Tag Archives: Basal cell carcinoma

Gorlin syndrome

Alternative Names:Nevoid basal cell carcinoma syndrome (NBCCS),basal cell nevus syndrome, multiple basal cell carcinoma syndrome and Gorlin–Goltz syndrome

Definition:
Gorlin syndrome is an inherited medical condition involving defects within multiple body systems such as the skin, nervous system, eyes, endocrine system, and bones. People with this syndrome are particularly prone to developing a common and usually non-life-threatening form of non-melanoma skin cancers.

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People with the syndrome have a predisposition to multiple basal cell carcinomas (a form of skin cancer), jaw cysts and other generally harmless abnormalities in the bone. The severity of the disease can be wide-ranging.

About 10% of people with the condition do not develop basal cell carcinomas (BCCs). the name Gorlin syndrome refers to researcher Robert J. Gorlin (1923–2006).

First described in 1960, NBCCS is an autosomal dominant condition that can cause unusual facial appearances and a predisposition for basal cell carcinoma, a malignant type of skin cancer. The prevalence is reported to be 1 case per 56,000-164,000 population. Recent work in molecular genetics has shown NBCCS to be caused by mutations in the PTCH (Patched) gene found on chromosome arm 9q. If a child inherits the defective gene from either parent, he or she will have the disorder

Incidence:
About 750,000 new cases of sporadic basal cell carcinomas (BCCs) occur each year in the United States. Ultraviolet (UV) radiation from the sun is the main trigger of these cancers, and people with fair skin are especially at risk. Most sporadic BCCs arise in small numbers on sun-exposed skin of people over age 50, although younger people may also be affected. By comparison, NBCCS has an incidence of 1 in 50,000 to 150,000 with higher incidence in Australia. One aspect of NBCCS is that basal cell carcinomas will occur on areas of the body which are not generally exposed to sunlight, such as the palms and soles of the feet and lesions may develop at the base of palmer and plantar pits. One of the prime features of NBCCS is development of multiple BCCs at an early age, often in the teen years. Each person who has this syndrome is affected to a different degree, some having many more characteristics of the condition than others.

Components:-
Some or all of the following may be seen in someone with Gorlin Syndrome:

1.Multiple basal cell carcinomas of the skin
2.Odontogenic keratocyst: Seen in 75% of patients and is the most common finding. There are usually multiple lesions found in the mandible. They occur at a young age (19 yrs average).
3.Rib and vertebrae anomalies
4.Intracranial calcification
5.Skeletal abnormalities: bifid ribs, kyphoscoliosis, early calcification of falx cerebri (diagnosed with AP radiograph)
6.Distinct faces: frontal and temporopariental bossing, hypertelorism, and mandibular prognathism

What genes are related to Gorlin syndrome?
Mutations in the PTCH1 gene cause Gorlin syndrome. This gene provides instructions for making a protein called Patched-1, which functions as a receptor. Receptor proteins have specific sites into which certain other proteins, called ligands, fit like keys into locks. Together, ligands and their receptors trigger signals that affect cell development and function. A protein called Sonic Hedgehog is the ligand for the Patched-1 receptor. Patched-1 prevents cell growth and division (proliferation) until Sonic Hedgehog is attached.

The PTCH1 gene is a tumor suppressor gene, which means it keeps cells from proliferating too rapidly or in an uncontrolled way. Mutations in this gene prevent the production of Patched-1 or lead to the production of an abnormal version of the receptor. An altered or missing Patched-1 receptor cannot effectively suppress cell growth and division. As a result, cells proliferate uncontrollably to form the tumors that are characteristic of Gorlin syndrome.

You may click to learn more about the PTCH1 gene.

How do people inherit Gorlin syndrome?
Gorlin syndrome is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the features that are present from birth, such as large head size and skeletal abnormalities. An affected person often inherits a PTCH1 mutation from one affected parent. Other cases may result from new mutations in the gene. These cases occur in people with no history of the disorder in their family. For tumors to develop, a mutation in the other copy of the PTCH1 gene must occur in certain cells during the person’s lifetime. Most people who are born with one PTCH1 mutation eventually acquire a second mutation in certain cells and develop basal cell carcinomas and other tumors.

Causes:-
Gorlin syndrome is an autosomal dominant condition. The abnormal gene is found on chromosome 9. New mutations (where neither parent carries the gene) are common.

Diagnosis:
Diagnosis of NBCCS is made by having 2 major criteria or 1 major and 2 minor criteria.

