You are on page 1of 143


Group of 15-C
01 Terminology

02 Problem Identification

03 Problem Analysis

04 Scheme

05 Learning Objective

Increased osteoclast cells
or cells of bone destruction Onion Skin
caused by neoplasm Appearance
The description of an
Periosteal Reaction obscure bone destruction
The formation of new bone in the form of layers found
layer due to injuries that in the diafisis
stimulate the layer of the
periosteum Open Biopsy
in the form of retrieval and
examination to see
microscopic cell body by
incision or excision of
tissue yg will be taken.
Problem Identification

What causes Erwin can suddenly fall and could not

1 stand while playing football and felt very sick?

What is the relationship of age and gender with

22. complaints suffered by Erwin?

What is the relationship of Erwin mother and

3 father jobs with the condition he suffered ?

Why Erwin experiencing swollen 6 months ago

4 and accompanied by pain at night?
What is the interpretation of the anamnesis and
5 physical examination on swelling in the right thigh?

6 What is the impact of the pain and swelling if not

Problem Identification

Why does the doctor recommend Erwin to

7 immediately referred to?

Why is arriving at the hospital carried out an

82. examination of x-rays and what the interpretation
of the results of his examination?
what types of tumors that become the cause of
9 complaint Erwin after an examination of x-rays?

Why do doctors diagnose for sure by using

10 open biopsy?
Why is the doctor going to do chemotherapy,
11 radiotherapy, even surgery in the case of the Erwin?

12 Is the disease can be cured by Erwin?

Problem Analysis
1. What causes Erwin can suddenly fall and could
not stand while playing football and felt very sick?

Football--> pathologic fractures (6 last month already swollen)-->so while playing

soccer (heavy activities) easy to occurrence of fracture

Growing pain--> muscle pain increased when doing activities on the damaged muscles

Pain can also be due to neoplasm  bone-somatic pain  quick and strong pains 
Ewing sarcoma

Ewing sarcoma-> intermitten, progressive and symptoms occur in children and adoles
cents aged 10-20 years
Problem Analysis
2. What is the relationship of age and gender with
complaints suffered by Erwin?

The age  Ewing sarcoma common older teens (10-20 yrs)

Because genetic occurrence of chromosomal translocation 11 & 22 with the type of

the primary tumor.

Gender  male: female = 1.5 : 1

Problem Analysis
3. What is the relationship of Erwin mother and father
jobs with the condition he suffered ?

The father was a laborer  exposure to carcinogenic substances against the child

The mother of a teacher  social economy enough .

Problem Analysis
4. Why Erwin experiencing swollen 6 months ago and
accompanied by pain at night?

6 months  the progressive growth of the tumor.

Ewing's tumor is felt after a few weeks/a few months

Pain at night :
 Osteosarcoma and Ewing's Sarcoma
 Radiologic result leads to ewing sarcoma
 An increase in prostaglandin E2 induced tumor cell proliferation  due to the
enzyme cox high on neoplasm cells metabolism--> activities less night so that
the body's energy usage in the evening focused on the proliferation of cells.
Problem Analysis
5. What is the interpretation of the anamnesis and
physical examination on swelling in the right thigh?

Anamnesis complaints neuromuskuloskeletal  PQRST



Problem Analysis
6. What is the impact of the pain and swelling if not treated ?

The prognosis of the disease If treated early in a disease that is known, then the
prognosis is better

If left untreated  the degree of severity is increasing  handling the more difficult
 prognosis gets worse with the survival rate decreases
Problem Analysis
7. Why does the doctor recommend Erwin
to immediately referred to?

Erwin fall suddenly  not because of trauma

Signs of malignancy  Swelling 6 months accompanied by pain symptoms at night

and when pressed there is swelling, tense, hard-elastic, as well as an increase in
the local temperature.

Competence of Ewing Sarcoma  Competency 1

Referred to  for X-rays, MRI , ct scan, and biopsy

Problem Analysis
8. Why is arriving at the hospital carried out an examination of x-
rays and what the interpretation of the results of his examination?

