case of osteosarcoma

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1 OSTEOSARCOMA A case report Abstract Osteosarcoma is a deadly form of musculoskeletal cancer that most commonly causes patients to die from pulmonary metastatic disease and which has a 5-year survival rate of 15-20%. It is regarded to be the most common highly malignant bone tumor which often affects the adolescents and young adults. The symptoms and chances of recovery for children and adolescents appear to be the same. Radiography is almost always the initial imaging modality used in the investigation and with histolopathologic studies done for the definitive diagnosis. The present management for osteogenic sarcoma involves amputation or disarticulation collaborated with chemotherapy. The proceeding section illustrates the etiology, pathophysiology, clinical course, diagnostic modalities and management of a patient with OSTEOSARCOMA.

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a case of 17 yo male with osteosarcoma in thailand

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Page 1: Case of Osteosarcoma

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OSTEOSARCOMAA case report

Abstract

Osteosarcoma is a deadly form of musculoskeletal cancer that most commonly causes

patients to die from pulmonary metastatic disease and which has a 5-year survival rate of 15-

20%. It is regarded to be the most common highly malignant bone tumor which often affects

the adolescents and young adults. The symptoms and chances of recovery for children and

adolescents appear to be the same.

Radiography is almost always the initial imaging modality used in the investigation and with

histolopathologic studies done for the definitive diagnosis. The present management for

osteogenic sarcoma involves amputation or disarticulation collaborated with chemotherapy.

The proceeding section illustrates the etiology, pathophysiology, clinical course, diagnostic

modalities and management of a patient with OSTEOSARCOMA.

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Table of contents

Title page 1

Abstract 2

Table of contents 3

Objective of the case study 4

Significance of the case study 4

Methodology

Patient’s profile 5

History 5

Physical examination 6

Differential diagnosis 7 - 8

Course in the ward 9 - 10

Laboratory results 11 - 13

Discussion of the case 14 -18

Conclusion 19

Recommendation 19

References 20

Appendix 21 - 31

Pictures of the case

Laboratory results

Biopsy results

Others

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Objective of the case report

1. To present a case of osteosarcoma in a 17 year old patient.

2. To emphasize the role of roentgenologic study in the detection of osteogenic sarcoma.

3. To emphasize the combined role of radiographic imaging and other laboratory

modalities in the early detection of osteosarcoma.

4. To discuss the pathophysiology, clinical and features, diagnosis, management, and

prognosis of osteosarcoma.

Significance of the case report

In**** , approximately there had been 9 recorded osteosarcoma cases admitted in *****

orthopedic ward. Only a few of these patients submitted for surgical management.

Osteosarcoma is considered to be the most common malignant tumor of the bone and it is also

fatal with the patients usually failing for pulmonary metastasis. It is known to affect the

younger age group.

Radiologic imaging has a crucial role in the detection of this tumor. When detected early, its

progression and metastases could be monitored through serial x-rays. Thus, when coupled

with histopathologic study and other essential laboratory modalities, its sensitivity and

specificity to diagnose the disease is doubled.

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Methodology

Patients’ profile

A case of J.M., 17 year old male, single, Asian of the Malayan race, Roman Catholic, from

***** City was admitted for the first time on ******* in ******** due to pain and deformity of the

left leg.

History of Present Illness

3 years prior to admission, patient noted a slowly growing mass on the left leg which initially

was not associated with pain or tenderness and there was no limitation of motion noted. The

condition was tolerated and no consultation sought.

Few months prior to admission, patient started to complain of dull, constant, aching pain with

progressive increase in the size of the mass in the left leg. The pain was also aggravated

during ambulation.

1 month PTA, patient was then brought by his mother for consultation in ****** OPD where an

x-ray and biopsy was advised. Patient complied and both x-ray and biopsy results revealed

osteosarcoma. He was then advised for surgery and thus this admission.

Past History

Patient is neither a known asthmatic nor had any history of allergies to food and medications.

He claims to have no history of previous hospitalization or serious illnesses.

