Raras
Buscar doenças, sintomas, genes...
Gangliocitoma
ORPHA:251937CID-10 · D36.1CID-11 · 2A00.21DOENÇA RARA

Neoplasia neuroepitelial bem diferenciada e de crescimento lento, composta por células ganglionares maduras e neoplásicas.

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Introdução

O que você precisa saber de cara

📋

Neoplasia neuroepitelial bem diferenciada e de crescimento lento, composta por células ganglionares maduras e neoplásicas.

Pesquisas ativas
2 ensaios
12 total registrados no ClinicalTrials.gov
Publicações científicas
428 artigos
Último publicado: 2026 Mar 23

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
<1 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Adolescent
+ childhood, infancy
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: D36.1
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

📏
Crescimento
5 sintomas
🧠
Neurológico
5 sintomas
🦴
Ossos e articulações
3 sintomas
💪
Músculos
1 sintomas

+ 12 sintomas em outras categorias

Características mais comuns

90%prev.
Ganglioneuroma
Muito frequente (99-80%)
55%prev.
Hemianopsia
Frequente (79-30%)
55%prev.
Neoplasia do sistema nervoso central
Frequente (79-30%)
55%prev.
Cefaleia
Frequente (79-30%)
55%prev.
Tumor da medula espinhal
Frequente (79-30%)
17%prev.
Adenoma de células nulas hipofisárias
Ocasional (29-5%)
26sintomas
Muito frequente (1)
Frequente (4)
Ocasional (15)
Muito raro (6)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 26 características clínicas mais associadas, ordenadas por frequência.

Ganglioneuroma
Muito frequente (99-80%)90%
HemianopsiaHemianopia
Frequente (79-30%)55%
Neoplasia do sistema nervoso centralNeoplasm of the central nervous system
Frequente (79-30%)55%
CefaleiaHeadache
Frequente (79-30%)55%
Tumor da medula espinhalSpinal cord tumor
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico428PubMed
Últimos 10 anos167publicações
Pico202522 papers
Linha do tempo
2026Hoje · 2026🧪 2013Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado1
3Fase 31
2Fase 21
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 3 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Gangliocitoma

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

12 ensaios clínicos encontrados, 2 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
169 papers (10 anos)
#1

Dysplastic cerebellar gangliocytoma: a six-decade study.

Journal of neurosurgical sciences2026 Feb

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.

#2

Recurrent multiloculated cystic variant of Lhermitte-Duclos disease managed with cyst fenestration and Ommaya reservoir placement: illustrative case.

Journal of neurosurgery. Case lessons2026 Mar 23

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.

#3

Underwater Endoscopic Resection for the Rare Periampullary Tumor.

The Korean journal of helicobacter and upper gastrointestinal research2026 Mar

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.

#4

Mixed gangliocytoma-pituitary neuroendocrine tumour: clinical, immunohistochemical, and molecular genetic profiles in a series of four patients.

Acta neuropathologica communications2026 Jan 30

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.

#5

Clinicopathological analysis of 19 cases of mixed gangliocytoma-pituitary neuroendocrine tumors.

Diagnostic pathology2026 Mar 23

Publicações recentes

Ver todas no PubMed

📚 EuropePMC218 artigos no totalmostrando 167

2026

Clinicopathological analysis of 19 cases of mixed gangliocytoma-pituitary neuroendocrine tumors.

Diagnostic pathology
2026

Recurrent multiloculated cystic variant of Lhermitte-Duclos disease managed with cyst fenestration and Ommaya reservoir placement: illustrative case.

Journal of neurosurgery. Case lessons
2026

Underwater Endoscopic Resection for the Rare Periampullary Tumor.

The Korean journal of helicobacter and upper gastrointestinal research
2026

Mixed gangliocytoma-pituitary neuroendocrine tumour: clinical, immunohistochemical, and molecular genetic profiles in a series of four patients.

Acta neuropathologica communications
2026

Composite Gangliocytoma/Neuroma and Neuroendocrine Tumor (CoGNET) of the Duodenum: An Image in Endocrine Pathology Exploring Patterns of Biomarker Expression in Tumor Elements.

