A conjunctival inclusion cyst is a colorless or whitish translucent cystic lesion, frequently occurring on the conjunctiva. Here, we describe a patient with a giant conjunctival cyst, mainly existing in the orbit, which clinically resembled lymphatic malformation. A 34-year-old male complained of a subconjunctival mass on his left infero-medial side persisting since childhood. It had gradually enlarged over the 3 months before the initial visit. He had no history of trauma, surgery, or conjunctivitis. The mass was dark red, with superficial dilated blood vessels. Magnetic resonance imaging showed a well-circumscribed mass in the inferior orbit, measuring 12 × 25 mm, which had an internal septum. The lesion was completely resected without rupture of the mass through a transcutaneous approach. Histopathologically, the mass was a cyst composed of columnar epithelium with goblet cells and lymphoid follicles adjacent to the wall. In conclusion, a giant orbital conjunctival cyst and a lymphatic malformation should be differentiated at diagnosis.

In this study, female patients are more predominant in IgG4-ROD. While inflammation recurs in one-third of patients, this study do not identify factors associated with recurrence after systemic corticosteroid administration.

Analyses of cell surface markers using FCM were useful in differentiating EMZL from IgG4-ROD/IOI. Sensitivity of Ig light chain restriction was relatively low in diagnosis of EMZL using FCM.

The aim of the present study was to investigate the clinicopathological features of cystic lesions in the eyelid. Clinicopathological profiles were retrospectively searched based on medical records of 35 patients with cystic lesions of the eyelids, which were surgically resected from January 2003 to June 2016. The cohort consisted of 16 men and 19 women. The mean age of the patients was 57±24 years. The main locations of the cysts were the upper eyelid in 22 patients, followed by the lower eyelid in 5 patients. Eyelid skin and its appendages were the most common as the origin of cysts, followed by the tarsus, palpebral conjunctiva and lacrimal gland. The histopathological diagnoses were 16 epidermal cysts, 5 intratarsal keratinous cysts, 3 conjunctival cysts, 2 trichilemmal cysts, 1 dermoid cyst, 1 apocrine hidrocystoma, 1 lacrimal gland cyst and 6 unclassifiable cysts. All cysts did not recur following resection. In summary, cystic lesions of the eyelid were frequently observed in the upper eyelid. The most frequent diagnoses were epidermal cysts, followed by intratarsal keratinous cysts, while a definitive diagnosis in some cysts could not be pathologically determined, as they lacked epithelia and/or the contents or they resembled apocrine hidrocystoma and intratarsal keratinous cysts with atypical findings. The results of the current study indicate that the incidence and differential diagnosis of eyelid cystic lesions may contribute to the application of appropriate treatment for patients with eyelid tumors.

Background/Aim: Orbital solitary fibrous tumor (SFT) is a rare lesion among orbital tumors, which can be misdiagnosed as another mesenchymal tumor. In this study we report two cases of orbital SFT, focusing on the imaging and pathological findings of the vascular structure inside the tumor. Case Report: A 26-year-old woman and 43-year-old man presented with orbital SFT. The pathological findings revealed a patternless growth pattern of the tumor cells and hemangiopericytoma-like vascularity as well as thickened, dilated blood vessels within the tumor tissue. Tumor cells revealed a diffuse strong positivity for cluster of differentiation 34 (CD34) and signal transducer and activator of transcription 6 (STAT6) in both cases, while Bcell lymphoma 2 (bcl-2) and CD99 were positive in one case. Characteristic findings within the tumor were the arterial components, where a variety of STAT6, CD99 and bcl-2positive smooth muscle cells were intermingled. Conclusion: Histologically, the tumor tissues might be characterized by not only conventional hemangiopericytoma-like vasculature but also dilated arterial vessels, which were shown to be part of the tumor components.Solitary fibrous tumor (SFT), a rare spindle-cell tumor, commonly arises from the pleura (1) but may also occur in extrapleural sites (2-5). Orbital SFT is a rare lesion and about 90 cases have been reported in the literature to date (6, 7). This tumor is likely to simulate other mesenchymal tumors such as fibrous histiocytoma, liposarcoma, synovial sarcoma, and neurofibroma. Since SFT is rich in feeding arteries, preoperative clinical diagnosis is important in assessing the likelihood of intraoperative bleeding (8), and total tumor resection is recommended (9). However, histopathological findings of the feeding vessels in SFT remain unknown. In this study, we report two cases of orbital SFT, focusing on the imaging and pathological findings of the vascular structure inside the tumor. The institutional review board in Hokkaido University and Teine Keijinkai Hospital waived the ethical assessment of the clinical study because of case reports. This study adhered to the principles of the Declaration of Helsinki. Case ReportCase 1. A 26-year-old woman presented with progressive and painless lower eyelid swelling for 2 months. An elastic hard, non-tender and non-pulsatile mass was palpable in the lower eyelid. Computed tomography revealed a well-defined heterogeneous mass along the lower wall of the right orbit (Figure 1A). Magnetic resonance imaging (MRI) demonstrated an orbital lesion with an isointense signal on T1-weighted images (T1WI) (Figure 1B) and a hyperintense signal on T2weighted images (T2WI) (Figure 1C). Postcontrast T1WI revealed a strong enhancement of the lesion that contained a linear flow void-like hypointensity (Figure 1D, arrow). Surgical excision was performed. There was bleeding from the 3649 This article is freely accessible online.