Personal History

J.M. was delivered in a hospital in****** . He is the youngest of 4 siblings. He was born a

healthy baby, was breastfed and personally taken cared of by his mother. His motor

development was average since he was able to walk and talk by 10 months old as claimed by

his mother. He received complete vaccination at the rural health center. During his childhood,

he was seldom afflicted with common illness like fever, cough and colds. Presently, he is still

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residing with his parents. His father worked as a plumber and his mother works as a clerk in a

nearby health center. He entered formal training in a nearby public school and it is where he

completed his high school education. Most of his closest friends are his schoolmates of which

he likes to spend time with when free from household errands. He claimed to be a non-smoker

and drinks alcoholic beverages occasionally. He also claimed to have tried prohibited drugs

with his friends out of curiosity but he claimed to have no dependency to such chemicals.

Physical examination

Examined a conscious, coherent, cooperative, ambulatory, sthenic and not in respiratory

distress with the following vital signs:

BP: 120/90 mmHg PR: 78 bpm RR: 22 cpm Temp.: 36.9 oC Wt.: 45 Kgs

Skin: warm, good turgor

HEENT: anicteric sclera, pinkish palpebral conjunctiva, no tonsillopharyngeal congestion

Neck: no masses, no tenderness, no lymphadenopathies noted

Chest/Lungs: equal chest expansion, clear breath sounds, no rales, no wheezes

CVS: distinct heart sound, normal rate and rhythm, no murmur

Abdomen: flat, normoactive bowel sounds, soft, no masses, no tenderness

GUT: (-) kidney punch sign

Extremities: presence of a firm mass approximately 12 X 14.5 cms at the proximal – middle

thirds of the left leg, (+) tenderness and warm when palpated. The right leg is unremarkable.

No limitation of motion noted. CRT < 2secs, strong pulses noted.

CNS: within normal limits

Impression: OSTEOSARCOMA, Left Tibia

Osteosarcoma is the primary consideration based on the history that the patient is still in his

adolescence. The patient is complaining of dull, constant, aching pain on his left leg. With

presenting signs of a solitary slowly growing lesion in the left leg which is also tender and

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warm when touched and is located near the left knee joint. The diagnosis is also supported by

documented plain film and biopsy results.

The plain film result of J.M. was described as a presence of cortical thickening and cloud-like

proliferation (sun-burst effect) in the external anterior surface of the proximal thirds of the left

tibia with associated soft tissue swelling of the left leg. Such radiographic finding coincides

with the textbook appearance and description of osteosarcoma.

Differential diagnosis

Chronic osteomyelitis

It is a chronic inflammatory process of the bones and its structures secondary to infection with

pyogenic organisms. It could affect all ages and has predisposition for males as it usually

results following trauma. It could present with a non-healing ulcer, flocculent mass and sinus

tract drainage, a history of fever, chronic fatigue and body malaise. It is included as one of the

differential diagnosis because its lesion usually is warm and is tender to palpate. And there is

a reduction in the use of the affected limb due to pain.

The presence and identification of the pathogenic organism could easily be documented

through hematologic studies and culture.

Radiographically, there would be evidences of soft tissue swelling which is noted as early as 3-

5 days after infection. And bony changes which are evident by 14 – 21 days and may initially

manifest as periosteal elevation followed by cortical or medullary lucencies usually by the 28 th

day. The infection extends through the vascular channels in the cortex to elevate and strip the

periosteum and the cortex. Approximately 40-50% of focal bone loss is necessary to cause

detectable lucency on plain films.

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Its exclusion in the differentials includes the fact that the patient did not have a history of

trauma or have a history of fever, chronic fatigue and body malaise. The gross appearance of

the lesion did not appear to be a flocculent mass, and did not have the usual sinus drainage

tract. The course of progression of osteosarcoma exceeds that of a chronic osteomyelitis.

The plain film appearance of osteomyelitis differs to that osteosarcoma in that the medullary

lucencies in osteomyelitis are highly evident usually appearing as a tubular lucency along the

shaft with cortical irregularity and with associated extensive demineralization of the peripheral

bone. It is further characterized by thick, irregular, cortical sclerosis with radiolucencies, an

elevated periosteum, and usually chronic draining sinuses are observed. The area of

destruction is poorly defined and has a fine, granular or slightly mottled appearance.

