Introduction Sarcomas are cancers that develop from connective tissues in the body, such as muscles, fat, bones,

membranes that line the joints, or blood vessels. There are many types of sarcomas. Rhabdomyosarcoma (RMS) is a cancer made up of cells that normally develop into skeletal muscles.The body has 3 main types of muscles. Skeletal (voluntary) muscles are muscles that e control to move parts of our body. Smooth muscle is the main type of muscle in internal or!ans (e"cept for the heart). #or e"ample, smooth muscles in the intestines push food alon! as it is di!ested. $e do not control this movement. %ardiac muscle is the main muscle type in the heart. &bout ' eeks into the development of an embryo, cells called rhabdomyoblasts ( hich ill eventually form skeletal muscles) be!in to form. These are the cells that can develop into rhabdomyosarcoma. (ecause this is a cancer of embryonal cells, it is much more common in children, althou!h it does sometimes occur in adults. Rhabdomyosarcoma (RMS) is a mali!nant tumor ()cancer)) that arises from a normal skeletal muscle cell. *ot very much is kno n about hy normal skeletal muscle cells become cancerous. (ecause skeletal muscle cells are found in virtually every site of the body, RMS can develop in almost any part of the body. Rhabdomyosarcoma (RMS) affects cells called rhabdomyoblasts, immature cells hich !o on to form the skeletal muscles of the body. &lthou!h it is the most common soft tissue cancer in children, only about 3+, cases are dia!nosed each year in the -nited States. Rhabdomyosarcoma occurs in t o distinct a!e !roups. children five years of a!e and under, ho are most likely to have the embryonal form of disease/ and adolescent ho more often have alveolar rhabdomyosarcoma. The overall hen detected in an early, ho develop RMS don5t have any clear risk factor for ill have an identifiable children a!ed 0123,,

five2year survival rate for rhabdomyosarcoma is ',4 treatable sta!e. Most children

!ettin! cancer. &fter takin! a careful family history and doin! a thorou!h physical e"amination, appro"imately one child in five to one child in ten )!enetic risk factor).

Symptoms of rhabdomyosarcoma depend on the location of the tumor. 6ye orbit. bul!in!, protrudin! eyeball

*asal passa!es. nosebleeds, con!estion, bloody mucus (ladder. blood in the urine, difficulty urinatin! 7a!ina. !rapelike lumps protrudin! from the va!ina (botryoid RMS), va!inal bleedin! Testicles. noticeable lump or s ellin! on one side, often painless &rms, le!s or trunk. s ellin! or lump similar to that of normal injuries 8elvis. constipation, pain or vomitin!

Diagnosis Rhabdomyosarcomas can often be detected ith a chest 92ray, MR: or %T scan. aspiration ill be performed is ;o ever, since the tumors can be similar to other cancers, a biopsy is needed to confirm the dia!nosis. &fter the dia!nosis, a bone marro to determine if the cancer has spread to the bone marro . & sample of marro e"tracted from the pelvic bones ith a needle and e"amined under the microscope. Sta!in! of rhabdomyosarcoma is done in three related steps. & sta!in! system & !roupin! system & risk !roup The sta!in! system is based on the si<e of the tumor, )favorable) site has a better pro!nosis. :f the site here it is in the body, and

Staging hether it has spread to other parts of the body. Rhabdomyosarcoma that occurs in a here cancer occurs is not one of the favorable sites listed belo , it is said to be an )unfavorable) site. Stage 1. %ancer is any si<e, has not spread to lymph nodes, and is found in only one of the follo in! )favorable) sites. 6ye or area around the eye. ;ead and neck (but not in the tissue ne"t to the brain and spinal cord). =allbladder and bile ducts. :n the testes or va!ina (but not in the kidney, bladder, or prostate).

