12: Soft tissue Flashcards
1
Q
surface of giant cell fibroma
A
papillary
2
Q
bilateral giant cell fibromas behind MD canines – treatment?
A
retrocuspid papilla, disappears with age
3
Q
retrocuspid papilla histo
A
giant cell fibroma
4
Q
frenal tag
A
fibrous hyperplasia, most frequent on maxillary labial frenum
5
Q
epulis fissuratum
A
hyperplasia of fibrous tissue with ill-fitting denture
6
Q
fibrous hyperplasia with ill-fitting denture
A
epulis fissuratum
7
Q
inflammatory papillary hyperplasia
A
usually under denture; mouth breathers or deep palatal vault
8
Q
palate of mouth breathers
A
inflammatory papillary hyperplasia
9
Q
benign fibrous histiocytoma histo
A
dermatofibroma
10
Q
dermatofibroma in mouth
A
benign fibrous histiocytoma
11
Q
other names for benign fibrous histiocytoma
A
sclerosing hemangioma, fibroxanthoma, nodular subepidermal ffibrosis
12
Q
most common site of benign fibrous histiocytoma
A
skin of extremities
13
Q
histo of benign fibrous histiocytoma
A
short intersecting fascicles: storiform (straw mat) or cartwheel pattern; fibroblasts, histiocytes, Touton giant cells, xanthoma cells, lymphocytes, HPC like areas
14
Q
short intersecting fascicles: storiform (straw mat) or cartwheel pattern
A
benign fibrous histiocytoma
15
Q
benign fibrous histiocytoma IHC
A
factor XIIIa+, Cd34 - (vs DFSP XIIIa and CD34+)
16
Q
variants of benign fibrous histiocytoma
A
cellular (higher recurrence) and eputhelioid (mimics Spitz nevus)
17
Q
which benign fibrous histiocytoma has higher recurrence
A
cellular
18
Q
touton giant cells look
A
“wreath-like” nuclei around scalloped edge of the cell like a florette
19
Q
“wreath-like” nuclei around scalloped edge of the cell like a florette
A
touton giant cells
20
Q
juvenile xanthogranuloma histo
A
sheets of histiocytes (xantho), touton giant cells, +/- eos
21
Q
juvenile xanthogranuloma IHC
A
CD68, antitrypsin + but S100 and CD1a negative
22
Q
reticulohistiocytoma aka
A
solitary epithlioid histiocytoma
23
Q
reticulohistiocytoma IHC
A
CD163+, CD68+
24
Q
reticulohistiocytoma histo
A
large epithelioid histiocytes with glassy cytoplasm, lymphocytes, neutrophils, giant cells
25
generalized reticulohistiocytoma
multiple skin lesions and granulomatous polyarthritis
26
xanthoma histo
localized collection of tissue histiocytes containing lipid (not a true tumor)
27
localized collection of tissue histiocytes containing lipid
xanthoma
28
xanthoma systemic conditions
most primary and some secondary hyperlipoproteinemias
29
xanthomas of eyelid
xanthelasma
30
lipid levels in xanthelasma
normal
31
fibrous proliferations intermediate between benign and fibrosarc
fibromatosis
32
fibromatosis entities
desmoid tumor, juvenile aggressive fibromatosis, extra-abdominal fibromatosis
33
fibromatosis in bone
desmoplastic fibroma
34
desmoplastic fibroma
fibromatosis in bone
35
conditions with higher risj for fibromatosis
familial adenomatous polyposis and gardner
36
fibromatosis histo
spindle cells infiltrate muscle and fat; cells run parallel to vessels and collagen runs alongsid each cell; abindant collagen )little or no cell-too-cell contact); no hyperchromasia; multinucleated giant cells: atrophic skeletal muscle remnants at periphery of lesion
37
fibromatosis IHC
vimentin, SMA, MSA, beta catenin
38
sternocleidomastoid tumor
fibromatosis colli, most common type, few weeks after birth
39
most common type of fibromatosis
sternocleidomastoid tumor aka fibromatosis colli
40
juvenile hyaline fibromatosis
hereditary; muliple cutaneous papiles/nodules/masses, gingival hyperplasia, joint contracures, osteolytic defects
41
juvenile hyaline fibromatosis histo
cords of spindle cells embedded in homogeneous eosinophilic matrix
42
prliferation of myofibroblasts
myofibroma/myofibromatosis
43
multicentric myofibromatosis
neonates/infants with tumors of skin, bone, visceral organs
44
histo look of myofibroma
may appear biphasic: darker central area and lighter pripheral areas; nofules or whorls of elongated spindle cells, myxoid stroma, staghprn vessels, chondroid areas; may have smooth muslce/fibroblastc features, HPC-like pattern, local infiltration
45
myofibroma IHC
vimentin, SMA, PTAH +; desmin S100 -
46
nodular fasciitis demographic
adults 20-40yo, classically rapid growth, 50% with pain
47
most nodular fasciitis location
upper extremities, trunk, head and neck
48
nodular fasciitis histo
red spindle cells in fascicles and bundles; feathery "tissue culture" appearance; keloid-like collagen fibers; inflammation, hemosiderin, extravasated RBC
49
feathery "tissue culture" look
nodular fasciitis
50
nodular fasciitic IHC
KP-1+
51
keloid natural history and predilection
abnormal wound healing in genetically predisposed patients; dark-skinned; hypertrophic scar confined to original wound site
52
systemic associations for keloid
Ehlers-Danlos, scleroderma, Rubinstein-Taybi syndrome
53
keloid histo