The major criteria consist of the following:

1.more than 2 BCCs or 1 BCC in a person younger than 20 years;
2.odontogenic keratocysts of the jaw
3.3 or more palmar or plantar pits
4.ectopic calcification or early (<20 years) calcification of the falx cerebri
5.bifid, fused, or splayed ribs
6.first-degree relative with NBCCS.

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The minor criteria include the following:

1.macrocephaly.
2.congenital malformations, such as cleft lip or palate, frontal bossing, eye anomaly (cataract, colobma, microphtalmia, nystagmus).
3.other skeletal abnormalities, such as Sprengel deformity, pectus deformity, polydactyly, syndactyly or hypertelorism.
4.radiologic abnormalities, such as bridging of the sella turcica, vertebral anomalies, modeling defects or flame-shaped lucencies of hands and feet.
5.ovarian and cardio fibroma or medulloblastoma (the latter is generally found in children below the age of two).
People with NBCCS need education about the syndrome, and may need counseling and support, as coping with the multiple BCCs and multiple surgeries is often difficult. They should reduce UV light exposure, to minimize the risk of BCCs. They should also be advised that receiving Radiation therapy for their skin cancers may be contraindicated. They should look for symptoms referable to other potentially involved systems: the CNS, the genitourinary system, the cardiovascular system, and dentition.

Genetic counseling is advised for prospective parents, since one parent with NBCCS causes a 50% chance that their child will also be affected.

Treatment:
Although there’s no cure, the carcinomas can be treated by surgery, lasers or photodynamic therapy, which reduces scarring.

If there’s a family history of the syndrome, it’s possible for family members to be tested to see if they carry the faulty gene.

Those with Gorlin syndrome are now advised to avoid – or to take advice before undergoing – any radiation treatment, as it’s thought it may exacerbate the condition.

Treatment is usually supportive treatment, that is, treatment to reduce any symptoms rather than to cure the condition.

*Enucleation of the odontogenic cysts can help but new lesions, infections and jaw deformity are usually a result.
*The severity of the basal cell carcinoma determines the prognosis for most patients. BCCs rarely cause gross disfigurement, disability or death .

*Genetic counseling

Advice and support:-
•Gorlin Syndrome Group
•Tel: 01772 496849
•Email: info@gorlingroup.org
•Website: www.gorlingroup.org

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.

Resources:
http://www.bbc.co.uk/health/physical_health/conditions/gorlinsyndrome1.shtml
http://en.wikipedia.org/wiki/Nevoid_basal_cell_carcinoma_syndrome
http://ghr.nlm.nih.gov/condition/gorlin-syndrome
http://dermnetnz.org/systemic/gorlins.html

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Skin Cancer

basal cell carcinoma removal scar

Image by safoocat via Flickr

Definition:
Skin cancer is the uncontrolled growth of abnormal skin cells. If left unchecked, these cancer cells can spread from the skin into other tissues and organs.It is a malignant growth on the skin which can have many causes. Skin cancer generally develops in the epidermis (the outermost layer of skin), so a tumor is usually clearly visible. This makes most skin cancers detectable in the early stages. There are three common types of skin cancer, each of which is named after the type of skin cell from which it arises. Cancers caused by UV exposure may be prevented by avoiding exposure to sunlight or other UV sources, and wearing sun-protective clothes. The use of sunscreen is recommended by medical organizations as a measure that helps to protect against skin cancer (see sunscreen).

Unlike many other cancers, including those originating in the lung, pancreas, and stomach, only a small minority of those afflicted will actually die of the disease.[citation needed] Skin cancers are the fastest growing type of cancer in the United States. Skin cancer represents the most commonly diagnosed malignancy, surpassing lung, breast, colorectal and prostate cancer. Melanoma is the least common skin cancer but it is potentially the most serious: there are over 8,000 new cases each year in the UK and 1,800 deaths. More people now die of Melanoma in the UK than in Australia. It is the second most common cancer in the young population (20 – 39 age group). It is estimated that approximately 85% of cases are caused by too much sun. Non-melanoma skin cancers are the commonest skin cancers. The majority of these are called Basal Cell Carcinomas. These are usually localised growths caused by excessive cumulative exposure to the sun and do not tend to spread.

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Types:-
There are different types of skin cancer. Basal cell carcinoma is the most common. Melanoma is less common, but more dangerous.

More rare types of skin cancer include:
*Dermatofibrosarcoma protuberans
*Merkel cell carcinoma
*Kaposi’s sarcoma

The BCC and the SCC often carry a UV-signature mutation indicating that these cancers are caused by UV-B radiation via the direct DNA damage. However the malignant melanoma is predominantly caused by UV-A radiation via the indirect DNA damage.[citation needed] The indirect DNA damage is caused by free radicals and reactive oxygen species. It has been shown, that the absorption of three sunscreen ingredients into the skin, combined with a 60-minute exposure to UV, leads to an increase of free radicals in the skin.