Endocrine pathology
2025

A Case of Mixed pitNET-Gangliocytoma of the Sella Turcica: Histopathologist's take on Extremely Rare Occurrence.

Pakistan journal of medical sciences
2025

Mixed gangliocytoma-silent somatotroph pituitary adenoma: a rare variant with pregnancy-associated growth.

BMJ case reports
2025

Dual Hormonal Presentation in a Rare Sellar Gangliocytoma: Diagnostic and Pathological Insights from a Collision Tumor.

Journal of neurological surgery reports
2025

Clinical spectrum and outcomes of anti-metabotropic glutamate receptor 5 encephalitis in Chinese patients: a case report and literature review.

Frontiers in immunology
2025

Case Report: A case series of Lhermitte-Duclos disease with surgical intervention.

Frontiers in oncology
2026

Dysplastic cerebellar gangliocytoma: a six-decade study.

Journal of neurosurgical sciences
2025

Nuances in the surgical treatment of gangliocytomas of the spinal cord: illustrative cases.

Journal of neurosurgery. Case lessons
2025

Atypical presentation of acute cerebellar infarct as Lhermitte-Duclos disease.

Surgical neurology international
2025

Surgical Strategy for Superior Cerebellar Peduncle Lesions: Utility of the Subtemporal Transtentorial Approach.

World neurosurgery
2025

Comparative analysis of growth hormone-secreting mixed gangliocytomas and pure growth hormone pituitary adenomas.

Clinical neurology and neurosurgery
2025

Composite gangliocytoma/neuroma and neuroendocrine tumour: a contemporary analysis of 71 cases shows risk factors for metastasis.

Histopathology
2025

Dysplastic gangliocytoma of the cerebellum with an unusual radiological finding.

Ceskoslovenska patologie
2025

Composite gangliocytoma/neuroma and neuroendocrine tumor: a clinicopathologic, immunohistochemical, and molecular genetic study of 11 cases.

Virchows Archiv : an international journal of pathology
2025

Endoscopic 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 lessons
2025

Extended Hormone Profiling Identifies a Wider Network of Duodenal Neuroendocrine Tumor Subtypes.

Endocrine pathology
2025

Utility of Intraoperative Ultrasound in Surgical Management of Lhermitte-Duclos Disease: A Case Report.

Asian journal of neurosurgery
2025

Two illustrative cases of adult Lhermitte-Duclos disease and a systematic review of literature related to surgical management.

Brain &amp; spine
2025

A Rare Case of Adult-onset Gangliocytoma in the Parietal Lobe: Case Report and Surveillance, Epidemiology, and End Results Registry Data Analysis.

NMC case report journal
2025

A Rare Case of Lhermitte Duclos Disease Associated with Somatic PTEN and Germline SUFU Variants.

Cerebellum (London, England)
2025

Composite Gangliocytoma/Neuroma and Neuroendocrine Tumor (CoGNET).

Mayo Clinic proceedings
2025

Ampullary composite gangliocytoma/neuroma and neuroendocrine tumor management.

Endocrine-related cancer
2024

Cauda Equina Neuroendocrine Tumors with Ganglioneuromatous Elements are Best Classified as Composite Gangliocytoma/Neuroma and Neuroendocrine Tumor (COGNET).

Endocrine pathology
2025

Pathologists' integration of prior biopsies of women with germline PTEN mutations may expedite the identification of this rare cancer predisposition syndrome.

Pathology
2025

Mixed Gangliocytoma-Pituitary Adenoma: A Systematic Review of Diagnostic Features, Clinical Management, and Surgical Outcomes.

World neurosurgery
2024

Clinical Neurologic Features and Evaluation of PTEN Hamartoma Tumor Syndrome: A Systematic Review.

Neurology
2024

Treatment and Diagnostic Approach for Lhermitte-Duclos Disease and Suspected Cowden Syndrome.

