Adult ; Humans ; Rhabdoid Tumor/surgery* ; Teratoma/pathology* ; Medulloblastoma ; Central Nervous System Neoplasms ; Cerebellar Neoplasms ; Brain Neoplasms/pathology*

Lhermitte-Duclos disease (LDD) is a type of rare brain tumor located in posterior fossa. A patient with LDD located in the left cerebellum and vermis was admitted by the Department of Neurosurgery, Xiangya Hospital, Central South University. MRI scan showed slightly heterogeneous enhancement at the region close to vermis. The patient underwent partial resection on August 11, 2016 without postoperative chemoradiotherapy. The progress free survival was 11 months and the overall survival was 17 months. What the case reveals is that the partial resection is not beneficial to these patients with LDD as the residual lesion probably recurs in a short term after operation. The pathogenesis, diagnosis and treatment of LDD are explored and summarized in combination with relevant literature.
Cerebellar Neoplasms/surgery* ; Cerebellum ; Hamartoma Syndrome, Multiple/diagnostic imaging* ; Humans ; Magnetic Resonance Imaging ; Neoplasm Recurrence, Local

Hemangioblastoma (HBL) in the pituitary stalk is extremely rare. Only 16 such cases have been reported in the past and 5 cases have been treated with surgical procedure. Here, we report surgical case of HBL in the pituitary stalk diagnosed in a 34-year-old woman. The patient underwent a gross-total resection via the modified lateral supra-orbital approach. No recurrence was observed in two years after surgery. To our knowledge, this is the 17th case of HBL in the pituitary stalk and the 6th surgical case. If the tumor is symptomatic and the volume is over 5 cubic centimeters as in our case, we recommend that the surgical resection of the HBL in the pituitary stalk is a more safe and reasonable than radiotherapy.
Adult ; Cerebellar Neoplasms/pathology/*surgery ; Female ; Hemangioblastoma/pathology/*surgery ; Humans ; Pituitary Gland/*pathology ; Treatment Outcome

OBJECTIVETo study the pathologic characteristics and prognosis in different subtypes of adult medulloblastoma (MB).

METHODSThe clinical information, imaging findings and pathologic characteristics of 151 cases of adult medulloblastomas were retrospectively reviewed and analyzed by chi-square test. The survival data were assessed using the Kaplan-Meier method.

RESULTSAmongst the 151 MB cases studied, there were 73 cases of classic MB, 36 cases of desmoplastic/nodular MB, 39 cases of anaplastic MB and 3 cases of large cell MB. The primary tumors were more frequently located in cerebral hemisphere in desmoplastic/nodular MB than in other subtypes (P=0.000).On the other hand, large cell/anaplastic MB were associated with more frequently local recurrence and distant metastasis (P=0.003). The post-operative overall survival time ranged from 6 to 150 months, with median survival being (103.3±5.7) months (95%CI, 92.52 to 115.09). The median survival of classic MB, desmoplastic/nodular MB and large cell/anaplastic MB was (110.7±7.8) months, (125.5±7.6) months and (57.6±7.6) months, respectively. The differences were statistically significant (P=0.000).

CONCLUSIONSThe three variants of MB show different biologic behavior. Large cell/anaplastic MB represents an independent poor prognostic indicator in adults.

Adolescent ; Adult ; Cerebellar Neoplasms ; classification ; metabolism ; pathology ; radiotherapy ; surgery ; Disease-Free Survival ; Female ; Follow-Up Studies ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Kaplan-Meier Estimate ; Ki-67 Antigen ; metabolism ; Male ; Medulloblastoma ; classification ; metabolism ; pathology ; radiotherapy ; surgery ; Middle Aged ; Neoplasm Recurrence, Local ; Phosphopyruvate Hydratase ; metabolism ; Radiotherapy, Adjuvant ; Retrospective Studies ; Survival Analysis ; Synaptophysin ; metabolism ; Young Adult

