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The article describes the case of a 60-year old male diagnosed with sebaceous carcinoma of the neck. The patient underwent surgical excision to remove the tumor and recovered well. He was also observed with expansion of the underlying sternocleidomastoic muscle and the subcutaneous adipose tissue into the substrate. The clinical manifestations of sebaceous carcinoma and its differential diagnosis are indicated.

Patients with Muir-Torre syndrome may have a systemic malignancy and a sebaceous neoplasm such as an adenoma, epithelioma, and/or carcinoma. The syndrome usually results from a germline mutation in one or more mismatch repair genes. Iatrogenic or acquired immunosuppression can promote the appearance of sebaceous tumors, either as an isolated event or as a feature of Muir-Torre syndrome and may unmask individuals genetically predisposed to the syndrome. Two iatrogenically immunosuppressed men with Muir-Torre syndrome features are described. Similar to these immunocompromised men, Muir-Torre syndromeassociated sebaceous neoplasms have occurred in solid organ transplant recipients, human immunodeficiency virus-infected individuals, and patients with chronic diseases who are treated with immunosuppressive agents. Muir-Torre syndromeassociated sebaceous neoplasms occur more frequently and earlier in kidney recipients, who are receiving more post-transplant immunosuppressive agents, than in liver recipients. The development of sebaceous neoplasms is decreased by replacing cyclosporine or tacrolimus with sirolimus or everolimus. Specific anti-cancer vaccines or checkpoint blockade immunotherapy may merit exploration for immune-interception of Muir-Torre syndrome-associated sebaceous neoplasms and syndrome-related visceral cancers. We suggest germline testing for genomic aberrations of mismatch repair genes should routinely be performed in all patients--both immunocompetent and immunosuppressed--who develop a Muir-Torre syndrome-associated sebaceous neoplasm. [ABSTRACT FROM AUTHOR]

Aim: To evaluate and compare the pathological outcomes, demographic and clinical features of benign and malignant tumors of the eyelid. Materials and Methods: The records of patients who were operated on for eyelid tumors in the oculoplastic surgery unit of our clinic were retrospectively reviewed. The age and sex of the patient, the location and laterality of the tumor, the histopathological diagnosis of the lesion, and whether it was benign or malignant were recorded. Results: A total of 251 patients who were operated on for eyelid tumors and underwent histopathological examination were included in the study. Histopathological results showed benign eyelid tumors in 230 patients (91.6%) and malignant eyelid tumors in 21 patients (8.4%). The age of the patients in the malignant eyelid tumors group was significantly higher than in the benign eyelid tumors group (p<0.001). The localization rate on the right side was significantly higher in malignant eyelid tumors compared to benign eyelid tumors (p=0.003). The lower eyelid localization rate was significantly higher in malignant eyelid tumors than benign eyelid tumors (p<0.001). Conclusion: Although the majority of eyelid tumors are benign, the pathological results of the patients are extremely important so that malignant masses that appear benign are not overlooked. It should be kept in mind that these tumors have a high probability of malignancy, especially in advanced age and lower eyelid locations, and may lead to serious morbidity if treated late. [ABSTRACT FROM AUTHOR]

Simple Summary: Sentinel lymph nodes are the first regional lymph nodes to receive lymph fluid from the primary cancer, sentinel lymph node biopsy (SLNB) can detect occult nodal metastasis. In addition, it may also have a therapeutic effect via regional disease control. SLNB was first introduced in melanoma among skin cancers and is currently used in many types of skin cancers. Previous randomized clinical trials suggested a prognostic benefit from SLNB in melanoma patients. However, whether SLNB affects the prognosis of patients with nonmelanoma skin cancer remains largely unknown, although SLNB may provide important information about nodal status. Since SLNB may be associated with adverse events including infection, lymphedema, and nerve injury, routine application of SLNB for all patients with skin cancer is not recommended. In this review, we summarize the evidence available in the literature regarding the role of SLNB in skin cancer. The sentinel lymph node is the first lymph node from the primary tumor. Sentinel lymph node biopsy (SLNB) is a surgical procedure that can detect occult nodal metastasis with relatively low morbidity. It may also have a therapeutic effect via regional disease control. The Multicenter Selective Lymphadenectomy-I (MSLT-I) trial revealed a prognostic benefit from SLNB in melanoma patients. However, it remains unclear whether there is a prognostic benefit from SLNB in patients with nonmelanoma skin cancer owing to a lack of randomized prospective studies. Nevertheless, SLNB provides important information about nodal status, which is one of the strongest factors to predict prognosis and may guide additional nodal treatment. Currently, SLNB is widely used in the management of not only patients with melanoma but also those with nonmelanoma skin cancer. However, the utilization and outcomes of SLNB differ among skin cancers. In addition, SLNB is not recommended for routine use in all patients with skin cancer. In this review, we provide a summary of the role of SLNB and of the indications for SLNB in each skin cancer based on previously published articles. [ABSTRACT FROM AUTHOR]

Sebaceous carcinoma is a rare and invasive tumor that originates from the Zeis and Meibomian glands around the eyes as well as in sebaceous glands in other head and neck areas and less commonly on the trunk. We report an 82-year-old Iranian man with a sebaceous carcinoma on the left external ear that was successfully treated with radiotherapy. [ABSTRACT FROM AUTHOR]

Sialoblastoma is an extremely rare embryonal tumor derived from salivary gland primordial cells. Treatment usually consists of surgery alone; however, in some cases, chemotherapy is required and is administered with good response. We present a case of a 5-week-old girl diagnosed with a parotid gland tumor and co-existing nevus sebaceous on the face. Initial tumorectomy was microscopically non-radical and histopathology revealed sialoblastoma. The patient received adjuvant chemotherapy consisting of vincristine, actinomycin, and cyclophosphamide. Due to imaging studies being inconclusive regarding response and possible residual disease, a second surgery (total parotidectomy) was performed. The histopathology results showed fields of necrosis in the parotid gland but no neoplastic cells in the material. The patient remains under watchful observation and there is no evidence of relapse 12 months after the second surgery. The adjuvant chemotherapy regimen with vincristine, actinomycin, and cyclophosphamide is a viable option of treatment in children with sialoblastoma. [ABSTRACT FROM AUTHOR]

