Does the histopathological subtype of primary basal cell carcinoma predict the subtype of secondary tumours? What role do genetic mutations play?

Background: Basal cell carcinoma (BCC) is one of the most common malignancies in the world. The frequency of histopathological subtypes and the distribution on the body of BCC has been well documented. Less has been written on the nature of secondary tumours. The genetics of BCC is starting to be understood, particularly with the advent of newer medical treatments (hedgehog inhibitors). Objectives: To determine if primary basal cell carcinoma histopathological subtype predicts secondary tumour subtype, as well as their anatomical distribution. Methods: A retrospective case series of patients over the age of 18 was performed from 2009 to 2014, with at least two separate diagnoses of BCC. Results: In 394 identified patients, a total of 1355 BCCs arose in the cohort over the 6-year study period. The number of secondary BCCs per patient ranged from 2 to 19 tumours. Nodular BCC was the most likely to reoccur in secondary tumours (53.3%), followed by mixed subtypes (45.7%). Conclusions: Within our study, we did find a predisposition for secondary BCCs to be of the same histopathological subtype as the primary, particularly with respect to nodular and mixed tumours. Furthermore, we found that secondary tumours were also more likely to occur on the same anatomical site as the primary tumour. We are only just beginning to under the genetic mutations involved in subtype formation.

Acute Leukemia Presenting in the Pediatric Orbit.

We present a case series to evaluate the clinical features of acute leukemia presenting with primary orbital manifestations. We undertook a retrospective case review of primary orbital presentations of acute myeloid leukemia (AML) and acute lymphocytic leukemia (ALL) over a 10-year period at two hospital sites (Hereford County Hospital and Leicester Royal Infirmary). Our case series included four patients - two with AML and two with ALL. Patients were young (mean age of four years and five months) at presentation, all with unilateral disease, and presented with orbital signs. Although there was some confusion with the diagnosis at the time of referral, a suspicion of malignancy was made rapidly once ophthalmic review was initiated. All four cases were diagnosed with the assistance of peripheral blood film and bone marrow biopsy, without the need for orbital biopsy. All four cases had resolution of the orbital mass and remain disease-free.

Reversible Charles Bonnet syndrome secondary to upper lid ptosis.

Complex visual hallucinations can occur in visually impaired individuals with no underlying psychiatric disorder. This phenomenon is known as Charles Bonnet syndrome (CBS). It is more common in elderly patients who are suffering from impaired vision due to ocular or neurological disease processes, resulting in sensory deprivation. We report a case of CBS in an elderly female with marked ptosis, which was exacerbated following a knee replacement surgery under general anaesthesia. Her CBS symptoms persisted until surgical correction of the ptosis, with a rapid and dramatic resolution of her hallucinations. Although CBS is typically a chronic condition, unusually in this case it was acute and reversible.

Ocular complications and mortality in peripheral ulcerative keratitis and necrotising scleritis: The role of systemic immunosuppression.

IMPORTANCE: Inflammatory ocular diseases are associated with systemic disease, ocular morbidity and mortality. BACKGROUND: To examine clinical characteristics of subjects with peripheral ulcerative keratitis (PUK) and necrotising scleritis. DESIGN: Retrospective case series in a single tertiary centre over 10 years. PARTICIPANTS: All patients presenting with necrotising scleritis, PUK or scleritis with corneal infiltrates. Fifty-two eyes (41 subjects) were identified. METHODS: Demographic and clinical data analysis. MAIN OUTCOME MEASURES: Visual loss, ocular complications and mortality. RESULTS: Mean follow up was 7.0 ± 5.3 years, presentation age 51.3 ± 18.6 years, and 23 subjects (56.1%) were female. Overall, 27 eyes presented with PUK, 26 with necrotising scleritis, and 22 with corneal infiltrates associated with active scleritis. Associated systemic diagnosis was observed in 12 subjects at presentation, and in 21 subjects (51.2%) by final follow up. Recurrence occurred in 23 eyes (44.2%): 10 eyes experiencing PUK or necrotising scleritis; 13 eyes with non-necrotising scleritis. Perforation occurred in six eyes (11.5%). Moderate vision loss occurred in 10 eyes (19.2%) and severe loss in six eyes (11.5%). Five subjects (12.2%) were deceased by study conclusion, of whom four were not on immunomodulatory therapy (IMT). A marked difference in estimated survival was observed with IMT, with mean estimated survival of 10.7 years without treatment, compared to 24.7 years with IMT (P = .045). CONCLUSIONS AND RELEVANCE: PUK and necrotising scleritis represent a severe inflammatory disease with high rates of perforation, visual loss and mortality. IMT was associated with a lower rate of mortality and longer estimated survival in this group.

