Periocular sebaceous cell carcinoma following chemical burn: a unique case of a Marjolin ulcer.

This study reports a unique case of a Marjolin ulcer in a patient who presented with periocular sebaceous cell carcinoma 16 years after a lye chemical burn. A 68-year-old woman presented with a long history of irregular and tender right upper and lower eyelid margins, periocular erythema, madarosis, trichiasis, ptosis, and chemosis, occurring many years after chemical burn to the area. After eyelid biopsy established the diagnosis of sebaceous cell carcinoma, subsequent map biopsies of the conjunctival surface revealed extensive sebaceous cell carcinoma with pagetoid spread, necessitating anterior exenteration for definitive treatment. This report describes the first case in which a sebaceous cell carcinoma has arisen in the area of a chemical burn. Sebaceous cell carcinomas should be included in the spectrum of Marjolin ulcers, and ophthalmologists should be aware of this entity when evaluating patients with a history of a burn to the eyelids.

Extra ocular sebaceous adenocarcinoma in HIV-positive patient--case report.

Sebaceous adenocarcinoma is a rare adnexal tumor that can affect the skin and is divided into ocular, a more common form and extra ocular, of a rarer occurrence. We report the case of a patient diagnosed with Acquired Immune Deficiency Syndrome (AIDS) who developed an extra ocular, bulky and fast-growing sebaceous adenocarcinoma on the face. The literature has suggested that transplanted patients and HIV-positive patients have an excess risk for developing adnexal tumors, including sebaceous adenocarcinoma.

Extra ocular sebaceous adenocarcinoma in HIV-positive patient - case report / Adenocarcinoma sebaceo extra-ocular em paciente HIV positivo - relato de caso

Sebaceous adenocarcinoma is a rare adnexal tumor that can affect the skin and is divided into ocular, a more common form and extra ocular, of a rarer occurrence. We report the case of a patient diagnosed with Acquired Immune Deficiency Syndrome (AIDS) who developed an extra ocular, bulky and fast-growing sebaceous adenocarcinoma on the face. The literature has suggested that transplanted patients and HIV-positive patients have an excess risk for developing adnexal tumors, including sebaceous adenocarcinoma.

Adenocarcinoma sebáceo é um tumor anexial raro que pode envolver a pele e é dividido em ocular, mais comum e extraocular, mais raro. Relatamos o caso de um paciente com diagnóstico de Síndrome da Imunodeficiência Adquirida que desenvolveu um adenocarcinoma sebáceo extra-ocular, na face, volumoso, de rápido crescimento. A literatura tem sugerido que pacientes transplantados e portadores do vírus da imunodeficiência humana têm um excesso de risco para o desenvolvimento de tumores anexiais, incluindo o Adenocarcinoma sebáceo.

Nonmelanoma skin cancer after renal transplantation: a single-center experience in 1736 transplantations.

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

Immunosuppression and sebaceous tumors: a confirmed diagnosis of Muir-Torre syndrome unmasked by immunosuppressive therapy.

Muir-Torre syndrome (MTS) is an autosomal dominant genodermatosis caused by mutations in the DNA mismatch repair genes MLH1 and MSH2. This case describes a patient with an extensive family history of colon cancer who experienced the onset of multiple sebaceous adenomas and carcinomas after undergoing kidney transplantation and receiving immunosuppressive therapy. The finding of deficient MSH2 expression in the immunohistochemical analysis of a sebaceous carcinoma prompted genetic testing for a systemic mutation in the mismatch repair gene. A systemic mutation of the MSH2 gene was detected and, despite the absence of a visceral malignancy, the diagnosis of MTS was made. Immunosuppression has previously been thought to play a possible role in unmasking a latent MTS phenotype in transplant recipients, but systemic mutations have not previously been analyzed. The relationship between immunosuppression and sebaceous tumors with the possibility of unmasking a MTS phenotype in transplant recipients is discussed.

A two-antibody mismatch repair protein immunohistochemistry screening approach for colorectal carcinomas, skin sebaceous tumors, and gynecologic tract carcinomas.

