Imaging manifestations of papillary glioneuronal tumors.

To analyse the imaging findings of papillary glioneuronal tumors (PGNTs), in order to improve the accuracy of preoperative diagnosis of this tumor. The clinical and imaging manifestations of 36 cases of PGNT confirmed by pathology were analyzed retrospectively. A total of 17 males and 19 females, averaging 22.47 (± 11.23) years. Initial symptoms included epilepsy in ten, headache in seven, and others in 19 cases. 97.2% (35/36) of the lesions were located in the supratentorial area, and 80.5% (29/36) in the intraventricular or deep white matter adjacent to the lateral ventricles. Twenty-four of the lesions (66.7%) were mixed cystic and solid, four (11.1%) were cystic with mural nodules, four (11.1%) were cystic, and four (11.1%) were solid. Four cases of PGNT of cystic imaging showed a "T2-FLAIR mismatch" sign. 69.4% (25/36) had septations. Nine lesions (25%) were accompanied by edema, and 9 (25%) of the mixed cystic and solid lesions were accompanied by hemorrhage. Among the 18 patients who underwent computed tomography (CT) or susceptibility-weighted imaging (SWI), nine had lesions with calcification. PGNTs mostly manifest as cystic mass with mural nodules or mixed cystic and solid mass in the white matter around the supratentorial ventricle, and the cystic part of the lesion is mostly accompanied by septations. Pure cystic lesions may exhibit the sign of "T2-FLAIR mismatch". PGNT is rarely accompanied by edema but sometimes by calcification and hemorrhage. Patients often present with seizures, headaches, and mass effect symptoms.

Middle temporal gyrus approach to mesial temporal lobe tumours in children.

AIM OF THE STUDY: To assess whether the middle temporal gyrus (MTG) approach to mesial temporal lobe (MTL) tumours is an effective procedure for the treatment of epilepsy in children. CLINICAL RATIONALE FOR THE STUDY: MTL tumours are a common cause of drug-resistant epilepsy in children. There is as yet no consensus regarding their treatment. One possibility is resection via a MTG approach. MATERIAL AND METHODS: We assessed the medical records of patients treated at the Department of Neurosurgery, Children's Memorial Health Institute,Warsaw, Poland between 2002 and 2020. A prospectively maintained database including clinical, laboratory, and radiographic presentation, as well as pre- and post-operative course, was analysed. Patients with at least a one- -year follow-up were included. RESULTS: There were 14 patients aged 4-18 years who underwent a MTG approach for a MTL tumour. All presented with epileptic seizure, and none had neurological deficit on admission to hospital. Median follow-up was 2.5 years. Neuronavigation was used to adjust the approach, localise the temporal horn, and achieve radical resection of the tumour and the hippocampus. Gross total resection was performed in all cases. In most patients, histopathological examination revealed ganglioglioma. One patient had transient aphasia. Two patients developed hemiparesis after surgery, which later improved. One of them also experienced visual disturbances. Acute complications were more frequent in younger patients (p = 0.024). In all cases, MRI confirmed complete resection and there was no tumour recurrence during the follow-up period. 13/14 patients remained seizure-free (Engel class I). CONCLUSIONS AND CLINICAL IMPLICATIONS: The MTG approach to MTL tumours is an effective procedure for the treatment of epilepsy in children. It avoids removal of the lateral temporal lobe and poses only a minor risk of permanent neurological complications.

Natural history and neuro-oncological approach in spinal gangliogliomas: a systematic review.