Skin cancer as a group:-
Many laymen and even professionals consider the basal cell carcinoma (BCC), the squamous cell carcinoma (SCC) and the malignant melanoma as one group – namely skin cancer. This grouping is problematic for two reasons:

*the mechanism that generates the first two forms is different from the mechanism that generates the melanoma. The direct DNA damage is responsible for BCC and SCC while the indirect DNA damage causes melanoma.

*the mortality rate of BCC and SCC is around 0.3 causing 2000 deaths per year in the US. In comparison the mortality rate of melanoma is 15-20% and it causes 138001 deaths per year.

Even though it is rare, malignant melanoma is responsible for 75 % of all skin cancer related death cases.

While sunscreen has been shown to protect against BCC and SCC it may not protect against malignant melanoma. When sunscreen penetrates into the skin it generates reactive chemicals. It has been found that sunscreen use is correlated with malignant melanoma. The lab-experiments and the epidemiological studies indicate that sunscreen use causes melanoma.

Causes:
The outer layer of skin, the epidermis, is made up of different types of cells. Skin cancers are classified by the types of epidermal cells involved:

Basal cell carcinoma develops from abnormal growth of the cells in the lowest layer of the epidermis and is the most common type of skin cancer.
Squamous cell carcinoma involves changes in the squamous cells, found in the middle layer of the epidermis.
Melanoma occurs in the melanocytes (cells that produce pigment) and is less common than squamous or basal cell carcinoma, but more dangerous. It is the leading cause of death from skin disease.
Skin cancers are sometimes classified as either melanoma or nonmelanoma. Basal cell carcinoma and squamous cell carcinoma are the most common nonmelanoma skin cancers. Other nonmelanoma skin cancers are Kaposi’s sarcoma, Merkel cell carcinoma, and cutaneous lymphoma.

Skin cancer is the most common form of cancer in the Unites States. Known risk factors for skin cancer include the following:

*Complexion: Skin cancers are more common in people with light-colored skin, hair, and eyes.
*Genetics: Having a family history of melanoma increases the risk of developing this cancer.
*Age: Nonmelanoma skin cancers are more common after age 40.
*Sun exposure and sunburn: Most skin cancers occur on areas of the skin that are regularly exposed to sunlight or other

*ultraviolet radiation. This is considered the primary cause of all skin cancers.

Skin cancer can develop in anyone, not only people with these risk factors. Young, healthy people — even those with with dark skin, hair, and eyes — can develop skin cancer.

Symptoms:
Skin cancers may have many different appearances. They can be small, shiny, waxy, scaly and rough, firm and red, crusty or bleeding, or have other features. Therefore, anything suspicious should be looked at by a physician. See the articles on specific skin cancers for more information.

Here are some features to look for:

*Asymmetry: one half of the abnormal skin area is different than the other half
*Borders: irregular borders
*Color: varies from one area to another with shades of tan, brown, or black (sometimes white, red, blue)
*Diameter: usually (but not always) larger than 6 mm in size (diameter of a pencil eraser)

Any skin growth that bleeds or will not heal
Use a mirror or have someone help you look on your back, shoulders, and other hard-to-see areas.

Risk factors:-
Skin cancer is most closely associated with chronic inflammation of the skin. This includes:

1.Overexposure to UV-radiation can cause skin cancer either via the direct DNA damage or via the indirect DNA damage mechanism. UVA & UVB have both been implicated in causing DNA damage resulting in cancer. Sun exposure between 10AM and 4PM is most intense and therefore most harmful. Natural (sun) & artificial UV exposure (tanning salons) are associated with skin cancer.[citation needed] Since sunbeds cause mostly indirect DNA damage (free radicals) their use is associated with the deadliest form of skin cancer, malignant melanoma.

2.UVA rays affect the skin at a deeper level than UVB rays, reaching through the epidermis and the dermis to the hypodermis where connective tissues and blood vessels are located. UVA activates the melanin of the epidermis causing changes in pigmentation as well as loss of elasticity of the skin, which contributes to premature wrinkling, sagging and aging of the skin.

3.UVB rays primarily affect the epidermis causing sunburns, redness, and blistering of the skin. The melanin of the epidermis is activated with UVB just as with UVA; however, the effects are longer lasting with pigmentation continuing over 24 hours.
Chronic non-healing wounds, especially burns. These are called Marjolin’s ulcers based on their appearance, and can develop into squamous cell carcinoma.