Subependymomas are rare benign tumors located in the ventricular system. Intraparenchymal subependymoma is extremely rare; only 6 cases have been reported, and all were located in the supratentorial region. We describe a case of infratentorial, intraparenchymal subependymoma in a 28-year-old man with intermittent headache. Imaging revealed a well-demarcated cystic and solid cerebellar mass near the fourth ventricle. The mass had a microcystic component and calcification without contrast enhancement. Complete surgical excision was performed, and histopathology confirmed a subependymoma.
Adult ; Calcinosis/diagnosis ; Cerebellar Neoplasms/*diagnosis/surgery ; Fourth Ventricle ; Glioma, Subependymal/*diagnosis/surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Rare Diseases/*diagnosis/surgery ; Tomography, X-Ray Computed

Meningioma is a common intracranial tumor in adults. Pediatric cases account for approximately 1.5% of all intracranial meningiomas, and very few cases show malignant histological features. Primary pediatric malignant meningioma in the cerebellopontine angle is extremely uncommon. Herein, we report a 2-year-old girl with malignant meningioma in the cerebellopontine angle. The clinical features, diagnosis, and treatment protocol are discussed.
Cerebellar Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Cerebellopontine Angle ; Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Meningeal Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Meningioma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Mucin-1 ; metabolism ; Radiography ; S100 Proteins ; metabolism ; Vimentin ; metabolism

OBJECTIVETo study the expression of β-catenin protein and the status of loss of heterozygosity (LOH) on chromsome 10q in medulloblastoma, with clinical correlation.

METHODSImmunohistochemical study for β-catenin protein was carried out in 50 cases of medulloblastoma encountered in the First Affiliated Hospital of Xinjiang Medical University during the period from 2002 to 2011, including 32 cases of classic medulloblastoma, 13 cases of desmoplastic medulloblastoma and 5 cases of medulloblastoma with extensive nodularity. The status of LOH on 10q was also detected by fluorescence in-situ hybridization. The clinicopathologic characteristics and prognostic parameters were studied by Kaplan-Meien and Cox analysis.

RESULTSThe rates of expression of β-catenin protein in classic medulloblastoma, desmoplastic medulloblastoma and medulloblastoma with extensive nodularity were 53.1% (17/32), 4/13 and 1/5, respectively. The rate of LOH on 10q was 33.3% (8/24) in classic medulloblastoma and 2/11 in desmoplastic medulloblastoma. There was no statistically significant difference between the two tumor types. Univariate analysis showed that the expression of β-catenin protein (P = 0.022), lack of LOH on 10q (P = 0.020), extensiveness of tumor resection (P < 0.01), radiotherapy (P = 0.002) and chemotherapy (P < 0.01) represented important prognostic factors.

CONCLUSIONSMedulloblastoma with expression of β-catenin protein and without LOH on 10q carries a better prognosis. Assessment of these parameters is helpful in evaluating prognosis and subsequent patient management.

Adolescent ; Adult ; Cerebellar Neoplasms ; genetics ; metabolism ; pathology ; surgery ; Child ; Child, Preschool ; Chromosomes, Human, Pair 10 ; genetics ; Female ; Follow-Up Studies ; Humans ; Infant ; Loss of Heterozygosity ; Male ; Medulloblastoma ; genetics ; metabolism ; pathology ; surgery ; Proportional Hazards Models ; Survival Rate ; Young Adult ; beta Catenin ; metabolism

OBJECTIVETo study the clinicopathologic features and immunophenotype of endolymphatic sac tumor (ELST) and normal endolymphatic sac.

METHODSThe clinical and histologic features were evaluated in 5 cases of ELST. Eight cases of choroid plexus papilloma at cerebellopontine angle and 2 cases of normal endolymphatic sac were used as controls. Immunohistochemical study for vimentin, AE1/AE3, CK8/18, CK5/6, EMA, GFAP, synaptophysin, S-100 protein, CEA, TTF-1, VEGF, D2-40, calponin, calretinin and Ki-67 was carried out.

