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1.
Can J Surg ; 64(1): E48-E50, 2021 02 03.
Article in English | MEDLINE | ID: mdl-33533578

ABSTRACT

At the start of the coronavirus disease 2019 (COVID-19) pandemic, hospitals and ambulatory surgical centres significantly decreased elective surgical procedures to facilitate capacity for in-hospital beds, preserve personal protective equipment (PPE), preserve anesthetic medications and limit spread of infection. Non-COVID-19­related ophthalmic disease continues to affect vision, and it is anticipated that the backlog of elective surgeries will require months to years to resolve. The delivery of ophthalmic surgical care in a prioritized, systematic and transparent way is vital to manage the surgical backlog while minimizing vision loss and consequent disability in the Canadian population. The Canadian Ophthalmology Society (COS) has modified the published Medically Necessary, Time Sensitive (MeNTS) Procedures scoring system to be applicable to all subspecialties within ophthalmology. This case prioritization process integrates medical necessity, consideration of resource preservation with risk of COVID-19 exposure, and factors unique to eye care. It provides guidance to Canadian ophthalmologists to facilitate decision making in triaging elective procedures.


Subject(s)
COVID-19/prevention & control , Health Priorities , Infection Control , Ophthalmologic Surgical Procedures , Patient Selection , Triage/organization & administration , COVID-19/epidemiology , COVID-19/transmission , Canada , Humans , Societies, Medical
3.
Orbit ; 40(4): 281-286, 2021 Aug.
Article in English | MEDLINE | ID: mdl-32552229

ABSTRACT

Purpose: To review the current literature on Coronavirus Disease 2019 (COVID-19) virology and transmission; to present a decision tree for risk stratifying oculofacial plastic and orbital surgeries; and to generate personal protective equipment (PPE) recommendations by risk category.Methods: A comprehensive literature review on COVID-19 was conducted. A two-stage modified Delphi technique involving 18 oculofacial plastic and orbital surgeons across Canada was used to determine consensus risk-stratification criteria and PPE recommendations for surgeries performed in the North American context.Results: COVID-19 is an infectious disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). We summarize COVID-19 virology and transmission, as well as practice considerations for oculofacial plastic and orbital surgeons. Although SARS-CoV-2 is known to be transmitted predominantly by droplet mechanisms, some studies suggest that transmission is possible through aerosols. Among common procedures performed by oculofacial and plastic surgeons, some are likely to be considered aerosol-generating. Risk of transmission increases when manipulating structures known to harbor high viral loads. We present an algorithm for risk-stratification based on the nature of surgery and the anatomical sites involved and offer recommendations for PPE.Conclusions: Although universal droplet precautions are now recommended in most healthcare settings, some clinical situations require more stringent infection control measures. By highlighting high-risk scenarios specific to oculofacial plastic and orbital surgery, as well as PPE recommendations, we hope to enhance the safety of continued care during the COVID-19 pandemic.


Subject(s)
COVID-19/transmission , Infectious Disease Transmission, Patient-to-Professional/prevention & control , Ophthalmologic Surgical Procedures , Orbital Diseases/surgery , Personal Protective Equipment/standards , SARS-CoV-2/pathogenicity , Surgery, Plastic , Decision Trees , Delphi Technique , Humans , Infection Control/standards , Pneumonia, Viral/prevention & control , Risk Assessment
4.
Can J Ophthalmol ; 55(3): 245-252, 2020 06.
Article in English | MEDLINE | ID: mdl-31901307

ABSTRACT

OBJECTIVE: The management of advanced basal cell carcinoma (BCC) in the periocular region remains a clinical challenge. Vismodegib (ErivedgeTM) has been approved in 2013 by Health Canada for adult patients with "histologically confirmed metastatic BCC or locally advanced BCC inappropriate for surgery or radiation." An expert consensus was sought to create a standardised approach in the use of this novel treatment. METHODS: Fourteen practicing oculoplastic surgeons across Canada were involved in formulating and reviewing guidelines until consensus was reached. A consultancy meeting was followed by further ratification of guidelines over email. Two voting surveys were performed of the group to objectively assess agreement over each statement within the guidelines. Ratification continued until at least two-thirds of the group agreed on every guideline statement. RESULTS: The guidelines summarize 21 statements in a major and minor criteria format. A multidisciplinary team review is suggested for each patient with the involvement of recommended specialists. The internal survey revealed 100% agreement over 9 statements, 91.7% agreement over 8 statements, 83.3% agreement over 4 statements, and 2 statements had 66.7% and 58.7% agreement each. All statements with less than 91.7% agreement were surveyed again, and they were kept, modified, or removed on the basis of a consensus of over 66.7%. CONCLUSIONS: These guidelines serve to act as a framework for physicians considering vismodegib for the medical management of patients with advanced or metastatic periocular BCC. Future applications, including neoadjuvant uses of the drug, may become apparent through further research.


