{"id":"62250b98-f01f-4f18-a56e-824b24328963","slug":"pediatric-angiocentric-glioma-with-acute-intracerebral-hemorrhage-a-case-report-with-36-months-follow-up","title":"Pediatric angiocentric glioma with acute intracerebral hemorrhage: A case report with 36 months follow-up","authors":["Rui Zhang","Xin Xu","Huakang Zhou","Dongying Yao","Ru Wei","Sajjad Muhammad"],"abstract":"Background: Angiocentric glioma (AG) is an extremely rare intracranial tumor that was first described in 2005 and identified as a special type of intracranial tumor in 2007 by the WHO, which mainly affects children and young adolescents. Epilepsy is the main presentation; therefore, it was recognized as a seizure-related tumor in the past. Here, we report a case of AG with acute intracerebral hemorrhage (ICH) as the first symptom who never had a seizure onset. Case Description: A 3-year-old girl with the right limb weakness was admitted to our hospital 4 h after onset in 2018. Computed tomography showed a hematoma of about 20 ml accompanied by a hyper/iso-dense spheroid lesion located in the sub-cortex of the left parietal lobe. Magnetic resonance image (MRI) showed signs of hypointense signal in T1, T2, and fluid-attenuated inversion recovery sequence, distinct enhancement of this tumefactive lesion in the contrast-enhanced sequence. Thus, the admission diagnosis was neoplasm with acute ICH. A gross total resection of the tumor was achieved by parietal craniotomy. The histopathological diagnosis was AG. No signs showed tumor recurrence after 36 months of follow-up. Conclusion: This is the sole case of AGs with acute intracranial hemorrhage as the first symptom without any kind of epilepsy by far. This case had unique MRI signs that were different from the previous description. This case enriches the clinical and radiological manifestations of AG and reveals that further investigations are needed to further understand AG. Angiocentric glioma (AG) is a rare intracranial tumor. WANG and Lellouch-Tubiana, respectively, 1st time reported in 2005. This special type of tumor mainly affects children and young adults.[ 8 , 12 ] Two years later, the WHO officially confirmed it as a central nervous system entity tumor for the 1st time in the classification of tumors of the central nervous system.[ 9 ] Considering the low incidence of this tumor, 108 cases had been reported until December 2019,[ 5 ] excluding this case we described. They had similar radiological features, such as magnetic resonance image (MRI) T1 hypointense signal, hyperintense signal in T2, and fluid-attenuated inversion recovery (FLAIR’s) without contrast enhancement.[ 1 , 5 ] Mostly AG is located in the supratentorial area in hemispheric lobes, with a benign biological behavior: slow-growing, possibility to be surgically removed, rare postoperation recurrence, and low proliferation index; so AG was classified as WHO Grade I.[ 10 ] Drug-refractory epilepsy is the most common complaint in this tumor, other symptoms such as headache, vision deficit, dizziness, earache, ataxia, dysphagia, weakness, numbness, and strabismus had been reported.[ 5 ] Here, we reported a case of AG with acute intracerebral hemorrhage (ICH) that never has been described in the literature. A 3-year-old girl was admitted to our emergency department due to the sudden onset of the right limb weakness. Physical examination showed a conscious child with poor spirit, normal-sized bilateral reactive pupils, hemiplegia of the right limb with low muscle tone, and muscle strength level 0. The child had vomited several times after the onset. No seizures occurred before and after onset. After admission, a computed tomography (CT) scan of the head showed a hematoma with the amount of 20 ml in the left parietal lobe. The primary diagnosis was ICH. However, a suspect lesion has a different density anterior to the hematoma suggesting the presence of neoplasm. The MRI scan and MRI contrast enhancement showed that admix signals (hypointense of the tumor part and isointense of the hematoma part) on T1 sequence, hypointense or isointense