Cerebellar Neoplasms ; diagnosis ; pathology ; radiotherapy ; surgery ; Follow-Up Studies ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Medulloblastoma ; diagnosis ; pathology ; radiotherapy ; surgery

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

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

Medulloblastoma is a common malignant central nervous system neoplasm found mainly in children. One the contrary, medulloblastoma of the cerebellopontine angle, the location of the tumor is very unusual. This is the the first case of the medullomyoblastoma, a rare form of medulloblastoma, occurring in the cerebellopontine angle. A 15-year-old boy experienced a sudden hearing loss in the left ear. Conservative medical treatment failed, and temporal MR imaging revealed a heterogeneously enhancing mass at the left cerebellopontine angle cistern and in the internal auditory canal; therefore, the lesion was regarded as a typical acoustic neuroma. Few days before surgery, an ipsilateral facial palsy developed, and a follow-up MR imaging showed a rapid growth of the previous lesion. The extended translabyrinthine approach permitted surgical removal. And under pathological diagnosis of malignancy, radiation therapy and series of chemotherapy was performed.
Adolescent ; Cerebellar Neoplasms/*pathology/surgery ; Cerebellopontine Angle/*pathology ; Diagnosis, Differential ; Humans ; Magnetic Resonance Imaging ; Male ; Medulloblastoma/*pathology/surgery ; Neuroma, Acoustic/*pathology

We describe herein a malignant glioma arising at the site of the resected hemangioblastoma after irradiation in a patient with von Hippel-Lindau disease (VHL). The patient was a 25 year-old male with multiple hemangioblastomas at the cerebellum and spinal cord, multiple pancreatic cysts and a renal cell carcinoma; he was diagnosed as having VHL disease. The largest hemangioblastoma at the right cerebellar hemisphere was completely removed, and he received high-dose irradiation postoperatively. The tumor recurred at the same site 7 years later, which was a malignant glioma with no evidence of hemangioblastoma. The malignant glioma showed molecular genetic profiles of radiation-induced tumors because of its diffuse p53 immunostaining and the loss of p16 immunoreactivity. The genetic study to find the loss of heterozygosity (LOH) of VHL gene revealed that only the cerebellar hemangioblastoma showed allelic losses for the gene. To the best of our knowledge, this report is the first to show a malignant glioma that developed in a patient with VHL disease after radiation therapy at the site of an excised hemangioblastoma. This report also suggests that radiation therapy should be performed very carefully in VHL patients with hemangioblastomas.
Adult ; Cerebellar Neoplasms/complications/*surgery ; Glioma/*diagnosis/*etiology ; Hemangioblastoma/complications/*surgery ; Humans ; Male ; Radiotherapy/*adverse effects ; von Hippel-Lindau Disease/complications/*radiotherapy

We report a very rare case of hemangioblastomatosis that developed after surgical removal of a solitary cerebellar hemangioblastoma (HB). A 51-yr-old man presented with back pain 10 yr after undergoing surgery for cerebellar HB. Magnetic resonance imaging showed numerous mass lesions along the entire neuraxis accompanied by prominent leptomeningeal enhancement. Genomic DNA analysis showed no mutation in the von Hippel-Lindau (VHL) genes. A surgical specimen obtained from a lesion in the cauda equina showed pathological findings identical to those of the cerebellar HB that had been resected 10 yr earlier. External beam radiation therapy and radiosurgery were subsequently performed; however, the patient succumbed one year after receiving the diagnosis of hemangioblastomatosis. The reduction of tumor cell spillage during surgery and regular long-term follow-up are recommended for patients with HBs.
Central Nervous System Neoplasms/*diagnosis/pathology/surgery ; Cerebellar Neoplasms/pathology/radiotherapy/surgery ; Hemangioblastoma/*diagnosis/pathology/surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Recurrence ; von Hippel-Lindau Disease/genetics

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

OBJECTIVETo study the clinical features, neuroimaging presentations, diagnosis and differential diagnosis, and operative warning events of the solid cerebellar hemangioblastoma.

METHODSThe clinical data of 48 patients with solid cerebellar haemangioreticuloma were retrospectively analysed. Preoperative examinations included CT and MRI in all patients, DSA in 20, preoperative embolization of feeding arteries of tumor in 16, a preoperative ventriculoperitoneal shunt was placed in 5 cases. The lesion distribution of 48 patients was as follows: 14 lesions were situated in the left hemisphere of cerebellum with an extent into the cerebellopontine angle in 2 cases, 12 in right hemisphere of cerebellum, 8 in superior vermis, 7 in inferior vermis, 6 in left subtentorium, 1 in left cerebellar tonsil.

RESULTSThe tumors of 48 patients were totally removed. One patient occurred normal perfusion pressure breakthrough during surgery, and gastrointestinal hemorrhage on the second day after operation. Other patients all were intra- and postoperatively uneventful. Except for autoinfusion, no patients transfused heterogenous blood. There were no operative mortality and serious complications in this series.

CONCLUSIONSThe solid cerebellar hemangioblastoma was benign neoplasm. Abiding by properly operative techniques, the optimal results can be obtained.

Adolescent ; Adult ; Angiography, Digital Subtraction ; Cerebellar Neoplasms ; diagnosis ; surgery ; Child ; Diagnosis, Differential ; Female ; Hemangioblastoma ; diagnosis ; surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Microsurgery ; Middle Aged ; Neurosurgical Procedures ; Retrospective Studies ; Tomography, X-Ray Computed

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