Rak gruczołów łojowych
Etiologia i przyczyny
Rak gruczołu łojowego (sebaceous carcinoma) to rzadki, agresywny nowotwór skóry wywodzący się z gruczołów łojowych, najczęściej lokalizujący się w obrębie głowy i szyi, zwłaszcza w okolicy oczodołowej (około 75% przypadków). Etiologia tego nowotworu jest wieloczynnikowa i obejmuje mutacje genów supresorowych (p53, Rb) oraz zaburzenia sygnalizacji Wnt/beta-katenina, szczególnie w kontekście zespołu Muira-Torre’a (MTS), który jest wariantem zespołu Lyncha. MTS wiąże się z mutacjami w genach naprawy DNA (MLH1, MSH2, MSH6, PMS2) i charakteryzuje się bardziej agresywnym przebiegiem raka gruczołu łojowego, z 5-letnim przeżyciem swoistym wynoszącym 52,5% w porównaniu do 78,2% u pacjentów bez tego zespołu. Czynniki ryzyka obejmują ekspozycję na promieniowanie UV, wcześniejszą radioterapię (zwłaszcza w obrębie głowy i szyi), immunosupresję (np. HIV/AIDS, leczenie po przeszczepach narządów, choroby autoimmunologiczne) oraz możliwy udział czynników wirusowych, takich jak HPV o wysokim ryzyku onkogennym. Mediana wieku rozpoznania wynosi 57-72 lata, a nowotwór częściej dotyczy kobiet i osób pochodzenia azjatyckiego.
Etiologia raka gruczołu łojowego (sebaceous carcinoma)
Rak gruczołu łojowego (sebaceous carcinoma) to rzadki, agresywny nowotwór skóry wywodzący się z gruczołów łojowych. Gruczoły te produkują łój (sebum), który pomaga nawilżać i chronić skórę. Dokładna etiologia tego nowotworu pozostaje w dużej mierze niewyjaśniona, a badacze nadal pracują nad pełnym zrozumieniem mechanizmów leżących u podłoża jego rozwoju.12
Mutacje genetyczne
Podobnie jak w przypadku innych nowotworów, rak gruczołu łojowego powstaje w wyniku mutacji genetycznych, które prowadzą do niekontrolowanego wzrostu i namnażania się komórek. Gdy w zdrowych komórkach gruczołów łojowych dochodzi do zmian w DNA, ulega zaburzeniu normalny cykl życiowy komórki. W normalnych warunkach DNA komórki zawiera instrukcje dotyczące tempa wzrostu i namnażania się oraz programowanej śmierci komórki. Mutacje genetyczne zmieniają te instrukcje, powodując szybki i niekontrolowany wzrost komórek nowotworowych, które nie obumierają w odpowiednim czasie. Skutkuje to nagromadzeniem zbyt dużej liczby komórek i formowaniem się guza nowotworowego.34
Według przeglądu badań z 2021 roku, w rozwój raka gruczołu łojowego zaangażowanych jest wiele genów.5 Wykazano, że w przypadkach sporadycznych, jak również tych związanych z zespołem Muira-Torre’a, ważną rolę odgrywają zmiany w sygnalizacji Wnt/beta-katenina. Dodatkowo mutacje genów supresorowych, takich jak p53 i Rb, zostały powiązane z rozwojem sporadycznego raka gruczołu łojowego.6
Czynniki środowiskowe
Chociaż dokładne przyczyny raka gruczołu łojowego nie są w pełni poznane, istnieją pewne czynniki środowiskowe, które mogą przyczyniać się do jego rozwoju:
Promieniowanie ultrafioletowe (UV)
Ekspozycja na promieniowanie UV jest potencjalnym czynnikiem ryzyka rozwoju raka gruczołu łojowego. Podobnie jak w przypadku innych nowotworów skóry, długotrwała ekspozycja na światło słoneczne może odgrywać rolę w patogenezie tego nowotworu.7 Obserwacje wskazujące na powstawanie raka gruczołu łojowego z choroby Bowena lub rogowacenia słonecznego oraz predyspozycja do rozwoju tego nowotworu w miejscach narażonych na działanie słońca sugerują rolę promieniowania UV w patogenezie niektórych przypadków raka gruczołu łojowego w obszarach eksponowanych na słońce.8
Radioterapia
Wcześniejsza ekspozycja na promieniowanie w ramach radioterapii, szczególnie w obrębie głowy i szyi, stanowi istotny czynnik ryzyka rozwoju raka gruczołu łojowego. Nowotwór ten może rozwinąć się nawet po wielu latach od zakończenia leczenia – osoby, które otrzymały radioterapię jako dzieci, mogą zachorować w wieku 60-70 lat.9 Rak gruczołu łojowego rozwija się również u dzieci, które otrzymały radioterapię z powodu siatkówczaka (retinoblastoma), nowotworu rozwijającego się w oku.10
Immunosupresja
Stan obniżonej odporności znacznie zwiększa ryzyko rozwoju raka gruczołu łojowego. Immunosupresja może być spowodowana różnymi czynnikami:1112
- Zakażenie wirusem HIV lub AIDS – u osób z AIDS ryzyko zachorowania na raka gruczołu łojowego jest 8-krotnie wyższe13
- Leki immunosupresyjne stosowane po przeszczepieniu narządów (np. cyklosporyna) – u biorców przeszczepu narządów ryzyko rozwoju raka gruczołu łojowego może być nawet 90-krotnie wyższe14
- Leki stosowane w leczeniu chorób autoimmunologicznych, takich jak reumatoidalne zapalenie stawów czy łuszczyca11