haphazard, thick, glassy, deeply eosinophilic collagen fibers
54
inflammatory myofibroblastic tumor location and cause
most common in lung, overexpression of ALK kinase
55
inflammatory myofibroblastic tumor IHC
MSA, SMA, desmin, ALK-1
56
oral focal mucinosis cause
overproduction of hyaluronic acid by fibroblasts (Alcian blue+)
57
oral focal mucinosis location and predilection
75% gingiva, young females
58
locations for PG
75% gingiva (poor hygiene), also tongue, cheek, lips (trauma related)
59
PG in pregnancy cause and name
increased progesterone/estrogen, granuloma gravidarum
60
PG in extraction socket
epulis granulomatosa
61
epulis granulomatosa
PG in extraction socket
62
peripheral giant cell granuloma clinical look and location
blue-purple mass, exclusive to gingiva or alveolar ridge
63
peripheral ossifying fibroma look and location, demographic
pink or red, exclusively gingiva, more anterior maxilla, teenagers (females), incisor-cuspid area
64
most common mesenchymal neoplasm
lipoma
65
location for lpoma
trunk and proximal extremities, more in obese people
66
metaplasia in lipoma
central ctlg or osseous
67
highest recurrence rate among lipomas
intramuscular 2/2 infiltrative growth
68
histo look of spindle cell lipoma
bland spindle cells, myxoid changes, ropy collagen bundles, scattered mast cells, mature adipocytes
69
IHC lipoma
CD34+
70
lipoblastoma histo
lipoblasts and adipocyte with fibrous connective tissue septa
71
lipoblasts and adipocyte with fibrous connective tissue septa
lipoblastoma
72
age for lipoblastoma
< 3 yo (almost exclusively childrins)
73
hibernoma origin
vestigial remnants of brown fat
74
which neoplasm arises from vestigial remnants of brown fat
hibernoma
75
hibernoma histo look
multivacuolated cells (brown fat, like in hibernating animals)
76
hibernoma IHC
S100+, Cd34 --
77
hibernoma molecular
11q13 and 11q21 rearrangement
78
11q13 and 11q21 rearrangement neoplasm
hibernoma
79
traumatic neuroma location
mental foramen, tongue, lower lip, often with trauma
80
special complication of parotid surgeries
traumatic neuroma of greater auricular nerve in 10%
81
pain in traumatic neuroma
25-33%
82
palisaded encapsulated neuroma aka
solitary circumscribed neuroma
83
solitary circumscribed neuroma aka
palisaded encapsulated neuroma
84
palisaded encapsulated neuroma location
90% face (nose and cheeks)
85
special artifacts for palisaded encapsulated neuroma
cracking and peeling
86
cracking and peeling characteristics artifacts of what
palisaded encapsulated neuroma
87
neurilemoma syndrome assoc
NF2
88
NF2 assoc with what mesenchymal neoplasm
neurilemoma
89
NF2 inheritance, gene, neoplasms
AD, MERLIN (schwannomin) gene mutation (chr 22). b/l neurilemomas of vestibular nerve (acoustic neuromas), neurilemmomas of peripheral nerves, meningiomas and ependymomas of CNS
90
multiple neurilemomas but no vestibular tumors
schwannomatosis
91
schwannomatosis vs NF2
NF2 acoustic neuromas
92
carney syndrome
psammomatous melanocytic schwannomas
93
psammomatous melanocytic schwannomas syndrome
carney syndrome
94
verocay bodies
reduplicated basement membrane and cytoplasmic processes
95
reduplicated basement membrane and cytoplasmic processes
verocay bodies
96
ancient schwannoma features
hemorrhage, vessel hyalinization, pleomorphic cells, verocay bodies, xanthomatous changes, cysts, fibrosis and calcification
97
schwannoma vs neurofibroma IHC
S100 stronger in schwannoma
98
neurites in schwannoma vs neurofibroma
absent in schwannoma, present in NF
99
most common peripheral nerve neoplasm
neurofibroma
100
special cell in neurofibromas
mast cells
101
many mast cells in spindly lesion
prolly neurofibroma
102
histo look of NF
shredded carrot
103
shredded carrot
NF
104
NF IHC
S100 scattered
105
neurofibromatosis aka
von Recklinghausen disease of the skin
106
von Recklinghausen disease of the skin aka
neurofibromatosis
107
NF1 inheritance, gene, chromosome, product
50% AD, 50% new mutation; NF1 gene on chr 17, neurofibromin protein
108
NF1 vs NF2 gene, chromosome, product
NF1: NF1 gene on chr 17, neurofibromin; NF2: MERLIN (schwannomin) gene mutation (chr 22)
109
large baggy neurofibromas
elephantiasus neuromatosa in NF1
110
elephantiasus neuromatosa
large baggy neurofibromas in NF1
111
pathognomonic feel of neurofibroma in NF1
plexiform, bag of worms, especially in trunk
112
bag of worms skin lesion esp trunk
pathognomonic plexiform feel of neurofibroma in NF1
113
NF Dx
cafe au ait (6+), axillary freckling (Crowe's sign), brown pigmented spots on the iris (Lisch nodules), optic glioma
114
optic glioma
NF1
115
axillary freckling in NF1
Crowe's sign
116
Crowe's sign
axillary freckling in NF1
117
Lisch nodules
brown pigmented spots on the iris in NF1
118
brown pigmented iris spots
Lisch nodules, NF1
119
most common oral finding in NF1
enlarged fungiform papillae in up to 50% of patients
120
most common medical problem in NF1
