IV-2a

Bones and Soft Tissue (from Robbins, old trans)

BONES
Composition of Bone A. Cells 1. Osteoblasts forms and mineralizes bone and produces ALP 2. Osteocytes inactive osteoblasts 3. Osteoclasts multinucleated - monocytes that resorb bone 4. Chondrocytes forms and maintains cartilage B. Organic and Inorganic Matrix Inorganic a. calcium hydroxyapatite - gives bone strength and hardness is the storehouse for 99% of the body's calcium, 85% of the body's phosphorus, and 65% of the body's sodium and magnesium. - formation of hydroxyapatite crystal is analogous to the conversion of water to ice Organic a. Cellular Components (mentioned earlier) b. Collagen fibers type 1 - 95% of organic matrix and derived mainly from osteoblasts b.1. Osteoid - Bones that are not mineralized Hydroxyproline - Two types: Woven (random weave) Lamellar (orderly layered manner) b.2. Proteoglycans Cell adhesion/ cytokines/ calcium/ GF/ enzymes C. Minerals (inorganic) - provides hardness and dependent on PTH 1. Calcium - 90% 2. Phosphorus - 80% D. Blood Vessels Bone Modeling and Remodeling formation and resorption process constant process adjustment of the skeletal system to stress Important for calcium and phosphate balance Bone Pathology (Non-NEOPLASTIC) DEVELOPMENTAL/GENETIC AND ACQUIRED ABNORMALITIES IN BONE CELLS MATRIX AND STRUCTURES

Gene Human Disorder Mutati on

Affected Molecule

Phenotype

Defects in Transcription Factors Producing Abnormalities in Mesenchymal Condensation and Related Cell Differentiation Synpolydactyly Waardenburg syndrome Greig syndrome Campomelic dysplasia Oligodontia HOXD13 PAX-3 GL13 SOX9 PAX9 Transcripti Extra digit with fusion on factor Hearing loss, abnormal Transcripti pigmentation, craniofacial on factor abnormalities Transcripti Synpolydactyly, on factor craniofacial abnormalities Transcripti Sex reversal, abnormal on factor skeletal development Transcripti Congenital absence of on factor teeth Hypoplastic nails, hypoplastic or aplastic Transcripti patellae, dislocated radial on factor head, progressive nephropathy Transcripti Congenital abnormalities, on factor forelimb anomalies Hypoplasia or absent ulna, Transcripti 3rd5th digits, breast, and on factor teeth, delayed puberty Abnormal clavicles, Transcripti wormian bones, on factor supernumerary teeth

Nail-patella syndrome Holt-Oram syndrome Ulnar-mammary syndrome Cleidocranial dysplasia

LMX1B

TBX5 TBX3

CBFA1

Defects in Extracellular Structural Proteins Osteogenesis imperfecta types 14 COL1A1 COL1A2 Achondrogenesis II Type 2 COL2A1 collagen Type 1 collagen Bone fragility, hearing loss, blue sclerae Dentinogenesis imperfecta Short trunk, severely shortened extremities, relatively enlarged cranium, flattened face Short trunk, shortened extremities, relatively enlarged cranium, flattened face Type 2 collagen Type 9 collagen Myopia, retinal detachment, hearing loss, flattened face, premature osteoarthritis Short or normal stature, small epiphyses, early onset osteoarthritis

Hypochondrogene COL2A1 sis

Stickler syndrome COL2A1 Multiple epiphyseal dysplasia

COL9A2

Gene Human Disorder Mutati on

Affected Molecule

Phenotype Mild short stature, bowing of lower extremities, coxa vara, metaphyseal flaring

Schmid COL10A Type 10 metaphyseal 1 collagen chondrodysplasia

Defects in Hormones and Signal Transduction Mechanisms Producing Abnormal Proliferation or Maturation of Chondrocytes and Osteoblasts Brachydactyly type C Jansen metaphyseal chondroplasia CDMP1 Signaling molecule Shortened metacarpals and phalanges Short bowed limbs, clinodactyly, facial abnormalities, hypercalcemia, hypophosphatemia Short stature, rhizomelic shortening of limbs, frontal bossing, midface deficiency Disproportionate short stature, micromelia, relative macrocephaly Severe limb shortening and bowing, frontal bossing, depressed nasal bridge Craniosynostosis C.