Cureus
2024

Lhermitte-Duclos Disease in an Eight-Year-Old Boy: A Case Report.

Cureus
2024

[MAX germline mutation-associated pheochromocytoma-paraganglioma syndrome: multiple endocrine neoplasia type 5].

Zhonghua wai ke za zhi [Chinese journal of surgery]
2024

Mixed pituitary adenoma/pituitary neuroendocrine tumor-gangliocytoma: Immunohistochemical insights.

Journal of neuropathology and experimental neurology
2024

Lhermitte-Duclos disease in a 51-year old patient.

Radiology case reports
2024

[Intraspinal composite gangliocytoma/neuroma and neuroendocrine tumor with vascular malformation: report of a case].

Zhonghua bing li xue za zhi = Chinese journal of pathology
2024

Cerebellar phenotypes in germline PTEN mutation carriers.

Neuropathology and applied neurobiology
2024

Clinical Characteristics, Diagnosis, and Treatment of Thyroid Stimulating Hormone-Secreting Pituitary Neuroendocrine Tumor (TSH PitNET): A Single-Center Experience.

Endocrinology and metabolism (Seoul, Korea)
2024

Anti-metabolic glutamate receptor 5 encephalitis with gangliocytoma: a case and review of the literature.

BMC neurology
2024

Lhermitte-Duclos disease with excessive calcification in a septuagenarian: A case report.

Medicine
2023

IgG4-associated hypophysitis coexisting with MALT lymphoma and gangliocytoma: first case report and literature review.

Frontiers in neurology
2023

A case report of giant gangliocytoma of mediastinum.

AME case reports
2023

Lhermitte-Duclos disease: A systematic review.

Surgical neurology international
2023

Anesthetic Management for a Patient with Rosai-Dorfman Disease, Cowden Syndrome, and Lhermitte-Duclos Disease: An Extremely Rare Disease Combination.

Cureus
2023

Lhermitte-Duclos Disease: A Case Series.

Cureus
2023

A young female of Cowden syndrome presenting with Lhermitte-Duclos disease: An illustrative case.

Surgical neurology international
2024

Neuropil-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 Neuropathology
2023

Lhermitte-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 Neurosurgery
2023

Association between Cowden syndrome and Lhermitte-Duclos disease: A case report of an uncommon Genetic Hamartomatous Disorder.

Radiology case reports
2023

Lhermitte-Duclos disease: A rare case of cerebellar tumor with successful surgical treatment.

Surgical neurology international
2023

[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 surgery
2023

Lhermitte-Duclos disease: A series of six cases.

Journal of neurosciences in rural practice
2022

A rare case of multiple paragangliomas in the head and neck, retroperitoneum and duodenum: A case report and review of the literature.

Frontiers in endocrinology
2023

Diffuse 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 Neurosurgery
2022

Posterior Fossa Intra-Axial Lesion: There Are Pathologies Beyond Metastases.

Asian journal of neurosurgery
2022

Case report: Clinical and single-cell transcriptome sequencing analysis of a mixed gangliocytoma-adenoma presenting as acromegaly.

Frontiers in oncology
2023

Cauda Equina Neuroendocrine Tumors: Distinct Epithelial Neuroendocrine Neoplasms of Spinal Origin.

The American journal of surgical pathology
2022

Lhermitte-Duclos Disease: A Rare Cerebellar Hamartoma Presenting Following Traumatic Brain Injury And A Review Of The Literature.

Journal of Ayub Medical College, Abbottabad : JAMC
2022

A Case Report of Adrenal Teratoma: Huge but Easy to Misdiagnose.

Urologia internationalis
2023

Advances in Adrenal and Extra-adrenal Paraganglioma: Practical Synopsis for Pathologists.

Advances in anatomic pathology
2021

The surgical resection of dysplastic cerebellar gangliocytoma assisted by intraoperative sonography: illustrative case.

Journal of neurosurgery. Case lessons
2022

Multiple tumorous lesions of the pituitary gland.

