Neoplasia neuroepitelial bem diferenciada e de crescimento lento, composta por células ganglionares maduras e neoplásicas.
Introdução
O que você precisa saber de cara
Neoplasia neuroepitelial bem diferenciada e de crescimento lento, composta por células ganglionares maduras e neoplásicas.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 12 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 26 características clínicas mais associadas, ordenadas por frequência.
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
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Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Gangliocitoma
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
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Ensaios clínicos abertos e novidades científicas recentes
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Pesquisa e ensaios clínicos
12 ensaios clínicos encontrados, 2 ativos.
Publicações mais relevantes
Dysplastic cerebellar gangliocytoma: a six-decade study.
Dysplastic cerebellar gangliocytoma (DCG) is a rare cerebellar tumor glioneuronal and neuronal tumor with phosphatase and tensin homolog (PTEN) identified as a key altered gene. The aim of this study is to establish DCG diagnostic and outcome trends over a six-decade and present cases from our institution. A literature review of online databases was performed using relevant terms (January 1970-October 2024). Our institution pathology database was queried for patients with DCG (2000-2024). Data was extracted and dichotomized in adult and pediatric cases. We report three new DCG cases in addition to the 170 DCG cases reported across 97 articles over seven decades, with 52% of articles published since 2010. DCG predominantly occurred in adults (>18 years, 82%), women (59%), at mean age 9.0±6.3 years and 38.1±13.7 years in pediatric and adult patients, respectively. Balance/coordination deficit, headache and visual deficit were the most common presenting symptoms. MRI "tiger-stripe" pattern was present in 67%. Surgical resection was the treatment of choice for >97% of patients. Association with Cowden Syndrome (CS) was confirmed in 67% of patients. PTEN mutations identified in 48.3% of cases, primarily affecting chromosome 10 (10q23.3). Progression-free survival was experienced by 74% of patients and reoperation for progression needed in 3.5% of cases. DCG is a rare tumor associated with PTEN mutations. MRI aids in diagnosis and surgery ensures favorable outcomes. Standardized genetic screening and targeted therapies require further study, as they hold promise for refining diagnosis and long-term management.
Recurrent multiloculated cystic variant of Lhermitte-Duclos disease managed with cyst fenestration and Ommaya reservoir placement: illustrative case.
Lhermitte-Duclos disease (LDD), or dysplastic gangliocytoma of the cerebellum, is a rare benign lesion typically characterized by slow growth and absence of cystic changes. Cyst formation in LDD is distinctly uncommon, and detailed clinical courses of such cases are rarely documented. The authors describe a case of a young male with multiloculated cystic LDD in the right cerebellar hemisphere. The lesion recurred with progressive cyst enlargement, ultimately causing brainstem compression. Despite multiple resections and fenestrations, histopathological analysis consistently revealed characteristic features of LDD, including thickened cerebellar folia with reduced Purkinje and granular cells, dysplastic neurons, and a very low Ki-67 index (≤ 1%). Final management, including cyst fenestration and placement of an Ommaya reservoir, successfully restored CSF communication, preventing further enlargement and stabilizing neurological symptoms. Although LDD generally follows a benign course, multiloculated cystic variants can present with progressive recurrence and neurological deterioration. This case emphasizes the importance of recognizing cystic changes in LDD and demonstrates that adequate fenestration with communication to cisternal spaces, supplemented by Ommaya reservoir placement when necessary, can provide durable disease control. https://thejns.org/doi/10.3171/CASE25907.
Underwater Endoscopic Resection for the Rare Periampullary Tumor.
Composite gangliocytoma/neuroma and neuroendocrine tumors (CoGNETs) are extremely rare periampullary neoplasms composed of neuroendocrine epithelial, Schwannian spindle, and ganglion cells. Although endoscopic papillectomy or surgery have been used to treat ampullary CoGNETs, underwater endoscopic mucosal resection (UEMR) for periampullary lesions has not been previously described. In this study, we present a case of a 53-year-old man referred to our hospital with an incidentally detected subepithelial tumor in the second portion of the duodenum. Endoscopic ultrasound revealed a 13×10-mm homogeneous, hypoechoic, and well-demarcated submucosal mass. UEMR was performed under conscious sedation without submucosal injection, achieving safe en bloc resection within 12 min. Histopathology revealed a 2.0×1.5×1.2-cm lesion composed of mixed neuroendocrine epithelial, Schwannian spindle, and ganglion cells. Immunohistochemical staining was positive for chromogranin A, synaptophysin, CD56, neuron-specific enolase, and S-100, confirming CoGNET with a Ki-67 index of <1% and no lymphovascular or perineural invasion. Follow-up endoscopy 3 months later revealed only a post-resection scar without residual or recurrent tumors. This appears to be the first reported case of periampullary CoGNET successfully treated with UEMR. Therefore, UEMR might represent a safe and effective therapeutic option for selected periampullary subepithelial tumors.
