2. PATH Pt 2: Bones Joints and Soft Tissue

Question 1

What is the MC benign bone tumor, aka exostosis, 85% are solitary in early adulthood, in AD* may see in younger+multiple lesions (20% progress to chondrosarcoma), more common in MEN (x3), assoc with EXT1/2 in sporadic osteochondromas, which encode heparin sulfate glycosaminoglycans, located at the METAPHYSIS near growth plate of tubular bone?

Answer 1

Osteochondroma (B9 cartilage forming tumor)

Question 2

Osteochondroma of bone appears as a bony projection, most being solitary, incidental lesions that may be excised if they cause local pain, there is a rare condition of multiple Osteochondromas marked by bone deformity and by a greater propensity to develop?

Answer 2

chondrosarcoma

Question 3

Osteochondroma has a bluish white cartilaginous cap which overlies the bony cortex, they are probably not true neoplasms but they are a lesion that extends outward from the metaphyseal* region, see disorganized growth, either sesile or pedunculated- continuous with?***

Answer 3

Medullary Cavity

Question 4

What is a benign cartilage, solitary metaphyseal lesion usually on hands and feet in 20-50 y/o, may be within medullary cavity (completely inside=enchondroma) or on the surface aka subperiosteal or juxtacortical?

Answer 4

Chondroma

Question 5

Ollier syndrome associated with multiple enchondroma, maffucci syndrome is associated with multiple enchondromas with angiomas leading to an increased risk of chondrosarcoma and other malignancies, what are two genes that are associated with Chondromas, same gene seen in GBM?

Answer 5

IDH1 and IDH2

Question 6

Enchondroma are less than 3cm, gray blue and translucent, well circumbscribed nodules of hyaline cartilage (encased in thin layer of reactive bone) containing benign chondrocytes, the periphery may ossify, and the center can calcify and infarct, ollier and maffuci (hereditary) are associated with what additional presence within the enchondroma?

Answer 6

Increased cellularity and atypia

radiolucent nodule with central calcification DOES NOT penetrate cortex

Question 7

What is the second MC malignant* matrix producing tumor of bone (osteosarcoma is first), more common in men (x2), usually in 40s+, lesions like axial skeleton (pelvis, shoulder, ribs), invade locally, painful enlarging mass may metastasize, 70% grade 3 which hematogenously spread to LUNGS, have a direct correlation between grade and behavior?

Answer 7

Chondrosarcoma

Question 8

Chondrosarcomas grow fast and have nodules of hyaline and myxoid cartilage permeating throughout the medullary cavity, growing through the cortex and forming well circumscribed soft tissue masses, see anaplastic chondrocytes amid hylaine cartilage , *bizarre giant cells w multiple nuclei, have a calcified matrix appears as foci of *flocculent densities, what are those?

Answer 8

Resembling tufts of wool = flocculent

Question 9

85% of Chondrosarcoma arise de novo or sporadically, the remainder occur in preexcisting osteochondromas or enchondromas, multiple osteochondroma syndrome assoc w mutations in EXT gene, chondromatosis-related and sporadic Chondrosarcomas are commonly associated with what gene?

Answer 9

IDH1/2 (+/-)

Question 10

What syndrome presents at age 10-15, due to APC gene mutation, associated with multiple GI adenomas, thyroid and desmoid tumors, skin cysts and **Osteomas?

Answer 10

Gardner Syndrome

Question 11

What is a benign lesions that is painful, usually worse at night and responds clinically to aspirin* and NSAIDs*, there is excess prostaglandin E2 production by osteoblasts, seen in young men - teens and 20s, located at appendicular skeleton = 50% FEMUR or TIBIA, lesions must be less than 2cm otherwise are considered osteoblastomas?

Answer 11

Osteoid Osteoma

severe nocturnal pain due to production of PGE2, releived via ASA

Question 12

Osteoid Osteoma has a central nidus of translucent woven bone surrounded by a rim of osteoblasts, associated with a thick rind of reactive cortical bone = radiographic clue, they are with what?

Answer 12

Radiofrequency Ablation - easily

Question 13

Osteoid Osteoma are composed of haphazardly interconnecting trabeculae of woven bone that are rimmed by prominent osteoblasts, what type of Osteoid Osteoma is in the cortex of the bone and has central mineralization surrounded by reactive bone?

