Questions regarding BCCNS Manifestations

Questions Regarding Ovarian Fibromas

How effective is laser treatment for the removal of BCCs?[+/-]

Laser treatment is one of a number of treatments for BCCs. It can be good on an early BCC, burning off superficial areas of the skin and leaving little damage. If laser treatment is used on thicker lesions there will be scarring and lots of laser treatment on the face may become a problem.

What is PDT?[+/-]

PDT or Photodynamic Therapy is a relatively new treatment which is increasingly being used for the treatment of BCCs. It is a non-invasive technique involving the interaction of light with a photosensitizing agent. The photosensitizer is applied to target tissue followed by exposure to the light source.

Can I donate blood?[+/-]

Yes. Once blood has been donated it goes through a process which ensures it is safe for transfusion.

Does the size of the head mean problems during childbirth?[+/-]

In pregnancy and childbirth 72% – 80% of women needed forceps or caesarean section during childbirth.

I would like children but I have gorlin syndrome. What should I do?[+/-]

There is a 50% risk that a child will inherit the condition. It is important to point out that you do have choices. Speak to your doctor or geneticist, they will be in a position to offer information and advice about the condition, as well as offering guidance on pregnancy and child birth.

How common are ovarian fibromas?[+/-]

Studies show that about 25% of women have fibromas. They cause no problems unless they are large. There is no evidence that they affect fertility.

What are ovarian fibromas?[+/-]

They are solid benign lumps on the ovary which often become calcified. 25% – 30% of Gorlin women are affected by ovarian fibroma and/or calcification. They cause no problems unless they are large.

If a fibroma is found does it normally have to be removed?[+/-]

Only if it is causing problems, i.e. sometimes when the ovary twists round the pedicle, it can stangulate the blood supply. Usually the fibroma, not the ovary is removed.

Can fertility be affected by fibromas/cysts?[+/-]

There is no evidence to suggest that fibromas or cysts affect fertility.

Is there a rule on the frequency of ultrasound scans for fibromas/cysts?[+/-]

There is at present no protocol for regular screening for ovarian fibromas/cysts. If the patient requested screening it would be advised that medical professionals with experience of Gorlin Syndrome did it.

Is hammer toe related to BCCNS?[+/-]

Yes. The second toe tends to be longer than the big toe resulting in the second toe getting pushed back.

My thumbs are very short from the middle join to the end. Is this part of the syndrome?[+/-]

It does appear that a large number of people with Gorlin Syndrome have this problem. It is nothing to worry about and will cause no problems.

What are bifid ribs and are they a symptom of the syndrome?[+/-]

Yes they are. Bifid ribs are ribs that are split in two. They cause no problems and need no attention.

I am worried that my children may inherit BCCNS. How can I find out?[+/-]

There is a 50 : 50 chance of children inheriting Gorlin Syndrome. It is suggested that an appointment is obtained with a Geneticist who will be in a position to confirm the condition in family members. The family GP can arrange an appointment

Is radiotherapy treatment recommended for the treatment of BCCs?[+/-]

In some patients with Gorlin Syndrome radiotherapy may lead to rapid development of new basal cell carcinomas and therefore should be used under special/exceptional circumstances

What is Moh’s surgery?[+/-]

The procedure is a form of surgical excision that has been modified with mapping the margins of the tissue specimen to determine whether tumour remains. This technique spares normal tissue because of the microscopic control involved. The pain, post-operative result, follow up care, and healing are similar as with standard surgical excisions. The overall chance of a cure with Moh’s surgery is 99%.

Our insurance company is denying coverage for KCOTs as they feel that it is a dental condition, not a medical condition, how do we educate them otherwise? What should we say?[+/-]

This is often a problem with what is called and ICD-9 or ICD-10 codes. ICD stands for International Classification of Disease. This is a code that insurance companies use to determine reimbursement. The World Health Organization (WHO) even classifies the odontogenic keratocyst as a tumor rather than a cyst. For this reason various ICD-9 codes are used and include 526.01 or 213.0 and 213.1. There are various reasons to use these three different codes which is beyond the scope of this discussion. However, some 526.XX or 213.X code should be on the list. If another code is on the list and they are denying reimbursement questions should be asked. If not you may need to ask your provider what they did use actually means.