Mixed gangliocytoma-pituitary neuroendocrine tumour: clinical, immunohistochemical, and molecular genetic profiles in a series of four patients.
The vast majority of tumours in the sellar region are pituitary neuroendocrine tumours, also called pituitary adenomas. Sellar gangliocytomas (GCs), benign tumours that originate from neuronal ganglionic cells, account for less than 1% of sellar tumours. Even rarer are mixed gangliocytoma-pituitary neuroendocrine tumours (GC-PitNET). These tumours are often associated with hormone hypersecretion, most commonly resulting in acromegaly. The histogenesis of mixed GC-PitNET is currently unclear. In this paper, we present comprehensive clinical, immunohistochemical, targeting enrichment next generation DNA sequencing, and genome-wide methylation data from four patients with mixed GC-PitNETs, three with acromegaly and one with Cushing's disease. Transcriptomic data are also included for two of the patients. Our findings indicate that mixed GC-PitNETs have different clinical course, with the acromegaly patients showing greater resistance to pharmacological therapy, as well as different protein expression and molecular features compared to respective pure PitNETs. The transcriptomic data on two patients with somatotroph GC-PitNET show involvement of mitochondrial and ribosomal genes, suggesting a distinct gene expression pattern, in comparison with pure somatotroph tumours. Furthermore, the expression pattern of selected stem cell markers, mainly SOX9, supports a common origin of the neuroendocrine and ganglionic tumour components, suggesting the involvement of stem cells in tumorigenesis.
Clinicopathological analysis of 19 cases of mixed gangliocytoma-pituitary neuroendocrine tumors.
Publicações recentes
Clinicopathological analysis of 19 cases of mixed gangliocytoma-pituitary neuroendocrine tumors.
Recurrent multiloculated cystic variant of Lhermitte-Duclos disease managed with cyst fenestration and Ommaya reservoir placement: illustrative case.
Underwater Endoscopic Resection for the Rare Periampullary Tumor.
Mixed gangliocytoma-pituitary neuroendocrine tumour: clinical, immunohistochemical, and molecular genetic profiles in a series of four patients.
Composite Gangliocytoma/Neuroma and Neuroendocrine Tumor (CoGNET) of the Duodenum: An Image in Endocrine Pathology Exploring Patterns of Biomarker Expression in Tumor Elements.
📚 EuropePMC218 artigos no totalmostrando 167
Clinicopathological analysis of 19 cases of mixed gangliocytoma-pituitary neuroendocrine tumors.
Diagnostic pathologyRecurrent multiloculated cystic variant of Lhermitte-Duclos disease managed with cyst fenestration and Ommaya reservoir placement: illustrative case.
Journal of neurosurgery. Case lessonsUnderwater Endoscopic Resection for the Rare Periampullary Tumor.
The Korean journal of helicobacter and upper gastrointestinal researchMixed gangliocytoma-pituitary neuroendocrine tumour: clinical, immunohistochemical, and molecular genetic profiles in a series of four patients.
Acta neuropathologica communicationsComposite Gangliocytoma/Neuroma and Neuroendocrine Tumor (CoGNET) of the Duodenum: An Image in Endocrine Pathology Exploring Patterns of Biomarker Expression in Tumor Elements.
Endocrine pathologyA Case of Mixed pitNET-Gangliocytoma of the Sella Turcica: Histopathologist's take on Extremely Rare Occurrence.
Pakistan journal of medical sciencesMixed gangliocytoma-silent somatotroph pituitary adenoma: a rare variant with pregnancy-associated growth.
BMJ case reportsDual Hormonal Presentation in a Rare Sellar Gangliocytoma: Diagnostic and Pathological Insights from a Collision Tumor.
Journal of neurological surgery reportsClinical spectrum and outcomes of anti-metabotropic glutamate receptor 5 encephalitis in Chinese patients: a case report and literature review.
Frontiers in immunologyCase Report: A case series of Lhermitte-Duclos disease with surgical intervention.