Answer 13

Intracortical Osteoid Osteoma (nidus)

Question 14

What is bigger than an osteoid osteoma (>2cm) involves the posterior spine*, has no bony reaction, achy pain that does NOT respond to aspiring, treated by curretted or excised in bloc, and malignant transformation is rare?

Answer 14

Osteoblastoma

Question 15

What is the MC primary malignant tumor of the bone with malignant mesenchymal cells that produce bone matrix, presents as a painful enlarging mass, 75% are less than 20 males AROUND THE KNEE**, 2nd peak in older males with pagets or due to PRIOR RADIATION?

Answer 15

Osteosarcoma

Question 16

Osteosarcoma is associated with Rb loss - 70% sporadic, TP53 is seens with Li-Fraumeni syndrome (breast cancer as well), INK4a gene, MDM2/CDK4 inhibit p53 and rb and are associated with large Osteosarcomas, on xray one sees mixed lytic and blastic mass with infiltrative margins, and what?***

Answer 16

Codman triangle- elevation of periosteum* due to proximal reactive bone

Chondrosarcoma in adults/Osteosarcoma in kids

Question 17

Osteosarcoma is MC around the KNEE and due to prior radiation, spreads early to lung, surgery and chemo have a 70% 5yr survival, spreads hematogenously to lungs- 90% mets to lung, bones, brain who died with tumor, on histo see bizarre tumor giant cells*, malignant osteoid, abnormal mitosis, and is it known for extension where?

Answer 17

extends through the cortex outside of the bone - formation of bone by tumor cells is diagnostic

Question 18

Where does osteosarcoma MC metastasize?

Answer 18

LUNGS

Question 19

What tumors occur younger than 20 (80%), with predilection for WHITES, not blacks or asians, 2nd MC bone sarcoma in children, aka small blue round cell tumors w SCANT cytoplasm, undifferentiated with neural differentiation is known as primitive neuroectodermal tumor or PNET?

Answer 19

Ewing Sarcoma

Question 20

Ewing Sarcoma is a painful enlarging mass with tenderness, warm and swelling, mimics infection because also has fever and increased ESR, a periosteal reaction occurs leading to reactive bone in onion-skin fashion on xray, MC in the diaphysis of LONG BONES especially?

Answer 20

FEMUR and flat bones

arises in medullary cavity and invades cortex

Question 21

Ewing Sarcoma has a 75% 5 year survival with surgery, chemo, and radiation, the amount of chemo-induced necrosis (more is better) is an important PROGNOSTIC factor- indicates sensitivity to drugs, 85% show t(11;22)(q24:q12) which is a fusion of what two genes?

Answer 21

EWS-FL11 (EWS gene)

necrosis prior to therapy could indicate rapid growth = BAD

Question 22

Sunburst pattern on xray and codman is associated with osteosarcoma, lamellated or onion-skin is associated with ewing, hair-on-end on xray is associated with spherocytosis, iron def anemia, neuroblastoma, hemolytic anemia, sickle cell disease, and thalassemia major…

Answer 22

meow

Question 23

Ewing Sarcoma arises in the medullary cavity and invades the cortex, periosteum and soft tissue, sheets of small round blue cells with scant cytoplasm are seen, contain hemorrhage and necrosis, what type of rosettes are seen, which are round groupings of cells with central fibrillary core signaling a neuroextodermal differentiation or PNET?

Answer 23

Homer Wright Rosettes

Question 24

What type of dysplasia is benign proliferation of fibrous tissue and bone that do not mature, may be a developmental anomaly, monostotic (70%) and usually asymptomatic- found incidentally but may cause pain/fracture/discrepancies in limb length, (polystotic is crippling), mostly occurs in early adolescence, M=F?

Answer 24

Fibrous Dysplasia

Question 25

Fibrous Dysplasia shows a ground glass appearance, with well defined margins radiographically because it is benign. What is a type of fibrous dysplasia associated with GNAS1 mutation in embryogenesis, causing polyostotic disease associated with cafe-au-lait skin pigmentations and endocrine abnormalities- esp precocious puberty?

Answer 25

McCune-Albright Disease*

Question 26

What syndrome is associated with fibrous dysplasia (usually polyostotic), along with multiple skeletal deformities in childhood such as soft tissue intramuscular myxomas?

Answer 26

Mazabraud Syndrome

Question 27

Fibrous Dysplasia is composed of curvilinear trabeculae of woven bone that lack conspicuous osteoblastic rimming and arise in a background of fibrous tissue, commonly described as what characters?

