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CASE STUDY: Merkel Cell and CLL (B-cell)

Case Study:
Merkel Cell Carcinoma and Chronic Lymphocytic Leukemia (B-cell)
Jessie Fredericks
Argosy University Twin Cities
Julie Yasgar

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CASE STUDY: Merkel Cell and CLL (B-cell)

Introduction
In This case study, an older male patient is treated for Merkel cell
carcinoma and chronic lymphocytic leukemia (B-cell). The patients medical
history, diagnosis and set-up/treatment planning is described in this case
study. Unfortunately, B-cell is an unfavorable prognosis for CLL patients, but
the patient had a wonderful outlook on his diagnosis and his life . It was a
great experience being able to watch this patients progress throughout his
treatment, all the way until the end.
Case study
Consult
Patient X is a 57 year old obese Caucasian male. Patient X went into
their primary doctor with complaints of night sweats, fatigue and satiety over
the past 3-6 months. After blood test and labs Patient X was referred over to
a medical oncologist for further testing. After labs, biopsy and scans, the
patient was diagnosed with Merkel cell carcinoma and chronic lymphocytic
leukemia (B-cell).
Before being diagnosed, the patients medical history was sleep apnea
and acid reflux. The patient had a Nissen Fundoplication 10 years ago for his
reflux, but no other surgeries. His family history is positive for Non-hodgkins
lymphoma, and his mother passed away at age 84 from a stroke. Patient
does not use alcohol or tobacco, and is a construction signal worker. A
physical examination was done to assess the patient. The patients overall

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CASE STUDY: Merkel Cell and CLL (B-cell)

appearance was clean and alert. He showed no signs of depression of

conflicting anger. The patient was found obese, but his vitals were all in
normal ranges. Normal respiratory, blood pressure and no edema. Patient
had enlarged lymph nodes around supraclave and neck region, along with a
large mass on his right trunk (mid-lower back) measuring 3.6 x 7.5 cm.
After the physical examination, the patient had multiple scans and
biopsies done to diagnosis Patient X. During the patients CT he was given
intravenous contrast and oral contrast. Patient was positioned prone, with
pillows for support and comfort. 5 mm axial images were obtained for the
chest, abdomen, and pelvis. During the CT the patient had a CT guided
biopsy done to the right soft tissue flank mass. The doctor used a 17 gauge
needle for the core biopsy, then she did 5, 18 gauge, 1.3 cm core biopsies.
The core samples showed two distinct histological patterns. 1. Diffuse
infiltrate of small lymphoid cells, 2. Epithelial configuration, malignant
neuroendocrine tumor (Merkel Cell Carcinoma). The CT scans showed
abnormal enlarged lymph nodes in the supraclavicular and axillary region
bilaterally measuring 2.9 cm in size., prevascular lymph nodes were present
measuring 2.3 cm in size cranial lymph nodes 3.7 cm, and an abnormal
spleen enlargement measuring 18.3 cm. Patient also received a PET scan
and the images were fused with the CT to later assist in treatment planning.
For the PET 15.08 mCi of F-18 FDG were injected for the exam. The scans
showed enlarged hypermetabolic lymph nodes throughout all nodal station in
the neck, along with abnormally enlarged supraclavicular, retropectoral and

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CASE STUDY: Merkel Cell and CLL (B-cell)

axillary lymph nodes. Also, showed extensive abnormal hypermetabolic

mesenteric and retroperitoneal adenopathy above and below the diaphragm.
After all the core biopsies and PET/CT scans were reviewed and
checked the patient was diagnosed with Stage 2 merkel cell carcinoma on
8/23/2016, with chronic lymphocytic leukemia, B-cell. Patient X was referred
over to Dr. McElveen at Radiotherapy Clinic of Georgia, Snellville, to being
radiation treatments.
Simulation
Patient X came into RCOG, Snellville, on 8/25/2016 for his CT
simulation. He was positioned in a new set-up than his first CT scan. Patient
X was positioned supine with a wing board, B6 grip, F head rest, with a vac
loc abutting the wingboard and a U-frame around his legs. 5 mm slices were
obtained from base of skull down to pelvis region. After the CT was
confirmed, tattoos (paint pen marks) were given to the patient on each
lateral and midline, based on the CT lasers. He also got tattoos on his
arms to insure accurate placement daily for treatments. After the CT was
obtained the doctor looked over the scans and prescribed a prescription for
the patient. Dosimetry began working on determing the accurate blocking a
calculation needed for the treatment. Contours were made and all structures
were outline, critical structures were avoided with MLC blocking. Critical
structures in this treatment included the liver, kidney and spinal cord. After