Hormones (Athens, Greece)
2024

Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma) in the setting of cowden syndrome: a case report and literature review on COLD syndrome.

British journal of neurosurgery
2022

Giant Pediatric Supratentorial Tumor: Clinical Feature and Surgical Strategy.

Frontiers in pediatrics
2022

Uncommon Glioneuronal Tumors: A Radiologic and Pathologic Synopsis.

AJNR. American journal of neuroradiology
2022

Primary collision tumors of the sellar region: Experience from a single center.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2022

Observation of Clinicopathologic Features of Pituitary Adenoma With Neuronal Differentiation.

Frontiers in endocrinology
2022

Overview of the 2022 WHO Classification of Neuroendocrine Neoplasms.

Endocrine pathology
2022

Overview of the 2022 WHO Classification of Pituitary Tumors.

Endocrine pathology
2022

Overview of the 2022 WHO Classification of Paragangliomas and Pheochromocytomas.

Endocrine pathology
2022

A rare case of intraventricular gangliocytoma.

Arquivos de neuro-psiquiatria
2022

Mixed gangliocytoma-pituitary adenoma in MEN1 syndrome: A case report and literature review.

Neuropathology : official journal of the Japanese Society of Neuropathology
2022

Dysplastic gangliocytoma of the cerebellum in a cat.

Veterinary pathology
2022

Adult gangliocytoma arising within the lateral ventricle: A case report and review of the literature.

Surgical neurology international
2022

Medulloblastoma and Cowden syndrome: Further evidence of an association.

Free neuropathology
2021

Dysplastic cerebellar gangliocytoma: a description of two cases.

Quantitative imaging in medicine and surgery
2022

Clinical-pathological study of 28 glial and mixed neuronal-glial tumors diagnosed within the first year of life.

Clinical neuropathology
2021

Sellar Gangliocytoma: Case Report and Review of an Extremely Rare Tumour.

Case reports in neurology
2021

Hypothalamic hormone-producing tumors.

Handbook of clinical neurology
2021

Unilateral hydrocephalus from a gangliocytoma-somatotrophinoma: first reported case.

Endocrinology, diabetes &amp; metabolism case reports
2021

An Update on Pituitary Neuroendocrine Tumors Leading to Acromegaly and Gigantism.

Journal of clinical medicine
2021

Adult Langerhans Cell Histiocytosis Diagnosed by Biopsy of the Skull Tumor Generated after Craniotomy.

NMC case report journal
2021

Efficacy of One-stage Paravertebral Approach using a Micro-Tubular Technique in Treating Thoracic Dumbbell Tumors.

Orthopaedic surgery
2021

Periodic discharges with high frequency oscillations recorded from a cerebellar gangliocytoma in an epileptic infant.

Surgical neurology international
2021

Lhermitte-Duclos Disease in Pediatric Population: Report of 2 Cases.

Pediatric neurosurgery
2021

BCR-NTRK2 fusion in a pediatric patient with spinal gangliocytoma.

Pediatric blood &amp; cancer
2021

[Dysplastic Cerebellar Gangliocytoma(Lhermitte-Duclos Disease)].

No shinkei geka. Neurological surgery
2021

Longitudinal Assessment of Enhancing Foci of Abnormal Signal Intensity in Neurofibromatosis Type 1.

AJNR. American journal of neuroradiology
2020

Lhermitte-duclos disease (dysplastic cerebellar gangliocytoma): A case report.

JPMA. The Journal of the Pakistan Medical Association
2020

Cerebellar gangliocytoma as a cause of fluctuating hearing loss.

Clinical case reports
2020

Radiographic Findings of Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) in a Woman with Cowden Syndrome: A Case Study and Literature Review.

Journal of radiology case reports
2020

Role 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 reports
2020

Posttreatment Maturation of Medulloblastoma into Gangliocytoma: Report of 2 Cases.

Pediatric neurosurgery
2020

Retrobulbar gangliocytoma and carcinoma of the corpuscles of Stannius in a pearl gourami, Trichopodus leerii (Bleeker, 1852).