RESULTSThe age of onset of ELST ranged from 23 to 35 years (median = 24 years). The male-to-female ratio was 2:3. The clinical presentation was tinnitus, otalgia, hearing loss, otorrhagia with effusion and headache. The duration of symptoms ranged from 6 months to 10 years. Local recurrences were noted in 3 cases. Radiologically, the tumors were located at cerebellopontine angle and demonstrated petrous bone destruction. Histologic examination showed that the tumors had a papillary-glandular pattern. The papillae were covered by a single layer of low cuboidal cells. The tumor cells had distinct cell borders and contained eosinophilic to clear cytoplasm. The nuclei were slightly atypical and sometimes apically located. Focal dilated glandular structures with colloid-like material were also identified. The surrounding stroma was vascularized. All of the 5 cases had dural or petrous bone infiltration. Immunohistochemical study showed that all of the 5 cases were positive for AE1/AE3, CK8/18, CK5/6 and VEGF, 4 cases for EMA, 3 cases for calponin (focal), 2 cases for vimentin, 2 cases for S-100 protein, 1 case for GFAP and 1 case for synaptophysin (focal and weak). The Ki-67 index measured less than 1%. The staining for D2-40, calretinin, CEA and TTF-1 was negative. The 2 cases of the normal endolymphatic sac were positive for AE1/AE3 and CK8/18, and negative for CK5/6, EMA, S-100 protein, GFAP and synaptophysin. The 8 cases of choroid plexus papilloma were positive for synaptophysin. Seven cases were also positive for S-100 protein, 2 cases for GFAP and 1 case for D2-40. All of the 8 cases were negative for EMA, CK5/6 and calponin.

CONCLUSIONSELST is a rare slow-growing and potentially malignant tumor with a tendency of bone invasion and local recurrence. Distant metastasis is not observed. It must be distinguished from choroid plexus papilloma occurring at cerebellopontine angle. Correlation with clinical, radiologic and immunohistochemical findings would also be helpful.

Adenocarcinoma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Adult ; Calcium-Binding Proteins ; metabolism ; Cerebellar Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Cerebellopontine Angle ; pathology ; Diagnosis, Differential ; Endolymphatic Sac ; pathology ; Female ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Keratin-5 ; metabolism ; Keratin-6 ; metabolism ; Male ; Microfilament Proteins ; metabolism ; Mucin-1 ; metabolism ; Neoplasm Recurrence, Local ; Papilloma, Choroid Plexus ; metabolism ; pathology ; Tomography, X-Ray Computed ; Young Adult

Gliosarcoma is a rare central nervous system tumor usually located in the supratentorial area. Here we report a rare case of a gliosarcoma that developed in the cerebellar hemisphere in a 70-year-old woman. Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain revealed an infratentorial mass of which radiological features were similar to those of glioblastoma. The tumor was diagnosed by pathology as a gliosarcoma. Though rare, gliosarcoma should be considered in the differential diagnosis of infratentorial tumors with radiological features of glioblastoma or metastasis in elderly patients.
Aged ; Cerebellar Neoplasms/*diagnosis/pathology/surgery ; Contrast Media/diagnostic use ; Female ; Gliosarcoma/*diagnosis/pathology/surgery ; Humans ; Magnetic Resonance Imaging ; Tomography, X-Ray Computed

We report a case of acute comitant esotropia in a child with a cerebellar tumor. A 3-year-old boy was referred for management of a 9 month history of acute acquired comitant esotropia. On first presentation, the patient's angle of esodeviation was 50 prism-diopters (PD) at distance and near fixation without any lateral incomitance. The cycloplegic refraction revealed +0.75 diopters in both eyes. Very mild bilateral papilledema was found on the fundus examination, but the neurological examination did not reveal any other pathological findings. Brain MRI showed a 5 cm mass located in the midline of the cerebellum as well as hydrocephalus. The mass was completely excised and histological examination confirmed the diagnosis of pilocytic astrocytoma. Despite neurosurgery, the patient's strabismus remained unresolved. One year after neurosurgery, both medial rectus muscles were surgically recessed by 6 mm, resulting in esotropia of 8PD at distant and near fixation without restoration of bifoveal fusion at follow-up 2 years after the eye muscle surgery. Therefore, acute onset comitant esotropia in a child can be the first sign of a cerebellar tumor, even without any other neurological signs and symptoms.
Acute Disease ; Astrocytoma/*complications/surgery ; Brain/pathology ; Cerebellar Neoplasms/*complications/diagnosis/surgery ; Child, Preschool ; Esotropia/*etiology/pathology/physiopathology/surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Neurosurgical Procedures ; Oculomotor Muscles/surgery ; Refraction, Ocular ; Time Factors