Mismatch repair protein immunohistochemistry is a widely used method for detecting patients at risk for Lynch syndrome. Recent data suggest that a two-antibody panel approach using PMS2 and MSH6 is an effective screening protocol for colorectal carcinoma, but there are limited data concerning this approach for extraintestinal tumors. The purpose of this study was to review the utility of a two-antibody panel approach in colorectal carcinoma and extraintestinal tumors. We evaluated mismatch repair protein expression in two cohorts: (1) a retrospective analysis of intestinal and extraintestinal tumors (n=334) tested for mismatch repair protein immunohistochemistry and (2) a prospectively accrued series of intestinal, gynecologic tract, and skin sebaceous neoplasms (n=98). A total of 432 cases were analyzed, including 323 colorectal, 50 gynecologic tract, 49 skin sebaceous, and 10 other neoplasms. Overall, 102/432 tumors (24%) demonstrated loss of at least one mismatch repair protein. Concurrent loss of MLH1 and PMS2 was the most common pattern of abnormal expression (50/432, 12%) followed by concurrent loss of MSH2 and MSH6 (33/432, 8%). Of 55 cases with abnormal PMS2 expression, 5 (9%) demonstrated isolated loss of PMS2 expression. Of 47 cases with abnormal MSH6 expression, 14 (30%) demonstrated isolated loss of MSH6 expression. Isolated loss of MLH1 or MSH2 was not observed. Colorectal carcinomas more frequently demonstrated abnormal expression of PMS2 (39/59, 66%). Skin sebaceous neoplasms more frequently demonstrated abnormal expression of MSH6 (18/24, 75%, respectively). A total of 65 tumors with abnormal mismatch repair protein expression were tested for microsatellite instability (MSI): 47 (72%) MSI high, 9 (14%) MSI low, and 9 (14%) microsatellite stable (MSS). Abnormal MSH6 expression accounted for 14/18 (78%) cases that were MSS or MSI low. Our findings confirm the utility of a two-antibody approach using PMS2 and MSH6 in colorectal carcinoma and indicate that this approach is effective in extraintestinal neoplasms associated with Lynch syndrome.

Sebaceous carcinoma: the great masquerader: emgerging concepts in diagnosis and treatment.

Sebaceous carcinoma (SC) is a rare tumor with a high rate of local recurrence and metastasis to lymph nodes and organs. The majority of SCs occur in the periocular region frequently presenting as painless, round subcutaneous nodules with a high tendency of diffuse and invasive growth in the eyelid and conjunctiva. It frequently masquerades as inflammatory conditions or as other tumors leading to delay in diagnosis, inappropriate treatment and increased morbidity and mortality. Sebaceous carcinoma is associated with Muir-Torre syndrome, a genetic condition presenting with sebaceous skin tumors associated with internal malignancy. Therefore, SC patients must be carefully evaluated and referred to an internist or gastroenterologist when indicated. Surgery is the definitive therapy for SC. In recent years, less radical surgical strategies are being used with improved outcomes. Current studies demonstrate that Mohs micrographic surgery (MMS) provides maximal tissue conservation and lower recurrence rates. Greater awareness and understanding of SC and its behavior has led to earlier diagnosis and appropriate treatment.

Cystic sebaceous carcinoma: is it a constant pathognomic marker for Muir-Torre syndrome?

Sebaceous carcinoma (SC) is a rare and aggressive cutaneous neoplasm. It may arise in ocular or extraocular sites. Approximately 25% of all reported cases of SC are extraocular. Cystic presentation of sebaceous neoplasm is rare. So far, cystic sebaceous neoplasia (CSN) has been reported only in association with Muir-Torre syndrome (MTS). Furthermore, CSN has recently been characterized as a marker lesion of MTS. We report a case of CSN of the nose that was not associated with MTS. Mohs micrographic surgery was performed with no recurrence for 2 years. Patients with MTS need long-term follow-up to detect possible future presentation of MTS.

Sebaceous carcinoma of the eyelid: a review of 14 cases.

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

[Multiple sebaceous tumors with colon cancer--Muir-Torre syndrome]. / Faggyúmirigy eredetú multiplex daganat coloncarcinomával--Muir-Torre-szindróma.