Frontiers in oncologyDysplastic cerebellar gangliocytoma: a six-decade study.
Journal of neurosurgical sciencesNuances in the surgical treatment of gangliocytomas of the spinal cord: illustrative cases.
Journal of neurosurgery. Case lessonsAtypical presentation of acute cerebellar infarct as Lhermitte-Duclos disease.
Surgical neurology internationalSurgical Strategy for Superior Cerebellar Peduncle Lesions: Utility of the Subtemporal Transtentorial Approach.
World neurosurgeryComparative analysis of growth hormone-secreting mixed gangliocytomas and pure growth hormone pituitary adenomas.
Clinical neurology and neurosurgeryComposite gangliocytoma/neuroma and neuroendocrine tumour: a contemporary analysis of 71 cases shows risk factors for metastasis.
HistopathologyDysplastic gangliocytoma of the cerebellum with an unusual radiological finding.
Ceskoslovenska patologieComposite gangliocytoma/neuroma and neuroendocrine tumor: a clinicopathologic, immunohistochemical, and molecular genetic study of 11 cases.
Virchows Archiv : an international journal of pathologyEndoscopic endonasal surgery using indocyanine green fluorescence endoscope for Cushing's disease caused by mixed intrasellar gangliocytoma and adrenocorticotropin adenoma: illustrative case.
Journal of neurosurgery. Case lessonsExtended Hormone Profiling Identifies a Wider Network of Duodenal Neuroendocrine Tumor Subtypes.
Endocrine pathologyUtility of Intraoperative Ultrasound in Surgical Management of Lhermitte-Duclos Disease: A Case Report.
Asian journal of neurosurgeryTwo illustrative cases of adult Lhermitte-Duclos disease and a systematic review of literature related to surgical management.
Brain & spineA Rare Case of Adult-onset Gangliocytoma in the Parietal Lobe: Case Report and Surveillance, Epidemiology, and End Results Registry Data Analysis.
NMC case report journalA Rare Case of Lhermitte Duclos Disease Associated with Somatic PTEN and Germline SUFU Variants.
Cerebellum (London, England)Composite Gangliocytoma/Neuroma and Neuroendocrine Tumor (CoGNET).
Mayo Clinic proceedingsAmpullary composite gangliocytoma/neuroma and neuroendocrine tumor management.
Endocrine-related cancerCauda Equina Neuroendocrine Tumors with Ganglioneuromatous Elements are Best Classified as Composite Gangliocytoma/Neuroma and Neuroendocrine Tumor (COGNET).
Endocrine pathologyPathologists' integration of prior biopsies of women with germline PTEN mutations may expedite the identification of this rare cancer predisposition syndrome.
PathologyMixed Gangliocytoma-Pituitary Adenoma: A Systematic Review of Diagnostic Features, Clinical Management, and Surgical Outcomes.
World neurosurgeryClinical Neurologic Features and Evaluation of PTEN Hamartoma Tumor Syndrome: A Systematic Review.
NeurologyTreatment and Diagnostic Approach for Lhermitte-Duclos Disease and Suspected Cowden Syndrome.
CureusLhermitte-Duclos Disease in an Eight-Year-Old Boy: A Case Report.
Cureus[MAX germline mutation-associated pheochromocytoma-paraganglioma syndrome: multiple endocrine neoplasia type 5].
Zhonghua wai ke za zhi [Chinese journal of surgery]Mixed pituitary adenoma/pituitary neuroendocrine tumor-gangliocytoma: Immunohistochemical insights.
Journal of neuropathology and experimental neurologyLhermitte-Duclos disease in a 51-year old patient.
Radiology case reports[Intraspinal composite gangliocytoma/neuroma and neuroendocrine tumor with vascular malformation: report of a case].
Zhonghua bing li xue za zhi = Chinese journal of pathologyCerebellar phenotypes in germline PTEN mutation carriers.
Neuropathology and applied neurobiologyClinical Characteristics, Diagnosis, and Treatment of Thyroid Stimulating Hormone-Secreting Pituitary Neuroendocrine Tumor (TSH PitNET): A Single-Center Experience.
Endocrinology and metabolism (Seoul, Korea)Anti-metabolic glutamate receptor 5 encephalitis with gangliocytoma: a case and review of the literature.
BMC neurologyLhermitte-Duclos disease with excessive calcification in a septuagenarian: A case report.
