Beating Melanoma --The Survival Manual

A 5-step guide for patients with melanoma

Introduction

The incidence of melanoma has risen dramatically over the past five decades.  In 2008, it was estimated that 62,190 individuals were diagnosed with this skin cancer in the United States, and 7910 deaths occurred due to melanoma.  On an individual patient's level, melanoma can have a profound physical, psychological, emotional, and economic impact.  As a dermatologist and skin cancer specialist working at the Memorial Sloan-Kettering Cancer Center, I diagnose, treat and care for many patients with melanoma. In addition, I care for individuals who are at high risk for developing melanoma.  This has afforded me the opportunity to hear their stories. While some of these stories are moving, inspirational and touching, some are sad.  Through my interactions with melanoma patients,  I have gained a deeper appreciation and understanding of the difficulties and challenges faced by these patients, especially in the beginning when they are first diagnosed with melanoma.

Some of the common reactions experienced by my patients are surprise, denial, frustration, confusion, fear and even despair. However, this is not the time to panic.  Denial is not an option. You need to take action and take action fast.  Certainly, your physician will urge you to undergo treatment(s) as soon as possible and this urgency is necessary, but some of my patients report feeling  themselves losing control as soon as this process starts.

There is no doubt that you will go through an intense and stressful period from the time of diagnosis to completing the treatment. I call this first period the “mad rush.” Many of my patients agree, because they have to learn much about the disease, seek experts in their areas, and decide on treatment options. When they finally start treatment, many patients go through the process feeling a sense of chaos and the loss of control. Navigating the “mad rush” phase can be challenging for most, even for many health care professionals. The first portion of this book is aimed to guide you through this phase.  I will provide the 5 step-by-step instructions to guide you through the “mad rush” phase. The 5-steps will teach you the relevant vocabulary, medical background information, and basic treatment options, as well as survival outcomes tailored to different individuals’ conditions and the stages of illnesses.  Such knowledge can be extremely helpful when moving through the “mad rush” phase.

After a successful transition through this phase, many melanoma patients feel a transient relief.  Neverthelss, many soon grapple with a fresh wave of frightful questions and nagging concerns regarding their disease: “Will I develop new melanoma?” “Will I get other skin cancers?” “Do I need more treatments? What about my children, my siblings? Will they get skin cancer?” From this point onward, many will undergo what I call the “marathon” phase of dealing with their melanoma.  With each passing year, most melanoma survivors gain a deeper understanding of their disease and become more comfortable and reassured. The second portion of the book is aimed to help you through the extended “marathon” phase. Information in this section will address many of your lingering concerns and questions. It will help you to develop habits and practices that can prevent additional skin cancers.

Lastly, before launching into the book, I want to talk about cancer and our society’s attitude towards cancer. In this and many other countries, the word “cancer” still carries frightful connotations. Many people consider cancer as a death sentence. Luckily,  this is far from the truth. Modern medicine has made enormous strides in the diagnosis and treatment of cancer. Progress in computer technology, non-invasive imaging, gene therapy, and other new discoveries have changed the medical management of cancers. In fact, I would even go sofar as to state that many types of cancer have been transformed into a group of chronic (rather than terminal) diseases, much like high blood pressure and diabetes. While melanoma is the most dangerous type of skin cancer most patients who are diagnosed early have excellent prognosis and survival outcomes. Treatment for early melanoma is straightforward. Because of public awareness and improved diagnostic techniques, more patients today are diagnosed with melanoma at an early stage. If you are diagnosed with melanoma, the odss are it is likely at an early or intermediate stage of the disease that can be treated with relative ease. Treating cancer early usually means you will have excellent chance for survival.

Chapter 1. You Have Melanoma, Now What?

Stephanie is a 31 year-old woman with light blonde hair, deep blue eyes, and fair skin. As a child, she grew up near the Atlantic oceans and had numerous sunburns. This is her third visit to our melanoma clinic, and she is due for her semi-annual skin exam. She is a melanoma survivor. Her melanoma was located on the left leg. It was diagnosed and treated a few years ago. After a few pointed questions about her overall health, I started my exam. As I proceeded through my usual routine, Stephanie assured me that she is avoiding excessive sun exposure on daily basis. I nodded in approval. She was both nervous and happy when I did not spot any troublesome lesions on her upper body. As I moved to examine her legs, I leaned in for a closer look and palpated the long scar on the left leg, the lasting remnant of her melanoma surgery, to check for any recurrence of the melanoma. She was silent and tense. Sensing her anxiety, I reassured her that everything looked good. As I completed the rest of my exam, I again reassured her that she was doing very well. The scar on her left leg was healing well. I did not see any moles or spots that were worrisome. As she listened, her eyes and left hand wandered to the scar on her left leg for a minute before she smiled in relief.