HTN
121
malignant transformation in NF
~5% of NF transform to malignant peripheral nerve sheath tumors; others: RMS, leukemia, pheochromocytoma, Wilms tumor
122
order of neural lesions occurrence
neuroma > schwannoma, NF > ancient schwannoma > plexiform NF
123
ganglioneuroma age location
average age 6y, most common in mediastinum
124
GI ganglioneuroma look and association
polypoid; associated with Cowden, tuberous sclerosis, juvenile polyposis, NF1, MEN2B
125
ganglioneuroma histo
scattered clusters of ganglion cells in a background of schwann cell bundles
126
ganglion cell look
large pink cytoplasm and 1-3 nuclei
127
neurothekeoma aka
nerve sheath myxoma
128
never sheath myxoma aja
neurothekeoma
129
neurothekeoma histo look
lobules of cells and myxoid stroma (hyaluronic acid or sulfated acid)
130
neurothekeoma IHC
S100 and PGP9.5+
131
cellular neurothekeoma
cellular, nuclear atypia, mitosis, extends into fat/mm/vessels
132
neurothekeoma ddx and how
focal mucinosis and myxoid neurofibroma -- lobulation is key difference
133
perineurioma differentiation
most cells show perineural differentiation
134
perineurioma forms
intraneural, extraneural )soft tissue), sclerosing, reticaulr
135
intraneural perineurioma histo look
tiny "onion bulbs", EMA+, S100+
136
ossifying fibromyxoid tumor of soft parts origin and location
possibly neuroectodermal, 70% in extrmities
137
ossifying fibromyxoid tumor of soft parts histo
lobules of uniform, round to fusiform cells in nests and cords, fibromyxoid stroma, incomplete shell of metaplastic hypocellular lamellar bone
138
ossifying fibromyxoid tumor of soft parts IHC
70% are vimentin and S100+; also desmin, Leu7, NSE, GFAP, SMA
139
granular cell tumor location
tongue > cheek, rarely parotid
140
granular cell tumor multiplicity demographic
black
141
granular cell tumor IHC
S100+ (schwann origin??), NK1C3, CD68, Leu7, NSE, MBP; GFAP and neurofilament --
142
examples of epulides
1- Giant cell epulis = PGCG
2- Ossifying fibroid epulis = peripheral ossifying fibroma
3- Congenital epulis
143
polyp on hard palate under denture
Leaf –like or fibroepithelial polyp
144
Granuloma gravidarum
Pyogenic granuloma
145
Proteus syndrome aka, presentation
(Wiedmann Syndrome ): overgrowth and atypical bone development, often
accompanied by tumors over half the body. Mistaken for NF1
146
other names for granular cell tumor
=Granular myoblastoma= Granular cell schwannoma
147
PHACES syndrome
```
Large segmental cervicofacial hemangioma
Posterior fossa brain anomaly (Dandy – walker malformation )
Hemangioma
Arterial anomalies
Cardiac defects
Eye anomalies
Sternal cleft or supraumbilical raphe
```
148
Kasabach-Merritt phenomenon:
hemangioma thrombocytopenia syndrome
Tufted hemangioma
Kaposiform hemangioendothelioma
149
hemangioma thrombocytopenia syndrome
Kasabach-Merritt phenomenon:
Tufted hemangioma
Kaposiform hemangioendothelioma
150
Non hereditary developmental condition of hamartomatous vascular proliferation
Sturge- Weber Syndrome; Encaphalotrigeminal Angiomatosis; Sturge -Weber Angiomatosis
151
portwine stain aka
nevus flammeus
152
nevus flammeus aka
portwine stain
153
Sturge Weber mutation
GNAQ 9q21
154
Noonan syndromw
o Heart defect
o Web neck
o Flat nose
o Pectum excavatum
155
fibrous histiocytoma not in the mouth
dermatofibroma
156
dermatofibroma in the mouth
Fibrous histiocytoma;
157
solitary fibrous tumor IHC
CD34+, BCL-2, STAT6
158
cafe au lait spots
Coast of Maine = FD
| Coats of California = NF
159
fibromatosis in bone
desmoplastic fibroma
160
fibromatosis syndrome
gardner
161
head and neck fibromatosis
Juvenile aggressive fibromatosis or extra-abdominal desmoids
162
fibromatosis recurrence
30%
163
criteria for neurofibromatosis type 1 (7)
1. 6 ≥ café au lait macules – prepubertal > 5mm / adults >15 mm
2. 2 ≥ NF or 1 plexiform
3. Freckling in the axillary region ( Crow sign)
4. Optic glioma
5. 2 ≥ Lische nodules ( iris hamartoma)
6. Osseous lesions
7. A first degree relative
164
MNTI: preilection, location, radio look, labs, recurrence
Male predilection
Anterior maxilla but other sites have been reported
Sunray radiograph
Lab : High levels of urinary vanillylmandelic acid (VMA)
20% recurrence
165
MNTI IHC
+ CK, HMB-45, NSE, CD 56, Synaptophysin in some cases
166
increased vanillylmandelic acid entities
o MNET
o MTC
o Pheochromocytoma
o Neuroblastoma
167
paraganglioma in head and neck syndromes
NF1, MEN 2, von Hippel-lindau
| syndrome
168
sustentacular cells in paragangliomas IHC
S100+
169
paraganglioma mutation and highest rate of malignancy
PGL1-4 (SDHB) / Type 4 has highest rate of malignancy 13% -23%
170
von hippel lindau chromosome and presentation
3p; Inherited disorder, formation of tumors and fluid-filled sacs (cysts) in many different
parts of the body. Tumors may be either noncancerous or cancerous and most
frequently appear during young adulthood.
