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Competing Interests: Conflicts of interest None disclosed.

Introduction: Sebaceous gland carcinoma (SGC) of the eyelid is an aggressive tumour with the ability to metastasise and an increased morbidity. Controversies regarding the epidemiology of this malignant eyelid tumour is widespread in the scientific literature. Western reports repeatedly describes eyelid SGC as a rare occurring tumour in general, accounting for 1%-3% of all eyelid tumours, however studies from Asia have uncovered a higher frequency of eyelid SGC including 54% of all eyelid tumours in Japan, and 43%-56% in India. We wish to retrieve observational data of eyelid SGC prevalence in proportion to total eyelid tumours, from pathological studies published worldwide to resolve this controversy., Methods and Analysis: We will search Ovid Medline, EMBASE, Cochrane Central Register of Controlled Trials, Scopus and Google Scholar to identify published reports on eyelid SGC prevalence proportions, aiming to clarify the incidence of the tumour. We will include observational clinicopathological studies reporting prevalence with confirmed histopathology. No limitations on publication date or language will be applied. Data from the individual studies and study quality will be extracted by two individual reviewers. Study quality will be assessed using the JBI Critical Appraisal Instrument for Studies Reporting Prevalence Data. Raw proportions will be transformed and pooled using a random effects model for meta-analysis. And subgroup analysis according to geography will be performed. If data are deemed unsuitable for a meta-analysis, a narrative synthesis will be presented. We will judge the certainty of evidence and present whether this has an overall effect on the results. The results may shed light on a long-standing academic disparity of the scientific literature., Ethics and Dissemination: This systematic review does not require ethical approval. The results of this proposed review will be the subject to a publication in an international peer-reviewed journal within the ophthalmic or pathological specialty., Prospero Registration Number: CRD42023487141., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Sebaceous carcinoma is a rare cutaneous malignancy which is typically regarded as relatively aggressive and has traditionally been subdivided into periorbital or extraorbital tumours. We conducted a retrospective review of all cases of sebaceous carcinoma reported to the Western Australian Cancer Registry between 1987 and May 2019. The incidence of sebaceous carcinoma in Western Australia has increased over the last three decades, with extraorbital tumours being much more common than periorbital tumours. Very few sebaceous carcinomas have led directly to the death of patients; however, adverse outcomes were more likely with periorbital tumours, in particular local recurrence and the need for major surgical intervention., (© 2023 The Authors. Australasian Journal of Dermatology published by John Wiley & Sons Australia, Ltd on behalf of Australasian College of Dermatologists.)

Background: Visceral malignancies in patients with Lynch syndrome behave less aggressively than in those without Lynch syndrome. The behavior of sebaceous carcinoma (SC) in Muir-Torre syndrome (MTS), a variant of Lynch syndrome, is incompletely investigated., Objective: To investigate features and survival of SC patients with and without MTS., Methods: Retrospective cohort study in the Surveillance, Epidemiology, and End Results 17 database from 2000 to 2019 of patients with SC. Patients were classified as MTS or non-MTS cases based on a threshold score of 2 on the Mayo MTS risk score., Results: We identified 105 (2.8%) MTS cases and 3677 (97.2%) non-MTS cases. On univariate analysis, MTS patients were younger, had a higher proportion of tumors outside the head/neck, and had fewer high-grade tumors. On Kaplan-Meier analysis, MTS patients trended toward having better SC-specific survival. On multivariate Cox proportional hazards analysis adjusting for other covariates, MTS status was an independent predictor of worse overall survival. However, there was no association between MTS status and SC-specific survival., Limitations: Given relatively high disease-specific survival in SC, our study may have been underpowered to detect a difference on Kaplan-Meier analysis., Conclusions: Our study suggests SC does not behave more aggressively in patients with MTS., Competing Interests: Conflicts of interest None disclosed., (Copyright © 2023. Published by Elsevier Inc.)

Purpose: To clarify the incidence and demographic characteristics of malignant eyelid tumors diagnosed in a single institute in Japan., Study Design: Retrospective, observational case series METHODS: Patients with malignant eyelid tumors diagnosed histopathologically at Tokyo Medical University Hospital between 1995 and 2019 were reviewed retrospectively. The incidence and demographic profile of malignant eyelid tumors were analyzed. The number of benign eyelid tumors diagnosed histopathologically during the same period was also counted., Results: A total of 412 patients with histopathologically proven malignant eyelid tumors were included. The most common malignant eyelid tumor was sebaceous carcinoma (n = 180, 44%), followed by basal cell carcinoma (n = 148, 36%), squamous cell carcinoma (n = 35, 9%), lymphoma (n = 28, 7%), Merkel cell carcinoma (n = 11, 3%) and others (n = 10, 2%). Mean age of all patients with malignant eyelid tumor at the time of diagnosis was 71.0 ± 13.0 years. For sebaceous carcinoma, the proportion of female patients was significantly higher than that of male patients (P = 0.0283) and the proportion of involvement of upper eyelid was significantly higher than that of lower eyelid (P = 0.0001). On the other hand, there was no sex predominance in basal cell carcinoma and squamous cell carcinoma. The proportion of involvement of lower eyelid was significantly higher than of upper eyelid in basal cell carcinoma (P = 0.001) and squamous cell carcinoma (P = 0.0012). There were 1433 patients with benign eyelid tumors accounting for 78% of all eyelid tumors during the study period., Conclusions: Sebaceous carcinoma is the major malignant eyelid tumor in Japan and is more frequent in women than in men. Epidemiology of malignant eyelid tumors may be affected by the trend of population age structure associated with the recent population aging., (© 2022. Japanese Ophthalmological Society.)