Stephanie’s melanoma was diagnosed more than 3 years ago in a fortuitous manner. She was working as a pharmaceutical representative visitng on doctors in their offices to educate them about the latest drugs from her company. During a summer visit, a nurse pointed out a dark mole on her left leg and urged her to see a dermatologist about it. Heeding the warning, Stephanie made an appointment with her dermatologist who shared the same concern and removed the mole. One week later, Stephanie received a phone call. The dermatologist shared the bad news, “Stephanie, the mole we removed from your leg was a melanoma. It is a type of skin cancer that needs to be treated quickly.” Stephanie was stunned at first. As she listened, her dermatologist told her other information about the disease, but Stephanie did not remember any of this. When she hung up the phone, all she remembered was that melanoma is a type of skin cancer. Judging by the urgency and tone of her dermatologist, she knew it  was something serious. One last thing she remembered was she had an appointment the following week for surgery.

That night, she went home and told her husband John about the diagnosis. They had heard about melanoma, but did not know too much about it. Soon, they both had many questions, but the hour was late to call her doctor. So they turned on the computer and started their own research on the Internet. Together they jumped from one site to the next and tried to grasp as much as possible about melanoma. They encountered confusing terms, such as Breslow thickness, sentinel lymph node biopsies, isolated limb perfusion and Interferons. They came across alarming warnings --“melanoma is the most dangerous type of skin cancer… One American dies from melanoma every hour… Lymph node dissection may be needed… Patient’s needs chemotherapy… Currently, there is no cure for advanced stage melanoma.” Stephanie recalled that sitting beside her husband sifting through those sites was a terrible experience. All the information was scattered. The medical terminologies confused her. The statistics and warnings terrified her. To make matters worse, some of the reputable sites had similar warnings. All she could think about was the impending big surgery, possible lymph node dissection, and possible chemotherapy. All kinds of thoughts raced through her head. As she tucked in her baby girl that night, tears streamed down her face. She could not read or think about this any more, and she could not wait for her next week’s appointment. She decided that she would trust her dermatologist and hope for the best. Fortunately, Stephanie’s melanoma was at a very early stage. She only needed surgery to remove the tumor. There was no need for chemotherapy or other additional treatment.

Unfortunately, Stephanie's experience is all too common. Many melanoma survivors have similar experiences after they are told about their diagnosis of melanoma. Many experience the same fear, frustration and worry in their quest to learn about the disease at this initial stage. Aside from their physicians, many seek advice or turn to their friends or family members who had melanomas. Some buy books. Other seek answers through the internet. Unfortunately, the information from many of these websites is fragmented and not relevant for most patients. Worst of all, some of the information is confusing, and this uncertainty frightens many patients. Finally, many patients hate the feeling of losing control as they rush ahead with their treatments.

There is a real need for a comprehensive and succinct guide that can help patients navigate through the “mad rush” phase of moving from diagnosis to treatment. During this period, you will meet different specialists who will consult you, help educate you, and work with you to come up with the best treatment plan. Many unfamiliar terminologies, statistics, and data will be thrown at you, and you may need to make some serious decisions. Again, all this will happen quickly. That is why I called this phase – “the Mad Rush.” Hence it is imperative to understand that critical steps have to be taken from the time of the initial diagnosis to the treatment.

I sincerely believe the best way to navigate through the “Mad Rush” phase is having accurate up to date information about melanoma as well as having support from friends and family. In the next sections of this book, I will share the 5-step plan that I believe will help you:

Step 1. Getting the information - the pathology report.
Step 2. Assessing the reliability of the information.
Step 3. Finding the clinical experts in your area.
Step 4. Knowing the treatment options by understanding the AJCC staging the systems.
Step 5. Understanding the survival rate or prognosis.