171
tumors with von hippel lindau
angiomatosis, hemangioblastomas, pheochromocytoma, renal cell carcinoma,
pancreatic cysts (pancreatic serous cystadenoma), endolymphatic sac tumor, and
bilateral papillary cystadenomas of the epididymis (men) or broad ligament of the
uterus (women
172
VHL signs and symptoms
headaches, problems with balance and walking, dizziness,
| weakness of the limbs, vision problems, and high blood pressure
173
```
favored site:
Adult rhabdomyoma
Granular cell tumor
Hibernoma
Paraganglioma
```
Adult rhabdomyoma H&N
Granular cell tumor skin, tongue
Hibernoma interscapular
Paraganglioma extra-adrenal ganglioma
174
```
electron microscopy:
Adult rhabdomyoma
Granular cell tumor
Hibernoma
Paraganglioma
```
Adult rhabdomyoma think/thick filaments
Granular cell tumor phagolysosomes
Hibernoma m/ch
Paraganglioma neurosecretory granules
175
```
S100:
Adult rhabdomyoma
Granular cell tumor
Hibernoma
Paraganglioma
```
Adult rhabdomyoma rare, focal
Granular cell tumor diffuse
Hibernoma diffuse
Paraganglioma sustentacular cells
176
```
MSA:
Adult rhabdomyoma
Granular cell tumor
Hibernoma
Paraganglioma
```
Adult rhabdomyoma diffuse
Granular cell tumor neg
Hibernoma neg
Paraganglioma neg
177
```
Chromogranin:
Adult rhabdomyoma
Granular cell tumor
Hibernoma
Paraganglioma
```
Adult rhabdomyoma neg
Granular cell tumor neg
Hibernoma neg
Paraganglioma diffuse
178
congenital epulis: sex, location, special cells
congenital granular cell lesion: 90% F, Midline, Maxilla
179
hemangioma of infancy special IHC
GLUT1
180
nasopharyngeal angiofibroma special radio
Anterior bowing of posterior wall of maxillary sinus
181
Anterior bowing of posterior wall of maxillary sinus
nasopharyngeal angiofibroma
182
nasopharyngeal angiofibroma: age, location, recurrence
```
Male
Pterygopalatine fossa
Sphenopalatine foramen
Anterior bowing of posterior wall of maxillary sinus
Recurrence 20 %-40%
```
183
lymphangiomas look
frog eggs or tapioca
184
frog eggs or tapioca
lymphangioma
185
macro vs microcystic lymphangioma
Macrocystic ( cystic hygroma) : 2cm > , 4% in blacks
| Microcystic: 2cm < / Mixed
186
cystic hygroma
macrocystic (>2cm) lemphangioma, 4% in blacks
187
spider web
rhabdomyoma
188
Batson plexus:
Valveless vertebral venous plexus
189
origin of congenital epulis of newborn
likely myofibroblastic
190
congenital epulis of newborn aka
congenital granular cell lesion
191
congenital granular cell lesion aka
congenital epulis of newborn
192
gender, location for congenital epulis of newborn
10% multiple, 90% females, Mx>md ib akveikar ridge (rarely on tongue also)
193
congenital epulis of newborn IHC
S100 - (vs granular cell tumor), KP-1+, vimentin+; no pseudoepitheliomatous hyperplasia (unlike granular cell tumor)
194
pseudoepitheliomatous hyperplasia granular cell tumor vs granular cell lesion of newborn
yes in GCT, not in newborn epulis
195
extracranial meningioma origin
ectopic arachnoid lining cells
196
ectopic arachnoid lining cells give rise to
extracranial meningioma
197
extracranial meningioma types, location, origin, histo
1 - congenital - skin of scalp, forehead, paravertebral; abnormal neural tube closure; intermediate between meningocele an dmeningioma (aka meningeal hamartoma)
2 - adults - close to sensory organs (eye ear nose) - syncytial pattern, swirling whorled balls of cells, collagenous septa, psammoma bodies; CK+
198
type 1 extracranial meningioma location, origin, histo, aka
1 - congenital - skin of scalp, forehead, paravertebral; abnormal neural tube closure; intermediate between meningocele an dmeningioma (aka meningeal hamartoma)
199
type 2 extracranial meningioma location, origin, histo, IHC
2 - adults - close to sensory organs (eye ear nose) - syncytial pattern, swirling whorled balls of cells, collagenous septa, psammoma bodies; CK+
200
congenital displacement of neuroglial tissue aka
glial heterotopia, nasal glioma, glial hamartoma, heterotopic glial tumor; variant of encephalocele
201
glial heterotopia location, clinical, age
6-% subQ tissue of nose, 30% nasal cavity; polypoid mass in nose of infant, gros with infant
202
glial heterotopia in mouth
glial choristoma
203
glial choristoma histo, IHC
glial heterotopia; mats of glial tissue with astocytes; neuronal elements absent; GFAP+ (glial fibrillary acidic proterin), S100+
204
