S1-Guideline Sebaceous Carcinoma.

Sebaceous gland carcinomas are rare malignant cutaneous adnexal tumors with sebocytic differentiation. The typical predilection area is the head and neck region, where sebaceous gland carcinomas are the most common malignant adnexal tumors of the skin. According to their localization a distinction is made between periocular and extraocular sebaceous gland carcinomas. Muir-Torre syndrome (MTS) should always be ruled out if it is suspected. In terms of prognosis, sebaceous gland carcinomas are potentially aggressive tumors with a clear tendency to recur and metastasize. Only small extraocular sebaceous gland carcinomas that have been completely resected have a very good prognosis. Sebaceous gland carcinomas most frequently metastasize lymphogenously to regional or distant lymph nodes; organ metastasis occurs less frequently. Periocular sebaceous gland carcinomas have a higher metastasis rate (up to 15%) than extraocular sebaceous gland carcinomas (up to 2%). Complete micrographically controlled surgery (MCS) of the primary tumor is the therapy of first choice, regardless of periocular or extraocular localization. Adjuvant or therapeutic radiotherapy may be considered. There is currently no established standard therapy for advanced, inoperable, or metastatic sebaceous gland carcinomas. Local procedures and systemic therapies such as chemotherapy or immunotherapy can be considered. The procedure should be determined individually by an interdisciplinary tumor board. Close follow-up care is recommended for these potentially aggressive carcinomas.

Sebaceous carcinoma: an updated review of pathogenesis, diagnosis, and treatment options.

Sebaceous carcinoma (SC) is a very rare and aggressive form of skin cancer that arises from the sebaceous glands. SC can occur anywhere on the body, but most commonly affects the head and neck, especially the upper eyelid. SC is the third most common malignancy of the eyelid and has the potential to metastasize and be fatal; therefore, it is vital for dermatologists to remain acquainted with this malignancy and its most current treatment options. Most commonly presenting as a painless lump or thickening of skin on the eyelid, SC has an insidious progression that may not prompt the patient to seek medical attention immediately. To avoid the potential of metastasis, early diagnosis and treatment is paramount. To assess if the cancer has spread, ophthalmology, imaging, and sentinel lymph node biopsy are recommended. This article provides a comprehensive review of SC's pathogenesis, current diagnostic methods, and treatments, including wide local excision, Mohs micrographic surgery, orbital exenteration, radiation, and other topicals. The prognosis of SC depends on several factors, including size, location, stage, and treatment method. After treatment of the neoplasm, diligent post-treatment surveillance remains the cornerstone of patient care. Continued dermatologic follow-ups are essential for early detection of reoccurrence, ensuring timely intervention and optimal long-term outcomes. In conclusion, this comprehensive review aims to equip dermatologists and other physicians with a nuanced understanding of SC, enabling them to provide effective care to support patients encountering this malignancy.

Adnexal and Sebaceous Carcinomas.

Adnexal carcinomas and sebaceous neoplasms are rare malignant neoplasms that are derived from eccrine and apocrine sweat glands or the pilosebaceous unit. Distinction of these neoplasms is essential, as treatment, workup, and prognosis varies widely among subtypes. For this comprehensive review, apocrine, eccrine, follicular, and sebaceous neoplasms are discussed. For each neoplasm, a review of clinical presentation, classic histologic findings, and management recommendations is provided.

Clinical Features and Prognosis of Young and Middle-Aged Adults With Skin Sebaceous Adenocarcinoma.

BACKGROUND: Sebaceous adenocarcinoma (SAC) mostly occurs in the elderly, and SAC in young and middle-aged population is inadequately investigated. OBJECTIVE: To explore the clinical features and prognosis of young and middle-aged adults with SAC. MATERIALS AND METHODS: Patients with skin SAC between ages 18 and 59 years from the Surveillance, Epidemiology, and End Results database (1975-2016) were eligible for this study. RESULTS: Seven hundred thirty-nine cases were identified. The proportion of extraocular SAC in the nonelderly increased from 1975-2005 to 2006-2016 ( p = .001), male predominance was observed in overall patients whereas female predominance in Asian population, and young patients had more head and neck SAC than middle-aged patients ( p = .014). The prognosis of young patients was better than middle-aged patients ( p = .004). Other independent prognostic factors included sex, marital status, tumor size, surgery, chemotherapy, and multiple primary cancer history. CONCLUSION: An increasing proportion of extraocular SAC was observed in young and middle-aged patients, and the young developed more head and neck SAC than the middle-aged. Female predominance was found in Asian population, and female patients had better prognosis. Younger age and married status indicated better prognosis, and around 20% of young and middle-aged patients might have poorer survival because of Muir-Torre syndrome.