Sebaceous carcinoma is a rare cutaneous malignant tumor, usually occurring on the eyelids, head, neck, and trunk. There have been few reports about sebaceous carcinoma with primary lung cancer, for which optimal therapy has not yet been established. A 70‐year‐old man presented with a mass in the left iliac bone and tumor of the lower left lung. The morphological characteristics of the iliac bone biopsy pathology and immunostaining results showed sebaceous gland differentiation. After systemic examination, we diagnosed a primary lung sebaceous carcinoma with intrapulmonary and bone metastases. PD‐L1 was positive in 1%–24% of tumor cells, and microsatellites were stable. We detected protein kinase B (AKT1) mutations using the Oncomine Dx target test. Palliative radiotherapy (RT) of a total of 45 Gy was provided in 15 fractions to the left iliac region, which resulted in a 25% reduction in the tumor size. Subsequently, four courses of first‐line pembrolizumab led to a 30% reduction in the total tumor count. RT and pembrolizumab may be treatment options for certain rare primary sebaceous carcinomas of the lungs. A synergistic effect from RT and subsequent administration of immune checkpoint inhibitors may have contributed to tumor reduction. [ABSTRACT FROM AUTHOR]

Background: Cutaneous adnexal tumours (CATs) are one of the commonest clinical presentations in dermatology outpatients. They constitute a subset of skin tumours with a common clinical presentation and variable histological findings. Almost all of them clinically present as a subcutaneous nodule. Depending upon the site and distribution, the clinician can suspect the diagnosis. However, histopathological examination is the gold standard for the definitive diagnosis and proper subtyping of CATs. Aims: The present study is conducted to see the overall spectrum, incidence and distribution of CATs in a large cohort at the tertiary care centre with their clinical profile. Methods: This was a retrospective study in which all the CATs diagnosed over a period of 5 years (2015 to 2019) in a tertiary care hospital were studied. Clinical data were recorded from the histopathology requisition forms. Results: Three hundred and ninety-five cases of CATs were retrieved. Approximately 90% of cases were benign and 10% were malignant. The age of presentation ranged from 8 months to 81 years with male preponderance in all the histological subtypes. The most common site was the head and neck followed by the extremities. Morphologically, the maximum cases showed a differentiation towards sweat glands (44%), followed by sebaceous (29%), follicular (26.5%) and apocrine differentiation (5.3%). Malignant tumours were common in the elderly age group with sebaceous carcinoma being the commonest. Conclusion: CATs comprise of a wide spectrum of tumours occurring in all age groups. Malignant CATs are rare and common in older age groups. Histopathological examination is the gold standard in distinguishing between the different subtypes. [ABSTRACT FROM AUTHOR]

Background: To investigate the correlation between the clinical and pathological characteristics and outcomes in patients with eyelid malignant tumors underwent wide local excision.Methods: This retrospective study included 141 cases of eyelid malignant neoplasms from January 2010 to December 2015 in Shanxi eye hospital. Demographic and clinical information were collected. The Kaplan-Meier method was used to calculate survival curves, and the log-rank test method was used to compare survival between groups. Cox proportional regression models were used to calculate the hazard ratios (HR) of total recurrence rate and metastasis rate.Results: Overall, there were 141 eyelid malignant neoplasms cases aged 65.34 ± 9.69 (range, 41-88) years old. The duration time range was from 1 to 828 (61.09 ± 122.21) months. Basal cell carcinoma (BCC) is the most common of all eyelid malignancies, accounting for 84 (59.5%), followed by Sebaceous gland carcinoma (SGC, 41, 29%), Squamous Cell Carcinoma (SCC, 11, 7.8%), Malignant Melanoma (MM, 3, 2.1%)。On cox-regression analysis, pathological classification (HR 1.959; 95% CI 1.012-3.790; p = 0.046) and eyelid tumor surgery history (HR 17.168; 95% CI 1.889-156.011; p = 0.012) were independently associated with recurrence in patients with eyelid malignant neoplasm. Pathological classification (HR 2.177; 95% CI 1.423 -3.331; p < 0.001) was independently associated with metastasis in patients with eyelid malignant neoplasm. Recurrence and metastasis were most likely to occur in 3 years after surgery.Conclusion: Wide local excision is an effective and economical treatment for eyelid malignant neoplasms. The prognosis is mainly related to pathological types, eyelid tumor surgical history and TNM stages. [ABSTRACT FROM AUTHOR]

Purpose: To provide a retrospective analysis of lesions of the caruncle which have been very rarely reported in the literature.Methods: A retrospective review of all the caruncular lesions between January 2000 and January 2020 was done at a single tertiary eye care hospital. The lesions were classified as benign and malignant lesions. Clinicopathological correlation was done for the excised lesions.Results: A total of 87 caruncular lesions were included in the study. Males (59%) were affected more than females (41%). The mean age at presentation was 44 ± 20 years. The mean duration of complaint was 36 ± 62 months. A total of 36 patients underwent surgical excision whereas the rest opted for conservative management. Recurrence was noted in five patients. Fifteen different types of lesions were identified histopathologically. Benign lesions (78%) were far more common than malignant ones (22%). Epithelial inclusion cyst was the most common benign lesion and sebaceous gland carcinoma was the most common malignant lesion. Correct clinicopathological correlation was seen in 52.7% of the cases. Caruncular tuberculosis, oncocytoma, and basosquamous cell carcinoma were some of the rare lesions.Conclusion: Caruncular lesions are uncommon and very diverse, which makes clinical diagnosis challenging. Epithelial inclusion cyst and sebaceous gland carcinoma were the most common benign and malignant lesions respectively. Correct clinicopathological correlation was seen in more than half of the cases. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the effect of direct cell injury of cryotherapy on eyelid sebaceous gland carcinoma cells by an ex vivo cryotherapy experiment.Methods: It was a prospective interventional case series. Six patients with biopsy-proven nodular sebaceous gland carcinoma were included. After excision of the mass, a thin slice of the mass resembling the thickness of the conjunctiva was shaved off and was oriented over the broad end of a tissue forceps. Cryotherapy was applied to both its anterior and posterior aspects by the triple freeze-thaw technique. The mass was then labeled and sent separately for histopathological evaluation by fixation and staining.Results: A total of six patients with a mean age of 58.2 ± 15.5 years were included. There were four females and two males. The mean duration of the lesion was 21.6 ± 17.51 months. All patients had involvement of the upper eyelid. The patients were clinically staged as T2b (n=2), T1a (n=2), T2c (n=1), and T3a (n=1) respectively. There was no regional lymphadenopathy or metastasis in any of the cases. The experimental cryo-tissue containing the cryo-treated lesion revealed the presence of viable tumor cells (>50%) in all six specimens.Conclusion: The direct cell injury caused by cryotherapy may not be sufficient to kill all the residual sebaceous gland carcinoma cells on the tumor bed. [ABSTRACT FROM AUTHOR]

