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1.
OBJECTIVE AND IMPORTANCE: Gangliogliomas are intra-axial, avascular masses located predominantly in the temporal lobe. A ganglioglioma that mimics a meningioma in that it is extra-axial and has a significant extracranial vascular supply has not been reported previously. CLINICAL PRESENTATION: A 12-year-old girl presented with a right temporoparietal mass. A neurological examination revealed nothing abnormal, and the girl's symptoms were limited to headaches. INTERVENTION: Magnetic resonance imaging revealed an extra-axial mass, and cerebral catheter angiography revealed a blood supply mainly from the posterior division of the right middle meningeal artery. Intraoperative findings confirmed the extra-axial location of the tumor, and histological analysis revealed that the tumor was a ganglioglioma. CONCLUSION: This report confirms that gangliogliomas can present as extra-axial, vascular masses that are similar to meningiomas. 相似文献
2.
Intramedullary Spinal Cord Ganglioglioma: A Report of Five Cases 总被引:1,自引:0,他引:1
We report five cases of ganglioglioma in various locations. The tumours were removed with gross total resection in most cases without any further neurological deterioration. Without any additional treatment, no evidence of recurrence or regrowth of tumours was observed in the mean follow-up period of 4.1 years. We suggest that intramedullary spinal cord gangliogliomas be resected totally due to the high risk of recurrence and regrowth rate of the tumours with subtotal resection in the long term considering especially that these tumours affect predominantly young patients. 相似文献
3.
Amer F. Samdani Andrew Torre-Healy Alexander Khalessi Matthew McGirt George I. Jallo Benjamin Carson 《Acta neurochirurgica》2009,151(6):635-640
The following review of the literature describes the ganglioglioma, an uncommon mixed glioneuronal neoplasm, most often of
low-grade histology, with a small, albeit well-documented, malignant potential. These tumors exhibit a strong epileptogenic
propensity and most often present as new onset seizures or are discovered after a long history of refractory epilepsy. Despite
their indolent course, the importance of gross total resection is well recognized to prevent anaplastic and malignant degeneration.
Morphologically, the neoplasm is often cystic with an enhancing mural nodule, but can also be entirely solid. They are most
often found in the temporal lobe but have been found throughout the neuraxis. An exceedingly rare location of the ganglioglioma
is within the lateral ventricle. A systematic literature search revealed only eight reports documenting the occurrence of
a ganglioglioma within the lateral ventricle. We describe an illustrative case of an intraventricular ganglioglioma with a
prominent cystic component and enhancing mural nodule, which represents the classic radiographic appearance of gangliogliomas
described in other locations. A superior parietal lobule approach offered excellent surgical access for tumor removal and
the patient has remained free of neurological deficits following surgery. Regardless of location within the central nervous
system, ganglioglioma should be on the differential diagnosis for any cystic mass with a mural nodule, particularly in the
setting of epilepsy. 相似文献
4.
A Lagares P A Gómez R D Lobato J R Ricoy A Ramos A de la Lama 《Surgical neurology》2001,56(5):315-22; discussion 322-4
BACKGROUND: Brainstem gangliogliomas are rare low-grade tumors that usually have a long clinical history. However, they may cause sudden death. There are only 31 cases of brainstem ganglioglioma reported in the literature, and only one has been studied with magnetic resonance (MR). We present three new cases of brainstem ganglion cell tumor studied with computed tomography (CT) (3 cases) and MR (2 cases) and discuss the clinical presentation, diagnostic imaging and treatment of these tumors. CASE DESCRIPTION: Age at presentation ranged from 19 to 59 years old. Two patients were female and 1 male. Duration of symptoms before diagnosis ranged from 1 year to nearly 14 years. Presenting complaints included syncope spells, cranial nerve deficits, headache, and gait instability. Imaging studies revealed well-circumscribed lesions involving the brainstem; the lesion was cystic in one case and calcified in one. They were iso- or hyperdense on CT scan, isodense on T1-weighted and hyperdense on T2-weighted MRI and frequently showed contrast enhancement.All tumors were operated through a posterior fossa craniectomy. Using microsurgical techniques only partial resection could be achieved, as there was no sharp delineation from the surrounding tissue in any case. Two of our patients had increased neurological deficits after surgery. Radiotherapy was not given. Follow-up of tumoral remnants has not shown clear tumor growth after 1, 3.5, and 10 years. CONCLUSIONS: Imaging characteristics of brainstem gangliogliomas do not seem to differ from those in other locations and are not specific. Radical surgery is rarely if ever possible, nor is it advisable because of the risk of functional deterioration. However, because of their benign histology, partial resection seems to carry a similar prognosis as tumors in other locations that are amenable to complete resection. 相似文献
5.