Probably the biggest problem with insurance companies comes not from paying for the surgical procedure, or paying of the pathologist for looking at the biopsy, but from repairing the consequences of the disease. One problem is the need for the radiographs on a yearly basis which insurance companies generally only want to pay for once every 5 years. Other problems would be with placement of implants, partials or dentures that need to be made because lost teeth. The biggest single “trick” is that the dentist/oral surgeon should always submit things through the medical insurance route first, when any surgery, exam or radiograph is provided. Unfortunately the restorations (implants, partials or dentures) almost always must be forwarded through dental insurance.

What is recurrence rate for KCOTs?[+/-]

This is a statistic that is thrown around in many publications. In our modern treatment of keratocystic odontogenic tumors, we can safely say that the recurrence rate is in the 2% to 10% range. Again, in basal cell nevus syndrome patients whether they are really recurrences or whether they are actually new occurrences is not fully known. In some older studies people will quote up to a 60% recurrance rate of KCOT’s with what they will term “simple enucleation”. The modern therapy with careful enucleation and scraping of the bone and roots with curettes is not simple enucleation. For this reason the rate is considered to be at the lower end of 2% to10%.

Should anyone who has an KCOT be screened for BCCNS or can people in the regular population have them as well?[+/-]

While KCOTs can be found in the general population, it is recommended that any individual with a KCOT occurrence under the age of 21 be screened for the syndrome, in addition to any patient that has more than one KCOT at a given time.

At what age should dental implants be placed for lost teeth?[+/-]

This varies by the area of the jaw where the teeth are lost. It also varies by whether the patient is male or female. Males generally need to have their implants placed approximately 2 years later than females. In the front part of the mandible from eye tooth to eye tooth, implants can often be placed as early as age 12. In other areas of both the upper and lower jaw implants are generally not possible until age 16 to 20. There is great variability in this and questions will center on the surgeon trying to determine whether growth has stopped in the child or not.

My dentist/oral surgeon wants to just wait and watch my KCOT, is that ok?[+/-]

Absolutely not. The KCOT or any radiolucent lesion seen on a panorex should be addressed in a basal cell nevus patient as soon as possible. The lesions will only get larger and more difficult to treat if they wait months before treatment.

What are the pros and cons of decompressions?[+/-]

Pro: decompression is more likely to save bone structure and teeth. With some patients having 10 or more tumors over a lifetime, if the surgeon is aggressive the patient will be left with few teeth at a very young age.

Con: decompression takes time, sometimes on the order of six months or more, for the decompression to result in the desired effect. During this time the patient will need to “flush out” the area multiple times each day. Depending on the location of the tumor this may be either an easy or laborious task. It may require help and the younger the child/patient the more difficult it might be for the patient to adequately flush it out. If not adequately flushed out, infections can ensue which could require emergency surgery. Some oral surgeons believe that decompression has a higher recurrence rate than other treatment modalities. However, as explained, with adequate follow up, decompression is a valuable tool in the management of many lesions.

What is the age range for KCOTs screening and how often is it recommended?[+/-]

This generally follows the same recommendations as the question on panorex x-rays. However, dental exams starting at age 2 with the normal twice a year dental exam and assessment of how teeth are erupting and assuring the bite is stable. The dentist will also look for any expansion of the jaws. At age 8, panorexes are recommended to begin. If an odontogenic keratocyst occurs in the maxilla which is the upper jaw, CT scans are often necessary. I still would recommend regular dental evaluation of the tissues and the jaws every 6 months and seeing the oral surgeon or ENT specialist for a more thorough evaluation on a yearly basis.

What should I be looking for in a dentist/oral surgeon?[+/-]

A general dentist will take care of the dental/tooth needs of a patient with basal cell nevus syndrome. It will generally be an oral surgeon who should be making the radiographs, panorexes and searching for, or following up keratocystic odontogenic tumors. My first question for the dentist would be what oral surgeon would they recommend in the area and whether they know the interest level of that oral surgeon with emphasis on whether they have treated basal cell nevus syndrome patients.