- Choroby nowotworowe, takie jak chłoniaki15
Znacznie podwyższone ryzyko raka gruczołu łojowego w populacjach z obniżoną odpornością, podobne do wzrostu obserwowanego w przypadku innych nowotworów związanych z infekcjami wirusowymi (mięsak Kaposiego, rak z komórek Merkla), sugeruje możliwy wirusowy czynnik etiologiczny również w przypadku raka gruczołu łojowego.13
Etiologia wirusowa
Istnieją przesłanki sugerujące możliwy udział czynników wirusowych w patogenezie raka gruczołu łojowego, chociaż bezpośrednie dowody pozostają niejednoznaczne. Wśród potencjalnych patogenów wirusowych wymienia się:16
- Wirusy brodawczaka ludzkiego (HPV) o wysokim ryzyku onkogennym – wykryto je w podgrupie młodszych pacjentów z rakiem gruczołu łojowego1417
- Inne wirusy, chociaż ich dokładna rola wymaga dalszych badań16
Ze względu na sprzeczne doniesienia i prawdopodobną złożoność potencjalnego zaangażowania wirusów w rozwój raka gruczołu łojowego, potrzebne są dalsze badania, aby wyjaśnić tę zależność.16
Predyspozycje genetyczne
Zespół Muira-Torre’a (MTS)
Zespół Muira-Torre’a jest rzadkim, autosomalnie dominującym zespołem nowotworowym, który znacząco zwiększa ryzyko rozwoju raka gruczołu łojowego. MTS stanowi wariant zespołu Lyncha (dziedzicznego niepolipowatego raka jelita grubego – HNPCC).718
Wyróżnia się dwa główne podtypy MTS:19
- MTS-I – charakteryzuje się mutacją germinalną w genach naprawy błędnie sparowanych zasad DNA (MMR), w tym mutL homolog1 (MLH1), mutS homolog2 (MSH2), mutS homolog6 (MSH6), postmejotyczne zwiększone segregowanie 2 (PMS2), oraz w genie niezwiązanym z MMR – cząsteczka adhezji komórek nabłonkowych (EBCAM), który epigenetycznie wycisza blisko związany gen MSH220
- MTS-II – wariant polipowatości związanej z genem MUTYH (MAP), stanowiący około 35% guzów u pacjentów z MTS21
Około 18,8% do 33,3% pacjentów z guzami łojowymi ma zdiagnozowany HNPCC.19 U pacjentów z MTS, oprócz raka gruczołu łojowego, często występują nowotwory trzewne, głównie w obrębie przewodu pokarmowego (rak jelita grubego), a także nowotwory układu moczowo-płciowego i endometrium.2223
Interesujące jest to, że w ponad połowie przypadków zmiany skórne rozwijają się po zdiagnozowaniu nowotworu trzewnego, natomiast w 22% przypadków guzy skóry poprzedzają rozwój nowotworu trzewnego nawet o 25 lat.19 Badania sugerują, że rak gruczołu łojowego u pacjentów z MTS ma bardziej agresywny przebieg niż u osób bez tego zespołu, z 5-letnim przeżyciem swoistym dla raka gruczołu łojowego wynoszącym 52,5% u pacjentów z MTS w porównaniu do 78,2% u osób bez tego zespołu.22
Zmiany związane z MTS często charakteryzują się niestabilnością mikrosatelitarną, w której występuje defekt naprawy DNA po replikacji z powodu mutacji w genach MSH-2, MLH-1 lub rzadziej MSH-6.24
Czynniki demograficzne
Wiek
Rak gruczołu łojowego najczęściej występuje u osób starszych, zwłaszcza między 60. a 80. rokiem życia, chociaż może wystąpić w dowolnym wieku. Według badań, rak gruczołu łojowego zdiagnozowano u dzieci już w wieku 3 lat, jak również u osób w wieku powyżej 90 lat.910 Mediana wieku w momencie rozpoznania wynosi od 57 do 72 lat.15
Chociaż rak gruczołu łojowego jest częstszy u osób starszych, może wystąpić u młodszych pacjentów, szczególnie tych z historią napromieniania twarzy.25
Płeć
Ten typ nowotworu skóry występuje częściej u kobiet niż u mężczyzn.18 W ponad 98% przypadków rak gruczołu łojowego występuje u osób powyżej 40. roku życia i jest najczęstszy u osób w wieku 60-80 lat, które urodziły się jako kobiety.26
Pochodzenie etniczne
Istnieją dowody sugerujące, że osoby pochodzenia azjatyckiego mogą być bardziej narażone na rozwój raka gruczołu łojowego.727 Z nieznanych przyczyn osoby pochodzenia azjatyckiego wydają się być bardziej dotknięte rakiem gruczołu łojowego niż osoby innych ras czy grup etnicznych.28 Szczególnie rak gruczołu łojowego powieki może być częstszy w populacji azjatyckiej.29
Inne czynniki
Dodatkowe czynniki, które mogą zwiększać ryzyko rozwoju raka gruczołu łojowego, obejmują:30
- Wcześniejsze łagodne gruczolaki gruczołów łojowych18
- Stosowanie tiazydowych leków moczopędnych30
- Istniejący wcześniej znamion łojowy (nevus sebaceous)31
Lokalizacja i patogeneza raka gruczołu łojowego
Rak gruczołu łojowego może rozwinąć się we wszystkich miejscach, gdzie występują gruczoły łojowe, jednak najczęściej pojawia się w obrębie głowy i szyi. Około 75% tych nowotworów powstaje w okolicy oczodołowej, podczas gdy pozostałe 25% rozwija się w lokalizacjach pozaoczodołowych.23