4.Genetic predisposition, including “Congenital Melanocytic Nevi Syndrome”. CMNS is characterized by the presence of “nevi” or moles of varying size that either appear at or within 6 months of birth. Nevi larger than 20 mm (3/4″) in size are at higher risk for becoming cancerous.

5.Skin cancer is one of the potential dangers of ultraviolet germicidal irradiation.
Skin can be protected by avoiding sunlight entirely, or wearing protective clothing while outdoors. Skin cancer is usually caused by exposing skin to UV rays excessively.

Treatment:-
Most skin cancers can be treated by removal of the lesion, making sure that the edges (margins) are free of the tumor cells. These excisions provide the best cure for both early and high-risk disease.

For low-risk disease, radiation therapy and cryotherapy (freezing the cancer off) can provide adequate control of the disease; both, however, have lower overall cure rates than surgery.

Mohs’ micrographic surgery is a technique used to remove the cancer with the least amount of surrounding tissue and the edges are checked immediately to see if tumor is found. This provides the opportunity to remove the least amount of tissue and provide the best cosmetically favorable results. This is especially important for areas where excess skin is limited, such as the face. Cure rates are equivalent to wide excision. Special training is required to perform this technique.

In the case of disease that has spread (metastasized), further surgical procedures or chemotherapy may be required.

Scientists have recently been conducting experiments on what they have termed “immune- priming”. This therapy is still in its infancy but has been shown to effectively attack foreign threats like viruses and also latch onto and attack skin cancers. More recently researchers have focused their efforts on strengthening the body’s own naturally produced “helper T cells” that identify and lock onto cancer cells and help guide the killer cells to the cancer. Researchers infused patients with roughly 5 billion of the helper T cells without any harsh drugs or chemotherapy. This type of treatment if shown to be effective has no side effects and could change the way cancer patients are treated.

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Prognosis:-
The outlook depends on a number of factors, including the type of cancer and how quickly it was diagnosed. Basal cell carcinoma and squamous cell carcinoma rarely spread to other parts of the body. However, melanoma is more likely to spread. See the specific skin cancer articles for additional information.

Prevention :-
Minimizing sun exposure is the best way to prevent skin damage, including many types of skin cancer:

*Protect your skin from the sun when you can — wear protective clothing such as hats, long-sleeved shirts, long skirts, or pants.
*Try to avoid exposure during midday, when the sun is most intense.
*Use sunscreen with an SPF of at least 15. Apply sunscreen at least one-half hour before sun exposure, and reapply frequently.
*Apply sunscreen during winter months as well.
*Reapply sun block every 2 hours and after swimming

Although it is generally accepted that UV exposure is the greatest risk factor in melanoma development, some sceptics say that there is no proven data that links moderate sun exposure with the appearance of melanoma.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.

Resources:
http://www.nlm.nih.gov/medlineplus/ency/article/001442.htm
http://en.wikipedia.org/wiki/Skin_cancer

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The Natural Cancer-Fighting Power of Tomatoes and Broccoli

Broccoli and tomatoes, both of which have been previously found to help fight cancer, have been found to be even more effective against prostate cancer when eaten together as part of a daily diet.

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Researchers fed rats who hd been implanted with prostate cancer cells a diet containing 10 percent broccoli powder and 10 percent tomato powder for a period of 22 weeks.

Other rats received either one or the other but not both, a dose of lycopene (the substance in tomatoes believed fight cancer), or the drug finasteride. Another set of rats was castrated.

At the end of the study, the tomato and broccoli combination diet was the most effective treatment; only castration even came close in terms of effectiveness.

The combination of vegetables may be more effective than either one alone because different compounds in each food work on different anti-cancer pathways.

Considering that nearly all of the conventional prostate cancer treatments may be unnecessary and harmful to your health, still more confirmation about a natural and completely healthy whole food treatment that doesn’t involve a toxic drug or dangerous procedure.

Even eating tomato sauce a couple of times a week may reduce prostate cancer risk, and the diet suggested by this study — 1.4 cups of fresh broccoli and 1/2 cup of tomato paste daily — is easily doable by those who wish to reduce their risk.

Still, it’s very important to realize, despite the good news, no food or vegetable is ideal for everyone. One of the best things you can do to honor your body, among many to virtually eliminate your cancer risks, remains eating the best foods for your unique metabolic type.

However, one of the most valuable and important natural approaches you can have to avoid prostate cancer is to make sure you have adequate sun exposure on your skin. There is overwhelming compelling evidence that optimal vitamin D levels are highly protective against prostate cancer.

Source: www.mercola.com