Purpose: To investigate the risk of second primary neoplasms (SPNs) after primary sebaceous carcinoma of the eyelid (SCE)., Methods: Data on patients diagnosed with primary SCE as their first malignancy were extracted from the Surveillance, Epidemiology, and End Results database from 2000 to 2016. Observed-to-expected ratios of SPNs were calculated to estimate standardized incidence ratios (SIRs). Patients were compared with a reference population (RP) matched for age, gender, and race., Results: Five hundred fifty-nine patients with primary SCE were identified, 16% of whom developed SPNs. SCE patients displayed a 61% increased risk of developing SPNs compared with the RP (p < 0.001). Overall, the risk of SPNs of the lungs (SIR = 1.82; p < 0.05), pancreas (SIR = 2.94; p < 0.05), salivary glands (SIR = 41.65; p < 0.001), and skin (SIR = 8.33; p < 0.05) was elevated. Only non-Hispanic Whites were at an increased risk (SIR = 1.51; p < 0.05). Patients 40-54 years old at the time of diagnosis were at the highest risk of developing SPNs compared with the RP (SIR = 3.15; p < 0.05). Women with SCE experienced an increased risk of breast cancer (SIR = 3.6; p < 0.05) and chronic lymphocytic leukemia (SIR = 8.8; p < 0.01)., Conclusion: SCE patients are more likely to develop SPNs of the lungs, pancreas, salivary gland, and skin than the RP. Forty to fifty-four years old Caucasian patients are at the highest risk. Women are at an increased risk of developing breast malignancies and chronic lymphocytic leukemia. Clinicians should be cognizant of these risks when managing SCE patients., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2022 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: To assess the relationship between age at presentation and outcomes of patients with periocular sebaceous gland carcinoma., Methods: Retrospective case series of 241 patients., Results: Of 241 cases, 29 (12%) were young adults (≤40 years), 122 (51%) were middle-aged adults (41-60 years), and 90 (37%) were older adults (>60 years). Based on the eighth edition of American Joint Committee on Cancer, most tumors belonged to T1 category (n = 78, 32%) on presentation. Wide excisional biopsy was the most common treatment modality (n = 183, 79%). There were no statistically significant differences in the clinical presentation, treatment approaches, and histopathology features amongst different age groups, except pagetoid spread which was higher in middle-aged adults (61%, p = 0.004). The incidence of tumor recurrence was higher in older age group compared to younger age groups, with 5-year, 10-year Kaplan-Meier estimate at 31%, 31% in young adults, 38%, 38% in middle-aged adults, and 45%, 100% in older adults (p = 0.03), respectively. The 10-year Kaplan-Meier estimate rate of locoregional lymph node metastasis, systemic metastasis, and death was higher in young adults (51%, 48%, and 48%, respectively) compared to middle-aged (30%, 17%, and 12%, respectively) and older adults (24%, 25%, and 27%, respectively), but the differences were not statistically significant., Conclusions: There is no difference in the clinical presentation of periocular sebaceous gland carcinoma based on age. However, the tumor recurrence rate is much higher in the older age group and death higher in younger patients despite the same treatment strategies in all age groups., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2021 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: To evaluate the clinical features, diagnostic challenges, management, and prognosis of sebaceous carcinoma (SC) of the eyelids and periocular region in a Nordic country., Methods: Patients were identified from the Finnish Cancer Registry and the Helsinki University Hospital databases during the 21-year period 1998-2018. Age, sex, location, clinical and histopathologic diagnosis, treatment and outcome were registered., Results: Sebaceous carcinoma (SC) was diagnosed in 32 patients. The incidence was 0.6 per million. Median age at the time of histopathologic diagnosis was 74 years, and 72% of patients were women. Diagnostic delay was often long, median 12 months. The most common cause for delay was misdiagnosis (72%): a chalazion in 34% and a benign tumour in 22%. The most common location was the upper eyelid (53%) and tumour type a solitary nodule (94%). The SC was not correctly diagnosed in 12 (40%) of 30 preoperative biopsies. The treatment for 31 (97%) patients was complete surgical removal with reconstruction. Conjunctival intraepithelial growth was found in 50%. The leading postoperative problem was ocular irritation (30%). During a median follow-up of 58 months, two patients (6%) experienced a local recurrence and one patient died from metastatic SC., Conclusions: The estimated incidence of SC in Finland was somewhat higher than in other Western countries. The diagnosis was often markedly delayed. Especially differentiation from chalazion continues to be essential. To improve outcomes, it is essential to inform the pathologist about the possibility of SC in eyelid biopsies and specimens and ideally submit them to an ophthalmic pathology service., (© 2020 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To describe the epidemiology of ocular and periocular tumors in patients presenting to a multi-tier ophthalmology hospital network in India using the electronic medical records (EMRs) system., Methods: A retrospective, observational, referral, hospital-based study of 1,142,098 patients. The data were collected using the in-house developed EyeSmart EMR system., Results: During a 6-year study period, 9633 (0.8%) new patients were diagnosed with eye tumors. Of the 9633 patients, 5209 (54%) were male and 4424 (46%) were female. Of all tumors, 6372 (65%) were benign, 282 (3%) were pre-malignant, and 3089 (32%) were malignant in nature, respectively. Overall, the three most common tumors included retinoblastoma ( n = 1167, 12%), ocular surface squamous neoplasia ( n = 957, 10%), and conjunctival nevus ( n = 903, 9%). The three most common benign tumors included conjunctival nevus ( n = 903, 9%), eyelid nevus ( n = 358, 4%), and orbital dermoid cyst ( n = 344, 4%). The three most common malignant tumors included retinoblastoma ( n = 1167, 12%), ocular surface squamous neoplasia ( n = 957, 10%), and sebaceous gland carcinoma ( n = 202, 2%). The most common tumor in 0-10 years' age group was retinoblastoma ( n = 1163, 42%), 11-30 years was conjunctival nevus ( n = 408, 16%), and > 30 years was ocular surface squamous neoplasia ( n = 801, 17%), respectively., Conclusions: The present study results indicate the incidence and distribution of ocular and periocular tumors in a large cohort in India. Retinoblastoma is the most common tumor encountered in a referral-based comprehensive ophthalmic oncology practice in India. The use of EMRs enables to capture the structured information and big data analysis of the same., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Middle East African Journal of Ophthalmology.)