Subject(s)
Antineoplastic Agents , Carcinoma, Basal Cell , Skin Neoplasms , Adult , Anilides , Antineoplastic Agents/therapeutic use , Canada , Humans , Pyridines , Treatment Outcome
5.
Plast Reconstr Surg ; 144(1): 43e-47e, 2019 07.
Article in English | MEDLINE | ID: mdl-31246809

ABSTRACT

BACKGROUND: Blindness from ophthalmic or central retinal artery embolism is one of the most devastating complications of cosmetic filler facial injections. A proposed therapy to mitigate visual loss is prompt retrobulbar injection of hyaluronidase into the retrobulbar space. Despite Zhu et al. showing a lack of evidence and very limited published literature for reversing visual loss with this intervention, it is still widely accepted as a treatment for filler-related emboli. The purpose of this study was to evaluate the penetration of hyaluronidase through optic nerve dura using an in vitro model. METHODS: At study conclusion, five 1-cm-long segments of fresh optic nerve were obtained and injected with highly crosslinked hyaluronic acid filler, then ligated on both ends in a watertight fashion. The sections were immersed in three concentrations of hyaluronidase solution for 24 hours. Histopathologic examination of the specimen was performed to assess the presence of filler. RESULTS: The optic nerve sections were 1.1 cm (range, 0.8 to 1.2 cm). Three were immersed in 20 ml of 1500 IU/ml hyaluronidase solution and two were immersed in saline as control. After 24 hours, there was a persistence of hyaluronic acid within the optic nerves. CONCLUSIONS: There is a lack of evidence for penetration of optic nerve sheath by hyaluronidase. This raises question about the effectiveness of retrobulbar injection of hyaluronidase in reversing filler-related blindness. Further studies are needed before this can be adopted as the treatment of choice. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.


Subject(s)
Dermal Fillers/pharmacokinetics , Hyaluronoglucosaminidase/pharmacokinetics , Optic Nerve/chemistry , Aged , Female , Humans , Male , Middle Aged
6.
Ocul Oncol Pathol ; 5(3): 186-189, 2019 Apr.
Article in English | MEDLINE | ID: mdl-31049326

ABSTRACT

BACKGROUND/AIMS: The treatment of orbital melanoma poses a management challenge. This case explores the delivery of high-dose melphalan to an orbital recurrence of uveal melanoma via intra-arterial delivery of melphalan to the orbit. A 62-year-old man developed recurrent orbital disease 7 months after enucleation for a large uveal melanoma. He received 6 monthly intra-arterial infusions of melphalan to the orbit, ranging in dose from 20 to 30 mg per infusion. Following the last infusion, mild temporary erythema was noted on the forehead along the distribution of the supratrochlear artery. The orbital recurrence was reduced in size by 66% in the longest dimension as measured by magnetic resonance imaging (MRI). However, 9 months following intra-arterial melphalan, tumor regrowth was detected on MRI, and additional treatment options were pursued. CONCLUSION: This case demonstrates that intra-arterial melphalan can result in nonsustained tumor regression of recurrent orbital uveal melanoma. It suggests that local delivery of high-dose melphalan may be helpful as a neoadjuvant treatment for uveal melanoma, and future studies will be useful to confirm the value of this approach in additional cases of recurrent and possibly in primary uveal melanoma.

7.
Am J Ophthalmol Case Rep ; 13: 119-121, 2019 Mar.
Article in English | MEDLINE | ID: mdl-30619977

ABSTRACT

PURPOSE: To review the clinical and histopathological features of nodular fasciitis, a rare benign periorbital tumor that mimics orbital malignancy, by presenting a case involving an infant with marked orbital wall erosion requiring repair. OBSERVATIONS: A 9-month-old boy developed a rapidly growing periorbital mass concerning for a soft tissue malignancy. Computerized tomography (CT) scans showed bony erosion of the lateral orbital wall. Incisional biopsy revealed nodular fasciitis. USP6 gene rearrangement was negative. The tumor was completely excised and the underlying orbital wall defect was repaired with polydioxanone (PDS) plate. CONCLUSIONS AND IMPORTANCE: Nodular fasciitis is a benign periorbital tumor that presents like malignancies and warrants prompt investigations, especially in children. Orbital wall erosion is rare and can be repaired to yield good functional and cosmetic outcome.

