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

Clinical article

Shinya Jinguji, Junichi Yoshimura, Kenichi Nishiyama, Hiroshi Aoki, Keisuke Nagasaki, Manabu Natsumeda, Yuichiro Yoneoka, Masafumi Fukuda, and Yukihiko Fujii

Object

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.

Methods

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.

Results

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.

Conclusions

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.

Open access

Preoperative three-dimensional multifusion imaging aiding successful microvascular decompression of a cerebellopontine angle lipoma: associated hemifacial spasm. Illustrative case

Hiroki Seto, Ryosuke Ogura, Tetsuya Hiraishi, Yoshihiro Tsukamoto, Taiki Saito, Satoshi Shibuma, Kohei Shibuya, Kouichirou Okamoto, Makoto Oishi, and Yukihiko Fujii

BACKGROUND

Cerebellopontine angle (CPA) lipoma–associated hemifacial spasm (HFS) is rare. As the removal of CPA lipomas has a high risk of worsening the neurological symptoms, surgical exploration is warranted only in selected patients. Preoperative identification of the lipoma affected site of the facial nerve, and offending artery are crucial for patient selection and successful microvascular decompression (MVD).

OBSERVATIONS

Presurgical simulation using three-dimensional (3D) multifusion imaging showed a tiny CPA lipoma wedged between the facial and auditory nerves, as well as an affected facial nerve by the anterior inferior cerebellar artery (AICA) at the cisternal segment. Although a recurrent perforating artery from the AICA anchored the AICA to the lipoma, successful MVD was achieved without lipoma removal.

LESSONS

The presurgical simulation using 3D multifusion imaging could identify the CPA lipoma, affected site of the facial nerve, and offending artery. It was helpful for patient selection and successful MVD.

Open access

Development and natural course of lateral posterior choroidal artery aneurysms arising from fragile choroidal collaterals in moyamoya disease: illustrative cases

Tomoaki Suzuki, Hitoshi Hasegawa, Kouichirou Okamoto, Kazuhiro Ando, Kohei Shibuya, Haruhiko Takahashi, Shoji Saito, Makoto Oishi, and Yukihiko Fujii

BACKGROUND

Choroidal collaterals are a risk factor for hemorrhagic stroke, even in the nonhemorrhagic hemisphere, among patients with moyamoya disease (MMD). Peripheral choroidal aneurysms rupture in fragile collaterals; however, the development and natural course of these aneurysms remain elusive.

OBSERVATIONS

A 51-year-old woman, who had experienced a right cerebral hemorrhage 3 years earlier, presented with asymptomatic minor bleeding from a left lateral choroidal artery aneurysm in a predeveloped choroidal anastomosis. Although the aneurysm spontaneously thrombosed within 2 months, the choroidal collaterals persisted. After bypass surgery, the choroidal anastomosis regressed, and neither a de novo aneurysm nor a hemorrhagic stroke occurred. A 75-year-old woman with MMD, who had experienced a left frontal infarction 6 years earlier, experienced recurrent right intraventricular hemorrhage from a ruptured lateral choroidal artery aneurysm that developed in the choroidal anastomosis. The aneurysm spontaneously regressed 3 days after the rebleeding with no recurrence over the following 7 years.

LESSONS

Choroidal artery aneurysms may develop in the choroidal anastomosis and rupture in the nonsurgical or contralateral hemispheres. Patients with MMD who have a history of hemorrhagic or ischemic stroke and impaired cerebral blood flow require careful observation. Although aneurysms may rapidly regress spontaneously, bypass surgery can stabilize hemodynamic stress and prevent further hemorrhage.

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Successful removal of a huge hypervascular tentorial cavernous angioma after preoperative endovascular embolization

Case report

Junichi Yoshimura, Yoshihiro Tsukamoto, Masakazu Sano, Hitoshi Hasegawa, Kazuhiko Nishino, Akihiko Saito, Masafumi Fukuda, Kouichirou Okamoto, and Yukihiko Fujii

The authors report a rare case of a huge hypervascular tentorial cavernous angioma treated with preoperative endovascular embolization, followed by successful gross-total removal. A 15-year-old girl presented with scintillation, diplopia, and papilledema. Computed tomography and MRI studies revealed a huge irregularly shaped tumor located in the right occipital and suboccipital regions. The tumor, which had both intra- and extradural components, showed marked enhancement and invasion of the overlying occipital bone. Angiography revealed marked tumor stain, with blood supply mainly from a large branch of the left posterior meningeal artery. Therefore, this lesion was diagnosed as a tentorium-based extraaxial tumor. For differential diagnosis, meningioma, hemangiopericytoma, and malignant skull tumor were considered. Tumor feeders were endovascularly embolized with particles of polyvinyl alcohol. On the following day, the tumor was safely gross totally removed with minimum blood loss. Histopathological examination confirmed the diagnosis of cavernous angioma. To date, there have been no reports of tentorium-based cavernous angiomas endovascularly embolized preoperatively. A tentorial cavernous angioma is most likely to show massive intraoperative bleeding. Therefore, preoperative embolization appears to be quite useful for safe maximum resection. Hence, the authors assert that the differential diagnosis of tentorium-based tumors should include tentorial cavernous angioma, for which preoperative endovascular embolization should be considered.