Journal of fish diseases
2020

Pituitary Gangliocytoma Producing TSH and TRH: A Review of "Gangliocytomas of the Sellar Region".

The Journal of clinical endocrinology and metabolism
2021

Dysplastic 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 Neurosurgery
2020

Bilateral Dysplastic Gangliocytoma with Concurrent Polyostotic Fibrous Dysplasia: A Case Report and Literature Review.

World neurosurgery
2020

[Design and clinical application of goal-oriented retroperitoneoscopic adrenalectomy].

Zhonghua yi xue za zhi
2019

AN INCIDENTAL COLLISION TUMOR OF THE SELLA TURCICA.

AACE clinical case reports
2020

Imaging of PTEN-related abnormalities in the central nervous system.

Clinical imaging
2020

Resection of a Symptomatic Dysplastic Cerebellar Gangliocytoma: 2-Dimensional Operative Video.

Operative neurosurgery (Hagerstown, Md.)
2020

Clinicopathologic Features of Diencephalic Neuronal and Glioneuronal Tumors.

Journal of neuropathology and experimental neurology
2020

Rare occurrence of gangliocytoma in the medulla oblongata.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2019

Hypothalamic Endocrine Tumors: An Update.

Journal of clinical medicine
2019

Mixed gangliocytoma-pituitary adenoma containing GH and GHRH co-secreting adenoma cells.

Endocrinology, diabetes &amp; metabolism case reports
2019

The Lhermitte-Duclos disease: a rare bilateral cerebellar location of a rare pathology.

The Pan African medical journal
2019

Glioneuronal 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 neurology
2019

Very Unusual Sellar/Suprasellar Region Masses: A Review.

Journal of neuropathology and experimental neurology
2019

Comprehensive Evaluation of Rare Pituitary Lesions: A Single Tertiary Care Pituitary Center Experience and Review of the Literature.

Endocrine pathology
2020

Early Basal Cortisol Level as a Predictor of Hypothalamic-Pituitary-Adrenal (HPA) Axis Function After Pituitary Tumor Surgery.

Experimental and clinical endocrinology &amp; diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association
2019

Bilateral Recurrent Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) in Cowden Syndrome: A Case Report and Literature Review.

World neurosurgery
2019

Updated Imaging Features of Dysplastic Cerebellar Gangliocytoma.

Journal of computer assisted tomography
2019

Hypothalamic Vasopressin-Producing Tumors: Often Inappropriate Diuresis But Occasionally Cushing Disease.

The American journal of surgical pathology
2019

Clinical Perspective on Dysplastic Gangliocytoma of the Cerebellum (Lhermitte-Duclos Disease).

World neurosurgery
2018

Lhermitte-Duclos Disease in a Six-Year Old Child: A Rare Presentation.

Pediatric neurosurgery
2018

Post-treatment maturation of medulloblastoma in children: two cases and a literature review.

The Journal of international medical research
2018

Securing Hemostasis in Pediatric Low-Grade Posterior Cerebral Fossa Tumors: The Value of Thrombin-Gelatin Hemostatic Matrix.

Pediatric neurosurgery
2018

Mixed gangliocytoma-pituitary adenoma in the sellar region: a large-scale single-center experience.

Acta neurochirurgica
2018

Multinodular and vacuolating neuronal tumor incidentally discovered in a young man: Conventional and advanced MRI features.

Radiology case reports
2018

Action Semantics at the Bottom of the Brain: Insights From Dysplastic Cerebellar Gangliocytoma.

Frontiers in psychology
2018

Growth hormone-secreting adenoma coexisted with gangliocytoma: a rare case.

International journal of clinical and experimental pathology
2018

Autonomic seizures and déjà vu in a patient with gangliocytoma of the orbitofrontal cortex.

Psychiatria Danubina
2018

A Case of Suspicious Gangliocytoma with Heterogeneously Distributed Lesions in the Thalamus and Basal Ganglia.

NMC case report journal
2018

Neurosurgical treatment of gangliogliomas in children and adolescents: long-term follow-up of a single-institution series of 32 patients.

