A 56-year woman presented eyeball pain and blurred vision. MRI revealed a small well-delineated solid tumor in the apex of right orbit with optic nerve compression. Intraoperatively, the tumor was found very fibrous, hypervascular and adhesive to surrounding structures. The tumor was completely removed with the combination of endoscopic and microscopic technique. Patient experienced transient oculomotor nerve palsy, which completely recovered 3 months after surgery. Herein we report a rare case of angioleiomyoma in the orbital apex.
Adhesives ; Angiomyoma ; Endoscopy ; Female ; Humans ; Magnetic Resonance Imaging ; Oculomotor Nerve Diseases ; Optic Nerve ; Orbit ; Orbital Neoplasms

OBJECTIVE: To investigate the effect of bevacizumab in the treatment of children with optic pathway glioma (OPG). METHODS: A retrospective analysis was performed for the clinical data of 30 children with OPG who underwent chemotherapy. According to whether bevacizumab was used, they were divided into conventional chemotherapy (carboplatin, vincristine and etoposide) group with 12 children and combined chemotherapy (bevacizumab, carboplatin, vincristine and etoposide) group with 18 children. The children were followed up to 6 months after chemotherapy, and the two groups were compared in terms of visual acuity and tumor size before and after chemotherapy and adverse reactions during chemotherapy. RESULTS: The combined chemotherapy group had a significantly higher proportion of children achieving tumor regression than the conventional chemotherapy group (P<0.05), while there were no significant differences between the two groups in the proportion of children with improved visual acuity or adverse reactions (P>0.05). No chemotherapy-related death was observed in either group. CONCLUSIONS Bevacizumab combined with conventional chemotherapy can effectively reduce tumor size. Compared with conventional chemotherapy, such combination does not increase adverse reactions and can thus become a new direction for the treatment of OPG in children.
Antineoplastic Combined Chemotherapy Protocols ; Bevacizumab ; Carboplatin ; Child ; Humans ; Optic Nerve Glioma ; Retrospective Studies ; Vincristine

The most cases with orbital metastases have been reported in patients with a prior established diagnosis of cancer and widespread systemic involvement. However, ocular symptoms can be developed as an initial presentation of cancer in patients without cancer history. We report a case of rapid progression from trochlear nerve palsy to orbital apex syndrome as an initial presentation of advanced gastric cancer.
Diagnosis ; Diplopia ; Humans ; Neoplasm Metastasis ; Optic Nerve Diseases ; Orbit ; Stomach Neoplasms ; Trochlear Nerve Diseases ; Trochlear Nerve

Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous syndrome caused by mutations in the neurofibromin gene. NF-1 patients have a high risk of tumors, and optic glioma is the most commonly observed central nervous system tumor in these patients. However, glioblastoma is extremely rare in pediatric NF-1 patients. Here we report the discovery of a novel heterozygous c.6766_6767insAA (p.Ser2256Lysfs*4), pathogenic mutation in the neurofibromin gene in a 17-year-old boy with NF-1-associated glioblastoma.
Adolescent ; Central Nervous System ; Glioblastoma* ; Humans ; Male ; Neurocutaneous Syndromes ; Neurofibromatoses* ; Neurofibromatosis 1 ; Neurofibromin 1* ; Optic Nerve Glioma

PURPOSE: Solitary plexiform neurofibroma of the eyelid without neurofibromatosis is a rare disease. We report a case of solitary plexiform pigmented neurofibroma of the eyelid without neurofibromatosis. CASE SUMMARY: A 12-year-old male visited our clinic with a painless palpable subcutaneous mass on the right lower eyelid. He had a history of Batter syndrome and attention deficit hyperactivity disorder. On initial presentation, clinical features regarding neurofibromatosis such as Lisch nodule, optic nerve glioma, or high myopia were not observed. We performed excision and biopsy of the lower lid mass under general anesthesia. Macroscopically, the tumor was 4.0 × 1.5 × 1.5 cm in size with irregular nodules. Microscopically, the tumor consisted of multiple, variably sized tortous enlarged nerve fascicles with clusters of pigmented cells. Immunohistochemical results revealed expression of S-100 protein. Pigmented cells express both S-100 and melan-A proteins, while nonpigmented cells express S-100 protein only. The tumor was finally diagnosed as plexiform pigmented neurofibroma. Dermatological evaluation revealed no evidence of systemic neurofibromatosis. CONCLUSIONS: Plexiform neurofibroma should be considered in the differential diagnosis of an eyelid mass, even if the patient does not have a history or clinical features of neurofibromatosis. Plexiform neurofibroma can be successfully managed with surgical excision.
Anesthesia, General ; Attention Deficit Disorder with Hyperactivity ; Biopsy ; Child ; Diagnosis, Differential ; Eyelids* ; Humans ; Male ; MART-1 Antigen ; Myopia ; Neurofibroma ; Neurofibroma, Plexiform* ; Neurofibromatoses ; Neurofibromatosis 1 ; Optic Nerve Glioma ; Rare Diseases ; S100 Proteins