8.
Ophthalmic Plast Reconstr Surg ; 35(2): 148-154, 2019.
Article in English | MEDLINE | ID: mdl-30371551

ABSTRACT

PURPOSE: A risk assessment score for metastasis based on age, tumor size, and mitotic figures has been suggested for nonorbital solitary fibrous tumor (SFT)/hemangiopericytoma. The authors herein examine the clinicopathological features of recurrent and metastatic orbital SFT and evaluate the existing risk assessment score for orbital SFT. METHODS: The American Society of Ophthalmic Plastic and Reconstructive Surgery Oncology Database was queried for patients with recurrent or malignant orbital hemangiopericytoma/SFT. The medical records were reviewed for clinical and pathologic findings, treatments, and outcomes. RESULTS: Eight patients from 3 institutions were identified with recurrent orbital hemangiopericytoma/SFT. Median age at diagnosis was 59 years, and 4 patients were women. The mean size of tumor was 2.1 ± 1.1 cm. All patients were initially treated with surgery and experienced local recurrence after a median of 4 (range 0.5-10) years. Five patients were treated with orbital radiation. Two patients also developed distant metastases and eventually died of their disease. Median Ki-67 was 5% (range 1-65%) and 5 mitotic figures/10 high-power fields (range 2-30). The previously described risk stratification model for nonorbital SFT did not correlate with the propensity to develop metastases in this cohort; however, both patients with distant metastasis had > 4 mitotic figures /10 high-power fields. CONCLUSIONS: In this cohort of recurrent orbital hemangiopericytoma/SFT, median time to recurrence was 4 years underscoring the importance of careful continued follow-up. The current risk stratification models have limited use for orbital lesions, mostly due to the fact that orbital SFTs are smaller than even the smallest size criteria in this risk assessment model.


Subject(s)
Hemangiopericytoma/pathology , Neoplasm Recurrence, Local/pathology , Orbital Neoplasms/pathology , Solitary Fibrous Tumors/pathology , Adult , Aged , Diagnosis, Differential , Female , Follow-Up Studies , Hemangiopericytoma/surgery , Humans , Incidence , Magnetic Resonance Imaging , Male , Middle Aged , Neoplasm Recurrence, Local/epidemiology , Ophthalmologic Surgical Procedures , Orbital Neoplasms/surgery , Retrospective Studies , Solitary Fibrous Tumors/surgery , Time Factors , United States/epidemiology
9.
J Natl Compr Canc Netw ; 16(10): 1209-1215, 2018 10.
Article in English | MEDLINE | ID: mdl-30323091

ABSTRACT

Background: Tumor board conferences (TBCs) are used by oncologic specialists to review patient cases, exchange knowledge, and discuss options for cancer management. These multidisciplinary meetings are often a cornerstone of treatment at leading cancer centers and are required for accreditation by certain groups, such as the American College of Surgeons' Commission on Cancer. Little is known regarding skin cancer TBCs. The objective of this study was to characterize the structure, function, and impact of existing skin cancer TBCs in the United States. Methods: A cross-sectional online survey was administered to physician leaders of skin cancer TBCs at NCI-designated Comprehensive and Clinical Cancer Centers. Results: Of the 59 centers successfully contacted, 14 (24%) reported not having a conference where skin cancer cases were discussed, and 45 (76%) identified 53 physician leaders. A total of 38 physicians (72%) completed the survey. Half of the meeting leaders were medical and/or surgical oncologists, and dermatologists led one-third of meetings. TBCs had a moderate to significant impact on patient care according to 97% of respondents. All respondents indicated that the meetings enhanced communication among physicians and provided an opportunity for involved specialists and professionals to discuss cases. The most frequently cited barrier to organizing TBCs was determining a common available date and time for attendees (62%). The most common suggestion for improvement was to increase attendance, specialists, and/or motivation. Conclusions: Results showed overall consistency in meeting structure but variability in function, which may be a reflection of institutional resources and investment in the conference. Future directions include defining metrics to evaluate changes in diagnosis or management plan after tumor board discussion, attendance, clinical trial enrollment, and cost analysis. Results of this survey may aid other institutions striving to develop and refine skin cancer TBCs.


