A Rare WHO Grade I Lesion of the Posterior Fossa with Recurrence Biological Behavior ­ Dysplastic Gangliocytoma of the Cerebellum: Case Report

Dysplastic gangliocytoma of the cerebellum (DGC) or Lhermitte-Duclos Disease is a rare lesion (World Health Organization [WHO] grade I) characterized by thickened folia and replacement of the internal granular layer by abnormal ganglion cells. More commonly, the compromised patients are young adults presenting ataxia, seizures, obstructive hydrocephalus, and increased intracranial pressure. Dysplastic gangliocytoma of the cerebellum is intimately associated with Cowden syndrome, a hereditary disorder caused by a germline mutation in the PTEN tumor suppressor gene on chromosome 10q23. Large neurons of DCG show vesicular nuclei with prominent nucleoli. Expansion of the internal granular layer determines vacuolization of the molecular layer and white matter, which can be related to the bright stripes identified on T2-weighted magnetic resonance imaging. Herein, the authors report a female patient who developed long- time recurrence of DGC and discuss pathological findings and differential diagnosis of this rare cerebellar lesion.

Imaging In A Case Of Lhermitte Duclos Disease - Dysplastic Cerebellar Gangliocytoma.

"Lhermitte-Duclos disease (LDD) is a rare cerebellar lesion of uncertain origin. It is linked to an autosomal- dominant phakomatosis known as Cowden's disease in 40% of patients. The MRI features of LDD are almost unique and can be considered diagnostic. (1) We report on a case of 48 year old female patient who presented to us with history of seizures and bilateral lower limb weakness with the typical MRI features of the above disease. We also discuss the pathology and genetics of this rare disease"

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

Ganglion cell tumors (GCT) are divided into two subtypes : gangliocytoma and ganglioglioma. Intramedullary gangliocytomas are extremely rare. A 20-year-old male patient with pain of neck, who also had a previously known neuroendocrine tumor of lung, was operated for mass found in the cervicomedullary junction with a presumptive diagnosis of metastases. Only partial resection could be performed. Pathological diagnosis had been reported as gangliocytoma. Only ten cases of intramedullary gangliocytoma have been reported in the literature. Although association with scoliosis and Von Recklinghausen's disease were previously reported in the literature, no gangliocytoma case concomitant with endocrine tumor of lung have been published. Pathological study is the most important diagnostic method for gangliocytomas. Surgical excision is the primary treatment, but difficulty in total surgical tumor resection is the most important problem.

MRI manifestations of dysplastic gangliocytoma of the cerebellum / 实用放射学杂志

Objective To discuss MRI characteristics of dysplastic gangliocytoma of the cerebellum.Methods MRI manifesta-tions of 13 cases with dysplastic gangliocytoma of the cerebellum verified by operation and pathology were analyzed retrospectively. Results All of 13 cases were located in the cerebellar hemisphere and vermis.The lesions presented striated pattern of iso-intensity signal and hypo-intensity signal on T1-weighted images.The sign of tiger stripes was the feature in dysplastic gangliocytoma on T2-weighted and FLAIR images.All of the tumors showed slightly striated enhancement after intravenous administration of contrast agent.The mass effects of dysplastic gangliocytoma of the cerebellum along with the forth ventricle and brain stem oppressed were seen in all cases.NAA was mildly reduced on magnetic resonance spectroscopy (MRS)in 7 cases of dysplastic gangliocytoma of the cerebellum.Conclusion There are distinctive manifestations in MRI images for dysplastic gangliocytoma of the cerebellum,which has crucial diagnostic value before operation.

Enfermedad de Lhermitte-Duclos o gangliocitoma displásico cerebeloso: reporte de un caso y revisión de la literatura / Lhermitte-Duclos disease or cerebellar dysplastic gangliocytoma: Case report and literature review

La enfermedad de Lhermitte-Duclos o gangliocitoma displásico cerebeloso es una entidad infrecuente que se presenta como un proceso expansivo de la fosa posterior, de lento crecimiento y patogenia desconocida. Puede estar asociada a otras anomalías congénitas. Presentamos el caso de un gangliocitoma displásico de cerebelo en un paciente de sexo masculino, de 77 años de edad, con antecedentes de adenocarcinoma prostático, que fue estudiado por tomografía computada (TC) y resonancia magnética (RM). Los hallazgos característicos, junto con una evaluación neuroquirúrgica y un manejo apropiados, permitieron efectuar el diagnóstico preoperatorio sin necesidad de recurrir a exámenes histopatológicos...