Chondrosarcoma

It is a malignant tumor which develops from cartilage cells. It is only about half as common as

osteogenic sarcoma, constituting 20 to 25% of all bone sarcomas and it is a tumor of

adulthood and old age with a peak incidence in the fourth to sixth decades of life. It can arise

de novo or as a malignant transformation of an enchondroma or, rarely, of the cartilaginous

cap of an osteochondroma. It has a predilection for the flat bones, especially the shoulder and

pelvic girdles. Like other bone sarcomas, high grade chondrosarcomas may spread to the

lungs. Radiographically, the lesion may have a lobular appearance with mottled or punctate

or annular calcification of the cartilaginous matrix.

It is included in our differential diagnosis because chondrosarcomas are also noted to affect

the diaphyseal portions of long bones. And it has an indolent natural history which typically

presents as pain and swelling with progressive increase in the size of the mass.

Its exclusion holds the fact that our patient is only in his adolescence as in opposition to older

age predisposition of chondrosarcoma. It differs radiographically, in that it has a lobular

appearance with mottled calcification versus that of our patient which has a sunburst

appearance. It is also noted to occur more commonly in the pelvis and less frequently seen in

the long bones. Its incidence is less common compared to osteosarcoma.

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Giant cell tumor (osteoclastoma)

It is a relatively uncommon tumor accounting for only 4-5% of primary bone tumors and 18.2%

of benign bone tumors. It is characterized by the presence of multinucleated giant cells. It is

often a solitary lesion, usually of slow rather than fast growing tumor and is seen most

frequently in young adults, peaking in those aged 20-30 years. It causes swelling with little

pain and appears in roentgenograms as an eccentric area of bone destruction. These tumors

do not have a uniform clinical course; some are quite aggressive in rapidity of growth and

destruction of bone. Most are considered benign, but many recur after curettement and a few

become frankly malignant. Metastasis to the lungs may occur.

Roentgenographic appearance shows an area of radiolucency in the epiphyseal and

metaphyseal regions of a long bone with expansion and thinning of the overlying cortex. There

are lines of relative thickening of the involved cortical bone and the reactive shell may produce

the x-ray appearance of bony septa, but these are only on the surface and do not extend

through the tumor. The lesion usually has a sharp zone of transition and is fairly well

demarcated from the normal bone, but there is no sclerotic rim.

It is included as one of the differentials due to the fact that most giant cell tumors, about 60%,

occur in the long bones including the proximal tibia, distal femur, distal radius, and proximal

humerus. Patients do often complain of pain and swelling at the affected site.

Its exclusion is made via radiography because clinically, its differentiation from osteosarcoma

is often difficult due to its comparable presentation and progression. In the plain film, this

tumor presents as an area of radiolucency in the metaphyseal and epiphyseal regions of a

long bone with expansion and thinning of the overlying cortex. Periosteal reaction is usually

minimal against that of osteogenic sarcoma.

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Course in the wards

On admission, patient came in ambulatory and vital sign were taken and were stable. Initial

laboratories were ordered and patient was then transported to the ward. In the ward, patient

was received per stretcher without IV fluid attached. Vital sign were monitored per shift and

were recorded. Patient was noted to complain of minimal dull aching pain in his left leg, but

was tolerable. The 1st hospital day was generally uneventful.

On 2nd hospital day, patient was still noted to complain of dull aching pain and was then given

Tramadol+Paracetamol (Dolcet) 325mg/37.5mg tab TID po for pain and claimed to have some

temporary relief. No other complaints noted. Laboratory results were then followed-up and

secured (refer to appendix B). Erythrocyte sedimentation rate was at 44 mm/hr (reference: 0-

10mm/hr) and the rest of the hematologic studies and urinalysis were unremarkable. The rest

of the day remained uneventful.

On the 3rd hospital day, patient was scheduled for Emergency Above Knee Amputation, Left

(AKA); and was advised to secure 1 unit of Fresh whole blood type A+. Patient was also seen

by Anesthesia and pre-operative medications were ordered and patient was then kept on NPO.

Patient was still complaining of slight breakthrough pain but claimed to be tolerable. Vital sign

still remained stable. No new complaints were noted.

4th hospital day, AKA was rescheduled due to failure to secure blood and some medications.