X-rays  look at the inside of the bone

 the existence of a periosteal reaction
 bone cortex thinning
 periosteum is elevated
 the boundaries that are not expressly

The results obtained by the onion skin appearance

Problem Analysis
9. what types of tumors that become the cause of complaint
Erwin after an examination of x-rays?

Ewing Sarcoma  Onion Skin Appearance

DD  Osteosarcoma
Problem Analysis
10. Why do doctors diagnose for sure by using open biopsy?

Make sure what type of malignancy and the degree of malignancy.

Problem Analysis
11. Why is the doctor going to do chemotherapy,
radiotherapy, even surgery in the case of the Erwin?

Chemotherapy radiotherapy  inhibits tumor cell growth and prevent metastasis.

Surgery  a high degree of severity  lifting these tumor cells

Problem Analysis
12. Is the disease can be cured by Erwin?

It is difficult because of the high rekurensi of Ewing Sarcoma (poor prognosis).

Life expectancy :
Stadium 1 & 2 = 70%
Stadium 3 & 4 = 15-30%
Learning Objective

1. Ewing Sarcoma
2. Osteosarcoma
3. Giant Cell Tumor
4. Multiple Myeloma
5. Bone Cyst
6. Osteoid Osteoma
7. Osteocondroma
8. Osteoblast
9. Soft Tissues Benign
10. Soft Tissues Malignancy
Ewing’s Sarcoma
• Ewing’s sarcoma is a small round-cell tumor typically arising in the
bones, rarely in soft tissues, of children and adolescents.
• All are characterised by recurrent chromosomal
translocation involving 11 & 22 (85%) & 21 &22 (15%)

2nd most common primary osseous

malignancy in children
Incidence is 2.1 / million
Rare in African-Americans and Asian
80% occur between ages 5 and 25

Male : Female
1.3:1 < 10 yrs
1.6:1 > 10 yrs

• Localised,painful, tendermas

• Systemicsymp.–fever,malaise,weight loss

• mistakenforOM

• disseminationoftumor

• Maymetastasizeto otherbones

• Elevated WBC & ESR,LDH

• pathological fracture
• The lesion is poorly defined
• Permeative or moth-eaten
type of bonedestruction
• Aggressive periosteal
onion peel
codman triangle
sunburst appearance

• Large soft-tissue mass

• Occasionally, the bone lesion it

self is almost imperceptible,
with the soft-tissue massbeing
the only prominent feature

• Cells are uniform and round

to oval

• Cytoplasm is scanty & lacy

• Nuclei are round to oval,

have a delicate ,finely di
spersed chromatin

• Nucleoli are inconspicuous

• Mitotic figures rare


1. CHEMOTHERAPY- control ofmicrometastasis

2. SURGERY - local control where possible

3. RADIOTHERAPY-local control where surgery

is not possible or incomplete
Radiosensitive tumor
• Poor prognostic factors:
• Age >17ys
• Large size tumor > 8cm
• Metastases
• Pelvic & Sacral tumors
• Fever, Leucocytosis & Raised ESRat presentation
• <90% necrosis after Chemo
• Extraosseous soft tissue extension
• Postradiation medullary tumor
• Small bones(strong CD99+ve) – better prognosis
Osteosarcoma Defined