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Preoperative depiction of cavernous sinus invasion by pituitary macroadenoma using three-dimensional anisotropy contrast periodically rotated overlapping parallel lines with enhanced reconstruction imaging on a 3-tesla system

Yuichiro Yoneoka, Naoto Watanabe, Hitoshi Matsuzawa, Itaru Tsumanuma, Satoshi Ueki, Tsutomu Nakada, and Yukihiko Fujii

Object

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.

Methods

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.

Results

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).

Conclusions

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.

Open access

Repeated cerebellar infarction in the affected nondominant vertebral artery distribution with reversible vertebral artery occlusion elicited by head tilt: illustrative case

Takanori Nozawa, Kouichirou Okamoto, Shinji Nakazato, Kunio Motohashi, Tomoaki Suzuki, Kotaro Morita, Hideki Tashi, Kei Watanabe, Hitoshi Hasegawa, Masato Watanabe, Hiroyuki Kawashima, and Yukihiko Fujii

BACKGROUND

Bow hunter’s syndrome or stroke (BHS) is characterized by rotational vertebrobasilar insufficiency elicited by rotation of the neck. It is caused by dynamic and reversible occlusion of the vertebral artery (VA). Reversible symptoms of rotational vertebrobasilar insufficiency are described as bow hunter’s syndrome, although brain infarction is rarely reported as bow hunter’s stroke.

OBSERVATIONS

A 70-year-old man experienced repeated cerebellar infarctions three times in the posterior inferior cerebellar artery (PICA) distribution of the nondominant right VA connecting the basilar artery. The onset of symptoms indicating cerebellar infarcts and the patient’s head position changes were unrelated. Dynamic digital angiography (DA) revealed that the nondominant right VA was occluded by an osteophyte from the C4 vertebral body, and the right PICA branches were shown to be passing through the distal right VA from the left VA. These findings were observed when the patient’s head was tilted to the right. An arterio-arterial embolic mechanism was suggested as the cause of repeated cerebellar infarctions.

LESSONS

Transient nondominant VA occlusion has been rarely reported as a cause of BHS when the head is tilted. To confirm the diagnosis of BHS, additional head tilt is recommended when performing dynamic DA in patients with a cervical osteophyte.

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Decreased wall shear stress at high-pressure areas predicts the rupture point in ruptured intracranial aneurysms

Tomoaki Suzuki, Christopher J. Stapleton, Matthew J. Koch, Kazutoshi Tanaka, Soichiro Fujimura, Takashi Suzuki, Takeshi Yanagisawa, Makoto Yamamoto, Yukihiko Fujii, Yuichi Murayama, and Aman B. Patel

OBJECTIVE

Degenerative cerebral aneurysm walls are associated with aneurysm rupture and subarachnoid hemorrhage. Thin-walled regions (TWRs) represent fragile areas that may eventually lead to aneurysm rupture. Previous computational fluid dynamics (CFD) studies reported the correlation of maximum pressure (Pmax) areas and TWRs; however, the correlation with aneurysm rupture has not been established. This study aims to investigate this hemodynamic correlation.

METHODS

The aneurysmal wall surface at the Pmax areas was intraoperatively evaluated using a fluid flow formula under pulsatile blood flow conditions in 23 patients with 23 saccular middle cerebral artery (MCA) bifurcation aneurysms (16 unruptured and 7 ruptured). The pressure difference (Pd) at the Pmax areas was calculated by subtracting the average pressure (Pave) from the Pmax and normalized by dividing this by the dynamic pressure at the aneurysm inlet side. The wall shear stress (WSS) was also calculated at the Pmax areas, aneurysm dome, and parent artery. These hemodynamic parameters were used to validate the correlation with TWRs in unruptured MCA aneurysms. The characteristic hemodynamic parameters at the rupture points in ruptured MCA aneurysms were then determined.

RESULTS

In 13 of 16 unruptured aneurysms (81.2%), Pmax areas were identified that corresponded to TWRs. In 5 of the 7 ruptured cerebral aneurysms, the Pmax areas coincided with the rupture point. At these areas, the Pd values were not higher than those of the TWRs in unruptured cerebral aneurysms; however, minimum WSS, time-averaged WSS, and normalized WSS at the rupture point were significantly lower than those of the TWRs in unruptured aneurysms (p < 0.01).

CONCLUSIONS

At the Pmax area of TWRs, decreased WSS appears to be the crucial hemodynamic parameter that indicates the risk of aneurysm rupture.