Primary ductal adenocarcinoma of the lacrimal gland with sarcomatoid differentiation: case report and genomic analysis.

We describe a case of primary ductal adenocarcinoma of the lacrimal gland with novel histopathological characteristics corresponding to a biphasic growth course and provide a comprehensive genomic profile of this malignancy. A 39-year-old male with a history of slowly progressive unilateral proptosis and hypoglobus presented after 1 month of hyperacute exacerbation. Orbital imaging revealed a superior mass with osseous erosion. The patient underwent orbital exploration and excisional biopsy via lateral orbitotomy. Histopathology demonstrated high-grade adenocarcinoma with a well-differentiated glandular component alongside a poorly differentiated sarcomatoid region. The glandular section was immunopositive for Her-2, CK7, GATA3, and androgen receptor. Tumor recurrence necessitated en-bloc exenteration with dural resection alongside adjuvant radiotherapy and chemotherapy. This represents the first report of sarcomatoid differentiation in primary ductal adenocarcinoma of the lacrimal gland, which may incite hyperacute progression. Conversely, GATA3 immunopositivity may correlate with indolent growth. Genomic variants such as SEMA3C represent potential therapeutic targets for this condition.

Kimura's disease: A short study of cytomorphologic features with its differential diagnosis and review of literature.

Kimura's disease (KD) is a chronic inflammatory disorder of unknown etiology, endemic in Asia. The typical clinical manifestations include a triad of painless unilateral cervical lymphadenopathy or subcutaneous masses predominantly in the head-and-neck region, blood and tissue eosinophilia, and elevated serum immunoglobulin (Ig) E levels. Many conditions including benign and malignant may mimic KD clinically. This study reports cytologic features of seven cases of KD which were studied and correlated with histology, Ig profile, and peripheral blood examination. KD shows a good response to medical treatment; hence, fine-needle aspiration cytology in conjunction with other laboratory findings lowers the need for additional biopsy procedures for early diagnosis as well as diagnosis of recurrent lesions.

Medication adherence and quality of life among type-2 diabetes mellitus patients in India.

BACKGROUND: Diabetes mellitus (DM) is a progressively increasing metabolic disorder and a significant public health burden that demands immediate global attention. However, there is a paucity of data about adherence to antidiabetic drugs among patients with type-2 (T2)DM in Uttarakhand, India. Outpatient research reported that more than 50% of patients do not adhere to the correct administration and appropriate medicine dosage. It has been reported that patients with chronic diseases who adhere to treatment may experience improvement in quality of life (QoL) and vice versa. AIM: To assess the adherence to antidiabetic medication and QoL among patients with T2DM. METHODS: This cross-sectional descriptive study was conducted at a tertiary care hospital in Uttarakhand, India. The Medication Adherence Rating Scale and World Health Organization QoL-BREF scale were used to assess medication adherence and QoL. RESULTS: Two hundred seventy-seven patients suffering from T2DM participated in the study. Their mean age was 50.80 (± 10.6) years, 155 (56%) had a poor adherence level and 122 (44%) had a good adherence level to antidiabetic medications. After adjusting for sociodemographic factors, multiple linear regression analysis found patients who were adherent to antidiabetic medications had significantly higher mean overall perception of QoL and overall perception of health, with beta scores of 0.36 and 0.34, respectively (both P = 0.000) points compared with nonadherent patients. CONCLUSION: There was an association between medication adherence and QoL in patients with T2DM. Hence, there is a need to plan awareness and counseling programs followed by regular follow-up to motivate patient adherence to recommended treatment and lifestyle regimens.

Surgical management of the orbit in thyroid eye disease: lateral orbital decompression.

PURPOSE OF REVIEW: Lateral orbital wall decompression is one of many well established techniques available to surgeons in management of patients with clinically significant thyroid eye disease (TED). Several different surgical approaches have been described in the literature and are reviewed herein. RECENT FINDINGS: Lateral orbital wall decompression remains a popular technique for surgical management of TED, with a recent American Society of Ophthalmic Plastic and Reconstructive Surgery survey showing that 22.6% of respondents preferred a single-wall procedure, with 36.8% of that subset preferring lateral wall decompression alone. Surgical techniques for lateral orbital wall decompression differ based on several steps, such as the incisional approach, whether to take an ab-interno versus ab-externo approach, and whether to remove orbital fat to achieve further decompression. In addition, technological advances have produced an array of tools available to the orbital surgeon to achieve efficient and accurate bone removal. SUMMARY: Lateral orbital wall decompression for TED, despite being an older technique, remains a popular and well established procedure for orbital decompression. Though no randomized controlled clinical trial supports one decompression technique over another for TED, lateral orbital wall decompression offers many benefits such as its ease of access and visualization of the orbital space.

Isolated Epitrochlear Filarial Lymphadenopathy: Cytomorphological Diagnosis of an Unusual Presentation.