Subject(s)
Cancer Care Facilities/organization & administration , Medical Oncology/organization & administration , Patient Care Team/organization & administration , Skin Neoplasms/therapy , Specialty Boards/statistics & numerical data , Cancer Care Facilities/statistics & numerical data , Congresses as Topic , Humans , Medical Oncology/statistics & numerical data , Patient Care Team/statistics & numerical data , Skin Neoplasms/diagnosis , Societies, Medical , Specialty Boards/organization & administration , Surveys and Questionnaires/statistics & numerical data , United States
10.
Am J Med Sci ; 355(1): 61-66, 2018 01.
Article in English | MEDLINE | ID: mdl-29289265

ABSTRACT

BACKGROUND: IgG4-related disease (IgG4-RD) is a recently recognized condition defined by characteristic histopathologic findings in affected organs. Serum IgG4 concentration is often but not always elevated. The sensitivity and specificity of serum IgG4 vary greatly across studies and has been anecdotally associated to ethnicity. Our study was conducted to investigate the difference in serum IgG4 levels between Asian and non-Asian patients with IgG4-RD. METHODS: This is a single-center retrospective study of 26 Asian and 10 non-Asian patients with histologically confirmed IgG4-RD. Serum IgG4 levels, clinical features and other laboratory findings were compared between the 2 groups, 31 Asian and 11 non-Asian patients with non-IgG4-RD rheumatic diseases were randomly identified to evaluate test characteristics of serum IgG4 measurement. RESULTS: Median serum IgG4 at time of diagnosis was significantly higher in Asian (median = 11.2g/L, interquartile range: 4.6-19.7) than non-Asian patients (median = 2.9g/L, interquartile range: 0.7-5.4, P = 0.0094), as well as the median serum IgG and total protein. Asian patients had more eosinophilia and polyclonal hypergammaglobulinemia than non-Asian patients (P = 0.016 and 0.001, respectively). Test sensitivity was higher in Asian (96%) than non-Asian patients (67%), whereas test specificity was higher in non-Asian patients (91% versus 71%). CONCLUSION: Asian patients with IgG4-RD have more exuberant serum IgG4, IgG and polyclonal hypergammaglobulinemia than non-Asian patients; the mechanism of this difference requires further study. These findings have significant clinical importance and must be accounted for in the diagnostic workup of patients in multiethnic settings.


Subject(s)
Arabs , Asian People/ethnology , Autoimmune Diseases/blood , Autoimmune Diseases/ethnology , Hispanic or Latino , Immunoglobulin G/blood , White People/ethnology , Aged , Biomarkers/blood , Cohort Studies , Ethnicity , Female , Humans , Male , Middle Aged , Reproducibility of Results , Retrospective Studies
11.
Br J Ophthalmol ; 102(5): 654-658, 2018 05.
Article in English | MEDLINE | ID: mdl-28844052

ABSTRACT

BACKGROUND: Orbital exenteration is a disfiguring procedure reserved for life-threatening malignancies. This study examines the clinical course and outcomes of a large series of patients who underwent orbital exenteration for malignant periocular neoplasms. METHODS: This is a retrospective review of patients who underwent orbital exenteration from 1 July 2005 to 30 June 2015 at four tertiary referral centres in the USA, Australia and Canada. Demographics, indication for surgery, pathology, surgical technique, reconstruction type and outcomes were reviewed. RESULTS: Orbital exenteration was performed on 102 patients. The mean age at surgery was 67.5 years. The most common malignant tumours encountered were squamous cell carcinoma, melanoma and basal cell carcinoma. Seventy-six patients (75%) underwent reconstruction with a local myocutaneous flap, twelve with partial-thickness skin grafts (PTSG), or split skin graft, two had a free flap, and one had a dermis fat graft. Sixteen patients had combined procedures of two of the above. Complete removal of the tumour was achieved with clear margins in 81 cases. Of all patients, 72% were alive at 48 months or more. CONCLUSION: The majority of orbital exenterations performed in this series were secondary to periocular malignancies with unsuccessful/insufficient previous treatments. Regional myocutaneous flaps, PTSG, full-thickness skin grafts and dermis fat grafts were all highly effective and durable reconstructive options, and were able to withstand radiation therapy without complications.