Breed predispositions to canine digital neoplasms are well known. However, there is currently no statistical analysis identifying the least affected breeds. To this end, 2912 canine amputated digits submitted from 2014–2019 to the Laboklin GmbH & Co. KG for routine diagnostics were statistically analyzed. The study population consisted of 155 different breeds (most common: 634 Mongrels, 411 Schnauzers, 197 Labrador Retrievers, 93 Golden Retrievers). Non-neoplastic processes were present in 1246 (43%), tumor-like lesions in 138 (5%), and neoplasms in 1528 cases (52%). Benign tumors (n = 335) were characterized by 217 subungual keratoacanthomas, 36 histiocytomas, 35 plasmacytomas, 16 papillomas, 12 melanocytomas, 9 sebaceous gland tumors, 6 lipomas, and 4 bone tumors. Malignant neoplasms (n = 1193) included 758 squamous cell carcinomas (SCC), 196 malignant melanomas (MM), 76 soft tissue sarcomas, 52 mast cell tumors, 37 non-specified sarcomas, 29 anaplastic neoplasms, 24 carcinomas, 20 bone tumors, and 1 histiocytic sarcoma. Predisposed breeds for SCC included the Schnauzer (log OR = 2.61), Briard (log OR = 1.78), Rottweiler (log OR = 1.54), Poodle (log OR = 1.40), and Dachshund (log OR = 1.30). Jack Russell Terriers (log OR = −2.95) were significantly less affected by SCC than Mongrels. Acral MM were significantly more frequent in Rottweilers (log OR = 1.88) and Labrador Retrievers (log OR = 1.09). In contrast, Dachshunds (log OR = −2.17), Jack Russell Terriers (log OR = −1.88), and Rhodesian Ridgebacks (log OR = −1.88) were rarely affected. This contrasted with the well-known predisposition of Dachshunds and Rhodesian Ridgebacks to oral and cutaneous melanocytic neoplasms. Further studies are needed to explain the underlying reasons for breed predisposition or “resistance” to the development of specific acral tumors and/or other sites. [ABSTRACT FROM AUTHOR]

Sebaceous neoplasms are rare adnexal tumors that can present a challenge to clinicians. Only four cases of sebaceous carcinoma with sebaceoma have been reported in the literature. Herein, we describe the case of a sebaceous carcinoma originating from a sebaceoma in a solitary nodule of the posterior neck. Immunohistochemically, the tumor cells were strongly positive for epithelial membrane antigen and p53. It is possible that adnexal carcinomas may arise from malignant transformation of their benign counterparts as well as de novo. Malignant transformation was likely in this case because the lesion was composed of distinct benign and malignant components, and the benign component showed the typical histopathological features of sebaceoma. This case underscores the fact that partial and superficial biopsies sometimes may not provide the correct diagnosis. If a surgeon suspects malignancy based on a clinical examination, then it is mandatory to perform a deep biopsy. [ABSTRACT FROM AUTHOR]

Fordyce granules of the oral mucosa are often discovered during routine dental examinations. They are considered anatomic variations and are typically seen on the labial and buccal mucosa in adults. The present case report describes for the first time in the literature an atypical location of an enlarged Fordyce granule with local bone destruction. The diagnostic process, surgical treatment, and follow-up are presented and discussed. [ABSTRACT FROM AUTHOR]

Purpose: To analyze data on eyelid malignancy in India, clinical and pathologic features, and outcomes.Methods: A multicentre study, from oculoplastic practices in four geographic zones in India. The centers perform similar documentation and protocol-based management for eyelid tumors. Clinical features, pathology, American Joint Committee on Cancer (AJCC) class, management, and the outcomes were analyzed.Results: The study included 129 patients, with slight female preponderance and mean age 62.7 years. The median delay to the consultation was 9 months. Rural patients traveled a mean distance of 115.2 km; there was no difference between the city and outstation patients in the delay to consultation or follow up. Pathology included 55/129 (42.6%) sebaceous gland carcinoma (SGC), 47/129 (36.4%) basal cell carcinoma (BCC), squamous cell carcinoma (SCC) in 15 (11.6%), and 12 (9.3%) other tumors. Commonest AJCC class was T2b/T3a in 80/111 (72%), invasion of the orbit was present in 16 (12.4%). Surgery with margin clearance was performed in 103. With a mean follow-up of 21.44 months, local recurrence and/or metastasis were seen in 12%. The diagnosis of SGC was strongly associated with adverse outcomes (odds ratio: 7.36). On multiple logistic regression analysis, diagnosis of SGC (P = 0.011) was significant in having adverse outcomes.Conclusion: The multicenter Indian data shows the highest prevalence of SGC, with the commonest AJCC class T2b. Most tumors were locally resectable at presentation. The histopathologic diagnosis of SGC is the factor strongly associated with adverse outcomes. [ABSTRACT FROM AUTHOR]

Context: Eyelid carcinoma is rare tumors of the head and neck. They are rarely lethal but can be associated with significant morbidity if not treated early and appropriately. There are limited data available from world over and in particular the Indian subcontinent regarding eyelid carcinoma and its prognostic factors influencing treatment outcomes. Setting and Design:Retrospective study of patients treated in a tertiary cancer center between 2005 and 2016.Methodology: In this study, 51 patients with eyelid carcinoma treated at single tertiary cancer center were included. The demographic, clinical data, which includes the treatment received, histopathology report and follow-up, were recorded. All the relevant variables influencing disease-free survival (DFS) were analyzed.Results: Sebaceous carcinoma was the most common eyelid carcinoma followed by squamous cell carcinoma and basal cell carcinoma in descending order in this series. Lower eyelid was involved most often. The incidence of nodal metastasis was low (14%). Multivariate analysis revealed that margin status influenced the DFS (P= 0.001) (hazard ratios = 15.9 [95% confidence interval: 1.8-135.2]). The 5 years' DFS was 70%.Conclusion: Eyelid tumors are less common cancer with good prognosis if treated appropriately. The morbidity associated with treatment can be reduced if treated early. [ABSTRACT FROM AUTHOR]

Sebaceous carcinoma of the breast is an extremely rare tumor. To the best of our knowledge only 15 cases have been reported in literature. Herein, we report a case of sebaceous carcinoma of the breast and describe the histopathologic features of sebaceous carcinoma of the breast in an elderly female. [ABSTRACT FROM AUTHOR]

