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Background: Dysplastic gangliocytoma is a sporadic cerebellar benign tumor with the characteristics of hamartoma and true tumor, also known as Lhermitte-Duclos disease (LDD). Bone fibrous dysplasia (FD) is a slowly progressive self-limited benign bone tissue disease. Cowden syndrome, an autosomal dominant genetic disorder caused by germline mutations in the PTEN gene, is considered to be closely related to dysplastic gangliocytoma. McCune-Albright syndrome is a disease characterized by café-au-lait skin macules, polyostotic FD, and precocious puberty. The etiologic mechanism of both conditions is not yet clear. We report a rare case of bilateral dysplastic gangliocytoma with concurrent polyostotic FD., Case Description: We describe a 16-year-old boy with both LDD and FD. He presented for medical examination with headache and poor eyesight. Magnetic resonance imaging revealed proliferation of the skull and abnormal signals in the cerebellum, and supratentorial hydrocephalus. Subtotal resection of the cerebellar tumor was performed, and the diagnosis of LDD and FD was confirmed by histopathology. No other abnormal changes were found in systemic medical examination and no PTEN gene mutation was found in the genetic analysis; therefore, the diagnoses of Cowden syndrome and McCune-Albright syndrome were excluded., Conclusions: LDD and FD are 2 rare diseases, and the simultaneous occurrence of the 2 conditions has not been reported before, to our knowledge. Our report challenges the etiology of the 2 diseases and the relationship between them, hoping to provide a reference for the study of the 2 diseases., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Dysplastic gangliocytoma (Lhermitte-Duclos disease [LDD]) typically presents as a PTEN (phosphatase and tensin homolog)-positive, insidious unilateral mass of the cerebellar cortex. Patients can present with symptoms of increased intracranial pressure. Magnetic resonance imaging (MRI) will reveal a characteristic laminar/tigroid appearance. Surgical management has been superior to conservative measures for symptomatic lesions. The outcomes for bilateral craniotomy have not yet been described., Case Description: A 50-year-old patient with Cowden syndrome had initially presented with vertigo and imbalance. T2-weighted MRI showed a striated pattern of hypointensity and hyperintensity in bilateral cerebellar hemispheres consistent with Lhermitte-Duclos disease. He underwent right posterior fossa craniotomy with near total surgical resection and achieved symptom resolution. However, 3 years later, asymptomatic recurrence of the right-sided LDD and progression of left-sided LDD were noted. These were managed with a 1-year course of temozolomide. Both lesions remained stable until 4 years after completion of temozolomide. MRI demonstrated progression in the left hemisphere requiring a second surgical resection. PTEN analysis of the resected lesion was negative for mutation., Conclusions: To the best of our knowledge, the present case is the first reported case of bilateral cerebellar hemisphere LDD ultimately managed by temporally dissociated bilateral surgical resections. Our patient was unique in that he had undergone surgery for resection of LDD, followed by a second surgery for contralateral progression 8 years later. The therapeutic options to prevent recurrence are limited, although temozolomide did seem to impede progression. Symptomatic patients will typically benefit most from surgical intervention. Given the strong association between adult LDD and Cowden syndrome, maintaining close follow-up care and possible surveillance imaging will be essential., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Dysplastic gangliocytoma of the cerebellum, also called Lhermitte-Duclos disease (LDD), is known as a rare, benign brain tumor. Around 200 cases have been reported., Case Description: Here we introduced a newly diagnosed adult case with intratumoral hemorrhage. Then we reviewed 18 cases diagnosed in our institute, including 12 adults and 6 children., Conclusions: Preoperative diagnosis based on magnetic resonance imaging is 50% in adults and 16.7% in pediatrics. Diagnostic tiger stripes are not always seen in pediatric cases. The prognosis of the disease is good if total resection can be achieved. Further examinations for PTEN mutation and other comorbidities are recommended., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Lhermitte-Duclos disease (LDD) is a rare neurologic disease that causes a hamartomatous lesion in the cerebellum. LDD is usually associated with mass lesion effects, but with appropriate surgical treatment, the clinical course is usually benign. We report a rare case of a patient with LDD with contrast enhancement on magnetic resonance imaging (MRI) who died as a result of intratumoral hemorrhage., Case Description: LDD was diagnosed in a 59-year-old woman after a routine MRI. She did not present with any symptoms initially or after follow-up MRI suggested minor hemorrhage in the tumor. Eleven months after her first visit to our department, she suddenly lost consciousness, and computed tomography revealed massive intratumoral hemorrhage. Surgical decompression was done, but she died 17 days after surgery. Histopathologic findings were consistent with LDD., Conclusions: Hemorrhagic events can occur with LDD, even though the growth of the lesion is slow. Long-term close follow-up of patients with LDD is needed, especially patients in whom MRI shows atypical enhancement. To