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Running head: RHABDOMYOSARCOMA 1 Embryonal Rhabdomyosarcoma Othman Saleh Grand Valley State University

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Page 1: Embryonal Rhabdomyosarcoma  · Web view2016. 3. 29. · rhabdomyosarcoma is a cancer of soft tissue arises from immature cells that form striated skeletal muscle. It affects the

Running head: RHABDOMYOSARCOMA 1

Embryonal Rhabdomyosarcoma

Othman Saleh

Grand Valley State University

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RHABDOMYOSARCOMA 2

PRESENTING SIGN AND SYMPTOMS OF PATIENTS (HISTORY &

PHYSICAL)

This is a 13 year old male with metastatic paratesticular embryonal rhabdomyosarcoma.

The patient noticed a left testicular mass but did not bring it to anyone’s attention. It is not

shocking to realize that a 13 year old being embarrassed to bring such sign to anyone. Therefore,

teenagers should be educated about signs and symptoms and encouraged to bring up any

abnormality to their parents or seek medical care attention in order to manage the disease before

it becomes aggressive. The patient never smoked nor drank. In addition, the patient’s father had

lung cancer. Overall, the patient is a well-developed African American male with no acute

distress. His vital signs are as follows:

Systolic- 106 mm

Diastolic- 63 mm

Pulse- 85/min

Respiration- 16/min

Pulse Oximetry- 99%

Weight- 52 kg

Pain assessment- 0

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EPIDEMIOLOGY

According to the article, Rhabdomyosarcoma; rhabdomyosarcoma is a cancer of soft

tissue arises from immature cells that form striated skeletal muscle. It affects the muscle,

connective tissue, and bone, which help the movement of the body. Additionally,

rhabdomyosarcoma is generally common in children and adolescents, approximately 5% of all

pediatric cancers and approximately 50 % of all soft tissue sarcomas. The annual incidence in

children and adolescents under 20 years of age is 4.3 cases per million children. 350 cases

diagnosed in the United States (Rhabdomyosarcoma 2008).

Moreover, according to American Cancer Society, rhabdomyosarcoma is more common

in male than female. Race and ethical group has not showed any cause to have an unusual high

rate in rhabdomyosarcoma (American Cancer Society 2016).

ETIOLOGY

According to a report from the Children’s Oncology Group and the Utah Population

Database, there is no sufficient information available about the risk factors in childhood

rhabdomyosarcoma. However, inherited genetic is mentioned to play a significant role in the

development of rhabdomyosarcoma. As revealed by Cancer Med, “5% of cases appear to be

associated with familial syndromes. Specifically, within Li- Fraumeni syndrome (LFS) families

that carry germline TP 5 3 mutations.” As discussed in Washington and Leaver and throughout

my radiation therapy school, inherited genes are most common risk factor to increase the risk of

developing cancer. Moreover, it was found that the prevalence of neurofibromatosis type 1 was

20 times higher in children with RMS compare to those with no evidence of the disease. In

addition, having a family history of cancer, especially with acute lymphoblastic leukemia, germ

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cell tumors, Hodgkin lymphoma, and non-Hodgkin lymphoma, has shown to be associated with

childhood Rhabdomyosarcoma (Cancer Med Cancer Medicine 2015).

According to National Cancer Institute, the cause of rhabdomyosacroma is not known.

However, additional inherited diseases are listed as follows:

Li-Fraumeni syndrome

Pleuropulmonary blastoma

Neurofibromatosis type 1

Beckwith-wiedemann syndrome

Costello syndrome

Noonan syndrome

In addition, overweight children at birth have a greater risk for developing embryonal

rhabdomyosarcoma. Furthermore, a lump or swelling, headache, trouble urinating, change in

bowel movements, blood in urine, bleeding in the nose, throat, vagina, or rectum are signs and

symptoms of rhabdomyosarcoma (National Cancer Institute 2016).

COMPARE PATIENT TO TYPICAL

As mentioned above, rhabdomyosarcoma is common in children and adolescents. This patient is

a 13 year old diagnosed with metastatic paratesticular embryonal rhabdomyosarcoma. One of the

common signs and symptoms is a lump; this patient noticed a left testicular mass. In addition, his

father had lung cancer, which probably placed him at a higher risk of developing cancer due to

inherited genes.