Rak gruczołu łojowego w lokalizacji ocznej
W okolicy oczodołowej rak gruczołu łojowego najczęściej wywodzi się z gruczołów Meiboma, które są wyspecjalizowanymi gruczołami łojowymi. Nowotwory mogą również pochodzić z gruczołów Zeissa i gruczołów łojowych w mięsku łzowym (caruncle).25 Zmiany częściej występują na górnej powiece, prawdopodobnie ze względu na większą liczbę gruczołów Meiboma (około 50 gruczołów w górnych powiekach, około 25 w dolnych).32
Rak gruczołu łojowego w lokalizacji pozaocznej
Rak gruczołu łojowego może również występować w innych obszarach ciała w 25% przypadków, w tym w innych obszarach głowy i szyi oraz innych obszarach skóry z włosami, a nawet w obrębie narządów płciowych.32 W lokalizacjach pozaoczodołowych rak gruczołu łojowego nie wydaje się pochodzić z gruczołów łojowych, co czyni ich pochodzenie dyskusyjnym.33
Patogeneza
Jedna z hipotez sugeruje, że nowotwór wywodzi się z nabłonka gruczołów łojowych, ale najnowsze badania wskazują, że może pochodzić z komórki pluripotencjalnej zdolnej do różnicowania się w dowolną linię komórkową, w tym komórki łojowe.23
Inwazja raka gruczołu łojowego odbywa się poprzez:34
- Bezpośrednie rozszerzenie się na tkanki podskórne i/lub nabłonkowe (epitheliotropism)
- Wieloogniskowe rozprzestrzenianie się na tkanki śródskórne i podskórne
- Onkoforezę, w której komórki nowotworowe są hipotetycznie uwalniane i przeszczepiane
- Bezpośrednie rozprzestrzenianie się śródnaskórkowe przez rozprzestrzenianie się pagetodiowe – do 50% nawracających raków gruczołu łojowego w okolicy oczodołowej może obejmować rozprzestrzenianie się pagetodiowe, a 100% w przypadku próbek po wytrzewieniu
Rak gruczołu łojowego charakteryzuje się agresywnym przebiegiem, z 5-letnim wskaźnikiem przeżycia wynoszącym 78% dla choroby zlokalizowanej/regionalnej i 50% dla choroby przerzutowej.13 Został udokumentowany jako nowotwór, który może miejscowo nawracać, a także dawać przerzuty regionalne i odległe.31
Zapadalność na ten nowotwór wzrasta w Stanach Zjednoczonych od kilku dekad, ale podstawowe przyczyny tego wzrostu nie są jasne.13 Szacowana ogólna częstość występowania wynosi od jednego do dwóch przypadków na milion osób rocznie.31
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Materiały źródłowe
- #1 Sebaceous Carcinoma: Causes, Symptoms And Treatmenthttps://www.netmeds.com/health-library/post/sebaceous-carcinoma-causes-symptoms-and-treatment?srsltid=AfmBOoqVpzKKAKudbAEelmjCM2gBR38l9yKqPxt-owHp_TqsY0RGQIM3
Just like any other kind of cancer, the exact cause of Sebaceous carcinoma is yet unknown, but studies suggest that they usually begin in the Sebaceous glands, which are found in the epidermal tissue that comprises the external layer of the skin. When the normal sebaceous glands have a change or mutation in the DNA or the genetic structure of the cells, it leads to erratic growth of cells both in size and number. These cells usually don’t die like healthy cells. They accumulate together to form tumorous growth. […] Although there is no solid evidence, but because most SCs develop on and around the head and neck regions, the sun may play a vital role in causing this rare form of skin cancer.
- #2 Sebaceous carcinoma: Symptoms, causes, treatments, and morehttps://www.medicalnewstoday.com/articles/sebaceous-carcinoma
Sebaceous carcinoma is a rare and aggressive skin cancer. […] Because sebaceous carcinoma is rare, the exact cause is still unclear. […] Possible risk factors include: Muir-Torre syndrome, a compromised immune system, radiation treatments, age, and Asian heritage. […] Sebaceous carcinoma may occur due to a viral infection. However, more research is necessary to confirm this. […] Experts also believe sun exposure and damage to the skin in these areas may play a role in its development.
- #3 Sebaceous carcinoma // Middlesex Healthhttps://middlesexhealth.org/learning-center/diseases-and-conditions/sebaceous-carcinoma
Sebaceous carcinoma may begin as a painless lump or thickening of skin on the eyelid. […] The cause of sebaceous carcinoma often isn’t known. This cancer starts as a growth of cells in the oil-producing glands of the skin. These glands are called sebaceous glands. […] Sebaceous carcinoma happens when sebaceous gland cells get changes in their DNA. A cell’s DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to grow and multiply quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells. The cancer cells form a growth or lump on the skin.