Initially discovered in bones of animals such

as bears in Europe (1776)
First Human Case Discovered in 1788 in
Osteosarcoma itself is a division of bone
cancer that targets specific areas around the
body, most often at joints.
The majority of those affected are in their teenage years. The
cancer also affects certain patients above the age of 40.
Factors such as ethnicity are irrelevant
Biggest majority of those affected are teenage boys of above
average height.
Exact Cause Unknown
Risk Factors Include Predisposition to:
Li-Fraumeni Syndrome
Loss of Mitotic Control Through p53
Broken Bones
Growth Spurts
Radiation Exposure
Infection Sites
Symptoms include pain and swelling:
distal femur
proximal tibia
proximal humerus
Symptoms may worsen at night or during exercise
Early symptoms often overlooked
Athletic Adolescents often sustain injuries which would
elicit the same result
“Growing Pains”
X-ray or CT/MRI Scan of suspected area is ordered
Scans also taken of Chest and Lung cavity
Pleural Cavity scan often reveals metastases
Tumor/Metastases identification results in a biopsy of the area
Biopsies often performed by the surgeon slated to remove the tumor
Malignant Tumors mandate start of a therapy routine
Loosen Cancerous Hold
Shrink Tumor Size
Aim to Kill 90% of cancerous cells
Limb Salvage
Checkup Visits

Mortality Rate:
400 New Cases per year in the US
Kills 1470 people per year Worldwide.
70% Survival Rate for those whose disease has not metastasized at
the time of diagnosis
30-50% Survival Rate for those people whose cancer has
Surviving Patients can expect to live a long, healthy life in
Giant Cell Tumor of Bone
• GCTs are benign tumors with potential for aggressive behavior and
capacity to metastasize.

• Although considered to be benign tumors of bone, GCT has a relatively

high recurrence rate. Metastases occur in 1% to 9% of patients with
• Neoplasm arising from non-bone forming supportive
connective tissue of marrow with network of stromal cells
regularly interspersed with giant cells
• These giant cells resemble osteoclasts
• 20% of biopsy analyzed benign bone tumors.
• It affects young adults between the ages of 20 and 40 years
• several authors have reported a slight predominance of
women over men.
• 1,5 : 1 = F :M
• The most common locations, in
decreasing order, are the distal
femur, the proximal tibia, the
distal radius, and the sacrum

• Ninety percent epiphyseal


• Fifty percent of GCTs arise

around the knee region
Clinical presentation
• Pain
• Swelling
• A soft tissue mass
• 12% present with pathologic fracture
• To rule out hyperparathyroidism
Blood :
– serum calcium
– Phosphorus
– Alk.phosphatase
• Surgical resection
is the universal standard of care for treatment of GCT of bone

• Intralesional Curettage  high recurrence rate

Reconstructing the defect after curettage : bone graft/ bone cement

• Amputation
– Severe disability
– Malignant tumor
– Recurrence after surgery
Multiple Myeloma

• Multiple myeloma is a clonal plasma cell neoplasm

• Usually accompanied by monoclonal antibody production
• 1% of all cancer
• Median age 65 years
• Incidence higher in African populations
Age-adjusted Incidence per 100,000

Male Female

White 6.2 4.1

Black 11.8 10.0


• Familial clustering
• African Americans
• Radiation
• Agriculture, Benzene, Radiation, Sheet metal work
• Chronic inflammatory disorders
Normal B cell Development
Pre B cell

B cell Follicles

Marrow Travel

Lymph Node
B cell finds “meaning”


B cell activation
Germinal Center
Plasma Cells travel
back to bone marrow

Memory B cell

“Activated B cell”

Plasma Cell
Properties of Plasma Cells

• Proliferate
• Secrete Immunoglobulins
• “Make space”
• Influence bone turnover
• Secrete Inflammatory mediators
Clinical Manifestations

• Plasma Cell proliferation

• Pancytopenia, bone damage, constitutional symptoms, anorexia, cachexia, hypercalcemia
• Monoclonal protein production
• Renal failure, hyperviscosity, amyloidosis, hypoalbuminemia, neurologic symptoms
• Immunodeficiency
• Infection, autoimmune phenomena
Presenting Symptoms and Signs

• Symptoms • Signs
• Back Pain • Lytic lesions
• Fatigue • Anemia, pancytopenia
• Anorexia • Hypercalcemia
• Recurrent infection • Renal insufficiency
• Constipation • Monoclonal proteins
• Somulence • Organomegaly
• Fracture • Bone tumors
• Neuropathy • Hypogammaglobulins
Initial Diagnostic Workup