Acta neurochirurgica
2018

An Unusual Case of Lhermitte-Duclos Disease Manifesting with Intratumoral Hemorrhage.

World neurosurgery
2018

Dysplasia and overgrowth: magnetic resonance imaging of pediatric brain abnormalities secondary to alterations in the mechanistic target of rapamycin pathway.

Neuroradiology
2017

Lhermitte-Duclos disease: an extremely rare cerebellar tumor.

The Pan African medical journal
2017

Transsphenoidal Surgery for Mixed Pituitary Gangliocytoma-Adenomas.

World neurosurgery
2017

Multinodular and Vacuolating Neuronal Tumor of the Cerebrum: A New "Leave Me Alone" Lesion with a Characteristic Imaging Pattern.

AJNR. American journal of neuroradiology
2017

[Curative effect analysis of 15 cases of cervical intra-and extra-spinal tumors by microsurgical resection].

Zhonghua yi xue za zhi
2017

Endoscopic Endonasal Resection of a Mixed Lesion of Gangliocytoma and Nonfunctioning Pituitary Adenoma.

World neurosurgery
2017

Lhermitte-Duclos Disease and Cerebellar Gangliocytoma-An Incidental Finding in a Patient with Gradual Vision Loss.

Neuro-ophthalmology (Aeolus Press)
2017

Lhermitte-Duclos Disease (Dysplastic Gangliocytoma of the Cerebellum) and Cowden Syndrome: Clinical Experience From a Single Institution with Long-Term Follow-Up.

World neurosurgery
2017

[Hereditary tumor syndromes in neuropathology].

Der Pathologe
2017

An intrasellar pituitary adenoma-gangliocytoma presenting as acromegaly.

Endocrinology, diabetes &amp; metabolism case reports
2018

Noninvasive assessment of isocitrate dehydrogenase mutation status in cerebral gliomas by magnetic resonance spectroscopy in a clinical setting.

Journal of neurosurgery
2017

Chiari I malformation in a child with PTEN hamartoma tumor syndrome: Association or coincidence?

European journal of medical genetics
2017

Gangliocytoma: outcome of a rare silent pituitary tumour.

BMJ case reports
2017

Clinicopathological 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 Neurosurgery
2017

Neuroglial intramedullary tumors: The collaboration between neurosurgeons and neuropathologists.

Neuro-Chirurgie
2017

Transdifferentiation of Neuroendocrine Cells: Gangliocytoma Associated With Two Pituitary Adenomas of Different Lineage in MEN1.

The American journal of surgical pathology
2017

Mixed Gangliocytoma-Pituitary Adenoma: Insights on the Pathogenesis of a Rare Sellar Tumor.

The American journal of surgical pathology
2016

Lhermitte-Duclos disease: A rare entity.

Medical journal, Armed Forces India
2017

Infantile Lhermitte-Duclos Disease Treated Successfully With Rapamycin.

Journal of child neurology
2016

Gangliocytoma Presenting With Tacrolimus Neurotoxicity in a Renal Transplant Recipient: Case Report.

Transplantation proceedings
2016

Lhermitte-Duclos disease with neurofibrillary tangles in heterotopic cerebral grey matter.

Folia neuropathologica
2016

The sellar and suprasellar region: A "hideaway" of rare lesions. Clinical aspects, imaging findings, surgical outcome and comparative analysis.

Clinical neurology and neurosurgery
2016

Mixed Pituitary Gangliocytoma and Prolactinoma Resistant to the Cabergoline Treatment.

World neurosurgery
2016

Gangliocytomas of the sellar region: A challenging diagnosis.

Clinical neurology and neurosurgery
2017

Characteristics of ganglion cells in pituitary gangliocytomas.

Neuropathology : official journal of the Japanese Society of Neuropathology
2016

Epidermal growth factor receptor is overexpressed in neuroblastoma tissues and cells.

Acta biochimica et biophysica Sinica
2016

High-Definition Fiber Tractography in the Evaluation and Surgical Planning of Lhermitte-Duclos Disease: A Case Report.