Answer 27

Chinese characters

Question 28

Somatic GNAS1 gain-of-function mutation, same gene is mutated in pituitary adenoma • Constitutively active GS protein, activating cAMP→ cellular proliferation • Extent of phenotype depends on stage in which the mutation occurs and the fate of cell harboring the mutation • McCune Albright occurs when mutations happens during embryogenesis • monostotic fibrous dysplasia happens when mutation occurs in one osteoblast precursor during or after skeletal formation

Answer 28

woof

Question 29

What type of tumor of the bone has osteoclastoma multinucleated osteoclast-type GIANT cells**, must consider brown tumor of hyperparathyroidism, occurs between 20-40s and is benign but locally aggressive, may be of monocyte-macrophage derivation, arises in epiphyses** and may extend into metaphysis?

Answer 29

Giant cell tumor of bone

Question 30

Giant cell tumor of bone is MC in knee- distal femur/proximal tibia causing arthritis like symptoms, tends to recur after curettafe, 4% mets to lungs and may regress and are seldomly fatal, histo shows predominantly lytic, expansile with destruction of the cortex, always see abundance of what?

Answer 30

Multinucleated giant cells

Question 31

Metastatic tumors are the MC form of skeletal malignancy, spread via direct extension, lymph/hematogenous, or intraspinal seeding, in adults- 75% are from *prostate, breast, kidney and lung, in kids see mets from neuroblastoma, wilms, osteosarcoma, rhabdomyosarcoma and?

Answer 31

Ewing*

Question 32

metastatic tumors are multifocal except kidney and thyroid are usually solitary, small bones of the hands usually mets from lung/kidney/colon, on XR you see lytic bone destroying lesions from kidney/lung/gi/MM, what facilitates implantation and growth - seen in the axial skeleton?

Answer 32

Red marrow (rich capillary and slow blood flow)

Question 33

Metastatic tumors can be both mixed lytic and blastic, there is a dim prognosis, manage sx to limit futher spread but will probs die, what is the ONLY blastic or bone forming metastasis to bone?

Answer 33

Prostatic Adenocarcinoma****Unique

Question 34

Joints allow movement while providing mechanical stability, they are classified as solid (nonsynovial) and cavitated. Solid joints aka synarthroses provide structural integrity and allow only minimal movement and they lack what?

Answer 34

Joint space (70% H20/10% Type II Collagen)

Question 35

Synovial joints in contrast, have a joint space, that allows for a wide range of motion, situated between the ends of bones formed via enchondral ossification, they are strengthened by a dense fibrous capsule and reinforced by ligaments and?

Answer 35

muscles

Question 36

Osteoarthritis is associated with thinned cartilage on the ends of the bones, allowing the bones to rub together causing creputis (later in life, progress over years, assymetrical to polyarthritis, weight bearing joints, stiff less than 1 hour* but END of day), while rheumatoid arthritis is do to what?

Answer 36

swollen inflamed synovial membrane (happen at any age, rapid onset, symmetrical polyarthritis, usually small joints of hands and feet, stiff >1hour at BEGIN of day)

Question 37

What is the MC type of joint disease, AKA degenerative joint dz DJD, primary or idiopathic due to aging, oligoarticular (few joints), increases exponentially >50 years, 40% of 70 year olds are affected, hands and knees in women and *hips in men?

Answer 37

osteoarthritis *pain better with rest and NSAIDs

Question 38

osteoarthritis can secondarily be caused due to joint deformation, trauma, or marked obesity and is seen in younger people, but only accounts for 5% of OA, other causes include DM, hemochromatosis, thyroid, acromegaly and charcot, is a disease of CARTILAGE- wear and tear, crepitus, dec range of motion, and worse with use, is it worse in morn or evening?

Answer 38

Evening stiffness ** also see morning stiffness less than 30min while RA has stiffness greater than 30mins in the AM

Question 39

DJD or OA is MOST common in proximal interphalanges PIP which are aka Bouchards nodes, and also common in distal interphalanges DIP which are are called what nodes?

Answer 39

Heberden Nodes

Question 40

Osteoarthritis is associated with increased water content of matrix and concentration of proteoglycans decreases causing cracks in the matrix, eventually hunks of cartilage slough causing *joint mice*, subchondral bone exposed and rubbed smoothed is bone on bone pain and also called?