 

Chapter 2. Navigating Through The "Mad Rush" Phase

2.1.  Step 1 -- Getting the Information – the Pathology Report

A pathology report is an important medical document that includes important information about the diagnosis.  The report is generated by a pathologist or dermatopathologist (I’ll explain the difference momentarily). After your clinician (e.g., dermatologist or family physician) performs the skin biopsy, the tissue sample is sent to a laboratory.  The tissue is processed through a series of steps.  First, the tissue is cut into very thin slices, 4 to 20 micrometer (um) in thickness.  This is thinner than the width of an average human hair. These skin slices are placed on a glass slide and stained with special dyes that help the physician (i.e., pathologist) see the diagnostic features of the sample.  Based on the tissues’ appearance and cell shapes and morphology, a diagnosis is rendered.  Finally, a written pathology report is generated.  This report is crucial. The information within this report will determine the management plan and can help predict the probability of surviving.  The report is mailed, faxed or delivered electronically to the clinician (the person who performed the biopsy), and the information will be conveyed to the patient.

Why do you need this report?

First, once you are given the diagnosis of melanoma, you will embark on a long road of medical care: you will work with many physicians from different specialties.  At each encounter, a physician will ask for a set of basic information in order to plan treatment and decide follow-up care.  Virtually all these physicians will want to know the information in your pathology report. With this important document in hand, you will make each visit more productive and efficient.  Without it, your physician will not have the critical information to plan the appropriate treatment. Most physicians will not rely on the patients’ words alone; they will want to see definitive evidence (i.e., the pathology report) before starting any treatment. Without the report in hand, your treatment and your care may be delayed. I assure you:  this type of delay can happen, and you want to avoid it.

Second, you are about to work with medical professionals to tackle a serious disease. I trust you will want to know as much about this disease as possible in order to make sound decisions. I know these esoteric medical terminologies may be confusing, but that should not stop you from becoming educated about your melanoma diagnosis. We educated ourselves in other areas of our lives, for example before making an expensive purchase, such as a house, car, plasma TV, stocks or appliances. The same principle applies here.  Knowledge is power, and you need to be informed.  The information contained in the pathology report is the first and necessary step in the learning process.

Third, as I said in the introduction, there are two phases in dealing with melanoma: (1) the Mad Rush and (2) the Marathon. The second phase may last many years when you need continual follow-up with dermatologist and other physicians.  During this time, you may move to another area or you may change physicians.  As you meet each new physician who treats your melanoma, he or she will want to know a great deal more than the simple answer "Yes, I had melanoma." Don’t trust your memory. You will invariably forget many crucial pieces information contained in this pathology report.

I hope you are convinced.  Obtaining the report is very easy. Just ask the dermatologist or the physician who performed the biopsy for a copy. By law, the physician has to give it to you.

Understanding the report

The pathology report can be confusing for a layperson.  Medical jargon and technical terms turn most people off. But let me assure you:  the information contained in the report is worth learning.  Here is some basic information you need to grasp, and a few basic things you need to do when you get your report.  See Figure 2.1 for a sample report.

Figure 2.1. An Example of A Pathology Report

A) Basic information:

- Verify your name, age (date of birth), and verify the date the biopsy specimen was submitted to the laboratory. Make sure they are correct on the report. The reason should be obvious. You need to be sure this report is for you not someone else.

- The report will contain the contact information and name of the physician who performed the biopsy.

- There should also be a name and contact information of the pathologist or dermatopathologist who rendered this diagnosis on this pathology report.

B) Diagnosis and description:

Different pathology labs generate their reports in different styles.  However, each has the same necessary elements: the diagnosis, microscopic description and macroscopic description.  The microscopic description discusses the tissue features seen under the microscope, and the macroscopic description details the tissue specimen when it first arrives in the lab before any tissue processing.  For our purposes, we need only to focus on the diagnosis and microscopic description and on a few key elements that you must pay attention to:

1.  Location: make sure the correct anatomic site is labeled.  Check to make sure left vs. right. This is a common mistake. If the biopsy was on the leg, make sure the report states “leg” rather than some other parts of the body.

2. Diagnosis: make sure the diagnosis of melanoma is clearly stated. If after reading the diagnosis and description you don’t see a definitive statement, be sure to ask your dermatologist for an explanation. Sometimes, it may be a good idea to have another pathologist or dermatopathologist review the slide (read Step 2. Assess the reliability of the information for more information).

