It has been reported that bevacizumab, an agent administered as an adjuvant therapy for high-grade gliomas, causes thromboembolic complications. We report a cerebral infarction with newly developed cerebral artery stenosis occurring during treatment with bevacizumab for an anaplastic astrocytoma. A 48-year-old female underwent excision surgery for an anaplastic astrocytoma on the right temporal lobe and received radiation therapy and chemotherapy with temozolomide. Twenty months after the maintenance therapy, treatment with bevacizumab was introduced for tumor recurrence. After the 14 th course of bevacizumab at 6 months, 27 months after radiation therapy, the patient began experiencing mild right hemiparesis. Magnetic resonance imaging revealed scattered cerebral infarcts on the left frontal lobe and diffuse cerebral artery stenosis of the bilateral internal carotid artery system both inside and outside the radiation-treated area. Antiplatelet medication was commenced, and there was no recurrence of ischemic stroke. The morphological transition of the cerebral arteries should be carefully monitored via magnetic resonance angiography during post-radiation treatment with bevacizumab.
Infantile myofibromatosis (IM) is a rare benign soft tissue tumor seen mainly in infants and toddlers. IM is the most common benign mesenchymal tumor observed in infancy, with an estimated incidence of 1/150,000 to 1/400,000 live births. The clinical spectrum ranges from solitary disease, which accounts for about half of all cases, to multiple and sometimes disseminated lesions that involve cutaneous, subcuta-(A) CT T1-FLAIR T1-FLAIR/FS +Gd X-ray T2-FLAIR DWI T1-FLAIR +Gd T2 MRI Pre-resection Tumor Post-resection (B) HE HE -SMA Ki67 (C) F I G U R E 1 Imaging studies, intraoperative findings, and histopathological analysis of solitary IM. (A) Anteroposterior plain radiograph and computed tomography (CT) images (axial slice and 3D volume rendering image) of the skull show a lytic lesion, measuring 2 cm, in the frontal bone. Axial magnetic resonance (MR) images of indicated sequences are shown. (B) Intraoperative pictures showing postscalp incision, the tumor, and postresection of the tumor. (C) Hematoxylin and eosin (H&E) staining (upper left and upper right panels) and immunohistochemical staining for α-smooth muscle actin (α-SMA) and Ki67 (lower left and lower right panels), respectively. FLAIR, fluid-attenuated inversion recovery; FS, fat-suppressed; Gd, gadolinium contrast; DWI, diffusion weighted imaging. neous, and visceral tissues. 1 The prognosis for solitary IM is generally good after surgical resection of the tumor; some cases regress spontaneously. However, multicentric disease involving visceral organs can be life-threatening, and patients may require systemic chemotherapy. 2,3 The World Health Organization (WHO) classification of soft tissue tumor categorizes IM to the family of benign pericytic/perivascular tumors. 4,5 Recent studies have investigated the genetic basis of IM. Gainof-function (GOF) variants of the PDGFRB gene have been found in IM tumor tissues. 6,7 A recent study of 69 patients with IM identified PDGFRB somatic variants in 68% of multicentric cases and 24% of solitary tumor cases. 6 Also, germline GOF variants in the PDGFRB gene have been reported in familial cases of IM. 8,9A 10-month-old male presented with a bump on his forehead. Physical examination revealed a palpable, firm, nonmobile, painless mass on
BACKGROUND “Diffuse midline glioma, H3 K27M-mutant’ was newly categorized as a separate pathological entity in the 2016 WHO classification, based on recently discovered mutation. Spinal cord glioma with H3 K27M-mutant is rare, so we reported the clinical course of two cases. CASE 1: A 17-year-old male presented with posterior headache and right limbs paralysis. MRI showed cervical spinal cord with expansion, T2-weighted high intensity and a part of enhancement. The biopsy revealed a diffuse midline glioma, H3 K27M-mutant. He received bevacizumab plus radiotherapy-temozolomide. In a few months, he had quadriplegia and cranial nerve paralysis and needed respirator. There was not expansion of mass, but intracranial dissemination. CASE 2: A 16-year-old male presented with posterior neck pain and right limbs paralysis. On brain stem and cervical spine, MRI findings were same to case 1. The biopsy was undergone and revealed H3 K27M mutation. He received bevacizumab in addition to radiotherapy-temozolomide. Although he also had quadriplegia, the progression of tumor has stopped. He has received chemotherapy with respirator at home. DISCUSSION It was previously reported that the prognostic factors for diffuse midline glioma were tumor location, H3 K27M-mutation and age, but there are few relevant studies. The consensus on the treatment is also not clearly determined. Because the cervical spinal cord gliomas are rapidly advanced miserably, we added bevacizumab to standard radiotherapy-temozolomide for initial treatment. In addition, whole brain and spine radiation may be considered to avoid dissemination. Multicenter study is important to collect information and improve treatment of H3 K27M-mutant glioma.
Objective: Retethering is not an unusual operation for a congenital lumbosacral dysraphic spinal lesion. The present study aimed to assess a new surgical technique for preventing retethering. Surgical technique: After untethering the spinal cord, the pia mater or scar tissue at the caudal end of the conus medullaris is anchored to the ventral dura mater loosely using 8-0 thread, and the dura mater is closed directly. This technique is called ventral anchoring. Results: Ventral anchoring was performed in 15 patients (aged 5 to 37 years old, average age: 12.1 years old) between 2014 and 2021. All but one patient showed improvement or stabilization of the preoperative symptoms. No complication directly related to the procedure was observed. Postoperative MRI demonstrated that the dorsal subarachnoid space was restored in 14 patients but was undetectable or absent in three patients on follow-up MRI. No patients have experienced a recurrence of the tethered cord syndrome during the follow-up period. Conclusion: Ventral anchoring is effective for restoring the dorsal subarachnoid space after untethering the spinal cord. This preliminary study suggested that ventral anchoring has the potential to prevent the postoperative recurrence of tethered cord syndrome in patients with a congenital lumbosacral dysraphic spinal lesion.
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