Primary sebaceous carcinoma of the breast is an exceedingly uncommon neoplasm that is defined as primary invasive carcinoma of the breast with sebaceous differentiation in more than 50% of cells. All confirmed cases have been reported in women. Herein, we report the case of a 70‐year‐old man with a primary sebaceous breast carcinoma, the first unequivocal case to be reported thus far in a man. [ABSTRACT FROM AUTHOR]

Purpose. In this study, we aimed to investigate the presence of Epstein–Barr virus (EBV) in the eyelid sebaceous gland carcinoma (SGC) and its association with the clinicopathologic features. Methods. One hundred and thirty paraffin-embedded SGC specimens were retrieved from the Clinical Pathology Department of Zhongshan Ophthalmic Center. Epstein–Barr virus-encoded RNA (EBER) was detected with in situ hybridization (ISH) using the Leica BOND system autostainer. The age and gender distributions of all patients were analyzed and compared with earlier reports. Pearson's χ2 and Fisher's exact tests were used to determine the association between clinicopathological features such as age, gender, laterality eye, tumor basal dimension, degree of tumor differentiation, and EBER positivity. Likewise, the relationship between the grade and tumor basal dimension in EBER-positive SGC of the eyelid was analyzed. Results. Thirty-four out of one hundred and thirty (26.2%) eyelid SGC specimens were positively stained for EBER. The age range of highest incidence was 46–75 years, and the female to male ratio was 1 : 0.9. No significant correlation was found between EBER-positivity and age (p = 0.5370), gender (p = 0.4758), and degree of tumor differentiation (p = 0.7787). However, EBV positivity was strongly correlated with the right eye (p = 0.0287), the tumor basal dimension (p = 0.0001). EBV positivity grade presented statistically associated with tumor size (p = 0.0329). Conclusion. We conclude that ISH is a sensitive method to identify EBV in SGC of the eyelid. A possible causal association of EBV in SGC patients is suggested by high frequency of EBER-ISH positivity and its association with the clinicopathologic features. [ABSTRACT FROM AUTHOR]

Sebaceous carcinoma (SC) is an aggressive skin tumor. Although ultraviolet radiation (UVR) is an important risk factor for some skin cancer types, no population-level study has evaluated for an association between UVR and SC risk. Herein, we examined satellite-based ambient UVR in relation to SC incidence using Surveillance, Epidemiology, and End Results 18 cancer registry data (2000–2016). There were 3503 microscopically confirmed cases of SC diagnosed during the study period. For non-Hispanic whites, there was an association between increasing ambient UVR and SC risk (incidence rate ratio [per UVR quartile] = 1.15, 95% confidence interval = 1.11 to 1.19; two-sided P  <  .001) including among individuals with and without putative Muir-Torre syndrome. In contrast, there was no association between ambient UVR and SC risk for other race and ethnicities. Our findings support a role for UVR in SC tumorigenesis, which suggests that photoprotection may reduce SC risk, particularly for high-risk populations (eg, Muir-Torre syndrome). [ABSTRACT FROM AUTHOR]

Background: The purpose was to describe the Nordic treatment practices and to reach a Nordic consensus for the treatment of sebaceous eyelid carcinoma.Methods: The treatment practices data was collected by a questionnaire with 37 questions to the Nordic oculoplastic surgeons and analyzed. A PubMed MEDLINE database search was done to gather data on the published treatment practices and recommendations. A working group that consisted of in minimum one senior consultant from each leading Nordic University Eye Hospital was assigned. A structured interactive method was used to establish the consensus.Results: Twenty-four doctors responded to the questionnaire. 23/24 (96%) of the respondents took a biopsy before surgery. Regional lymph node scanning was routinely done by 14/23 (61%) and a systemic screening of a metastatic disease by 13/23 (57%). 6/22 (27%) never took conjunctival mapping biopsies and 12/23 (52%) never screened for Muir- Torre. Respondents used Mohs surgery, frozen section or multi-stage excision with delayed closure, and 5-6 mm was the mostly preferred margin. Sentinel lymph node biopsy was a possible option for 9/22 (41%) and cryotherapy and Mitomycin C for 6/22 (27%) respondents. 50% of respondents considered radiation as a treatment option. 15/16 (94%) respondents always followed-up their patients, most for 5 years. Two thirds scanned regional lymph nodes during the follow-up. Consensus was reached for 18 statements representing three domains: preoperative work-up, treatment and follow-up.Conclusion: Treatment practices differ in between the five Nordic countries which have similar public health care systems. In the article the authors present a Nordic consensus for the treatment of eyelid sebaceous carcinoma. [ABSTRACT FROM AUTHOR]

To shed more light on the pathogenesis of sebaceous carcinoma, we analysed the expression of proteins related to angiogenesis in 18 ocular and 22 extraocular sebaceous carcinomas using a broad panel of immunohistochemical markers. To quantify the expression of D2-40, vascular endothelial growth factor, vascular endothelial growth factor receptor-2 and -3, we calculated a quantification score by considering the percentage of positive tumour cells (0=0%, 1=up to 1%, 2=2-10%, 3=11-50%, and 4=>50%) in relation to the staining intensity (0=negative, 1=low, 2=medium, and 3=strong). Additionally, lymphatic microvessel density in the D2-40 stained sections was counted. Vascular endothelial growth factor receptor-3 (quantification score 9.42 ± 2.94) was significantly more strongly expressed than vascular endothelial growth factor receptor-2 (quantification score 2.15 ± 2.42, p < 0.001). Furthermore, epidermal vascular endothelial growth factor expression was negatively correlated with the intratumoural lymphatic vessel density, and the ratio of small lymphatics to large lymphatics was much higher in intratumoural tissue than in paratumoural tissue and in intraindividual control tissue, suggesting a lymphangiogenetic potential of sebaceous carcinoma. [ABSTRACT FROM AUTHOR]

