Blok 3.

2
Tahun 2018

BONE AND SOFT TISSUE

TUMORS
dr. Aswiyanti Asri,M.Si.Med,SpPA
 Bone tumors
• 2400 bone sarcoma per tahun di U.S
• 50% mortalitas
• Sebagian besar : dekade pertama
• Usia pasien : sangat penting dalam diagnosis
• GK : fraktur patologis, nyeri
 CLASSIFICATIONS OF PRIMARY TUMOURS INVOLVING BONES
Metastatic cancers are the most frequent malignant tumors found in bone

Histological Types Benign Malignant

Hematopoietic (40%) Myeloma
Malignant lymphoma
Chondrogenic (22%) Osteochondroma Chondrosarcoma
Chondroma Dedifferentiated chondrosarcoma
Chondroblastoma
Chondromyxoid fibroma
Osteogenic (19%) Osteoid osteoma Osteosarcoma
Osteoblastoma
Unknown origin (10%) Giant cell tumour Ewing tumour
Giant cell tumour
Adamantinoma
Histiocytic origin Fibrous histiocytoma MFH
Fibrogenic Fibroma Fibrosarcoma
Notochordal Chordoma
Vascular, Cystic, lipogenic
neurogenic
0 - 10 simple bone cyst Ewing's sarcoma
eosinophilic granuloma leukemic involvement
metastatic neuroblastoma

10 - 20 non-ossifying fibroma osteosarcoma,

fibrous dysplasia Ewing's sarcoma,
simple bone cyst adamantinoma
aneurysmal bone cyst
osteochondroma (exostosis)
osteoid osteoma
osteoblastoma
chondroblastoma
chondromyxoid fibroma

20 - 40 enchondroma chondrosarcoma
giant cell tumor

> 40 osteoma metastatic tumors

myeloma
leukemic involvement
chondrosarcoma
 Osteosarcoma
• Sel kanker membentuk matriks osteoid atau
mineralized bone
• 20% primary bone cancer
• Bimodal age distribution (75% usia < 20 tahun)
• Metafise, >> sekitar lutut
• Codman triangle
 Osteosarcoma : genetik
• RB : 70% osteosarcoma
• TP53 : herediter (Li-Fraumeni syndrome) dan
sporadik
• INK4a (inaktivasi)
• MDM2 dan CDK4
Osteosarcoma
 Chondrosarcoma
• Cartilage forming tumors
• Dewasa
• Conventional, dedifferentiated, clear cell,
mesenchymal
• Pelvis, shoulder, ribs
• Genetically heterogen
• Gen yang diketahui : gen EXT, IDH1, IDH2, CDKN2A
Chondrosarcoma
 Ewing Sarcoma
• PNET (primitive neuroectodermal tumors)
• 80% < 20 tahun
• Diafise, femur, pelvis
• Onion skin fashion
• Translokasi 11;22 (q24;q12) ok fusi bingkai gen EWS
di kromosom 22 dengan gen FLI1
Ewing Sarcoma
 Giant Cell Tumor
• = Osteoclastoma
• Epifise tapi dapat meluas ke metafise
• Sekitar lutut
• 40 – 60% rekurens lokal
Giant Cell Tumor
 Soft tissue tumors
• Tumor yang berasal dari jaringan nonepithelial,
kecuali glial dan sistem RES
• otot, vaskuler, jaringan lemak, jaringan fibrous, sistem
syaraf perifer
• seluruh tubuh
• > 200 jenis tumor, 90 jenis ganas
• 15% pada usia < 15 tahun dan 40% usia > 55 tahun
Lokasi
Soft tissue tumors : Etiologi
• >> sporadik, tidak jelas faktor risiko
• 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
 Klasifikasi
• 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
 Soft tissue tumors
• Bervariasi
• Benign – self limited lesion
• Intermediate grade – locally aggressive
• Highly agressive
 Lipoma

Karakteristik Mikroskopik
 The most common soft tissue

tumors of adulthood

 Subcutis ekstremitas, tubuh

 Soft, mobile, painless

 Mirip sel lemak normal

 Liposarcoma
• 2nd most common sarcoma of adult
• Kompartemen dalam ekstremitas atau
retroperitoneum
• Lipoblast
• Subtipe : well differentiated, pleomorphic, myxoid
Liposarcoma
 GIST (Gastrointestinal Stromal Tumors)
• Separate subtype of sarcoma defined by expression
of c-Kit (CD117)
• Surgery: complete resection without local or regional
lymphadenectomy
• Very resistant to traditional chemotherapy
• Gleevec (imatinib mesylate)
GIST
 Hemangioma
• Benign lesion
• Resemble normal blood vessels
• Congenital or non congenital
• >> infants and children
• Head and neck, internal organ – liver
• Capillare, cavernosum
 Fibrosarcoma
• Fibrosarcomas are rare but may occur anywhere in the body, most
commonly in the retroperitoneum, the thigh, the knee, and the
distal extremities.
• Typically, these neoplasms are unencapsulated, infiltrative, soft,
fish-flesh masses often having areas of hemorrhage and necrosis.
Better-differentiated lesions may appear deceptively encapsulated.
Histologic examination discloses all degrees of differentiation, from
slowly growing tumors that closely resemble cellular fibromas
sometimes having spindled cells growing in a herringbone fashion
to highly cellular neoplasms dominated by architectural disarray,
pleomorphism, frequent mitoses, and areas of necrosis.
 Rhabdomyosarcoma
• Rhabdomyosarcomas, the most common soft tissue
sarcomas of childhood and adolescence, usually appear
before age 20. They may arise in any anatomic location,
but most occur in the head and neck or genitourinary
tract.
• embryonal, alveolar, and pleomorphic variants.
• rhabdomyoblast--the diagnostic cell in all types--contains
eccentric eosinophilic granular cytoplasm rich in thick
and thin filaments, tadpole cells
Rhabdomyosarcoma
 Leiomyoma vs Leiomyosarcoma

Leiomyoma Leiomyosarcoma
• Uterus, skin, subcutis • Uterus, GIT,
• Fasikulus sel spindel yang retroperitoneum
membentuk anyaman saling • Sel spindel – spt cerutu,
silang plemorfik