Subject(s)
Orbit Evisceration/methods , Orbital Neoplasms/surgery , Plastic Surgery Procedures/methods , Skin Transplantation/methods , Carcinoma, Basal Cell/surgery , Carcinoma, Squamous Cell/surgery , Humans , Melanoma/surgery , Retrospective Studies , Surgical Flaps
12.
Br J Ophthalmol ; 102(3): 291-294, 2018 03.
Article in English | MEDLINE | ID: mdl-29055904

ABSTRACT

BACKGROUND: The 1981 Nepal Blindness Survey first identified the Narayani Zone as one of the regions with the highest prevalence of blindness in the country. Subseuqently, a 2006 survey of the Rautahat District of the Narayani Zone found it to have the country's highest blindness prevalence. This study examines the impact on blind avoidable and treatable eye conditions in this region after significant increase in eye care services in the past decade. METHODS: The rapid assessment of avoidable blindness (RAAB) methodology was used with mobile data collection using the mRAAB smartphone app. Data analysis was done using the standard RAAB software. Based on the 2011 census, 100 clusters of 50 participants aged 50 years or older were randomly sampled proportional to population size. RESULTS: Of the 5000 participants surveyed, 4771 (95.4%) were examined. The age-adjusted and sex-adjusted prevalence of bilateral blindness, severe visual impairment (SVI) and moderate visual impairment (MVI) were 1.2% (95% CI 0.9% to 1.5%), 2.5% (95% CI 2.0% to -3.0%) and 13.2% (95% CI 11.8% to 14.5%), respectively. Cataract remains the primary cause of blindness and SVI despite cataract surgery coverage (CSC) of 91.5% for VA<3/60. Women still account for two-thirds of blindness. CONCLUSION: The prevalence of blindness in people over the age of 50 years has decreased from 6.9% in 2006 to 1.2%, a level in keeping with the national average; however, significant gender inequity persists. CSC has improved but continues to favour men.


Subject(s)
Blindness/epidemiology , Cataract Extraction/statistics & numerical data , Cataract/epidemiology , Vision, Low/epidemiology , Age Distribution , Aged , Blindness/prevention & control , Cross-Sectional Studies , Female , Health Services Accessibility , Health Surveys , Humans , Male , Middle Aged , Nepal/epidemiology , Prevalence , Sex Distribution , Visual Acuity
13.
Ophthalmic Plast Reconstr Surg ; 33(2): 101-105, 2017.
Article in English | MEDLINE | ID: mdl-26974419

ABSTRACT

PURPOSE: To characterize the clinical presentation, management, and outcomes of patients with sweat gland carcinoma of the periocular region. To review the pathologic classification scheme, compare the clinical behavior between subtypes, and discuss treatment recommendations for periocular sweat gland carcinomas. METHODS: Retrospective study from 2 tertiary centers, which are part of the American Society of Ophthalmic Plastic and Reconstructive Surgery Oncology Database. RESULTS: Twenty-two patients, of whom 13 (59%) were female, were included in the study. The median age was 69 years. The median follow-up time was 23 months. Twenty (91%) patients had lesions that were slow growing and were present for several months to years before diagnosis. Presentation was varied, and pathology included mucinous carcinoma (7; 32%), microcystic adnexal carcinoma (4; 18%), endocrine mucin-producing sweat gland carcinoma (4; 18%), eccrine carcinoma (2; 9%), apocrine carcinoma (2; 9%), poorly differential adnexal carcinoma (2; 9%), and hidroadenocarcinoma (1; 5%). Microcystic adnexal carcinoma tended to present with the largest tumor dimension (range: 25-32 mm) and mucinous carcinoma the smallest (range: 5-13 mm). Treatments included Mohs micrographic excision in 10 patients (45.5%), wide local excision in 10 patients (45.5%), and orbital exenteration in 2 patients (9%). No lesions ≤T2b (by AJCC 7th edition classification for eyelid carcinomas) had local recurrence or nodal metastasis. No patients had distant metastasis. CONCLUSIONS: Sweat gland carcinomas tend to grow slowly and be present for several months to years before diagnosis. Overall, microcystic adnexal carcinoma subtype showed more aggressive clinical behavior than mucinous subtypes. Eyelid carcinomas AJCC category T2b or less were associated with better outcomes.


