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Neuromusculoskeletal & Psychiatry II Exam #3 > Pathology of Bones, Joints and Soft Tissue Part 2 > Flashcards

Pathology of Bones, Joints and Soft Tissue Part 2 Flashcards

1
Q

What is the most common benign bone tumor?

A

Osteochondroma (AKA Extostis)

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2
Q

How do Osteochondromas (Exostis) present?

Which sex is affected more often?

What genes are involved?

Where does the tumor grow in the bone?

A

Solitary tumors in adulthood.

Men 3x > women.

EXT1 and EXT2 in sporadic osteochondromas, which encode heparin sulfate glycosaminoglycans.

At the metaphysis near the growth plates of tubular bone.

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3
Q

What is Multiple Hereditary Exostis?

A

An AD disorder that progresses to chondrosarcoma 5-20% of the time.

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4
Q

What is a gross/morphologic feature of osteochondromas?

A

The medullary cavity is continuous into the tumor.

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5
Q

What are Chondromas/Enchondromas?

What age do they occur?

Where do they occur?

What genes are involved?

A

Benign hyaline cartilage, solitary metaphyseal lesions on hands and feet.

Can be within medullary cavity (enchondroma) or on the surface (subperiosteal or juxtacortical).

IDH1 and IDH2.

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6
Q

What is Ollier syndrome?

What is Maffucci syndrome?

A

Ollier syndrome: multiple enchondromas.

Maffucci syndrome: multiple enchondromas + angiomas which increase the risk of chondrosarcoma and other malignancies.

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7
Q

What is the gross appearance of an enchondroma?

A

< 3 cm. gray-blue and translucent.
*Well-circumscribed nodules of hyaline cartilage containing benign chondrocytes.
they periphery may ossify and the center may calcify and infarct.

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8
Q

How are endochondromas in Ollier and Maffucci syndrome different that regular endochondromas?

A

They have increased cellularity and atypia.

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9
Q

What is Chondrosarcoma?

What sex is more common? What age does it present?

What does it affect most?

How does it present?
How does it metastasize? Where does it metastasize?

A

The second most common malignant matrix producing tumor of bone.

Males are 2x more common; usually presents in the 40s.

Axial skeleton (pelvis, shoulders, ribs).

Presents as a locally invasive painful enlarging mass.
It metastasizes hematogenously, mostly to the lungs.

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10
Q

Which tumor has a direct correlation between grade and behavior?

A

Chondrosarcoma

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11
Q

What is the prognosis of chondrosarcoma?

A

80-90% survival at 5 years for for grade 1 vs. 43% at grade 3.

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12
Q

What is seen on XR in chondrosarcoma?

A

Calcified matrix that appears as a foci of flocculent densities.

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13
Q

How do chondrosarcomas develop?

Multiple osteochondroma syndrome has mutations in…

Chondromatosis-related and sporadic chondrosarcomas have mutations in…

A

85% develop de novo, remainder occur in pre-existing osteochondroma or enchondromas.

EXT gene.

+/- IDH1 or IDH2.

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14
Q

What is the presentation of Osteiod osteoma?

What causes it?

At what sex and age does it onset?

Where does it occur?

What is the size of it by definition?

What are the histologic and XR findings?

What is the treatment?

A

Painful, usually worse at night, that classically responds to aspirin and NSAIDs.

Excess PGE2 production by osteoblasts.

Young men in their teens and 20s.

Appendicular skeleton - 50% femur or tibia.

< 2 cm. in greatest dimension (> 2 cm. = osteoblastoma).

Histology: central nidus of translucents woven bone surrounded by a rim of osteoblasts.
XR: thick rind of reactive cortical bone.

Radiofrequent ablation.

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15
Q

What is the definition of Osteoblastoma by size?

What is involved classically?

There is NO ________, as compared to osteoid osteoma.

What is the presentation?

What is the treatment?

Does it become malignant?

A

> 2 cm.

Involves the posterior spine.

NO bony reaction.

Achy pain that is non-responsive to aspirin/NSAIDs.

Curettage or excision en bloc.

Rarely.

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16
Q

What is the most common primary malignant tumor of bone?

A

Osteosarcoma

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17
Q

What is the presentation of Osteosarcoma?

What ages does it most often present? (bimodal distribution)

What genes increase your risk for it mainly? (2)

What is the findings on XR?

