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Dermatologic Surgery : Official... Jan 2021Sebaceous carcinoma (SC) of the eyelid is a rare, aggressive malignancy associated with high rates of recurrence, metastasis, and tumor-related mortality. (Meta-Analysis)
Meta-Analysis
BACKGROUND
Sebaceous carcinoma (SC) of the eyelid is a rare, aggressive malignancy associated with high rates of recurrence, metastasis, and tumor-related mortality.
OBJECTIVE
Provide a collective analysis of clinical presentations, management techniques, and outcomes, and compare outcomes of common treatment methods.
METHODS AND MATERIALS
Observational studies reporting management and outcomes of SC of the eyelid were included. Patient and clinical data were extracted, and meta-analysis of proportions was performed.
RESULTS
One thousand three hundred thirty-three subjects were included with a mean age of 65.2 years and 803 (60.2%) women. Of 647 initial diagnoses reported, 277 (42.8%) were correct, and the mean diagnostic delay was 14.7 months (range 8.5-34.8). The tumor location was reported in 1,246 subjects and involved the upper eyelid in 780 (62.6%), lower eyelid in 409 (32.8%), and 57 (4.8%) involved both. Overall rates of recurrence, metastasis, and tumor-related mortality were 15.9%, 12.1%, and 6.2%, respectively. There were no statistically significant differences in wide local excision (WLE) versus Mohs micrographic surgery (MMS) outcomes.
CONCLUSION
Sebaceous carcinoma of the eyelid is more common in women, on the upper eyelid, and is frequently misdiagnosed initially. Rate of recurrence, metastasis, and tumor-related mortality were similar in subjects managed with WLE versus MMS.
Topics: Delayed Diagnosis; Eyelid Neoplasms; Female; Humans; Male; Mohs Surgery; Sebaceous Gland Neoplasms
PubMed: 33347004
DOI: 10.1097/DSS.0000000000002660 -
Journal of the European Academy of... Sep 2022Cutaneous adnexal tumours (ATs) encompass a variegated group of hamartomas and benign or malignant tumours, originating from the hair follicle, sebaceous, eccrine or... (Review)
Review
Cutaneous adnexal tumours (ATs) encompass a variegated group of hamartomas and benign or malignant tumours, originating from the hair follicle, sebaceous, eccrine or apocrine glands that may simulate other cutaneous neoplasms. This study aims to provide a comprehensive overview of the spectrum of clinical and dermoscopic features of ATs, to better define these lesions and assist in the differential diagnosis. We performed a two-step systematic search of the literature in PubMed, Embase and Cochrane Library databases from inception until 4 September 2020. In the first step, we aimed to define histological variants of ATs with descriptions of dermoscopic criteria. The second step included a search for the name of each previously identified AT variants in the same databases adding 'AND (epilum* or dermosc* or dermatosc*)'. All study types in English language reporting dermoscopic images of ATs were included. Collisions between ATs and other inflammatory or neoplastic skin lesions were excluded, with the exception of collisions with a sebaceous nevus. The protocol of this study was prospectively registered in PROSPERO (CRD42021244677). In total, 206 articles met our inclusion criteria, encompassing 372 ATs in 365 patients. Most ATs were apocrine-eccrine (n = 217, 58.3%, n = 173 benign) with a prevalence of poromas (n = 82), followed by follicular ATs (n = 88, 23.7%, n = 83 benign) and sebaceous ATs (n = 67, 18.0%, n = 49 benign). Most patients had a single AT lesion (320, 86.0%), while 42 (11.3%) had multiple ATs. A syndrome causing multiple ATs was identified in 15 patients. Histopathological analysis revealed 82% benign (n = 305) and 18.0% malignant (n = 67). ATs were classified according to their ability to mimic four groups of more common skin tumours: basal cell carcinoma, squamous cell carcinoma, melanocytic lesions and benign cutaneous lesions. Moreover, we have highlighted the ability of malignant variants of ATs to simulate benign skin lesions. This systematic review offers a comprehensive overview of the common clinical and dermoscopic features of follicular, sebaceous and apocrine-eccrine ATs and details possible differential dermoscopic features.
