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The city of Hyderabad has had a distinct linguistic and political history. It was a multilingual city with Urdu (and Persian) as the court languages under the Asaf Jahi dynasty until 1948, when it ...

Primary sinonasal neuroendocrine carcinoma (SNEC) is a rare aggressive sinonasal malignancy which typically occurs in the ethmoidal or maxillary sinuses, with or without nasal cavity involvement, of middle-aged patients (median age 53 years), with a slight male preponderance. No risk factors have been identified. Most patients present at advanced stages due to the lack of significant symptoms.1,4,5,8 Advanced tumours may invade the skull, orbit or brain. Staging is of limited value in predicting prognosis and recent literature clearly highlights the importance of histological diagnosis, particularly differentiation grade, in determining the prognosis and predicting treatment response. Nomenclature has been ambiguous, but broadly SNECs can be classified as well-, moderately- or poorly differentiated. The latter group includes sinonasal undifferentiated carcinoma and sinonasal small cell carcinoma. On histological examination, well-to-moderately differentiated tumours show medium-sized cells with large nuclei containing stippled or ‘salt/pepper’ chromatin and scant cytoplasm. Nuclear moulding, increased mitoses and apoptotic bodies are commonly seen. Immunohistochemistry reveals expression of neuroendocrine markers.1,4–7 Poorly-differentiated tumours may lose expression of neuroendocrine markers and differentiation from other poorly differentiated malignancies can be extremely difficult.1,4–7 Due to the limited number of reported cases, there is no clear consensus on management, although oncologists now advocate multimodal therapy. Combined surgery and radiotherapy is thought to beneficial in moderately and poorly-differentiated subtypes.1,4–8 We describe a classical case of SNEC with secondary orbital involvement, with a review of the current literature.

Hyperkeratosis lenticularis perstans (Flegel disease) is a rare, benign hyperkeratotic skin disorder which typically occurs in lower extremities of Caucasian middle-aged patients. Most cases are sporadic although familial cases with an autosomal dominant mode of inheritance have been reported.1–5 Clinically the condition mimics many other hyperkeratotic and inflammatory disorders and the diagnosis is only confirmed on histopathological and clinical correlation.2,5,6 The condition typically presents with asymptomatic keratotic/scaly red/brown papules which histomorphologically show lamellar hyperkeratosis with abrupt peripheral basket-weave orthokeratosis, irregular acanthosis and underlying lichenoid lymphocytic infiltrate.1–7 The pathogenesis remains unclear and thus various topical and/or systemic treatments have shown variable success rates. Herein we describe a classical case with review of the current literature.

Neo-liberal reforms of the 1990s in India brought new modes of operation which made cities as the central nodes of the economy. The cities were now reconfigured as servicing hubs for the gl...

Current treatments for metastatic uveal melanoma (mUM) are limited and rarely prolong patient survival. Immunotherapy trials for mUM are few and to date have demonstrated only marginal success. High densities of tumor-associated macrophages (TAMs) and infiltrating T lymphocytes (TILs) in primary UM are associated with poor prognosis. Little is known about the immune microenvironment of mUM. Our aim was to examine the presence and distribution of TAMs and TILs in mUM within the liver. Whole-tissue sections of liver mUM (n=35) were examined by immunohistochemistry. For TAMs, monoclonal antibodies against CD68 and CD163 were used. Macrophage density and morphology were scored using previous established systems. Density and spatial distribution of TILs were highlighted using antibodies against CD3 (pan-lymphocyte marker), CD4 (T-helper cells), and CD8 (T-cytotoxic cells). CD68+ and CD163+ TAMs were seen within the tumor in all 35 specimens; their density was "moderate" in 50% of cases and "few" in 43%, and the majority showed an "indeterminate" phenotype. CD3+ TILs were noted both within mUMs and surrounding the tumor. Of these, CD8+ TILs were "few" in number within mUM but were predominantly seen peritumorally at the tumor/normal liver interface, whereas CD4+ TILs showed a high perivascular density within mUM. CD68+ and CD163+ TAMs of "indeterminate" morphology were observed in mUM, suggesting a tendency toward the protumorigenic M2 phenotype. CD4+ TILs were seen within the mUM, whereas CD8+ TILs tended to be peritumoral. The biological and functional roles of inflammatory cells in mUM require further investigation to determine if they represent potential targets for future therapies in mUM.