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CASE STUDY: Merkel Cell and CLL (B-cell)

reviewing the plan the dosimetrist made the doctor approved the plan for
treatment.
The treatment card/ doctors orders read 60 Gy total to the right flank,
2 cGy/ fraction for 30 fractions, bolus for first 7 treatment. Treatment was
delivered using static IMRT. KV images were ordered daily, with a cone-beam
done once a week. IMRT was the best approach to use the MLCs to block
critical structures, no wedges were needed to lessen hotspots. Treatment
was given at 2 gantry angles using 2 different energies. First angle was RAO
at gantry angle 103* using 10 MV energies, then the gantry rotated to LPO at
291.5* using 10 MV energies. The gantry would then rotate back to RAO at
103* and treat with 15 MV energies, then back to LPO at 291.5* using 15 MV
energies. They planned the treatment with two different energies to get a
deeper dose deposition to the treatment site. Patient X was scheduled to
begin treatment on 9/7/2016, and finish 10/18/2016
Treatment/ doctor visits
During Patient X treatment, he was positioned just as he was in the CT
simulation. Supine, wingboard, B6 grip, F head rest, vac loc abutting
wingboard and a U-frame. Patient X was lined up using the tattoos given to
him during the CT simulation and position was verified with KV orthogonal
images lining up to the patients bony anatomy. KV images were done daily
as prescribed by the doctor, the therapist would match the images taken
daily to the images obtained during the CT simulation. After images were

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CASE STUDY: Merkel Cell and CLL (B-cell)

verified by two different therapist, we would begin delivering the radiation.

Patient X always came in, in a good mood, making jokes and laughing with
the therapist. He never complained of being in pain or having any
discomfort. As his treatments went on he loved showing off his right flank
mass, and showing how much it has shrank in size. He would also discuss
this with his physician during their weekly visits. The doctor also noticed his
upbeat personality and shrinking mass. As documented in her note she
discussed how he was dropping weight, but not at an alarming weight, which
was a good thing. His vitals were all still in check and his mass was shrinking
. They discusses how he was feeling, any tenderness in his right side and if
he had any concerns about side effects with his treatment. As a student
observer I did not interfere with the doctor or patient while they talked, but it
was unique to see how the patient reacted differently with the doctor
opposed to how he acted with the therapist. He seemed more professional
around the doctor, and would let her know more about little things that
worried him. Such as sleeping habits changing, or feeling more fatigued
than normal, but nothing to concerning. As his treatments progressed he
started to mention tenderness of his right flank and feeling fatigued, which
he discussed with the Dr. Around Patient X 20th fraction he said he was not
feeling well and has been under the weather. We proceeded with his
treatment on that day with okay from both the patient and the doctor. On
October 7th Patient X was admitted into the hospital for puking up blood. On
October 25th the patient decided to go to hospice care at home, where on

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CASE STUDY: Merkel Cell and CLL (B-cell)

October 28th he had a stroke and passed away. Dr. McElveen did go to the
hospital to do a follow-up visit with him and see how he was doing. Patient X
personality showed signs of depression even though he tried to keep a brave
face for his wife. He expressed stopping the radiation treatments and
moving on to hospice care.
Usually in a follow-up visit it would occur 4 weeks after the patients
final treatment date and then every 6 months for 3 years after that. In the
follow-up visit there is a physical examination to asses the patient, vitals
would be checked and some blood work may be ordered. The doctor would
talk with the patient on how he is doing? If there is any side effects of
treatment he is concerned about, or if he is having any issues. The doctor
would then look over his treatment site to see how it is recovering and if
there is any signs of latent effects on the patients skin. She would talk
about the follow-up care he should be doing, such as which ointments to use
and how to care for his mass. She would also discuss the possibility of
recurrence and the plan of action they would take if it ever came to that.
The doctor would answer any question the patient and his wife would have
and would continue to monitor the patient.
Research and Analysis
Etiology and Epidemiology
Merkel cell is an aggressive, rare, neuroendocrine tumor of the skin. It
has a 5 year mortality rate in 46% of patients, and effects about 1,600 new