Visual acuity and pattern of visual field loss at presentation in pituitary adenoma.

Our purpose was to analyse the demographics, prevalence and pattern of visual field defects in patients with pituitary adenoma. We prospectively recruited 103 consecutive patients (206 eyes) presenting to a neurosurgical unit with pituitary adenoma. Ophthalmological examination and standard automated perimetry (Humphrey, 24-2 threshold) was performed. Severity of visual field defects was also assessed. The mean population age was 53.9 years (standard deviation=15). Visual loss was the most common reason for presentation (39%) followed by endocrine abnormality (21%) and headache (15%). Patients with endocrine abnormality on presentation were 10.9 years younger than those presenting with visual loss (p=0.001). Bitemporal defects were the most prevalent pattern (n=22, 41%) followed by homonymous defects (n=7, 13%). Of the patients with visual field loss, 33% had unilateral visual field defects. The mean visual acuity in those with bitemporal defects was 6/7.5 with half of these patients having 6/6 vision in both eyes. In conclusion, the majority of patients with pituitary adenoma have visual acuity better than 6/7.5 despite having visual field defects. While a bitemporal pattern of visual field loss is the most common, a significant proportion of patients had unilateral and altitudinal defects. Assessment of the visual field is essential to rule out chiasmal compression.

Autoimmune retinopathy associated with carcinoid tumour of the small bowel.

Cancer associated retinopathy (CAR) is an immune mediated paraneoplastic condition associated with vision loss. It has been associated with a variety of systemic malignancies. The primary clinical presentation is rapid, progressive vision loss. Rod and cone dysfunction can cause other associated symptoms, such as nyctalopia. Electrophysiological testing and detection of anti-retinal antibodies are used to confirm the diagnosis. To our knowledge we describe the first patient with CAR associated with a carcinoid tumour of the gastrointestinal system. Auto-antibodies against alpha enolase and carbonic anhydrase II were detected with western blotting. Electroretinogram findings were consistent with rod and cone dysfunction.

The price of healthy eating: cost and nutrient value of selected regular and healthier supermarket foods in New Zealand.

AIM: To estimate the difference in cost and nutrient value for selected regular and healthier supermarket food items in New Zealand. METHOD: Electronic sales data from supermarket shoppers (n=882) from February 2004 to January 2005 were used to determine the 1000 top-selling food items. These items were categorised according to food type. Five regular items and five healthier options were selected per category to create two shopping baskets for which cost and nutrient data were then compared. RESULTS: The total weekly household cost did not differ substantially overall: NZ90.21 dollars for the regular basket vs NZ96.63 dollars for the healthy basket. However, healthier options were more expensive for some specific food categories: meat and poultry (27%); butter and margarine (44%); and cheese (19%). Healthier options were less expensive for canned fish (10%). Per unit weight, the healthier basket contained considerably less energy (29%), total fat (35%), saturated fat (52%), sugar (56%), and sodium (20%) than the regular basket. CONCLUSIONS: Cost is frequently perceived to be a barrier to a healthy diet, yet our analyses demonstrate that substantial improvements in nutrition are possible without incurring an increase in price for many staple food categories. However, healthier options within some important food categories, notably meat and spreads, are more expensive. Consideration should be given to ways in which this cost differential can be addressed to favour key healthy diet choices.