• H&P • Bone Marrow Biopsy

• CBC • 24-hour urine
• BUN/creat, lytes • UPEP/immunofix
• Calcium/albumin • Beta2-microglobulin
• Quant Ig • Skeletal survey
• SPEP/immunofix
Lytic Bone Lesions in Myeloma

•Important for diagnosis

•Treatment of impending fracture
Greater than 20% plasma cells

• Stage I (All) • Stage III (Any)

• Hgb > 10 g/dl • Hgb < 10 g/dl
• Normal calcium • Hypercalcemia
• Normal bones or Solitary plasmacytoma • Multiple lytic lesions
• Low M-protein • High M-protein
• IgG < 5 g/dl • IgG > 7 g/dl
• IgA < 3 g/dl • IgA > 5 g/dl
• Light chains < 4 g/24 h • Light chains > 12 g/24 h

•Stage II – not fitting I or III

Smoldering Myeloma

• Monoclonal gammopathy
• IgG > 3.5 g/dl and < 5 g/dl
• IgA > 2 g/dl and < 3 g/dl
• Urine light chains > 1 g/dl
• Bone Marrow Plasma cells
• Greater than 10% and less than 20%
• No anemia, renal insufficiency, hypercalcemia
• No lytic lesions or diffuse osteopenia
NCCN Treatment Guidelines

• National Comprehensive Cancer Network

• Group of NCI Cancer Centers
• Evidence based guidelines of appropriate care for general population
• Reviewed annually and updated by panel members
• Available online:

• Myeloma is a cancer of plasma cells

• Patients suffer primarily from bone disease, anemia and renal disease
• Conventional treatment is non-curative
• Aggressive treatment with high-dose chemotherapy preserves quality life
• Supportive care improves quality life (and survival)
Bone Cyst
Osteoid Osteoma
• Bony (eksostosis) with the cover of which is derived from the
external surface of the bone.
• Osteokondroma contribute to more than the external surface
of the bone
• 80-85% of cases are often aged 10-25 years
• More commonly found in men than women
• The second most common benign tumors (32.5%) of the whole
of the benign tumors of the bone and is mainly found in
teenagers growth off and on young adults.
• Hard lumps can be found in the area around the lesion.
Clinical Manifestations
• The symptoms of pain in the jointsVery slow tumor growth and
50% of the tumors become malignant
• Radiological Examination-X-rays look clear on bone retraksi
cancellous bone is stretched beyond the line of the epifisis.
• The form of oddball with a thin cortex but the penetration
came out rare.
• Borderline tumors are irreguler, not expressly accompanied by
calcification spots the presence of calcium deposition.
• Rare benign bone tumor. Just 1 % from primar bone tumor
• Wide age range (6-75) years old. Often in +- 20 years old
• Can affect to any bone. 40 % in spina but also can attack arm
leg and limbs
 Rad The Radhiographic picture is often diverse and less
 Most have sharp edges on the edges that have sclerotic
bone edge skin
 Most tumors remain confined to the bones and do not
destroy or penetrate the cortex
 25% of its appearance can mimic malignancy

At the examination FNAB was received

 Hip Hypocellular cells, mineralization from tumors that
are not the same, many traabular cells that are not
maatang, iant cell may be appearance
Difference between Osteoblastoma and Osteoid Osteoma
• Osteoblastoma usually occurs in the axial skeleton while
the osteoid osteoma is often long bone
• Ostoblastom usually has persistent disease while the
osteoid osteoma has an increased pain during the night
• Both of them are the same as often in young adults but at
all ages can

• main treatment is still surgical, exciciary of tumor tissue
(stage 1-2 es internal excision) (stage 3 wide excision)
• Chemotherapy and radiation provide controversy
Soft Tissues Benign
Tumor jaringan lunak yang paling sering ditemuk
an, biasanya muncul antara usia 40 dan 60 tahun

•Tumor mesenkim jinak ya SKDI : 4A.