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PATIENT WORKUP INFO

Date Procedure Lab Tests Lab Results Diagnosis

April 5, 2015 Physical exam Testicular mass Possibly tumor

April 7, 2015 Imaging CT Testicular mass Paratesticular

embryonal

rhabdomyosarcoma

May 1, 2015 Urology Tumor Embryonal

rhabdomyosarcoma

July 30, 2015 Biopsy Paratesticular

rhabdomyosarcoma

Tumor size 9.8 cm

July 14, 2015 Testicularectomy Removal of the

tumor

July 24, 2015 CT Retroperitoneal mass and

metastatic disease in his

lungs

Metastasis

July 30, 2015 chemo Mass Manage disease

August 19, 2015 PET/CT Retroperitoneal mass

decreased to 16x28x60

mm and showed mild

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focal uptake

December 2, 2015 PET/CT Malignant

neoplasm of

connective and soft

tissue of abdomen

December 28, 2015 CT Simulation RT treatment

planning

January 14, 2015 V-SIM Trial run for

managing pain

ANATOMY DISCUSSION

This patient is receiving treatment to the left abdomen. The abdomen is the area between

the thorax and pelvis. The digestive organs are found in the stomach. It includes the stomach,

pancreas, liver, gallbladder, large and small intestines. Mesentery is the loose connective tissues

that holds them in place and allow expansion. In addition, the spleen and kidneys are found in

the abdomen. The aorta and inferior vena cava are the main vessels running through the

abdomen to deliver and drain blood from organs (Web MD 2014). Refer to figure 1.1 under the

ANATOMY AND LYMPHATIC GRAPHICS section for graphic illustration.

The abdominal aorta begins from the diaphragm traveling anterior to the spinal column.

The first branch of the abdominal aorta is the celiac trunk, which branches off to the left gastric,

splenic, and common hepatic arteries to supply blood to the stomach, the spleen, the pancreas,

and the liver. The superior mesenteric artery extends from the anterior portion of the aorta. The

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superior mesenteric artery divides into the inferior pancreaticoduodenal artery, the ileocolic,

right colic, and middle colic arteries to feed the ileum, cecum, appendix, and colon. The inferior

mesenteric artery branches off to the left colic and sigmoid arteries. The aorta also gives of the

middle suprarenal, renal, and gonadal arteries to supply the kidney and testicular/ ovarian. On the

other hand the veins collect deoxygenated blood to the inferior vena cava (Basic Human

Anatomy pg.268-271). Refer to figure 1.2 under the ANATOMY AND LYMPHATIC

GRAPHICS section for graphic illustration.

The abdomen includes muscles in order to main body position and enables movement.

The rectus abdominis is located in the middle of the abdomen, which provides the ability to tilt

the pelvis and the curvature of the lower spine. The internal oblique muscles are found next to

the rectus abdominis. The internal oblique muscles originate from the front of the abdomen to the

back of the torso. Its functions are to rotate the spine and helps with breathing. The external

oblique muscles originate from the distal ribs to the pelvis, which enables the function of

bending sideways and rotate the torso. The erector spinae, iliocostalis lumborum, longissimus,

and spinalis located parallel with the spine to allow the spine to flex laterally and provide erect

posture. The latissimus dorsi is an important back muscle which arises from the upper arm to the

hip and joins the abdominal and pectoral muscles. It aids with breathing by enabling the

movement of the ribs. The longissimus muscle is another important back muscle that aid to keep

the head up and allow to it to flex to the side (Healthline 2014). Refer to figure 1.3 under the

ANATOMY AND LYMPHATIC GRAPHICS section for graphic illustration.

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REGIONAL LYMPHATIC DRAINAGE

According to the article, Management of spermatic cord tumors: a rare urologic malignancy; the

regional lymphatic drainage of the spermatic cord are as follows:

Ipsilateral pelvic Inguinal nodes External iliac nodes Hypogastric nodes Common iliac nodes Para-aortic nodes (Management of spermatic cord tumors: a rare urologic malignancy

2012).