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CASE STUDY: Merkel Cell and CLL (B-cell)

cases a year. It is not clear what exactly causes Merkel cell, yet it is more
common in highly sun-exposed skin areas such as the head/neck and arms,
but it can occur anywhere on the body. (Merkelcell.org, 2016). It is more
likely to effect older people above the age of 57 year old and people with a
weaker immune system. Merkel cell is a deadly disease with a poor
prognosis. There is local recurrence in 44% of patients and multiple
recurrence in 15%, happening within 5 months after treatment. (Pearson, J,
MD, 2015). 34% of all patients will get distant mets, and mortality rate with
distance mets is 75-100%.
For Chronic Lymphocytic Leukemia (B-cell), the exact cause is
uncertain, yet B-cell CLL is the only leukemia not associated with radiation
exposure. (Fayad,L,MD and OBrien,S,MD, 2015). There is a higher incidence
rate among whites than blacks and is higher in males than in females 1.7:1
ratio. Median age for CLL is 58 years, but can occur at any age. Prognosis
depends on disease stage at diagnosis, but most patients live 5-10 years. If
metastasize are involved patients average prognosis is 2-3 years.

Symptoms and Causes

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CASE STUDY: Merkel Cell and CLL (B-cell)

First sign of Merkel cell carcinoma is usually a fast-growing, painless

nodule on your skin. (Mayo Staff, 2015). Merkel cells are found in the
epidermis of our skin, and are responsible for the sense of touch, so as the
tumor grows the skin may appear red or purple and become painful to touch.
They may spread to surrounding skin, or to nearby lymph nodes. (Cancer.org,
2016). Excessive exposure to artificial or natural sunlight may increase your
risk of getting Merkel cell, along with older age. Your risk increase as you
age, most common in people over 50 years of age. It also more common in
lighter skinned people.
For CLL (B-cell), most patients have no early symptoms, for those who
may experience early symptoms they may have: enlarged, painless, lymph
nodes, fatigue, fever, night sweats, and weight loss. Just like many cancers,
doctors are not certain what causes CLL. What doctors do know is that
something has to happen to cause a genetic mutation in the DNA of blood
producing cell, which causes the production of abnormal, ineffective
lymphocytes. (Mayo clinic, 2015). Doctors and researchers are working
together to understand what causes the genetic mutation of CLL. Risk
factors for increasing your chance of getting CLL are increasing age, family
history of blood or bone marrow cancer, and your race. Whites are more
likely to develop CLL than any other race. (Mayo Clinic, 2016).

Diagnosis and Treatment

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CASE STUDY: Merkel Cell and CLL (B-cell)

In order to diagnosis Merkel cell carcinoma, there is a series of test and

biopsy that need to be done. A physical examination is done to check for
unusual moles, freckels, pigmented spots or any abnormal growths on the
skin. A physical examination usually leads to lab and biopsy of the abnormal
mass. The diagnosis of Merkel cell can only be made with a skin biopsy,
either a core biopsy or shaving part of the top lesion with a scalpel.
(Merkelcell.org, 2016). After the biopsy is obtained, a special stain is used to
determine if it is Merkel cell or another form of cancer. The stains are called
immunohistochemistry stains, it shows the unique characteristics of Merkel
cell. After the biopsy patients will either undergo a CT scan or a PET-CT of
the chest, abdomen and pelvis region to determine if there is evidence the
cancer has already spread. After all the test and scans are reviewed and
studied, the TNM staging system is used to determine a patients stage and
grade. For Merkel cell carcinoma the staging system was based on an
analysis of over 5000 patients using the Natioanl Cancer Database as stated
in 8th Edition of the AJCC Staging Manual. Stages I & II is localized to the skin
at the primary site. Stage I: is for less than or equal to 2 centimeters lesions.
Stage II: is for lesions greater than 2 centimeters. Stage III is for disease that
involves nearby lymph nodes. Stage IV is disease found beyond regional
lymph nodes. After diagnosis of Merkel cell, doctors will being to prepare a
custom treatment plan for that patient.
The treatment decisions usually depend on tumor stage, general
health and location/size of the mass. Popular treatment options for patients