ng berasal dari jaringan le Dokter diharapkan dapat m
mak. endiagnosis lipoma dan me
lakukan penatalaksanaan li
•Dapat muncul di mana saj poma secara mandiri dan tu
a di tubuh, umumnya pada
jaringan subkutis.
Definisi Lipoma
• Tumor adiposa yang tumbuh lambat dan jinak
• Umumnya memiliki konsistensi yang lunak, dan sering mempunyai kapsul, kadang-
kadang tumbuh infiltratif
• Paling sering ditemukan pada jaringan subkutan, dapat ditemukan di jaringan yang
lebih dalam seperti otot, saraf, sendi, tendon, septa intermuskular, organ abdomen,
rongga mulut, dll.
Epidemiologi Lipoma

• 16% dari semua tumor mesenkim adalah lipoma

• Dapat terjadi pada semua usia, biasanya muncul antara usia 40 dan 60 tahun.
Etiologi Lipoma

• Penyebab sebenarnya tidak diketahui

• Spekulasi tentang hubungan potensial antara trauma dan pembentukan lipoma
• Spekulasi hubungan penyusunan ulang kromosom 12 pada kasus lipoma soliter
karena adanya kelainan pada fusi gen HMGA2-LPP
• Gangguan metabolisme lemak, terjadi proliferasi baik histologi dan kimiawi
Manifestasi Klinis


Intestines  Subkutis 
nyeri benjolan
abdomen, teraba lunak,
perdarahan mobile

Saluran napas
Lipoma Tidak nyeri -
 gagal

Esofagus 
obstruksi, Sendi 
disfagia, ROM terbatas

Pemeriksaan Fisik
Anamnesis 1. Benjolan teraba lunak
2. Lobulasi Pemeriksaan Penunjang
1. Gejala klinis
3. Mobilitas bebas dari kulit 1. USG
2. Waktu pertumbuhan di atasnya (slippage sign)
lipoma lambat 2. CT-scan
4. Bentuk bulat atau
ireguler 3. MRI
5. Single atau multiple 4. Biopsi
6. Ukuran nodul ±2-10 cm
Diagnosis Banding

Liposarcoma Epidermoid cyst Abses

• Ukuran >5cm, bisa • Kista epidermoid • Dikelilingi oleh

lebih besar subkutan biasanya eritema
• tumbuh perlahan bulat dan kencang • Berkembang dengan
selama bertahun- • Punctum sentral cepat selama
tahun atau cepat sering terlihat, keluar beberapa hari
dalam waktu singkat eksudat putih • Biasanya hangat,
• Teraba lebih kencang lunak dan fluktuatif
• Pemeriksaan saat diraba.

Eksisi Liposuction


++ • Jaringan
lebih kecil
 • Jarum ukuran 16-gauge
dan jarum suntik ukuran
• Anastesi dengan lidokain

Lipoma lunak
-- • Sulit eliminasi
jaringan ikat • Sulit untuk
yang lebih kecil diagnosis

 • Injeksi
Atrofi jaringan

• Lipoma
berdiameter <2,5

-- • Tidak ada eliminasi  kekambuhan

• Jaringan parut dan fibrosis

Fungsi otot
ROM terbatas Nyeri saraf

Soft Tissues Malignancy

• Rare: only 8300 new cases annually in U.S.

• 3900 die annually from STS
• Mesodermal origin
Location and Type

• h/o Radiation therapy increases grade of tumors and risk for metastasis
• Chemical exposure
• Thorotrast, vinyl chloride, arsenic for hepatic angiosarcoma
• Genetic syndromes
• Neurofibromatosis – nerve sheath tumors
• Familial gastrointestinal stromal tumor syndrome – KIT mutation
• Skin hyperpigmentation, uticaria, cutaneous mast cell dx

• Soft tissue and bone

• viscera (gastrointestinal, genitourinary, and gynecologic organs)
• nonvisceral soft tissues (muscle, tendon, adipose, pleura, and connective tissue)
• By differentiation (usually with IHC staining)
• adipocytic tumors
• fibroblastic/myofibroblastic tumors
• fibrohistiocytic tumors
• smooth muscle tumors
• pericytic (perivascular) tumors
• primitive neuroectodermal tumors (PNETs)
• skeletal muscle tumors
• vascular tumors
• osseous tumors
• tumors of uncertain differentiation