avoid possible hemorrhagic events, surgical treatment should be considered even if only subtle changes are seen on neuroimaging., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Adult-onset Lhermitte-Duclos disease (LDD) and Cowden syndrome (CS) are considered a single phakomatosis that belongs to PTEN hamartoma tumor syndrome (PHTS) now. There is still controversy regarding the diagnosis and treatment. The authors describe the clinical features of LDD and CS with long-term follow up., Methods: From January 2001 to January 2017, 18 patients were admitted to the neurosurgery department of Beijing Tiantan Hospital. The authors analyzed the medical records of each patient and followed every case., Results: Seventeen of 18 patients underwent surgery to remove the tumor. The results of pathologic analysis revealed LDD. There was obvious enhancement on magnetic resonance imaging (MRI) in 2 patients who received gamma knife and radiotherapy before surgery. During surgery, it is difficult to determine the exact margin. Tumors were removed gross totally in 9 patients, partially in 6 patients, and only subtotally in 2 patients. CS was diagnosed in 11 patients. Two patients received DNA analysis, revealing heterozygous mutation of exon 5 in an 11-year-old girl. There was no recurrence of the tumor during follow-up., Conclusions: LDD has the unique appearance on T2-weighted MRI. The most difficult aspect of surgery is determining the actual margins of the tumor. Total resection is difficult in some patients. There was no tumor recurrence after long-term follow-up in our case series. For pediatric LDD patients, DNA analysis should be performed to rule out CS., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Preoperative delineation of normal tissue displacement patterns in Lhermitte-Duclos disease has not been feasible with conventional imaging means. Surgical resection of this type of lesion remains challenging, because the boundaries of the lesion are indistinguishable during surgery., Case Description: The clinical presentation, preoperative and postoperative magnetic resonance imaging (MRI) findings, high-definition fiber tractography (HDFT) and histopathological studies, are presented in a 46-year-old male subject with symptomatic Lhermitte-Duclos disease. HDFT was performed using a quantitative anisotropy-based generalized deterministic tracking algorithm to define fiber tracts. Displacement of the cerebellar and brainstem tracts on the affected side was performed using the unaffected contralateral side as a comparison. The displacement of the normal tissues was not apparent on preoperative MRI but was immediately evident on the preoperative HDFT. Of note, there was a relative paucity of fiber tracts within the lesion. By tailoring our operative boundaries based on the HDFT findings, we were able to spare the displaced fiber tracts when debulking the tumor. Restoration of normal fiber tract anatomy on postoperative HDFT imaging was correlated with clinical resolution of preoperative symptoms., Conclusions: This case report suggests that HDFT may be a powerful surgical planning tool in cases of Lhermitte-Duclos disease, in which the pattern of normal tissue displacement is not evident with conventional imaging, allowing maximal lesion resection without damage to the unaffected tracts. Therefore, this report contributes to solving the greatest challenge when operating on this type of lesion, which has not been resolved in any previous report in our review of the English literature., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: The simultaneous presence of Lhermitte-Duclos disease (LDD) with focal areas of nodular dysembryoplastic neuroepithelial tumor (DNET) differentiation in the cerebellar hemisphere is reported in a patient who showed the characteristic magnetic resonance imaging (MRI) appearance of "tiger striping"., Case Report: A 25-year-old man presented with a 7-month history of holocranial headache, progressive vision diminution, and right-sided cerebellar signs. Computed tomography (CT) scan revealed a hypodense, nonenhancing right cerebellar lesion effacing the fourth ventricle. There were thin hyperdense, linear striations across the tumor with hypodense streaks between them. The third and lateral ventricles were dilated with periventricular lucency. MRI showed the hypointense-to-isointense right cerebellar lesion with linear striations on T1-weighted images. The lesion was hyperintense on T2-weighted images with areas of linear hypointensity streaks running throughout the lesion characteristic of the "tiger striping" effect of LDD. Sagittal MRI revealed tonsillar herniation. Gross total removal of the tumor was accomplished with right paramedian suboccipital craniectomy. The postoperative course was unremarkable, and the patient remained symptom-free at 3-month follow-up examination. Histology revealed the simultaneous presence of LDD with DNET., Conclusions: To the best of the authors' knowledge, the simultaneous presence of cerebellar LDD with DNET has never been reported before. This case report may point to their common genesis with cortical dysplasia and neuronal migrational abnormalities playing an important role. LDD and DNET may represent different points in the spectrum of the lesion-from being purely hamartomatous to having a greater proliferative potential. The simultaneous presence of LDD and DNET was perhaps responsible for the unusually rapid neurologic deterioration in this patient before surgical intervention., (Copyright © 2011 Elsevier Inc. All rights reserved.)