When talking to the oral surgeon, ask whether they are familiar with the syndrome. If they are familiar with the syndrome, ask some questions to try and determine their familiarity. Be sure to ask questions of their knowledge when treating keratocystic odontogenic tumors as well.

One important to consider when searching for any healthcare provider is personality, communication skills and how comfortable you feel with that healthcare provider.

Is there a particular specialist for treating KCOTs?[+/-]

Oral and maxillofacial surgeons generally have more consistent experience with keratocystic odontogenic tumors. With that said, not all oral surgeons may have experience with BCCNS patients.

What are the treatment options for KCOTS?[+/-]

Many people use marsupialization as a term to describe opening up a hole into the wall of the tumor where the patient can “flush out” the cavity several times a day. There have been some reports of this being successful to the point where the cysts totally resolves and the last portion of the area allowing the cavity to be flushed out is eventually removed and the area remodels itself to normal. However, this is generally not the way that we would recommend treatment in people with the basal cell nevus syndrome. Decompression is more common for the treatment of KCOT’s in BCCNS patients and involves making the same kind of opening produced in marsupialization, but when the cyst size becomes small enough (1/2-1 ½ inches in size) the surgeon then reopens the area and removes the remaining portion of the tumor by enucleation. Enucleation is the careful removal of the tumor wall. In some cases, the surgeon will then use curettage or the purposeful removal of bone with a sharp instrument or with a burr to prevent regrowth. The goal of these treatments is to retain the maximum number of teeth and with minimal loss of bone within the jaw. Some tumors can become so large, expansile and destructive that teeth do need to be removed to remove the tumor. Some surgeons also use what is called Carnoy’s solution to prevent regrowth of the tumors.

What is the time frame you can start and stop getting jaw cysts?[+/-]

Generally the earliest that the jaw cysts occur is about the time that the permanent teeth begin erupting into the mouth. Although there is lifetime risk of cyst development the incidence of cysts is greatest in the teenage years and early 20’s then decreases as the patient gets older.

How frequently should people with NBCNS have panorex x-rays?[+/-]

Panorex radiographs are recommended to begin on patients around the age 7 or 8. In patients with BCCNS, the odontogenic keratocystic tumors are often the first destructive expression of the syndrome. Though the tumors can occur at any age, it is not uncommon for them to ceomce detectable from the ages of 8 through the early 20’s. For this reason, panorex radiographs are recommended every year from 8 through age 21. If the jaw tumors appear in older patients, panorex should also be taken on a yearly basis for 5 years following the removal of the last tumor. If no new cysts occur in that 5 year period, panorexes can then be lengthened to every two years. If no cysts have occurred for eleven years the panorex radiographs can then be made at 5 year intervals.

Do the cysts always have to be surgically removed or can they resolve on their own?[+/-]

The cysts will not resolve themselves on their own and do need to be addressed. There are various ways for them to be addressed. If left alone, the cysts will only become bigger, more destructive and more difficult to remove with surgery.

Are the cysts that are associated with BCCNS always the OKC type?[+/-]

The huge majority of cysts associated with BCCNS are indeed the odontogenic keratocyst, however, there have been occasional reports of other odontogenic cysts that are in patients with BCCNS and some reported cases of ameloblastoma which is an odontogenic tumor.

My child has hundreds of BCC, what is the best treatment options?[+/-]

This type of scenario usually involves a history of some type of childhood cancer treatment. In cases such as these, pursuing a very knowledgeable doctor, preferably one who has worked with children, is highly recommended when possible. PDT treatment has been a true gift to patients and their families, but usually an extensive time/travel commitment is involved. Contact the BCCNS Life Support Network for more information.

BCCNS Fact Sheet-Dr. John Hellstein, University of Iowa

Do BCC’s have to be removed or do they tend to go away on their own?[+/-]

Skin cancer must be removed or it will continue to grow and infiltrate. There is some evidence that “spontaneous disappearance” can occasionally happen, but trusting in this phenomena is clearly not advised. BCC can appear to be dormant, or even seem to disappear, and often these same lesions flare up or return with a vengeance. BCCNS patients simply must be under a doctor’s careful and consistent care.