3. Is it “in situ”? This is the next question you should keep in mind when reading your pathology report. “In situ” means the tumor has not penetrated beyond the epidermis (outer layer of the skin).  If the pathology report states that the melanoma is only an “in situ” lesion, the treatment is very straight forward (i.e., simple removal). Chances for survival and cure are excellent with in situ tumors. Please note that, in the report, the wording may be “Melanoma in situ.” If your melanoma is an in situ lesion, you can skip the item #4 to #8 below as these information will not be applicable to your case.

4. Is it invasive? If so, what is the Breslow thickness? In general, if the melanoma is not an “in situ” lesion, it is an invasive lesion. Invasive melanoma means the tumor has penetrated the dermis (below the outer  layer of the skin). If the report clearly states “invasive melanoma,” then look for the phrase "Breslow thickness." THIS IS ONE OF THE MOST IMPORTANT PIECES OF INFORMATION! Breslow thickness measures the depth of penetration by the tumor cells.  It is measured from the very top of the epidermis (first layer of the skin) to the deepest melanoma cells in the skin tissue (See figure 2.2).  The unit of measurement is millimeters. If you have trouble in finding this information, look for a numerical number followed by the abbreviation "mm" (e.g.,  0.5 mm, 1.2mm or 0.2mm). Again, let me reiterate: Breslow thickness is a vital piece of information. This will dictate the stage of the disease, treatment options and survival rate. It is important.

Figure 2.2. Breslow thickness measurement. The depth is measured from the very top of the epidermis to the deepest melanoma cells in the skin.

5. Clark level: Clark level is another way to express the depth of melanoma invasion (see figure 2.3). Clark level is a 5-level grading scale.

• Level I describes tumor growth along the junction between the epidermis (first layer of the skin) and the dermis (second layer of the skin).
• Level II describes lesions that have penetrated the first layer of the skin, but only reached the papillary dermis, a very superficial part of the dermis (second layer of the skin).
• Level III describes lesions that have extended to the border of the papillary (superficial dermis) and reticular (deep dermis) layer of the dermis.
• Level IV describes lesions have penetrated directly into the reticular layer of the dermis.
• Level V describes lesions have extended into the subcuataneous layer or the fatty tissue of the skin.

Figure 2.3 Clark's Level Measurement

IT IS IMPORTANT NOT TO CONFUSE CLARK LEVEL WITH STAGE. THE NUMBER FOR CLARK LEVEL HAS COMPLETELY DIFFERENT MEANING THAN THE NUMBER FOR STAGE OF THE DISEASE. For example, Clark level III does not mean stage III disease.  Stages of disease will be discussed in later section (Step 4 – Knowing the treatment option by understanding the AJCC staging system).

6. Ulceration: As the name suggests, indicates a loss of the intact skin overlying the tumor.   Presence of ulceration often indicates advanced stages of disease.

7. Mitosis: This term indicates cells undergoing division, splitting from one cell to two cells. As a tumor expands, the number of tumor cells increases. The number of tumor cells undergoing  mitosis reflects the activity of the tumor.  In general, tumors with a high number of mitoses (cell divisions) are more worrisome and aggressive.

8. Lymphocytic Infiltration: lymphocytes are the body's immune cells. They play a major role in fighting and destroying melanoma cells.  Therapies using vaccines or interferon aim to boost the ability of these cells to track, target, and destroy the melanoma cells.  The presence of brisk or active lymphocytes in a melanoma is a good sign.

It is important to keep in mind that different pathologists or dermatopathologists provide different degrees of    detail in the reports they generate.  In general, most reports will contain information #1 to 5.  Again, for in situ melanoma, the information in #4 to #8 may not be included in the report, because it is not applicable.

2.2. Step 2 -- Assessing the Reliability of the Information

Which specialist makes the diagnosis?

I have used the terms, pathologist and dermatopathologist, on a number of occasions in the above section.  At this point, you may guess that both are types of physicians who examine the tissue under a microscope, render a diagnosis, and generate the pathology report. What are the difference between a pathologist and dermatopathologist?