Evaluation of electrochemotherapy in the management of apocrine gland anal sac adenocarcinomas in dogs: A retrospective study.

Background: Electrochemotherapy (ECT) combines the administration of anticancer drugs with the delivery of electric pulses, thus increasing the drug uptake through the cell membranes, resulting in increased efficacy. Aim: The aim of our study was to describe the tolerability and efficacy of ECT alone or in association with other treatment modalities for the management of apocrine gland anal sac adenocarcinoma (AGASAC). Methods: Medical records of dogs with a diagnosis of AGASAC that were treated with ECT alone or in combination with surgery/chemotherapy were retrospectively evaluated. Each dog received 20 mg/m2 of bleomycin intravenously. Based on the clinician's decision, the primary tumor or tumor bed was also infiltrated with cisplatin at the dose of 0.5 mg/cm2. Trains of permeabilizing biphasic electric pulses were then applied under general anesthesia. Results: Ten dogs were enrolled in the study. Of those 10 dogs, only one received ECT for treatment of microscopic local disease, while in six cases ECT was the only treatment modality. In three dogs, ECT was followed by systemic medical treatment. Six dogs (60%) had a partial response (PR), three dogs (30%) had stable disease, and one dog treated for microscopic disease did not show any sign of local relapse for 305 days after treatment, being still alive and in complete remission at the time of writing this article. The median time to progression was 303 days and the median survival time was 365 days. The treatment was well tolerated and local side effects were minimal. No systemic effects were documented. Conclusion: This preliminary study suggests that ECT may be beneficial for dogs with AGASAC and could be a useful addition to the current therapeutic options in consideration of its low cost, limited toxicity, and ease of administration.

Eyelid carcinoma: An experience from a tertiary cancer center.

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.

Periocular skin cancer: Diagnosis and management.

The periocular region sees high rates of cancer compared to the rest of the body and can be affected by several unique tumors. Treatment typically involves surgical excision, although adjunctive therapies and prognosis vary depending on the diagnosis. Basal cell carcinoma (BCC) is the most common malignancy affecting this region and should be excised promptly. This is followed by squamous cell carcinoma, which is often mistaken for BCC but is treated similarly. Melanoma is less common but more deadly, with relatively high rates of metastasis and death. Rare tumors that may be found in this region include sebaceous carcinoma, Merkel cell carcinoma, and microcystic adnexal carcinoma. While uncommon, these tumors are associated with poor outcomes and frequent recurrence. Recognition of periocular skin cancer is an important skill for primary care physicians, as prompt diagnosis and treatment can be sight or life-saving.

Metastatic Prostatic Adenocarcinoma in Patient With Muir-Torre Syndrome Misdiagnosed as Metastatic Sebaceous Carcinoma: Case Report and Systematic Literature Review.

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.

Nordic treatment practices survey and consensus for treatment of eyelid sebaceous carcinoma.

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.

Cutaneous sebaceous carcinoma.

Cutaneous sebaceous carcinoma occurs almost exclusively on the head and neck and has a significant propensity for recurrence and metastasis. It is easily mistaken for benign conditions, resulting in inappropriate management. Thus, it is important to maintain a high index of suspicion. Despite previous reports, sebaceous carcinoma may occur with similar frequency in Asians and whites. Recent genetic data suggest there are multiple mutational groups of sebaceous carcinoma, paving the way for targeted treatment. After a diagnosis, investigations for staging and for Muir-Torre syndrome should be considered. The available evidence on the treatment options for sebaceous carcinoma is discussed, and specific recommendations for management are made.

Eyelid abscess masking underlying sebaceous cell carcinoma.

Sebaceous cell carcinoma is well known as the "great masquerader" mimicking other benign or malignant eyelid conditions and lesions. We present a case of a middle age male presenting with a subacute left upper lid abscess who was ultimately diagnosed with malignant sebaceous cell carcinoma after incision and drainage and treatment with broad spectrum antibiotics. This case highlights the deceptive clinical and radiographic appearance of this tumor and the importance of histologic examination in atypical or refractory periorbital abscesses.

Impact of surgery and adjuvant treatment on the outcome of extraocular sebaceous carcinoma: a systematic review and individual patient's data analysis of 206 cases.

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.

Sebaceous carcinoma: evidence-based clinical practice guidelines.

Sebaceous carcinoma usually occurs in adults older than 60 years, on the eyelid, head and neck, and trunk. In this Review, we present clinical care recommendations for sebaceous carcinoma, which were developed as a result of an expert panel evaluation of the findings of a systematic review. Key conclusions were drawn and recommendations made for diagnosis, first-line treatment, radiotherapy, and post-treatment care. For diagnosis, we concluded that deep biopsy is often required; furthermore, differential diagnoses that mimic the condition can be excluded with special histological stains. For treatment, the recommended first-line therapy is surgical removal, followed by margin assessment of the peripheral and deep tissue edges; conjunctival mapping biopsies can facilitate surgical planning. Radiotherapy can be considered for cases with nerve or lymph node involvement, and as the primary treatment in patients who are ineligible for surgery. Post-treatment clinical examination should occur every 6 months for at least 3 years. No specific systemic therapies for advanced disease can be recommended, but targeted therapies and immunotherapies are being developed.

