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Neuro Muscular Psych Block 3 > Bone Tumors, Joints and Soft Tissue Path > Flashcards

Bone Tumors, Joints and Soft Tissue Path Flashcards

1
Q

Osteochondroma

Behavior, Mutation, Location, Severe Form (2)

A

Benign

EXT1/EXT2 Mutation

Metaphysis

Mutliple hereditary exostosis: progresses to chondrosarcoma

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2
Q

Enchondroma

Behavior, Location, Mutation, Composition, Syndromes (2) with Descriptions (1/3)

A

Benign

Metaphysis

IDH1/IDH2

Hyaline Cartilage

Ollier Syndrome: mutliple enchondromas

Maffucci Syndrome: multiple enchondromas + angiomas with increased risk of chondrosarcoma

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3
Q

Chondrosarcoma

Behavior (2) Locations (2) Mutation, Morphology (2) Clinical Feature

A

Malignant
Metastasizes to lungs (Grade III)

Axial Skeleton
Shoulders

EXT mutation

Calcified matrix making flocculent densities
Multinucleated giant cells

Direct correlation between Grading and prognosis

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4
Q

Osteoid Osteoma

Location, Morphology (3) Clinical Features (2) Treatment

A

Lower Extremity

<2 cm
Central nidus surrounded by rim of osteoblasts
Thick rind of reactive cortical bone

Pain worst at night
Pain responsive to Aspirin and NSAIDS

Treat with Radiation

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5
Q

Osteoblastoma

Location, Morphology (2) Clinical Feature, Treatment

A

Posterior spine

> 2 cm
No bony reaction (formation of new bone)

Achy pain unresponsive to aspirin

Curetted or Excised

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6
Q

Osteosarcoma

Behavior (2) Location, Mutations (2) Morphology, Clinical Features (2)

A

Most common bone malignancy
Metastasis to lungs

Knees

Rb gene
TP53 (Li-Fraumeni syndrome)

Mixed lytic and blastic mass
Codman Triangle (Periosteum lifted off bone)

Associated with prior radiation
Painful rapidly enlarging mass

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7
Q

Ewing Sarcoma Family Tumors

Behavior, Location, Mutation (2) Morphology (4) Clinical Features (2) Prognostics

A

Malignant

Femur diaphysis

t(11;22)(q24:12) translocation
Makes EWS-FL11 fusion gene

Onion-skin lamellations on X ray
Small round blue cell tumor
PNET if neural differentiated: Homer-Wright Rosettes

Tender, warm, swollen mass
Fever

Amount of chemotherapy-induced necrosis is prognostic

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8
Q

Fibrous Dysplasia
Behavior, Locations (3) Mutation, Morphology (2)
McCune Albright Syndrome Features (3)

A

Benign

Craniofacial bones
Lower extremity
Ribs

GNAS mutation

Ground glass appearance
Curvilinear Trabeculae (Chinese characters)

Unilateral Fibrous Dysplasia
Ipsilateral Cafe au Lait spots (hyperpigmented)
Precocious puberty

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9
Q

Giant Cell Tumor of Bone

Behavior, Location (2) Cell type, Clinical Features (2)

A

Benign

Epiphysis of knee

Osteoclastoma: multinucleated osteoclast-type giant cells

Arthritis-like symptoms
Recur after curretage

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10
Q

Metastasis to Bone
Most Common Primary Tumors
Adult (4) Child (5)
Locations of Secondary tumors (2)

A 
Adult
Prostate
Breast
Kidney
Lung
Children
Neuroblastoma
Wilms
Osteosarcoma
Ewing
Rhabdomyosarcoma

Small bones of hands/feet (lung, kidney, colon)
Axial skeleton

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11
Q

Lytic vs Blastic Lesions

Descriptions with Examples (4/1)

A 
Lytic: bone destroying
Lung
Kidney 
GI Tract
Melanoma

Blastic: bone building
Prostatic adenocarcinoma

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12
Q

Osteoarthritis

Locations (3) Pathogenesis, Deformities (2) Pathological Course (4) Presentation (3)

A

Large Joints: Knees/hands in women, hips in men

Water increases in matrix and proteoglycans decrease

Bouchard’s Nodes: PIP joints
Heberden Nodes: DIP joints

Cartilage sloughed (joint mice)
Subchondral bone rubbed smooth (eburnation)
Subchondral cysts develop
Osteophytes form

Joint stiffness worst in evening
Worse with use
Crepitus

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13
Q 
Rheumatoid Arthritis
Clinical Features (2) Deformities (4) Morphology (3)
A

Morning stiffness and pain that gets better with use
Affects small joints

Boutonniere: flexion of PIP with extension of DIP
Swan-Neck
Ulnar Finger Deviation
Radial Wrist Deviation

Pannus: hyperplasia of synovium
Lymphoid aggregates
Fibrous/Bony Ankylosis

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14
Q

Rheumatoid Arthritis

Genetics (3) Pathogenesis (3) Diagnostic Tests (2)