The authors present the case of a 33-year-old patient with a bifocal ganglioglioma located in the right superior temporal
gyrus. He had a history of tonic–clonic seizures and developed intermittent nausea and vertigo later on. Magnetic resonance
imaging showed two distinct, small lesions in the right temporal lobe. Both tumors were removed microsurgically with ultrasound
guidance. Intraoperatively, two distinct tumors were found. Histological diagnosis of both tumors was of ganglioglioma WHO
II. Postoperatively, the patient was free of symptoms. Bifocal occurrence or the coincidence of two distinct gangliogliomas
is a very uncommon finding. So far, it has not yet been reported in benign gangliogliomas.
Received: 16 December 1998 / Accepted: 12 January 1999 相似文献
6.
Neuroblastomatous recurrence of ganglioglioma. Case report 总被引:3,自引:0,他引:3
This case is believed to be the first reported recurrent intracranial ganglioglioma with purely neuroblastomatous malignant transformation. A complete macroscopic resection of a right frontal lobe tumor in an 18-year-old woman revealed differentiated ganglioglioma. Seven years later a large, well-demarcated recurrent tumor was again macroscopically totally resected in the same patient. Histological analysis showed malignant transformation in only the neuronal component of the original tumor. A review of the literature on recurrent gangliogliomas and their malignant transformation is included. 相似文献
7.
Kinoshita Y Kiya K Satoh H Sadatomo T Mizoue T Tsumura R Shirozu H Sugiyama K Kurisu K 《No shinkei geka. Neurological surgery》2002,30(5):503-507
Gangliogliomas represent approximately 0.2% of all the intracranial tumors. Ganglioglioma arising from the cerebellum is rare, with a rate of 1.5-9% of CNS gangliogliomas. The authors report a case of cerebellar ganglioglioma with a huge cyst. A 28-year-old man presented headache and ataxia. Computerized tomography (CT) demonstrated a huge cyst at the vermian region with calcification located at the peripheral side of the cyst. Magnetic resonance imaging (MRI) with administration of Gd-DTPA showed a slightly enhanced small mass at the left side of the cyst. Preoperative diagnosis of the lesion seemed to be a cystic astrocytoma. The tumor was removed subtotally through the midline suboccipital approach. Pathological examination of the tumor specimen revealed a ganglioglioma. The postoperative course was uneventful with no sign of tumor regrowth on repeated MRI. According to the previous 17 reports of cerebellar ganglioglioma including our case, the shorter interval from onset to the diagnosis and clinical symptoms such as increased intracranial pressure were conspicuous as compared with supratentorial ganglioglioma because of the anatomically narrow space of the posterior fossa. Neuroradiological findings showed tumor enhancement in 86% of the cases, calcification in 67%, and cyst formation in 53%. 相似文献
8.
《Neuro-Chirurgie》2022,68(5):e8-e15
BackgroundGangliogliomas 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 methodsWe 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.ResultsFifteen 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.ConclusionICH 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. 相似文献
9.
Y Hayashi M Iwato M Hasegawa O Tachibana A von Deimling J Yamashita 《Journal of neurosurgery》2001,95(1):138-142
A gangliocytoma/ganglioglioma with no atypical or malignant features was subtotally resected from the right temporal lobe of a 16-year-old woman. A second resection was performed 8 years later to treat a locally recurrent lesion with increased cellularity that was diagnosed as a World Health Organization Grade II ganglioglioma on the basis of neuropathological examination. Molecular analysis of the recurrent tumor revealed a TP53 gene mutation, but no amplification of the epidermal growth factor receptor (EGFR) gene. Radiotherapy (60 Gy) was administered after the second resection. The patient returned 1 year later with a second focal recurrence. The specimen obtained during the third resection of tumor exhibited exclusively astrocytic differentiation, cellular pleomorphism with multinucleated cells, high mitotic activity, and endothelial proliferation. Therefore, the tumor was diagnosed to be a glioblastoma multiforme (GBM). Molecular analysis of tumor DNA from the second recurrent tumor demonstrated the presence of the TP53 mutation, which previously had been observed in the first recurrent tumor, but again no evidence of EGFR amplification. Findings demonstrate that the presence of TP53 mutation in progressed gangliogliomas should be interpreted as a progression-associated mutation rather than a consequence of treatment. This is the first report to indicate that the molecular pathways of gangliocytomas/gangliogliomas progressing to become GBMs may parallel those of diffuse astrocytomas progressing to become GBMs. 相似文献
10.