Answer 40

Eburnation

Question 41

Osteoarthritis- the bone tries to buttress stress, microfractures and subchondral cysts develop due to the stress, osteophytes then form and nerve root compression and radicular pain can occur, there is narrowing of the?

Answer 41

Joint space

Question 42

With Osteoarthritis you see characteristic fibrillation of the articular cartilage on histo, and grossly can see eburnation, cyst formation, and residula?

Answer 42

articular cartilage (location of eburnation is where cartilage is no longer)

Question 43

Rheumatoid Arthritis is associated with overgrowth of synovial tissue which is also called?*

Answer 43

PANNUS, also see inflam, ankylosis, and redness

Question 44

What occurs in 2-4th decades and MC in women (x3), usually autoimmune, nonsuppurative proliferative and inflammatory synovitis, causing destruction of the articular cartilage leading to ankylosis which is stiffness of a joint due to abnormal adhesion and rigidity of the bones of the joint, which may be the result of injury or disease?

Answer 44

Rheumatoid Arthritis

Question 45

Rheumatoid Arthritis has a symmetrical distribution, and focuses on small joints, causing them to be swollen, warm and painful- commonly see morning stiffness for longer than *1 hour*, can see marked synovial hypertropht with formation of villi, along with the tissue containing dense?

Answer 45

lymphoid aggregate

Question 46

HLA DRB1 (DR4**) and environmental factors together, causes B and T cells to respond to self antigens (antigen antibody complexes contain citrullinated peptides CCP), CD4+ T helper cells initiate the aut0immune response in RA by reacting with an arthritogenic agent, perhaps microbial or self-antigen, of these which agent is most directly linked to RA, alng with IL1?

Answer 46

TNF* TFN and IL1 together stimulate synovial cells to secrete RANKL which causes bone resorption IFN activates Mø and IL17 recruits neutros and monocytes to area from TH1/TH17

Question 47

There are four tests that are commonly associated with Rheumatoid Arthritis, 2 less common are C reactive protein and erythrocyte sedimentation rate ESR, the 2 that are seen in 80% of RA patients is anti-cyclic citrullinated peptide (CCP) antibodies and what else?

Answer 47

Rheumatoid Factor RF

Question 48

Rheumatoid Arthritis is associated with boutonniere which is deformity of fingers- see hyperextension of DIP and flexion of PIP, swan neck - hyperextension of PIP and flexion of DIP, ulnar deviation of fingers which decreases joint space, causing periarticular bony erosions, what deviation of the wrist is seen?

Answer 48

Radial Deviation of the wrist ****PIPs and MCPs****

Question 49

Rheumatoid Arthritis is *characterized by pannus which is edematous, thickened, hyperplastic synovium, synovial cell hyperplasia and dense inflammatory infiltrates frequently forming?

Answer 49

Lymphoid Aggregates

Question 50

Rheumatoid Arthritis is also associated with increase vascularity due to angiogenesis, fibrinopurulent exudate on the synovial surfaces and joints and osteoclast activity, pannus is granulation tissue which leads to what?

Answer 50

bony akylosis

Question 51

Other commonalities presenting with Rheumatoid Arthritis includes what, which are extra articular lesions that may involve skin heart blood vessels and lungs and therefore the clinical manifestations can resemble other systemic autoimmune disorders such as SLE or scleroderma?

Answer 51

Rheumatoid Nodules

Question 52

Rheumatoid Nodules are composed of a central necrosis rimmed by palisaded histioytes, they are firm, nontender, and round to oval and in the skin arise in the subcutaneous tissue, microscopically they resemle what, with a central zone of fibrinoid necrosis surrounded by a prominent rim of activate macrophages and numberous lymphocytes and plasma cells?

Answer 52

Necrotizing granulomas

Question 53

``` Arthritis:? disease of cartilage wear and tear large-weight bearing joints not inflammatory heberdens and bouchard nodes no systemic effects no morning stiffness (or less than 30 mins) worse* with activity ```

Answer 53

Osteoarthritis

Question 54

``` Arthritis? Disease of synovium Autoimmune Small, symmetrical joints Inflammatory Swan-neck deformities and Ulnar deviation Systemic effects Morning stiffness >1hr Worse with REST ```

Answer 54

Rheumatoid Arthritis

Question 55

Seronegative spondyloarthropathies are autoimmune, Tcell response is initiated by environmental factors in predisposed, there is no specific antibodies (seronegative), there are pathogenic changes in the ligamentous attachments rather than the synovium, negative rheumatoid factor RF and has a huge associated with what HLA?