Pathologists are trained to examine tissue from all parts of human body, such as the brain, muscle, colon, heart, bone and other tissue. By and large, most pathologists do not specialize in looking at skin tissues. They do not exclusively examine the skin tissue on daily basis. By contrast, dermatopathologists are dermatologists or pathologists who have received additional training in examining skin specimens. They are focused on skin diseases. In general, dermatopathologists are more knowledgeable when it comes to skin disease. However, the important point is not whether a pathologist or a dermatopathologist reviewed the slides and made the diagnosis, but rather whether the physician who made the diagnosis is experienced and knowledgeable when comes to melanoma.

Why should I care who made the diagnosis and generated the pathology report?

When it comes to melanoma, I believe it is crucial to have a knowledgeable and experienced physician study the tissue under the microscope and make the final diagnosis.  I learned this lesson very early on in my medical training.

There are a number of reasons why you should step back and assess the reliability of the diagnosis.  First, there are skin lesions or tumors that can look like melanomas both clinically and under the microscope.  I have personally seen a number of cases when the diagnosis of melanoma was reversed to a severe dysplastic nevus (a type of atypical mole) after an experienced dermatopathologist or pathologist was asked to render a second opinion on a pathology slide.  Another type of benign mole, a Spitz nevus, can also mimic melanoma.  I vividly remember a young woman who had tears of relief after learning that she did not have a deeply invasive melanoma. Instead, she had a Spitz nevus.  As a result of this change in the final diagnosis, she did not need to undergo a very large excision, sentinel lymph node biopsy (see explanation in a later section), or other more aggressive treatments.  We helped her avoid many of the unnecessary problems associated with the extensive surgery and toxic systemic treatments.

Second, in the last section, I mentioned a number of elements in the pathology report that are important.  The most critical element is the Breslow thickness.  This measures the depth of melanoma cells in the skin. This information dictates the treatment plan (See Step 4. Know the treatment options by understanding the AJCC staging the systems). Patients with thicker Breslow measurements need more aggressive treatment. It is also used as a prognostic indicator for predicting survival outcome (See Step 5. Understand the Survival Rate and Prognosis). Hence, an accurate measurement of the Breslow thickness is very important. However, the measurement of the deepest tumor cells in the skin tissue can be a challenge in certain cases. Like many high skill tasks, experience and knowledge matter. Inaccurate measurement of the tumor thickness can have dire consequences. Overestimation of the Breslow thickness can lead to an unnecessarily large surgery, longer surgical scars, and slower recovery time. Even worse, an overestimation of tumor thickness may lead to more invasive surgical procedures such as a lymph node biopsy or chemotherapy. On the other hand, underestimation of the Breslow thickness can lead to inadequate surgical intervention and appropriate treatment, which have the potential for causing tumor to recur or spread. In fact, this point is proven in a clinical study conducted by a group from Netherlands. The investigators showed that the results of 1887 lesions that were submitted as a second opinion to the expert dermatopathologists/pathologists of the Dutch Melanoma Working Group Pathology Panel for consultation. The results showed that the experts changed the diagnoses in 27% of the cases. That is more than one in every four samples.

How do you verify the accuracy and reliability of the pathology report

The easiest approach is to ask your dermatologist or the clinician who performed the biopsy of the initial skin lesion.  Ask your dermatologist if he or she has confidence in the pathologist or dermatopathologist who read the slides. Another clue is to look at the pathology report itself.  Usually at the end of the report, the pathologist or dermatopathologist will state if he or she is board certified in pathology or dermatopathology.

What to do when you're not satisfied with the reliability and accuracy of the diagnosis?

There are times when the diagnosis stated in a pathology report is not clear. The pathologist or dermatopathologist may simply give a microscopic description loaded with detailed discussion, but not state the diagnosis. This is because in biology, as well as in nature in general, things are not always black and white – sometimes, they’re grey.  In other words, at times it is difficult to know if a lesion is a melanoma or severe atypical nevus. Also, you or your physician may wish to seek a second opinion, and have the slides reviewed by an experienced dermatopathologist. Just ask your physician to “please have the slides reviewed by another dermatopathologist to confirm the histologic diagnosis.” (I specifically placed the text in quotes, because that is what many dermatologists say to each other in consultation of difficult cases). If you don’t feel comfortable using the above language, here is another way to make the request. “Dr. Smith, I trust your judgment. Do you think it may be a good idea to have another dermatopathologist or pathologist who is experienced in melanoma to review the slides and to confirm the diagnosis?” Remember, it is a good idea to have a board certified and experienced dermatopathologist or pathologist review your slides.