Background: McCune Albright syndrome (MAS) is a rare heterogeneous clinical syndrome without any predilection for ethnic group. Classic form includes triad of fibrous dysplasia, caf' au late spots and autonomous hyper function of one or more endocrine pathways. Case Report: We report the case of an 18-year old girl with non-classic form of MAS .New aspect of this case report attributed to multiple sebaceous adenoma. Conclusion: The new finding of our case of MAS was not reported before. Periodic followup with different radiologic and laboratory tests should be considered after suspicion to MAS. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate prognostic factors and survival of patients with sebaceous carcinoma of the eyelid through a population-based analysis. Methods: A total of 940 patients with primary sebaceous carcinoma of the eyelid were derived from the Surveillance, Epidemiology, and End Results (SEER) database in the United States from 1973 to 2013. Kaplan–Meier univariate analysis and Cox Regression multivariate analysis were performed to examine prognostic factors in overall survival (OS). 5- and 10-year survival rates, median survival, and prognostic variables with statistical significance were measured. Results: Kaplan-Meier analysis showed that OS is 66% and 44% at 5 years and 10 years respectively. Median OS is 9.4 years. Multivariate Cox regression analysis demonstrated that independent prognostic factors for OS are age at diagnosis (HR = 4.61 [95% CI 1.93–11.0], P = 0.001), surgical treatment (HR = 0.196 [95% CI 0.07–0.55], P = 0.002), combined surgical and radiation treatment (HR = 0.227 [95% CI 0.06–0.81], P = 0.023), and greater tumor size at diagnosis (HR = 3.381, [95% CI 1.77–6.45], P < 0.001). Conclusion: We report the largest population study to date to evaluate prognostic factors of patients with sebaceous carcinoma of the eyelid. Multivariate analysis shows that older age, and greater tumor size correlate with decreased overall survival, whereas surgical treatment or combined surgical and radiation treatment correlate with increased overall survival. Interestingly, tumor grade, lymph node involvement, and distant extent of tumor have not demonstrated to be independent prognostic factors for overall survival. [ABSTRACT FROM AUTHOR]

A 61-year-old man presented with a dome-shaped tumor in the right eyelid. Histology revealed a cutaneous neoplasm with lobular growth pattern. It was composed of large basaloid cells, cells with sebaceous differentiation, and glandular structures with apocrine features. The immunohistochemical study revealed diffuse expression of CK7, EMA, and androgen receptors in the neoplastic cells. Gross cystic disease fluid protein-15 highlighted the luminal cells, consistent with apocrine differentiation. These findings fit with a sebaceous carcinoma with focal glandular apocrine differentiation ("sebo-apocrine" carcinoma). Metastasis to regional lymph nodes were diagnosed 12 months after the surgical procedure. After 3 years of follow-up, the patient is alive, with evidence of persistent locoregional metastasis, but no evidence of distant metastasis. Most cutaneous adnexal tumors present only one cell type of differentiation, but hybrid/combined tumors can occur. Despite "sebo-apocrine" tumors are not unexpected, due to their common origin in the follicularsebaceous apocrine unit, they are very rarely reported. [ABSTRACT FROM AUTHOR]

An external mass was observed on the ear of a free-ranging Baird's Tapir Tapirus bairdii. The mass was surgically removed under general anesthesia and was histopathologically identified as sebaceous gland adenoma. Hematological and biochemical analyses were also performed. The animal showed a mild anemia and the other values were unremarkable. Only few cases of tumors have been reported in tapirs and this is the first report of a tumor in a free ranging Baird's Tapir. The presence of any disease in free-ranging wildlife should call our attention in order to develop a better understanding of disease ecology, especially in threatened species. [ABSTRACT FROM AUTHOR]

Introduction: Mature sebaceous glands do not contain Merkel cells. Neither do sebaceous tumors in the vast majority of cases. Recently, however, it was showed that rare sebaceous adenomas with a carcinoid‐like or labyrinthine pattern can contain intratumoral Merkel cells. Our purpose in this study is to examine for the presence of Merkel cells in extraocular sebaceous carcinomas. Methods: Nineteen cases of extraocular sebaceous carcinoma were retrospectively studied with cytokeratin 20. Results: One out of 19 cases (5.3%) contained Merkel cells in the basal layers of the tumoral nests of a sebaceous carcinoma from the cheek of a 76‐year‐old woman. Conclusions: Merkel cells are rarely evident in extraocular sebaceous carcinoma, and therefore, their evidence does not exclude this diagnosis. [ABSTRACT FROM AUTHOR]

Hepatoid gland adenocarcinoma are malignant tumors seen in the perianal region in dogs, mostly affecting sexually intact male dogs between 7 and 14 years of age. The surgical removal of such a tumour was performed in a male dog of eight-year age under general anaesthesia. The histopathological findings revealed cells with moderate degree of anisocytosis and anisokaryosis and multifocal infiltration of inflammatory cells predominantly neutrophils. Based on histo-pathological observation the mass was diagnosed as hepatoid gland adenocarcinoma. Complete removal of the tumour mass and appropriate wound care lead to an uneventful recovery. [ABSTRACT FROM AUTHOR]

Aims: Sebaceous carcinomas are uncommon malignant cutaneous tumours originating from the pilosebaceous unit. Although its occurrence is mostly common in peri‐ocular glands, other anatomical regions of the head and neck may be affected, including major and minor salivary glands. Methods and results: We describe a series of sebaceous adenocarcinomas of the parotid and submandibular glands. The mean age was 62.1 (range = 31–90) years. Two patients (20%) presented regional or distant metastasis to mandible and lungs. All cases were positive for cytokeratins (AE1AE3 and CK‐5), epithelial membrane antigen and adipophilin and negative for androgen receptor, Factor XIIIa, S‐100, vimentin and perforin. MLH1 and MSH2 were expressed in the nuclei of most tumour cells, and one case showed loss of MSH2 expression. Proliferative index (assessed by Ki‐67 expression) and microvessel density (CD34‐positive vessels) were higher in metastasis‐associated cases. P63 expression was noted in the periphery of the tumour nests, in the basaloid cells, with a mean of 69.2% nuclear positivity. Conclusions: The sebaceous adenocarcinoma of salivary glands is rare and may show an unfavourable outcome; therefore, its correct diagnosis may be challenging. For this reason, immunohistochemical studies, including adipophilin in particular, constitute an important diagnostic tool. [ABSTRACT FROM AUTHOR]