To describe the natural history of spinal gangliogliomas (GG) in order to determine the most appropriate neuro-oncological management. A Medline search for relevant publications up to July 2023 using the key phrase "ganglioglioma spinal" and "ganglioglioma posterior fossa" led to the retrieval of 178 studies. This corpus provided the basis for the present review. As an initial selection step, the following inclusion criteria were adopted: (i) series and case reports on spinal GG; (ii) clinical outcomes were reported specifically for GG; (iii) GG was the only pathological diagnosis for the evaluation of the tumor; (iv) papers written only in English was evaluated; and (v) papers describing each case in the series were included. The World Health Organization (WHO) 2021 grading criteria for gangliogliomas were applied. A total of 107 tumors were evaluated (63 from male patients and 44 from female patients; 1.43 male/1.0 female ratio, mean age 18.34 ± 15.84 years). The most common site was the cervical spine, accounting for 43 cases (40.18%); GTR was performed in 35 cases (32.71%) and STR in 71 cases (66.35%), while this information was not reported in 1 case (0.94%). 8 deaths were reported (7.47%) involving 2 males (25%) and 6 females (75%) aged 4-78 years (mean 34.27 ± 18.22) years. GGs located on the spine displayed the same gender ratio as these tumors in general. The most frequent symptom was pain and motor impairment, while the most prevalent location was the cervical spinal cord. GTR of the tumor posed a challenge for neurosurgeons, due to the difficulty of resecting the lesion without damaging the spinal eloquent area, explaining the lower rate of cure for this tumor type.

Metameric pruritus as the sole manifestation of an intramedullary tumor in a toddler.

An 18-month-old child presented with persistent pruritus and excoriation involving the right T9 and T10 dermatomes. She did not exhibit any other dermatological or neurological anomalies. Based on magnetic resonance imaging investigation of the spine, T8 ganglioglioma was diagnosed and surgically removed resulting in resolution of the pruritus within a few days. This observation underlines the importance of neuroimaging in patients presenting with metameric pruritus without specific skin lesions, especially in young children.

Epilepsy Outcome and Pathology Analysis for Ganglioglioma: A Series of 51 Pediatric Patients.

BACKGROUND: The postoperative epilepsy outcome and clinicopathological features in children with ganglioglioma (GG) are not well understood. METHODS: Data from 51 consecutive pediatric patients diagnosed with GGs who underwent surgery were collected. The correlations between the expression of CD34 and BRAF V600E mutations and clinical features were analyzed. The related factors affecting the outcome of epilepsy were analyzed. RESULTS: The average follow-up was 44.2 months, and 48 patients were seizure-free. A high proportion of BRAF V600E mutation (78.8%) and CD34 expression (77.8%) was detected in GG. The onset age of epilepsy with the BRAF V600E mutation was earlier than that without. The expression of CD34 increased with the age of onset, the duration of epilepsy, and the age of operation. Focal cortical dysplasia (FCD) I was found in 62.7% of patients, and FCD II was found in 11.8% of patients approximately in the cortex surrounding GG. There was no significant correlation between the outcome of epilepsy and BRAF V600E mutation, CD34 expression, and combination with FCD. CONCLUSIONS: The overall outcome of GG and epilepsy in children is optimistic, and the outcome is not closely related to the presence of BRAF V600E mutation and CD34 (+). The FCD surrounding GG could be type I or type II. Incomplete resection of the surrounding FCD has the risk of unsatisfactory control of epilepsy. Children with the BRAF V600E mutation may be prone to early-onset epilepsy. The expression of CD34 is more likely to be detected in children with older age and a long duration of epilepsy.

Unusual Locations of Gangliogliomas: Intraventricular and Posterior Fossa.

AIM: To report a series of patients diagnosed with gangliogliomas (GG) in unusual locations; and to review the clinical and imaging features as well as surgical treatment and outcomes. MATERIAL AND METHODS: A series of consecutive patients who underwent surgery for GGs at unusual locations, such as intraventricular region and posterior fossa, from 2010 to 2022 were included in the study. RESULTS: Nine patients with GGs located in unusual areas, one in the intraventricular region and 8 in the posterior fossa, were included. There were 5 males and 4 females, with a mean age 31±8.5 years. We performed GTR in 6 cases and STR in 3 cases. Seven tumors were grade I WHO while the remaining two were anaplastic. Five patients also had preoperative hydrocephalus. We found a positive correlation between midline GG of the posterior fossa and solid aspect of the tumor (p=0.05). Univariate analysis found no other statistically significant associations, but this was due to the small patient sample. Recurrence was seen in 2 cases with STR, after 1 and 10 years, respectively. CONCLUSION: GG should be considered in the differential diagnosis of patients with tumors in the intraventricular region or posterior fossa. Maximal tumor resection and restoration of CSF flow pathways ensure a good outcome. Growth patterns correlate with resection and can help choose the best candidates for surgery. However, further studies on large patient samples are needed.