- #4https://content-dev.irisoncology.com/library/sebaceous-carcinoma
Sebaceous carcinoma is a rare type of cancer that begins in an oil gland in the skin. […] The cause of sebaceous carcinoma often isn’t known. This cancer starts as a growth of cells in the oil-producing glands of the skin. These glands are called sebaceous glands. […] Sebaceous carcinoma happens when sebaceous gland cells get changes in their DNA. A cell’s DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to grow and multiply quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells. The cancer cells form a growth or lump on the skin.
- #5 Sebaceous Carcinoma: Symptoms, Photos, Staging, Treatmenthttps://www.healthline.com/health/skin-cancer/sebaceous-carcinoma
Sebaceous carcinoma is a rare but aggressive skin cancer that often affects the eyelids but can develop in any sebaceous gland in your body. […] Like all cancers, sebaceous carcinomas occur due to genetic mutations that cause healthy cells to become cancerous. In the case of sebaceous carcinomas, the affected cells are in the sebaceous glands. According to a 2021 research review, multiple genes are involved. […] Scientists aren’t entirely sure what causes the mutations, but they have a few ideas. Environmental factors, like ultraviolet (UV) radiation, may play a role. […] Other known risk factors include: exposure to UV radiation, Muir-Torre syndrome, a rare genetic condition that causes tumors to develop in the sebaceous glands, a weakened immune system, often due to HIV or organ transplantation. […] Research links Muir-Torre syndrome (MTS), a rare genetic condition, with sebaceous carcinoma. In fact, MTS has links to an increased risk of several different kinds of tumors, including colorectal, endometrial, and ovarian cancers.
- #6 Sebaceous carcinoma – Wikipediahttps://en.wikipedia.org/wiki/Sebaceous_carcinoma
While there are markedly increased rates of cutaneous neoplasms in solid organ transplant recipients, there is evidence to suggest that solid organ transplantation may increase the risk of SGc up to 90 times. […] Others have observed a significantly increased risk of SGc in patients with AIDS, suggesting some role for immunosuppression. […] Muir-Torre syndrome (MTS) is an autosomal dominant cancer syndrome characterized by multiple sebaceous and visceral neoplasms, the most common being colorectal adenocarcinoma. […] Besides mutations in mismatch repair genes, Wnt/beta-catenin signaling is known to be altered and play a significant role in the development of sebaceous tumors. […] More recently, mutations in tumor suppressor genes including p53 and Rb have been associated with the development of sporadic SGc as well as SGc in MTS patients with intact mismatch repair and subsets of younger patients presenting with SGc harboring transcriptionally active high-risk human papillomavirus (HPV).
- #7 Sebaceous Carcinoma: Symptoms, Causes & Treatmenthttps://my.clevelandclinic.org/health/diseases/24087-sebaceous-carcinoma
Sebaceous carcinoma is a rare type of skin cancer that starts in your skins oil-producing (sebaceous) glands. […] Experts dont know why some people develop sebaceous carcinoma. Like other types of skin cancer, exposure to the suns ultraviolet (UV) rays may contribute to this cancer. It may also develop in people, especially younger people, who have had radiation therapy to the head or neck. […] People with a rare inherited disease called Muir-Torre syndrome are more likely to develop sebaceous carcinoma, as well as colorectal (colon) cancer. Muir-Torre syndrome is a form of Lynch syndrome. It causes tumors to form in your sebaceous glands. […] Some studies suggest that people of Asian descent are also more at risk.
- #8 Sebaceous carcinoma – Wikipediahttps://en.wikipedia.org/wiki/Sebaceous_carcinoma
Sebaceous carcinoma, also known as sebaceous gland carcinoma (SGc), is an uncommon malignant cutaneous (skin) tumor. […] The cause of these lesions in the vast majority of cases is unknown. […] The pathogenesis of SGc remains poorly understood. […] The majority of SGc are solitary and sporadic and believed to be associated with such factors as ultraviolet exposure, radiotherapy, and immunosuppression. […] Other SGc including those occurring outside of the head and neck region and the presentation of multiple at a time are believed to be associated with genetic defects including defects in mismatch repair genes, MuirTorre syndrome (MTS), and familial retinoblastoma. […] The observation of extraocular SGc arising from Bowen disease or actinic keratosis and the predisposition of SGc to arise in sun-exposed areas suggest a role for ultraviolet exposure or intraepidermal neoplasia in the pathogenesis of some SGc in sun-exposed areas.
- #9 Skin cancer types: Sebaceous carcinoma causeshttps://www.aad.org/public/diseases/skin-cancer/types/common/sebaceous/causes
People diagnosed with this rare skin cancer tend to be between: […] Sebaceous carcinoma (SC) can begin earlier or later. According to research studies, SC has been diagnosed in a 3-year-old child and people in their 90s. […] For SC, age is a common risk factor. Other risk factors for SC are: […] A weakened immune system greatly increases the risk for SC. […] Radiation treatments to the head or neck: People who received these radiation treatments as a child have been diagnosed with this rare skin cancer in their 60s and 70s. […] SC is rare, so scientists still have much to learn, including what causes it. Because most SCs develop on the head and neck, the sun may play a role in causing this rare skin cancer.