• Most present as painless mass leading to delayed diagnosis as lipoma or

• Core needle biopsy guided by palpation or by image guidance if not palpable
• Few cases of tumor seeding with closed biopsy so some recommend
tattooing site for later excision with specimen
• Excisional biopsy for superficial small lesions if needle biopsy non-diagnostic
• Incision biopsy
• Longitudinal incision without tissue flaps with meticulous hemostasis to
prevent tumor seeding in hematomas
• Send biopsy fresh and orientated
Tumor seeding after biopsy

• For extremity masses
• Gives good delineation between muscle, tumor and blood vessels
• CT for abdominal and retroperitoneal
• May help determine high vs. low grade
• May be helpful in recurrences

• AJCC/UICC Staging System for Soft Tissue Sarcomas

• T1: <5cm
• T1a: superficial to muscular fascia
• T1b: Deep to muscular fascia
• T2: >5cm
• T2a: superficial to muscular fascia
• T2b: Deep to muscular fascia
• N1: Regional nodal involvement
• Grading
• G1: Well-differentiated
• G2: Moderately differentiated
• G3: Poorly differentiated
• G4: Undifferentiated

Stage IA G1,2 T1a,b N0 M0

Stage IB G2,2 T2a,b N0 M0
Stage IIA G3,4 T1a,b N0 M0
Stage IIB G3,4 T2a N0 M0
Stage III G3,4 T2b N0 M0
Stage IV Any G Any T N1 M1

Staging system predicts survival and risk of metastasis, but not local recurrence
**Does not take into account extremity vs. visceral
Relative risk for recurrence and survival

• Age >50 years 1.6

• Local recurrence at presentation 2.0
• Microscopically positive margin 1.8
• Size 5.0–10.0 cm 1.9
• Size > 10.0 cm 1.5
• High-grade 4.3
• Deep location 2.5
• Local recurrence 1.5

• Limb-sparing vs amputation
• Comparison study with post-op radiation in limb sparing showed no
difference in survival
• Amputation still may be indicated for neurovascular or bone involvement

• Arbitrary 2 cm margin if no plan for post-op radiotherapy

• Negative margins may be adequate for post-op radiation therapy
• Presence of positive margins increases local recurrence by 10-15%
• No need for lymph node dissection as only 2-3% have nodal metastasis
Adjuvant radiotherapy

• Small, low grade tumors resected with 2 cm margins may not require radiation
• Improves local control but not survival
• Whether improved local control leads to improved survival is controversial
Pre-op or post-op radiation?

• Some avoid pre-op use because of increased wound complications (although this
is debatable)
• RCT looking at wound complication rate pre-op vs post-op radiation showed
35% vs 17%
• Risk confined to lower extremity
• Conclusions: pre-op may be better for upper extremity and head & neck
because of equal wound complication risk and benefit of lower radiation
doses to more vital tissues
Pre-op vs post-op radiotherapy

• Can improve local control, but not survival

• Doxorubicin and ibosfamide have response rates of 20%
• Use only in advanced disease
• Combination with radiation or neoadjuvant therapy are controversial
• Hypothermic isolated limb perfusion may be used for palliation
Treatment of Recurrence

• 20-30% of STS patients will recur

• More common in retroperitoneal and head & neck high grade tumors because
hard to get clear margins
• 38% for retroperitoneal
• 42% for head and neck
• 5-25% for extremity
• After re-resection recurrence is 32% for extremity and much higher for visceral
Metastatic disease

• Lung most common site of mets, but visceral often go to liver

• Median survival from development of metastatic disease is 8-12 months
• Resection of pulmonary mets can give 5 year survival of 32% if all mets can be
• >3 mets is poor prognosticator
Thank You for Your Attention
Group of 15-C