Purpose: Comparison of outcomes of localized eyelid sebaceous carcinoma (American Joint Committee on Cancer (AJCC) 8th edition categories T1, T2, T3) following initial management with posterior lamellar resection (PLR) versus (vs) lumpectomy (nonposterior lamellar resection, non-PLR).Methods: This was a retrospective, comparative, interventional case series. Of 54 patients, 26 were managed by PLR and 28 by nonPLR. The main outcome measures were vision loss, orbital exenteration, lymph node metastasis, and remote metastasis were the main outcome measures.Results: A comparison of PLR vs nonPLR revealed no difference in mean age at presentation (70 vs 72 years), race (Caucasian 100% vs nonCaucasian 93%), gender (female 54% vs male 71%), entering mean visual acuity (20/30 vs 20/50), or corneal involvement (23% vs 11%). There was significant difference in clinical features with PLR demonstrating greater mean basal diameter (21 mm vs 12 mm, P = 0.004), and positive margins were seen more frequently in PLR (69% vs 46%). There was significantly less number of surgical procedures in PLR to achieve complete tumor control (1.7 vs 2.5, P = 0.001). Outcomes at mean 55 months follow-up revealed significantly better control with PLR (vs nonPLR) with fewer orbital exenterations (15% vs 43%, P = 0.038), fewer lymph node, and systemic metastases (0% vs 39%, P < 0.001). There was no significant difference in final mean visual acuity (20/60 vs 20/200).Conclusion: For eyelid sebaceous carcinoma, the initial management is critical to the patient's outcome. PLR demonstrates superior outcomes with regard to preservation of visual acuity and avoidance of exenterations, lymph node metastases, and systemic metastases compared to patients managed with other techniques. [ABSTRACT FROM AUTHOR]

Ocular sebaceous carcinoma (SC) is an exceedingly rare but aggressive malignancy that can impair patients' visual acuity or even cause tumor-specific mortality. This tumor usually mimics chronic benign eyelid lesions, thus leading to delayed diagnosis, thereby causing high recurrence and metastasis. Ophthalmologists should be more aware of SC in order to offer correct diagnosis and treatment at the onset of symptoms. Prompt surgical excision with frozen section margin control is the mainstay of SC management after patient evaluation and accurate biopsy. Mohs micrographic surgery has been associated with better tumor control outcomes than wide local excision. Radiation therapy should be considered as adjuvant treatment for locally advanced (stage T3a or higher) or high-risk (pagetoid spread) SC, nodal metastasis, or palliative care. Cryotherapy and topical chemotherapy are used for pagetoid spread. Targeted therapy has an emerging role in more complicated cases. For lymph node and distant metastasis, combination treatments should be provided, including lymph node and neck dissection, radiation therapy, systemic chemotherapy, and even orbital exenteration. The rarity of ocular SC precludes a comprehensive perspective on standard treatment. This paper offers a brief review of recent advances in the clinical diagnosis and management of ocular SC based on current scientific literature. [ABSTRACT FROM AUTHOR]

A rare case of cerebriform nevus sebaceous of Jadassohn is being reported in Images of Surgery. [ABSTRACT FROM AUTHOR]

Purpose: Overexpression of the inhibitors of apoptosis proteins have been demonstrated in a variety and of solid tumors including melanomas and nonmelanomas skin cancers. X-linked inhibitor of apoptosis protein (XIAP) is an inhibitor of apoptosis which prevents apoptosis by inhibiting caspases 9, 7, and 3. The prognostic value of XIAP in sebaceous gland carcinoma (SGC) remains unexplored.Methods: The immunohistochemical expression of XIAP was evaluated in 29 SGC cases.Results: The cytoplasmic overexpression of XIAP was detected in 62% SGC cases. XIAP expression was found to be significantly associated with advanced age, large tumor size, and with reduced disease-free survival (P = 0.0174). XIAP expression and advance tumor Grade III emerged as significant risk factors on univariate analysis. On stepwise multivariate analysis, both increased cytoplasmic XIAP expression and high tumor grade were found to be significantly associated with recurrence. Patients with low XIAP immunoexpression had a longer disease-specific survival than those with high expression in the 5-year follow-up.Conclusion: The present study demonstrates at the immunohistochemical level that XIAP is overexpressed in SGC and that high expression could be of biological significance in the development of eyelid SGC. Our finding suggests that up-regulation of XIAP may aggravate tumor metastasis in SGC. [ABSTRACT FROM AUTHOR]

Background Though a rare tumor, sebaceous carcinoma is relatively well-described in immunocompetent patients, in whom it often occurs in a periorbital distribution where it has an overall poor prognosis with a high metastasis rate. The effect of transplant-related immunosuppression on the development of sebaceous carcinoma and its outcomes has not been characterized. Methods We collected 9 cases from a single institution of patients developing sebaceous carcinoma after solid organ transplantation. We analyzed clinicopathologic features. Results We estimate the prevalence of sebaceous carcinoma post-solid organ transplantation to be 0.09%. The mean age at diagnosis was 66.1 years (std 7.0 years). The mean time between transplantation and sebaceous carcinoma diagnosis was 7.1 years (std 5.1 years). All tumors occurred in extra-ocular distribution. Two patients likely had Muir-Torre syndrome, of whom 1 died from metastatic sebaceous carcinoma. No other patients developed metastatic disease or had disease-related death. Mohs micrographic surgery and wide local excision were equally effective and there were no recurrences with either procedure. Conclusions Our study found that sebaceous carcinoma in solid organ transplant recipients occurs in in an extraorbital distribution with only 1 patient developing metastatic disease. Both Mohs micrographic surgery and wide local excision are acceptable treatment modalities for sebaceous carcinoma in transplant recipients. [ABSTRACT FROM AUTHOR]

The increasingly successful and widespread use of Tumor Necrosis Factor inhibitors ( TNFi) to treat autoimmune and inflammatory conditions has also been accompanied by adverse reactions, both systemic and cutaneous. Psoriasiform cutaneous rashes are well described. Recently, TNF inhibitor associated psoriatic alopecia ( TiAPA) is being more frequently reported. The purpose of this study is to describe the features of TiAPA, including marked atrophy of sebaceous lobules as a histologic clue to diagnosis, helping to distinguish it from other types of alopecia. Clinical and histopathological features of 3 patients who developed scalp alopecia while on TNFi treatment were examined. Clinical follow up was conducted after discontinuation of TNFi. A review of the previous literature on the subject was also conducted. Atrophy of sebaceous lobules is a potentially reversible, characteristic and conspicuous feature of TiAPA that can be distinguished from idiopathic psoriatic alopecia by clinical history of drug exposure and sometimes by histologic presence of a mixed inflammatory response including plasma cells and eosinophils. [ABSTRACT FROM AUTHOR]

Objective: We describe the case of a patient with metastatic breast cancer who presented with eyelid margin thickening and madarosis more suggestive of sebaceous cell carcinoma than metastatic disease. Histopathology confirmed metastatic breast adenocarcinoma. Case Report: A 59-year-old woman with a known history of metastatic breast carcinoma actively enrolled in a clinical trial presented with a thickened right upper eyelid margin with madarosis and without ulceration. Although the possibility of metastasis was considered, a biopsy was performed to ensure the patient did not have a primary eyelid malignancy such as sebaceous cell carcinoma given her immunocompromised state. Histopathology revealed metastatic breast adenocarcinoma. Conclusions: To the authors' best knowledge, metastatic breast carcinoma presenting as eyelid margin thickening without ulceration has not previously been reported. Eyelid metastasis is rare, and this patient's clinical presentation was found to be unusual as well. It is important to establish metastatic disease even in a small focus such as the eyelid, as it may alter disease management. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented that discusses the medical condition of multifocal sebaceous carcinoma of the vulva.