Micromelic shortening of the limbs Frontal bossing with relative macrocephaly Small chest cavity Bell shaped abdomen Die due to respiratory insufficiency

Diseases associated with defects in extracellular structure proteins Type I collagen diseases (Osteogenesis Imperfecta) group of phenotypically related disorders caused by deficiency in the synthesis of collagen type I Brittle bones / too little bone Marked cortical thinning and attenuation of trabeculae 4 subtypes according to severity of mutation Types 2, 10, 11 collagen diseases - affects hyaline cartilage

PTHrp Receptor receptor

1.

Achondroplasia

FGFR3

Receptor

Hypochondroplasi FGFR3 a Thanatophoric dwarfism Crouzon syndrome

2.

Receptor

*pahabol: subtypes of osteogenic imperfecta

Subtype Inheritance Collagen Defect Decreased synthesis pro1(1) chain Abnormal pro1(1) or pro2(1) chains Major Clinical Features Compatible with survival

FGFR3

Receptor

FGFR2

Receptor

Postnatal OI Autosomal fractures, blue I dominant sclerae

Normal stature Skeletal fragility Dentinogenesis imperfecta Hearing impairment Joint laxity Blue sclerae

A. 1.

Caused by defects in nuclear proteins and transcription factors Dysostoses uncommon developmental anomaly due to localized disorder of migration/condensation of the mesenchymal cells Genetic alteration that affects transcription factors Homeobox genes (HOXD-13
Most OI Perinatal lethal autosomal II recessive Some autosomal dominant ?New mutations OI Progressive III deforming Autosomal dominant (75%) Autosomal recessive (25%)

B. 1.

Caused by defects in hormones and signal transduction mechanism Achondroplasia Defect in the paracrine cell signaling resulting in the reduction in the proliferation of chondrocytes in the growth plates Most common disease of the growth plate Most common cause of dwarfism without cartilage formation Autosomal dominant Shortened proximal extremities Trunk has normal length Enlarged head with bulging forehead and conspicuous depression of the root of the nose Not associated with longevity, intelligence and reproductive status

Abnormally short Death in utero or within pro-1(1) chain days of birth Unstable triple helix Abnormal or insufficient pro2(1) Skeletal deformity with excessive fragility and multiple fractures Blue sclerae

Altered structure of pro-peptides Compatible with survival of pro-2(1) Impaired formation of triple helix Growth retardation Multiple fractures Progressive kyphoscoliosis Blue sclerae at birth that become white Hearing impairment

2.

Thanatophoric Dwarfism Diminished proliferation of chondrocytes and poor columnization in the zone of proliferation Most common lethal form dwarfism Mutation in FGFR3 (missense/point mutation)

Dentinogenesis imperfecta Postnatal OI Autosomal fractures, IV dominant normal sclerae Short pro-2(1) chain Unstable triple Compatible with survival Moderate skeletal

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Subtype

Inheritance

Collagen Defect helix

Major Clinical Features fragility Short stature Sometimes dentinogenesis imperfecta

G.

Disease caused by osteoclasts dysfunction Pagets Disease/ Osteitis Deformans Initial osteoclastic activity due to defective remodeling followed by disorganized hyperplastic bone formation

1.

OI, osteogenesis imperfecta.

3 phases 1. Osteolytic stage 2. Osteoclastic-osteoblastic stage 3. Osteosclerotic stage Etiology uncertain (viral infection) M>F / most patient > 55 years Most commonly involves lumbosacral spine, pelvis and skull; very rare in ribs/ usually polyatomic Pain

D. 1.

Diseases associated with defects in folding and degradation of macromoleculess Mucopolysaccharidoses group of lysosomal storage diseases Deficiencies in enzymes that degrade heparan sulfate/ dermatan sulfate/ keratan sulfate Associated with acid hydrolases Abnormalities in hyaline cartilage: cartilage anlage, growth plates, costal cartilages and articular surfaces Short stature Chest wall abnormalities Malformed bones

