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

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

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 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 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