PURPOSE: To present a case of orbital inflammation and optic perineuritis preceding vesicular eruption in herpes zoster ophthalmicus(HZO). CASE SUMMARY: An 84-year-old woman with a history of gall bladder cancer and hypertension complained of left periorbital erythematous edema and discomfort. On examination, visual acuity was 20/25 bilaterally; no tenderness, proptosis or ophthalmoplegia was observed. Pupils were equal, round, and reactive to light without relative afferent pupillary defects. Slit-lamp examination revealed severe conjunctival injection and chemosis without keratitis or uveitis. The remainder of the ocular examination was unremarkable. Magnetic resonance imaging confirmed left-sided preseptal swelling with an enlarged left lacrimal gland, high signal intensity of the retrobulbar fat and optic nerve sheath. Systemic antibiotic therapy with steroids was started under a presumed diagnosis of idiopathic orbital inflammatory disease, but the clinical presentation was unresolved. After 2 days, vesicular lesions confined to the first division of the trigeminal nerve and pseudodendritic keratitis developed on the left side leading to a diagnosis of HZO. Treatment with acyclovir immediately resolved anterior segment inflammation and periorbital edema. While on therapy, visual acuity deteriorated to 20/125 and the pupil became dilated and unresponsive to light over a few days. All signs and symptoms of acute orbitopathy and postherpetic neuralgia had resolved 3 months later with the exception of pupil abnormality and visual acuity. CONCLUSIONS: HZO may present with symptoms and signs of orbital inflammation and optic perineuritis even in the absence of a vesicular rash. Thus, HZO should be considered in the differential diagnosis of unexplained acute orbital syndromes.
Acyclovir ; Aged, 80 and over ; Dacryocystitis ; Diagnosis ; Diagnosis, Differential ; Edema ; Exanthema ; Exophthalmos ; Female ; Gallbladder Neoplasms ; Herpes Zoster Ophthalmicus* ; Herpes Zoster* ; Humans ; Hypertension ; Inflammation* ; Keratitis ; Lacrimal Apparatus ; Magnetic Resonance Imaging ; Neuralgia, Postherpetic ; Ophthalmoplegia ; Optic Nerve ; Orbit* ; Pupil ; Pupil Disorders ; Steroids ; Trigeminal Nerve ; Uveitis ; Visual Acuity

Pilocytic astrocytomas (PAs) are World Heath Organization Grade I tumors and are most common in children. PA calcification is not a common finding and has been reported more frequently in the optic nerve, hypothalamic/thalamus and superficially located cerebral tumors. We present a cerebellar PA in a 3-year-old male patient with cystic components and massive calcification areas. The residual tumor grew rapidly after the first operation, and the patient was operated on again. A histopathological examination revealed polar spongioblastoma-like cells. Massive calcification is not a common feature in PAs and can lead to difficulties in radiological and pathological differential diagnoses.
Astrocytoma* ; Child ; Child, Preschool ; Diagnosis, Differential ; Humans ; Male ; Neoplasm, Residual ; Neoplasms, Neuroepithelial ; Optic Nerve ; Recurrence*