Answer 55

HLAB27**

Question 56

What spondylitis is associated with HLAB27, and affects vertebral and sacroiliac joints in the 20-30s, along with uveitis, aortitis, amyloidosis are complications, progressive dz, 1/3 pts have peripheral joint involvement such as hip, knees and shoulders?

Answer 56

Ankylosing Spondylitis Bamboo spine from fusion of vertebrae

Question 57

What arthritis is a seronegative disease, will see arthritis, urethritis or cervicitis, and conjunctivitis* (triad), 80% have HLAB27, *men 20-30s, also in HIV, might have prior GI/GU infections (chlamydia, shigella, campy, salmonella, yersinia), affects ankles knees and feet in asymmetric pattern, also see cardiac conduction abnl, and aortic regurg?

Answer 57

Reactive Arthritis (cant see cant climb a tree cant pee)

Question 58

Enteritis associated athritis include yersinia, slamonella, shigella, and campylobacter, lipopolysaccharides of the outer cell membranes stimulate immunological resonse, appears sudden and lasts for 1 year, most commonly at what two joints?

Answer 58

Knees and ankles (+/- wrist, fingers, toes)

Question 59

What arthritis affects peripheral hands and feet and axial joints, ligaments and tendons, associated with HLAB27 and less common HLA Cw6, 30-50s, *pencil in cup* deformity of dips and is assymetrical, frequent remissions, less joint destruction?

Answer 59

Psoriatic Arthritis | psoriaisis in 10%- nails turn thick, rough, rigid with pits

Question 60

What type of arthritis is caused by microorganisms that seed joints via hematogenous dissemination, direct inocultion or contiguous spread via soft tissue abscess or osteomyelitis, there is rapid destruction of the joint causing permanent deformities?

Answer 60

Infectious Arthritis

Question 61

Suppurative arthritis is usually spread to the joint hematogenously, and the bacteria differs in different age groups. For kids less than 2, H. influenza causes the arthritis, in older kids and adults what two are most common?

Answer 61

S. Areus and Gonococcus

Question 62

Gonococcal arthritis is most common in sexually active women, and MAC deficiency makes the patient susceptible to disseminated GC, where you see acutely painful swollen joint with restricted range of motion, sometimes associated with fever, leukocytosis and elevated ESR, 90% of non-gonococcal cases involve?

Answer 62

a SINGLE joint - KNEE is MC

Question 63

For suppurative arthritis, joint aspiration is diagnostic, and you see purulent fluid, if mycobacterial there is an insidious onset, with gradual progressive pain at hip* or knee, what disease is caused by borrelia burgdorferi in which 60-80% dev days or weeks after the skin lesion, mc in knee/shoulders (large joints)?

Answer 63

Lyme Disease

Question 64

What organism is seen in healthy adults (particularly young sexually active), associated with tenosynovitis, vesicular pustules, late complement deficiency, and has a negative synovial fluid culture and gram stain?

Answer 64

Neisseria Gonorrhea

Question 65

What is transient attacks of acute arthritis initiated by crystallization of monosodium urate (MSU) within and around joints, preceipitation MSU into joint causes cytokines that recruit leukocytes, due to overproduction or reduced excretion --- plasma levels of MSU greater than 6.8 mg/dl?

Answer 65

Gout

Question 66

Gout is associated with other factors that predispose a person to it, besides hyperuricemia, including age, genetic predisposition, heavy alcohol* consumptions, obesity, drugs such as thiazide diuretics which decreases excretion of urate and what toxicity?

Answer 66

Lead Toxicity purine catabolism increases uric acid

Question 67

Primary gout is usually due to unknown enzyme defects, the most commonly known enzynme defect is HGPRT deficiency (hypoxanthine guanine phosphoribosyl transferase) which causes increased uric acid production, secondary gout is due to increased nucleic acid turnover (leukemia), congenital like lesch-nyhan syndrome, and chronic?

Answer 67

Renal disease (decrease in uric acid excretion) uric acid is usually completely reabsorrbed in the proximal tubule

Question 68

Hyperuricemia (MSU>6.8) causes preceiptation of urate crystals in LOWER temperatures- usually peripheral joints, which causes phagocytosis via mø and activation of complement, mø release IL-1B**** causing acute gouty arthritis and bringing in neutrophils, and the crystals activate complement system which ultimately ends in?