Complications: 1. Fracture 2. Degenerative arthritis 3. Bone tumors (osteosarcoma, fibro sarcoma, chondrosarcoma, and GCT) 4. High-output cardiac failure 5. Mosaic pattern (likened to a jigsaw puzzle) histologic hallmark H. Disease associated with abnormal mineral homeostasis Rickets and Osteomalacia Accumulation of unmineralized bone matrix resulting from a diminished rate of mineralization Causes: Dietary deficiency in vitamin D Defective bone mineralization Congenital or acquired defects in vitamin D or phosphate metabolism Malabsorption (most common cause in US)
Crohns disease Celiac disease Homeostatic liver disease Biliary obstruction Chronic pancreatitis

1.
E. Diseases associated with defects in metabolic pathways (enzymes/ion channels and transporters) Osteopetrosis a rare genetic disease in which there is reduced osteoclasts bone resorption resulting in diffuse symmetric skeletal sclerosis Stone like quality of the bones which are abnormally brittle and fractures like a chalk - A.k.a. Marble bone disease/ Albers Schonberg disease Deficient osteoclast activity

1.

Bone lack medullary canal (Erlenmeyer flask deformity) Ends of long bones are bulbous and misshapen No room for bone marrow Fracture anemia and hydrocephaly

Soft spot on babys head is slow to close Bony necklace Curved bones Big, lumpy joints Bowed legs (knees bent out)

F .

Diseases associated with decreased bone mass Osteoporosis Increased porosity of the skeleton resulting in reduced bone mass that may predispose the bone to fracture Localized (disused osteoporosis) vs. generalized (metabolic bone disease) Most common - senile/ post menopausal osteoporosis Pathogenesis: 1. Age related - Senile/low turn over variant 2. Reduce physical activity 3. Genetic factors 4. Vitamin D receptor molecule 4. Calcium nutrition status 5. Hormonal influences (Estrogen vs.

2.

Hyperparathyroidism Increased bone resorption 2 to increased PTH Osteitis Fibrosa Cystica - classic pathologic change a. Replacement of marrow by fibrous tissue b. Numerous microfractures c. Hemosiderin-laden macrophages d. Eventually cystic degeneration and classic gross appearance referred to as brown tumor

1.

Disease Of Bone)

Generalized Osteitis Fibrosa Cystica (Von Recklinghausen a. b. c. - hallmark of severe hyperparathyroidism Increased bone cell activity peritrabecular fibrosis cystic brown tumors

glucocorticoids)

3.

Renal Osteodystrophy - used to describe collectively all of the skeletal changes of chronic renal disease,

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inclufing the ff. 1. 2. 3. 4. Increased orthoclastic bone resortion Delayed matrix mineralization Growth retardation Osteoporosis

2.

Klebsiella Pseudomonas (tennis shoe)

genitourinaty tract infxn seen in px with

3.
4.

E.coli Heamophilus influenza in neonatal pxs Group B Strep. Neisseria Salmonella in pxs with sickle cell dse Mycobacterium tuberculosis

5.
6. 7. 8. 9.

Divided into two major types a. High-turnover osteodystrophy characterized by increased bone resorption and formation, with the former predominating b. low-turnover or aplastic disease manifested by adynamic bone (little osteoclastic and osteoblastic activity) and osteomalacia.

B.

Tuberculous osteomyelitis patients present with pain on motion, localized tenderness, low-grade fevers, chills, and weight loss - Pott disease if occurs in the spine (most common site) Skeletal syphilis - syphilis (Treponema pallidum) and yaws (Treponema pertenue) characterized by edematous granulation tissue - Saber Shin produced by massive reactive periosteal bone deposition on the medial and anterior surfaces of the tibia. Can be:
I - medullary II - superficial III - localized IV - diffuse

C.