Background/aims Iris melanomas (IM) are rare and have a lower mortality than posterior uveal melanomas (UM). Our aims were to determine the prevalence of genetic changes associated with prognosis of posterior UM, in both treated and non-treated IM. Methods Retrospective database review and molecular analysis of all patients diagnosed with IM at the Liverpool Ocular Oncology Centre (LOOC) between 1993 and 2015. Archival pathology specimens of confirmed IM cases were analysed for chromosomal alterations, using multiplex ligation-dependent probe amplification (MLPA) or microsatellite analysis (MSA) depending on DNA yield, and BRAF mutation status. Results 5189 patients were diagnosed with intraocular melanoma at LOOC from 1993 to 2015. Of these, 303 (5.8%) patients were diagnosed with IM. Tissue samples were available for 26 IM cases. Twelve of these cases had biopsies taken post-proton beam radiotherapy (PBR). Histological subtyping showed 14 IM being spindle, 2 epithelioid and 10 were of mixed cell type. Twenty of the 26 IM cases (77%) analysed genetically were classified as either disomy 3 (n=16) or monosomy 3 (n=4). Chromosome 6p gain was detected in 4/18 (22%) IM, and polysomy 8q in 6%. BRAF mutations were not detected in any of the four IM cases examined. One patient with IM died from metastatic disease: this tumour was disomy 3 with 6p and 8q gains. All other patients were alive with no evidence of metastases at study closure. Conclusions Chromosomal aberrations seen in posterior UM can also be demonstrated using MLPA or MSA in both treatment naive and PBR-treated IM. Most IM display a low-metastatic risk chromosomal profile.

Purpose Sarcoidosis is a chronic idiopathic granulomatous inflammatory disease that can affect many major organ systems, primarily the lungs, and hence has remarkable clinical heterogeneity. At least 50% of patients with systemic sarcoidosis develop inflammatory eye disease, and in approximately 21% of cases, it may be the first clinical manifestation. Neuro-ocular involvement occurs in, Highlights • Optic nerve head (ONH) sarcoidosis without uveal tract involvement is rare. • ONH sarcoidosis can be the first manifestation of systemic sarcoidosis. • ONH sarcoidosis may be the first manifestation of neuro-ocular sarcoidosis. • ONH masses present diagnostic challenges due to the impact of tissue biopsy. • ‘Sarcoid-like’ reaction or sarcoid-lymphoma syndrome may occur with concurrent lymphoma.

Background: Lymphomas of the lacrimal sac are rare, accounting for less than 10% of lacrimal sac malignant tumours. They may present with symptoms typical of secondary acquired nasolacrimal duct obstruction and are thus often misdiagnosed. Methods: Case series and literature review. Results: Herein we describe 3 cases of chronic lymphocytic leukaemia (CLL)/small-cell lymphocytic lymphoma (SLL) of the lacrimal sac with immunohistochemical and in 1 case molecular confirmation. Conclusion: Lymphomas of the lacrimal sac should be suspected in patients with known CLL presenting with epiphora and dacryocystitis. During dacryocystorhinostomy, an incisional biopsy of the lacrimal sac is essential for confirming CLL/SLL involvement and may guide treatment.

Chondroid syringoma or mixed skin tumour is a rare, benign skin adnexal tumour which typically occurs in the head and neck region of middle-aged patients. Due to their rarity, they are often clinically misdiagnosed and with the diagnosis only confirmed on histological examination. They typically comprise sweat gland elements within a prominent ‘cartilage-like' stroma. Treatment involves total excision with clear surgical margins. Where excision is incomplete, routine follow up is required due to risk of recurrence or malignant transformation, though the latter is extremely rare. Herein we describe a classical case with review of the current literature.

Tumors of the lacrimal sac are rare but their recognition and early management are imperative, as they are locally invasive and potentially life-threatening. Because of their rarity, large clinical studies with statistically significant data on the natural course, management, and prognosis of these neoplasms are unavailable. Current practices are therefore based on a few case series and a small number of isolated case reports. Most tumors are primary and of epithelial origin (60-94%), of which 55% are malignant. Lacrimal sac tumors typically present with epiphora and a palpable mass over the medial canthus and are thus often erroneously diagnosed as chronic dacryocystitis. A full history with clinical and diagnostic workup is essential to plan treatment, which is often multi-disciplinary. Statistically significant associations have been shown with higher tumor staging and size with increased metastatic risk and lower survival rates. Management usually involves complete surgical resection with adjuvant radiotherapy and/or chemotherapy for malignant lesions. Long-term follow-up is required, as recurrences and metastases can occur many years after initial treatment.

This review aims to cover the transplant procedures that have been developed and investigated as potential treatments for age-related macular degeneration. The choice of transplant materials that will be discussed ranges from isolated cells (including stem cells, iris pigment epithelial cells and retinal pigment epithelial cells) to monolayers of cells on artificial or nonartificial substrates and also to whole patches of tissue (e.g., fetal retina, full-thickness patch grafts and macular relocation). Finally, we will address the current and future technologies and questions that need to be addressed in order that transplant procedures can have an effective role in the treatment of patients with age-related macular degeneration.