World neurosurgery
2016

Hypothalamic hamartoma with neurofibrillary tangles.

Neuropathology : official journal of the Japanese Society of Neuropathology
2016

Growth hormone secreting pituitary adenoma with admixed gangliocytoma and ganglioglioma.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2016

Management of unusual dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) in a developing country: Case report and review of the literature.

Asian journal of neurosurgery
2016

A Rare Case of Concomitant Intramedullary Gangliocytoma at the Cervicomedullary Junction in Patient with Neuroendocrine Tumor of Lung.

Journal of Korean Neurosurgical Society
2015

Challenge in preoperative diagnosis of retroperitoneal mucinous cyst in a pediatric patient.

International journal of clinical and experimental medicine
2015

Pituitary adenoma-neuronal choristoma is a pituitary adenoma with ganglionic differentiation.

Experimental and molecular pathology
2015

Ganglion cell tumours in the sella turcica in close morphological connection with pituitary adenomas.

Folia neuropathologica
2015

Tumor-to-tumor metastases in Cowden's disease: an autopsy case report and review of the literature.

Diagnostic pathology
2016

Sellar gangliocytoma with adrenocorticotropic and prolactin adenoma.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2016

Autopsy case of undiagnosed gangliocytoma in the medulla oblongata complicated with cerebral palsy.

Legal medicine (Tokyo, Japan)
2016

Middle infratemporal fossa less invasive approach for radical resection of parapharyngeal tumors: surgical microanatomy and clinical application.

Neurosurgical review
2015

Intracranial hypertension and cerebellar symptoms due to Lhermitte-Duclos disease.

Iranian journal of neurology
2015

Evaluation of amygdala pathology using (11)C-methionine positron emission tomography/computed tomography in patients with temporal lobe epilepsy and amygdala enlargement.

Epilepsy research
2015

Malignant peripheral nerve sheath tumor in cowden syndrome: a first report.

Journal of neuropathology and experimental neurology
2014

L'hermitte-Duclos disease in an elderly patient: A case report and review of the literature.

Asian journal of neurosurgery
Ver todos os 218 no EuropePMC

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Dysplastic cerebellar gangliocytoma: a six-decade study.
    Journal of neurosurgical sciences· 2026· PMID 41148183mais citado
  2. Recurrent multiloculated cystic variant of Lhermitte-Duclos disease managed with cyst fenestration and Ommaya reservoir placement: illustrative case.
    Journal of neurosurgery. Case lessons· 2026· PMID 41871401mais citado
  3. Underwater Endoscopic Resection for the Rare Periampullary Tumor.
    The Korean journal of helicobacter and upper gastrointestinal research· 2026· PMID 41846450mais citado
  4. Mixed gangliocytoma-pituitary neuroendocrine tumour: clinical, immunohistochemical, and molecular genetic profiles in a series of four patients.
    Acta neuropathologica communications· 2026· PMID 41618392mais citado
  5. Clinicopathological analysis of 19 cases of mixed gangliocytoma-pituitary neuroendocrine tumors.
    Diagnostic pathology· 2026· PMID 41872867mais citado
  6. Composite Gangliocytoma/Neuroma and Neuroendocrine Tumor (CoGNET) of the Duodenum: An Image in Endocrine Pathology Exploring Patterns of Biomarker Expression in Tumor Elements.
    Endocr Pathol· 2026· PMID 41537937recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:251937(Orphanet)
  2. MONDO:0016730(MONDO)
  3. GARD:10638(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. 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

Gangliocitoma
Compêndio · Raras BR

Gangliocitoma

ORPHA:251937 · MONDO:0016730
Prevalência
<1 / 1 000 000
CID-10
D36.1 · Neoplasia benigna dos nervos periféricos e sistema nervoso autônomo
CID-11
Ensaios
2 ativos
Início
Adolescent, Childhood, Infancy
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C5190517
EuropePMC
Wikidata
Papers 10a
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