The Muri-Torre syndrome is an autosomal-dominant genodermatosis. The criteria of the diagnosis is the occurrence of at least one sebaceous skin tumour and at least one malignant internal tumour. Skin tumours must be considered as very important signs because they may precede visceral malignancies. The authors present the case of a 62 years old man. Three tumours were excised from three different locations of his trunk in the Plastic Surgery Ward of our hospital. The tumours proved to be sebaceous adenoma, sebaceous epithelioma and sebaceous carcinoma by histology. Because of this result, we had conducted a detailed tumour screening which found a non-symptomatic malformation of the proximal colon tract. Histology of the resected colon part revealed adenocarcinoma. With the presentation of this case the authors call attention on the fact, that in case of the diagnosis of a sebaceous skin tumour, careful tumour screening could be lifesaving.

Sebaceous carcinoma of the eyelid associated with retinoblastoma.

OBJECTIVE: To analyze the association between retinoblastoma (Rb) and sebaceous carcinoma (SC) of the eyelid to improve surveillance of survivors of RB: DESIGN: Case report and systematic literature review. METHODS: Ten patients who had SC develop after Rb were identified by systematic literature review, and a child who died with lymph node, lung, and liver metastases 7 years after irradiation for Rb is described. The data were analyzed by univariate statistics, including cumulative frequency distribution plots and Kaplan-Meier analysis. RESULTS: Of 11 children with SC of the eyelid who all had hereditary RB, 9 (82%; 95% confidence interval, 48-98) received a median of 46 Gy (range, 21-89) of radiotherapy at a median age of 16 months (range, 0.5-15 years) and had SC develop within the field of radiation. Their median age at diagnosis of SC was 14 years (range, 8-30 years), median diagnostic delay 12 months (range, 6 months-3 years), and median interval from irradiation 11 years (range, 5-26 years); 7 of them (78%; 95% confidence interval, 40-97) were diagnosed between 5 and 15 years after radiotherapy. SC also developed at the age of 32 and 54 years in two nonirradiated Rb patients. Five patients had regional lymph node metastases after a median time of 12 months (range, 1 month-24 years). The 5-year cumulative probability of survival was 87%. CONCLUSIONS: SC of the eyelid may occur in patients with hereditary Rb regardless of primary treatment, especially within the field 5 to 15 years after radiotherapy.

Carcinoma sebáceo nasal. A propósito de un caso / Nasal sebaceus carcinoma. About a case

Se presenta un caso clínico de un carcinoma sebáceo extra-ocular, en un varón de 71 años de edad con una tumoración en ala nasal derecha (AU)

Four-eyelid sebaceous cell carcinoma following irradiation.

BACKGROUND: Sebaceous cell carcinoma is a distinctive tumor of the eyelid associated with a high rate of metastasis and mortality. Involvement of both upper and lower lids has been described; however, involvement of all 4 eyelids is extremely rare. OBJECTIVE: To describe the evaluation and diagnosis of the clinicopathologic features in a 74-year-old patient with 4-eyelid sebaceous carcinoma and a history of whole face irradiation for eczema. METHODS: Bilateral eyelid, conjunctiva, map, and fine needle biopsy of enlarged, submandibular lymph node specimens were stained with hematoxylin-eosin and oil-red-O for light microscopy and fresh-frozen sections were evaluated by electron microscopy. RESULTS: The biopsy specimens from the eyelids and conjunctiva showed sebaceous carcinoma cells in the meibomian glands and scattered within the conjunctival epithelium. The cells were moderately well differentiated. Similar cells stained with oil-red-O were found in the submandibular lymph nodes. CONCLUSIONS: The appearance of bilateral sebaceous carcinoma supports the concept of simultaneous occurrence of multiple primaries in specific patients, especially after radiation therapy. Patients with a history of facial irradiation and atypical eyelid lesions should be evaluated for malignant tumors such as sebaceous carcinoma.

Malignant tumors of the eyelid: a population-based study of non-basal cell and non-squamous cell malignant neoplasms.

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

Sebaceous adenomas, squamous cell carcinoma and skin infections in a patient with carcinoma of the colon, rectum and bladder.

Sebaceous adenomas and squamous cell carcinoma developed in a male patient in addition to viral, mycotic and bacterial infections, several years after the removal of three malignant tumors from his lower gastrointestinal and urinary tract. Skin tests with trichophytin, candidin, and mixed bacteria were negative. Various aspects regarding cutaneous changes associated with colorectal and bladder carcinomas are discussed.