Objective: To evaluate the incidence and management of recurrent periocular sebaceous gland carcinoma at a tertiary ocular oncology service in the United Kingdom., Methods: This was a retrospective cohort study of 62 patients with sebaceous gland carcinoma treated between 2004 and 2017. A total of 10 eyes were treated for local recurrence. The following variables were recorded: age and sex of patient; tumour location, histological subtype; recurrence type; treatment and outcome., Results: Of the 62 cases with eyelid SGC, 10 (16%) had recurrences during the study period and satisfied inclusion criteria. There were six (60%) females and four males in the recurrent group. The mean time interval between initial excision and tumour recurrence was 37 months (median 23 months; range 4 to 84 months). Four patients received cryotherapy to the lids and conjunctiva to control recurrent disease and two patients were treated with topical or intralesional chemotherapy. Four patients (40%) underwent orbital exenteration during the study period. Metastasis occurred in 20% over a mean follow-up of 113 months (median 106; range 47-184 months)., Conclusions: The risk factors for local recurrence of SGC after wide excision with paraffin section control were reported, and an approach to these recurrent lesions was proposed. The results of this study will help guide surgeons dealing with the medical and surgical conundrum of recurrent disease. The risk of recurrence is highest in the first 2 years after initial excision.

Background: Sebaceous neoplasms (SN) may appear sporadically in the general population but may also be part of the Muir-Torre variant of Lynch syndrome (MT-LS). There are few studies in southern Europe on the incidence of MT-LS in the population of patients with SN., Aim: To retrospectively review patients with SN and to analyse their clinical features and the incidence of MT-LS., Methods: Patients with SN diagnosed between 1995 and 2015 were enrolled in the study. The diagnosis of MT-LS was made according to established clinical criteria and, whenever possible, was confirmed by germline mutation analysis., Results: In 60 patients (32 men, 28 women, mean age 69.22 years), 96 SN were diagnosed: 65 adenomas (67.7%), 16 sebaceomas (16.7%) and 15 carcinomas (15.6%). Of the 60 patients, 50 (83.3%) had a single SN and 10 (16.7%) had multiple lesions. Patients diagnosed with MT-LS (12 patients, 20%) were younger (63.25 years vs. 70.71 years), and had a higher incidence of extrafacial SN (4/12 patients, 33.3%), and were significantly (P < 0.001) more likely to have multiple SNs (8/12, 75%) and keratoacanthomas (KAs) (6/12, 50%)., Conclusion: Our study confirms that all patients with SN should be investigated, as 20% of our patients were diagnosed with MT-LS. The most specific features of SN associated with MT-LS in our study were the presence of multiple lesions and association with KAs., (© 2018 British Association of Dermatologists.)

Purpose of Review: Periocular sebaceous cell carcinoma is a rare malignancy that is often misdiagnosed leading to increased morbidity and mortality. This review aims to describe current knowledge on the disease to aid clinicians in timely diagnosis and treatment., Recent Findings: New research has shown that the diagnosis of sebaceous cell carcinoma can be aided with immunohistochemical stains. A revision in the American Joint Committee on Cancer staging system incorporates new tumor size classifications in the staging of eyelid carcinomas. Wide local excision and Mohs micrographic surgery continue to represent the standard of care for initial treatment of localized disease., Summary: The current review highlights diagnostic and treatment modalities to allow clinicians to make timely diagnosis and tailor treatment plans for individual patient presentations.

Background: The incidence of rare cutaneous malignancies is unknown. Current estimates of rare cutaneous malignancy incidences are based on broad epidemiologic data or single institution experiences, not population-based data., Objective: To determine the incidence of several rare nonmelanoma skin cancers., Materials and Methods: The authors conducted a retrospective chart review of a population-based cohort between the years 2000 and 2010. Residents of Olmsted County, Minnesota, who were diagnosed with a biopsy-proven nonmelanoma skin cancer-excluding basal cell carcinoma and squamous cell carcinoma-were included in this study. The primary outcome was tumor incidence. Additionally, the authors extracted patient demographics, tumor characteristics, treatment modalities, and outcomes., Results: The age-adjusted and sex-adjusted incidences per 100,000 persons of multiple rare cutaneous malignancies were: atypical fibroxanthoma (1.8), sebaceous carcinoma (0.8), dermatofibrosarcoma protuberans (0.4), microcystic adnexal carcinoma (0.7), eccrine carcinoma (0.4), eccrine porocarcinoma (0.2), and leiomyosarcoma (0.2)., Conclusion: The authors report population-based incidences and clinical characteristics for these rare cutaneous malignancies. The immune status and smoking status of patients and the treatment and outcomes of these tumors are reported. Additional studies in a broader population are needed to further define the epidemiology and outcomes of these malignancies.

Background: Patients with chronic lymphocytic leukemia (CLL), small lymphocytic lymphoma (SLL), or non-Hodgkin lymphoma (NHL) are at increased risk for the development of skin malignancies., Objectives: This study was conducted to estimate the incidences of rare skin malignancies in patients with CLL/SLL or NHL., Methods: Patients with a diagnosis of CLL/SLL or NHL recorded in the Surveillance, Epidemiology and End Results (SEER) database during 1992-2007 were identified. Diagnoses of specific skin malignancies were identified from SEER files., Results: During 1992-2007, a total of 128,674 patients with first diagnoses of CLL/SLL or NHL were recorded in SEER; 4743 were excluded because follow-up data were unavailable. Among the remaining 123,931 patients, 28,964 had CLL/SLL and 94,967 had NHL. Standardized incidence ratios (SIRs) for invasive malignant melanoma, Merkel cell carcinoma, malignant fibrous histiocytoma, dermatofibrosarcoma protuberans, Kaposi's sarcoma, and sebaceous carcinoma were 2.3, 8.2, 3.6, 2.5, 2.9, and 1.4, respectively, in CLL/SLL patients and 1.6, 3.2, 1.5, 1.3, 17.6, and 0.8, respectively, in NHL patients. When invasive melanoma was stratified by patient age and sex, the highest SIR (17.8) was found in men aged 0-49 years with CLL (P < 0.001)., Conclusions: Patients with CLL/SLL or NHL have a higher risk for the subsequent development of rare skin cancers. Given the more aggressive nature of these malignancies in this setting, regular monitoring for the development and prompt treatment of cutaneous malignancy is prudent in patients with NHL and particularly in patients with CLL. Regular use of sun protection may decrease the morbidity associated with skin cancer in this immunosuppressed population., (© 2015 The International Society of Dermatology.)