Stage 2. %ancer is found in any one area not included in sta!e 0. The tumor is + centimeters or smaller and has not spread to lymph nodes. Stage 3. %ancer is found in any one area not included in sta!e 0 and one of the follo in! is true. The tumor is + centimeters or smaller and cancer has spread to nearby lymph nodes. The tumor is lar!er than + centimeters and cancer may have spread to nearby lymph nodes. Stage 4: The tumor may be any si<e and cancer may have spread to nearby lymph nodes. %ancer has also spread to distant parts of the body such as the lun!, bone marro , or bone. Grouping System The !roupin! system is based on =roup :. %ancer hether the cancer has spread and ho here it started and it much cancer remains after sur!ery to remove the tumor. as found only in the place as completely as removed by sur!ery. Tissue removed. The tissue cells ere seen. =roup :: is divided into !roups ::&, ::(, and ::%. ::&. %ancer patholo!ist. ::(. %ancer had spread to nearby lymph nodes and the cancer and lymph nodes removed by sur!ery. ::%. %ancer had spread to nearby lymph nodes and the cancer and lymph nodes removed by sur!ery. Tissue removed. The tissue cells ere seen. =roup :::. %ancer as partly removed by sur!ery and there are cancer cells (a lump or mass) remainin! that can be seen by "2ray or other ima!in! test. %ancer has not spread to distant parts of the body. as taken from the ed!es of here the tumor ere as ere as removed by sur!ery but cancer cells here the tumor as removed, ere seen hen the tissue, taken from the ed!es of as vie ed under a microscope by a as taken from the ed!es of here the tumor

as checked under a microscope by a patholo!ist and no cancer

as checked under a microscope by a patholo!ist and no cancer

=roup :7. %ancer had spread to distant parts of the body at the time of dia!nosis. Risk Group The risk !roup is based on the sta!in! system and the !roupin! system and is used to plan treatment. :t describes the chance that rhabdomyosarcoma The follo in! risk !roups are used. >o 2risk rhabdomyosarcoma is one of the follo in!. ithout a microscope. The cancer may have areas are )favorable) sites. ill recur (come back).

&n embryonal tumor of any si<e that is found in a )favorable) site. There may be tumor remainin! after sur!ery that can be seen spread to nearby lymph nodes. The follo in! 6ye or area around the eye ;ead or neck (but not in the tissue ne"t to the brain and spinal cord) =allbladder and bile ducts :n the testes or va!ina (but not in the kidney, bladder, or prostate) &n embryonal tumor of any si<e that is not found in one of the )favorable) sites listed above. There may be tumor remainin! after sur!ery that can be seen only microscope. The cancer may have spread to nearby lymph nodes. :ntermediate2risk rhabdomyosarcoma is one of the follo in!. ith or ithout a ith a

&n embryonal tumor of any si<e that is not found in one of the )favorable) sites listed above. There is tumor remainin! after sur!ery, that can be seen microscope. The cancer may have spread to nearby lymph nodes. &n alveolar tumor of any si<e in a )favorable) or )unfavorable) site. There may be tumor remainin! after sur!ery that can be seen have spread to nearby lymph nodes. ;i!h2risk rhabdomyosarcoma may be the embryonal type or the alveolar type. :t may have spread to nearby lymph nodes and has spread to one or more distant parts of the body. Rhabdomyosarcoma Screening %ancer screenin! e"ams are important medical tests done hen you5re at risk but don5t hen the chances for have symptoms. They help find cancer at its earliest sta!e, ith or ithout a microscope. The cancer may

successful treatment are hi!hest. ;o ever, there are currently no standardi<ed screenin! e"ams for childhood rhabdomyosarcoma. Medical history and physical e am :f your child has any si!ns or symptoms that may su!!est RMS, the doctor ant to !et a complete medical history to learn about any symptoms and ho child has had them. The doctor abnormal mass in the body. :f symptoms or the results of the physical e"am su!!est your child mi!ht have RMS, other tests tests. Imaging tests :ma!in! tests use "2rays, ma!netic fields, radioactive substances, or sound reasons, includin!. To help find out hether a suspicious area mi!ht be cancerous To determine the e"tent of a tumor or learn ho To help determine if treatment is orkin! far a cancer may have spread aves to create pictures of the inside of the body. :ma!in! tests may be done for a number of ill need to be done. These mi!ht include ima!in! tests, biopsies, and?or lab ill lon! your

ill also e"amine your child to look for possible si!ns of

RMS or other health problems. #or e"ample, the doctor may be able to see or feel an