lesion with annularity hyperintense around on T2 and FLAIR sequence, and part of the lesion can be significantly enhanced on the contrast sequence. Thus, the modified diagnosis was the left parietal lobe tumor with ICH [ Figure 1 ]. In the computed tomography images (a): an ICH located in the parietal lobe surrounded by circle edema. An ellipse entity that had the same density as the cortex can be seen in the anterior part of this lesion. In MRI images: this lesion can be significantly enhanced in the contrast sequence (b), with an extremely low signal in the T1 sequence (c), and in the T2 (d) and FLAIR (e) sequence the signals were still low, which were different from the previous literature. This is a cortex-sub-cortex lesion that includes tumor and hematoma situated at the central region of the left hemisphere. With the assistance of the neuronavigation, a left parietal craniotomy was performed. A trans-sulcus approach was used to perform the minimal cortical incision. We successfully removed all the hematoma and the gross total resection of the tumor was achieved [ Figure 2 ]. Three-dimensional reconstruction virtual image of the preoperative computed tomography scan showed the relationship between the tumor hematoma and the central sulcus [the left part of Figure 2]. The intro-operation image showed a gross total resection of the tumor, meanwhile, the cortical incision was limited [the right part of Figure 2]. The tumor is located in the superficial layer of the sub-cortex, with clear borders, 2 cm × 2 cm × 2.3 cm in size, gray-pink cut surface, and slightly tough texture. The tumor is composed of glial components, blood vessels, and degenerated neurons. The tumor cells are spindle-shaped, bipolar, single-layered or multi-layered, arranged in a chrysanthemum-like structure, or forming a pseudo-chrysanthemum-like structure around the blood vessels, and ependymal-like structure; in some areas, spindle cells are interwoven into sheets to form a nerve sheath-like structure; tumor cell nuclei are oval, round, or spindle-shaped, with visible nucleoli and abundant cytoplasm. No nucleic mitosis was observed; the proliferation of microvessels, necrosis, and hemorrhage can be seen. Tumor cells are diffusely positive for glial fibrillary acid protein (GFAP), S-100, CD99, blood vessels CD31, and F-8 are positive; proliferation index KI-67 hot spot is about 5%. Syn, CgA, NSE, AE1/AE3, and epithelial membrane antigen (EMA) are negative [ Figure 3 ]. (1 and 2) Hematoxylin and eosin staining. Spindle tumor cells, bipolar, single-layered or multi-layered, arranged in a chrysanthemum-like structure, or forming a pseudo-chrysanthemum-like structure around the blood vessels. Glial fibrillary acidic protein (3), S-100 (4), CD99 (5), CD31 (6), and F-8 (7) are positive. KI-67 hot spot is about 5% (8) EMA is negative (9). Original magnification ×100. No postoperative complications occurred. After 2 weeks, the muscle strength of the right arm was reached level 3, and level 4 in her right leg. Then, this patient was transferred to a rehabilitation center. Due to its benign characteristic, adjuvant treatment such as chemo- or radiotherapy was not needed. Three months after discharge, the patient’s right limb hemiplegia was completely resolved. Twelve months later, there was no sign of tumor recurrence in the cranial MRI. After that, MRI examination was performed every 12 months, and the past MRI scan was 36 months later. No tumor recurrence was found till the past follow-up [ Figure 4 ]. The child now is in stable condition and is living a normal life. Postoperative MRI checks from the year 2019 (a) to 2021 (c). The tumor has a total resection. No signal of recurrence was found.","thumbnailUrl":"https://sni-digital-videos.s3.amazonaws.com/articles/62250b98-f01f-4f18-a56e-824b24328963/featured/hero-1781561958125.png","publishDate":"2021-10-06T00:00:00.000Z","doi":"10.25259/SNI_791_2021","categories":["Pediatric Neurosurgery","Case Report"],"fullTextUrl":"https://surgicalneurologyint.com/wp-content/uploads/2021/10/11161/SNI-12-499.pdf"}