Subject(s)
Carcinoma/epidemiology , Eyelid Neoplasms/epidemiology , Sweat Gland Neoplasms/epidemiology , Aged , Aged, 80 and over , Carcinoma/pathology , Carcinoma/surgery , Eyelid Neoplasms/pathology , Eyelid Neoplasms/surgery , Female , Humans , Male , Middle Aged , Mohs Surgery , Neoplasm Metastasis/pathology , Neoplasm Recurrence, Local , Ophthalmologic Surgical Procedures/methods , Orbital Neoplasms/pathology , Retrospective Studies , Sweat Gland Neoplasms/pathology , Sweat Gland Neoplasms/surgery , United States/epidemiology
14.
Curr Pharm Des ; 23(4): 655-659, 2017.
Article in English | MEDLINE | ID: mdl-27928965

ABSTRACT

Basal cell caricnoma (BCC), the most common periocular magliancy, is treated with complete surgical excision. However, in patients not amenable to surgery or when surgical resection means loss of vital organs or disfiguring procedures due to locally advanced or metastatic disease, targeting the hedgehog pathway offers a novel treatment approach for such patients. Mutation in PTCH1 and SMO has been identified in patients with basal cell nevoid syndrome as well as in patients with sporadic BCC. Inhibition of SMO by vismodegib or sonidegib, the two sonic hedgehog inhibitor drugs approved by the Food and Drug Administration in the United States, has shown overall response rate for locally advanced and metastatic BCC around 50%. The most common side effects were muscle cramps, weight loss, fatigue, and lost appetite. Resistance to vismodegib has been attributed to mutation in SMO or activation of RAS/MAPK pathway. New research into dual inhibition aims to overcome this resistance and provide more lasting response.


Subject(s)
Antineoplastic Agents/pharmacology , Carcinoma, Basal Cell/drug therapy , Eye Neoplasms/drug therapy , Hedgehog Proteins/antagonists & inhibitors , Animals , Antineoplastic Agents/chemistry , Carcinoma, Basal Cell/metabolism , Eye Neoplasms/metabolism , Hedgehog Proteins/genetics , Hedgehog Proteins/metabolism , Humans
16.
Head Neck ; 38(8): 1258-62, 2016 08.
Article in English | MEDLINE | ID: mdl-27027275

ABSTRACT

BACKGROUND: We analyzed local control and early ocular toxicity after eye-sparing management of lacrimal gland carcinoma. METHODS: For consecutive patients with lacrimal gland carcinoma treated during 2007 to 2014, we reviewed tumor characteristics, treatment details, ocular toxic effects, and recurrence. RESULTS: Twenty patients, median age 55 years, were treated for lacrimal gland carcinoma during the study period; 11 had globe-sparing surgery. Seven patients had adenoid cystic carcinoma, 2 had carcinoma ex pleomorphic adenoma, and 1 each had high-grade and low-grade adenocarcinoma. Ten patients underwent postoperative radiotherapy, median 60 Gy (range, 52-64 Gy), 6 with concurrent chemotherapy. At a median of 30 months after radiation, all patients had dry eye syndrome, and 1 patient had severe corneal and conjunctival damage leading to enucleation. All 11 patients were disease free at last contact, median follow-up after surgery of 33 months. CONCLUSION: An eye-sparing approach with surgery followed by adjuvant radiotherapy or chemoradiotherapy is feasible for selected patients with lacrimal gland carcinoma and is associated with a reasonable locoregional control and ocular toxicity profile. © 2016 Wiley Periodicals, Inc. Head Neck 38:1258-1262, 2016.


Subject(s)
Adenocarcinoma/surgery , Eye Neoplasms/surgery , Lacrimal Apparatus/surgery , Ophthalmologic Surgical Procedures/methods , Organ Sparing Treatments/methods , Academic Medical Centers , Adenocarcinoma/mortality , Adenocarcinoma/pathology , Adenocarcinoma/therapy , Adenoma, Pleomorphic/mortality , Adenoma, Pleomorphic/pathology , Adenoma, Pleomorphic/surgery , Adult , Aged , Cancer Care Facilities , Chemoradiotherapy/methods , Cohort Studies , Combined Modality Therapy , Eye Neoplasms/mortality , Eye Neoplasms/pathology , Eye Neoplasms/therapy , Female , Humans , Lacrimal Apparatus/pathology , Male , Middle Aged , Prognosis , Retrospective Studies , Risk Assessment , Survival Rate , Texas , Treatment Outcome
17.
JAMA Ophthalmol ; 133(10): 1109-16, 2015 Oct.
Article in English | MEDLINE | ID: mdl-26203913