Instead, was rescheduled for elective surgery on the next day and patient was made to secure

an additional unit of FWB type A+ for the said contemplated surgery. Patient remained to be

stable and no new complaints noted.

5th hospital day, IVF was started with PLR 1L and regulated at 30gtts/min prior to transport.

FWB, medications and consent were secured and patient was then transported to the OR.

Surgery went uneventful lasting for almost 3 hours under spinal anesthesia and no blood

transfusion was done. The amputated extremity was given to family for biopsy. Patient was

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then transported to the recovery room where vital signs were closely monitored. Post-surgery

medications were given like Tramadol 100mg IVTT initially and then 50mg IVTT every 6 hours

thereafter; Ketorolac 30mg IVTT initially then 15mg IVTT for 3 doses; Ranitidine 50mg IVTT

initially then every 8hrs for 3 doses; and Cefuroxime 750mg IVTT every 8hrs after an initial

skin test was done. Patient displayed stable vital signs after few hours of close monitoring

thus was then transported back to the ward. In the ward, pain scoring was recorded every 2

hours, patient was already complaining of pain in the left groin and thigh. Patient was noted to

have difficulty of sleeping due to post-operative pain.

6th – 7th hospital day, patients recovery was noted to be at par. Pain was gradually

deteriorating to tolerable state. Daily wound care was done. Vital signs and pain score

monitored regularly. Intravenous fluid and medications were continued.

8th hospital day, family was noted to refuse sending the amputated limb for biopsy for financial

reasons. Post-operative recovery was still at par. No signs of fever or infection noted. Pain

was gradually deteriorating.

9th hospital day, post-op recovery was still at par. Pain was tolerable and vital signs remained

stable. No signs of fever or infection noted. Daily wound care and intravenous medications

were still continued.

10th hospital day, patient was seen by IM-oncology section and was advised for chemotherapy

and also advised to take Multivitamins+Iron 1 tab po daily. No new complaints noted.

11th hospital day, intravenous fluid and parenteral medications were discontinued. Oral

medications were started with Cefuroxime 500mg cap bid po and Mefenamic acid 500mg cap

TID po.

12th hospital day, patient was again visited by IM-oncology section and was advised to follow-

up as OPD on ******* in IM office for scheduling of chemotherapy.

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13th hospital day, patient was allowed to go home with take home meds of Cefuroxime 500mg

cap BID po for 10 days more and Mefenamic acid 500mg cap TID po for 7 days more. Patient

was also schedule to visit the Rehab department for his physical therapy management on

******** and to follow-up in orthopedics in ****** OPD on the same day. Patient was

discharged improving.

Laboratory results

Complete blood count

Hematocrit = 0.44/L

White blood cell = 10. 25 x 109/L

Segmenters = 0.66

Monocytes = 0.01

Lymphocytes = 0.26

Platelets 345x109/L

Blood type A+

Urinalysis

Color = Yellow Red blood Cells = 0-1/hpf

Transparency = Cloudy Pus Cells = 0-1/hpf

Reaction = pH 6.0 Amorphous Urates = Abundant

Specific Gravity = 1.030 Bacteria = Rare

Sugar = Negative

Protein = Negative

CT-BT

Clotting Time = 3 mins x 5 secs

Bleeding Time = 1 min x 2 secs

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Partial Thromboplastin Time (PTT/APTT)

Patient 39.2 secs

Control 35.5 secs

ESR

Erythrocyte Sedimentation Rate = 45 mm/hr

Prothrombin Time

Patient = 12 secs

Activity = 91.3%

INR = 1.01

Control = 11.3 secs

Control Activity = 102.8%

Chest X-ray report

The trachea and mediastinum are in the midline. The heart is normal in size and shape. The

lung fields on both sides are also normal. The diaphragms are smooth. The costophrenic

angles are sharp. The bony thorax shows no abnormality.

Conclusion: Negative Chest

Chest X-ray report

Both lung fields are clear. The heart is normal in size and shape. The trachea is at the

midline. The diaphragms are depressed. The osseous thoracic cage showed no significant

bony abnormality.

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Conclusion: Hyperaeration of both lungs. This raises the question of lower airway obstruction

(Bronchial Asthma).