Answer 68

Tissue Injury and Inflammation *IL1 and IL1B*****

Question 69

What is associated with needle-shaped crystals and have a negative birefringence?

Answer 69

Gout-monosodium urate crystals

Question 70

What is associated with rhomboid-shaped crystals, weakly positive birefringent- yellow under parallel light and blue under perpendicular light- associated with underlying metabolic disease usually?

Answer 70

Pseudogout (calcium pyrophosphate dehydrate)

Question 71

Gout's MC lesion is the first metatarsophalangeal joint in the GREAT TOE, assoc with excruciating joint pain, localized hyperemia and warmth, can see urate crystals which are needle shaped and negative birefringent under polarized light, and WHAT is a pathognomonic lesion for gout which is an aggregate of dissolved urate crystals surrounded by reactive fibroblasts, mononuclear inflamm cells and giant cells?

Answer 71

Gouty Tophus**

Question 72

Caclium Pyrophosphate crystal deposition disease CPPD is aka as chondrocalcinosis or what, which is seen in >50s and increases with age, sporadic, hereditary and secondary, secondary MCC are assoc with previous joint damage, hyperparathyroidism, hemochromatosis, hypothyroidism, diabetes and ochronsis (syndrome caused by accum of homogentisic acid in CT- yellow discoloration of the tissue seen on microscope)?

Answer 72

Pseudogout or Chondrocalcinosis

Question 73

Pseudogout has crystals that form chalky, white friable deposits, and on histo show oval blue-purple aggreagates, deposits are present in the cartilage and consist of amorphous basophilic material, the individual crystals are rhomboid shaped and are what under light?

Answer 73

Positively* birefringent

Question 74

What type of cyst is usually in the joint capsule or tendon seath, 1-1.5cm cyst, usually on the wrist-firm fluctuant pea-sized translucent nodule, cysts LACKs cell lining and on FNA see a thick myxoid material (degerenation)?`

Answer 74

Ganglion Cyst

Question 75

A synovial cyst is herniation of synovium through a joint capsule or massive enlargement of bursa, synovial lining may be hyperplastic and contain inflam cells and fibrin, what is an example- which is seen in the popliteal space in rheumatoid arthritis?

Answer 75

Baker Cyst

Question 76

Soft tissue is non-epithelial tissue, benign tumors are much more common than malignant, sarcomas are malignancies arising from soft tissue, they are uncommon but aggressive, usually arise in extremities - THIGH, and are associated with many tumor suppresor genes including gardner, li fraumeni, osler weber rendu and?

Answer 76

NF1

Question 77

Soft Tissue Sarcomas are also associated with environmental exposure such as radiation, burns and toxins, there are simple karyotype tumors which are in young and and have limited chormosomal change, and there are what kind of karyotype (85%) which is seen in adults and has genomic instability?

Answer 77

Complex Karyotype (examples include leiomyosarcomas, and undifferentiated sarcomas-morphogenically pleomorphic)

Question 78

Sarcoma types include angiosarcoma, osteosarcoma, liposarcoma, ewings sarcoma, chondrosarcoma, hemangioendothelioma...

Answer 78

moo

Question 79

What is a benign tumor of adipose tissue, is soft mobile and painless, MC soft tissue tumor of adults, which many variants including angiolipoma, spindle cell, pleomorphic, myelolipoma, usually in subcutis but can occur in deep soft tissue- extremities and trunk, on histo see encapsulated mass of normal appearing adipose tissue?

Answer 79

Lipoma

Question 80

What is a common malignant ST tumor in adults 50-60, deep soft tissue of proximal extremities and retroperitoneum, assoc with 12q13-q15 & t(12;16)- well differentiated and myxoid type respectively, one of the key genes in the amplified area of chr 12q is MDM2* encodes a potent inhib of p53?

Answer 80

Liposarcoma

Question 81

Liposarcomas recur locally and repeatedly, unless completely excised, variants include well differentiated, myxoid (intermediate), round cell and pleomorphic which are high grade, what is the MC variant?

Answer 81

Myxoid Liposarcoma - t(12;16) MDM2 = no p53

Question 82

What type of fibrous tissue is self limited fibroblastic and myofibroblastic proliferation seen in young adults in the UPPER extremity, 25% assoc w hx of trauma, rapid growth- <5cm, t(17;22) = MYH9-USP6 fusion gene = clonal self-limited proliferation- spontaneously regresses?