FRACTURES Most common pathologic condition of bones 1. Traumatic 2. Non traumatic Classification 1. Complete vs. incomplete Complete - bali ng buo Incomplete- may nakadikit pa 2. Simple (close) vs. compound Simple - hindi lumabas sa tissue Compound - kita na 3. Comminuted vs. displaced Comminuted - bali ng maliliit (splintered) Displaced - malinis ang pagkabali (straight) 4. Pathologic and stress Pathologic - disease process Stress - increased physical activity/more load OSTEONECROSIS / AVASCULAR NECROSIS Infarction of bone and marrow. This is a relatively common event that occurs in the medullary cavity of the metaphysis, diaphysis and the subchondral regions of the epiphysis. May results from ischemia Mechanisms: 1. Mechanical vascular interruption (fracture) 2. Corticosteroids 3. Thrombosis and embolism 4. Vessel injury 5. Increased intraosseous pressure with vascular compression 6. Venous hypertension OSTEOMYELITIS Inflammation of the bone and commonly implies infections. May be a complication of any systemic infection but frequently manifests as a primary solitary focus of disease 50% of cases no pathologic organisms are isolated Local, exogenous or hematogenous infection - Sequestrum dead piece of bone - Involucrum - deposited reactive woven or lamellar bone that forms a sleeve of living tissue around a segment of devitalized bone Chronic osteomyelitis often requires surgery

HOST FACTORS THAT AFFECT WOUND HEALING Local factors

o o o o o Chronic edema Vascular diseases Extensive scarring Previous radiation Tissue loss

Systemic factors
o o o o o o o

Malnutrition Immune deficiencies Organ failures Diabetes, old age Cancers, obesity Use of tobacco products Steroids, other medications

Bone Pathology (NEOPLASTIC) Can be: 1. Bone Forming Tumor 2. Cartilage FormingTumor 3. Fibrous and Fibro-osseous Tumor 4. Miscellaneous Tumors Classification of Primary Tumors Involving Bones Histologic Type Hematopoietic (40%) Benign Malignant Myeloma Malignant lymphoma Chondrogenic (22%) Osteochondroma Chondroma Chondroblastoma Chondromyxoid fibroma Chondrosarcoma Dedifferentiated chondrosarcoma Mesenchymal chondrosarcoma

A.

Pyogenic Osteomyelitis or Bacterial infection of bone

1.

Staphylococcus aureus - 80-90% of cases

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Histologic Type Osteogenic (19%)

Benign Osteoid osteoma Osteoblastoma

Malignant Osteosarcoma

characterized by Codman Triangle - triangular shadow between the cortex and raised ends of periosteum

Several subtypes of osteosarcoma are recognized and are grouped according to: The anatomic portion of the bone from which they arise (intramedullary, intracortical, or surface) Degree of differentiation Multicentricity (synchronous, metachronous) Primary (underlying bone is unremarkable) or secondary (e.g., osteosarcoma associated with pre-existing disorders such as benign tumors, Paget disease, bone infarcts, previous irradiation) Histologic variants (osteoblastic, chondroblastic, fibroblastic, telangiectatic, small cell, and giant cell) CARTILAGE FORMING TUMORS Characterized by the formation of hyaline or myxoid cartilage; fibro cartilage and elastic cartilage Osteochondroma Chondroma Chondroblastoma Chondromyxoid fibroma Chondrosarcoma

Unknown origin Giant cell tumor (10%)

Ewing tumor Giant cell tumor Adamantinoma

Histiocytic origin Fibrogenic

Fibrous histiocytoma Metaphyseal fibrous defect (fibroma)

Malignant fibrous histiocytoma Desmoplastic fibroma Fibrosarcoma

Notochordal Vascular Hemangioma

Chordoma Hemangioendothelioma Hemangiopericytoma

Lipogenic Neurogenic

Lipoma Neurilemmoma

Liposarcoma

BONE FORMING TUMORS common features is the production of bone by the neoplastic cells Woven trabeculae (except osteoma) and variably mineralized A.

1. 2. 3. 4. 5.

1. Osteoma 2. Osteoid osteoma / osteoblastoma 3. Osteosarcoma

A.

