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Flashcards in BS - Pathology Deck (74):
1

Rothmund-Thomson syndrome

 growth retardation, thin eyebrows and lashes, juvenile cataracts, sunlight sensitivity, hypogonadism, and teeth
abnormalities. RTS is associated with an increased risk for cancer, such as cutaneous epitheliomas (basal, squamous), gastric adenocarcinoma, fibrosarcoma, and osteosarcoma

2

What is the structure
outlined by the black arrow in Figure 85b?

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Birbeck granules (diagnostic of LCH)

 

LCH is also confirmed by CD1a
immunohistochemical staining.

3

cell membrane protein that conveys chemotherapeutic resistance to tumor cells

P-glycoproteins

protein produced from multidrug resistance gene 1 (MDR1)

4

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scurvy

5

What percent of patients initially diagnosed with classic, high-grade osteosarcoma of the extremity have visible evidence of pulmonary metastasis on CT of the chest?

10-20%

6

Tumor syndromes + eponyms (7)

Ollier's disease (enchondromatosis)

  • Mafucci syndrome - enchondromatosis + soft tissue hemangiomas

MHE

Polyostotic Fibrous dysplaisa

  • McCune Albright Syndrome: add cafe au lait spots in coast of Maine pattern, 
    endocrine abnormalities (precocious puberty), renal phosphate wasing due to FGF-23
  • Mazabraud's Syndrome: add intramuscular myxomas

NOF

  • Jaffe-Campanacci syndrome: add cafe au lait, mental retardation, heart, eyes, gonads involved

EOG

  • Hand-Schuller-Christian disease (HSC): chronic, disseminated form with bone and visceral lesions
     
  • Letterer-Siwe disease (LSD): fatal form that occurs in young children

7

Frassica differential SURFACE LESION (5)

osteochondroma

MHE

parosteal osteosarcoma

periosteal osteosarcoma

periosteal chondroma (rare)

 

8

Frassica differential TIBIA (4)

fibrous dysplasia

osteofibrous dysplasia

adamantinoma

osteomyelitis

 

9

Frassica differential MULTIPLE LESIONS IN YOUNG PERSON

MHE

Ollier's (enchondromatosis)

Fibrous dysplasia

EOG

Hemangioendothelioma of bone

10

Frassica differential ADULT BONE LESION

metastatic carcinoma

multiple myeloma

primary lymphoma of bone

chondrosarcoma

MFH (pleomorphic undifferentiated sarcoma)

11

Frassica differential HAND

enchondroma

GCT

ABC

giant cell reparative granuloma

12

Frassica differential EPIPHYSIS

GCT

clear cell chondrosarcoma

chondroblastoma

13

Frassica differential SYNOVIAL PROLIFERATIVE DISORDERS

synovial chondromatosis

gout

RA

PVNS

infection

14

Frassica differential SACRUM

chordoma

metastatic carcinoma

myeloma

lymphoma

15

Frassica differential BONE LESION YOUNG PERSON

osteosarcoma

Ewing's sarcoma

osteomyelitis

EOG

16

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fibrous dysplasia

17

most common distal met (distal to elbow/knee)

lung

18

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GCT

curretage and BG or cement

19

Treatment?

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CR + long leg cast

20

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gout

21

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pseudogout = pos birefringent

22

EOG treatment

observation

23

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chordoma

-wide resection alone

24

60 yo, CD20 positive

Staging and treatment

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bone marrow aspiration and biopsy are required for staging

cyclophosphamide, doxorubicin, prednisone and vincristine

25

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ABC

treatment is curretage and bone grafting

26

multiple myeloma on bone scan

COLD in 30%

27

16 yo

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chondroblastoma

extended intralesional curettage and bone grafting

histology:  chondroid matrix, chondroblasts in "cobblestone" or "chickenwire" pattern, giant cells

S100+ cells

28

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multiple myeloma

29

infant

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infantile fibrosarcoma

- unresectable lesion, treat with vincristine, actinomycin-D, and cyclophosphamide, followed by excision if there is an adequate decrease in the size of the lesion