Background/aims: Sebaceous adenomas (SAs) are rare, benign sebaceous gland tumours of the eyelid. SAs may be associated with primary internal malignancies. This association is known as Muir-Torre Syndrome (MTS). The purpose of this study was to approximate the prevalence of SAs, to determine the reliability of the clinical diagnosis of SAs and to demonstrate immunohistochemical staining of DNA mismatch repair proteins mutL homologue 1 (MLH1) and mutS homologue 2 (MSH2) for a case of MTS., Methods: We reviewed the histopathology reports from all eyelid specimens collected between 1993 and 2013 at the Henry C Witelson Ocular Pathology Laboratory to determine the proportion of SAs. For the SAs identified on histopathology, we looked at patient charts to see what diagnosis was originally suspected on clinical examination. Immunohistochemical staining for MLH1 and MSH2 was performed on all SAs to screen for MTS., Results: Of the 5884 eyelid specimens collected, 9 were SAs (6 women, 3 men; 42-72 years old). The diagnosis of SA was suspected clinically in only one of the nine cases based on the gross appearance of the eyelid lesion. Immunohistochemistry revealed one SA case with positive MLH1 expression and negative MSH2 expression. These findings prompted systemic work-up and this patient was diagnosed with MTS after discovery of a colon adenocarcinoma T2M0N0., Conclusions: The diagnosis of eyelid SA is rare. The importance of this benign eyelid tumour stems from its association with internal malignancies in MTS. Immunohistochemical staining of mismatch repair proteins MLH1 and MSH2 is a valid and accessible strategy for investigating MTS in patients with SAs., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Aim: To describe the clinical features, management and correlation of the American Joint Committee on Cancer tumour node metastasis (TNM) staging for eyelid carcinoma with outcomes in Japanese patients with sebaceous carcinoma., Methods: Multicentre retrospective review of 63 Japanese patients. Tumours were staged using the American Joint Committee on Cancer 7th edition TNM criteria., Results: A distinct mass was the initial presentation in 94% and correct initial diagnosis made in 57% patients. Most tumours (60%) presented at stage T2aN0M0. The remaining TNM stages were: T2bN0M0 (25%); T3aN0M0 (9%); T3bN0M0 (2%); T2bN1M0 (2%); T3bN1M1 (2%). Frozen section controlled excision was performed in 81%. One patient required an orbital exenteration. Median follow-up was 4.2 years. Local recurrence occurred in four patients treated with frozen section controlled excision. Five patients had regional nodal metastases, two of which had T2aN0M0 lesions. T3a tumours and greater were significantly associated with local recurrence but not regional nodal metastasis. One patient died due to disease. One patient is alive with disease, and remaining patients were alive without disease at last follow-up., Conclusions: In this Japanese cohort, an eyelid mass was the main clinical presentation of sebaceous carcinoma. Contrary to previous reports, T2a tumours smaller than 10 mm were associated with regional nodal metastases.

Objective: To describe the clinicopathological characteristics of patients with eyelid tumours in Hong Kong., Design: Retrospective case series., Setting: A tertiary eye centre in Hong Kong., Patients: A computerised retrieval system was used to identify all patients who underwent eyelid mass excisions with histological reports, encountered in the period 2000 to 2009, in a tertiary eye centre. The demographics (age, gender), clinical features (laterality, tumour topography), and the pathological diagnosis of each patient were documented. Descriptive statistical tabulation and analyses were performed on the data., Results: In all, 198 patients were identified; all were Chinese. Their mean age was 54 years for benign lesions and 68 years for malignant ones. Women were more commonly affected. Benign tumourous lesions occurred more commonly on the upper (n=91; 54%) than lower eyelid (n=79; 47%), whereas malignant lesions more often affected the lower (n=17, 61%) than upper (n=11, 39%) eyelid. The distribution of left and right eye involvement was similar (103 vs 101, respectively). In six patients, there were bilateral benign lesion. Regarding benign masses, 45 (27%) were intradermal neavi, 38 (22%) were squamous papillomas, 25 (15%) were seborrhoeic keratosis lesions, 14 (8%) were epidermoid cysts, and 7 (4%) were compound naevi. Regarding malignant eyelid tumours, the most common was basal cell carcinomas (n=12, 43%), 5 (18%) were squamous cell carcinomas, 3 (11%) were actinic keratosis lesions, and 2 (7%) each were sebaceous gland carcinomas and melanomas., Conclusion: Benign lesions constituted the majority of these eyelid tumours. Among the malignant lesions, basal cell carcinoma was the commonest type, with lower lid involvement in majority. Sebaceous gland carcinoma is not rare, which is in contrast to Caucasian populations. The relative frequencies of the most common malignant tumours in Hong Kong differed substantially from those reported in other Asian studies.

