Optic nerve motion and gaze direction: Their impact on intraorbital tumor radiotherapy.

PURPOSE: Management of inter- and intra-fraction movements of target volumes and organs at risk (OARs) during radiotherapy is essential. While there is little OAR or target volume movement, the movements and orientation of the eyes can be significant during radiotherapy and they can affect the position of the optic nerve. The objective of the present study was to assess the variations of the optic nerve position due to gaze direction and to discuss their clinical consequences on the radiation treatment of intraorbital tumors. MATERIAL AND METHODS: Three patients without a history of oculomotor nerve palsy underwent six CT acquisitions with a thermoplastic mask: eyes open with different gaze directions (straight ahead, left, right, up, down) and eyes closed. The acquisition with the straight-ahead gaze was chosen as the reference position. Left and right optic nerves were segmented on the six acquisitions, and total volumes and maximum amplitude motions were calculated in three dimensions. RESULTS: Maximum differences were observed while looking left and up, with a median maximum amplitude of 5 and 6mm [range: 2-7mm], respectively. These motions induced a position variation of more than 50% of the volume of the optic nerve (compared to the reference position). Greater variations of motion were observed for the anterior portion of the nerve. The gaze position with the fewest variations compared to the reference position was eyes closed. CONCLUSION: Optic nerve positions vary significantly due to the gaze direction, especially for the anterior portion of the nerve. These variations should be taken into account for the treatment of small intraorbital tumors involving the anterior third of the optic nerve.

The diagnostic value of 123I-IMP SPECT in ocular adnexal lymphoma.

BACKGROUND: N-isopropyl- (123I) p-iodoamphetamine (123I-IMP) is specifically accumulated in primary central nervous system lymphoma (PCNSL) during single-photon emission tomography (SPECT) and contributes to its diagnostic imaging. However, whether 123I-IMP is accumulated in ocular adnexal lymphoma (OAL), one of the malignant intraorbital tumors, remains unclear. This study aimed to evaluate the diagnostic value of 123I-IMP SPECT in OAL. METHODS: Between August 2005 and June 2020, 26 patients with intraorbital tumors underwent neurosurgery at the tertiary care center. Of these, 15 patients who underwent 123I-IMPSPECT before surgery were retrospectively examined. The region of interest was set in the cerebellum ipsilateral to the intraorbital tumor on 123I-IMP SPECT, and the tumor-to-cerebellum ratio (T/C ratio) was calculated using the following formula: T/C ratio = [accumulation of tumor (count/pixel)]/[accumulation of ipsilateral normal cerebellar hemisphere (count/pixel)]. RESULTS: Six patients were included in the OAL group, who were pathologically diagnosed with extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), diffuse large B-cell lymphoma (DLBCL), and plasmacytoma. The T/C ratio in the OAL group was statistically higher than that in the non-OAL group (p < 0.01). The optimal cutoff values for both groups were between 0.76 and < 0.93. The sensitivity and specificity were 1.00, respectively. CONCLUSIONS: 123I-IMP SPECT is useful as one of the examinations in the differential diagnoses of OAL, because it showed a significantly higher accumulation in OAL group than in non-OAL group.

Alveolar soft part sarcoma of the orbit: A case report.

Alveolar soft part sarcoma is a rare soft tissue neoplasm that accounts for approximately 1% of all sarcomas and is usually identified in the extremities in adults. The occurrence of alveolar soft part sarcoma in the orbit is extremely rare, estimated at approximately 5% - 15% among all cases of alveolar soft part sarcoma . Here, we present a case of 29-year-old woman with orbital alveolar soft part sarcoma. We describe the magnetic resonance and F-18 2-fluoro-2-deoxy-D-glucose-position emission tomography/computed tomography findings of this case. This young woman had a spindle-shaped mass. A higher signal compared to the extraocular muscle on T1-weighted images, numerous flow voids on T2-weighted images, and intense enhancement could be key findings of this disease.

Infantile intraorbital Ewing's sarcoma: case report and review of the literature.