Filariasis is a major public health problem in tropical countries like India. Despite the large number of people at risk, detection of eggs with or without larva (microfilaria) on fine-needle aspiration cytology is very unusual, especially in an uncommon site or incidentally detected in clinically unsuspected cases of filariasis with the absence of microfilariae in the peripheral blood. A 19-year-old male presented with swelling over medial aspect of left arm (just above the elbow), with no other specific signs and symptoms. Fine needle aspiration cytology revealed an adult gravid female filarial worm in a background of reactive lymphoid cells and lymphohistiocytic clusters. We report a case with elaborate fine needle aspiration cytology findings of filarial worm infestation with unusual presentation of isolated epitrochlear lymph node involvement in a clinically unsuspected case and recommend clinicians and pathologists to consider a high index of suspicion for such infections at uncommon sites especially in endemic territories, as early diagnosis and treatment prevent the more severe manifestations of disease.

Radiographic predictors of visual outcome in orbital compartment syndrome.

PURPOSE: The goal of this study is to evaluate parameters on orbital CT as predictors of visual outcome in orbital compartment syndrome from retrobulbar hematoma or orbital cellulitis. The study will assess the assertion that certain patients are anatomically predisposed to vision loss in these conditions. METHODS: A retrospective chart review of consecutive patients with the diagnosis of either orbital cellulitis or orbital hematoma from the clinic of a single provider in an academic practice from 2006 to 2009 was performed. Exclusion criteria included preexisting vision loss, lack of CT scan for analysis, or lack of 1-month follow up for final visual acuity. Measurements of final visual acuity, medial wall length, lateral wall length, distance from the globe to the apex, and a novel measurement of posterior globe tenting (stretch angle) were obtained. Patients were divided into 2 groups: normal visual acuity and vision loss. Statistical analysis was performed to identify significant differences between the 2 groups. RESULTS: The normal vision group consisted of 11 patients, all with vision of 20/30 or better. The average length of the medial and lateral wall was 43.9 and 41.6 mm, respectively. The average distance from the globe to the apex was 26.3 mm in the uninvolved eye and 30.3 mm in the involved eye, resulting in an average difference of 4.18 mm. The average stretch angle measurement was 28.9° in the uninvolved eye and 28.5° in the involved eye, resulting in an average difference of 0.41°. The vision loss group consisted of 4 patients, all with vision of count fingers or worse. The average length of the medial and lateral wall was 46.9 and 45.7 mm, respectively. The average distance from the globe to the apex was 32.2 mm in the uninvolved eye and 36.7 mm in the involved eye, resulting in an average difference of 4.50 mm. The average stretch angle measurement was 32.3° in the uninvolved eye and 21.1° in the involved eye, resulting in an average difference of 11.2°. The difference in stretch angle between eyes in the vision loss and normal vision groups were found to be highly significant with a p value of less than 0.001. The difference between the 2 orbits for globe to apex is a rough measurement of proptosis and was not statistically different in the 2 groups (p = 0.71), whereas the length from the globe to the apex in the uninvolved eye was statistically different between the vision loss group and normal vision group (p = < 0.001). CONCLUSIONS: Orbital compartment syndrome is a potentially vision-threatening condition. Minimal objective data are currently available in the literature to guide physicians in making clinical judgments regarding these patients. The results of this study indicate that comparing the novel stretch angle between the patient's 2 eyes and measuring the length from the globe to the orbital apex can help identify patients at risk for poor visual outcome. This study provides objective measurements that can aid ophthalmologists and radiologists in determining the relative threat to vision in patients presenting with orbital compartment syndrome from orbital cellulitis or retrobulbar hematoma. Hopefully, the data can help select patients that may benefit from more aggressive intervention and will ultimately result in superior visual outcomes.

Elovl4 5-bp deletion knock-in mouse model for Stargardt-like macular degeneration demonstrates accumulation of ELOVL4 and lipofuscin.

The mechanism underlying photoreceptor degeneration in autosomal dominant Stargardt-like macular degeneration (STGD3) due to mutations in the elongation of very long chain fatty acids-4 (ELOVL4) gene is not fully understood. To evaluate the pathological events associated with STGD3, we used a mouse model that mimics the human STGD3 phenotype and studied the progression of retinal degeneration. Morphological changes in the retina of Elovl4 5-bp deletion knock-in mice (E_mut(+/-)) were evaluated at 22 months of age. The localization of ELOVL4, and the expression pattern of inner retinal tissue marker proteins, and ubiquitin were determined by immunofluorescence labeling of retinal sections. Levels of the retinal pigment epithelium (RPE) lipofuscin fluorophores were measured by quantitative HPLC. Morphological evaluation of the retina revealed an accumulation of RPE debris in the subretinal space. A significant increase in the amount of ELOVL4 was observed in the outer plexiform layer in E_mut(+/-) mice compared to controls. Apart from the accumulation of ELOVL4, E_mut(+/-) mice also exhibited high expression of ubiquitin in the retina. Analysis of lipofuscin fluorophores in the RPE showed a significant elevation of A2E and compounds of the all-trans-retinal dimer series in retinas from four and ten month old E_mut(+/-) mice compared to wild-type littermates. These observations suggest that abnormal accumulation of ELOVL4 protein and lipofuscin may lead to photoreceptor degeneration in E_mut(+/-) mice.