Where does it spread?

What is the treatment and prognosis?

A

A painful enlarging mass.

75% < 20 y/o males around the knee + 2nd peak in older males with Paget’s diseaseor prior radiation (secondary osteosarcoma).

Rb gene (70%)
TP53 gene (Li-Fraumani syndrome)

Mixed lytic and blastic mass -> Codman triangle (elevation of periosteum).

Early to the lung hematogenously.

Surgery or chemo with 60-70% survival at 5 years.

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18
Q

What is seen on XR in Ewing sarcoma?

A

“Lamellated and focally interrupted periosteal reaction.”

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19
Q

What age and ethnicity is at a greater risk for Ewing Sarcoma Family Tumors (ESFT)?

What kind of tumor is it?
What kind of tumor is it with neural differentiation?

How does it present?

What is seen on XR?

What bones (and where?) does it tend to affect?

A

80% < 20 y/o Caucasians (2nd most common bone sarcoma in kids).

Small blue round cell tumor.
With neural differentiation -> primitive neuroectodermal tumor (PNET).

Painful enlarging mass; frequently tender, warm and swollen (fever and high ESR) mimics infection!

XR: periosteal reaction -> reactive bone in “onion-skin” fashion.

Diaphysis of long bones, especially the femur and flat bones that arises in medullary cavity and invades the cortex.

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20
Q

What is the prognosis of ESFT?

What chromosomal abnormality is seen in 85% of the tumors?

What is the most important prognostic finding?

A

75% survival at 5 years with surgery, chemo and radiation.

t(11;22)(q24:q12) - fusion gene EWS-FL11.

The amount of chemo-induced necrosis!

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21
Q

“Onion skin” on XR =

Codman on XR =

A

“Onion skin” on XR = Ewing sarcoma

Codman on XR = Osteosarcoma

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22
Q

What is Fibrous Dysplasia?

How many lesions usually occur?

When do they present?

What bones are involved?

What is seen on XR?

A

Benign proliferation of fibrous tissue and bone that do not mature; possibly a developmental anomaly.

70% are monostotic - asymptomatic.
27% are polyostotic - crippling deformities.

Early adolescence; M = F.

1/3 ribs, 1/3 femur/tibia, 1/3 craniofacial bones.

Groundglass appearance with well-defined margins on XR.

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23
Q

What diseases/syndrome does Fibrous Dysplasia occur? (2)

A

McCune-Albright disease: bone lesions, pigmented skin lesions (Cafe au lait spots).

  • precocious puberty in females.
  • GNAS mutations.

Mazabraud syndrome: fubrous dysplasia (polystotic) and multiple skeletal deformities in childhood.
-soft tissue myxomas.

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24
Q

“Chinese characters” (curvilinear trabeculae) on histology =

A

Fibrous dysplasia

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25
Q

What is a Giant Cell tumor of bone made of?

What is the age of onset?

Where does it occur within the bone?

What are the symptoms?

What is the prognosis?

A

An osteoclastoma multinucleated osteoclast-type giant cells - must consider brown tumor of hyperparathyroidism.

20-40 y/o.

In the epiphyses, may extend into the metaphyses.

Arthritis-like symptoms.

It is benign, but locally aggressive.It tends to recur after curettage, occasional with metastasize and be fatal.

26
Q

How do bone metastases spread?

What malignancies are commonly implicated in adults vs. kids?

They tend to be…

What do they affect most?

Prognosis?

A

Direct extension, lymphatic/hematogenously, intraspinal seeding.

Adults: prostate, breast, kidney and lung.
Kids: neuroblastoma, Wilms tumor, osteosarcoma, Ewing, Rhabdomyosarcoma.

They tend to be multifocal.

Axial skeleton and small bones of hands and feet.

Poor prognosis.

27
Q

What primary malignancies have lytic vs. blastic features on XR?

A

Lytic (bone destroying): kidney, lung, GI, melanoma.

Blastic (bone forming): prostatic adenocarcinoma.

28
Q

Solid joints =

Cavitated joints =

A

Solid joints = non-synovial (synarthroses) - no joint space.

Cavitated joints = synovial.

29
Q

What collagen type is seen in hyaline cartilage?

A

Type II collagen

30
Q

OA is a disease of…

What symptoms are classic?

