Musculoskeletal

Neoplasms

Hasna Dewi, dr., Sp.PA., M.Kes.

Bagian Patologi Anatomi FKIK UNJA
 KUIS
1. A sarcoma is most likely to develop from cells derived from which germ
layer?
A. Ectoderm B. Mesoderm
C. Endoderm D. Retroderm

2. What bone tumour is associated with lifting of periosteum off bone

producing the Codman triangle and a sunburst appearance on X-ray?
A. Osteosarcoma B. Osteoma
C. Ewing sarcoma D. Chondroma

3. Known as undifferentiated malignant tumor. Biopsy of this mass reveals

sheets of primitive cells with small, fairly uniform nuclei and only scant
cytoplasm. This neoplasm more common in children
A. Osteosarcoma B. Chondrosarcoma
C. Ewing sarcoma D. Rhabdomyosarcoma
 4. A 45 yo male has experienced pain
about the hip and upper thigh for
several months. A radiograph reveals
an upper femoral mass lesion. Thre
proximal femur is excised and on
sectioning has the gross appearance
shown here. The cells that proliferate in
this lesion sre most likely to be..
A. Osteoblast B. Chondroblast
C. Osteoclast D. Primitive cells

5. Which of the following is a cancer of the skeletal muscle?

A. Osteosarcoma B. Chondrosarcoma
C. Leiomyosarcoma D. Rhabdomyosarcoma
6. Correlation of both ___________ and __________ data
have lead to criteria for diagnosing tumors as either
benign & malignant
A.Clinical, statistical
B.Histologic, clinical
C.Histologic, statistical
D.Demographical, hitological

7. Who determines the definitive diagnosis of tumors?

• A.Oncologist B.Pharmacist
• C.Physician D.Pathologist
8. Benign tumor of cartilage is called an:
A.Osteoma B.Chondroma
C.Leiomyoma D.Lipoma

9. Benign tumor of osseous tissue is called an:

A.Osteoma B.Chondroma
C.Leiomyoma D.Lipoma

10. What does TNM stand for?

A. Tumor size, lymph node, malignancy
B. Tumor size, leimyoma, malignancy
C. Tumor shape, lymph node, metastasis
D. Tumor size, lymph node, metastasis
Musculoskeletal
Neoplasms
Hasna Dewi, dr., Sp.PA., M.Kes.
Bagian Patologi Anatomi FKIK UNJA
 WHO classification of bone
tumours
1. Cartilage tumours
2. Osteogenic tumours
3. Fibrogenic tumours
4. Fibrohistiocytic tumours
5. Ewing sarcoma/Primitive Neuroectodermal tumour
6. Haematopoietic tumours
7. Giant cell tumour
8. Notochordal tumours
9. Vascular tumours
10. Smooth muscle tumours
11. Lipogenic tumours
12. Neural tumours
13. Miscellaneous tumours
14. Miscellaneous lesions
15. Joint lesions
 Bone tumors
• The diagnosis of bone lesion should
be established by a combination of :
clinical,
radiological, and
pathological studies
• Most bone tumors can be diagnosed by
routine H.E stains, but in some cases, I.H.C
studies are needed
• Bone tumors are classified into:
 Primary bone tumors
 Secondary bone tumors ( Metastasis)
Analytic approach to evaluation of the
bone neoplasm
 Evaluation; imaging
• Plain radiograph
• CT scan
• MRI
• Radionuclide scanning
• PET
Radiographic features of bone tumors
 Site of the lesion.
Distribution of various lesions Distribution of various lesions in a
in a long tubular bone in a long tubular bone after skeletal
growing skeleton maturity
 Site of the lesion.
Location of epicenter of lesion usually determines site of its origin
(medullary, cortical, periosteal, soft tissue, or in the joint)
 Borders of the lesion
determine its growth rate.
sharp sclerotic sharp lytic ill-defined.
 Borders of the lesion.
A: Sclerotic border B: A wide zone of transition
typifies a benign lesion e.g typifies an aggressive or
nonossifying fibroma in the malignant lesion e.g
distal femur. plasmacytoma involving the pubic
bone and supraacetabular portion
of the right ilium
 Patterns of bone destruction.
geographic permeative type
moth-eaten
a uniformly affected characteristic of
rapidly growing round cell tumors
area within sharply infiltrating lesions
defined borders

giant cell tumor. myeloma Ewing sarcoma

 Types of periosteal reaction.