For this patient with a metastatic stage 4 paratesticular rhabdomyosarcoma; the inguinal,

hpogastric, and paraaortic nodes should be included in radiation therapy treatment. Refer to

figure 1.4 under the ANATOMY AND LYMPHATIC GRAPHICS section for graphic

illustration.

ANATOMY AND LYMPHATIC GRAPHICS

The Abdomen (Human Anatomy) - Picture, Function, Parts, Definition, and More. (2014). Retrieved February 17,

2016, from http://www.webmd.com/digestive-disorders/picture-of-the-abdomen

Figure 1.1

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Lesson 36: Vessels and nerves of the abdomen & pelvis - Anatomy 3300 with Susan Turner at The Ohio State University - StudyBlue. (n.d.). Retrieved February 18, 2016, from https://www.studyblue.com/notes/note/n/lesson-36-vessels-and-nerves-of-the-abdomen--pelvis/deck/6141413

Figure 1.2

Abdomen, Pelvis and Perineum - Allied Health Sciences Ortho with Taylor at Texas Tech Health Sciences Center University - StudyBlue. (n.d.). Retrieved February 18, 2016, from https://www.studyblue.com/notes/note/n/abdomen-pelvis-and-perineum/deck/1022704

Figure 1.3

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DipNB CLINICAL TUTORIALS. GENERAL SURGERY. DR T M JOSEPH : February 2015. (n.d.). Retrieved

February 18, 2016, from http://drjosephtm.blogspot.com/2015_02_01_archive.html

Figure 1.4

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PATHOLOGY

According to the article, Rhabdomyosarcoma; Rhabdomyosarcoma is a cancer of

soft tissue arises from immature cells that form striated skeletal muscle. It affects the muscle,

connective tissue, and bone, which help the movement of the body. Additionally,

rhabdomyosarcoma is generally common in children and adolescents, approximately 5% of all

pediatric cancers and approximately 50 % of all soft tissue sarcomas. The annual incidence in

children and adolescents under 20 years of age is 4.3 cases per million children. 350 cases

diagnosed in the United States ( Rhabdomyosarcoma 2008).

Moreover, there are three main types of Rhabdomyosarcoma; embryonal, alveolar, and

pleomorhic. The most common type in children is the embroyonal rhabdomyosarcoma. As

described by Paulino and Okcu; these tumors are made of mixture of dense spindle cell and loose

myxoid foci. It occurs in the genital, urinary organs, and head and neck area

(Rhabdomyosarcoma 2008).

According to Paulino and Okcu, alveolar is about 20% of all rhabdomyosarcomas. They

are characterized by thick fibrous septa with loss of cellular cohesion. They consist of solid

round cells with acidophilic to clear cytoplasm and round to oval multilobated, hyperchromatic

nuclei. It occurs in the chest, abdomen, genital organs, arms, legs, and anal area. Pleomorphic

rhabdomyosarcoma is very rare in children and present in adults. These tumors are made up of

uncontrollable arrangement of small, primitive and large pleomorphic cells with an unpredictable

growth guide (Rhabdomyosarcoma 2008).

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GRADING

The staging and grading of cancer are critical factors in deciding the best treatment

options for a neoplasm. In addition, stage and grading describes the extent and how aggressive

the neoplasm is. According to Stanford Medicine on staging and clinical risk group,

rhabdomyosarcoma is staged using the TNM staging system. The TNM system described in the

AJCC cancer Staging Manual as follows:

T- Describes the tumor size:

T1- confined to site of origin

T1a- tumor size < 5 cm

T1b- tumor size ≥ 5 cm

T2- extension to/ infiltration of surrounding tissue

T2a- tumor size < 5 cm

T2b- Tumor size ≥ 5 cm

N- Describes regional lymph nodes

N0- Lymph nodes not clinically involved

N1- Lymph nodes clinically involved

NX- Clinical lymph node status unknown

M- Metastasis

M0- No distant metastasis

M1- Distant metastasis present

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Subsequently, based on the finding from the above information about the tumor size, lymph

nodes involvement, and metastasis, it would be grouped and characterized under stage 1-4.

Stages T N M

1 T1-T2 N0, N1, NX M0

2 T1-T2 N0, NX M0

3 T1-T2 N0, N1, NX M0

4 T1-T2 N0, N1 M1

In addition, a grouping system is used to describe the disease extent and whether it has been

removed by surgery.