Answer 82

Nodular Fasciitis (20-30s)

Question 83

What group of fibrous tumors are infiltrative, with local deformity and MC in men, such as palmar fibromatosis = dupuytren contracture where there is slow progressive flexion contracture of the 4th/5th fingers?

Answer 83

Superficial Fibromatosis

Question 84

Other superficial fibromatoses include plantar which is seen in young patients and are unilateral with no contractions, or penile which is aka as what, and is on the dorsolateral aspect of the penis, may lead to abnl curvature of the shaft, constriction of the urethra or both?

Answer 84

Peyronie Disease

Question 85

What is aka deep fibromatosis and is a large infiltrative tumor that frequently recurs and often is painful, seen in teens-30s mostly women, see mutations in APC/Bcatenin genes increasing WNT signaling, associated with familial adenomatous polyposis or gardner syndrome- who have APC mutations?

Answer 85

Desmoid tumor*

Question 86

In nodular fascitis you see plump, randomly oriented stellate and spindle cells (separate by collagen bundles) surrounded by myxoid stroma, assoc with mitotic acitvity and extravasated red blood cells. What fibrous tissue is seen on histo as infiltrating between skeletal muscle cells-nodular or poorly defined broad fascicles of fibroblasts in long, sweeping fascicles surrounded by abundant dense collagen?

Answer 86

Fibromastosis

Question 87

What sarcoma in adults is deep seated slow growing mass in thigh or trunk of young or middle aged adult 35% 5yr survival, infantile- children less than 2 often congenital arises in extremities, smaller and more superficial 85% 5yr, histo shows striking uniform, spindle cells arranged in herringbone fascicles***?

Answer 87

Fibrosarcoma

Question 88

What sarcoma is a malignant skeletal muscle tumor with 3 subtypes- the 2 most important are alveolar and embyronal, in kids**** in the sinuses, head and neck, and GU tract (vagina is MC)?

Answer 88

Rhabdomyosarcoma

Question 89

Rhabdomyosarcoma has two main varients both seen in kids, which type has a variant known as sarcoma botryoides which has a cambium layer, see as grape like clusters that grow in cavities, and is associated with the BEST prognosis?

Answer 89

Embryonal which is the MC

Question 90

Rhabdomyosarcoma has another type which is what, less common but seen in kids and is due to fusion of FOXO1 gene to either PAX3 (2;12) or PAX7 (1;13), PAX 3 is a trancription factor that initiates skeletal muscle differentiation?

Answer 90

Alveolar Rhabdomyosarcoma

Question 91

Rhabdomyoblasts are said to look like a tadpole cell or elongated strap cells (with crossing striations intermixed with SMALL ROUND BLUE CELLS**). In embryonal Rhabdomyosarcoma of the uterine cervix (MC) a cellular subepithelium what is seen which contrasts with underlying hypocellular tumor?

Answer 91

Cambium Layer - dense purple thick

Question 92

What is a benign smooth muscle tumor, seen in the uterus (fibroid) is the MC neoplasm in women, pilar version of erector pili muscle in skin and can be multiple and painful, can also be seen in the hereditary leiomyomatosis and renal cell cancer syndrome which is AD, many uterine leiomyomas are seen with RCC* due to LOF at chr 1q42.3 (fumarate hydratase enzyme deficiency)?

Answer 92

Leiomyoma

Question 93

What makes up 10-20% of ST sarcomas, MC in adult women in the extremities and retroperitoneum, treatment is based on tumor size, location and grade, local extension occurs commonly with mets to the lung, there is lots of necrosis due to lack of blood supply cause of its quick growth?

Answer 93

Leiomyosarcoma

Question 94

What is a tumor of uncertain origin, first tumor found adjacent to a joint, also found in chest wall, head and neck, 10% of all ST sarcomas, 4th MC, seen in 20-40s, deep mass for years, t(x;18)(p11;q11) producing ss18-ssx1, -ssx2 or ssx4 fusion genes that encode chimeric transcription factors, ***+for keratins and epithelial markers***, 5yr survival is 25%, mets to lungs common?

Answer 94

Synovial Sarcoma On histo see classic biphasic spindle cell and glandlike histo appearance with epithelial cells