X-ray of the Left Leg APL

There is cortical thickening and cloudlike proliferation in the external anterior surface of the

proximal 3rd to the middle 3rd of the left tibia with soft tissue swelling. The epiphyses are not

fused yet.

Conclusion: The above findings is suggestive of periosteal osteosarcoma, suggest biopsy.

Fine Needle Aspiration Biopsy, Left Anterior Leg

Smears are moderately cellular consisting of sheets, clusters and individually scattered of

highly atypical spindle-shaped cells. These cells have round to ovoid, pleomorphic, and

hyperchromatic nuclei with coarse chromatin pattern surrounded by a scanty cytoplasm. No

organoid pattern is seen. The background is bloody.

Diagnosis: Cellular findings highly suggestive of sarcoma

Surgical Pathology report

Gross description: The specimen consists of a few fragments of firm, light brown tissue

admixed with blood clots measuring 1 cm in aggregate. These are entirely processed.

Microscopic: Section shows fragments of a malignant neoplasm composed of tumor cells with

large, pleomorphic and hyperchromatic nuclei, some vesicular with conspicuous nucleoli and

some with intranuclear inclusions. Several mitotic figures are seen. These cells are

surrounded by small irregular trabeculae of eosonophilic osteoid.

Diagnosis: Tibia, left, middle third, shaft: OSTEOSARCOMA

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Discussion of the case

Background

It is the most common malignant bone tumor (Vander Griend, 1996). And it is thought to arise

from primitive mesenchymal bone-forming cells and later becoming a malignant osteoid. It is a

deadly form of musculoskeletal cancer that most commonly causes patients to die from

pulmonary metastatic disease.

History

Osteosarcoma is an ancient disease that is still incompletely understood. The term sarcoma

was introduced by the English surgeon John Abernathy in 1804 and was derived from Greek

roots meaning fleshy excrescence (Peltier, 1993). In 1805, the French surgeon Alexis Boyer

(personal surgeon to Napoleon) first used the term osteosarcoma and he realized that it is a

distinct entity from other bone lesions such as osteochondromas (exostoses). Its gross

appearance was described 1847 by Baron Guillaume Dupuytren as a whitish or reddish mass,

lardaceous and firm at an early stage of the disease; but presenting at a later period, points of

softening, cerebriform matter, extravasating blood, and white or straw colored fluid of a viscid

consistence in its interior.

Incidence

The incidence of osteosarcoma is slightly higher in males than in females with males (5.2 per

million per year) and females (4.5 per million per year). It is very rare in young children (0.5

cases per million per year in children <5 y), incidence increases steadily with age, increasing

more dramatically in adolescence, corresponding with the growth spurt.

Etiology

Rapid bone growth appears to predispose persons to osteosarcoma, as suggested by the

increased incidence during the adolescent growth spurt, and osteosarcoma’s typical location in

the metaphyseal area adjacent to the growth plate (physis) of long bones.

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There are some evidences of genetic predisposition for osteosarcoma in patients with bone

dysplasia (Paget disease, fibrous dysplasia, enchondromatosis, and hereditary multiple

exostoses and retinoblastoma). It is thought that the combination of constitutional mutation of

the RB gene and radiation therapy might develop chances for osteogenic mutation. The only

known environmental risk factor is exposure to radiation. But in most cases of osteogenic

sarcoma no definite cause can be determined.

Pathophysiology

Osteosarcoma is a malignant mesenchymal sarcoma characterized by the direct formation of

bone or osteoid by tumor cells. Osteosarcoma is subclassified depending on the histologic

characteristics of the cells. When the majority of the cells produce osteoid, the tumor is called

osteoblastic. Where production of chondroid matrix is predominant, the tumor is classified as a

chondroblastic osteosarcoma. When spindle cells are in the majority and little matrix is formed,

the term fibroblastic osteosarcoma is used.