MedicineIgG4-associated hypophysitis coexisting with MALT lymphoma and gangliocytoma: first case report and literature review.
Frontiers in neurologyA case report of giant gangliocytoma of mediastinum.
AME case reportsLhermitte-Duclos disease: A systematic review.
Surgical neurology internationalAnesthetic Management for a Patient with Rosai-Dorfman Disease, Cowden Syndrome, and Lhermitte-Duclos Disease: An Extremely Rare Disease Combination.
CureusLhermitte-Duclos Disease: A Case Series.
CureusA young female of Cowden syndrome presenting with Lhermitte-Duclos disease: An illustrative case.
Surgical neurology internationalNeuropil-like islands are a possible pathogenetic link between glioblastoma and gangliocytoma/ganglioglioma in a case of synchronous bilateral brain tumors.
Neuropathology : official journal of the Japanese Society of NeuropathologyLhermitte-Duclos disease with concomitant KCNT2 gene mutation: report of an extremely rare combination.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryAssociation between Cowden syndrome and Lhermitte-Duclos disease: A case report of an uncommon Genetic Hamartomatous Disorder.
Radiology case reportsLhermitte-Duclos disease: A rare case of cerebellar tumor with successful surgical treatment.
Surgical neurology international[Treatment of intraspinal benign tumors in upper cervical vertebrae by modified recapping laminoplasty].
Zhongguo xiu fu chong jian wai ke za zhi = Zhongguo xiufu chongjian waike zazhi = Chinese journal of reparative and reconstructive surgeryLhermitte-Duclos disease: A series of six cases.
Journal of neurosciences in rural practiceA rare case of multiple paragangliomas in the head and neck, retroperitoneum and duodenum: A case report and review of the literature.
Frontiers in endocrinologyDiffuse paediatric cerebellar glioma: two identical imaging phenotypes of an extremely rare entity with disparate pathology.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryPosterior Fossa Intra-Axial Lesion: There Are Pathologies Beyond Metastases.
Asian journal of neurosurgeryCase report: Clinical and single-cell transcriptome sequencing analysis of a mixed gangliocytoma-adenoma presenting as acromegaly.
Frontiers in oncologyCauda Equina Neuroendocrine Tumors: Distinct Epithelial Neuroendocrine Neoplasms of Spinal Origin.
The American journal of surgical pathologyLhermitte-Duclos Disease: A Rare Cerebellar Hamartoma Presenting Following Traumatic Brain Injury And A Review Of The Literature.
Journal of Ayub Medical College, Abbottabad : JAMCA Case Report of Adrenal Teratoma: Huge but Easy to Misdiagnose.
Urologia internationalisAdvances in Adrenal and Extra-adrenal Paraganglioma: Practical Synopsis for Pathologists.
Advances in anatomic pathologyThe surgical resection of dysplastic cerebellar gangliocytoma assisted by intraoperative sonography: illustrative case.
Journal of neurosurgery. Case lessonsMultiple tumorous lesions of the pituitary gland.
Hormones (Athens, Greece)Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma) in the setting of cowden syndrome: a case report and literature review on COLD syndrome.
British journal of neurosurgeryGiant Pediatric Supratentorial Tumor: Clinical Feature and Surgical Strategy.
Frontiers in pediatricsUncommon Glioneuronal Tumors: A Radiologic and Pathologic Synopsis.
AJNR. American journal of neuroradiologyPrimary collision tumors of the sellar region: Experience from a single center.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaObservation of Clinicopathologic Features of Pituitary Adenoma With Neuronal Differentiation.
Frontiers in endocrinologyOverview of the 2022 WHO Classification of Neuroendocrine Neoplasms.
Endocrine pathologyOverview of the 2022 WHO Classification of Pituitary Tumors.
Endocrine pathologyOverview of the 2022 WHO Classification of Paragangliomas and Pheochromocytomas.
Endocrine pathologyA rare case of intraventricular gangliocytoma.
Arquivos de neuro-psiquiatriaMixed gangliocytoma-pituitary adenoma in MEN1 syndrome: A case report and literature review.
Neuropathology : official journal of the Japanese Society of NeuropathologyDysplastic gangliocytoma of the cerebellum in a cat.
Veterinary pathologyAdult gangliocytoma arising within the lateral ventricle: A case report and review of the literature.
Surgical neurology internationalMedulloblastoma and Cowden syndrome: Further evidence of an association.