Chordoid glioma of the third ventricle is a rare and challenging tumor to surgery because of its unique anatomical location and its close juxtaposition to the neurovascular structures and hypothalamus. The authors report a case of chordoid glioma of the third ventricle in a 43-year-old woman, who presented with headache and somnolence. The tumor was approached by endoscopic transnasal technique with a favorable result. Histopathologic examination disclosed a neoplastic tissue composed of eosinophilic epithelioid cells, mucinous, periodic acid Schiff-diastase positive, extracellular matrix, and scattered lymphoplasmacytic infiltrates. The best treatment option remains controversial. Customarily, the surgical route to remove chordoid glioma is transcranial; however, the undersurface of the optic chiasm and optic nerves preclude an adequate surgical visualization. In contrast, an expanded endoscopic transnasal approach provides a direct midline corridor to this region without any brain retraction.
Adult ; Brain ; Eosinophils ; Epithelioid Cells ; Extracellular Matrix ; Female ; Glioma* ; Headache ; Humans ; Hypothalamus ; Mucins ; Optic Chiasm ; Optic Nerve ; Periodic Acid ; Third Ventricle*

OBJECTIVETo observe the treatment and prognosis of optic nerve invasion in retinoblastoma (RB).

METHODThe children who had been diagnosed with unilateral RB and had received enucleation from January 2006 to December 2013 in our hospital were recruited. Tumor extension into the optic nerve were disclosed. Optic nerve involvement was classified into four grades according to the degree of invasion. Grade I is superficial invasion of the optic nerve head only, grade II is involvement up to and including the lamina cribrosa, grade III is involvement beyond the lamina cribrosa, and grade IV is involvement up to and including the surgical margin. Grade I and II are called invasion of the optic nerve before the sieve plate. Grade III and IV are called invasion of the optic nerve after the sieve plate. Other high-risk factors included extensive invasions of the choroid, sclera, anterior chamber, iris, and ciliary body. They were divided into two groups according to whether the merger of other high histopathologic risk factors. Treatment was delivered accordingly, and the prognosis of different degrees of optic nerve invasion was observed. The subjects were followed up for 6 months to 7 years (average: 43 months).

RESULTThere were 465 subjects in this study, including 279 boys and 186 girls. The right eye was affected in 260 patients and the left eye in 205 patients. The average time from onset of symptoms to visit was 2.7 months (range 1 day-24 months). Twenty-five patients died, resulting in an overall survival rate of 94.6%. The mortality rate of patients with optic nerve involvement with grade I was 0.4%, grade II was 1.0%, grade III was 8.7% was and grade IV was 60.9%. Of the 338 with invasion of the optic nerve before the sieve plate, two died of recurrence, with a survival rates of 99.4% (336/338). Of the 127 patients who had invasion of the optic nerve after the sieve plate, twenty-three died of recurrence, with a survival rate of 81.9% (104/127) , the difference was statistically significant (χ² = 52.299, P = 0.000). A total of 379 patients did not have any other merged pathology high-risk factors, 8 died, the mortality rate was 2.1%. Of the 86 patients who had complicated with other high-risk factors, 17 died, the mortality rate was 19.8%, the difference was statistically significant (χ² = 42.955, P = 0.000). Of the 338 patients, 304 had invasion of the optic nerve before the sieve plate had not merged other pathology high-risk factors, none died, of the 34 patients who had complicated with other pathology high-risk factors, 2 had died, the mortality rate was 5.9%, the difference was statistically significant (P = 0.010). Of the 127 patients with invasion of the optic nerve after the sieve plate, 76 had not complicated with other pathological high-risk factors, 9 of whom had died, the mortality rate was 11.8%, 51 had complicated with other pathological high-risk factors, 14 of whom had died, the mortality rate was 27.5%, outcomes did significantly differ between the two subgroups (χ² = 5.014, P = 0.025). Cox multivariate analysis showed that invasion of the retrolaminar optic nerve, surgical margin of the optic nerve and sclera were influential factors of colorectal cancer.

CONCLUSIONPatients with optic nerve invasion have an excellent outcome with current therapy. But for those whose resection margin was invaded, which has a high incidence of recurrence, chemotherapy is recommended for patients with postlaminar optic nerve involvement.

Child ; Female ; Humans ; Male ; Multivariate Analysis ; Neoplasm Invasiveness ; Neoplasm Recurrence, Local ; Optic Nerve Neoplasms ; pathology ; surgery ; Prognosis ; Retinoblastoma ; pathology ; surgery ; Retrospective Studies ; Survival Rate

No abstract available.
Diagnosis, Differential ; Female ; Humans ; Middle Aged ; Optic Disk Drusen/*diagnosis ; Optic Nerve Neoplasms/*diagnosis ; Tomography, Optical Coherence