8atients ho have or may have RMS ill !et one or more of these tests. Plain x-rays These are sometimes used to look for tumors, but their use is limited mainly to lookin! at bones because they do not sho much detail in internal or!ans. &n "2ray of the chest is sometimes done to look for cancer that mi!ht have spread to the lun!s, althou!h it isn@t needed if a chest %T scan is bein! done. Computed tomography (CT or CAT) scan The %T scan is an "2ray test that produces detailed cross2sectional ima!es of parts of the body, includin! soft tissues such as muscles. :nstead of takin! one picture, like a re!ular "2ray, a %T scanner takes many pictures as it rotates around your child

hile he or she lies on a table. & computer then combines these pictures into ima!es of slices of the part of the body bein! studied. This test can provide fairly detailed information about a tumor, includin! ho nodes, as spread. (efore the scan, your child may be asked to drink a contrast solution and?or !et an intravenous (:7) injection of a contrast dye that helps better outline abnormal areas in the body. Aour child may need an :7 line throu!h contrast may cause some flushin! (a feelin! of or lo hich the contrast dye is injected. The armth, especially in the face). Some ell as the lun!s or other areas of the body lar!e it is and if it has invaded nearby structures. :t can also be used to look at nearby lymph here the cancer mi!ht have

people are aller!ic and !et hives. Rarely, more serious reactions like trouble breathin! blood pressure can occur. (e sure to tell the doctor if your child has any aller!ies %T scans take lon!er than re!ular "2rays. Burin! the test, the table slides in and out of the scanner, a rin!2shaped machine that completely surrounds the table. Aour child ill need to lie still on a table hile this is bein! done. Aoun!er children may be hich completes the scan more !iven medicine to help keep them calm or even asleep durin! the test. Many medical centers no use spiral CT (also kno n as helical %T), Cuickly. :t also yields more detailed pictures and lo ers the dose of radiation received durin! the test. Magnetic resonance imaging (MRI) scan >ike %T scans, MR: scans !ive detailed ima!es of soft tissues in the body. (ut MR: scans use radio radio aves and stron! ma!nets instead of "2rays. The ener!y from the aves is absorbed and then released in a pattern formed by the type of body or has ever had a reaction to any contrast material used for "2rays.

tissue and by certain diseases. & computer translates the pattern into a very detailed ima!e of parts of the body. & contrast material called !adolinium may be injected into a vein before the scan to better sho cause aller!ic reactions. This test may be used instead of a %T scan to look at the tumor and the tissues around it. MR: is especially useful if the tumor is in certain parts of the body, such as the details. The contrast material usually does not

head and neck, an arm or le!, or the pelvis. MR: scans can help determine the e"act e"tent of a tumor, as they provide a detailed vie tissue around the tumor. This is important spinal cord or brain. MR: scans take lon!er than %T scans, often up to an hour. Aour child may have to lie inside a narro tube, hich is confinin! and can be distressin!. *e er, more open ith this, but the test still reCuires stayin! still for lon! periods of MR: machines can help of the muscle, fat, and connective hen plannin! sur!ery or radiation therapy.

MR: is also very useful if your child@s doctor is concerned about possible spread to the

time. The MR: machine also makes loud bu<<in! and clickin! noises that may be disturbin!. Sometimes, youn!er children are !iven medicine to help keep them calm or even asleep durin! the test. Bone scan & bone scan can help sho of the orkup for children the entire skeleton at once. #or this test, a small amount of lo 2level radioactive material is injected into a vein (:7). The substance settles in areas of dama!ed bone throu!hout the entire skeleton over the course of a couple of hours. Aour child then lies on a table for about 3, minutes hile a special camera detects the radioactivity and creates a picture of the skeleton. Aoun!er children may be !iven medicine to help keep them calm or even asleep durin! the test. &reas of active bone chan!es attract the radioactivity and sho up as )hot spots) on the scan. These areas may su!!est cancer in an area, but other bone diseases can also cause the same pattern, so other ima!in! tests such as plain "2rays or MR: scans, or even a bone biopsy mi!ht be needed. ltrasound -ltrasound uses sound aves and their echoes to make a picture of internal ith !el). :t !ives off sound aves or!ans or tumors. #or this test, a small, microphone2like instrument called a transducer is moved around on the skin ( hich is first lubricated if a cancer has spread to the bones, and is often part ith RMS. This test is useful because it provides a picture of