Desmoplastic infantile gangliogliomas (DIG) are uncommon supratentorial brain tumors with a usually good prognosis despite
an aggressive radiological appearance that typically occurs in infants below the age of 24 months. DIGs are exclusively supratentorial,
generally have a voluminous size, and are partially cystic. Total surgical removal is sufficient for the treatment of these
tumors, and no chemotherapy or radiotherapy is indicated if complete resection is achieved. Except for age difference, DIG
and desmoplastic non-infantile gangliogliomas are radiologically and histologically similar. Non-infantile variants of this
biologically benign intracranial neoplasm are rare; only 15 cases of non-infantile DIGs have been reported in the literature.
As far as we know, this case is the 16th in literature. We reported the magnetic resonance imaging and histological findings
of desmoplastic ganglioglioma in a 6-year-old boy. 相似文献
11.
Summary Gangliogliomas are glioneuronal tumours of the young, and occur more frequently supratentorially. Among those uncommon cases
in the spinal cord, the conus medullaris is an extremely rare site. We reviewed the literature of such cases and report another
case of a ganglioglioma of the conus in a 13-year-old boy with an insidious sensory sensitive and motor deficit of the right
lower limb. Subtotal resection was accomplished with no recurrence after two years of follow-up. As for the supratentorial
locations, the extent of surgical resection is the main prognostic factor for gangliogliomas of the spinal cord, and they
should be considered in the differential diagnosis of intramedullar neoplasm. 相似文献
12.
Desmoplastic gangliogliomas (DIG) are rare primary neoplasms that comprise 0.5–1.0% of all intracranial tumors. Clinically,
there are two forms of DIG, the infantile and the non-infantile. These tumors invariably arise in the supratentorial region
and commonly involve more than one lobe, preferentially the temporal and frontal. On neuroimaging are seen as large hypodense
cystic masses with a solid isodense or slightly hyperdense superficial portion. The histologic diagnosis is characterized
by the presence of three different cell lines: astrocytic, neuronal, and primitive neuroectodermal marker sites, which were
demonstrable. The treatment of choice is radical surgical excision, and if this is done, achieved complete healing of the
patient does not require additional treatment. A literature review of DIG was compiled through Medline/Ovid using the keywords
“desmoplastic infantile ganglioglioma”, “desmoplastic non-infantile ganglioglioma” covering the years 1984–2009. We present
a review of a total of 113 cases of infantile (94) and non-infantile gangliogliomas (19) published to date, examining the
clinical, radiologic, surgical, and pathological aspects, as well as the outcome. Desmoplastic gangliogliomas represent a
rare tumor group with two well-defined age groups, the children and non-children. Desmoplastic infantile gangliogliomas are
the most common and occur in children below 5 years of age, and the large majority of them present within the first year of
life. Surgery is the treatment of choice and no complementary treatment is needed in cases of complete tumor resection. 相似文献
13.
Nael Al-Sarraf Rashid Aziz Kathy Gately Julie Lucey Lorraine Wilson Eillish McGovern Vincent Young 《European journal of cardio-thoracic surgery》2008,33(1):104-109
OBJECTIVE: We sought to assess the incidence, pattern and predictors of occult mediastinal lymph node involvement (N2) in non-small cell lung cancer patients with negative mediastinal uptake of 2-deoxy-2-[(18)F]-fluoro-d-glucose ((18)FDG) on integrated positron emission tomography-computerised tomography (PET-CT). METHODS: All patients who underwent surgical resection in our unit over a 30-month period were reviewed (n=215). All patients had preoperative PET-CT prior to lung resection as an adjunct to a dedicated chest CT. Diabetic patients, patients who received neoadjuvant chemotherapy and those with positive mediastinal nodes on PET-CT (N2/N3) were excluded from this study. The population of interest was 153 non-small cell cancer patients (NSCLC), all of which had no FDG uptake in the mediastinum. No preoperative mediastinoscopy was carried out in this group and all underwent curative intent surgical resection. The pathological results were retrospectively reviewed and correlated with CT and integrated PET-CT findings. RESULTS: The incidence of occult N2 disease in NSCLC patients with negative mediastinal uptake of (18)FDG on PET-CT was 16% (25 of 153). The highest incidence of occult N2 involvement was in American thoracic society (ATS) 7 (16 of 25 patients, 64%) followed by ATS 4 (seven patients of 25, 28%). In univariate analysis, the following were significant predictors of occult N2 disease: centrally located tumours (P=0.049), right upper lobe tumours (P=0.04), enlarged lymph nodes (>1cm) on CT (P=0.048) and PET positive uptake in N1 nodes (P=0.006). In multivariate analysis, the following were independent predictors of occult N2 disease: centrally located tumours, right upper lobe tumours and PET positive uptake in N1 nodes (P<0.05). CONCLUSIONS: In NSCLC patients who are clinically staged as N2/N3 negative in the mediastinum by integrated PET-CT, 16% will have occult N2 disease following resection. Patients with the following: centrally located tumours, right upper lobe tumours and positive N1 nodes on PET should have preoperative cervical mediastinoscopy to rule out N2 nodal involvement, especially in ATS stations 7 and 4 as the incidence of occult nodal metastasis in these nodes is high. This study has potential implications in decision-making and planning best treatment approach. 相似文献
14.