Can BCC metastasize?[+/-]

This is a rare occurrence, but it is possible.

What is a perineural BCC, a syringoma, a keocanthoma…?[+/-]

These are all varieties of skin cancer lesions, some are more aggressive than others, and all must be removed.

Any advice on sunscreen and sun protection?[+/-]

Apply sun protection thoroughly and consistently. Educate yourself and consider sun protection to be an investment in your overall health. Involve the entire family, not just the BCCNS patient. Evaluate your clothing and your surroundings (covered porches, tinted windows). Learn about the good, the bad, and the proper use of sunscreens. Carefully consider your activities during the heat of the day (10-3). Each state also has regulations regarding the tinting of car windows, particularly for health reasons. The BCCNS Life Support Network does not promote total avoidance of the sun because life is best when it is lived — just live wisely.

What do the SPF numbers on sunscreen bottles mean?[+/-]

SPF = Sun Protection Factor The higher the number the greater the protection. However, all sunscreens are removed by rubbing, sweating, and with water. So all sunscreens, regardless of the SPF Factor, need to be reapplied every two hours (yes, even if they state that they are “waterproof” or “sweatproof”). The American Academy of Dermatology recommends nothing less than an SPF 30.

Are tanning beds ok?[+/-]

No, No, NO. A tan or burn is *skin damage* whether you get it inside or outside.

What is “buffering” (used with lidocaine injections)?[+/-]

“Buffering” is when an additive is mixed with lidocaine that reduces the pain of the injection. You can ask if your dermatologist/doctor does this, and if they don’t, request that they do.

Any experience with numbing creams (ie Emla)?[+/-]

There are several creams of this type on the market. Some patients find them very effective, especially with children. They are typically applied to the area to be injected and left on for a period (approximately an hour), wiped off, and the injection proceeds. The cons are that they are messy, feel funny, and can be difficult to leave on certain areas for the required time.

Are there non-surgical BCC treatments available?[+/-]

There are topical creams that show some promise, and there is research being done in other areas, but nothing is as effective as surgery. Avoiding surgery should not be a motivation for trying such treatments. Use your pathology report to discuss your options with your doctor.

What is a “sub-type” of BCC? Why is it important?[+/-]

Basal Cell Carcinoma has several sub-types: “Superficial” is the least invasive, other types are nodular, cystic, and the most aggressive form is sclerosing (also called morpheaform). A biopsy and pathology report are the only definitive way to know the sub-type. This information can be very valuable to determine what type of treatment is needed. Because a BCCNS patient is looking at multiple treatments for the long-term, the attitude toward treatment can differ from the “typical” skin cancer patient who may only have a couple of surgeries in their lifetime. Informing yourself about all the sub-types and treatments is a valuable tool for the BCCNS patient.

What is a biopsy? How is it done?[+/-]

A biopsy is the removal of a small piece of skin to be sent to a lab for microscopic examination in order to confirm the presence of cancer cells. For the BCCNS patient, biopsies can seem redundant since we have so many BCC and we “know” what they look like. However, biopsies and pathology reports can give you even more information that can help determine your course of treatment. For instance, “marginal involvement” which tells you whether or not the biopsy actually removed the entire lesion; and “sub-type” which can help you determine how aggressive to be with your treatment. There are several different types of biopsies, but the most common are (1) Shave – where a thin layer of skin is removed, and (2) Punch – where a tubular scalpel is used to remove a “core sample” of skin. Biopsies are often required by insurance companies in order to approve other types of treatment.

What are milia?[+/-]

Milia are small whitish cysts that look like pimples and often develop around the eyes but can be found in other areas. They are not normally anything more than a cosmetic concern. As with anything, if they grow or change in some way, a biopsy should be considered.

