Schwannomas rarely present as intraocular tumors and are often misdiagnosed as malignant melanoma. We describe a choroidal schwannoma confirmed by sclerouvectomy. A 30-year-old woman presented with a large nonpigmented intraocular mass of the choroid in the right eye and underwent surgical excision by sclerouvectomy. Histologically, the tumor was composed of a mixture of cellular solid components (Antoni A) and loose myxoid components (Antoni B). The tumor was eventually diagnosed as a schwannoma. Currently available ancillary studies are still of little value in definitively differentiating schwannomas from other choroidal tumors. In the case of atypical findings for a malignant melanoma, a benign neoplasm should be included in the differential diagnosis. This patient avoided enucleation by first having the mass excised. We are unaware of previous reports in which a choroidal schwannoma was diagnosed by surgical excision.
Adult ; Choroid/*pathology ; Choroid Neoplasms/*diagnosis/surgery ; Diagnosis, Differential ; Eye Enucleation/*methods ; Female ; Humans ; Magnetic Resonance Imaging ; Neurilemmoma/*diagnosis/surgery

BACKGROUNDNeurofibroma is a kind of benign neoplasm that derives from nervous tissues. Though this tumor is the most common types in the peripheral nervous system, it is rarely seen in the choroid and easy to be misdiagnosed of choroidal melanoma. The aim of this study was to review the clinical features of neurofibroma of the choroid in the Chinese race.

METHODSA retrospective case series design was used. Two male and one female patients diagnosed with choroidal neurofibroma in Beijing Tongren Eye Center were included in this study. The clinical histories were abstracted from the patients' medical records. Routine eye examinations including visual acuity, intraocular pressure, slit lamp and ophthalmoscope were performed. Auxiliary examinations included fluorescein fundus angiography (FFA), AB-ultrasound scan, color doppler imaging (CDI), and magnetic resonance imaging (MRI). Local resection of the tumors was performed and the specimens underwent pathological examinations.

RESULTSThe tumors were of yellow-pink color with pigmentation on the surface. CDI showed arterial blood signals in the tumor and MRI showed high-intensity in the T1-weighted image and a slightly increased intensity in the T2-weighted image. FFA and indocyaninegreen angiography demonstrated the tumors were of hypofluorescence at early stage and hyperfluorescence with prominent leakage at late stage. The pathological examination confirmed the tumors were choroidal neurofibroma. After 5 - 10-year follow-up, there were no recurrences of the tumors and the retinas were well attached.

CONCLUSIONSChoroidal neurofibroma is difficult to be diagnosed clinically and pathological confirmation is important. These tumors can be managed conservatively by local resection.

Adult ; Choroid Neoplasms ; diagnosis ; surgery ; Female ; Humans ; Male ; Middle Aged ; Neurofibroma ; diagnosis ; surgery ; Retrospective Studies

Adult ; Choroid Plexus Neoplasms ; pathology ; Diagnosis, Differential ; Ear Neoplasms ; pathology ; radiotherapy ; surgery ; Endolymphatic Sac ; pathology ; Glomus Jugulare Tumor ; pathology ; Humans ; Immunohistochemistry ; Magnetic Resonance Imaging ; Male ; Meningioma ; pathology ; Skull Neoplasms ; pathology ; radiotherapy ; surgery ; Temporal Bone ; pathology ; 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