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CASE STUDY: Merkel Cell and CLL (B-cell)

with Merkel cell are Surgery, radiation therapy and chemotherapy. The goal
of surgery is to remove the tumor so that there is no recurrence near the
primary site or in the lymph nodes. To obtain this the whole tumor must be
removed with clear margins, and a >2cm margin around the tumor.
(Merkelcell.org, 2016). Yet, even with clean margin, surgery alone can have
a high recurrence rate up to 42%, but it can be cut to less than 5% if
combined with radiation therapy. Radiation therapy is delivered to the
cancer cells and a margin of surrounding normal tissue. Radiation therapy is
used with surgery to destroy any cancer cells that may remain after surgery
has removed the visible tumor. The goal of radiation is to damage the
genetic material of cancer cells making them unbale to grow.
(Merkelcell.org, 2016). Radiation total dose is usually equal to or greater
than 50Gy, and administered for 5-7 weeks, 5 days a week. Studies have
shown radiotherapy significantly improve the local and nodal recurrence,
some may also suggest it improves a patients chance of survival. Another
form of treatment is chemotherapy. Chemotherapy may be used to destroy
cancer cells that may remain after surgery and/or radiation therapy. Since
Merkel cell has a high recurrence rate and quickly gains resistance, the
tumor can start to grow again despite receiving radiation, surgery and
chemotherapy. The chemotherapy drugs may be given intravenously or
orally over a set number of weeks to kill the cancer cells in the body.
Chemotherapy is not as common as surgery and radiation because most
patients are elderly and it effects their immune system, quality of life and

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CASE STUDY: Merkel Cell and CLL (B-cell)

has a 4 to 7% acute death rate. (Merkelcell.org, 2016). So the best

treatment option for patients with Merkel cell carcinoma is surgery combined
with radiation thearpy
Diagnosis of Chronic lymphocytic leukemia are usually obtained through
tests and procedures. A patients medical history will be checked for
symptoms and possible risk factors for CLL, and the patients general health
will be taken into account. A physical examination will also provide
information about the patients health, paying close attention to lymph
nodes. A complete blood count will be tested to measure the different cells
in the blood, such as red blood cell, white blood cells, and the platelets.
People with CLL have a higher than normal white blood cell count and might
have too few red blood cell and blood platelets. (Fayad,L,MD and
OBrien,S,MD, 2016). Another test that is very important in diagnosing CLL is
a flow cytometry. This test can see if a lymphocytes contain CLL cells. CLL
cells have a marker called CD5, which is normally found on T-cells, but not on
normal B-cells. In order for someone to be diagnosed with CLL (B-cell), there
must be at least 5,000 CD5 (per mm3) in the blood. (Fayad,L,MD and
OBrien,S,MD, 2016). Imaging test such as CT, PET-CT, and ultrasound are
also done to determine spread of cancer and to help determine the best
treatment options. Determining the stage for CLL is different than Merkel cell
in the way they use two different staging systems. CLL staging is based off
of the Rai stages and Bienet classification. The Rai stages have 5 different
stages: Stage 0 only has high levels of lymphocytes. Stage I has high levels

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CASE STUDY: Merkel Cell and CLL (B-cell)

of lymphoctyes and enlarged lymph nodes. Stage II has high level of

lymphoctyes, enlarged lymph nodes and enlarged spleen. Stage III has high
levels of lymphocytes, may or may not have enlarged lymph nodes, enlarged
spleen and anemia. Stage IV has all of the symptoms listed above and low
levels of platelets. For the Binet staging, the stages are listed in A, B, and C.
Stage A: The patient does not have anemia or low platelets and the leukemia
can be felt in fewer than 3 areas of lymph nodes. Stage B: The patient does
not have anemia or low platelets and the leukemia is in 3 or more areas of
lymph nodes. Stage C: The patient has anemia and/or low levels or platelets
and the leukemia is in many number of lymph nodes.
Treatment of CLL is mainly chemotherapy, since leukemia usually
spreads throughout the body. Doctors would give chemotherapy in cycles,
with each treatment followed by a rest period to allow the body time to
recover. Chemotherapy is usually given in 3 to 4 weeks, with adequate rest
time in between treatments. Major types of chemo drugs used to treat CLL
include: Purine analogs; fludarabine, pentostatin, and cladribine. And
Alkylating agents; chlorambucil and cyclophosphamide. (American Cancer
Society, 2015). Chemotherapy comes with a lot of side effects and is not
recommended for patients in poor health. Side effect can include increased
risk of infection, hair loss, loss of appetite, NVD, and low blood counts.
Analysis

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CASE STUDY: Merkel Cell and CLL (B-cell)