Osteoma Bosselated, round to oval sessile tumors that project from the subperiosteal or endosteal surfaces of the cortex Skull and facial bone - Gardnes syndrome if multiple osteoma - Composed of woven and lamellar bone Reactive bone induced by infection, trauma or hemangioma - Slow growing tumor with little clinical significance and interfere with function Osteoid Osteoma / Osteoblastoma Benign tumors with identical histologic patterns but differ in size, site of origin and symptoms 1. Osteoid Osteoma <2cm 10-20 y/o Appendicular bone / cortex Painful lesion (PGE) nocturnal - relieved by Osteoblastoma Spine Dull pain, achy - not responsive to salicylates No marked bony reaction

Osteochondroma - A.k.a. exostosis, is a benign cartilage-capped outgrowth that is attached to the underlying skeleton by a bony stalk - Inactivation of both copies of the EXT gene develop only in bones of endochondral origin and arise from the metaphysis near the growth plate of long tubular bones, especially about the knee. mushroom shaped and range in size from 1 to 20 cm Chondroma - Benign lesion of hyaline cartilage Intraosseous cartilage - 20-50 y/o Most are asymptomatic - O ring Sign radiographic feature (unmineralized nodules of cartilage produce wellcircumscribed oval lucencies that are surrounded by a thin rim of radiodense bone)

B.

B.

1.
cavity

Enchondroma - if arises within the medullary

2.

aspirin

2.

Subperiosteal or juxtacortical chondroma if arises on surface of bone 3. Olliers disease - syndrome of multiple enchondromas, or enchondromatosis 4. Maffuci syndrome - enchondromatosis is associated with soft tissue hemangiomas

C.

Osteosarcoma Malignant mesenchymal neoplasm in which the cell produce bone matrix - 20% of primary bone tumors with Bimodal age distribution (<20/75% - elderly) Metaphyseal region of long bones (knee) Mutation in RB gene painful and progressively enlarging masses

C.

Chondroblastoma - Rare benign tumor - 1% of primary tumors Young / male / knee Epiphyses / apophyses Painful Polyhedral chondroblasts Chondromyxoid Fibroma rarest Commonly mistaken as sarcoma

D.

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Male/teens frequently arise in the metaphysis of long tubular bones x-rays demonstrate an eccentric geographic lucency

D.

E.

Chondrosarcoma Group of tumors with a broad spectrum of clinical and pathologic findings Production of neoplastic cartilage Second most common primary bone tumor 40yo / women commonly arise in the central portions of the skeleton, including the pelvis, shoulder, and ribs SUBCLASSIFICATION: a. According to site: 1. Intramedullary 2. Juxtacortical b. According to histologic feature: 1. Conventional (hyaline and/or myxoid) variants o large bulky tumors are made up of nodules of gray-white, somewhat translucent glistening tissue 2. Clear cell variants o unique in that it originates in the epiphyses of long tubular bones o Hallmark: sheets of large malignant chondrocytes that have abundant clear cytoplasm, numerous osteoclast-type giant cells, and intralesional reactive bone formation 3. Dedifferentiated variants o Combination of malignant fibrous histiocytoma, fibrosarcoma, or osteosarcoma 4. Mesenchymal variants o composed of islands of well-differentiated hyaline cartilage surrounded by sheets of small round cells, which can mimic Ewing sarcoma. A.

Fibrosarcoma / Malignant Fibrous Histiocytoma Fibroblastic collagen producing sarcoma of the bone Overlapping clinical, radiological and pathologic features Any age/ equal sex distribution Enlarging painful masses Metaphysis of long bones and flat bones of the pelvis Pathologic fracture is a frequent complication MISCELLANEOUS TUMORS Ewing Sarcoma and PNET highly malignant tumor (small round cell) A type of peripheral primitive neuroectodermal tumor (PNET) Translocation of t(11;22)(q24;q12) Lower extremity more than the upper extremity, but any long tubular bone may be affected Most common sites are the metaphysis and diaphysis of the femur followed by the tibia and humerus First and second decade but may affect persons from age 2 to 8 - second most common malignant tumor of the bone in children Whites more than blacks and Asians Male to female (3:2) Giant Cell Tumor - benign lesion that is usually solitary and locally aggressive potentially malignant Numerous multinucleated giant cells most common bone tumor in the young adults age 25 to 40 Women than men Long bones, most often the distal femur, proximal tibia, and distal radius Most common primary bone lesions in the distal phalanx Metastatic Disease Most common form of skeletal malignancy Pathway of spread: Direct extension Lymphatic Hematogenous Intraspinal seeding Originates usually: Prostate Breast Kidney Lung

B.

