✓ The authors discuss the results obtained in patients who underwent foramen magnum decompression for longstanding advanced Chiari I malformation in which marked spinal cord atrophy was present. This 50-year-old woman presented with progressive quadriparesis and sensory disorders. Magnetic resonance imaging revealed the descent of cerebellar tonsils and medulla associated with remarkable C1—L2 spinal cord atrophy. After a C-1 laminectomy—based foramen magnum decompression, arachnoid dissection and duraplasty were undertaken. These procedures resulted in remarkable neurological improvement, even after 40 years of clinical progression. Spinal cord atrophy may be caused by chronic pressure of entrapped cerebrospinal fluid in the spinal canal.
Yoshiro Ito, Koji Tsuboi, Hiroyoshi Akutsu, Satoshi Ihara and Akira Matsumura
Yosuke Masuda, Hiroyoshi Akutsu, Eiichi Ishikawa, Masahide Matsuda, Tomohiko Masumoto, Takashi Hiyama, Tetsuya Yamamoto, Hidehiro Kohzuki, Shingo Takano and Akira Matsumura
MRI scans obtained within 48–72 hours (early postoperative MRI [epMRI]), prior to any postoperative reactive changes, are recommended for the accurate assessment of the extent of resection (EOR) after glioma surgery. Diffusion-weighted imaging (DWI) enables ischemic lesions to be detected and distinguished from the residual tumor. Prior studies, however, revealed that postoperative reactive changes were often present, even in epMRI. Although intraoperative MRI (iMRI) is widely used to maximize safe resection during glioma surgery, it is unclear whether iMRI is superior to epMRI when evaluating the EOR, because it theoretically shows fewer postoperative reactive changes. In addition, the ability to detect ischemic lesions using iMRI has not been investigated.
The authors retrospectively analyzed prospectively collected data in 30 patients with glioma (22 and 8 patients with enhancing and nonenhancing lesions, respectively) who underwent tumor resection. These patients had received preoperative MRI within 24 hours prior to surgery, postresection radiological evaluation with iMRI during surgery, and epMRI within 24 hours after surgery, with all neuroimaging performed using identical 1.5T MRI scanners. The authors compared iMRI or epMRI with preoperative MRI, and defined a postoperative reactive change as a new postoperative enhancement or T2 high-intensity area (HIA), if this lesion was outside of the preoperative original tumor location. In addition, postoperative ischemia was evaluated on DWI. The iMRI and epMRI findings were compared in terms of 1) postoperative reactive changes, 2) evaluation of the EOR, and 3) presence of ischemic lesion on DWI.
In patients with enhancing lesions, a new enhancement was seen in 8 of 22 patients (36.4%) on iMRI and in 12 of 22 patients (54.5%) on epMRI. In patients with nonenhancing lesions, a new T2 HIA was seen in 4 of 8 patients (50.0%) on iMRI and in 7 of 8 patients (87.5%) on epMRI. A discrepancy between the EOR measured on iMRI and epMRI was noted in 5 of the 22 patients (22.7%) with enhancing lesions, and in 3 of the 8 patients (37.5%) with nonenhancing lesions. The occurrence of ischemic lesions on DWI was found in 5 of 30 patients (16.7%) on iMRI, whereas it was found in 16 of 30 patients (53.3%) on epMRI (p = 0.003); ischemic lesions were underestimated on iMRI in 11 patients.
Overall, given the lower incidence of postoperative reactive changes on iMRI, it was superior to epMRI in evaluating the EOR in patients with glioma, both with enhancing and nonenhancing lesions. However, because ischemic lesions can be overlooked on iMRI, the authors recommend only the additional DWI scan during the early postoperative period. Clinicians need to be mindful about not overestimating the presence of residual tumor on epMRI due to the high incidence of postoperative reactive changes.
Takuma Hara, Hiroyoshi Akutsu, Shingo Takano, Hiroyoshi Kino, Eiichi Ishikawa, Shuho Tanaka, Hidetaka Miyamoto, Noriaki Sakamoto, Keiichiro Hattori, Mamiko Sakata-Yanagimoto, Shigeru Chiba, Takashi Hiyama, Tomohiko Masumoto and Akira Matsumura
The Wnt/β-catenin signaling pathway is strongly implicated in the pathogenesis of adamantinomatous craniopharyngioma (adaCP). However, there is no evidence that the CTNNB1 mutation activates the target gene of Wnt/β-catenin signaling, and it is unknown whether it affects the tumorigenesis of adaCP. To assess the effect of the CTNNB1 mutation of adaCP, the authors analyzed the correlation between the mutation and clinical, radiological, pathological, and biological findings.
Between 2003 and 2015, 42 patients (24 male and 18 female, median age 42 years) with either papillary craniopharyngioma (papCP) or adaCP underwent tumor resection at the authors’ institution. BRAF V600E and CTNNB1 in papCP and adaCP samples were sequenced by next-generation sequencing and the Sanger method, and mRNA expression levels of Axin2 and BMP4 were evaluated by RT-PCR. Axin2, BMP4, β-catenin, and BRAF expression were evaluated by immunohistochemistry. Other data were collected from clinical reports.
The BRAF V600E mutation was detected in all 10 cases of papCP (100%). CTNNB1 exon 3 mutations were detected in 21 of 31 (68%) cases of adaCP, excluding 1 case for which there were no available sequence data. The mRNA expression level of Axin2 was significantly higher in adaCPs with a CTNNB1 mutation than in those without (p < 0.05). The immunohistochemical findings of Axin2 and BMP4 did not correlate with CTNNB1 mutation positivity. When patients who received adjuvant radiation therapy were excluded, progression-free survival was shorter in the mutation-positive group than in the mutation-negative group (log-rank test, p = 0.031). Examination of clinical characteristics and immunohistochemical findings of adaCPs showed that there was no significant correlation between CTNNB1 mutation positivity and age, sex, tumor volume, gross-total resection, optic tract edema, calcification, or T1 signal intensity of cyst fluid on MRI, β-catenin, and MIB-1 index.
These results raise the possibility that the CTNNB1 mutation in adaCP may be associated with disease recurrence, and genes related to the Wnt/β-catenin signaling pathway might represent a therapeutic target.