A

PTPN22 gene
HLA-DRB1 gene encoding HLA DR4

Th17 cells secrete IL17, Th1 cells secrete IFN-gamma
Neutrophils recruited, cause synovial proliferation
TNF and IL1 cause protease production

Rheumatoid Factor
CCP

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15
Q

Ankylosing Spondylitis

Genetics, Locations (2) Complications (3)

A

HLA B27

Vertebrae
Sacroiliac joint

Uveitis
Aortitis
Amyloidosis

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16
Q

Reactive Arthritis

Genetics, Locations (4) Pathogenesis (2) Presentation (4)

A

HLA B27

Ankles
Knees
Feet
Severe Spine involvement

Prior GI or GU infection

Arthritis
Urethritis/Cervicitis
Conjunctivitis
Cardiac issues

17
Q

Enteritis Associated Arthritis

Etiologies (4) Locations (2)

A

Yersinia
Shigella
Salmonella
Campylobacter

Knees
Ankles

18
Q

Psoriatic Arthritis

Genetics, Deformities (2)

A

HLA B27

Pencil in cup deformity to DIP’s
Nail changes

19
Q

Suppurative Arthritis

Etiologies (6) Clinical Features (2) Diagnosis

A 
S. aureus*
Gonococcus*
H. influenzae (under 2 years)
TB
Borrelia burgdorferi
Viral: HIV, HBV, HCV, EBV, parvo, Rubella

Acutely painful and swollen joint
Knee most affected

Purulent fluid from joint aspiration

20
Q

Gout

Crystal Morphology, Risk Factors (4) and Clinical Features (3)

A

Monosodium urate needle-like crystals

Alcohol consumption
Obesity
Thiazide diuretic use
Lead toxicity

Peripheral joints affects (lower temperature)
Excruciating pain
Elevated IL-1

21
Q

Gout
Primary Characteristics (3)
Secondary Etiologies with Descriptions (3)

A

Primary:
Enzyme deficiency (HGPRT)
Elevated uric acid production
Normal uric acid excretion

Secondary:
Leukemia: increases nucleic acid turnover
Chronic renal disease: decreased uric acid excretion
Lesch-Nyhan: Elevated uric acid production and decreased uric acid excretion

22
Q 
Pseudo-Gout
Crystal Morphology (4)
A

Calcium Pyrophosphate crystals
Blue-purple crystals on histology
Chalky white friable deposits
(+) Birefringence

23
Q

Ganglion Cyst

Location (2), Pathogenesis, Morphology (3)

A

Wrist
Joint capsule or tendon sheath

Cystic or myxoid degeneration

Fluctuant
Pea sized translucent nodule
Wall lacks cell lining

24
Q

Synovial Cyst

Pathogenesis, Baker Cyst Description (2)

A

Herniation of synovium through joint capsule

Baker Cyst: found in popliteal space, associated with rheumatoid arthritis

25
Q

Liposarcoma

Behavior, Locations (2) Mutation (2) Most Common Variant

A

Malignant
Recur repeatedly unless completely excised

Retroperitoneum
Proximal Extremities

t(12;16)(q13:p11) translocation
FUS-DDIT3 fusion gene

Myxoid most common

26
Q

Nodular Fasciitis

Location, Mutation (2) Prognosis

A

Upper extremity

t(17;22)
MYH9-USP6 fusion gene

Spontaneously regresses

27
Q

Fibromatoses with Complications

Palmar, Plantar, Penile

A

Palmar: Dupuytren contracture (digit 4/5)

Plantar: in kids, no contracture

Penile: Peyronie Disease, dorsolateral penis

28
Q

Desmoid Fibromatosis

Mutations (3) Predisposition

A

APC gene, Beta-catenin genes
Increased WNT signaling

Gardner syndrome (FAP) with APC gene defects

29
Q 
Rhabdomyosarcoma
Behavior, Locations (3)
Subtype Descriptions
Alveolar Genetics (2) Embryonal Morphology (3) Pleomorphic Population (1)
A

Malignant

Alveolar:
Kids with FOXO1-PAX3/PAX7 fusion gene

Embryonal:
Kids
Rhabdomyoblasts
Sarcoma botyroides in cambium layer

Pleomorphic:
Adult

Locations:
Sinuses
Head and neck
GU tract

30
Q

Smooth Muscle Tumors

Leimyoma (2) and Leimyosarcoma (3) Descriptions

A

Leimyoma:
Benign
Fibroids in uterus

Leimyosarcoma:
Malignant, metastasizes to lungs
Starts in extremities and retroperitoneal

31
Q

Synovial Sarcoma

Genetics (4) Staining Morphology (2) Clinical Features (2)

A

SS18-SSX1/SSX2/SSX4 fusion genes

(+) for keratins
(+) for epithelial markers

Primary tumors found adjacent to joints
Metastasis to lungs common

Neuro Muscular Psych Block 3 (7 decks)

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