Group Extent of Disease

1 localized disease, excised

2 total gross resection with regional disease spread

3 gross residual disease

4 distant metastasis

Eventually, the staging system and grouping system would lead to the finding of the risk

group. The risk group is based on all the workup findings as follow:

Histologic Subtype Clinical Group Clinical Stage Risk group

Embryonal 1, 2, 3 1 Low Risk

Embryonal 1, 2 2, 3 Low Risk

Embryonal 3 2, 3 Intermediate Risk

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Embryonal 4 4 High Risk

Alveolar 1, 2, 3 1, 2, 3 Intermediate Risk

Alveolar 4 4 High Risk

(Staging and Clinical Risk Groups 2016)

PATIENTS PATHOLOGY STAGE AND GRADE

Based on above data and patient’s chart, the patient had diagnosis of malignant neoplasm

of connective and soft tissue of abdomen. His primary is a paratesticular embryonal

rhabdomyosarcoma, which had spread to the lungs and the connective and soft tissue of the

abdomen. Based on the data collected, the patient has a stage 4 embryonal rhabdomyosarcoma.

Therefore, it would be characterized as a high risk according to the clinical risk group.

RT TREATMENT PLAN & RX FOR PATIENT

IMRT planning was preformed per physician request to deliver a total dose of 5040 cGy over 28

fractions, 180 cGy per fraction to the left abdomen.

TREATMENT INFO AND PATIENT SET-UP

Patient setup was supine, headfirst. Vacloc, large knee sponge, feet banded used as

immobilization devices. In addition, suprasternal notch to chin separation must be 12 cm. 3

points tattoos on low chest with anterior superior straightener were given for leveling purposes

during the delivery of radiation therapy treatment.

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TREATMENT TYPE AND HOW IT WAS DELIVERED

Based on patient’s chart, he was treated using external radiation therapy with rapid arc technique.

Details are listed as follows:

Beam ARC1 (CW) ARC2 (CCW)

Gantry

Angles

0.0-179.0 179.0-0.0

Collimator

Angle

30 330

Couch

Angle

0 0

Wedges None None

Block MLC MLC

Bolus None None

Planned

SSD

95.0 cm 89.4 cm

Monitor

Units

178 MU 188 MU

Energy 6 MV

Photon

6 MV

Photon

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RT COMPLICATION & SIDE EFFECTS

According to the patient chart, the patient was experiencing fatigue, nausea, vomiting, abdominal

pain, and skin irritation. The side effects of radiation the patient experienced was mentioned to

him and his mom during the consult. It was recommended for the patient to drink a lot of fluid

and use aquaphor to manage his skin irritation.

ADJUVANT THERAPIES

Treatment options are determined based on the stage, grade, histologic, and site of

disease. Additionally, the patient overall health is an important factor in deciding which

treatment option is best for the patient. The goal of the treatment is to improve not to hurt the

patient. Some patients are not considered a good candidate to undergo some treatments. Due to

advantages in technology in the medical field and better understanding of cancer, there are many

options available to treat cancer victims. According to American Cancer Society, surgery,

chemotherapy, and radiation therapy are the traditional treatment used to treat

rhabdomyosarcoma. Surgery is usually the first option to be used if the tumor can be removed

without causing major deformity. In some cases, chemotherapy or radiation therapy may be used

to shrink the tumor before the operation. The goal of the surgery is to completely remove the

main tumor plus some surrounding normal tissue in order to prevent tumor spread or

reoccurrence. The possible side effects from surgery may include pain, physical changes, and it

can affect how organs function (Surgery for Rhabdomyosarcoma 2014).

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American Cancer Society mentioned that all rhabdomyosarcoma patients will receive

chemotherapy. Chemotherapy is the use of drugs to enter through the blood to kill or manage

cancer cells, which makes it helpful to treat cancer that has spread to other body parts. Chemo

drugs used to treat patients with rhabdomyosarcoma include:

o Vincristine

o Dactinomycin

o Irinotecan

o Ifosfamide

o Etoposide

o Doxorubicin

The possible side effects of chemo may include; hair loss, mouth sores, loss of appetite,

diarrhea, fatigue, nausea and vomiting (Chemotherapy for Rhabdomyosarcoma 2014).