An uninterrupted periosteal reaction usually indicates a benign process, whereas an

interrupted reaction indicates a malignant or aggressive nonmalignant process
 Interrupted type of periosteal reaction

lamellated or Ewing sarcoma -

sunburst onion-skin lamellated type
pattern - type in ewing
sarcoma
osteosarcoma

Codman
triangle
(arrow)
Radiographic features that may help differentiate
benign from malignant lesions
 Primary Bone Tumors
Bone-Forming tumors
– Osteoid osteoma and
osteoblastoma
– Osteosarcoma Miscellaneous tumors
− Ewing’s sarcoma
Cartilage-Forming tumors – Giant cell tumor of
bone
– Chondroma
(Enchondroma)
– Chondrosarcoma
 Osteogenic Tumors
• Osteoid osteoma
Benign
• Osteoblastoma
• Osteosarcoma  Malignant

• Bone sarcoma ± 0,2% (overall neoplasms)

• The majority of primary bone malignancies arise de novo
• Etiology : radiation, chronic inflammatory, exposures of
chromium, nickel, cobalt, alumunium, titanium,
methylmethacrylate and polyethyelene. Implanted
metallic hardware and joint prostheses
• Clinical features:
The clinical features of bone tumours are non-
specific. Pain, swelling and general discomfort are
the cardinal symptoms. However, limited mobility
and spontaneous fracture may also be important
features.
Osteoid osteoma
- Benign bone tumor seen often in teens and young adults
- < 2 cm diameter and have predilection for cortex of long bones
- Painful lesions and can mimic osteomyelitis
- Well circumscribed mass of woven bone rimmed by osteoblasts
- This is called the nidus and is surrounded by reactive bone
 OSTEOBLASTOMA
• Clinic • Imaging
– Rare –
a lytic well
– 1% of all bone tumors
circumscribed oval or
– >> male teenagers and
round defect almost
young adults always confined by a
• Location periosteal shell of
– Vertebra – sacrum reactive bone.
– Prox. And distal femur Calcification +
and prox. Tibia → – Size : >> 3–10 cm
metafisis

KPTJL, 27-28/05/06, IAPKOL

 OSTEOBLASTOMA
• Macroscopy
– Rich vascular supply  red or red
brown and often with a gritty or
sandpaper consistency due to the
tumour bone.
– The tumour is usually round to
oval with a thinned cortex and
always with a thin periosteal
reactive bone shell if the cortex is
destroyed

KPTJL, 27-28/05/06, IAPKOL

 OSTEOBLASTOMA
• Histophatology
– has identical histological
features to osteoid
osteoma
– composed of woven bone
spicules or trabeculae.
These spicules are
haphazardly or
chaotically arranged and
are lined by a single layer
of osteoblasts. The
vascularity is rich, often
– with extravagated red
blood cells
KPTJL, 27-28/05/06, IAPKOL
 CONVENTIONAL
OSTEOSARCOMA
• a primary intramedullary high grade malignant tumour in
which the neoplastic cells produce osteoid
• Clinic • Location
– the most common,
– the long bones  Metafisis
nonhaemopoietic, primary
(90%) diafisis (9%)  distal
malignant tumour of bone
femur , prox. tibia &
– frequently occurs in the humerus
second decade with some
60% of patients < 25 years • Lab
– patients > 40 yo  Paget – Alkali fosfatase ↑ (N:0-270)
disease of bone, post- – Lactic acid dehydrogenase
radiation sarcoma ↑ (N:100-190)
– >> ♂
– a painful, tender mass
KPTJL, 27-28/05/06, IAPKOL
• Radiographic : blastic, lytic, or lytic-blastic lesion. Periosteal
reaction :
a. Codman’s triangle
b. sunburst
• Microscopic :
malignant pleomorphic cells
malignant osteoid
may produce cartilage,
fibrous or mixoid tissue
Osteosarcoma

Based on Subtypes
Intramedullary / intracortical /
Location
periosteal etc

Differentiation Well / poorly

Osteoblastic / chondroblastic /
Histology fibroblastic, telangiectatic / giant
cell / small cell

Solitary / multicentric
Others
Primary / secondary
 EWING SARCOMA / PRIMITIVE
NEUROECTODERMALTUMOUR (PNET)
Ewing Sarcoma
may be confused with osteomyelitis

Commonly affects diaphysis with onion

skin appearance
 EWING SARCOMA/PNET

KPTJL, 27-28/05/06, IAPKOL

GIANT CELL TUMOR
Gross:

- Large, red-brown
- Hemorrhage and cystic degeneration
- Uniform mononuclear cells
in sheets with increased
mitoses
- Numerous osteoclast type
of giant cells with > 100
nuclei
Cartilage-forming tumors
 CHONDROBLASTOMA
• a benign, cartilage producing neoplasm usually
arising in the epiphyses of skeletally immature
patients
• << 1% of all bone tumours. Patients >> are
between 10 and 25 years of age at diagnosis
and ♂ >>
• > 75% involve the long bones  >> epiphyseal
and epimetaphyseal regions of the distal and
proximal femur, proximal tibia, and proximal
humerus

KPTJL, 27-28/05/06, IAPKOL

 CHONDROBLASTOMA
• patients >> complain of
localized pain, often mild,
but sometimes of many
years duration
• Ro : typically lytic, centrally
or eccentrically placed,
small lesions (3 - 6 cm),
occupying < ½ of the
epiphysis and are sharply
demarcated,with or without
a thin sclerotic border

KPTJL, 27-28/05/06, IAPKOL

 CHONDROBLASTOMA
• Histologically, the characteristic cell is
uniform, round to polygonal cell with
well defined cytoplasmic borders, clear
to slightly eosinophilic cytoplasm and a
round to ovoid nucleus (chondroblasts).
• The nucleus often displays clefts or
longitudinal grooves and contains one
or more small to inconspicuous nucleoli.
• Chondroblasts are packed in pseudo-
lobulated sheets often showing a
pavement-like pattern.
• Pericellular calcification  chicken wire
calcification”
• numerous randomly-distributed
osteoclast type giant cells

KPTJL, 27-28/05/06, IAPKOL

 Chondrosarcoma

Local swelling and pain,

alone or in combination.
The symptoms are usually
of long duration (several
months or years).
 Chondrosarcoma
Microscopic

These tumors are composed of lobules of cartilage with anaplastic

chondrocytes in the lacunae and with focal enchondral ossification and
calcification.
 TUMOR OTOT SKELET
• Benign:
– Rhabdomyoma
• Adult
• Fetal
• Genital
• Malignant
– Rhabdomyosarcoma
• Embryonal
• Alveolar
• Pleomorphic

KPTJL, 27-28/05/06,
 Rhabdomyoma
• a benign mesenchymal tumour with skeletal muscle
differentiation that is classified into cardiac and
extracardiac types based on location
• Rare
• The head and neck region (90%)
• The median age is 60 years (33 - 80 years) with a 3:1
male predominance.
• Symptoms include upper airway obstruction and
mucosal or soft tissue mass (median duration 2 years,
range 2 weeks to 3 years); in 10% the mass is
asymptomatic . A-RM is often solitary (70%),

KPTJL, 27-28/05/06,
KPTJL, 27-28/05/06,
 RHABDOMYOSARCOMA
 Highly malignant neoplasm arising from
embryonal mesenchyme with capacity for
skeletal muscle differentiation
 Clinical Presentation

• Occurs in multiple primary sites

• Usually presents as a asymptomatic mass
• symptoms relate to mass effect on asso. Organ
• Orbital : Proptosis, ophthalmoplegia
• Parameningial : Nasal, Aural or sinus obstruction, CN
Palsies & headache etc.
• Genitourinary : Hematuria, Urinary obstruction
 • Most common soft tissue sarcoma of children
under 15 years of age
• Tumors in extremities present as painful, rapidly-
growing masses
• Age:
• Predominantly infants and children
• Sex:
• M>F

KPTJL, 27-28/05/06,
 Histology
 Embryonal  Spindle cell
 Most common  Subtype of embryonal
 60-70% of all childhood  Most common site is
RMS paratesticular
 H&N, GU sites  Superior Prognosis
 Intermediate prognosis  Alveolar
 20% of RMS
 Boytroid
 More common in adolescents
 Subtype of embryonal
 Tumors involving extremities,
 10% of all childhood RMS trunk, perianal and perineal
 Bladder, vagina,  Undifferentiated
nasopharynx, nares,  Diagnosis of exclusion
middle ear, biliary tree
 Previously called pleiomorphic
 Superior prognosis  Rare in children, more common
in adults
KPTJL, 27-28/05/06,
GROSS: URINARY: BLADDER: RHABDOMYOSARCOMA, BOTRYOID TYPE
KPTJL, 27-28/05/06,
 Embryonal
Rhabdomyosarcoma

Alveolar
Rhabdomyosarcoma

KPTJL, 27-28/05/06,
 Pleomorphic rhabdomyosarcoma

KPTJL, 27-28/05/06,
 Selamat Belajar
Dan
Terima Kasih

KPTJL, 27-28/05/06, IAPKOL