- #10 Sebaceous carcinoma: Foothill Dermatology Medical Center: Dermatologyhttps://www.foothillderm.com/blog/sebaceous-carcinoma
Sebaceous carcinoma (SC) can begin earlier or later. According to research studies, SC has been diagnosed in a 3-year-old child and people in their 90s. […] Anything that increases your risk of getting a disease is a risk factor. For SC, age is a common risk factor. Other risk factors for SC are: […] A weakened immune system greatly increases the risk for SC. The immune system may be weakened by: […] Radiation treatments to the head or neck: People who received these radiation treatments as a child have been diagnosed with this rare skin cancer in their 60s and 70s. SC also develops in children who receive radiation treatments for retinoblastoma, a cancer that develops in the eye. […] SC is rare, so scientists still have much to learn, including what causes it. Because most SCs develop on the head and neck, the sun may play a role in causing this rare skin cancer.
- #11 Sebaceous Carcinoma Treatment – Lexington Dermatologisthttps://dermatologistlexington.com/skincancer/sebaceous-carcinoma/
Asian Descent: Some studies suggest that people of Asian descent might be more vulnerable to sebaceous carcinoma. […] Age: Getting older can increase the risk. […] Sun Exposure: Spending a lot of time in the sun without protection can also make it more likely. […] Weakened Immune System: If your immune system is weakened due to conditions like cancer, HIV/AIDS, organ transplant medications, or drugs for arthritis or psoriasis, you are at a higher risk.
- #12 What Is Sebaceous Carcinoma?https://www.icliniq.com/articles/skin-care/sebaceous-carcinoma
Multi-Torre Syndrome: It is an autosomal dominant cancer syndrome that runs in families. This type of syndrome increases the risk of developing sebaceous carcinoma. Not only is it associated with sebaceous carcinoma, but also it can increase the chance of developing other types of cancer, colon cancer being the most common. […] A weakening of the immune system due to any of the below reasons increases the risk of developing sebaceous carcinoma: Drugs used in the treatment of arthritis or psoriasis. Immunosuppressant medications taken to prevent organ rejection following transplantation of organs. Immune deficiency diseases like cancer or human immunodeficiency virus (HIV).
- #13 Sebaceous carcinoma epidemiology and genetics: Emerging concepts and clinical implications for screening, prevention, and treatmenthttps://pmc.ncbi.nlm.nih.gov/articles/PMC7854832/
Sebaceous carcinoma is an aggressive skin cancer with a 5-year overall survival rate of 78% for localized/regional disease and 50% for metastatic disease. The incidence of this cancer has been increasing in the United States for several decades, but the underlying reasons for this increase are unclear. […] We further discuss emerging evidence of a possible viral etiology for this cancer. […] The highly elevated risk for sebaceous carcinoma among immunosuppressed populations (solid organ transplant recipients; individuals with HIV infection), which is similar to the elevation observed for other virus-associated cancers (Kaposi sarcoma; Merkel cell carcinoma), suggests that sebaceous carcinoma may also be caused by a virus. […] Recently, immunosuppression has been identified as a strong risk factor for sebaceous carcinoma. Among individuals with acquired immunodeficiency syndrome (AIDS), there is an 8-fold elevation in risk for this cancer.
- #14 Sebaceous carcinoma – Wikipediahttps://en.wikipedia.org/wiki/Sebaceous_carcinoma
While there are markedly increased rates of cutaneous neoplasms in solid organ transplant recipients, there is evidence to suggest that solid organ transplantation may increase the risk of SGc up to 90 times. […] Others have observed a significantly increased risk of SGc in patients with AIDS, suggesting some role for immunosuppression. […] Muir-Torre syndrome (MTS) is an autosomal dominant cancer syndrome characterized by multiple sebaceous and visceral neoplasms, the most common being colorectal adenocarcinoma. […] Besides mutations in mismatch repair genes, Wnt/beta-catenin signaling is known to be altered and play a significant role in the development of sebaceous tumors. […] More recently, mutations in tumor suppressor genes including p53 and Rb have been associated with the development of sporadic SGc as well as SGc in MTS patients with intact mismatch repair and subsets of younger patients presenting with SGc harboring transcriptionally active high-risk human papillomavirus (HPV).
- #15 Sebaceous Carcinoma – EyeWikihttps://eyewiki.org/Sebaceous_Carcinoma
Older age is a risk factor, as the reported median age at presentation has ranged between 57 and 72 years. However, tumors can arise in younger individuals who have been treated with periocular radiotherapy. The role of immunosuppression is unclear, but solid organ transplantation increases the risk of sebaceous carcinoma. In addition, other immunosuppressed states such as lymphoma may increase the likelihood of periocular sebaceous carcinoma.
- #16 Sebaceous carcinoma epidemiology and genetics: Emerging concepts and clinical implications for screening, prevention, and treatmenthttps://pmc.ncbi.nlm.nih.gov/articles/PMC7854832/
The strongly increased risk for sebaceous carcinoma among people with AIDS and solid organ transplant recipients is similar to that of other virus-induced cancers such as Merkel cell carcinoma (caused by Merkel cell polyomavirus) and Kaposi’s sarcoma (caused by Kaposi’s sarcoma-associated herpesvirus/human herpes virus 8), which suggests that sebaceous carcinoma may also be caused by a viral infection. […] However, direct evidence supporting a viral agent has been contradictory. […] Further complicating the story of a potential viral etiology in sebaceous carcinoma is that multiple viruses have been implicated. […] Ultimately, due to the conflicting reports to date and the likely complexity of potential viral involvement in sebaceous carcinoma, several clear lines of evidence will be needed to bring clarity.