The Lhermitte-Duclos disease or dysplastic cerebellar gangliocytoma is an uncommon disorder that presents as an expansive, slow growing process of the posterior fossa, of unknown pathogenesis, that may be associated with other congenital anomalies.A case is presented of a dysplastic cerebellar gangliocytoma in a 77 year-old male patient, with a history of prostatic adenocarcinoma. A study was made using Computed Tomography (CT) and Magnetic Resonance (MRI), where the characteristic findings enabled a preoperative diagnosis to be made without need for histopathological examination, in addition to a neurosurgical evaluation and appropriate management...

Assessing a Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) with 7T MR Imaging

Lhermitte-Duclos disease (LDD; dysplastic cerebellar gangliocytoma) is a rare hamartomatous lesion of the cerebellar cortex and this was first described in 1920. LDD is considered to be part of the autosomal-dominant phacomatosis and cancer syndrome Cowden disease (CS). We examined the brain of a 46-year-old man, who displayed the manifestations of CS, with 7 Tesla (T) and 1.5T MRI and 1.5T MR spectroscopy (1H-MRS). We discuss the possible benefits of employing ultrahigh-field MRI for making the diagnosis of this rare lesion.

Enfermedad de Lhermitte-Duclos: Informe de un caso y revisión de la literatura / Lhermitte-Duclos disease: Case report and review of literature

Introducción. El gangliocitoma displásico del cerebelo fue descrito originalmente en 1920 por Lhermitte y Duelos, autores a quienes se les debe el epónimo de esta enfermedad. Es una entidad rara, caracterizada por ser una masa tumoral unilateral de la corteza cerebelosa, de crecimiento muy lento, que produce un aumento de la presión intracraneana. En 1991 Padberg y col. describieron la asociación de la enfermedad Lhermitte-Duclos y el síndrome de Cowden (SC). Aún no se ha comprendido completamente la biología molecular que asocia a estas enfermedades, sin embargo se ha comprobado en estudios modelos animales (ratones) mutaciones del gen homólogo de fosfatasa y tensina suprimido del cromosoma 10 (PTEN). Las imágenes de resonancia magnética son características de esta entidad, de tal manera que permiten hacer el diagnóstico preoperatorio sin la necesidad de estudios histopatológicos, permitiendo una evaluación y manejo neuroquirúrgico adecuados. Caso clínico. Se informa el caso de una mujer de 17 años con enfermedad de Lhermitte-Duclos del hemisferio cerebeloso derecho no asociado a SC, en quien el diagnóstico se estableció en el transoperatorio con improntas de la lesión cerebelosa. Conclusión. No existen publicaciones previas que describan las características cito lógicas de la lesión, y en este caso sí fue posible estudiarlas.

Introduction. Dysplastic cerebellar gangliocytoma was first described in 1920 by Lhermitte and Duelos, authors who gave their name to the entity. It is a rare condition, which is characterized by a very slowly growing unilateral tumor mass of the cerebellar cortex, producing increased intracranial pressure. In 1991 Padberg et al. described the association of Lhermitte-Duclos disease and Cowden syndrome. The relationship between these 2 entities has been associated with mutations of the phosphatase and tensin homolog deleted on chromosome (PTEN) gene. Magnetic resonance imaging findings are the hallmark of this entity, and the diagnosis is established without hystopathologic studies. Case report. We inform a case of a 17 years old girl with Lhermitte-Duclos of the right cerebellar hemisphere without Cowden syndrome.

Dysplastic Gangliocytoma of Cerebellum(Lhermitte-Duclos Disease): Relation to Cowden Disease

We report a case of dysplastic gangliocytoma of the cerebellum(Lhermitte-Duclos disease) that can cause progressive mass effect in the posterior fossa. Cowden disease is a rare autosomal dominant disorder characterized by mucocutaneous hamartoma and high incidences of systemic malignancies. The patient had no mucocutaneous lesions indicating Cowden disease. With recent advances in molecular genetics, the association between Lhermitte-Duclos disease and Cowden disease has been recognized, it is considered that Cowden disease is a new phakomatosis. Recognition of this association has direct clinical relevance and long term follow up may lead to the early detection of malignancy.