Anatomical PredispositionIt usually arise as solitary lesions within the fastest growing areas of the long bones, with the

top three sites includes the femur (42%, 75% of which are distal femur), tibia (19%, 80% of

which are proximal tibia), humerus (10%, 90% of which are proximal humerus), and other

significant locations are the skull and jaw (8%) and pelvis (8%), but virtually any bone can be

affected. Not all osteosarcomas arise in a solitary fashion, as multiple sites may become

apparent within a period of about 6 months (synchronous osteosarcoma), or multiple sites may

be noted over a period longer than 6 months (metachronous osteosarcoma). Such multifocal

osteosarcoma is decidedly rare, but when it occurs, it tends to be in patients younger than 10

years

Clinical Presentation

Pain, as a rule the first symptom, is of dull, constant, aching character and often interferes with

sleep. The usual physical examination findings include a palpable mass which may or may not

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be present. The mass may be tender and warm, though these signs are indistinguishable from

osteomyelitis. In the case of lower limb lesions, the discomfort may result in a slight limp.

Pathologic fracture is uncommon in osteogenic sarcoma. In rapidly growing tumors, distention

of the superficial veins and elevation of the skin temperature over the lesion are common.

Pulsations or a bruit may sometimes be detectable. The range of motion might be decreased if

a joint is involved. Involvement of local or regional lymph nodes is unusual. Auscultation is

usually uninformative unless disease is extensive. In more than half of patients the serum

alkaline phosphatase level is elevated, reflecting the osteoblastic activity of the tumor cells.

Laboratory

Important laboratory studies include the following:

LDH (it is helps to determine the present state of cell turnover)

Alkaline phosphatase (is of prognostic significance because an elevated alkaline

phosphatase at diagnosis is more likely to have pulmonary metastases)

CBC count with Platelet count (hematology may also help to determine if concomitant

anemia is existing)

Liver function tests: Aspartate aminotransferase, alanine aminotransferase, bilirubin,

and albumin (it helps to assess liver status and possible involvement)

Electrolytes: Sodium, potassium, chloride, bicarbonate, calcium, magnesium,

phosphorus

Renal function tests: BUN, creatinine

Urinalysis

Imaging Studies

X-rayRoentgenographic picture shows areas of both bone destruction and of new bone

formation. The degree of each is highly variable, some tumors being very radiopaque

and others showing extensive demineralization with areas of cortical destruction where

tumor has broken through and elevated overlying periosteum or cortical extension is

seen. Beneath this expanded periosteal sleeve, small spicules of bone radiating at right

angles to the shaft (sunburst effect) are frequently present. At proximal and distal

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extremes of this elevated periosteum a small triangle of reactive bone, Codman’s

triangle, may be found.

It is advisable to obtain plain films of the suspected lesions in 2 views. It is important to

note that no single feature on radiograph is diagnostic. Taking chest film is of value in

order to determine if the pulmonary system is already involved.

CT scanA CT-scan of the primary tumor is best for defining bone destruction and the pattern of

the calcification. It is critical for surgical planning

MRIIt is the best method to assess the extent of intramedullary disease as well as

associated soft tissue masses and skip lesions. It is also the single most important

study for accurate surgical staging of the lesion using the Enneking staging system.

Enneking staging system (1983)

The purpose of staging tumors is to stratify risk groups. It is also referred to as the staging

system of the Musculoskeletal Tumor Society and this system applies to al`l musculoskeletal

tumors (both bone and soft tissue). The key components to the staging system are the

histologic grade of the tumor (low grade versus high grade), anatomic location of the tumor

(intracompartmental versus extracompartmental), and the absence or presence of metastatic

disease.

The staging system is typically depicted as follows:

Low-grade tumor, intracompartmental - I-A

Low-grade tumor, extracompartmental - I-B

High-grade tumor, intracompartmental - II-A

High-grade tumor, extracompartmental - II-B

Any tumor with evidence of metastases– III

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Substage A - Intracompartmental lesion (intramedullary lesion for bone

tumors)

Substage B - Extracompartmental lesion (extramedullary spread for bone

tumors)

Radionuclide bone scansIt uses technetium Tc 99-methylene diphosphonate and it is important to evaluate for

the presence of metastatic or multifocal disease with a bone scan.

Angiography It is not helpful for diagnosis, but it is the most sensitive test for assessing the response

to preoperative chemotherapy. Most osteosarcomas are high grade.