We reported a 7-month-old female with intraorbital Ewing's sarcoma. Infantile Ewing's sarcoma is rare and its prognosis is poor. Ewing's sarcoma of orbital origin is even rare. There has been only 1 case of infantile intraorbital Ewing's sarcoma reported, and only 5 infantile primary orbital ESFTs (Ewing's sarcoma family of tumors) have been reported. Among these 5 cases, 2 infants who did not receive multimodal therapy died, whereas 3 who received multimodal therapy demonstrated long-term survival. The present case was also treated with multimodal therapy consisting of surgery, chemotherapy, and proton beam radiotherapy. There is no recurrence at 15 months follow-up. No specific treatment strategies have been established yet, and accumulation of cases is necessary. Ewing's sarcoma should be included in the differential diagnosis of infantile intraorbital tumors.

Intraorbital Solitary Fibrous Tumor Requiring Preoperative Embolization of Feeding Artery.

Solitary fibrous tumors (SFTs) are derived from mesenchymal cells and commonly develop in thoracoabdominal organs; however, their occurrence in orbit is rare. The first-choice treatment is to surgically remove as much of the SFT as possible; however, if total removal is not achieved, the recurrence rate is high, resulting in poor prognosis. A 42-year-old man presented with painless right-sided proptosis and diplopia 4 years ago. Orbital computed tomography revealed a right extraconal mass medial to the optic nerve, measuring 25 mm. Magnetic resonance imaging demonstrated iso-signal intensity on T1- and T2-weighted imaging, including flow-void signals. During biopsy of the intraorbital mass, which was performed by ophthalmologists 3 years earlier, difficulty with hemostasis occurred due to massive hemorrhage from the mass. The mass grew to reach a maximal diameter of 33 mm, resulting in referral to the authors' department. Diagnostic cerebral angiography revealed a hypervascular orbital tumor with multiple feeding arteries. To control intraoperative bleeding, the patient underwent preoperative endovascular embolization. Subsequently, the tumor was completely removed using a combination of microsurgical craniotomy and endoscopic endonasal approach, without the occurrence of massive intraoperative hemorrhage from the tumor. Postoperatively, his clinical course was uneventful except for the remaining preoperative diplopia. The tumor was diagnosed histologically as SFT and has not recurred for 8 months since surgery. Preoperative intravascular embolization of branches of the ophthalmic artery can be performed safely, resulting in excellent control of intraoperative bleeding and facilitating complete removal of SFT without additional complications.

[Primary meningioma of the optical nerve sheet in infancy as initial presentation of neurofibromatosis type 2]. / Primäres Meningeom des Nervus opticus im Säuglingsalter als Erstmanifestation einer Neurofibromatose Typ 2.

Intraorbital meningiomas are rare tumors, making up less than 4% of all intraorbital tumors. Intraorbital meningiomas of childhood are curiosities with only few documented cases. We present the case of an 8­month-old male infant, presenting with strabismus and nystagmus. Magnetic resonance imaging showed a long segment thickening of the optical nerve and an intraocular tumor. The tumor was suspicious for retinal dysplasia and enucleation of the eye was performed to exclude malignancy. Histological examination revealed a meningothelial meningioma (WHO grade I), extending along the optical nerve and into the eye accompanied by retinal dysplasia and epiretinal membranes. Meningiomas of childhood, retinal dysplasia, and epiretinal membranes are regularly associated with neurofibromatosis type 2. Subsequent genetic analysis led to the final diagnosis. This case documents a very unusual early beginning of a neurofibromatosis type 2.

The tragedy of Thomas Crawford, sculptor of the Statue of Freedom.

The Statue of Freedom, which sits on top of the Capitol Dome in Washington, DC, was created by Thomas Crawford. At the peak of his career in 1856, while busy completing multiple commissions for the new Capitol extensions, he developed diplopia and consulted the leading oculists of the time in Paris, Drs. Desmarres and Sichel, who were certain he had an intraorbital tumor. Two American physicians were also involved in Crawford's care. Both became controversial after treating Crawford, one because of his method of diagnosis, the other because of his unproven method of treating cancer. Desperate, Crawford agreed to undergo an experimental treatment that destroyed the eye and orbital contents. He died 5 months later at age 44 and never saw any of his sculptures placed in the Capitol building.