and picks up the echoes as they bounce off the or!ans. The echoes are converted by a computer into an ima!e that is displayed on a computer screen. -ltrasound can be used to see if tumors in the pelvis (such as prostate or bladder tumors) are !ro in! or shrinkin! over time. (This test can@t be used to look at tumors in the chest because the ribs block the sound aves.) This is an easy test to have done, and it uses no radiation. Aour child simply lies on a table, and a technician moves the transducer over the part of the body bein! looked at. Biopsy methods The results of ima!in! tests may stron!ly su!!est that someone has RMS, but a biopsy (removin! some of the tumor for vie in! under a microscope and other lab testin!) is the only ay to be certain. -sually several different kinds of lab tests are ays. The approach that is used ill depend on done on the sample to sort out hat kind of tumor it is. (iopsies can be done in several the doctor doin! the biopsy. !urgical biopsy The most common biopsy approach is to sur!ically remove a small piece of tumor (a doctor hile the patient is under !eneral anesthesia (asleep) and have it looked at by a patholo!ist ho dia!noses diseases from the results of lab tests). :n some cases, nearby lymph nodes may also be removed and tested to see if the tumor has spread. "eedle biopsies :f for some reason a sur!ical biopsy cannot be done, a less invasive biopsy usin! a hollo needle may be used. There are 3 kinds of needle biopsies, each of hich has needle pros and cons. %ore needle biopsy. #or a core needle biopsy, the doctor inserts a hollo into the tumor to ithdra a piece of tissue (core sample). :f the tumor is near the here the mass is located, the a!e of the patient, and the e"pertise and e"perience of

surface of the body, the doctor can !uide the needle into the tumor by touch. (ut if the

tumor is deep

ithin the body, ima!in! tests such as ultrasound or %T scans may be

needed to !uide the needle into place. The core sample that is removed is then used in lab tests to help make the dia!nosis. The main advanta!e of a core needle biopsy is that it does not reCuire sur!ery, so there is no lar!e incision. Bependin! on here the tumor is, adults and older children ith a may not need !eneral anesthesia ( here they are asleep for the biopsy), but some children may still need it. Dn the other hand, the specimen is smaller than specimen is not a !ood sample of the tumor, another biopsy ill be needed. #ine needle aspiration (#*&) biopsy. This techniCue uses a very small hollo needle attached to a syrin!e to easily. The do nside of #*& is that the sample is very, very small. The patholo!ist must be e"perienced ith this techniCue and be able to decide hich lab tests ill be most helpful on a very small sample. :n cancer centers that have the e"perience to e"tract the most information from very small amounts of tissue, #*& can be a valuable E thou!h certainly not foolproof E dia!nostic approach, but it is not usually the preferred biopsy techniCue. Bone marro# aspiration and biopsy These tests aren@t used to dia!nose RMS, but they may be done after the dia!nosis to find out if the tumor has spread to the bone marro . The 3 tests are usually done at the same time. The samples are usually taken from the back of both of the pelvic (hip) bones, but in some patients they may be taken from the sternum (breastbone) or other bones. These tests may be done durin! the sur!ery to treat the main tumor ( hile the child is still under anesthesia), or they may be done as a separate procedure. :f the bone marro aspiration is bein! done as a separate procedure, the child lies ith local anesthetic, hich may cause a on a table (on his or her side or belly). &fter cleanin! the skin over the hip, the doctor numbs the area and the surface of the bone ithdra (aspirate) a small tumor sample. &n #*& biopsy is ideally suited to tumors near the surface of the body that can be reached sur!ical biopsy, and if it is not aimed correctly, the needle may miss the cancer. :f the

brief stin!in! or burnin! sensation. 6ven have some brief pain hollo hen the marro

ith the local anesthetic, most patients still

is removed. :n most cases, the child is also

!iven other medicines to reduce pain or even be asleep durin! the procedure. & thin, needle is then inserted into the bone, and a syrin!e is used to suck out a small & bone marro bone and marro done, pressure biopsy is usually done just after the aspiration. & small piece of ith a sli!htly lar!er needle that is t isted as it is pushed amount of liCuid bone marro . is removed

do n into the bone. The biopsy may also cause some brief pain. Dnce the biopsy is ill be applied to the site to help stop any bleedin!.