- #17 Sebaceous carcinoma | MyPathologyReporthttps://www.mypathologyreport.ca/diagnosis-library/sebaceous-carcinoma/
Sebaceous carcinoma is a type of skin cancer. Sebaceous carcinoma develops from specialized cells called sebocytes in the dermis and subcutaneous tissue of the skin. […] Doctors do not know exactly what causes sebaceous carcinoma. However, risk factors for developing this type of cancer include prior radiation to the skin, excessive sun exposure, and viral infections (HPV and HIV). […] People with Muir Torre syndrome are at an increased risk of developing sebaceous carcinoma.
- #18 Sebaceous gland carcinoma | Skin cancer | Cancer Research UKhttps://www.cancerresearchuk.org/about-cancer/skin-cancer/types/sebaceous-gland-carcinoma
Sebaceous gland carcinoma is a very rare type of skin cancer. It starts in the sebaceous glands. These are the glands that produce our natural skin oils. […] There are some factors that increase your risk of developing sebaceous gland carcinoma. But having a risk factor doesn’t mean that you will develop this cancer. […] Some factors that can increase your risk include: non cancerous lumps (benign adenomas) of the sebaceous glands, exposure to radiation for example, previous treatment with radiotherapy, a genetic condition called Muir Torr syndrome, having a weakened immune system following organ transplant. […] Muir Torr syndrome can mean that you have another primary cancer elsewhere in your body. So people diagnosed with sebaceous gland cancer are often checked over for signs of cancer elsewhere. […] This type of skin cancer is more common in women than men. It’s more common in elderly people.
- #19 Sebaceous Carcinoma – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK610689/
Sebaceous carcinoma development is linked to various factors, including ultraviolet radiation (UVR) exposure, organ transplants, and immunosuppression with medications like cyclosporine. […] Additionally, this malignancy is associated with genetic cancer predisposition syndromes, such as MTS-I, which is a distinct variant of hereditary nonpolyposis colorectal cancer (HNPCC), sometimes referred to as Lynch syndrome. […] Sebaceous carcinoma is also associated with MTS-II, a MUTYH-associated polyposis (MAP) variant. […] Approximately 18.8% to 33.3% of patients with sebaceous tumors are diagnosed with HNPCC. […] Notably, sebaceous skin lesions develop after a visceral malignancy is diagnosed in over half of the cases. […] In 22% of cases, skin tumor growth precedes visceral neoplasia by up to 25 years.
- #20 Sebaceous Carcinoma | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/149613
Sebaceous carcinoma development is linked to various factors, including ultraviolet radiation (UVR) exposure, organ transplants, and immunosuppression with medications like cyclosporine. […] Additionally, this malignancy is associated with genetic cancer predisposition syndromes, such as MTS-I, which is a distinct variant of hereditary nonpolyposis colorectal cancer (HNPCC), sometimes referred to as Lynch syndrome. […] Sebaceous carcinoma is also associated with MTS-II, a MUTYH-associated polyposis (MAP) variant. […] MTS-I is characterized by a germline mutation in mismatch repair (MMR) genes, including mutL homolog1 (MLH1), mutS homolog2 (MSH2), mutS homolog6 (MSH6), postmeiotic segregation increased 2 (PMS2), and the non-MMR gene epithelial cell adhesion molecule (EBCAM), which epigenetically silences the closely linked MSH2 gene.
- #21 Sebaceous Carcinoma | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/149613
Approximately 18.8% to 33.3% of patients with sebaceous tumors are diagnosed with HNPCC. […] Notably, sebaceous skin lesions develop after a visceral malignancy is diagnosed in over half of the cases. […] In 22% of cases, skin tumor growth precedes visceral neoplasia by up to 25 years. […] The subtype MTS-II, a variant of MAP, comprises around 35% of tumors in patients with MTS. […] In addition to developing numerous adenomatous polyps and, consequently, colorectal cancer, affected individuals are predisposed to sebaceous adenomas, sebaceous epitheliomas, and sebaceous carcinomas.
- #22 Sebaceous Carcinoma – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK610689/
Malignancies commonly associated with HNPCC include gastrointestinal (colorectal), endometrial, and genitourinary types. […] Research suggests a more aggressive sebaceous carcinoma in patients with MTS than in those without, with a 5-year sebaceous carcinoma-specific survival of 52.5% in patients with MTS compared to 78.2% in individuals without the syndrome. […] The subtype MTS-II, a variant of MAP, comprises around 35% of tumors in patients with MTS. […] In addition to developing numerous adenomatous polyps and, consequently, colorectal cancer, affected individuals are predisposed to sebaceous adenomas, sebaceous epitheliomas, and sebaceous carcinomas.