Topics: Carcinoma, Basal Cell; Dermoscopy; Humans; Nevus, Sebaceous of Jadassohn; Skin Neoplasms; Sweat Gland Neoplasms
PubMed: 35536546
DOI: 10.1111/jdv.18210 -
Familial Cancer Apr 2023A subset of patients with Lynch Syndrome demonstrates cutaneous manifestations of the disorder. Characterization of these Lynch-related skin lesions could help in early... (Review)
Review
A subset of patients with Lynch Syndrome demonstrates cutaneous manifestations of the disorder. Characterization of these Lynch-related skin lesions could help in early recognition of patients with Lynch Syndrome. A broad search of the literature on OVID Medline and Embase was carried out to capture papers reporting cutaneous manifestations in Lynch Syndrome patients. The results were uploaded into Mendeley reference management software. The PRISMA workflow was used in the literature selection process. In this systematic review, data were collected from 961 cases from 413 studies, including 380 molecularly confirmed Lynch Syndrome cases. The main skin lesions were: Sebaceous adenomas (43%), sebaceous carcinomas (27%), keratoacanthomas (16%), sebaceomas (13%), squamous cell carcinomas (23%), and basal cell carcinomas (10%). MSH2 variants were the most common underlying genotype (72%). Assessment of mismatch repair by immunohistochemistry, microsatellite instability analysis, or both were performed on 328 skin lesions from 220 (58%) molecularly confirmed cases. In those skin lesions, 95% of Immunohistochemistry and 90% of the microsatellite instability test results were concordant with the underlying genotype. Sebaceous skin lesions are well-recognised phenotypic features of Lynch Syndrome. Our results show that squamous and basal cell carcinomas are relatively common in patients with Lynch syndrome; however, available evidence cannot confirm that Lynch syndrome is causal. Immunohistochemistry and/or microsatellite instability testing of skin tumours in patients with a family history of Lynch Syndrome-associated cancers may be a useful approach in identifying patients requiring referral to Clinical Genetics and/or consideration of germline genetic testing for Lynch Syndrome.
Topics: Humans; Muir-Torre Syndrome; Microsatellite Instability; Sebaceous Gland Neoplasms; Genotype; Carcinoma, Basal Cell; MutS Homolog 2 Protein
PubMed: 36418753
DOI: 10.1007/s10689-022-00319-8 -
International Journal of Dermatology Jun 2024Sebaceous carcinoma (SC) is a rare neoplasm affecting periocular and extraocular sites. If inadequately treated, it can recur and cause morbidity. Specific management... (Review)
Review
Sebaceous carcinoma (SC) is a rare neoplasm affecting periocular and extraocular sites. If inadequately treated, it can recur and cause morbidity. Specific management guidelines have not been established. Wide local excision (WLE) has been traditionally used; however, Mohs micrographic surgery (MMS) can be advantageous because of complete margin assessment and tissue-sparing nature. This analysis aims to systematically review the surgical modalities used for the management of SC. Articles meeting eligibility criteria were identified using MEDLINE (via PubMed), Embase, Cochrane, and Scopus databases. All studies investigating surgical management of SC with WLE or MMS were considered. Seventy studies met inclusion criteria, including retrospective cohort studies, case series, and case reports. WLE was used in 32 studies, MMS in 29, and MMS and WLE in 9. Subgroup analysis showed that MMS has lower recurrence rates. For WLE, local, regional, and distant recurrence rates were 23.4%, 13.3%, and 11.0%, respectively, and for MMS, 6.8%, 4.3%, and 4.6%, respectively. Patients treated with WLE were more likely to have local recurrence than patients treated with MMS (P = 0.001). WLE cases were more likely to have a regional (P = 0.05) and distant recurrence (P = 0.001). Limitations of the study include heterogeneity of case reports, case series, and retrospective studies, variable follow-up times between the two groups, and large tumors included in the WLE category. In addition, disease-specific survival was not evaluated. MMS cases showed a superior outcome for local, regional, and distant recurrence, making it a good option for the management of SC.
PubMed: 38856083
DOI: 10.1111/ijd.17283 -
Cancer Treatment Reviews Nov 2021Rare cutaneous cancers require early management given their aggressive nature; however, few therapeutic options exist for managing these rare cancers.
BACKGROUND
Rare cutaneous cancers require early management given their aggressive nature; however, few therapeutic options exist for managing these rare cancers.
OBJECTIVE
To identify emerging therapies for extramammary Paget's disease, Merkel cell carcinoma, sebaceous gland carcinoma, microcystic adnexal carcinoma, Kaposi sarcoma and cutaneous angiosarcoma.
METHODS
A systematic review was conducted using PubMed database from October 2010 to October 2020. Published clinical trials and case reports/series were included if they involved primarily a targeted agent rather than classic cytotoxic chemotherapy or photosensitizing medication. Active clinical trials were evaluated using ClinicalTrials.gov, the Japanese University Hospitals Clinical Information Network, and the ISRCTN registry. Quality of evidence for each study was rated using the Oxford Centre for Evidence-Based Medicine Level of Evidence Rating Scale.
RESULTS
There are several emerging therapies for rare cutaneous cancers with many clinical trials actively recruiting. PD-1 receptor inhibitors were the most investigated treatment, targeting several cancers. Merkel cell carcinoma and Kaposi sarcoma had the most clinical trials while microcystic adnexal carcinoma and sebaceous gland carcinoma had the least. The main limitation was a lack of key findings from clinical trials still in progress.