encephalocele vs glial heterotopia
encephalocele connects to CNS via defect in cribriform plate; when in nose, same as glial heterotopia
205
encephalocele histo
mixture of astrocytes, glial fibers, and neuronal elements (latter absent in glial heterotopia)
206
MEN1 aka, findings
Werner sybdrome: 3P: benign tumors of parathyroid, pancreas, and pitutary
207
benign tumors of parathyroid, pancreas, and pitutary which MEN
MEN1 aka Werner syndrome
208
MEN2A aka and findings
Sipple syndrome, pheochromocytoma and medullary thyroid ca
209
pheochromocytoma and medullary thyroid ca whih MEN
MEN2A aka sipple syndrome
210
MEN2B findings and mutation
Pheochromocytoma (50%), medullary thyroid ca (90%), mucosal neuromas; RET mutation (chrom 10); protuberant lips in narrow face, eversion of eyelids; marfanoid body (thin long limbs and muscle wasting)
211
mucosal neuromas which MEN
MEN2B
212
protuberant lips in narrow face, eversion of eyelids which MEN
MEN2B
213
neuromas in MEN2B location
lips, anterior tongue, bilatrally on commissures
214
marfanoid body which MEN
MEN2B
215
medullary thyroid ca labs
increased serum or urinary calcitonin
216
increased serum or urinary calcitonin labs
medullary thyroid ca
217
pheochromocytoma labs
increased vanillylmandelic acid and epi/norepi ratio
218
increased vanillylmandelic acid and epi/norepi ratio which conditions
pheochromocytoma, MNTI, neuroblastoma
219
MNTI labs
increased vanillylmandelic acid and epi/norepi ratio
220
neuroblastoma labs
increased vanillylmandelic acid and epi/norepi ratio
221
MNTI origin, age, location, gender, xray look
neural crest origin, <1 yo, Mx, M>F, sunray pattern on xray
222
MNTI histo and IHC
○ Large epithelioid cells CK+, HMB45+, neurospecific enolase +
○ Smaller cells neurospecific enolase +, CD56+, +/- other neuroendocrine markers
223
biphasic: NSE and CD56+ small round cells; CK+ epithelioid cells
MNTI
224
what are paraganglia
chemoreceptors that detect changes i blood pH and O tension
225
paraganglioma aka
chemodectoma
226
paraganglioma origin
neural crest
227
carotid body paraganglioma origin, gender, condition
neural crest, may arise in response to hypoxia; more in females at higher altitudes
228
cartoid body paraganglioma clinical
deep mass below angle of MD with pharyngeal swelling; Fontaine's sign: lesion moved side to side but no vertical movement
229
Fontaine's sign
lesion moved side to side but no vertical movement -- carotid body paraganglioma
230
lesion moved side to side but no vertical movement
Fontaine's sign, carotid body paraganglioma
231
% rule for carotid body paraganglioma (3)
10% multlifocal, 10% familial history (genomic imprinting), 10% metastasize
232
carotid body paraganglioma histo and IHC
zellballen, highly vascular; chief cells (type 1) -- synapto/chromo; sustentacular cells (type 2) S1--+
233
carney's triad
extra-adrenal paraganglioma., gastric leiomyosarcoma, pulmonary chondroma
234
when is carotid body paraganglioma malignant
when mets are present
235
jugulotympanic paraganglioma aka
glomus jugulare
236
glomus jugulare aka
jugulotympanic paraganglioma
237
jugulotympanic paraganglioma location and origin
paraganglia or auricular branch of vagal nerve or tympanic branch of glossopharyngeal nerve; temporal bone and middle ear;; glomus jugulare -- when arises from jugular bulb;; glomus tympanicum -- involves middle ear
238
glomus jugulare
jugulotympanic paraganglioma in jugular bulb
239
glomus tympanicum
jugulotympanic paraganglioma in middle ear
240
most common neoplasm of middle ear
jugulotympanic paraganglioma/glomus tympanicum
241
vagal body tumor aka
vagal paraganglioma
242
vagal paraganglioma aka
vagal body tumor
243
vagal paraganglioma findings and location
high cervical masses between mastoid process and angle of jaw; above carotid bifurcation without widening of bifurcation point
244
high cervical masses between mastoid process and angle of jaw; above carotid bifurcation without widening of bifurcation point
vagal paragnglioma
245
glomus tumor origin and function
glomus body: regulates temperature, is an AV shunt
246
arterial segment of glomus body
Sucquet-Hoyer
247
glomus body composition
varying proportions of glomus cells, blood vessels, and smooth muscle
248
glomus tumor clinical, location
papulonodular lesions, red/pink/blue, extremely painful;; acral distribution (hand, foot, forearm)
