease appear to represent vessels dilated due to cerebral hypoperfusion. High-field T2-reversed MR imaging is useful in estimating cerebral circulation in patients with moyamoya disease.
Yuichiro Yoneoka, Naoto Watanabe, Hitoshi Matsuzawa, Itaru Tsumanuma, Satoshi Ueki, Tsutomu Nakada and Yukihiko Fujii
Three-dimensional anisotropy contrast (3DAC) magnetic resonance (MR) imaging provides clear depiction of neuronal fibers. The aim of this study was to identify intracavernous cranial nerves in patients with pituitary macro-adenoma and in healthy volunteers by using 3DAC MR imaging on a 3-tesla system and to preoperatively predict cavernous sinus invasion by pituitary macroadenoma.
Thirty-three patients (cavernous sinuses in 66 sides) with pituitary macroadenomas and 25 healthy volunteers (50 sides) participated in this study. Coronal 3DAC MR images constructed from diffusion weighted images, acquired with periodically rotated overlapping parallel lines with enhanced reconstruction (PROPELLER) sequences, and T2-weighted reverse images were obtained at the same anatomical locations using a 3-tesla MR imaging system. Attempts were made to identify the cranial nerves.
The oculomotor and ophthalmic/maxillary nerves were preoperatively identified in all sides (66 sides in patients and 50 sides in healthy volunteers) on 3DAC MR images. In the 33 patients, cavernous sinus invasion was revealed in 10 (12 [18.2%] of 66 sides) by intraoperative endoscopic observation. Coronal 3DAC MR images revealed that the oculomotor nerves were half surrounded with adenoma in all 12 of these sides, and the ophthalmic/maxillary nerves were half encapsulated with tumor (sensitivity/specificity: 100%/100% and 83%/100%, respectively).
Preoperative evaluation of pituitary macroadenomas using 3DAC PROPELLER MR imaging on a 3-tesla system is likely to be a powerful noninvasive method of detecting cavernous sinus invasion, which can potentially dominate the therapeutic strategy for these lesions.