30

internal hemipelvectomy types

1:  resection of ilium

2: resection of the periacetabular region

3: ischiopelvic region resection (no reconstruction required)

31

19 year old

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GCT

32

20 yo, painless mass

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ZEBRA (nodular fasciitis)

benign soft-tissue lesion that usually arises from the fascia and is often misdiagnosed as a sarcoma

 

Classic characteristics are short irregular bundles and fascicles dense reticulum network small amounts of mature collagen

marginal resection (recurrence rare)

33

13 yo

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EOG

- usually observation

34

14 yo mass, desmin+, vimentin+

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rhabdomyosarcoma

remember lymph node biopsy and BM biopsy

- kids get chemo + surgery, adults get radiation + surgery

REMEMBER (2:13) Pax3-FKHR with alveolar

35

small round blue cell tumors (6)

Ewing's

lymphoma

PNET

rhabdomyosarcoma

small cell lung

neuroblastoma

36

18 yo hand mass

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BPOP

hands and feet in young person

very cellular cartilage, a proliferation of bizarre fibroblasts, and disorganized bone with spindle shaped fibroblasts in the intertrabecular spaces, leading to confusion with

37

best immunohistochemistry marker for PNET family tumors

MIC-2 = CD99

high sensitivity and specificity

38

DEXA indications screening

over 65 women and postmenopausal and one risk factor

39

14 yo 

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chondromyxoid fibroma

- lytic eccentric metaphyseal lesion that is sharply demarcated from adjacent bone, can be locally agressive

- intralesional curretage and bone grafting (or PMMA), high recurrence rate

HISTOLOGY: lobules fibromyxoid tissue, STELLATE CELLS

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40

mets to lymph nodes (5)

synovial sarcoma

clear cell sarcoma

epithelioid sarcoma

angiosarcoma

rhabdomyosarcoma

41

35 yo wrist soft tissue mass

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clear cell sarcoma

- Histology is characteristic with solid nests and fascicles of tumor cells with clear cytoplasm and prominent nucleoli

 

remember (12:22) EWS-ATF1

42

soft tissue mass

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synovial sarcoma

HISTO:  classic biphasic pattern with spindle cells and epithelial cells

vimentin+, epithelial membrane antigen+

43

17 yo

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chondroblastoma

treatment: extended curettage and bone grafting

Resection of rare benign pulmonary metastasis

44

30 yo with radiating pain

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neurofibroma

NOT = Schwannoma

 

rich wire-like collagen fiber network +/- myxoid

45

molecular biology neurofibromatosis

Autosomal dominant, NF-1

codes for neurofibromin protein


negatively regulates Ras signaling pathway


neurofibromin deficiency (affected cells have inactive other copy NF-1) leads to increased Ras activity


affects Ras-dependent MAPK activity (essential osteoclast function and survival)

46

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osteofibrous dysplasia

- anterior cortex

- osteoblastic rimming

47

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epithelioid sarcoma

- most common soft‐tissue sarcoma in the hand and most commonly occurs in young adults. The tumors can be superficial and may become ulcerated. Deeper lesions are often attached to tendons, tendon sheaths, or fascial structures. These are usually minimally symptomatic.

- The biopsy specimen reveals the typical appearance of a nodular pattern with central necrosis. They can mimic a necrotizing granulomatous process. Usually there are chronic inflammatory cells along the margin of the tumor nodules

48

60 yo

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renal cell CA

remember preop embolization

49

15 yo

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chondroblastoma

(remember GCT is rare in children, typically metaphyseal)

 

50

Mirels scoring system

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51

15 yo

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ABC

-remember local recurrence in up to 25% and more common in children with open physes

52

33 yo

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Giant celltumor of tendon sheath

a lobulatedmassthatmay bemulticolored;typically yellow, brown,red, and gray.

it is most common on palmar surface of radial three digits near DIPJ

decreased signal intensity on both T1-and T2-weighted MR imaging

Histologically the lesion consists ofmultinucleated giant cells, polygonalmononuclear cells, and histiocytes
thatmay contain abundant hemosiderin orlipid

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53

80 yo knee pain

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popliteal cyst

-no further workup (characteristic lesion on MRI)