Purpose: To investigate the epidemiology and clinicopathological management for ocular sebaceous carcinoma (OSC) in the UK., Methods: Observational prospective cohort study of patients with newly-diagnosed OSC. The British Ophthalmological Surveillance Unit captured incident cases of OSC between 2008 and 2010. Incident and 6-month follow-up questionnaires from reporting ophthalmologists captured OSC demographic and clinical data., Results: Data were available on 51 patients with unilateral OSC (response rate 85%). The UK estimated annual incidence was 0.41 cases per million population (95% CI 0.31 to 0.54). Median age was 70 years (SD 14, range 28-98) with 57% women. OSC location was upper lid (54%), lower lid (20%), multicentric (14%) and caruncle (12%). Most common misdiagnoses included chalazion (42%), basal cell carcinoma (30%) and blepharoconjunctivitis (16%), with median delay in diagnosis of 10 months (SD 9, range 0.5-36). Specialist ophthalmic pathologists performed diagnostics in 62%, with pagetoid/intraepithelial spread present in 39%. Misdiagnosis of chalazion (p=0.019) and pagetoid tumour spread (p=0.016) was associated with a significant diagnostic delay (one-way ANOVA/R(2)). Primary surgical management involved excision with reconstruction (49%), primary exenteration (10%) and Mohs surgery (8%). There were three deaths (out of 51) during the study period; one patient died of OSC-related disease and the other two due to other causes., Conclusions: This population-based prospective study confirms OSC as a rare cancer in the UK. Masquerade syndromes result in significant diagnostic delays and increase the risk of pagetoid tumour spread. There is considerable UK variation in pathological and surgical management, and ocular reconstruction and radical surgery is often required for OSC due to delayed presentation.

Background: Sebaceous carcinoma (SbCC) is a rare malignancy that often mimics benign conditions. Lymphatic involvement, large T3 tumors herald a dismal survival for patients. We present our series of 13 cases of locally advanced SbCC of the eyelid treated at a surgical oncology unit and describe the clinical profile, patterns of nodal spread and recurrence pattern in this subset of SbCC., Methods: A retrospective analysis of case records was carried out for patients presenting with orbital tumors between January 1997 and April 2010 in the department of Surgical Oncology, AIIMS, New Delhi, India. All patients underwent orbital exenteration and superficial parotidectomy with neck dissection was added to patients with clinically significant lymphadenopathy. All patients who underwent OE after 2002 were advised radiotherapy as adjuvant therapy. The end point was development of recurrence or end of two year follow up period which ever occurred earlier., Results: Thirteen patients underwent orbital exenteration. Eleven patients had clinically palpable lymphadenopathy. Ten patients (76.9%) had pathologically confirmed metastatic nodes. Parotid lymph node involvement was present in all patients (100%); two of these ten patients also had level II b cervical lymph node involvement. Recurrence was observed in seven patients (53.8%). All recurrences were loco-regional only and no systemic metastases was seen. There were only two recurrences in the group that received PORT., Conclusions: Eyelid SbCC is a loco-regionally aggressive malignancy and adequate disease control can be achieved with combined modality approach of radical surgery followed by post operative radiotherapy.

Background: Renal transplantation is associated with an increased incidence of nonmela-noma skin cancer (NMSC) caused by immunosuppression. Squamous cell carcinoma (SCC) and basal cell carcinoma (BCC), the two major histological types of NMSC, exhibit more aggressive biological and clinical courses in renal transplant recipients (RTRs), with higher rates of recurrence and mortality than in the general population., Methods: We retrospectively analyzed our experience of NMSC in 1736 renal transplantations performed over a 25-year period. All cases of skin cancer after renal transplantation were included except those of skin cancer resulting from melanoma and mesenchymal skin tumors., Results: In our series, the overall incidence of NMSC after transplantation was 2.2% (n = 39), and SCC represented the most frequent skin malignancy (64.1%), followed by BCC (17.9%), Bowen's disease (10.2%), basosquamous carcinoma (5.1%), and a rare case of invasive sebaceous carcinoma (2.6%). A shift to newer immunosuppressive regimens after the initial diagnosis of NMSC had been implemented in eight cases (20.5%). The recurrence rate after initial treatment was 41% (n = 16), and distant metastatic disease was diagnosed in 15.4% (n = 6) of NMSC patients. The NMSC-specific mortality rate was 25.6% (n = 10)., Conclusions: Nonmelanoma skin cancer remains a significant source of morbidity and mortality in RTRs, and post-transplant surveillance should be increased., (© 2011 The International Society of Dermatology.)

Purpose: To determine the epidemiologic and clinical characteristics of patients with malignant eyelid tumors in Hong Kong., Methods: A computerized retrieval system was used to identify all patients with histologically confirmed malignant eyelid tumors residing in Western Kowloon and the Southern New Territories, Hong Kong, during the 13-year period from 1997 to 2009. The patients' medical records were reviewed for clinical and demographic data., Results: A total of 36 patients were identified; all were Chinese. The mean age at diagnosis was 73.4 ± 11.5 years. Women were more commonly affected. Each patient had 1 tumor. Tumors developed more commonly on the lower eyelid (n = 19; 52.8%) than on the upper eyelid (n = 11; 30.6%). The right eye was more often involved (n = 20; 55.6%). With regard to the type of tumor, 27 were basal cell carcinoma (BCC) (75%), 4 sebaceous gland carcinoma (11.1%), 2 squamous cell carcinoma (5.6%), 1 mucinous eccrine carcinoma (2.8%), 1 verrucous carcinoma (2.8%), and 1 metastatic carcinoma (2.8%). Most were treated by frozen section-guided excision and eyelid reconstruction. Recurrence occurred in 1 case only. Incidence was low until approximately 45 years of age, after which it rose sharply. The incidence continued to increase until above the age of 85 years. There was an overall increase in incidence from 0.6 per million in 1997 to 2.3 per million in 2009., Conclusion: Increasing incidence of malignant eyelid tumors was noticed in Hong Kong from 1997 to 2009, with BCC dominating the incidence trend. Recurrence after complete surgical excision was uncommon.