BackgroundOphthalmologists and retina specialists may consider choroidal detachment if patients with rhegmatogenous retinal detachment present with choroidal elevation. That misdiagnosis may lead to inappropriate treatments, development of tumor cell dissemination, and eventual promotion of patient death. We report a case of a patient with rhegmatogenous retinal detachment associated with choroidal melanoma simulating choroidal detachment according to fundus findings.Case presentationA 78-year-old Japanese woman with blurred vision in her right eye was referred to our hospital because of rhegmatogenous retinal detachment with complicated atypical choroidal detachment. Her intraocular pressure was normal with clear anterior chamber. Retinal detachment involving the inferior and nasal retina was observed, and a retinal hole was noted in the same quadrant. A small yellowish choroidal elevation was located in the inferonasal site. Gadolinium-enhanced magnetic resonance imaging revealed enhancement corresponding to the elevation, leading to the identification of a choroidal tumor. Enucleation of the patient’s right eye was eventually performed. The enucleated eye histologically demonstrated malignant melanoma.ConclusionsIf hypotony or an inflammatory sign is absent, ophthalmologists should pay attention to the differential diagnosis of choroidal elevations observed in such patients.

PurposeTo report an anti-recoverin antibody-positive cancer-associated retinopathy (anti-recoverin CAR) patient with remarkable improvements of visual function and outer retinal morphology following spontaneous regression of cancer.ObservationsA 65-year-old woman with small cell lung carcinoma developed progressive, bilateral vision loss with diffuse loss of the ellipsoid zone at the macula on optical coherence tomography and marked reduced responses of a- and b-waves on electroretinography. Western blot analysis led to a diagnosis of anti-recoverin CAR. The visual function and outer retinal morphology gradually improved following spontaneous regression of the cancer and the initiation of systemic corticosteroid. Subsequent intermittent chemotherapy and continuation of corticosteroid maintained reduction of the cancer and prevented the recurrence of CAR, with preservation of improvements of the visual function and macular outer retinal morphology.Conclusions and importanceThese results suggest that requirement for obtaining good visual prognosis in CAR patients is to make the cancer regress prior to falling into photoreceptor apotosis.

Purpose: Flow cytometry (FCM) is used to evaluate cell surface markers of various leukocyte populations quantitatively. However, little is known about the usefulness of FCM in follicular lymphoma (FL) of the ocular adnexa. The aim of this study was to evaluate the clinicopathological features and FCM results in ocular adnexal FL. Materials: This is a retrospective multicenter case study on clinical and immunohistochemical features. All tumors, surgically excised, were diagnosed based on histopathology, immunoglobulin heavy chain gene rearrangement, and FCM. The percentages (%) of B-cell markers, T-cell markers, a natural killer cell marker, and cell surface kappa/lambda measured by FCM analysis in tumor tissues were searched based on medical records. Results: This study enrolled nine tumors in eight FL patients (three men and five women). The median age at the time of initial presentation was 74 years. All the tumors surgically excised histologically exhibited cluster of differentiation (CD)10, CD20, and BCL2-positive cells. At the time of ophthalmic diagnosis, lymphomas were already disseminated throughout the body in five cases. FCM demonstrated high percentage of B-cell markers including CD10, CD19, CD20, and CD23 in all nine tumors. CD10 population was 73.5% ± 11.9% in seven out of nine tumors, while that in the other two tumors was particularly low being 11.7% ± 1.13%, which showed the relatively high T-cell lineages compared to the other seven tumors. Conclusion: For ophthalmologists involving managements of ocular adnexal tumors, FCM can provide useful information for complementing the diagnosis and understanding pathophysiology of FL.