- #23 Extraocular Sebaceous Carcinoma: A Report of 2 Cases | Actas Dermo-Sifiliográficashttps://www.actasdermo.org/es-extraocular-sebaceous-carcinoma-a-report-articulo-S1578219012003113
Sebaceous carcinoma is a rare, aggressive adnexal tumor of unknown etiology; 75% of these tumors arise in the periocular region while 25% arise at extraocular sites. […] Its etiology is unknown. […] One hypothesis is that the tumor originates in the epithelium of sebaceous glands, but recent studies suggest that it may be derived from a pluripotent cell able to differentiate into any cell line, including sebaceous cells. […] Sebaceous carcinoma has been associated with Muir-Torre syndrome, a hereditary disease of autosomal dominant transmission. […] The most frequently associated visceral tumors are gastrointestinal, located mainly in the proximal colon, followed by tumors of the genitourinary tract. […] Approximately 23% of the patients with Muir-Torre syndrome have sebaceous carcinoma.
- #24 Extraocular Sebaceous Carcinoma: A Report of 2 Cases | Actas Dermo-Sifiliográficashttps://www.actasdermo.org/es-extraocular-sebaceous-carcinoma-a-report-articulo-S1578219012003113
Thus, when patients present with a sebaceous carcinoma, they should be carefully screened by taking a personal and family history of cancer and performing a full physical examination and baseline laboratory tests. […] Lesions associated with Muir-Torre syndrome often have unstable microsatellites, in which there is DNA repair defect after replication due to a mutation in the MutS homolog (MSH)-2, the MutL homolog (MLH)-1, and more recently and less often the MSH-6 genes. […] The treatment of choice for the sebaceous carcinoma is excision with margins of 5 to 6mm.
- #25 Sebaceous Gland Carcinoma: Background, History of the Procedure, Epidemiologyhttps://emedicine.medscape.com/article/1213781-overview
In the periocular area, the most common sites for sebaceous carcinoma typically are the Meibomian glands, which are specialized sebaceous glands. However, tumors also can originate from the Zeiss glands and sebaceous glands in the ocular caruncle. […] Sebaceous carcinoma is a fast-growing form of skin cancer that has a 5-year survival rate of 78% for localized/regional disease and 50% for metastatic disease. The incidence of this cancer has been on the rise in the United States for many years, but the factors contributing to this increase are not fully understood. […] Although sebaceous cell carcinomas are more common in elderly patients, they may be seen in younger patients with a history of radiation to the face.
- #26 Sebaceous Gland Carcinoma: Symptoms, Causes, and Treatmentshttps://resources.healthgrades.com/right-care/skin-cancer/sebaceous-gland-carcinoma
Sebaceous gland carcinoma is a rare type of skin cancer. It typically begins on the eyelid, though it may start in sebaceous glands elsewhere. Itâs an aggressive type of cancer, which means it can grow or spread quickly. […] Researchers donât know the exact cause of sebaceous gland carcinoma. However, certain risk factors may make you more likely to develop the condition. […] In over 98% of cases, sebaceous gland carcinoma occurs in people over 40 years old. Itâs most common in people aged 60â80 years who were assigned female at birth. […] Other risk factors for sebaceous gland carcinoma include having a weakened immune system, receiving head or neck radiation treatment, being of Asian heritage, having Muir-Torre syndrome, a rare inherited condition that causes sebaceous gland tumors, and having regular sun exposure. […] Since the exact cause of sebaceous gland carcinoma is unclear, it may not be possible to prevent it.
- #27 Sebaceous Carcinoma Treatment – Lexington Dermatologisthttps://dermatologistlexington.com/skincancer/sebaceous-carcinoma/
Sebaceous carcinoma, pronounced âsuh-BAY-shus,â emerges as a rare skin cancer originating from oil-producing glands within the skin. These sebaceous glands, responsible for producing protective sebum, can be found extensively beneath the skinâs surface, especially in areas with hair. […] The precise triggers behind sebaceous carcinomaâs emergence are unknown. Experts are still piecing together the puzzle of its origins. However, common threads are starting to emerge. Exposure to the sunâs ultraviolet rays, similar to other skin cancers, might play a role in its development. Notably, those who have undergone radiation therapy, particularly around the head or neck, could be more susceptible. […] Certain factors that can make you more likely to get sebaceous carcinoma: Muir-Torre Syndrome: People with a rare genetic condition called Muir-Torre syndrome have a higher risk of getting sebaceous carcinoma, as well as colorectal cancer.
- #28 Sebaceous Carcinoma of Skin – DoveMedhttps://www.dovemed.com/diseases-conditions/sebaceous-carcinoma-of-skin
Sebaceous Carcinoma of Skin is a very rare, malignant tumor of the skin, arising from the sebaceous gland. They are thought to occur due to sun damage […] For some unknown reasons, individuals of Asian origin seem to be affected by Sebaceous Carcinomas more than individuals of any other race or ethnic group. […] A majority of cases of Sebaceous Carcinoma of Skin occur in a sporadic manner, without any genetic defect or abnormality. […] Though, some cases occur due to genetic defects, caused by mutations in the DNA mismatched repair gene.
- #29 Sebaceous carcinoma | Acclaim Dermatologyhttps://acclaimdermatology.com/blog/sebaceous-carcinoma
Sebaceous carcinoma (SC) is rare, so scientists still have much to learn, including what causes it. […] Because most SCs develop on the head and neck, the sun may play a role in causing this rare skin cancer. […] A weakened immune system greatly increases the risk for SC. […] People who received radiation treatments as a child have been diagnosed with this rare skin cancer in their 60s and 70s. […] SC on the eyelid may be more common in Asians. […] Muir-Torre syndrome is a rare medical condition. Many people do not know they have this condition until they are diagnosed with SC.