Objective: Eyelid carcinomas are rare, and the management strategy of regional lymph node metastasis linked to eyelid carcinomas has not been standardized to date. The aim of the present study was to analyze the patterns of regional metastasis and to assess the optimal extent of surgical treatment for lymph node metastasis of eyelid carcinoma.Methods: This study was a retrospective review of patient data from a single institution. From a series of 268 eyelid carcinomas, we selected the 21 patients with lymph node metastasis, and we analyzed the patterns of lymphatic spread, approach to treatment and outcomes.Results: The most common histological type of eyelid carcinoma with regional metastasis was sebaceous carcinoma (17/21, 81.0%). Submandibular area metastases were seen only in the patients with the primary tumor originating in the medial half of the eyelid, but parotid area metastases were seen in both the patients whose tumors had a medial-half origin and those with a lateral-half origin. Although 11 of the 16 patients with parotid-area metastases underwent a tumorectomy or superficial parotidectomy (which resulted in four cases of recurrence in the parotid area), none of the five patients who underwent a total parotidectomy developed parotid-area recurrence. The incidence of regional recurrence of the patients who received adjuvant radiotherapy (14.3%) was lower than that of the patients without adjuvant radiotherapy (57.1%).Conclusion: Continued surveillance and optimal management of regional lymph node metastases are important for the control and survival of eyelid carcinomas. [ABSTRACT FROM AUTHOR]

Muir-Torre syndrome (MTS) is a rare autosomal dominant inherited genodermatosis that is considered to be a phenotypic subtype of hereditary nonpolyposis colorectal cancer (HNPCC), commonly referred to as Lynch syndrome. We describe the clinical course of a 57-year-old female patient with MTS. She has a confirmed HMSH2 mutation. Recently she presented with two nodular lesions. Histologic examination confirmed these lesions to be sebaceous neoplasms. The patient has a history of endometrial and colorectal adenocarcinoma as well as several nonspecific sebaceous lesions throughout her life. She has a confirmed extensive family history of MTS with both male and female family members harbouring either HMLH1 or HSMH2 mutations. Affected relatives have presented at different ages throughout their lives with cutaneous neoplasms and visceral malignancies, including malignancies rarely associated with MTS. MTS presents a diagnostic challenge for clinicians. The case demonstrates that the management of MTS, a potentially underreported syndrome, requires a multiprofessional approach incorporating vigilance, screening, and expert knowledge for successful diagnosis and potentially improved prognosis for patients and their families. The case also demonstrates the varied heritability of MTS and prompts the question of how MTS is expressed in succeeding generations. [ABSTRACT FROM AUTHOR]

Purpose: To report a successful eyelid reconstruction performed using the residual tarsus after excision of a sebaceous gland carcinoma. Case Report: An 86-year-old woman presented with a sebaceous gland carcinoma of the upper eyelid margin. After excision of the tumor, she underwent reconstructive surgery of the superior eyelid performed using the residual tarsus. Because the tumor was localized on the upper lid margin, a tarsus of about 4-5 mm in height remained after the excision; we therefore used the residual tarsus to reconstruct the upper eyelid. No functional or cosmetic problems arose as a result of this method. Conclusions: Reconstructive surgery of the upper eyelid using the residual tarsus may be a viable option provided that surgeons can ensure a sufficient safety margin, and that more than 4 mm of the tarsus remains. [ABSTRACT FROM AUTHOR]

Sporadic sebaceous gland hyperplasia (SGH) is a benign skin lesion, with a high prevalence in the general population. Although SGH has been attributed to both extrinsic and intrinsic factors, the underlying genetic changes have not yet been characterized. Recently, HRAS and KRAS mutations have been identified in sebaceous naevus, a hamartoma sharing histological characteristics with SGH. Therefore we screened 43 SGH for activating mutations in RAS genes and other oncogenes. We identified a wide spectrum of mutually exclusive activating HRAS (8/43), KRAS (11/43) and EGFR mutations (7/31) in altogether 60% of the lesions investigated. A RAS and EGFR wildtype status was found in 15 normal sebaceous glands in the head and neck area. Our findings indicate that activating HRAS, KRAS and EGFR mutations play a major role in the pathogenesis of sporadic SGH. These results support the concept that SGH is a true benign neoplasm rather than a reactive hyperplasia. [ABSTRACT FROM AUTHOR]

The article presents a study which examines the use of electrochemotherapy as possible new treatment modality for metastatic extraorbital sebaceous carcinoma (SC). A systematic literature review for case reports of extra-ocular, cutaneous, multifocal metastasizing SC conducted between 1960 and 2015 is offered. Results of the extensive literature search highlight the objective response of the tumour to ECT as well as good quality of life of patients.

Background: Sebaceous carcinoma is a rare malignancy primarily with aggressive growth affecting the cutaneous tissues of the periocular region. Sebaceous carcinoma of the parotid gland is exceedingly rare, with only 32 cases reported in the literature. Our case brings this total to 33.Case Presentation: We present a case of a 57-year-old Moroccan woman with a firm, painless, slowly enlarging swelling at her left parotid area, with normal overlying skin and no palpable neck nodes. Parotidectomy with facial nerve preservation was performed, and microscopic examination showed sebaceous carcinoma. Then, she underwent adjuvant radiotherapy. With a follow up of 20 months, head and neck computed tomography revealed no recurrence.Conclusions: The optimal treatment is unclear. With more cases reported, clinicopathological characteristics and histogenesis are increasingly understood. Therefore the treatment for this rare tumor continues to evolve. [ABSTRACT FROM AUTHOR]