CONCLUSIONS
Emerging therapies exist for rare cutaneous cancers; results of ongoing studies will provide more robust evidence in the future.
Topics: Antineoplastic Agents; Humans; Molecular Targeted Therapy; Skin Neoplasms
PubMed: 34418780
DOI: 10.1016/j.ctrv.2021.102266 -
International Journal of Dermatology Apr 2020Extraocular sebaceous carcinoma (EOSC) is an aggressive malignancy of the sebaceous gland. Surgery is considered the cornerstone of treatment, but there is lack of... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Extraocular sebaceous carcinoma (EOSC) is an aggressive malignancy of the sebaceous gland. Surgery is considered the cornerstone of treatment, but there is lack of clarity about extent and adjuvant treatment.
METHODS
We conducted a systematic review and analysis of individual patient data of all published cases of EOSC to look into demography, pattern of care, importance of type of surgery, and other adjuvant treatment and survival outcome. A search of PubMed and Google Scholar was done with the key words sebaceous carcinoma, extraocular sebaceous carcinoma, and Muir-Torre syndrome till December 2017. The data were compiled in an Excel chart and analyzed using SPSS IBM software.
RESULTS
Data of 206 patients were retrieved. Median age at presentation was 65 years (range: 11-96 years). Surgery was performed in all except 13 patients. Of these 13, eight were deemed inoperable for extensive disease, and five had metastatic disease. Median PFS and OS for the entire cohort were 84 months (95% CI: 10-158 months) and 92 months (95% CI: 59-126 months). Univariate analysis revealed significantly poor survival for patients with a metastatic disease, regional nodal metastasis, and those with Mohs micrographic or incomplete surgery.
CONCLUSION
EOSC is a disease of elderly patients with good prognosis. Complete surgery with regional lymph node dissection is standard treatment. The role of adjuvant radiotherapy is debatable but can be considered in patients with incomplete surgery or high-risk factors.
Topics: Adenocarcinoma, Sebaceous; Adolescent; Adult; Aged; Aged, 80 and over; Chemoradiotherapy, Adjuvant; Chemotherapy, Adjuvant; Child; Cohort Studies; Female; Humans; Kaplan-Meier Estimate; Male; Middle Aged; Mohs Surgery; Prognosis; Progression-Free Survival; Radiotherapy, Adjuvant; Sebaceous Gland Neoplasms; Sebaceous Glands; Young Adult
PubMed: 31850516
DOI: 10.1111/ijd.14739 -
The American Journal of Dermatopathology Sep 2020Muir-Torre syndrome (MTS) is a rare autosomal dominant condition characterized by the presence of at least one cutaneous sebaceous tumor and one visceral malignancy,...
Muir-Torre syndrome (MTS) is a rare autosomal dominant condition characterized by the presence of at least one cutaneous sebaceous tumor and one visceral malignancy, arising mostly from the gastrointestinal tract. We present the case of a 63-year-old man with several cutaneous and visceral neoplasias in the context of MTS, and a pelvic lymph node lesion diagnosed initially as metastatic sebaceous carcinoma, but later identified as metastasis from a newly diagnosed prostatic adenocarcinoma. Histological similarities between these 2 lesions are discussed. A systematic literature review was conducted evaluating all published cases of patients with MTS in which metastases were reported. Eighteen articles were included in the final synthesis, representing 20 patients with a total of 26 metastases. Seventeen patients (85%) exhibited metastases originating from MTS-related neoplasms, whereas only 2 patients (11%) exhibited metastases from concomitant malignancies. Of the 85% of patients with metastases from MTS-related malignancies, most originated from noncutaneous sources (78% from visceral neoplasms and 22% from sebaceous carcinomas). When stratifying according to metastases, 23 cases (88%) originated from MTS-related lesions, whereas only 3 (12%) originated from unrelated malignancies. Our findings thus demonstrate that most metastases found in MTS patients (88%) do indeed originate from MTS-related neoplasms. Nevertheless, it remains imperative that a broad differential diagnosis is maintained when assessing a novel lesion, to avoid misdiagnoses, as in the present case, with significant therapeutic and prognostic implications.
Topics: Adenocarcinoma; Adult; Aged; Aged, 80 and over; Biomarkers, Tumor; Biopsy; Clinical Decision-Making; Diagnostic Errors; Female; Humans; Immunohistochemistry; Lymphatic Metastasis; Male; Middle Aged; Muir-Torre Syndrome; Predictive Value of Tests; Prostatic Neoplasms; Sebaceous Gland Neoplasms
PubMed: 32568842
DOI: 10.1097/DAD.0000000000001633