Lacrimal Sac Sebaceous Carcinoma.

Sebaceous carcinoma is an aggressive malignancy with a high mortality rate that commonly arises in the periorbital area. Rarely, the lacrimal apparatus may be involved by either contiguous or noncontiguous spread. The authors describe 2 unusual cases of sebaceous carcinoma in the lacrimal sac, presenting as a medial canthal mass simulating chronic dacryocystitis. In Case 1, the sebaceous carcinoma occurred primarily in the lacrimal sac, in the absence of concurrent or previous ocular adnexal sebaceous neoplasia. Mapping biopsies found no evidence of intraepithelial disease. Case 2 developed a late noncontiguous disease recurrence involving the lacrimal sac 5 years postprimary resection of an ipsilateral eyelid sebaceous carcinoma. These cases demonstrate atypical mode of spread of sebaceous carcinoma in lacrimal excretory system. It is important to regard the nasolacrimal drainage system as a potential reservoir for neoplastic sebaceous cells and minimize the risk of tumor cell dissemination during surgical management of eyelid or conjunctival sebaceous cell tumors.

Impact of clinicopathologic factors on survival in patients with sebaceous carcinoma of the eyelid - a population-based analysis.

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.

[Establishment of standardized diagnosis and treatment of eyelid sebaceous carcinoma in China].

Eyelid sebaceous carcinoma is a malignant tumor originating from Meibomian glands, Zeis glands, and other glands surrounding hair follicles. It is the second most common eyelid malignancy, following basal cell carcinoma, in China. Considering its diversified clinical manifestations and strong tendency for recurrence and metastasis, standardized cancer staging, surgical excision with frozen section control, and postsurgical follow-up has been internationally adopted with the aim to considerably reduce the risk of recurrence and metastasis. However, the lack of standardized and normalized staging, surgical treatment and follow-up in China inevitably caused high recurrence rate and low predictability of prognosis of eyelid sebaceous carcinoma. Therefore, it is of great significance to standardize the clinical diagnosis and treatment of eyelid sebaceous carcinoma in China. (Chin J Ophthalmol, 2018, 54: 481-483).

Sebaceous Carcinoma: A Review of the Scientific Literature.

OPINION STATEMENT: Sebaceous carcinoma is a rare and potentially aggressive cutaneous malignancy. Commonly reported in the periocular area and the head and neck region, sebaceous carcinoma can arise from any sebaceous gland in the skin. The clinical presentation may be nonspecific, and a biopsy is important to establish a diagnosis and to differentiate from mimickers including benign sebaceous neoplasms, other adnexal tumors, and basal cell carcinoma. A diagnosis of Muir Torre syndrome should be considered in patients presenting with a sebaceous neoplasm. Early treatment is important given the potential of sebaceous carcinoma to spread to the regional lymph nodes and beyond. Sentinel lymph node biopsy and imaging to complete tumor staging may be indicated for larger or more aggressive tumors. Surgery, including Mohs micrographic surgery, remains the primary treatment modality for sebaceous carcinoma. Mohs micrographic surgery has the advantage of complete margin evaluation and low recurrence rates. Advanced cases may be treated with orbital exenteration, radiation therapy, chemotherapy, or combination therapy.

Updates on the Molecular Pathology of Selected Ocular and Ocular Adnexal Tumors: Potential Targets for Future Therapy.

Ophthalmic pathologic studies of retinoblastoma first definitively elucidated a genetic etiology for cancer three decades ago. Advances in DNA sequencing, protein expression profiling, and the exploration of epigenetics have since led to categorization of tumors and clinical prognostication based on genetic aberrancy. There are now many neoplasms that are defined by a characteristic genetic signature. In the past several years alone, much has been discovered in regard to the original tumor-suppressor gene initially defined in retinoblastoma as well as in other intraocular tumors such as medulloepithelioma. Our further understanding of ocular adnexal tumors that result in substantial morbidity and mortality, such as sebaceous carcinoma, has also benefited from a genetic approach. In this article, we review the clinicopathologic features of the foregoing three entities--retinoblastoma, medulloepithelioma, and sebaceous carcinoma--in order to highlight discoveries in their underlying abnormal molecular genetic functioning.

Patterns of lymphatic spread and the management of eyelid carcinomas.

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.