A 72-year-old male visited a nearby hospital with a large tumor in his occipital region, which had existed since 20 years. Since malignant tumor was suspected, he was referred to our department. At the initial consultation, an elastic-hard, yellow-brown, sessile tumor, measuring 8 x 7 x 5 cm and with a flat surface, was observed in the occipital region. The tumor was resected and covered with artificial dermis. Histopathologically, the lesion was composed of basal-cell-like cells with nest formation in the dermis. A rippled pattern, or the single-line arrangement of tumor cells involving the stroma, was present. In addition, some tumor clusters revealed the differentiation to sebaceous glands, and these cells were positive for cytokeratin (AE1/AE3) and epithelial membrane antigen, which is consistent with the staining of sebaceous glands. On the contrary, tumor cells were negative for epithelial antigen (Ber-EP4), and Ki67 (MIB1) index was 5% or lower. Therefore, we diagnosed the tumor as rippledpattern sebaceoma and not as basal cell carcinoma. Although this case was quite unique in its large size, immunostaining was useful for the definite diagnosis. [ABSTRACT FROM AUTHOR]

Background: Sebaceous glands contribute significantly to the barrier functions of the skin. However, little is known about their homeostasis and tumorigenesis. Recently, increased expression of stem cell marker Lrig1 has been reported in sebaceous carcinoma-like tumors of K14?NLef1 transgenic mice. In this study, we analyzed the Lrig1 expression in human sebaceous tumors. Methods: Twenty-eight formalin-fixed paraffin-embedded sebaceous tumor specimens (7 sebaceous hyperplasias, 7 sebaceous adenomas, 10 sebaceomas and 4 sebaceous carcinomas) were stained with anti-Lrig1, anti-CD44v3 and anti-Ki67 antibody. Results: Four (100%) sebaceous carcinomas, 8 (80%) sebaceomas, 3 (43%) sebaceous adenomas and no sebaceous hyperplasia showed Lrig1 overexpression. Discussion and Conclusion: Lrig1 is a known tumor suppressor gene and is usually considered to be an indicator of poorly aggressive tumors. In human sebaceous tumors, the stronger Lrig1 staining in sebaceous carcinoma compared to other sebaceous tumors might be a feature of an advanced stage in tumorigenesis and a bad prognosis. In our study, 100% of sebaceous carcinomas revealed Lrig1 overexpression. We propose that Lrig1 may be used as a possible new marker of poorly differentiated sebaceous carcinoma. [ABSTRACT FROM AUTHOR]

Although the histogenesis of sebaceous carcinomas remains unclear, the occurrence of intraepidermal or intraepithelial sebaceous carcinoma in the epidermis or conjunctiva may suggest de novo histogenesis. This report describes a case of sebaceous carcinoma within preexisting rippled/carcinoid pattern sebaceoma. This lesion was composed of two (benign and malignant) components, and the benign component of the lesion showed the typical features of a rippled/carcinoid pattern sebaceoma. Although evidence of trauma as well as a vertical orientation was seen in this lesion, the malignant component of the lesion showed histopathological evidence of malignancy (sebaceous carcinoma), such as the aggregations with irregular and infiltrated borders, a sheet-like growth pattern, and the cytopathological findings of the neoplastic cells, showing a high-grade of malignancy (a high mitotic index and abnormal mitotic figures). The immunohistochemical staining for p53, Ki-67 and D2-40 also favored this diagnosis. This sebaceous carcinoma component was considered to be the incipient stage of carcinoma within preexisting sebaceoma, therefore, it was still considered to be a vertically oriented lesion. This case shows the possibility that abnormal (malignant) sebaceous germinative cells may originate within a sebaceoma, thereby suggesting that some sebaceous carcinomas may develop from preexisting sebaceomas. [ABSTRACT FROM AUTHOR]

Introduction: Sebaceous carcinoma is a very rare malignant tumor primarily found in the area of the eyelid. We have reported here a case of sebaceous carcinoma of the eyelid with neck node metastasis diagnosed on fine needle aspiration cytology with emphasis on the unique cytomorphology of this tumor. Case history: A 35 year old woman presented with complaints of swelling in left upper eyelid, proptosis, loss of vision and cervical node enlargement. Fine needle aspiration (FNA) from the mass showed scattered and loose clusters of pleomorphic cells with increased nucleocytoplasmic ratio, prominent nucleoli, and abundant dense as well as vacuolated cytoplasm in a lipid backgound. A diagnosis of sebaceous carcinoma with cervical metastasis was made and confirmed on histopathology. Conclusion: FNA is a simple, cost effective technique for diagnosis of eyelid tumors. Familiarity with the cytomorphological features of sebaceous carcinoma can lead to early diagnosis of this malignancy with minimum discomfort to the patient. [ABSTRACT FROM AUTHOR]

Muir-Torre syndrome (MTS) is a rare genodermatosis, diagnosed by the presence of sebaceous neoplasms along with an internal malignancy, most commonly colorectal carcinomas. MTS is most commonly caused by microsatellite instabilities of the hMLH1 and hMSH2 mismatch repair genes, and is rarely caused by mutations of the hMSH6 gene. We describe the case of a 56-year-old male who presented with an enlarging mass on his back as well as hematochezia. The back mass was excised, and pathology confirmed microsatellite instability in MSH2 and MSH6. Abdominal CT and colonoscopy confirmed the presence of synchronous masses in the cecum, ascending colon, and the transverse colon. He refused any further workup or treatment, only to return 8 months later complaining of hematochezia and discomfort due to an enlarging mass protruding from the rectum. After consenting to surgical intervention, he agreed to outpatient chemotherapy treatment. The presence of sebaceous neoplasms should raise suspicion for the possibility of an associated internal malignancy. [ABSTRACT FROM AUTHOR]

Muir-Torre syndrome is a rare, autosomal dominant genodermatosis characterized by the presence of at least one sebaceous gland neoplasm, associated with an underlying visceral malignancy. Muir-Torre syndrome is believed to be a subtype of Lynch Syndrome. Affected individuals are found to have germline mutations predominantly in DNA mismatch repair gene MSH2, and much less frequently, MLH1. The authors report the case of a 55-year-old woman presenting with multiple cutaneous neoplasms including sebaceoma, basal cell carcinoma, and squamous cell carcinoma; personal history of colorectal and endometrial cancer; and family history of colorectal cancer; found to have a deletion at mismatch repair gene MLH1. It is important to recognize the role of these less common gene deletions in producing the Muir-Torre syndrome phenotype, and consider the correlation of cutaneous manifestations with internal disease. The authors discuss the clinical presentation of Muir-Torre syndrome, methods of diagnosis, and the importance of regular medical surveillance to detect and prevent disease progression in Muir-Torre syndrome patients and their family members. [ABSTRACT FROM AUTHOR]