Radiation therapy for rhabdomyosarcoma is used along with chemo to treat leftovers

from surgery. Radiation therapy delivers high dose of radiation to destroy cancer cells.

Unlike chemo, radiation is given to a specific area of the body. Radiation therapy is usually

administered to cure, manage, or relieve pain for metastases. The possible side effects from

radiation therapy depending on the treatment site may include; fatigue, skin reactions of the

area treated, nausea, vomiting, slowing of bone growth, and induces second malignancies

(Radiation Therapy for Rhabdomyosarcoma 2014).

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WHAT OTHER THERAPIES AND COMPLICATIONS DID PATIENT HAVE

According to the patient’s chart, patient had undergone radical orchiectomy and went through

chemotherapy with vincristine and irinotecan.

CRITICAL STRUCTURES AND DOSE TOLERANCES

In order to deliver the dose to the left abdomen, there are some critical structures the beam has to

pass through to reach the target. An IMRT plan was generated using a computer based

optimization in order to accomplish specific dose constraints and goals for the targets and

identified at risk structures. According to Washington and Lever, these structures are listed as

follows with their TD 5/5’s as well:

Structures Side Effect TD 5/5’S (cGy)

Spinal cord Myelitis/necrosis 4500

Right kidney Nephritis 2300

Heart Pericarditis 4000

Liver Liver diseases 3500

bladder Contracture 6500

Muscle Fibrosis, atrophy 6000

Skin Necrosis, ulcerateon 5500

Stomach Ulceration/perforation 5000

Bowel Obstruction/perforation 4000

(Principles and Practice of Radiation Therapy pg. 82)

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ROUTES OF SPREAD

Argawala revealed in the article, Pediatric Rhabdomyosarcomas and

Nonrhabdomyosarcoma Soft Tissue; rhabdomyosarcoma can extend locally, regionally, or

distantly. Local spread means that the tumor invades the tissues around the area where it started.

Regional spread reveals that the tumor has spread to the lymph nodes. Lastly, distant spread

indicates that the tumor spread through the bloodstream to another location in the body.

Argawala stated, “In case there is scrotal violation then hemiscrotectomy should be done to

prevent metastases to the inguinal nodes (which are considered distant metastases and not local)

(Pediatric Rhabdomyosarcomas and Nonrhabdomyosarcoma Soft Tissue 2016).” For this patient,

the primary diagnosis was paratesticular rhabdomyosarcoma, which arises in the distal area of

the spermatic cord. It invaded the testicle. In addition, it spread to the inguinal, retroperitoneal,

and paraspinal nodes. Moreover, it has traveled through the blood to the lung.

PROGNOSIS AND SURVIVAL

Age, stage, site, and histologic type are prognosis factors commonly for all cancer. Refer to the

Grading section to locate the clinical risk group, which includes the stage and histologic type of

the tumor. According to the American Cancer center, the 5 year survival based on the risk group

is as follow:

Low risk group 90%

Intermediate risk group 60-80%

High risk group 20-40%

(Survival Rates for Rhabdomyosarcoma by Risk Group 2014)

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PATIENT PROGNOSIS AND SURVIVAL

This patient pathology is a stage 4 paratesticular embryonal rhabdomyosarcoma. He has

metastatic disease to multiple sites. Using all the data given, the patient disease is considered a

high risk. The prognosis rate in his case is 20-40% for a 5 year survival rate. Moreover, an

article, Paratesticular Rhabdomyosarcoma with Metastatic Encasement of the Abdominal Aorta;

discussed a case similar to my patient with the same age, gender, stage, and histologic type. It

stated, “Stage 4 patients who have metastatic disease in multiple sites at the time of diagnosis are

considered high-risk. The 5 year survival rate in this population is 50% or less (Paratesticular

Rhabdomyosarcoma with Metastatic Encasement of the Abdominal Aorta pg 1064).”

