BS - Pathology Flashcards
1
Q
Rothmund-Thomson syndrome
A
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
Q
What is the structure
outlined by the black arrow in Figure 85b?
A
Birbeck granules (diagnostic of LCH)
LCH is also confirmed by CD1a
immunohistochemical staining.
3
Q
cell membrane protein that conveys chemotherapeutic resistance to tumor cells
A
P-glycoproteins
protein produced from multidrug resistance gene 1 (MDR1)
4
Q
A
scurvy
5
Q
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?
A
10-20%
6
Q
Tumor syndromes + eponyms (7)
A
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
Q
Frassica differential SURFACE LESION (5)
A
osteochondroma
MHE
parosteal osteosarcoma
periosteal osteosarcoma
periosteal chondroma (rare)
8
Q
Frassica differential TIBIA (4)
A
fibrous dysplasia
osteofibrous dysplasia
adamantinoma
osteomyelitis
9
Q
Frassica differential MULTIPLE LESIONS IN YOUNG PERSON
A
MHE
Ollier’s (enchondromatosis)
Fibrous dysplasia
EOG
Hemangioendothelioma of bone
10
Q
Frassica differential ADULT BONE LESION
A
metastatic carcinoma
multiple myeloma
primary lymphoma of bone
chondrosarcoma
MFH (pleomorphic undifferentiated sarcoma)
11
Q
Frassica differential HAND
A
enchondroma
GCT
ABC
giant cell reparative granuloma
12
Q
Frassica differential EPIPHYSIS
A
GCT
clear cell chondrosarcoma
chondroblastoma
13
Q
Frassica differential SYNOVIAL PROLIFERATIVE DISORDERS
A
synovial chondromatosis
gout
RA
PVNS
infection
14
Q
Frassica differential SACRUM
A
chordoma
metastatic carcinoma
myeloma
lymphoma
15
Q
Frassica differential BONE LESION YOUNG PERSON
A
osteosarcoma
Ewing’s sarcoma
osteomyelitis
EOG
16
Q
A
fibrous dysplasia
17
Q
most common distal met (distal to elbow/knee)
A
lung
18
Q
A
GCT
curretage and BG or cement
19
Q
Treatment?
A
CR + long leg cast
20
Q
A
gout
21
Q
A
pseudogout = pos birefringent
22
Q
EOG treatment
A
observation
23
Q
A
chordoma
-wide resection alone
24
Q
60 yo, CD20 positive
Staging and treatment
A
bone marrow aspiration and biopsy are required for staging
cyclophosphamide, doxorubicin, prednisone and vincristine
25
ABC
treatment is curretage and bone grafting
26
multiple myeloma on bone scan
COLD in 30%
27
16 yo
chondroblastoma
extended intralesional curettage and bone grafting
histology: chondroid matrix, chondroblasts in "cobblestone" or "chickenwire" pattern, giant cells
S100+ cells
28
multiple myeloma
29
infant
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
GCT
32
20 yo, painless mass
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
EOG
## Footnote
- usually observation
34
14 yo mass, desmin+, vimentin+
rhabdomyosarcoma
## Footnote
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
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
chondromyxoid fibroma
## Footnote
- 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
40
mets to lymph nodes (5)
synovial sarcoma
clear cell sarcoma
epithelioid sarcoma
angiosarcoma
rhabdomyosarcoma
41
35 yo wrist soft tissue mass
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
synovial sarcoma
HISTO: classic biphasic pattern with spindle cells and epithelial cells
vimentin+, epithelial membrane antigen+
43
17 yo
chondroblastoma
treatment: extended curettage and bone grafting
Resection of rare benign pulmonary metastasis
44
30 yo with radiating pain
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
osteofibrous dysplasia
- anterior cortex
- osteoblastic rimming
47
epithelioid sarcoma
## Footnote
- 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
renal cell CA
remember preop embolization
49
15 yo
chondroblastoma
(remember GCT is rare in children, typically metaphyseal)
50
Mirels scoring system
51
15 yo
ABC
-remember local recurrence in up to 25% and more common in children with open physes
52
33 yo
Giant celltumor of tendon sheath
## Footnote
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
53
80 yo knee pain
popliteal cyst
-no further workup (characteristic lesion on MRI)
54
38 yo enlarging mass
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
giant celltumor of bone
* txt: extended curettage plus a local adjuvant
* purely lytic, eccentrically located and abuts the subchondral bone
56
33 yo
adamantinoma
* treatment is wide resection (NO chemo or rads)
* high local recurrence rate
* keratin +
57
treatment of known renal cell isolated met?
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
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
_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
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
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
Dermatofibrosarcoma Protuberans
## Footnote
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
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
periosteal chondroma
## Footnote
- 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
osteoid osteoma
nidus contains uniform osteoid seams of immature osteoid trabeculae (woven bone) with a sharp border of osteoblastic rimming
67
incidental finding
Melorheostosisis
hyperostosis may be on the periosteal or endostealsurface ofthe bone
68
17 yo boy
osteoblastoma
## Footnote
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
Elastofibroma
70
40 yo M
synovial sarcoma
biphasic, young, distal extremity
71
28 yo F tender shoulder mass
Schwannoma
## Footnote
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
73
Enneking benign bone lesion
1 - latent
2- active
3- aggressive
(arabic numerals)
74
giant cell tumor of tendon sheath
- remember hemosiderin (gross appearance of tumor and dark on T1/T2)
- giant cells
- hand/foot