Free neuropathologyDysplastic cerebellar gangliocytoma: a description of two cases.
Quantitative imaging in medicine and surgeryClinical-pathological study of 28 glial and mixed neuronal-glial tumors diagnosed within the first year of life.
Clinical neuropathologySellar Gangliocytoma: Case Report and Review of an Extremely Rare Tumour.
Case reports in neurologyHypothalamic hormone-producing tumors.
Handbook of clinical neurologyUnilateral hydrocephalus from a gangliocytoma-somatotrophinoma: first reported case.
Endocrinology, diabetes & metabolism case reportsAn Update on Pituitary Neuroendocrine Tumors Leading to Acromegaly and Gigantism.
Journal of clinical medicineAdult Langerhans Cell Histiocytosis Diagnosed by Biopsy of the Skull Tumor Generated after Craniotomy.
NMC case report journalEfficacy of One-stage Paravertebral Approach using a Micro-Tubular Technique in Treating Thoracic Dumbbell Tumors.
Orthopaedic surgeryPeriodic discharges with high frequency oscillations recorded from a cerebellar gangliocytoma in an epileptic infant.
Surgical neurology internationalLhermitte-Duclos Disease in Pediatric Population: Report of 2 Cases.
Pediatric neurosurgeryBCR-NTRK2 fusion in a pediatric patient with spinal gangliocytoma.
Pediatric blood & cancer[Dysplastic Cerebellar Gangliocytoma(Lhermitte-Duclos Disease)].
No shinkei geka. Neurological surgeryLongitudinal Assessment of Enhancing Foci of Abnormal Signal Intensity in Neurofibromatosis Type 1.
AJNR. American journal of neuroradiologyLhermitte-duclos disease (dysplastic cerebellar gangliocytoma): A case report.
JPMA. The Journal of the Pakistan Medical AssociationCerebellar gangliocytoma as a cause of fluctuating hearing loss.
Clinical case reportsRadiographic Findings of Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) in a Woman with Cowden Syndrome: A Case Study and Literature Review.
Journal of radiology case reportsRole of Long-Term Vestibular Rehabilitation in a Patient with Posterior Fossa Tumor: A Case Report with 2 Years of Follow-Up.
The American journal of case reportsPosttreatment Maturation of Medulloblastoma into Gangliocytoma: Report of 2 Cases.
Pediatric neurosurgeryRetrobulbar gangliocytoma and carcinoma of the corpuscles of Stannius in a pearl gourami, Trichopodus leerii (Bleeker, 1852).
Journal of fish diseasesPituitary Gangliocytoma Producing TSH and TRH: A Review of "Gangliocytomas of the Sellar Region".
The Journal of clinical endocrinology and metabolismDysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) presenting as a prenatally heterotopic hamartoma.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryBilateral Dysplastic Gangliocytoma with Concurrent Polyostotic Fibrous Dysplasia: A Case Report and Literature Review.
World neurosurgery[Design and clinical application of goal-oriented retroperitoneoscopic adrenalectomy].
Zhonghua yi xue za zhiAN INCIDENTAL COLLISION TUMOR OF THE SELLA TURCICA.
AACE clinical case reportsImaging of PTEN-related abnormalities in the central nervous system.
Clinical imagingResection of a Symptomatic Dysplastic Cerebellar Gangliocytoma: 2-Dimensional Operative Video.
Operative neurosurgery (Hagerstown, Md.)Clinicopathologic Features of Diencephalic Neuronal and Glioneuronal Tumors.
Journal of neuropathology and experimental neurologyRare occurrence of gangliocytoma in the medulla oblongata.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryHypothalamic Endocrine Tumors: An Update.
Journal of clinical medicineMixed gangliocytoma-pituitary adenoma containing GH and GHRH co-secreting adenoma cells.
Endocrinology, diabetes & metabolism case reportsThe Lhermitte-Duclos disease: a rare bilateral cerebellar location of a rare pathology.
The Pan African medical journalGlioneuronal Tumor With Features of Ganglioglioma and Neurocytoma Arising in the Fourth Ventricle: A Report of 2 Unusual Cases and a Review of Infratentorial Gangliogliomas.
Journal of neuropathology and experimental neurologyVery Unusual Sellar/Suprasellar Region Masses: A Review.
Journal of neuropathology and experimental neurologyComprehensive Evaluation of Rare Pituitary Lesions: A Single Tertiary Care Pituitary Center Experience and Review of the Literature.