After researching Merkel cell carcinoma and Chronic lymphocytic

leukemia (B-cell), Patient X treatment follows the treatment plan listed in all
the online research papers. Patient X did not go through with surgery
because his mass was so large on his back ( > 3cm) and he showed signs of
lymph node involvement. He underwent radiation therapy of 60Gy, for 5
days a week (Monday-Friday), for 6 weeks. The patients actual course of
treatment follows the same guidelines listed on current treatment options.
When asking the therapist in the department about this patients setup, they agreed that this treatment was preferred for this patients cancerous
mass. IMRT radiation was the best course of treatment to avoid critical
structures while treating the cancerous cells. They agreed with the total
dose being described and saw an improvement in Patient X mass while under
treatment.
Radiation therapy is a vital role in the treatment of Merkel cell
carcinoma. Most patients undergo surgery first to remove as much, if not all,
the tumor with a 2-3 cm margin. Radiation is used to treat the area where
the tumor bed lies to kill off any of the microscopic cancerous cells left
behind, and help diminish recurrence rates. Since Patient X did not have
surgery, radiation was his only source to killing off the cancerous cells and
shrinking his right flank mass. The patient was to receive chemotherapy to
treat his CLL, since that is the main treatment for CLL (B-cell). It would have
entered the patients bloodstream to treat all areas of the body, since
leukemia tends to spread throughout the body. Chemotherapy works by

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CASE STUDY: Merkel Cell and CLL (B-cell)

attacking the cells in the body that are dividing quickly, but they dont just
harm cancerous cells, they can also damage healthy cells. Which is why
chemotherapy is not recommended to patients in poor health or with a
weakened immune system. But, it plays a vital role in the treatment of
leukemias.
Conclusion
In conclusion, it was an honor to be in every step of a patients cancer
experience. From doctor visits and CT scans, to watching the dosimetrist
and physicist contour and create the actual treatment plan for the patient, to
being there with him every day of treatment.

I wish there would have been

a better outcome for Patient X, but I believe he was getting the appropriate
treatment for his diagnosis. He seemed to be taking the treatment well up
until he became sick. He had a great outlook on life and was a very jolly
man. It was a great experience to watch how every member of the team
works together to care for this patient. I hope that one day there will be a
better prognosis for Merkel Cell carcinoma, but until then I will do all that I
am able to help patients with MCC.

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CASE STUDY: Merkel Cell and CLL (B-cell)

Resources
American Cancer society. (May 23rd, 2016). Skin Cancer: Merkel Cell Carcinoma. Retrieved
from: http://www.cancer.org/cancer/skincancer-merkelcell/detailedguide/skin-cancer-merkelcell-carcinoma-signs-and-symptoms
By Mayo Clinic Staff. Chronic Lymphocytic Leukemia. Retrieved from:
http://www.mayoclinic.org/diseases-conditions/chronic-lymphocytic-leukemia/symptomscauses/dxc-20200674
Created in partnership with : Seattle Multidisciplinary MCC Team, University of Washington
MCC Research, Fred Hutchinson Cancer Research Center, and the Seattle Cancer Care
Alliance/Skin Cancer (December 6th, 2016). About Merkel Cell Carcinoma. Retrieved from
https://merkelcell.org/about-mcc/
Fayad,L,MD and OBrien, S, MD. (April 1st, 2015). Chronic Lymphocytic Leukemia and
Associated Disorders. Retrieved from www.cancernetwork.com/articles/chronic-lymphocyticleukemia-and-associated-distorderd
Hoghes, M. (Jan 22nd, 2014). Merkel Cell Carcinoma: Epidemiology, Target, and Therapy.
Retrieved from www.ncbi.nlm.nih.gov/pmc/articles/PMC3931972
Mir, M , MD. (Sep 7th, 2016) . Chronic lymphocytic leukemia (CLL). Retrieved from
www.emedicine.medscape.com/article/199313Pearson,J ,MD. (August 20th, 2015). Skin Cancer- Merkel Cell Carcinoma. Retrieved from
emidicine.medscape.com/article/870538-treatment
Strati, P and Shanafelt, T. (2015). CLL B-cell. Retrieved from
www.bloodjournal.org/content/126/4/454?sso-checked=true
Written by Mayo Clinic Staff. (Dec 9th, 2015) Mayo Clinic: Merkel Cell Carcinoma. Retrieved
from www.mayoclinic.org/disease-conditions/merkel-cell-carcinoma/home/ovc-20165247

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CASE STUDY: Merkel Cell and CLL (B-cell)