FIBROUS AND FIBRO-OSSEOUS TUMORS 1. 2. 3. A. Misshapen bony trabeculae interspersed with fibrous tissue Woven bone never is transformed to lamellar bone Fibrous Cortical Defect Non-ossifying Fibroma Fibrous Dysplasia C.

Fibrous Cortical Defect - extremely common (30-50% <2years old) - A developmental defect rather than neoplasm - Metaphysis of distal femur and proximal tibia Nonossifying Fibroma >5-6 cm - Not detectable until Adolescence Spontaneous resolution Fibrous Dysplasia all of the components of normal bone are present, but they do not differentiate into their mature structures lesions appear in three distinctive patterns: (1) involvement of a single bone (monostotic) (2) involvement of multiple, but never all, bones (polyostotic); and (3) polyostotic disease, associated with caf au lait skin pigmentations and endocrine abnormalities known as McCune-Albright syndrome 1. 2. 3.

JOINTS
Osteoarthritis Rheumatoid arthritis Spondyloarthopathies Gout Pseudogout Osteoarthritis - Most common form of joint disease characterized by the progressive erosion of articular cartilage A.k.a. Degenerative joint disease Slowly progressive - Characteristic in women, but not in men, are Heberden nodes in the fingers, representing prominent osteophytes at the distal interphalangeal joints.

B.

4.
5. A.

C.

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Pathology: a. increased water in the cartilage b. decreased concentration of proteoglycans c. weakening of the collagen network d. IL-1, TNF and nitric oxide are increased e. Cartilage attrition due to IL-1

SOFT TISSUES
Soft tissue tumors are defined as mesenchymal proliferations that occur in the extraskeletal, nonepithelial tissues of the body, excluding the viscera, coverings of the brain, and lymphoreticular system. - They are classified according to the tissue they recapitulate (muscle, fat, fibrous tissue, vessels, and nerves) Soft Tissue Tumors
Tumors of adipose tissue Lipomas Liposarcoma Tumors and tumor-like lesions of fibrous tissue Nodular fasciitis Fibromatoses Superficial fibromatoses Deep fibromatoses Fibrosarcoma Fibrohistiocytic tumors Fibrous histiocytoma Dermatofibrosarcoma protuberans Malignant fibrous histiocytoma Tumors of skeletal muscle Rhabdomyoma Rhabdomyosarcoma Tumors of smooth muscle Leiomyoma Smooth muscle tumors of uncertain malignant potential Leiomyosarcoma Vascular tumors Hemangioma Lymphangioma Hemangioendothelioma Hemangiopericytoma Angiosarcoma Peripheral nerve tumors Neurofibroma Schwannoma Granular cell tumor Malignant peripheral nerve sheath tumors Tumors of uncertain histogenesis Synovial sarcoma Alveolar soft part sarcoma Epithelioid sarcoma

B.

Rheumatoid arthritis - chronic systemic disease of unknown etiology Joints of hands and feet nearly always involved; may use involves elbows, knees, ankles, hips, spine and TMJ - F>M (3:1), 4th to 6th decade Strongly associated with HLA-DR4 and several nonMHC genes - pannus formation mass of synovium and synovial stroma consisting of inflammatory cells, granulation tissue, and fibroblasts, which grows over the articular cartilage and causes its erosion

Rheumatoid factor Positive in 70-80% of patients with classic RA Autoantibodies of IgM, IgG or IgA that react with Fc region of IgG Not specific for RA Circulating complexes bind complement Synovial hyperplasia driven by IL-1

C.
1.

Seronegative Spondyloarthropathies Ankylosing spondylitis - A.k.a. Rheumatoid sodalities/ Marie-Strumpell disease HLA-B27 - chronic synovitis with destruction of articular cartilage and bony ankylosis, especially in the sacroiliac, apophyseal joints and Vertebral column Reiter syndrome episode of noninfectious arthritis of the appendicular skeleton that occurs within one month of a primary infection Post-venereal Urethritis, conjunctivitis, and seronegative polyarthritis Psoriatic Arthritis distal interphalangeal joints of the hands and feet are first affected in an asymmetric distribution produces the sausage finger has sx of conjunctivitis and iritis Infectious Arthritis Suppurative Arthritis Tuberculous Arthritis Lyme Arthritis Viral Arthritis Gout Common end point of a group of disorders that produce hyperuricemia Endogenous crystals Monosodium urate (gout) Calcium pyrophosphate dihydrate Calcium phosphate (pseudo gout) - Contributing factors: Age, Genetic predisposition, Alcohol, Obese, drugs and Lead - Tophi are the pathognomonic hallmark of gout Acute arthritis - crystallization of urates Chronic arthritis tophi

2.