- #30 Sebaceous carcinomahttps://dermnetnz.org/topics/sebaceous-carcinoma
Sebaceous carcinoma is a rare aggressive skin cancer arising from a sebaceous gland. […] The exact cause of sebaceous carcinoma is unclear. The following have been reported to possibly increase the risk of these tumours: […] Underlying Muir-Torre or Lynch syndrome […] Previous radiation therapy to the area for a variety of benign and malignant conditions, such as retinoblastoma […] History of oral thiazide diuretic use […] Mutations to the tumour suppressor gene p53 […] Immunosuppression.
- #31 Sebaceous Carcinoma: A Rare Extraocular Presentation of the Cheek – European Medical Journalhttps://www.emjreviews.com/dermatology/article/sebaceous-carcinoma-a-rare-extraocular-presentation-of-the-cheek/
Sebaceous carcinoma is a rare and aggressive malignant cutaneous cancer that arises from the sebaceous gland epithelium. […] The estimated overall incidence rate is one to two per million people per year. […] Sebaceous carcinoma has been documented to have the ability to locally recur, as well as metastasise regionally and distantly. […] Additional risk factors for this cancer include male sex, ethnicity (Caucasian, Asian, and Indians), irradiation, ultraviolet exposure, pre-existing nevus sebaceous, Muir-Torre syndrome, and immunosuppression. […] The varying appearance allows sebaceous carcinoma to mimic other benign tumours or inflammatory conditions. […] The diagnosis of sebaceous carcinoma is established via incisional or partial-thickness biopsy. […] Sebaceous carcinomas are known to have a significant association with Muir-Torre syndrome, a subset of hereditary nonpolyposis colorectal carcinoma. […] Wide excision with clear margins is the treatment of choice for well-differentiated sebaceous carcinoma. […] It is important to be aware of sebaceous carcinomas risk for recurrence and distant metastasis: more common in the ocular type as compared to the extraocular.
- #32 Sebaceous Carcinoma – EyeWikihttps://eyewiki.org/Sebaceous_Carcinoma
Sebaceous carcinoma is a rare, highly malignant, and potentially lethal tumor of the skin, which most commonly occurs in the eyelid. The neoplasm arises from sebaceous glands, such as those of the meibomian glands in the tarsus, the Zeis glands of the eyelashes, the caruncle, and the skin of the eyebrow. The lesion occurs more often in the upper eyelid, most likely due to the presence of a greater number of meibomian glands (~50 glands in upper eyelids, ~25 in lower). […] Sebaceous carcinoma can also occur in other body areas in 25% of cases including other areas of the head and neck and other areas of skin with hair, even genitalia. […] Muir-Torre Syndrome (MTS) is an important consideration when a patient is diagnosed with sebaceous carcinoma. MTS is an autosomal dominant condition of sebaceous tumors associated with gastrointestinal, endometrial, and urologic tumors (without any predisposing factors). The sebaceous tumors included in this syndrome are: sebaceous adenoma, basal cell epithelioma with sebaceous differentiation, and sebaceous carcinoma. Cohen et al. reported that 24% of 120 MTS patients had sebaceous carcinoma. The sebaceous carcinomas in MTS are less aggressive and less likely to metastasize than solitary sebaceous carcinoma.
- #33 Sebaceous Carcinoma (Sebaceous Gland Carcinoma, Sebaceous Cell Carcinoma, Meibomian Gland Carcinoma) – Dermatology Advisorhttps://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/sebaceous-carcinoma-sebaceous-gland-carcinoma-sebaceous-cell-carcinoma-meibomian-gland-carcinoma/
Sebaceous carcinoma (SC) has been termed the great masquerader of adnexal tumors. […] The majority of sebaceous carcinomas arise spontaneously and de novo. In the periocular region, they arise from sebaceous glands, most commonly the Meibomian glands and the glands of Zeis. Other sebaceous glands in this region include the sebaceous glands of the eyebrows (follicular), sebaceous glands of the fine hairs of the eyelids and lacrimal glands, and sebaceous glands of the caruncle. In the extracutaneous sites, sebaceous carcinomas do not appear to arise from sebaceous glands, making their origin debatable. […] A minority of sebaceous carcinomas occur in patients with Muir-Torre syndrome, providing clues that mutations in tumor suppressor genes can be causative. Muir-Torre syndrome is an autosomal dominant disease with high penetrance and variable expressivity.
- #34 Sebaceous Carcinoma (Sebaceous Gland Carcinoma, Sebaceous Cell Carcinoma, Meibomian Gland Carcinoma) – Dermatology Advisorhttps://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/sebaceous-carcinoma-sebaceous-gland-carcinoma-sebaceous-cell-carcinoma-meibomian-gland-carcinoma/
MSH-1 and MLH-2 are genes important for the repair of DNA mismatch. Germline mutations in these genes lead to microsatellite instability in DNA and in the process lead to inactivation of tumor suppressor genes. […] The invasion of sebaceous carcinoma is believed to occur by direct extension into subdermal and/or epithelial tissues (epitheliotropism), multifocal proliferation into intradermal and subcutaneous tissues, and by oncophoresis whereby cancer cells are hypothetically shed and transplanted. […] There can also be direct intraepidermal extension by pagetoid spread. Up to 50% of recurrent periocular sebaceous carcinomas may involve pagetoid spread and 100% in exenterated specimens.