249
glomus tumor types
solid, glomangioma, glomangiomyoma (60%), infiltrating, glomangiosarcoma
250
glomangioma histo
cookie cutter cells (ptominent cell borders); round, centrally placed, almost neuroendocrine like; cells around vascular lakes
251
glomangiomyoma histo
smooth muscle cells near vascular spaces blending with glomus cells
252
hemangioma vs vascular malformation
hemangioma arises during first 8 wks, involutes; vascular malformation present at birth, no involution
253
most common tumor of infance
hemangioma
254
hemangioma gender, location
most common tumor of infancy, F:M 5:1, 60% H&N
255
PHACES syndrome
Posterior fossa brain anomaly (Dandy Walker), Hemangioma, Arterial anomalies, Cardiac defects, Eye anomalies, Stenral cleft/supraumbilical raphe
256
Kasabach Merritt phenomenon
thrombocytopenia and hemorrhage due to platelets being trapped in tufted hemangioma/or kaposiform hemangioendothelioma
257
thrombocytopenia and hemorrhage due to platelets being trapped in tufted hemangioma/or kaposiform hemangioendothelioma
Kasabach Merritt phenomenon
258
intrabony hemangioma radio and clinical
bruit/pulsation; multi or unilocular; radiolucent or sunburst
259
hemangioma histo types
juvenile or cellular (aka juvenile hemangioendothelioma), capillary, cavernous
260
most common slow growing tumor of infants
juvenile hemangioendothelioma aka jivenile/cellular hemangioma
261
hemangioma of infancy IHC
GLUT1+ (negative in vascular malformations)
262
intravascular papillary endothelial hyperplasia aka
Masson's tumor, Masson's hemangioma
263
papillary endothelial hyperplasia origin and histo
reactive pseudoneoplastic proliferation of endothelial cells associated with thrombosis; dilated vascular channels with endolthelial-lined papillary circles and stroma
264
reactive pseudoneoplastic proliferation of endothelial cells associated with thrombosis; dilated vascular channels with endolthelial-lined papillary circles and stroma
papillary endothelial hyperplasia aka Masson's tumor
265
Sturge Weber aka
Sturge Weber sybdrome or angiomatosis, encephalotrigeminal angiomatosis
266
Sturge Weber clinical
port wine stain (nevus flammeus), leptomeningeal angiomas, seizzures, mental retardaton
267
sturge weber intraoral
gingiva may become hyperplastic or with PG(from vascular component or use of phenytoin for seizures)
268
sturge weber skull film
tramline calcifications
269
tramline calcifications on skull film
sturge weber
270
fibrous hyperplasia on maxillary labial frenum
frenal tag
271
frenal tag
fibrous hyperplasia on maxillary labial frenum
272
retrocuspid papilla
fibroma on gingiva lingual to MD canines, disappears with age
273
fibroma on gingiva lingual to MD canines
retrocuspid papilla
274
leaflike denture fibroma
fibroepithelial polyp on hard palate beneath denture, with serrated edges
275
polyp on hard palate beneath denture
leaflike denture fibroma or fibropithelial polyp with serrated edges
276
polyp with serrated edges
leaflike denture fibroma
277
fibromatosis of head and neck
juvenila aggressive fibromatosis or extra-abominal desmoid tumors
278
higher risk conditions for fibromatosis
familial adenomatous polyposis and Gardner
279
familial adenomatous polyposis and Gardner higher risk for
fibromatosis
280
multicentric myofibroma
myofibromatosis; infants with tumors of skin, bone, viscera
281
infants with tumors of skin, bone, viscera
multicentric myofibromatosis
282
oral focal mucinosis location and chemistry
increased hyaluronic acid, 75% on gingiva
283
PG in extraction socket
epulis granulomatosa
284
epulis granulomatosa
PG in extraction socket
285
lipoma with higher recurrence rate
intramuscular
286
risk in surgery for PA
traumatic neuroma in 10%
287
traumatic neuroma association
10% of surgeries for PA
288
schwannoma condition association
NF2
289
NF2 inheritance, genetics, chromosome, tumors
AD, MERLIN gene mutation (chr 22); acoustic neuromas, neurilemomas of peripheral nerves, meningiomas, ependymomas of CNS
290
NF1 inheritance, genetics, chromosome
50% AD, 50% new mutation; NF1 gene on chrom 17, neurofibromin protein
291
chrom 17 which NF
1 -- neurofibromin
292
chrom 22 which NF
2 -- MERLIN
293
MERLIN gene
NF2
294
large baggy neurofibromas special name
elephantiasis neuromatosa
295
bag of worms
plexiform neurofibroma