If you and your physician decide to have the slides reviewed, it is important to move through this process quickly.  The process usually takes 4 to 10 days. It usually involves mailing of slides from one lab to another lab. The consultant dermatopathologist or pathologist will review the slides and make his or her diagnosis. The results will be communicated to your physician through phone calls, email, or fax. Again, this should be accomplished in a speedy manner.

When you receive the second diagnosis from your physician, proceed with the same action stated in Step 1 – obtaining the pathology report. Now you will have two reports in hand. You can compare them.  Of course your dermatologist should explain the results to you in detail. In checking the second pathology report, here are few important steps:

1.      Confirm the report is for you not someone else. Again check for name and date of birth. Also, check for the pathology accession number in both reports. Make sure the pathology accession numbers on both reports are identical. This means that the new dermatopathologist has looked at the same specimen.

2.      Confirm the report is for the correct anatomic site. For example, if you had 3 biospsies but only the lesion on the left leg was in question, make sure the second report is generated for the left leg and not for the other two lesions. Although mistakes are rare, they can happen. I have seen it.

3.      Compare the diagnosis. Is it melanoma? If so, is it invasive or is it in situ? If it is invasive, compare the Breslow thickness measurement in both reports.

If the two sets of independent reviews of your slides come to the same conclusion, you will have assurance of the accuracy and reliability of your diagnosis.

What happens if reports conflict with one another?

Again, it does not occur frequently, but it can happen. In those cases, you can have a third dermatopathologist or pathologist review the slides.

 

Table of Contents:

Suggestions To the Readers

Introduction

Chapter 1. You Have Melanoma, Now What?

Chapter 2. The 5-Step Plan: Navigating Through the “Mad Rush” Phase

2.1. Step 1 – Getting the Information – the Pathology Report

-Why do you need this report?

-Understanding the report.

2.2. Step 2 – Assessing the Reliability of the Information

-Which specialist makes the diagnosis?

-Why should you care?

-How to verify the accuracy of the information?

-What to do when you are not satisfied with the reliability and accuracy of the

Diagnosis?

-What happen if reports conflict with one another?

2.3. Step 3 -- Find the Clinical Experts In Your Area

-Stacy and Sarah’s stories

-How to find the experts in your area

-What happens if you cannot find the experts in your area?

2.4. Step 4 -- Knowing the Treatment Options by Understanding the AJCC Staging System

-What is the AJCC system?

-How to interpret the AJCC staging system

-How does your doctor determine the stage of your disease?

-Knowing your treatment options.

2.5. Step 5 -- Understand the Survival Rate or Prognosis

Chapter 3. “The Marathon” Phase – Surviving Melanoma

3.1. Section 1 -- Medical Follow-Up

-Why do you need continual follow-ups?

-Which specialist or specialists should you see for follow-ups?

-How frequently should you have follow-up exams?

-What to expect during follow-up visits

3.2. Section 2 -- Who Is At Risk For Developing Melanoma?

-What is your skin color?

-How much sun exposure have you had?

-What is your personal and family history?

-Do you have large moles found at birth?

3.3. Section 3 -- How to Prevent Melanoma?

-Primary Prevention -- ways to avoid excessive UV exposure

-Sun avoidance and shade

-Hats and clothing

-Sunscreens

-Secondary Prevention -- How to detect melanoma at an early stage

-Clinical Exam

-Total body skin exam

-Dermoscopy

-Total body photography

-Digital clinical and dermoscopy photos

-What should you look for?

-Use the ABCDE rule

-Look for the Ugly Duckling”sign

-Pay attention to symptoms.

-Why should you not use tanning booths?

3.4. Section 4 -- Current & New Medical Developments

-Computer assisted diagnostic devices

-MoleSafe

-Confocal laser microscopy

-Tape stripping research

Chapter 4. Closing Remarks

Bonus #1: Basal Cell Cancer

Bonus #2: Squamous Cell Cancer

Bonus #3: Beating Melanoma Checklist

Bonus #4: Melanoma Image Library