Background: Sebaceous neoplasms are adnexal neoplasms that contain a varying number ofsebocytes, i.e. large cells with lipid-laden vacuolated cytoplasm, soap-bubble in appearance, and crenate nuclei. They are uncommon compared to other adnexal neoplasms. Various sebaceous neoplasms with complex histopathologic features and varying degree ofsebaceous cells differentiation have been described in the literature., Objectives: To study the prevalence of sebaceous neoplasms, i.e., nevus sebaceus, sebaceous hyperplasia, sebaceous adenoma, sebaceoma, sebaceous epithelioma, superficial epithelioma with sebaceous differentiation, and sebaceous carcinoma diagnosed in the Department of Pathology, Siriraj Hospital, Mahidol University during the 9-year-period between 1997 and 2005. To study the prevalence of tumor transformation that occurs in nevus sebaceus., Material and Method: A retrospective study of all sebaceous neoplasms including Nevus sebaceous, sebaceous hyperplasia, sebaceous adenoma, sebaceoma, sebaceous epithelioma, superficial epithelioma with sebaceous differentiation, sebaceous carcinoma, and all neoplasms containing the term "sebaceous" was performed. All slides were re-analyzed and re-diagnosed, without knowledge of the previous diagnosis or any clinical data, according to the criteria described in the standard textbooks of dermatopathology by Elder, McKee. Comparison between the previous diagnoses and the reviewed diagnoses was performed to assess the initial accuracy of all sebaceous neoplasms diagnosed. Small-sized biopsies or biopsies that possess incomplete sebaceous differentiation, in which the sebocytes were few and subtle, sometimes are difFicult to diagnose. In these instances, the clinical correlation was needed for positive diagnosis. Afterwards, these reviewed diagnoses were recorded and classified according to the patient's age, gender, and localization., Results: Two hundred seven sebaceous neoplasms (2.34%) from the 8819 skin biopsies that were taken in the Department of Pathology, Siriraj Hospital during the 9-year-period, were included After exclusion of some authentically non-sebaceous neoplasms, 182 sebaceous neoplasms were found Nevus sebaceus (n=85, 46.7%) and sebaceous hyperplasia (n=64, 35.1%) were the two most common benign lesions. The others were sebaceoma (n=3, 1.6%), sebaceous adenoma (n=2, 1.1%), sebaceous epithelioma (n=1, 0.5%), sebaceous carcinoma (n=26, 14.3%), and one unclassified sebaceous lesion that could not be considered a neoplasm. Tumor degeneration was found in 14 nevus sebaceus in which 21 neoplasms existed, namely, trichilemmoma (wart)-like lesion (n=4), primitive follicular induction (n=7), syringocystadenoma papilliferum (n=3), trichoblastoma (n=3), and one of each of trichoepithelioma, sebaceous adenoma, tumor of follicular infundiculum, and mucoepidermoid carcinoma., Conclusion: Twenty-six sebaceous carcinomas out of 182 sebaceous neoplasms, occurring mostly on the patients'eyelids, were found The most common sebaceous neoplasm was nevus sebaceus (n=85); the prophylactic excision of this lesion was recommended as tumor degeneration was frequent (14 out of 85 cases). Epithelial membrane antigen (EMA) usually decorated both normal and abnormal sebocytes. It was very helpful in the detection of sebocytes in basaloid cells in sebaceous neoplasms and among lymphoid cells within metastasized lymph nodes and a discriminant between sebaceous and nonsebaceous neoplasms.

Objective: Immunosuppression may increase risk for some skin cancers. We evaluated skin cancer epidemiology among persons with AIDS., Design: We linked data from population-based US AIDS and cancer registries to evaluate risk of nonkeratinocytic skin cancers (melanoma, Merkel cell carcinoma, and appendageal carcinomas, including sebaceous carcinoma) in 497 142 persons with AIDS., Methods: Standardized incidence ratios (SIRs) were calculated to relate skin cancer risk to that in the general population. We used logistic regression to compare risk according to demographic factors, CD4 cell count, and a geographic index of ultraviolet radiation exposure., Results: From 60 months before to 60 months after AIDS onset, persons with AIDS had elevated risks of melanoma (SIR = 1.3, 95% confidence interval 1.1-1.4, n = 292 cases) and, more strongly, of Merkel cell carcinoma (SIR = 11, 95% confidence interval 6.3-17, n = 17) and sebaceous carcinoma (SIR = 8.1, 95% confidence interval 3.2-17, n = 7). Risk for appendageal carcinomas increased with progressive time relative to AIDS onset (P trend = 0.03). Risk of these skin cancers was higher in non-Hispanic whites than other racial/ethnic groups, and melanoma risk was highest among men who have sex with men. Melanoma risk was unrelated to CD4 cell count at AIDS onset (P = 0.32). Risks for melanoma and appendageal carcinomas rose with increasing ultraviolet radiation exposure (P trend <10 and P trend = 10, respectively)., Conclusion: Among persons with AIDS, there is a modest excess risk of melanoma, which is not strongly related to immunosuppression and may relate to ultraviolet radiation exposure. In contrast, the greatly increased risks for Merkel cell and sebaceous carcinoma suggest an etiologic role for immunosuppression.

Background: Sebaceous gland tumors are a rare type of neoplasm. In some cases they have been associated with visceral tumors in patients with Muir-Torre syndrome, a hereditary form of nonpolyposis colorectal cancer. The aim of this study was to review the diagnosis and follow-up of a series of patients with sebaceous gland tumors to assess how many met the criteria for Muir-Torre syndrome., Patients and Methods: A search was performed of records from 1990 to 2005 in the database of the Department of Dermatology of the Consorcio Hospital General Universitario de Valencia in Valencia, Spain, to identify patients with sebaceous gland tumors. The biopsy material was reviewed to confirm the diagnosis. We also searched the patient histories for information suggestive of a diagnosis of Muir-Torre syndrome; when the histories were incomplete, we contacted the patients by telephone., Results: We identified 20 patients diagnosed with sebaceous gland tumors, but after reviewing the biopsy material diagnosis was only confirmed in 12. Two patients belonged to a family with a history of visceral tumors that met the clinical criteria for hereditary nonpolyposis colorectal cancer syndrome. Follow-up was not uniform in all patients and not all underwent the same tests., Conclusions: It is essential to rule out the presence of Muir-Torre syndrome in patients with sebaceous gland tumors. The use of new techniques such as immunohistochemistry or detection of microsatellite instability may help to identify families at increased risk of Muir-Torre syndrome.