What are palmar and plantar pits?[+/-]

The pits look similar to large pores and can be more noticeable when the skin has absorbed water (like after a bath). The words palmar and plantar refer to the areas where they are found most often – on the palms of the hands and the bottoms of the feet. The usual caution applies: if a pit should grow or change, have it looked at by a dermatologist.

Any advice on bandaging and caring for wounds?[+/-]

Most doctors will provide you with clear wound-care instructions and any questions should be referred to them. However, some patients have found themselves sensitive to bandage adhesive and antibiotic ointments, so care should be taken and advice should be sought out from your doctor and the Network if you have any of these issues.

What do BCCs look like?[+/-]

The various sub-types have different appearances and can vary (which is why a biopsy is highly recommended). Typically, skin cancers are “sores that don’t heal” or skin blemishes that appear or change suddenly. They can appear reddish and scaley with flaking or scabbing skin. They can also appear more nodular with rolled edges and/or a “pearly” appearance. Some patients have described “little volcanos” as they can often have an indentation in the center, and some can look like moles. The more aggressive sub-types can also be more indistinct, even looking like flat, white scars. A good health “law” is to listen to any signals that your body sends you and get anything suspicious checked, in addition to being seen regularly by a dermatologist.

Are there any herbal remedies or treatments?[+/-]

There are no herbal or homeopathic treatments that have been clinically proven successful, however, several BCCNS patients have found personal improvements through nutritional guidelines and supplements.

Is stress a factor in BCC growth/development? What about hormones (from puberty, pregnancy, menopause)?[+/-]

There definitely seems to be evidence that both stress and hormones play a part in BCC growth and development. The “average” skin cancer patients (and their doctors) don’t notice this since they do not deal with the numbers of BCC’s that BCCNS patients deal with. People connected with the Network, and doctors that have taken an interest in the condition, have noticed it. Taking steps to manage your stress, and being aware of the impact of hormones, may be helpful and beneficial.

Is there any research being done for BCC treatment?[+/-]

The BCCNS Life Support Network endeavors to keep patients informed of current studies/research and also maintain connections with doctors that are working toward better management and treatment of this condition. Contact the Network for current information.

Why did you set up your calcium calculator on a weekly basis instead of a daily basis?[+/-]

Most calcium calculators use your daily diet as the basis for calculating your daily dietary intake of calcium. For us, that proves difficult to calculate when very few people eat the exact same thing every day. When creating our calcium calculator, we decided to give you the opportunity to tell us what you eat on a weekly basis, and then we divide that number by seven to give you how much calcium you ingest in an average day. This just made more sense to us, and hopefully to you, too!

Why take a calcium dietary supplement?[+/-]

Most people do not consume enough calcium in their diet. Certain foods, mainly dairy products, are high in calcium. If a person does not consume enough of these types of food, they may become calcium deficient. To correct this deficiency a daily calcium supplement may be necessary. [Try our calcium calculator to find out how much calcium you are currently getting from your diet.]

Can I ignore my calcium needs until I am 30?[+/-]

No. Your bone density builds up in your early years. The denser bones are by age 30, the longer it will normally take for them to become thinner. It is vitally important that young people get enough calcium so that their bones remain strong and dense into their later years.

Why does your body need calcium?[+/-]

Your body uses the mineral calcium to build and maintain strong bones and teeth. Calcium is required by many cellular functions throughout your body. A small but absolutely essential amount of calcium is found in your blood and your body’s soft tissue to help with those cellular functions.

What happens if I don’t get enough calcium?[+/-]

Your body is an amazing creation. If you do not ingest enough calcium, your body will go to your bones and extract the calcium it needs. When this happens, your bones can become less dense (osteopenia) or brittle (osteoporosis) over time. We have all seen the elderly woman who is bent over – she is an example of a person who has osteoporosis.

Who is likely to have calcium deficiency?[+/-]

Basically we are all vulnerable. Starting at about age 30, bones begin to thin. As we grow older, our bodies reabsorb existing bone tissue faster than they can create new bone. Therefore, we need more calcium each day. In addition, when a woman goes through menopause she experiences a decline in the hormone estrogen. This causes her bones to thin faster than a man’s bones.