A rare tumor in the sellar region: ganglioglioma, a case report and a general overview.

BACKGROUND: Gangliogliomas are rare mixed neuronal-glial tumors of the central nervous system, accounting for less than 2% of intracranial tumors. CASE DESCRIPTION: This report presents a rare case of ganglioglioma in the sellar region of a 3-year-old and 5-month-old pediatric patient. The patient underwent surgical intervention initially through a transnasal transsphenoidal approach and subsequently through a transcranial pterional craniotomy approach. Subsequently, radiotherapy and chemotherapy were administered for residual tumor tissue. The purpose of this report is to highlight the presence of ganglioglioma as a distinct diagnosis in sellar region tumors, discuss the surgical, radiotherapy, and/or chemotherapy treatment options for sellar region gangliogliomas based on the literature, and contribute the patient's follow-up and treatment outcomes to the existing literature. CONCLUSION: Complete tumor resection may not be feasible in sellar region gangliogliomas, especially in pediatric cases, due to endocrinological and vision-related complications. In cases where complete resection is not possible, radiotherapy and/or chemotherapy may be considered. However, the optimal treatment approach has not yet been established, and further research is needed.

A unique case of intracranial collision tumor composed of ganglioglioma WHO gr I and supratentorial ependymoma WHO gr III: case-based literature review.

PURPOSE: Intracranial collision tumor is a rare entity that represents the coexistence of two histopathological different tumor types in the same area without histological admixture or an intermediate cell population zone. So far, several cases of collision tumors with ganglioglioma as its component have been reported in the literature, while supratentorial ependymoma has never been reported as a collision tumor component. We are presenting a unique case of collision tumor in patient without previous history of head trauma, neurological surgery, radiotherapy, or phakomatosis. METHODS AND RESULTS: A 17-year-old male with no previous history of head trauma, neurological surgery, radiotherapy, or phakomatosis was presented to our clinic with grand mal seizure. Brain magnetic resonance imaging with gadolinium contrast was done revealing a contrast-enhancing lesion of right frontal lobe closely related to dura, surrounded by perifocal edema. The patient underwent a gross total tumor resection. Histological examination revealed collision tumor with two distinct components: ganglioglioma and supratentorial ependymoma. CONCLUSION: To our best knowledge, no previous reports of collision tumor composed of ganglioglioma and supratentorial ependymoma in a single patient have been reported. We believe that this report could significantly contribute to further surgical practice as well as to treatment decision for these types of collision tumors.

Spinal anaplastic ganglioglioma.

Anaplastic gangliogliomas of the spinal cord are extremely rare with only four cases reported in the literature. Here we present the case of a 22-year-old female who presented acutely with quadraparesis and urinary retention. Radiographic imaging demonstrated an intramedullary lesion within the cervical spine. She underwent a cervical laminectomy and resection of the lesion under neurophysiological monitoring. Post-operatively, she regained some function, but remained paraparetic. Histopathology demonstrated an anaplastic ganglioglioma (WHO Grade 3). She subsequently underwent radiotherapy. Following surgery, she remained stable and had MRC Grade 3 Power in all four limbs. Herein, we describe a previously undescribed case of cervical anaplastic ganglioglioma and review the existing literature.

Seizure-outcome after surgery of low-grade epilepsy associated neuro-epithelial tumors.