A

Cartilage - “wear and tear”.

Evening stiffness, crepitus, poor ROM and worse with use.

31
Q

Bouchard’s nodes =

Heberden nodes =

A

Bouchard’s nodes = OA at PIP joint

Heberden nodes = OA at DIP joint

32
Q

What are the molecular changes in OA?

What is “joint mice”?

What is eburnation?

What are subchondral cysts?

What forms as a result of this?

A

Water content of matrix increases and concentration of proteoglycans decreases and causes cracks in the matrix.

“Joint mice” - hunks of cartilage sloughed off the joint.

Eburnation - subchondral bone exposed and rubbed smooth.

Cysts that develop as a result of trying to repair microfractures/stress in OA.

Osteophytes (bone spurs) - nerve root compression and radicular pain and neurological deficits.

33
Q

What age of onset is most common in RA?

It is characterized as destruction of…

What is the distribution?

What is a major symptom/feature?

A

2nd-4th decades; women 3x more common.

Articular cartilage -> ankylosis.

Symmetrical distribution of small joints; swollen, warm and painful.

Morning stiffness, which may be > 1 hr. in active disease.

34
Q

What lab findings are seen in 80% of RA?

A

+ Rheumatoid factor (RF)

+ anti-cyclic citrulinated peptide (CCP) antibodies

35
Q

Features of RA:

Boutonniere deformity

Swan-neck deformity

Ulnar deviation of fingers

Radial deviation of wrist

A

Boutonniere deformity - hyperextension of DIP, flexion of PIP.

Swan-neck deformity - hyperextension of PIP, flexion of DIP.

Ulnar deviation of fingers - decreased joint space causing periarticular bony erosions.

Radial deviation of wrist

36
Q

What changes are seen in joints with RA? (3)

A

Pannus: hyperplastic synovium.

Synovial hypertrophy w/ villi.

Lymphoid aggregates.

37
Q

What is a Rheumaoid Nodule?

What other diseases may it be seen in? (2)

A

Extra-articular lesions that occur on extensor surfaces at pressure points; may have central necrosis and palisading histocytes.

SLE and Scleroderma.

38
Q

OA vs. RA

Which is worse with rest?

Which has evening stiffness?

A

RA is worse with rest; OA is worse with activity.

OA has evening stiffness; RA has morning stiffness.

39
Q

+ HLA-B27

A

Ankyosing spondylitis

40
Q

Triad of symptoms in Reactive Arthritis (3)

What gene is positive?

What patients are most common?

What tends to precipitate this process?

What extra-articular findings may occur? (3)

A

Arthritis, urethritis or cervicitis, conjunctivitis.

80% have + HLA-B27.

Men in 20s-30s; HIV+.

Prior GI or GU infection - symptoms onset several weeks later.

Conjunctivitis, cardiac dconduction abnormalities, aortic regurgitation.

41
Q

What bugs cause Enteritis Associated Arthritis? (4)

What stimulates the immunological response?

What is the presentation?

A

Yersinia, Salmonella, Shigella, Campylobacter.

LPS of outer cell membranes stimulate the response.

Sudden onset of ankle and knee pain that lasts about 1 year.

42
Q

What is affected in Psoriatic Arthritis (PA)?

What genes are implicated? (2)

What ages are most common?

What deformity is classic?

A

Peripheral joints (hands and feet) and axial joints, ligaments and tendons.

+ HLA-B27 and HLA-Cw6.

“Pencil in cup” deformity - DIPs affected asymmetrically.
Symptoms may have frequent remissions.

43
Q

What disease is common in onycholysis?

A

Psoriatic arthritis

44
Q

How do infections spread to cause infectious arthritis?

What is the progression of the infection?

A

Can be via hematogenous, direct inoculation or contiguous spread from soft tissue abscess or osteomyelitis.

Rapid joint destruction leading to permanent deformities.

45
Q

What are symptoms of Suppurative Arthritis?

A

Acutely painful and swollen joint with restricted ROM.

46
Q

Suppurative arthritis in < 2 y/o is due to:

Older kids and adults:
Who is at risk for disseminated GC?

Other than these, what can cause this infection?