Background: Adnexal skin tumors share many features in common and differentiate along one line. Their detailed morphological classification is difficult because of the variety of tissue elements and patterns seen. They may be clinically confused with other cutaneous tumors. The aim of this report is to review and classify all adnexal tumors seen in a pathology department over a 16-year period., Method: A 16-year retrospective analysis of all adnexal skin tumors seen in a large University Teaching Hospital in Nigeria from January 1991- December 2006. All tissue specimens were fixed in 10% formalin, processed in paraffin wax and stained with Haematoxylin and Eosin. Histology slides were retrieved, studied and lesions characterized., Results: Fifty-two adnexal tumors were seen, accounting for 0.9% of all cutaneous tumors seen within the same period. The median age was 33 years (range: 4 days-70 years). Clinical presentations varied from discreet swellings and nodules to ulcerated masses. Five patients presented with recurrent lesions. Only two cases had a clinical diagnosis of adnexal tumor. Twenty-four (46%) of the lesions were distributed in the head and neck region. Duration of symptoms was 2 months to 15 years (median: 12 months). Tumours of the sweat gland were the commonest--41 (78.8%); they comprised predominantly eccrine acrospiroma (17), characterized histologically by solid nests of round to polygonal cells with clear to eosinophilic cytoplasm, forming tubules in areas. Tumours of sebaceous gland were 7 (13.5%); they comprised mainly Nevus sebaceous of Jadassohn (6), composed of immature sebaceous glands and pilar structures microscopically and a solitary sebaceous adenoma. Tumours of hair follicle were 4 (7.7%) and included trichoepithelioma, characterized microscopically by multiple horn cysts and epithelial tracts connecting abortive pilar structures and a trichofolliculoma. Forty-six lesions (88.5%) were benign and six (11.5%) malignant., Conclusion: Adnexal skin tumors have distinct histological patterns which differentiates them from other cutaneous tumors. They are commonly distributed in the head, neck and trunk. The commonest variants are those of eccrine sweat gland origin. Malignant adnexal tumors are uncommon in our setting.

Background: Cutaneous sebaceous carcinoma (SC) is a rare malignancy derived from adnexal epithelium of sebaceous glands. Periorbital SC is approximately three times more common than extraorbital SC. To date, there are few data from an Australian source to document the prevalence or clinical outcome of this tumour., Methods: A retrospective review was undertaken of the cases from the Institute of Clinical Pathology and Medical Research using their histopathology database for patients with a diagnosis of SC. Patients were identified over a period of 34 years from 1971-2005 and their management and follow up are reported., Results: There were 25 patients in all. Seventeen were men and eight were women. Of the 25 cases, 12 were extraorbital and 13 were periorbital. Three metastases and one tumour showed extensive local invasion. There were nine cases of incomplete excisions and 13 patients were reoperated on for either incomplete excisions, to obtain a wider margin or for local recurrences. Adjuvant radiotherapy was used on five patients, three of whom had lymph node deposits., Conclusion: Sebaceous carcinoma is a rare tumour. Extraorbital SC is an aggressive and invasive malignancy. Excision margins may need to be wider than previously thought. Exposure to ultraviolet radiation (sunlight) may play a role in the aetiology. Wide excision and selective use of radiotherapy remain the treatment of choice.

Objective: To provide clinical details and long-term outcome data for a series of patients with eyelid or conjunctival melanoma or eyelid sebaceous cell carcinoma who underwent sentinel lymph node (SLN) biopsy., Design: Retrospective interventional case series with review of clinical records and pathologic specimens., Setting: Tertiary comprehensive cancer center., Patients: Twenty-five consecutive patients treated at 1 institution for eyelid or conjunctival melanoma or eyelid sebaceous cell carcinoma from December 2000 to October 2004., Interventions: Surgical removal of the eyelid or conjunctival tumor and SLN biopsy., Main Outcome Measures: Local treatment modalities; lymphatic basins in which SLNs were identified; status of SLNs; false-negative rate; and long-term patterns of local recurrence, regional and distant metastasis, and survival., Results: Seven patients had conjunctival melanoma, 8 had eyelid-margin melanoma with a considerable palpebral conjunctival component, and 10 had eyelid sebaceous cell carcinoma. The SLNs were identified in all but 1 patient by using technetium Tc 99m sulfur colloid as a tracer. Intraoperatively, in 16 patients in whom blue dye was used in addition to technetium Tc 99m sulfur colloid during mapping, no SLN was blue. One patient with conjunctival melanoma and 1 patient with eyelid melanoma had a histologically positive SLN. Two patients with eyelid melanoma and 2 patients with eyelid sebaceous cell carcinoma had negative findings from SLN biopsy but developed recurrence in their regional lymph nodes during the follow-up period. Overall, during follow-up, 2 of 10 patients with sebaceous cell carcinoma (20%) and 5 of 15 patients with eyelid or conjunctival melanoma (33%) had regional lymph node metastasis. Four patients with melanoma who had regional metastasis also developed distant organ metastasis. Two patients with sebaceous cell carcinoma--1 with regional metastasis and 1 without--developed distant organ metastasis., Conclusions: The detection of histologically positive SLNs in this series of patients may justify further study of SLN biopsy for high-risk patients with ocular adnexal melanoma or eyelid sebaceous cell carcinoma. The false-negative rate is higher than that reported for SLN biopsy at most other anatomic sites. Patients with negative findings from SLN biopsy still require careful long-term follow-up because they may develop regional or distant metastasis.