BACKGROUND: Most patients with glioneuronal tumors present with seizures. Although several studies have shown that greater extent of resection improves overall patient survival, few studies have focused on postoperative seizure outcome after resection of these tumors. The aim of this study was to characterize seizure control rates in patients undergoing glioneuronal tumor resection and evaluate the association between poor seizure outcome and tumor recurrence or progression. METHODS: The study population included patients who had undergone resection of glioneuronal tumors between 2014 and 2019 at our institution. Seizure outcome was assessed using Engel grading. Preoperative seizure characteristics, tumor characteristics, surgical factors, and postoperative seizure outcomes were reviewed. RESULTS: Twenty-six patients (N.=16, temporal lobe; N.=6, frontal lobe; N.=4, parietal lobe) with mean seizures duration of 56.9-months, were assessed. Histopathologically, N.=15 dysembryoplastic neurepithelial tumor, N.=7 ganglioglioma and N.=4 Diffuse lepto-meningeal neuroepithelial tumor. There were 2 cases of complex DNET and one case of DLMNT had associated cortical dysplasia. At mean follow-up of 49.7 months, N.=20 Engel 1, N.=4 Engel 2 and N.=2 had Engel 3 outcome. N.=20 underwent gross total excision (N.=18 Engel 1 and N.=2 Engel 2) and N.=6 sub-total excision. Among the 4 patients who needed re-surgery, two were in Engel 2 and another two were in Engel 3. CONCLUSIONS: Good seizure-outcome is likely associated with extent of resection. Younger age of patient, less than one-year of seizure duration and absence of generalization of seizure are good prognostic indicators. The best seizure-control can be achieved by early surgical intervention.

Suprasellar Ganglioglioma Arising from the Third Ventricle Floor: A Case Report and Review of the Literature.

Gangliogliomas are uncommon intracranial tumors that include neoplastic and abnormal ganglion cells, and show positive immunohistochemical staining for GFAP and syn. This type of lesion occurs more frequently in the temporal lobe than in other areas; they are extremely rare in the suprasellar region. To the best of our knowledge, including our case, 19 cases of GGs have been found in the suprasellar region. Among them, five tumors invaded the optic nerve, nine tumors invaded the optic chiasm, one tumor invaded the optic tract, and two tumors invaded the entire optic chiasmal hypothalamic pathway. In the present study, we describe the first case of suprasellar GGs arising from the third ventricle floor that was removed through the endoscopic endonasal approach. In addition, we summarize the clinical characteristics of GGs, such as age of onset, gender distribution, MRI signs, main clinical symptoms, and treatment methods for GG cases.

Peritoneal metastasis of a brainstem anaplastic ganglioglioma in a 2-year-old boy: case report and literature review.

Anaplastic gangliogliomas (AGG) are rare tumors of the central nervous system (CNS) that commonly affect children and young adults, with an unusual infratentorial presentation, which is related to hydrocephalus and a worse prognosis. We report a case of a brainstem AGG in a 2-year-old boy who underwent a ventriculoperitoneal shunting (VPS) and later presented peritoneal metastasis. We also reviewed the related literature. Even though rare, disease dissemination through VPS should be sought in patients with CNS tumors and VPS who develop new abdominal symptoms. The early diagnosis and intervention may minimize morbidity and improve quality of life of such patients.

Ganglioglioma revealed by spontaneous intracerebral hematoma: A cohort study.

BACKGROUND: Gangliogliomas are rare tumors of the central nervous system. Intracerebral hemorrhage (ICH) is a frequent pathological event, rarely related to intracranial tumor. Here we report a cohort of 14 gangliogliomas, 3 of which were revealed by ICH. We also performed a systematic review of the literature on gangliogliomas revealed by ICH. PATIENTS AND METHODS: We retrospectively collected data for all gangliogliomas operated on in our department between January 2015 and December 2019: clinical history, radiological data and outcome, with a special focus on gangliogliomas revealed by spontaneous ICH. All cases had pathological validation. For the systematic review, relevant studies were identified by systematic search of the scientific literature in PubMed. RESULTS: Fifteen patients underwent surgery for ganglioglioma during the study period. Six cases were revealed by seizures, 3 by headache, 1 by hydrocephaly and 1 by developmental disorder. Three patients (21%) had hemorrhagic presentation. All patients were male, aged 30 to 48 years, diagnosed with atypical ICH without vascular abnormality on cerebral angiogram. Conservative management was first decided. Diagnosis of ganglioglioma was made within 3 months for 2 patients and 9 years later for 1, after surgical removal and histological analysis. All hemorrhagic gangliogliomas were BRAF wild type. The systematic review of the literature identified 8 other cases of ganglioglioma revealed by ICH. CONCLUSION: ICH may be a mode of revelation of ganglioglioma, and ganglioglioma is a possible etiology of atypical ICH in young patients. Long-term imaging follow-up is mandatory in patients with unexplained ICH.