54

38 yo enlarging mass

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aggressive fibromatosis = desmoid tumor

  • well differentiated fibroblasts
  • uniform spindle cells with elongated nuclei and occasional mitoses
  • abundant collagen
  • tumor infiltrates adjacent tissues

100% positive for Estrogen receptor-beta , can use TAMOXIFEN in treatment (inoperable)

wide surgical resection with radiotherapy (reduces local recurrence)

55

35 yo

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giant celltumor of bone

  • txt: extended curettage plus a local adjuvant
  • purely lytic, eccentrically located and abuts the subchondral bone

56

33 yo

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adamantinoma

  • treatment is wide resection (NO chemo or rads)
  • high local recurrence rate
  • keratin +

57

treatment of known renal cell isolated met?

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WIDE resection and prosthetic reconstruction

 

Wide resection of isolated
renal cell carcinomametastasis, which presents distantto the nephrectomy, may improve long‐termsurvival.

58

14 yo.  Discuss treatment with and without path fx

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Nonoperative

  • ABC with acute fracture until fracture has healed. Once healed, treat as an ABC without fracture unless the fracture has led to spontaneous healing of the ABC

Operative

  • aggressive curettage and bone grafting
  • some use adjuvant treatment (phenol)
  • local recurrence in up to 25% and more common in children with open physes 

59

40 yo enlarging soft tissue mass

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Nodular fasciitis

  • may be mistaken for sarcoma

Keys are Young person (20-40)

Fast growing, related to trauma

HISTO:  plump, immature‐appearing fibroblasts; short, irregular bundles and fascicles and are adjacentto collagen and reticulin (green stain)

 

marginal resection

60

7 yo mass

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Hemangioma 

 

first line of treatment: observation, NSAIDS, vascular stockings, and activity modification, esp childhood lesions

consider embolization if sx

wide resection : lesions resistant to nonoperative management, HIGH RATE LOCAL RECURRENCE

61

16 yo

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syndovial sarcoma

(X:18)

Clues are young person (15-40)

Biphasic on path: spindle + epithelial cells

wide surgical resection with adjuvant radiotherapy

62

45 yo

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Dermatofibrosarcoma Protuberans

wide surgical resection

has a tendency to recurr locally

A characteristic histologic finding can be seen in the deepmargins of the tumor where itintricately interdigitates with normalfat

63

46 yo

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lipoma

marginal resection

64

GCT pulmonary met risk

 

Giant celltumor of bone has about a 2% risk of benign pulmonary metastasis in all cases and 6%
risk in recurrent cases

65

10 yo

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periosteal chondroma

- difficult dx, rare

- CLUES:  young person (10-20 yo)

- Surface lesion, saucerization of underlying bone

- benign cartilage histology

marginal excision including underlying cortex

66

23 yo

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osteoid osteoma

 

nidus contains uniform osteoid seams of immature osteoid trabeculae (woven bone) with a sharp border of osteoblastic rimming

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incidental finding

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Melorheostosisis

 

hyperostosis may be on the periosteal or endostealsurface ofthe bone

68

17 yo boy

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osteoblastoma

lytic or mixed lytic-blastic lesion with radiolucent nidus > 2cm

osteoblastic rimming

curettage or marginal excision with bone grafting

69

60 yo shoulder pain/snapping

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Elastofibroma

70

40 yo M

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synovial sarcoma

biphasic, young, distal extremity

71

28 yo F tender shoulder mass

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Schwannoma

MRI features fairly nonspecific, fairly homogeneous appearance with a high water content and
often fusiformshape.

Classically,the histology shows alternating Antoni A (dense spindle cell region) areas and Antoni B (loosemyxoid tissue) areas

uniformintense immunostaining with S‐100 protein

72

AJCC Bone Sarcoma

I and II same as Enneking

III skip lesions

IV A/B are based on mets to lung/other

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Enneking benign bone lesion

1 - latent

2- active

3- aggressive

(arabic numerals)

74

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giant cell tumor of tendon sheath

- remember hemosiderin (gross appearance of tumor and dark on T1/T2)

- giant cells

- hand/foot