ABSTRACT

IMPORTANCE: The molecular-genetic alterations contributing to the pathogenesis of sebaceous carcinoma and sebaceous adenoma remain poorly understood. Given that sebaceous carcinoma is associated with substantial morbidity and mortality, there is a critical need to delineate the pathways driving sebaceous carcinoma and candidate molecules for targeted therapy. OBJECTIVE: To describe differentially expressed microRNAs (miRNAs) in a series of periocular sebaceous carcinomas compared with sebaceous adenomas in order to identify pathways driving the pathogenesis of sebaceous carcinoma. DESIGN, SETTING, AND PARTICIPANTS: Thirty sebaceous carcinomas and 23 sebaceous adenomas (including 11 that were confirmed to be related to Muir-Torre syndrome and 6 that were confirmed to be sporadic) were obtained from archives (from 48 patients) of 2 institutions (University of Texas MD Anderson Cancer Center, Houston, and Siriraj Hospital, Mahidol University, Bangkok, Thailand) and profiled. MAIN OUTCOMES AND MEASURES: Expression of miRNAs was determined using total RNA from formalin-fixed, paraffin-embedded tissue and real-time reverse transcription-polymerase chain reaction performed in a microfluidics card containing 378 unique miRNAs. Fold change was determined using the ΔΔCt method (reference probe, RNU48). Median centering was used to normalize the data. Two-sample t tests were used to identify differentially expressed miRNAs. The false discovery rate was assessed by ß-uniform mixture analysis of P values from the t statistics. Significance was defined by this estimated false discovery rate. RESULTS: Serial testing and validation confirmed overexpression of 2 miRNAs previously reported to be oncogenic, miR-486-5p (4.4-fold; P = 2.4 × 10-8) and miR-184 (3.5-fold; P = 1.7 × 10-6), in sebaceous carcinoma compared with sebaceous adenoma and downregulation of 2 miRNAs previously reported to have tumor-suppressive properties, miR-211 (-5.8-fold; P = 2.3 × 10-9) and miR-518d (-4.5-fold; 6.7 × 10-5), in sebaceous carcinoma compared with sebaceous adenoma. CONCLUSIONS AND RELEVANCE: Sebaceous carcinoma exhibits an miRNA expression profile distinct from that of sebaceous adenoma, implicating dysregulation of NF-κB and PTEN (targets of miR-486-5p) and TGF-ß signaling (target of miR-211) in the pathogenesis of sebaceous carcinoma. The identification of miRNAs whose expression is altered in sebaceous carcinoma compared with sebaceous adenoma provides a novel entry point for a more comprehensive understanding of the molecular-genetic alterations pivotal to the development of sebaceous carcinoma.


Subject(s)
Adenocarcinoma, Sebaceous/genetics , Eyelid Neoplasms/genetics , Gene Expression Regulation, Neoplastic/physiology , MicroRNAs/genetics , Muir-Torre Syndrome/genetics , Adenocarcinoma, Sebaceous/secondary , Adult , Aged , Aged, 80 and over , Eyelid Neoplasms/pathology , Female , Gene Expression Profiling , Humans , In Situ Hybridization, Fluorescence , Lymphatic Metastasis , Male , Middle Aged , Muir-Torre Syndrome/pathology , Real-Time Polymerase Chain Reaction
18.
Am J Ophthalmol ; 160(2): 220-227.e2, 2015 Aug.
Article in English | MEDLINE | ID: mdl-25935097

ABSTRACT

PURPOSE: To review our experience treating patients with the Hedgehog pathway inhibitor, vismodegib, in patients with orbital or periocular locally advanced or metastatic basal cell carcinoma (BCC) or basal cell nevus syndrome. DESIGN: Retrospective interventional case series. METHODS: We reviewed all patients with locally advanced or metastatic orbital or periocular BCC or basal cell nevus syndrome treated with the Hedgehog pathway inhibitor, vismodegib, at a comprehensive cancer center from 2009 through 2015. Reviewed data included age; sex; American Joint Commission on Cancer tumor, node, metastasis staging system designation; type and grade of drug-related side effects; response to treatment; duration of follow-up, and status at last follow-up. RESULTS: The study included 10 white men and 2 white women; the median age was 64.5 years. Ten patients had locally advanced BCC; 2 had basal cell nevus syndrome. Among the patients with locally advanced BCC, 5 had T3bN0M0 disease at presentation; 1 each had T3aN0M0, T3bN1M0, T2N1M1, T4N1M1, and T4N2cM1 disease. Overall, 3 patients had a complete response, 6 had a partial response, and 3 had stable disease at last follow-up. Two patients developed progressive disease after a complete response for 38 months and stable disease for 16 months, respectively. All patients developed grade I drug-related adverse effects, most commonly muscle spasms (12 patients), weight loss (10), dysgeusia (9), alopecia (9), decreased appetite (5), and fatigue (4). Five patients developed grade II adverse effects. At last follow-up, none of the 5 patients presenting with T3bN0M0, nor the patient with T3bN1M0 disease, had required orbital exenteration. CONCLUSION: Hedgehog pathway inhibition produces a significant clinical response in most patients with locally advanced or metastatic orbital or periocular BCC or basal cell nevus syndrome and can obviate orbital exenteration in some patients. Drug-related adverse effects are manageable in most patients.