Endocrine pathologyEarly Basal Cortisol Level as a Predictor of Hypothalamic-Pituitary-Adrenal (HPA) Axis Function After Pituitary Tumor Surgery.
Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes AssociationBilateral Recurrent Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) in Cowden Syndrome: A Case Report and Literature Review.
World neurosurgeryUpdated Imaging Features of Dysplastic Cerebellar Gangliocytoma.
Journal of computer assisted tomographyHypothalamic Vasopressin-Producing Tumors: Often Inappropriate Diuresis But Occasionally Cushing Disease.
The American journal of surgical pathologyClinical Perspective on Dysplastic Gangliocytoma of the Cerebellum (Lhermitte-Duclos Disease).
World neurosurgeryLhermitte-Duclos Disease in a Six-Year Old Child: A Rare Presentation.
Pediatric neurosurgeryPost-treatment maturation of medulloblastoma in children: two cases and a literature review.
The Journal of international medical researchSecuring Hemostasis in Pediatric Low-Grade Posterior Cerebral Fossa Tumors: The Value of Thrombin-Gelatin Hemostatic Matrix.
Pediatric neurosurgeryMixed gangliocytoma-pituitary adenoma in the sellar region: a large-scale single-center experience.
Acta neurochirurgicaMultinodular and vacuolating neuronal tumor incidentally discovered in a young man: Conventional and advanced MRI features.
Radiology case reportsAction Semantics at the Bottom of the Brain: Insights From Dysplastic Cerebellar Gangliocytoma.
Frontiers in psychologyGrowth hormone-secreting adenoma coexisted with gangliocytoma: a rare case.
International journal of clinical and experimental pathologyAutonomic seizures and déjà vu in a patient with gangliocytoma of the orbitofrontal cortex.
Psychiatria DanubinaA Case of Suspicious Gangliocytoma with Heterogeneously Distributed Lesions in the Thalamus and Basal Ganglia.
NMC case report journalNeurosurgical treatment of gangliogliomas in children and adolescents: long-term follow-up of a single-institution series of 32 patients.
Acta neurochirurgicaAn Unusual Case of Lhermitte-Duclos Disease Manifesting with Intratumoral Hemorrhage.
World neurosurgeryDysplasia and overgrowth: magnetic resonance imaging of pediatric brain abnormalities secondary to alterations in the mechanistic target of rapamycin pathway.
NeuroradiologyLhermitte-Duclos disease: an extremely rare cerebellar tumor.
The Pan African medical journalTranssphenoidal Surgery for Mixed Pituitary Gangliocytoma-Adenomas.
World neurosurgeryMultinodular and Vacuolating Neuronal Tumor of the Cerebrum: A New "Leave Me Alone" Lesion with a Characteristic Imaging Pattern.
AJNR. American journal of neuroradiology[Curative effect analysis of 15 cases of cervical intra-and extra-spinal tumors by microsurgical resection].
Zhonghua yi xue za zhiEndoscopic Endonasal Resection of a Mixed Lesion of Gangliocytoma and Nonfunctioning Pituitary Adenoma.
World neurosurgeryLhermitte-Duclos Disease and Cerebellar Gangliocytoma-An Incidental Finding in a Patient with Gradual Vision Loss.
Neuro-ophthalmology (Aeolus Press)Lhermitte-Duclos Disease (Dysplastic Gangliocytoma of the Cerebellum) and Cowden Syndrome: Clinical Experience From a Single Institution with Long-Term Follow-Up.
World neurosurgery[Hereditary tumor syndromes in neuropathology].
Der PathologeAn intrasellar pituitary adenoma-gangliocytoma presenting as acromegaly.
Endocrinology, diabetes & metabolism case reportsNoninvasive assessment of isocitrate dehydrogenase mutation status in cerebral gliomas by magnetic resonance spectroscopy in a clinical setting.
Journal of neurosurgeryChiari I malformation in a child with PTEN hamartoma tumor syndrome: Association or coincidence?
European journal of medical geneticsGangliocytoma: outcome of a rare silent pituitary tumour.
BMJ case reportsClinicopathological features and microsurgical outcomes for giant pediatric intracranial tumor in 60 consecutive cases.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryNeuroglial intramedullary tumors: The collaboration between neurosurgeons and neuropathologists.
Neuro-ChirurgieTransdifferentiation of Neuroendocrine Cells: Gangliocytoma Associated With Two Pituitary Adenomas of Different Lineage in MEN1.