$umbar puncture (spinal tap) >umbar puncture is not a common test for RMS, but it may be done for tumors in the head near the coverin! of the brain (the menin!es). This test is used to look for cancer cells in the cerebrospinal fluid (%S#), and spinal cord. #or this test, the doctor first numbs an area in the lo er part of the back near the spine. The doctor may also recommend that the child be !iven somethin! to make him or her sleep so the spinal tap can be done hollo fluid. $ab tests on the biopsy samples & patholo!ist ill look at the biopsy samples under a microscope to try to determine if they contain cancer cells. :f the patholo!ist finds cancer, the ne"t step is to fi!ure out if the cancer is RMS. :n rare cases, the patholo!ist can see that the cancer cells have small muscle striations (myofibrils), hich confirm that the cancer is RMS. (ut in most cases, other lab tests are needed to confirm the dia!nosis. 8atholo!ists may use special stains on the samples to identify the type of tumor. The stains contain special proteins (antibodies) that specifically attach to substances in RMS cells but not other cancers. The stains produce a distinct color that can be seen ithout difficulty or causin! harm. & small, ithdra some of the needle is then placed bet een the bones of the spine to hich is the liCuid that bathes the brain

under a microscope. This lets the patholo!ist kno rhabdomyosarcoma. Sometimes the tumor section

that the tumor is a

ill also be tested for !ene abnormalities. =enetic tests look for

chromosome translocations and other B*& chan!es such as those discussed in the

!atient !ro"ile *ame. 8atient 9A9 &!e. 3 years old (irthday. Fanuary 3,, 3,,G (irth 8lace. (r!y.Bamuhan Matalahib &ddress. H013 (ry. 8a!2ibi! Sa in! 8alad Reli!ion. %atholic *ationality. #ilipino Bate and Time of &dmission %hief %omplaint. difficulty in urination, pelvic pain, vomitin! and diarrhea Bia!osis. 6mbryonal rhabdomyosarcoma 8hysician. Bra. &nita Mahajan M.B #istory I$ !ast #istory

The patient was born Fanuary 3,, 3,,G , the first daughter of Mr. and Mrs. Magdiwa. He was well taken care of her parents starting her intrauterine life. He had already illness like Measles, Chicken Pox, Mumps, Diarrhea .This was his first confinement to the hospital as erbali!ed by her mother.
II$!resent history

" days prior to admission patient e"perienced pain in the lo er

abdomen and diarrhea for 3 days dehydration. The patient ith vomitin!. ;is mother !ave him #buprofen 0,,m!?+ml for abdominal pain but the pain continue. $ater therapy to prevent as brou!ht by her mother to the hospital because of pelvic as admitted in the pedia ard then as !iven ith :7# of H0 B+:M( +,, ml. &fter a series of pain, diarrhea and vomitin!. ;e anal!esic and anti emetic dru!s dia!nostic test ;e

as referred to MB &nderson 8roton Therapy %enter for treatment of

an embryonal rhabdomyosarcoma attached to his bladder.

%natomy and !hysiology

The urinary system consists of the kidneys, ureters, urinary bladder, and urethra. The kidneys filter the blood to remove astes and produce urine. The ureters, urinary bladder, hich acts as a plumbin! system to drain urine and urethra to!ether form the urinary tract, eliminatin!

from the kidneys, store it, and then release it durin! urination. (esides filterin! and astes from the body, the urinary system also maintains the homeostasis muscular or!an that acts as a reservoir for urine. of ater, ions, p;, blood pressure, calcium... The urinary bladder is a hollo The adult bladder can hold a pint or more of fluid. :t lies behind the pubic bone and is protected by the pelvis. The bladder alls are made up of muscle and an inner linin!. &t the back are t o ureters, hich carry the urine from the kidneys. The bladder collects and stores urine until it can be e"pelled from the body.

!athophysiology The tumor is believed to arise from primitive muscle cells, but tumors can occur any here in the body/ ho ever, a primary bone rhabdomyosarcoma has not been reported. The most common sites are the head and neck (3I4), e"tremities (314), and !enitourinary (=-) tract (0I4). Dther notable sites include the trunk (004), orbit ('4), and retroperitoneum (G4). Rhabdomyosarcoma occurs at other sites in less than 34 of patients. The botryoid variant of 6RMS arises in mucosal cavities, such as the bladder,

va!ina, nasopharyn", and middle ear. >esions in the e"tremities are most likely to have an alveolar type of histolo!y. Metastases are found predominantly in the lun!s, bone marro , bones, lymph nodes, breasts, and brain. &s ith most tumors of childhood, the cause of rhabdomyosarcoma is unkno n.