A

H. flu.

S. aureus and Gonococcal (sexually active women).
Patients with MAC deficiency.

Mycobacterium, Lyme disease and viruses (HIV, HBV, HCV, EBV, parvo B19 and Rubella).

47
Q

Most non-gonoccocal cases of suppurative arthritis affect what?

A

Knee mostly

48
Q

What is Gout?

What is the lab value associated with it?

What are risk factors?

A

Transient attacks of acute arthritis initiated by crystallization of monosodium urate (MSU) within and around joints.

MSU > 6.8 mg/dl.

Age (after 20s-30s)
Genetics
EtOH
Obesity
Drugs (thiazides)
Lead toxicity
49
Q

What cytokine is vital in the development of Gout?

A

IL-1B

50
Q

What is pathognomonic for Gout of histology?

A

Gouty tophus

51
Q

What age does Calcium Pyrophosphate Crystal Deposition (CPPD) onset? (AKA pseudogout/chondrocalcinosis)

How can it develop?

What is seen on histology?

How are these crystals different than Gout?

A

> 50 y/o and increases with age.

Sporadic, hereditary or secondary (previous joint damage).

Crystals forming chlaky, white friable deposits.
Oval blue-purple aggregates.

These crystals are positively birefringement; Gout is negative birefringement.

52
Q

Ganglion cyst occurs where?

How big?

What are features of the cyst?

A

In the joint capsule or tendon sheath.

1-1.5 cm. cyst.

Cystic or myxoid degeneration leads to the cyst; NO cell wall!

53
Q

What is a synovial cyst?

What is a Baker cyst?

A

Herniation of synovium through the joint capsule.

A cyst occurring in the popliteal space, associated w/ RA.

54
Q

Where do most soft tissue tumors arise?

Which class of genes are commonly associated?

A

Extremities - mostly the thigh.

Tumor suppressor genes (NF1, Gardner, Li-Fraumeni, O-W-R).

55
Q

What is the most common soft tissue of adults?

Where do these occur most?

What is seen on histology?

A

Lipoma - soft, moveable and painless.

Usually in subcutis, but may happen in deep soft tissue.

Encapsulated mass of normal appearing adipose.

56
Q

What ages are most common in the development of Liposarcoma?

Where do they occur?

What chromosomal change is associated?

What is the prognosis?

What histological variant is most common?

A

50-60 y/o; common malignant ST tumor.

Deep soft tissue of proximal extremities and retroperitoneum.

12q13-q15 and t(12;16); p53 inhibitor.

They recur locally and repeatedly unless completely excised.

Myxoid (intermediate varant)

57
Q

What is Nodular Fascitis?

What fusion gene is associated?

What is the progression?

A

Self-limited fibroblastic and myofibroblastic proliferation in the UE of young adults.

t(17;22) -> MYH9-USP6 fusion gene.

Regresses spontaneously.

58
Q

What sex is most common for Fibromatoses?
What are the following?

Palmer (Depuytren contracture)

Plantar fibromatosis

Penile (Peyronie disease)

A

M > F.

Palmer (Depuytren contracture): slowly progressive flexion contracture of 4th and 5th digits.

Plantar fibromatosis: young patients, UL and no contractures.

Penile (Peyronie disease):abnormal curvature and possible urethral constriction.

59
Q

What is a Desmoid tumor?

What age and sex is most common?

What signaling pathway is implicated?

What syndrome is associated?

A

Large, infiltrative tumos that frequently recur and are painful.

Teenage to 30s, mostly women.

Increased Wnt pathway.

Gardner syndrome.

60
Q

What are the 3 subtyoes of Rhabdomosarcoma?

In kids, what areas are affected most?

A

Alveolar (20%): kids; fusions of FOX01 to PAX3 or PAX7.

Embryonal (60%): kids; rhabdomyoblasts and sarcoma botryoides (cambium layer).

Pleomorphic (20%): adults.

Sinuses, head/neck and GU tract.

61
Q

Leiomoma is associated with: (2)

A

Hereditary Leiomyomatosis and RCC syndrome

62
Q

Synovial sarcomas are found where?

What ages are common?

What markers are positive?

What is the prognosis?

A

Adjacent to joint spaces.

20s-40s.

+ for keratins and epithelial markers.

5 year survival is 25-60% with metastases to lung common.

Neuromusculoskeletal & Psychiatry II Exam #3 (12 decks)

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