296
plexiform neurofibroma special feeling
bag of worms
297
elephantiasis neuromatosa
large baggy neurofibromas
298
diagnostic criteria for NF1
6+ cafe au lait, axillary freckling (Crowe's sign), iris hamartoma )Lisch nodules), optic glioma
299
Lisch nodules are
iris hamartomas; NF1
300
freckling in NF1
Crowe's sign
301
Crowe's sign
axillary freckling in NF1
302
iris hamartomas in NF1
Lisch nodules
303
NF1 syndrome associations
Noonan syndrome and CGCG
304
most common problem in NF1
HTN
305
MPNST in NF1
5% of NF
306
anterior bowing of posterior wall of mx sinus
nasopharyngeal angiofibroma
307
nasopharyngeal angiofibroma special radio
anterior bowing of posterior wall of mx sinus
308
leiomyoma histo types
solid, angioleiomyoma, epithlioid leiomyoma (leiomyoblastoma)
309
leiomyoblastoma aka
epithlioid leiomyoma
310
epithlioid leiomyoma aka
leiomyoblastoma
311
special stain in rhabdomyoma
PTAH
312
MPNST and NF1
50% of MPNSTs are in pts with NF1
313
MPNST radio
widening of MD canal or mental foramen
314
widening of MD canal or mental foramen
suspect MPNST
315
triton tumor
MPSNT + malignant skeletal muscle
316
MPSNT + malignant skeletal muscle
triton tumor
317
olfactory neuroblastoma location
nasal cavity close to cribifrom plate
318
botryoid rhabdomyosarcoma histo
cambium layers: zone of increased cellularity below mucosa
319
zone of increased cellularity below mucosa in botryoid rhabdomyosarc
cambium layers
320
alevolar rhabdomyosarcs translocations
PAX3-FKHR and PAX7-FKHR
321
PAX3-FKHR
alveolar rhabdomyosarc
322
PAX7-FKHR
alveolar rhabdomyosarc
323
embryonal rhabdomyosarc generics
loss of heterozygosity 11p15
324
cowden syndrome
multiple trichilemmomas, adnexal hamartomas and nevi, hamartomatous intestinal polyps, breast and other malignancies
325
multiple trichilemmomas, adnexal hamartomas and nevi, hamartomatous intestinal polyps, breast and other malignancies
cowden syndrome
326
loss of heterozygosity 11p15
embryonal rhabdomyosarc
327
alveolar soft part sarc genetics
x17 translocation ASPL-TFE3 fusion protein
328
x17 translocation
alveolar soft part sarc
329
ASPL-TFE3 fusion protein
alveolar soft part sarc
330
synovial sarc genetics
x18 translocation SSx-SS18
331
x18 translocation
synovial sarc
332
SSx-SS18
synovial sarc
333
synovial sarc IHC
CK+, EMA+
334
batson plexus
vertebral plexus that bypasses lung filtration (explains distant mets in jaws but not in lungs)
335
distant mets in jaws but not in lungs
batson plexus -- vertebral plexus that bypasses lung filtration
336
vertebral plexus that bypasses lung filtration
batson plexus (explains distant mets in jaws but not in lungs)
337
men soft tissue mets
lung, renal, melanoma; prostate goes to bone
338
women soft tissue mets
breast (IHC ER PR), genital, lung, bone, kidney
339
Klippel Trenaunay Weber syndrome
multiple facial hemangiomas, vascular masses with enlargement of extremities, ocular disorders
340
multiple facial hemangiomas, vascular masses with enlargement of extremities, ocular disorders
Klippel Trenaunay Weber syndrome
341
Klippel Trenaunay Weber syndrome oral findings
premature tooth eruption and bony overgrowth --> malocclusion
342
nasopharyngeal angiofibroma age gender location
exlusively in young males (10-17yo), pterygopalatine fossa
343
lesion of pterygopalatine fossa exclusively in young males
nasopharyngeal angiofibroma
344
nasopharyngeal angiofibroma imagina
anterior bowing of posterior wall of mx sinus, angiogram useful
345
anterior bowing of posterior wall of mx sinus
nasopharyngeal angiofibroma
346
hemangiopericytoma aka
solitary fibrous tumor
347
solitary fibrous tumor aka
hemangiopericytoma
348
hemangiopericytoma location in mouth and in general
75% cheek; common on pleura
349
hemangiopericytoma pattern
tightly packed cells around staghorn vessels
350
patternless pattern: spindle cells in short fascicles
solitary fibrous tumor/hemangiopericytoma
351
solitary fibrous tumor histo
remember aka hemangiopericytoma ~ alternating hyper/hypocellular zone; hypocellular with prominent hyalinized collagen bundles; bands of dense collagen separate individual cells; myxoid bluish background around islands of pleomorphic bland cells; HPC like areas with staghorns
352
solitary fibrous tumor IHC
CD34+, CD99+, bcl-2+, SMA -, STAT6
353
tightly packed cells around staghorn vessels