Background: Eyelid malignancies are completely treatable if detected early. The treatment depends on the invasiveness of the cancer which in turn depends on the type of malignancy., Aim: The aim of the study was to characterize the distribution of the types of eyelid malignancies in central India., Settings and Design: The study was conducted in the Department of Plastic and Maxillofacial Surgery at a tertiary care hospital., Materials and Methods: We report a series of 27 cases of eyelid malignancies. In the same case series, we also include a case of malignant hemangiopericytoma which is an extremely rare form of eyelid malignancy worldwide., Statistical Analysis: Depending on the underlying statistical distribution, either analysis of variance (ANOVA) or the Kruskal-Wallis (K-W) test was used to assess the differential distribution of these variables across the types of eyelid malignancies observed in this study., Results: We observed that sebaceous cell carcinoma (approximately 37%) was almost as prevalent as basal cell carcinoma (approximately 44%) in the study subjects and had an earlier age of occurrence and a more rapid clinical course., Conclusions: Sebaceous cell carcinoma of the eyelid is almost as common as basal cell carcinoma in a large tertiary care centre in central India.

Purpose: To describe the epidemiologic characteristics and incidence trends of primary eyelid cancers in the Chinese population in Taiwan between 1979 and 1999., Design: Retrospective population-based cancer registry data review., Participants: A total of 1166 primary eyelid cancers coded to International Classification of Diseases 8 or 9, site 172.1 (malignant melanoma of eyelid) and 173.1 (other malignant neoplasm of eyelid) were retrieved from the Taiwan National Cancer Registry between January 1979 and December 1999. Of these, 1121 (96.1%) were histopathologically verified and were used for analysis., Methods: Age- and gender-specific incidence rates were calculated and were age-adjusted to the 2000 world standard population. Trends in incidence rates were estimated by calculating the annual percentage change. We further examined the effects of age, period of diagnosis, and birth cohort on incidence trends using age-period-cohort analysis., Main Outcome Measure: Age-standardized incidence rate., Results: The mean age at diagnosis of eyelid cancers was 62.6+/-14.1 years. The average annual age-standardized incidence rate was 3.2 per million during the study period. There was an overall increase of incidence rates from 1.5 per million in 1979 to 5.1 per million in 1999, with an annual percentage change of 4.63% (P<0.001). The most common eyelid malignancy was basal cell carcinoma (BCC; 65.1%), followed by squamous cell carcinoma (12.6%), and sebaceous cell carcinoma (7.9%). The increasing rates were mainly driven by an increase in the incidence of BCC. Cohort effects were found to play a major role in terms of model fit for the incidence trends of BCC., Conclusions: These national data reveal a rapidly increasing incidence of eyelid cancers from 1979 to 1999 in Taiwan. Basal cell carcinoma dominates the incidence trends, and the significant cohort effects give a warning of increasing risk of BCC in younger birth cohorts.

Background: Sebaceous carcinoma of the eyelid is a rare tumor. Treatment can be complicated by noncontiguous spread of the tumor., Objective: Review a series of patients with sebaceous carcinoma to illustrate clinical presentations, treatments, and outcomes., Methods: We retrospectively reviewed medical records of patients with sebaceous carcinoma treated at Mayo Clinic (Rochester, MN)., Results: Fourteen patients had sufficient follow-up data available for review. Mean follow-up was 57 months (range 18-134 months). Treatment included wide local excision with frozen and permanent section control (9 patients, 64%), Mohs micrographic surgery (2 patients, 14%), external beam radiation (2 patients, 14%), and exenteration and total parotidectomy with cervical lymph node dissection (1 patient, 7%). Two patients (14%) had local recurrence of the tumor after wide local excision, and 1 patient (7%) had tumor recurrence after Mohs micrographic surgery., Conclusion: Treatment should be chosen on the basis of the extent of the tumor and the specific needs of the patient. The mainstay of treatment of tumors without orbital involvement has been wide local excision, with the margins checked in both permanent and frozen sections, in combination with conjunctival map biopsies when warranted. Mohs micrographic surgery is an alternative that may provide tissue conservation and lower recurrence rates. Recurrence rates between treatments are difficult to assess because of the small number of cases reported in the literature. In cases with orbital involvement, exenteration may be warranted. Radiation may be useful when surgery cannot be tolerated.

Objective: To determine the relative frequencies, average annual incidences, and patient characteristics of non-basal cell and non-squamous cell malignant neoplasms of the eyelid in a defined geographic population., Design and Setting: A retrospective study using the Florida Cancer Data System to identify malignant tumors of the eyelid, except for basal cell and squamous cell carcinomas, from 1981 through 1994. Cases were limited to persons who resided within Florida., Main Outcome Measure: Incidence of histologically confirmed malignant eyelid tumors., Results: Two hundred six primary malignant eyelid tumors were identified. The 3 most common, in order of frequency, were melanoma, sebaceous carcinoma, and lymphoma. The median age at diagnosis for all patients was 73 years. Only 3 of the 206 malignant neoplasms occurred in blacks. The annual incidence of eyelid melanoma and sebaceous carcinoma in whites older than 20 years was 0.6 and 0.5 per million, respectively. Kaposi sarcoma was the most common type of mesenchymal tumor. Eleven different histologic types of lymphoma were found in the eyelid. Only 2 of 27 lymphomas had T-cell lineage., Conclusions: Malignant tumors of the eyelid other than basal cell and squamous cell carcinoma are uncommon and usually occur in elderly white persons. Primary eyelid tumors of any type are rare in blacks. The risk of a non-basal cell and non-squamous cell malignant neoplasm of the eyelid in Florida is 6.4 times greater for whites than for blacks (95% confidence interval [CI], 2.1-20.2). A variety of B-cell lymphomas can be manifested as primary eyelid tumors.