Histopathology

The tumor cells have a frankly sarcomatous appearance, with large, bizarre, and

hyperchromatic nuclei and frequent mitotic figures. The cells contain high levels of

alkaline phosphatase. The hallmark of osteogenic sarcoma is the osteoid matrix

produced by the tumor cells. The tumor interdigitates with trabeculae of normal

metaphyseal bone.

Treatment

The orthopedic surgeon is of paramount importance in the care of patients with osteosarcoma.

Since osteosarcomas are not particularly responsive to radiotherapy, surgery is the only option

for definitive tumor removal (local control). In addition, an oncologic type of total joint

prosthesis or complex bone reconstruction may be required following surgical resection.

Therefore, close involvement of the orthopedic surgeon with the medical oncologist at the time

of diagnosis, as well as during and after chemotherapy, is critical.

The primary aim of definitive resection is patient survival. As such the margins on all sides of

the tumor must contain normal tissue (wide margin). The thickness of the margin is important

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only for the marrow, where an adequate margin is thought to be 5-7 cm from the edge of

abnormality depicted on MRI or bone scan. But amputation may be the treatment of choice in

some circumstances.

When amputation is decided, a well planned rehabilitative program should be designed in

partnership with the physiatrist and physical therapist for the patient’s post-amputation

recovery.

The most important prognostic factor for long-term survival is response to chemotherapy.

Preoperative (neoadjuvant) chemotherapy followed by limb-sparing surgery (which can be

accomplished in > 80% of patients) followed by postoperative (postadjuvant) chemotherapy is

standard management. The use of neoadjuvant chemotherapy has been found to facilitate

subsequent surgical removal by causing tumor shrinkage and has also provided oncologists

with an important risk parameter. Patients who have a good histopathological response to

neoadjuvant chemotherapy (>95% tumor cell kill or necrosis) have a better prognosis than

those whose tumors do not respond as favorably. The effective drugs are doxorubicin,

ifosfamide, cisplatin, and high-dose methotrexate with leucoverin rescue.

Prognosis

The present understanding of outcome and prognosis for osteosarcoma is driven by certain

serum markers, clinical staging, and histologic response to chemotherapeutic agents. The

overall 5-year survival rate for patients diagnosed between 1974 and 1994 was 63% (59% for

males, 70% for females). Patients with elevated alkaline phosphatase at diagnosis are more

likely to have pulmonary metastases. In patients without metastases, those with an elevated

LDH are less likely to do well than are those with a normal LDH. Long term survival rates in

extremity osteosarcoma range from 60 to 80%. Osteosarcoma is radioresistant; radiation

therapy has no role in the routine management.

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Conclusion

Osteosarcoma is a serious and fatal disease of the bones in which patients would die of

pulmonary cause. It usually affects the younger age group, peaking during the adolescent and

in the young adult. Clinically, it usually manifests as a slowly growing solitary mass in the long

bones and is frequently seen around the joint area. It is also associated with dull, aching pain

around the lesion. Its diagnosis is based mainly on clinical findings. Radiologic study of

osteosarcoma plays an essential role in the surgeon’s diagnosis. It is a diagnostic modality

that is easily available; it is non-invasiveness, and is highly sensitive in illustrating the disease.

The definitive diagnosis is eventually made through biopsy. Once diagnosis is made, it is

usually managed surgically with amputation of the affected limb and supported with

chemotherapy.

Recommendation

The occurrence of osteosarcoma in our medical center is common with early detection and

management being crucial in improving the outcome patient’s recovery. The author would like

to make the following recommendations:

To educate patients who are diagnosed with osteosarcoma about the disease, signs and

symptoms, different kinds of treatment and its outcome.

To create an organized multi-disciplinary body geared especially in the treatment of

osteosarcoma. This involves the team approach of the departments of Orthopedic, IM-

Oncology, Radiology, Pathology, Rehabilitation-Medicine and Psychiatry, especially if it is a

pediatric patient with a contemplated amputation being perceived.

I would also recommend that a formal inquiry of the present statistic on the occurrence of

osteosarcoma in our hospital be conducted. This includes the management and outcome, and

recurrence after chemotherapy if present.

The participation of psychiatry department be encourage in the present management of

osteosarcoma patients as they play a crucial role in its pre-and post- operative management.

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6. American Cancer Society

7. Radiologic Society of North America 1995