The American journal of surgical pathologyMixed Gangliocytoma-Pituitary Adenoma: Insights on the Pathogenesis of a Rare Sellar Tumor.
The American journal of surgical pathologyLhermitte-Duclos disease: A rare entity.
Medical journal, Armed Forces IndiaInfantile Lhermitte-Duclos Disease Treated Successfully With Rapamycin.
Journal of child neurologyGangliocytoma Presenting With Tacrolimus Neurotoxicity in a Renal Transplant Recipient: Case Report.
Transplantation proceedingsLhermitte-Duclos disease with neurofibrillary tangles in heterotopic cerebral grey matter.
Folia neuropathologicaThe sellar and suprasellar region: A "hideaway" of rare lesions. Clinical aspects, imaging findings, surgical outcome and comparative analysis.
Clinical neurology and neurosurgeryMixed Pituitary Gangliocytoma and Prolactinoma Resistant to the Cabergoline Treatment.
World neurosurgeryGangliocytomas of the sellar region: A challenging diagnosis.
Clinical neurology and neurosurgeryCharacteristics of ganglion cells in pituitary gangliocytomas.
Neuropathology : official journal of the Japanese Society of NeuropathologyEpidermal growth factor receptor is overexpressed in neuroblastoma tissues and cells.
Acta biochimica et biophysica SinicaHigh-Definition Fiber Tractography in the Evaluation and Surgical Planning of Lhermitte-Duclos Disease: A Case Report.
World neurosurgeryHypothalamic hamartoma with neurofibrillary tangles.
Neuropathology : official journal of the Japanese Society of NeuropathologyGrowth hormone secreting pituitary adenoma with admixed gangliocytoma and ganglioglioma.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaManagement of unusual dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) in a developing country: Case report and review of the literature.
Asian journal of neurosurgeryA Rare Case of Concomitant Intramedullary Gangliocytoma at the Cervicomedullary Junction in Patient with Neuroendocrine Tumor of Lung.
Journal of Korean Neurosurgical SocietyChallenge in preoperative diagnosis of retroperitoneal mucinous cyst in a pediatric patient.
International journal of clinical and experimental medicinePituitary adenoma-neuronal choristoma is a pituitary adenoma with ganglionic differentiation.
Experimental and molecular pathologyGanglion cell tumours in the sella turcica in close morphological connection with pituitary adenomas.
Folia neuropathologicaTumor-to-tumor metastases in Cowden's disease: an autopsy case report and review of the literature.
Diagnostic pathologySellar gangliocytoma with adrenocorticotropic and prolactin adenoma.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaAutopsy case of undiagnosed gangliocytoma in the medulla oblongata complicated with cerebral palsy.
Legal medicine (Tokyo, Japan)Middle infratemporal fossa less invasive approach for radical resection of parapharyngeal tumors: surgical microanatomy and clinical application.
Neurosurgical reviewIntracranial hypertension and cerebellar symptoms due to Lhermitte-Duclos disease.
Iranian journal of neurologyEvaluation of amygdala pathology using (11)C-methionine positron emission tomography/computed tomography in patients with temporal lobe epilepsy and amygdala enlargement.
Epilepsy researchMalignant peripheral nerve sheath tumor in cowden syndrome: a first report.
Journal of neuropathology and experimental neurologyL'hermitte-Duclos disease in an elderly patient: A case report and review of the literature.
Asian journal of neurosurgeryAssociações
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Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Dysplastic cerebellar gangliocytoma: a six-decade study.
- Recurrent multiloculated cystic variant of Lhermitte-Duclos disease managed with cyst fenestration and Ommaya reservoir placement: illustrative case.
- Underwater Endoscopic Resection for the Rare Periampullary Tumor.The Korean journal of helicobacter and upper gastrointestinal research· 2026· PMID 41846450mais citado
- Mixed gangliocytoma-pituitary neuroendocrine tumour: clinical, immunohistochemical, and molecular genetic profiles in a series of four patients.
- Clinicopathological analysis of 19 cases of mixed gangliocytoma-pituitary neuroendocrine tumors.
- Composite Gangliocytoma/Neuroma and Neuroendocrine Tumor (CoGNET) of the Duodenum: An Image in Endocrine Pathology Exploring Patterns of Biomarker Expression in Tumor Elements.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:251937(Orphanet)
- MONDO:0016730(MONDO)
- GARD:10638(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q18555074(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