Challenges in the Intraoperative Consultation of Low-Grade Epilepsy-Associated Neuroepithelial Tumors by Cytomorphology in Squash Preparations.

INTRODUCTION: Low-grade epilepsy-associated neuroepithelial tumors (LEATs) create a diagnostic challenge in daily practice and intraoperative pathological consultation (IC) in particular. Squash smears are extremely useful in IC for accurate diagnosis; however, the knowledge on cytopathologic features of LEATs is based on individual case reports. Here, we discuss the 3 most common and well-established entities of LEATs: ganglioglioma (GG), dysembryoplastic neuroepithelial tumor (DNT), and papillary glioneuronal tumor (PGNT). METHODS: Thirty patients who underwent surgery for GG, DNT, and PGNT between 2001 and 2021 were collected. Squash smears prepared during intraoperative consultation were reviewed by 1 cytopathologist and an experienced neuropathologist. RESULTS: Among the 30 tumors, 16 (53.3%) were GG, 11 (36.6%) DNT, and 3 (10%) PGNT. Cytomorphologically, all of the 3 tumor types share 2 common features such as dual cell population and vasculocentric pattern. GG smears were characteristically composed of dysplastic ganglion cells and piloid-like astrocytes on a complex architectural background of thin- to thick-walled vessels. DNT, on the other hand, showed oligodendroglial-like cells in a myxoid thin fibrillary background associated with a delicate capillary network. Common cytological features of PGNT were hyperchromatic cells with narrow cytoplasm surrounding hyalinized vessels forming a pseudopapillary pattern and bland cells with neuroendocrine nuclei dispersed in a neuropil background. CONCLUSION: A higher diagnostic accuracy can be obtained when squash smears are applied with frozen sections. However, it is important to integrate clinical and radiologic features of the patient as well as to know the cytopathologic features of the LEAT spectrum in the context of differential diagnosis to prevent misinterpretation in the IC.

Anaplastic gangliogliomas of the spinal cord: a scoping review of the literature.

Gangliogliomas (GGs) are rare, usually low-grade tumors that account for 1-2% of all central nervous system (CNS) neoplasms. Spinal GGs are exceedingly rare (1% of all spinal tumors) and the presentation of anaplastic features in them is even rarer. According to the last World Health Organization (WHO) classification of CNS neoplasms, anaplastic GG (AGG) is classified as a malignant neoplasm (grade III). We performed a scoping review of the literature to elucidate the epidemiology, clinical features, histopathology, treatment, and outcome of primary spinal AGGs, which, to the best of our knowledge, is the first such review. Relevant studies were identified by a search of the MEDLINE and SCOPUS databases, using the following combination of search strings: (anaplastic ganglioglioma or malignant ganglioglioma or high grade ganglioglioma) AND (spine or spinal or spinal cord). We included studies related to primary or recurrent AGGs and malignant transformation of low-grade GGs. The search produced 15 eligible studies, plus two studies from the references, all of which were case reports of patients with spinal AGGs (17 studies with 22 patients). The mean age of the patients was 21.4 years and the sex ratio was 1:1, with male predominance. Motor impairment was the most common presentation, followed by sensory impairment, gait problems, urinary disturbances, and back pain. The thoracic spine was the most frequently involved area (14/22) followed by the cervical (6/22) and lumbar (5/22) spine. In terms of histology, the anaplastic features were usually predominant in the glial element, resembling high-grade astrocytomas, while the neuronal element was composed of the so-called dysplastic ganglion (neuronal) synaptophysin-positive cells, without mitotic figures. Complete surgical resection of the tumor without neurological compromise, plus adjuvant chemotherapy and radiotherapy, was the treatment protocol implemented in the two patients with the best outcome. Primary spinal AGG is an exceedingly rare entity, with only 22 cases being retrieved after an extensive literature search. They appear to affect children and young adults and tend to manifest aggressive behavior. Most studies report that only the glial component of AGGs presents high-grade malignant features, with low mitotic activity in the neuronal component. We therefore suggest that, pending novel targeted therapy, AGGs should be treated as high-grade gliomas, with an aggressive treatment protocol consisting of maximal safe resection and adjuvant chemotherapy and radiotherapy.