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Work Citation

Paulino, A. C., & Okcu, M. F. (2008). Rhabdomyosarcoma [Abstract]. Current Problems in

Cancer, 32(1), 7-34. Retrieved from

http://www.sciencedirect.com.ezproxy.gvsu.edu/science/article/pii/S0147027207000712

What are the key statistics about rhabdomyosarcoma? (n.d.). Retrieved February 17, 2016, from

http://www.cancer.org/cancer/rhabdomyosarcoma/detailedguide/rhabdomyosarcoma-key-

statistics

Lupo, P. J., Danysh, H. E., Plon, S. E., Curtin, K., Malkin, D., Hettmer, S., . . . Schiffman, J. D.

(2015). Family history of cancer and childhood rhabdomyosarcoma: A report from the

Children's Oncology Group and the Utah Population Database [Abstract]. Cancer Med

Cancer Medicine, 4(5), 781-790. Retrieved from

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4430270/?tool=pmcentrez

Childhood Rhabdomyosarcoma Treatment. (n.d.). Retrieved February 18, 2016, from

http://www.cancer.gov/types/soft-tissue-sarcoma/patient/rhabdomyosarcoma-treatment-

pdq

The Abdomen (Human Anatomy) - Picture, Function, Parts, Definition, and More. (2014).

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Retrieved February 17, 2016, from http://www.webmd.com/digestive-disorders/picture-

of-the-abdomen

O'Rahilly, R., & Muller, F. (1983). Basic human anatomy: A regional study of human structure.

Philadelphia: Saunders.

Lesson 36: Vessels and nerves of the abdomen & pelvis - Anatomy 3300 with Susan Turner at

The Ohio State University - StudyBlue. (n.d.). Retrieved February 18, 2016, from

https://www.studyblue.com/notes/note/n/lesson-36-vessels-and-nerves-of-the-abdomen--

pelvis/deck/6141413

Muscles. (2014, November 5). Retrieved February 18, 2016, from

http://www.healthline.com/human-body-maps/abdomen-muscles

Abdomen, Pelvis and Perineum - Allied Health Sciences Ortho with Taylor at Texas Tech

Health Sciences Center University - StudyBlue. (n.d.). Retrieved February 18, 2016, from

https://www.studyblue.com/notes/note/n/abdomen-pelvis-and-perineum/deck/1022704

Rodríguez, D., & Olumi, A. F. (2012). Management of spermatic cord tumors: a rare urologic

malignancy. Therapeutic Advances in Urology, 4(6), 325–334.

http://doi.org/10.1177/1756287212447839

DipNB CLINICAL TUTORIALS. GENERAL SURGERY. DR T M JOSEPH : February 2015.

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(n.d.). Retrieved February 18, 2016, from

http://drjosephtm.blogspot.com/2015_02_01_archive.html

Staging and Clinical Risk Groups. (n.d.). Retrieved February 18, 2016, from

http://surgpathcriteria.stanford.edu/srbc/rhabdomyosarcoma/staging.html

Agarwala, S. (2006, January-March). Pediatric rhabdomyosarcomas and nonrhabdomyosarcoma

soft tissue sarcoma. Journal of Indian Association of Pediatric Surgeons, 11(1).

Retrieved from http://go.galegroup.com.ezproxy.gvsu.edu/ps/i.do?id=GALE

%7CA145087233&sid=summon&v=2.1&u=lom_gvalleysu&it=r&p=AONE&sw=w&asi

d=08d4f905a846b4638c96a27d4b802bbf

Surgery for rhabdomyosarcoma. (2014, November 21). Retrieved February 19, 2016, from

http://www.cancer.org/cancer/rhabdomyosarcoma/detailedguide/rhabdomyosarcoma-

treating-surgery

Chemotherapy for rhabdomyosarcoma. (2014, November 21). Retrieved February 19, 2016,

From

http://www.cancer.org/cancer/rhabdomyosarcoma/detailedguide/rhabdomyosarcoma-

treating-chemotherapy

Radiation therapy for rhabdomyosarcoma. (2014, November 21). Retrieved February 19, 2016,

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from

http://www.cancer.org/cancer/rhabdomyosarcoma/detailedguide/rhabdomyosarcoma-

treating-radiation-therapy

Survival rates for rhabdomyosarcoma by risk group. (2014, November 21). Retrieved February

19, 2016, from

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