Subject(s)
Anilides/antagonists & inhibitors , Antineoplastic Agents/therapeutic use , Basal Cell Nevus Syndrome/drug therapy , Carcinoma, Basal Cell/drug therapy , Pyridines/antagonists & inhibitors , Skin Neoplasms/drug therapy , Adult , Aged , Aged, 80 and over , Anilides/metabolism , Basal Cell Nevus Syndrome/metabolism , Basal Cell Nevus Syndrome/pathology , Carcinoma, Basal Cell/metabolism , Carcinoma, Basal Cell/pathology , Female , Humans , Male , Middle Aged , Pyridines/metabolism , Retrospective Studies , Skin Neoplasms/metabolism , Skin Neoplasms/pathology
19.
Clin Dermatol ; 33(2): 159-69, 2015.
Article in English | MEDLINE | ID: mdl-25704936

ABSTRACT

Eyelid cancers account for 5% to 10% of all cutaneous malignancies. The incidence of eyelid cancer is approximately 15 cases per 100,000 individuals per year. Basal cell carcinoma is by far the most common cutaneous malignancy in the periocular area; other cutaneous malignancies that occur in this area include, in decreasing order of frequency, squamous cell carcinoma, sebaceous carcinoma, melanoma, and Merkel cell carcinoma. The most common treatment for eyelid carcinomas is surgical resection with frozen section examination for margin control, but exenteration may be needed when there is orbital invasion. Adjuvant radiotherapy may be needed in patients at high risk for local recurrence; sentinel lymph node biopsy may be considered in patients at high risk for lymph node metastasis. Primary or residual in situ disease of the conjunctiva can be treated with topical chemotherapy, such as mitomycin C, 5-fluorouracil, or interferon alpha-2 b. For patients with metastatic or locally advanced basal cell or squamous cell carcinoma not amenable to surgical excision or radiotherapy, targeted therapy against the hedgehog pathway (for basal cell carcinoma) or epidermal growth factor receptor (for squamous cell carcinoma) has been shown to be effective in preventing disease progression. Patients with eyelid and ocular surface malignancies need to be monitored with careful clinical examination for at least 5years after surgical treatment, and additional investigations may be warranted in some cases.


Subject(s)
Carcinoma, Basal Cell/therapy , Carcinoma, Squamous Cell/therapy , Conjunctival Neoplasms/therapy , Eyelid Neoplasms/therapy , Neoplasm Recurrence, Local/pathology , Carcinoma, Basal Cell/epidemiology , Carcinoma, Basal Cell/pathology , Carcinoma, Squamous Cell/epidemiology , Carcinoma, Squamous Cell/pathology , Combined Modality Therapy , Conjunctival Neoplasms/epidemiology , Conjunctival Neoplasms/pathology , Eyelid Neoplasms/epidemiology , Eyelid Neoplasms/pathology , Female , Follow-Up Studies , Humans , Male , Monitoring, Physiologic/methods , Neoplasm Invasiveness/pathology , Neoplasm Recurrence, Local/therapy , Neoplasm Staging , Ophthalmologic Surgical Procedures/methods , Radiotherapy, Adjuvant , Rare Diseases , Time Factors , Treatment Outcome
20.
Ocul Oncol Pathol ; 1(2): 111-20, 2015 Feb.
Article in English | MEDLINE | ID: mdl-27171013

ABSTRACT

Benign mesenchymal tumors of the craniofacial complex present unique challenges for orbital surgeons because of their potential for orbital compartment syndrome, ocular morbidity, and facial disfigurement and because definitive surgical management may be associated with significant morbidity. While the precise classification of such lesions depends on radiologic as well as histologic evaluations and remains controversial, benign tumors involving the bony walls of the orbit share features of bony expansion, facial deformity, and the potential to cause significant orbital and ophthalmic morbidity. We herein present 2 cases of benign mesenchymal tumors with bony involvement in the orbitofacial region (1 juvenile ossifying fibroma and 1 central giant cell granuloma) and review the current management of similar benign fibro-osseous and reactive bone lesions of the orbit. These rare entities presented share common orbital and ophthalmic manifestations and remain without any effective definitive treatment options.

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