Gangliocytoma Mimicking Extra-axial Tumor: A Report of Two Cases

We report two cases of supratentorial gangliocytomas mimicking an extra-axial tumor. MR imaging indicated that the tumors were extra-axial, and meningiomas were thus initially diagnosed. Relative to gray matter, the tumors were hypointense on T1-weighted images and hyperintense on T2-weighted images. On contrast-enhanced T1-weighted images, homogeneous enhancement was observed, while CT scanning revealed calcification in one of the two cases.

Lennox-Gastaut Syndrome Associated with Gangliocytoma: A Case of Surgical Treatment

We report a case of Lennox-Gastaut syndrome successfully treated by the removal of hippocampal gangliocytoma. A17-year-old girl was admitted due to intractable tonic seizure, generalized tonic clonic seizure, and frequent fallingdespite the administration of polypharmacotherapy. She was mentally retarded and had developmental delays. She had the first seizure at the age of 12 months and the pattern of seizures seemed to be drop attacks. Electroencephalogram (EEG) showed background slowing of about 7 Hz, multifocal spike and wave, and generalized spike and wave complex-es (1.5~3.5 Hz). Magnetic resonance image (MRI) revealed a small polycystic mass in the left parahippocampal gyrus and hippocampal atrophy. A left hippocampectomy and total resection of the tumor was performed. Histopathologically, the cysts were identical to gangliocytoma. Sixth months later, the frequency of seizures decreased to once a month No seizures have been reported since August 1998.

A Case of Acromegaly Caused by Mixed Gangliocytoma-Adenoma of the Pituitary Gland / 대한내분비학회지

The tumors containing ganglion cells are rare in the pituitary gland. These tumors are divided into two groups. The first group is the cases of mixed gangliocytoma-adenoma that contained both gangliocytoma and pituitary adenoma elements in the same tumor and the second group is those of gangliocytoma that contained only gangliocytoma element. Since the first description by Kiyono in 1926, 45 cases have been reported. The most common presentation of these tumors is acromegaly and the cases of Cushings disease and galactorrhea-amenorrhea also have been reported. In immunohistochemical study, hypothalamic-releasing hormones are stained in gangliocytoma cells and adenohypophyseal hormones are stained in adenoma cells. The releasing hormones stained in gangliocytoma element and the pituitary hormones stained in adenoma elements are usually closely related but unrelated cases also have been reported. We report a case of a 41 year-old lady with acromegaly which was diagnosed as mixed gangliocytoma-adenoma after surgical removal of the pituitary tumor.

Mixed Gangliocytoma-Pituitary Adenoma: A case report

The mixed gangliocytoma-pituitary adenoma is a very rare intracranial neoplasm, and it is frequently associated with endocrine symptoms; acromegaly, Cushing's disease, galactorrhea and amenorrhea. Morphologically it shows a mixture of gangliocytoma and pituitary adenoma in various proportions. In the area of gangliocytoma, there is no neoplastic glial component. The portion of pituitary adenoma consists mainly of chromophobe cells. There are three hypotheses in its histogenesis. We report a case of a 41 year-old lady presented with acromegaly and amenorrhea existing for 3 years. On magnetic resonance image, there is a dumbell-shaped mass in the sellar region. Histologically it showed typical features of mixed gangliocytoma-pituitary adenoma. This case is presented in the view of its rarity and interesting possible histogeneses.

Lhermitte-Duclos Disease in a Sudden Death: An autopsy case

Lhermitte-Duclos disease of the cerebellum manifests itself as a slowly growing mass formed by abnormal neurons that appear more malformative than tumoral. Although most cases show satisfactory clinical evolution, several cases, not recognized in life, died abruptly, usually from respiratory arrest. We recently experienced a 19 year old male, who died suddenly during alcohol drinking. At autopsy, the cerebellum revealed thickening of the folia over two limited areas. Microscopically, the lesion was characterized by proliferation of abnormal neuronal cells with excessive myelination of their axons, disappearance of Purkinie cells and greatly reduced white mater. Theories regarding the pathogenesis of Lhermitte-Duclos disease were reviewed and discussed.

Lhermitte-Duclos Disease(Dyslastic Gangliocytoma of the Cerebellum): A Case Report with Review of the Literature

No abstract available.