Purpose To investigate the incidence and outcome of cornea transplant rejection following endothelial keratoplasty (EK) and penetrating keratoplasty (PK) for Fuchs endothelial dystrophy (FED) and pseudophakic bullous keratopathy (PBK). Design Multicenter cohort study. Methods Patients registered on the United Kingdom Transplant Registry (UKTR) who had an EK or PK for FED or PBK between April 1, 2005 and March 31, 2011 were included. Data were collected from UKTR forms at 1 and 2 years. Postoperative steroid use varies between surgeons and cannot be captured in this reporting system. Rejection events were identified as those recorded as endothelial rejection. Results A total of 3486 corneal transplants were undertaken: 1973 for FED, 1513 for PBK. For FED, 2-year rejection-free survival was 93% (95% confidence interval [CI] 90%–94%) for PK and 94% (95% CI 92%–96%) for EK ( P = .3). In transplants that had a rejection episode, 50% of PKs (17) and 60% of EKs (15) subsequently failed. For PBK, 2-year rejection-free survival for PK was 88% (95% CI 86%–90%) and 90% (95% CI 86%–92%) for EK ( P = .6). In transplants that had a rejection episode, 85% of PKs (41) and 76% of EKs (22) subsequently failed. Inflammation (ie, conjunctival injection, presence of keratic precipitates and intraocular signs) at the time of surgery for patients with FED was significant for developing rejection: 3.5 times greater compared with those with no inflammation ( P = .02). Conclusions There is no significant difference in rejection-free survival between EK and PK for FED or PBK. The presence of inflammation is an important risk factor, and attention to its control before and following surgery is important.

The aim of this study was to produce a heavy tamponade with a specific gravity greater than 1.06 g/mL that was optically transparent, could be manufactured using simple processing, could be injected using standard clinical equipment, and would have appropriate biocompatibility.Aerosil silica was added to a phenyl trimethicone and mixed via a roller, overhead stirring, and ultrasonics. The refractive index, visible absorbance, and shear viscosity were measured. The injectability of the solutions was evaluated using the Accurus Viscous Fluid Injection system. The tamponade efficiency was assessed using a model eye chamber and compared with that of Densiron 68, Oxane HD, and F6H8. The biocompatibility was evaluated in vitro and in vivo in rabbits.Tamponade agents were produced with specific gravities of 1.10, 1.11, 1.13, and 1.16 g/mL that had good optical clarity. Mixing using overhead stirring was sufficient to produce tamponade agents with shear viscosities in the range 1000 to 5000 mPa·s that were reproducible and stable during storage. The solutions were easier to inject using the Accurus Viscous Fluid Injection system than silicone oil 1000 mPa·s. The 11% silica solution had greater tamponade efficiency than Densiron 68 or Oxane HD. There was no evidence of cytotoxicity in vitro. Silica solution 11% induced cataract earlier than Polydimethylsiloxane 1000 (PDMS 1000). Silica solution 11% and phenyl trimethicone reduced the a-wave value at 1 week after vitrectomy, but recovery was observed at later time points. Silica solution 11% caused inner nuclear layer (INL) nuclei dropdown in inferior retina from 4 weeks postoperation. Polydimethylsiloxane 1000 induced a similar phenomenon in superior retina 12 weeks postoperation.We have produced a heavy tamponade with good clarity that has appropriate shear viscosity, injectibility, enhanced tamponade efficiency, and biocompatibility similar to that of PDMS 1000.

Background Retinal pigment epithelial (RPE) transplantation presents a potential treatment for age-related macular degeneration (AMD). A suitable transplant membrane that can support an intact functioning RPE monolayer is required. Expanded polytetrafluoroethylene (ePTFE) possesses the physical properties required for a transplanting device; however, cells do not attach and spread on ePTFE. This study investigated the ability of surface-modified ePTFE to optimise the growth and function of healthy RPE monolayers. Methods ePTFE discs were modified by ammonia gas plasma treatment. ARPE-19 cells were seeded on the membranes and maintained in media supplemented with retinoic acid and reduced serum. Cell number, morphology and proliferation were analysed. RPE monolayer function was investigated through formation of cell–cell junctions and phagocytosis of photoreceptor outer segments (POS). Results Ammonia gas plasma treatment resulted in enhanced cell growth and good monolayer formation with evidence of cell–cell junctional proteins. Furthermore, RPE monolayers were able to phagocytose POS in a time-dependent manner. Conclusions ePTFE can be surface-modified to support an intact functional monolayer of healthy RPE cells with normal morphology and the ability to perform RPE-specific functions. Following further investigation ePTFE may be considered for use in transplantation.