Desmoplastic gangliogliomas are rare mixed glial and neuronal cerebral tumors, especially described in infants below 4 years of age but exceptional cases have been reported in young adults. These tumors are generally localised in parietal or temporal lobes, present as a large cystic lesion with peripheral contrast enhancement. They also have characteristic histological features: extensive desmoplasia and tumoral cells of variable size exhibiting immunohistochemical and ultrastructural features of glial and neuronal differentiation. Total surgical removal is sufficient for the treatment of these tumors and no radiotherapy or chemotherapy are indicated if complete resection is achieved. We report a case of desmoplastic ganglioglioma in a 19-year-old male. This tumor presented as a large parieto- temporal cystic lesion with rimmed contrast enhancement. At histological examination, this tumor exhibited extensive desmoplasia and comprised 2 types of tumoral cells: small cells with round nuclei, positive for NSE, neurofilaments and synaptophysin and sometimes presenting typical morphological features of neuronal differentiation, and large cells with abundant eosinophilic strongly staining for GFAP. This observation emphazises on the fact that desmoplastic ganglioglioma can no more be considered as a specific entity of infancy and must be well recognised even in young adults because it may be misdiagnosed as malignant glioma. 相似文献
15.
Intracerebral ganglioglioma: clinical and radiological study of eleven surgically treated cases with follow-up 总被引:6,自引:0,他引:6
Background. Gangliogliomas are rare benign tumors of the CNS consisting of differentiated neural elements and low-grade glial cells. Methods. We reviewed our experience of 11 patients with histologically proven ganglioglioma who were surgically treated since 1986 at Çukurova University Medical Center. These patients presented at 18 to 45 years of age. Five were women and six were men. The most common initial symptom was seizures (in nine of 11 patients), which had sometimes persisted over long periods of time. At the time of diagnosis, four patients had focal neurological deficits and three had signs or symptoms of increased intracranial pressure. The cystic and well-circumscribed characteristics of these lesions were detected on computed tomography (CT). Despite their appearance on CT, all but one of the lesions were found to be mostly solid at operation. Magnetic resonance imaging (MRI) in six patients revealed abnormally high signal intensity on T2-imaging. The temporal lobe was the main tumor location (seven patients). All cases were diagnosed according to the Russel and Rubinstein histological criteria for ganglioglioma. Results. Ten patients had radical total resection and one had subtotal resection. No patient underwent postoperative radiation or chemotherapy. Except for one, all are still alive and free of progressive disease 1 to 11 years (mean 6.2) after operation. Six are seizure-free and three have improved seizure control under anticonvulsant therapy. Conclusions. We conclude that ganglioglioma is a distinct histological phenomenon with mildly predictable clinical symptoms (seizures), mildly characteristic radiological features, and long-term survival after surgical resection without the need of adjuvant treatment such as radiotherapy. 相似文献
16.
A 6-month-old girl had a gradually increasing head circumference. A preoperative computed tomographic (CT) scan of the head revealed an enhancing calcified partially cystic right frontal mass that was removed through a right frontotemporal craniotomy. On microscopic examination, the tumor was composed of sheets of neurons in a glial background alternating with highly cellular anaplastic areas. The diagnosis of anaplastic ganglioglioma was made. The child has done well for the 20 months since the operation without any evidence of tumor recurrence on subsequent CT scans. Because of the immaturity of the child's developing central nervous system, we have elected not to initiate radiotherapy at this time. The pertinent literature regarding gangliogliomas is reviewed. 相似文献
17.