3.

D. 1. 2. 3. 4. E.

Morphology of Cells in Soft Tissue Tumors Cell Type Spindle cell Features Rod-shaped, long axis twice as great as short axis Tumor Type Fibrous, fibrohistiocytic, smooth muscle, Schwann cell

1. 2.

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Cell Type Small round cell

Features Size of a lymphocyte with little cytoplasm

Tumor Type Rhabdomyosarcoma, primitive neuroectodermal tumor

synovial sarcoma - young adults

pleomorphic high grade sarcoma/ liposarcoma/leiomyosarcoma - elderly Distinguishing features: 1. Lipoma - mature white fat cells with no pleomorphism 2. liposarcoma - lipoblasts 3. Fibrosarcoma - herring bone pattern 4. Malignant Fibrous Histiocytoma - storiform pattern 5. Rhabdomyosarcoma tadpole or strap cells 6. Leiomyoma 7. Leiomyosarcoma - malignant spindle cells that have cigar-shaped nuclei arranged in interweaving fascicles 8. Biphasic Synovial Sarcoma dual line of differentiation of the tumor cells Note: sa libro, mahaba pa ang topic pero sa ppt ni doc, distinguishing features na lang ang binanggit nya.. dinagdagan ku na ung mga walang distinguishing features.. wala tlaga sa leiomyoma.. basta most common neoplasm in women lang sya.. hindi ku na din sinama pa ung iba, aksaya oras.. hehe!!

Polyhedral with abundant Smooth muscle, Schwann Epithelioi cytoplasm, nucleus is cell endothelial, epithelioid d centrally located sarcoma

Architectural Patterns in Soft Tissue Tumors Pattern Tumor Type Fascicles of eosinophilic spindle cells intersecting at Smooth right angles muscle Short fascicles of spindle cells radiating from a central point (like spokes on a wheel)storiform Nuclei arranged in columnspalisading Herringbone Mixture of fascicles of spindle cells and groups of epithelioid cellsbiphasic Fibrohistiocyt ic Schwann cell Fibrosarcoma Synovial sarcoma

Epidemiology Rate of benign vs. sarcoma 100:1 Benign soft tissue annual clinical incidence 3000/mi Sarcoma annual clinical incidence 30/mi No significant geographic differences

A.

Benign soft tissue tumors (epidemiology) 1/3 lipoma 1/3 fibrohistiocytic and fibrous tumors 10% vascular tumors 5% nerve sheath tumors 99% are superficial 95% are <5cm in diameter

Note: sabi sa ppt ni doc, there is a relationship between type of tumors,

symptoms, location and patients age and gender

EXAMPLE: Lipoma - painless, rare in hand, lower leg and foot are uncommon in children Multiple Angiolipoma - painful, in young men Angioleiomyoma - painful, lower leg, middle aged women Vascular tumors () - younger than 20yo

B.

65yo)

Soft tissue Sarcomas (epidemiology) may occur anywhere extremeties (thigh) 10% trunk wall and retroperitoneum Slight male predominance More common in increasing age (median age

*epidemiology pa din to ng soft tissue ah? Panalo sa epidemiology ang lecture ni doc sa soft tissue..siguro wala na kasing mailecture.. =)

Size: Extremities/trunk wall tumors 1/3 superficial with a median diameter of 5cm 2/3 deep seated with median diameter of 9cm Retroperitoneal tumors Large on diagnosis and 1/10 have metastasis (most common lung) 1/3 die because of the tumor are high grade pleomorphic (MFH-like), liposarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumors are highly malignant (grade 3-4) Age: vary/type

embryonal rhabdomyosarcoma - exclusive in children

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