The alveolar variant is so named because of the thin criss2crossin! fibrous bands that appear as spaces bet een cellular re!ions of the tumor (reminiscent of lun! alveoli). This variant is usually associated ith 0 of 3 chromosomal translocations, namely, t(3/03) or t(0/03). These result in the fusion of the B*&2bindin! domain of the neuromuscular developmental transcription factors, encoded by PA%& on chromosome 3 or PA%' on chromosome 01 , to the transcriptional activation domain of a relatively ubiCuitous transcription factor, #J;R (or #D9D0a), rare cases.+ The resultin! hybrid molecule is a potent transcription activator. :t is believed to contribute to the cancerous phenotype by abnormally activatin! or repressin! other !enes. The embryonal subtype usually has a loss of hetero<y!osity at band 00p0+.+/ this observation su!!ests the presence of a tumor suppressor !ene. Dther molecular aberrations that may provide clues to the ori!in of the tumor and that may be useful for future treatment strate!ies include TP(& mutations ( hich occur in appro"imately one half of patients), an elevated *2myc level (in 0,4 of patients ith &RMS), and point mutations in *2ras and J2ras onco!enes (usually embryonal). :n addition, levels of insulinlike !ro th factor23 may be elevated, su!!estin! path ays involvin! autocrine and paracrine !ro th factors.G &aboratory Result &n abdominal?pelvic MR: revealed a 3.G " 3.3 " 3.32cm lobulated bladder base mass that indented the bladder base but had no evidence of invasion. There ere no adenopathy or perivesical fat strandin! notes. #B= %T286T revealed increased #B= uptake at the ri!ht ureter and ri!ht kidney, possible hydronephrosis on the ri!ht side, as ell as distal ureteral obstruction. & bone marro biopsy tested ne!ative for metastatic hich is encoded on chromosome 03. >ess common translocations involvin! the PA% !enes have been found in some

disease. The tumor rhabdomyosarcoma.

as sta!ed as T0a *, M,, sta!e 3, !roup 3 embryonal as potentially risky. Treatment ith

(ecause of the tumor5s location, sur!ical removal

conventional radiation could have resulted in serious late effects on 8atient5s developin! body, includin! intellectual impairment, decreased bone and soft tissue !ro th, hormonal deficiencies and second tumors. Medication %hemotherapy is an essential part of treatment to prevent further spread of the cancer. Many different chemotherapy dru!s are active a!ainst rhabdomyosarcoma. Some of these dru!s include.

7incristine &ctinomycin2B %yclophosphamide (%yto"an) Bo"orubicin (&driamycin) Melphalan :fosfamide 6toposide Topotecan

'R(%'M()' $ith its capability to precisely deliver hi!h doses of radiation to the tumor little dama!e to surroundin! normal tissue, proton therapy ith as an ideal treatment for

the patient. 8roton therapy is increasin!ly bein! used to treat cancer in pediatric patients. 8roton therapy, also called 8roton beam therapy, is the most advanced radiation therapy available today 2 it destroys cancer cells but does not attack surroundin! healthy tissue nearly as much as traditional radiation therapy does. 8roton therapy, a kind of particle therapy, directs proton beams cells. ;e received vincristine concurrently ith proton therapy at MB &nderson. The ith non2coplanar beam care plan called for doses of +,.1 cobalt !ray eCuivalents ith !reat precision at cancer

!eometry at 0.I =y per fraction to the !ross tumor volume. T o anterior obliCue fields, as ell as 8& field ere utili<ed. T enty2ei!ht proton therapy treatments ere !iven.

!rognosis &fter t o eeks of proton therapy, his symptoms resolved. &fter three eeks of proton therapy and concurrent chemotherapy, physicians sa returned home to 7ir!inia year2old. no evidence of disease and he

ith his family. *o , three years post treatment, Fake has not

had a recurrence or residual effects. ;is parents describe him as a normal, active '2