Delphine Trousse Xavier Beno?t D'Journo Jean-Philippe Avaro Christophe Doddoli Roger Giudicelli Pierre Antoine Fuentes Pascal Alexandre Thomas 《European journal of cardio-thoracic surgery》2008,33(1):99-103
OBJECTIVE: T4-disease for non-small cell lung cancer (NSCLC) includes different conditions: mediastinal invasion, neoplastic pleural cytology, and multifocal disease in the same lobe; regarding the last category, no strict criteria allow to differentiate satellite nodules from synchronous multiple primary tumours. METHODS: Retrospective study of 56 patients who underwent a complete resection from 1985 to 2006 of a NSCLC graded pT4N0 due to multifocal disease. A small nodule (<1cm) closed to the primary tumour, in a same pulmonary segment with an identical histology was considered as a satellite nodule (pT4sn). Multiple tumours, sized more than 1cm, with an identical histology, located in the same lobe but in different segment were considered as synchronous cancers (pT4sc). RESULTS: There were 44 males and 12 females: 35 patients were graded T4sn and 21 patients T4sc. The median age was 62.5 years. The two groups were similar for sex, age, tobacco consumption, ASA score, NYHA, Charlson's index, spirometric parameters, cardiovascular comorbidity and history of previous extra-thoracic malignancies. All had a complete anatomic resection with mediastinal lymphadenectomy. Thirty-day mortality rate was 3.6%. Overall 5-year and 10-year survival rates were 48.2% and 29.9%, respectively. There was a non-significant trend for a worse survival in T4sn group patients when compared to that of T4sc group patients: 42.9% vs 52.3% at 5 years, and 25% vs 34.9% at 10 years (p=0.62). CONCLUSIONS: Multifocal T4 stage IIIB disease is a heterogeneous category where overall prognosis is far better than those of other T4 subgroups. Survival rates associated with pT4sn and pT4sc look roughly similar because of the small size of the subgroups usually submitted to comparison in most series. In the present experience, respective survival figures diverge, suggesting different biological behaviours. 相似文献
18.
Ganglioglioma of the brain stem. Case report 总被引:2,自引:0,他引:2
A case of brain-stem ganglioglioma is reported. A review of the literature revealed only 13 other reported cases. Brain-stem gangliogliomas usually become symptomatic in the first and second decades of life and involve the medulla and pons. Clinical improvement and prolonged survival have been reported after partial resection. 相似文献
19.
The records of eight patients treated for intestinal obstruction resulting from bands that have no identifiable embryologic or acquired basis were reviewed retrospectively. All patients presented with symptoms and signs indicative of intestinal obstruction. Patients older than 2 years of age additionally had a history of chronic abdominal pain. One thick anomalous congenital band with blood vessels in it was found to be the cause of obstruction in each patient. Bands were located between ascending colon and terminal ileum in four patients (50%), ligament of Treitz and terminal ileum in two patients (25%), right lobe of liver and terminal ileum in one patient (12.5%), and right lobe of liver and ascending colon in one patient (12.5%). The obstructive mechanisms were compression of bowel by band in five patients (62.5%) and entrappment of an intestinal loop between the band and mesenterium in three patients (37.5%). These bands are suggested to be the anomalies of mesenterium that may cause intestinal obstruction and chronic abdominal pain in children. 相似文献
20.
Ganglioglioma is usually a well differentiated slowly growing mixed neuronal and glial neoplasm corresponding to WHO grade I or II. However, some gangliogliomas are considered to be WHO grade III because they exhibit anaplastic features in their glial component. Finally there are exceptionally rare cases of newly diagnosed gangliogliomas with grade IV changes in the glial component. We report a case of a 25-year-old woman with a family history of neurofibromatosis who presented initially with a World Health Organization grade IV anaplastic ganglioglioma (a mixed ganglion cell multiform tumor glioblastoma). Despite aggressive management, the patient died of disease in a relatively short period. Histologically, two cell populations were noted: a predominant glial component consisting in a multiform glioblastoma and ganglion cells supporting a diagnosis of ganglioglioma. Immunohistochemical analysis clearly distinguished the two tumor cell populations. Although other cases of grade III gangliogliomas and twelve cases of grade IV gangliogliomas have been reported, the present case is exceptional in that, to our knowledge, it is the second case of a patient who presented initially with a composite grade IV ganglioglioma and who was clinically followed up to the time of death. This case allows direct comparison between the histological findings in a multiform glioblastoma and a ganglioglioma. It also documents the aggressive biologic behavior of this complex neoplasm. 相似文献