hemangiopericytoma (=SFT) like pattern
354
glomangiopericytoma aka
sinonasal hemangiopericytoma, glomus tumor, HPC-like tumor; distinct from soft tissue hemangiopericytome
355
sinonasal hemangiopericytoma aka
glomangiopericytoma, glomus tumor, HPC - like tumor; distinct from soft tissue hemangiopericytome
356
glomagiopericytoma histo
remember aka sinonasal hemangiopericytoma ~ monomorphic spindle to ovoid cells with lightly eosinophilic cytoplasm and bland nuclei forming short fascicles or a storiform/whorled/palisaded pattern; tumor cells aggregate around staghorn vessels
357
PEComa = ; examples, association
perivascular epithelioid cell neoplasm; derived from perivascular epithelioid cells. eg renal angiomyolipoma, lymphangiomyomatosis, clear cell sihar tumor of lung; associated with tuberous sclerosis complex
358
tuberous sclerosis classic organ tumors
renal angiomyolipoma, cardiacrhabdomyoma; subependymal giant cell astrocytoma
359
renal angiomyolipoma type of tumor and syndrome
PEComa ~ tuberous sclerosis
360
PEComa IHC, histo
melanocytic and muscle markers, can look like granular cell tumor
361
lymphangiomyomatosis what is it and demographic
PEComa, tuberous sclerosis association, exclusively in women of child-bearing age
362
lymphangioma types
simplex (capillary), cavernous, cystic (cystic hygroma)
363
lymphangioma location, age
50-75% head and neck, 90% <2yo
364
cystic lymphangioma location
most in posterior triangle of neck; anterior lesions more complications
365
oral lympahngiomas location and look
anterior 2/3 of tongue, frog eggs or tapioca pudding
366
neonates and lymphangiomas?
4% of black babies, esp boys, have bilateral lymphangiomas on mandibular alveolar ridge
367
leiomyoma locationas
most in uterus, GI tract, skin
368
leiomyoma histo types
solid, angioleiomyoma, epithelioid leiomyoma (leiomyoblastoma)
369
leiomyoma staining
Masson red muscle, SMA+, MSA+
370
rhabdomyoma types and locations
adult (pharynx, larynx, FOM, soft palate, base of tongue) and fetal (face, periauricular)
371
adult rhabdomyoma look and location
pharynx, larynx, FOM, soft palate, base of tongue; spider-web ~ peripheral vacuoles with filaments
372
fetal rhabdomyoma look and location
face, periauricular ~ haphazard spindle cells (muscle looking) in a myxoid stroma
373
rhabdomyoma IHC
myoglobin, desmin, MSA, PTAH + (PTAH myofibrils stain purple)
374
PTAH staining
purple myofibrils and rhabdomyoma
375
normal tissue in abnormal location
choristoma
376
excess tissue in normal location
hamartoma
377
hamartoma vs choristoma
hamartoma excess tissue in normal location; choristoma normal tisue in abnormal location
378
osseous and cartilaginous choristoma location gender
85% posterior tongue, 70% women
379
ectomesenchymal chondromycoid tumor location, histo, IHC
always anterior tongue; well defined multilobulated; spindle to round cells in a myxoid or chondroid background; GFAP+
380
multinucleate cell angiohistiocytoma origin and presentation
chronic inflammatory disorder of unknown cause; multiple firm, red-purple, dome shaped, coalescing/linearly arranged papules on skin of limbs
381
multiple firm, red-purple, dome shaped, coalescing/linearly arranged papules on skin of limbs
multinucleate cell angiohistiocytoma, chronic inflammatory disorder of unknown cause
382
multinucleate cell angiohistiocytoma histo, IHC
Histo: proliferation of small blood vessels with unusual dendritic cells and multinucleate cells; IHC endothelial - factor VIII-RA, CD34, CD31; dendritic - factor XIIIa, lysozyme, a1 antichymotrypsin, vimentin, CD68; giant cells - vimentin only
383
fibrosarcoma location and diagnosis
diagnosis of exclusion; most in deep soft tissues of lower extremities, especially thigh and knee
384
fibrosarcoma histo
herrinbone: fascicles of spindle cells
385
infantile fibrosarcoma aka and age
congenital fibrosarcoma, aggressive infantile fibromatosis; congenital or within first year of life
386
infantile fibrosarcoma histo
identical to adult but more prominent HPC-like growth
387
infantile fibrosarcoma genetics
12 15 translocation --> ETV6-NTRK3 fusion protein
388
12 15 translocation
ETV6-NTRK3 fusion protein
| secretory Ca
389
ETV6-NTRK3 fusion protein
secretory carcinoma, 12 15 translocation
390
dermatofibrosarcoma protuberans genetics
translocation 17, 22 (Col11A1-PDGFbeta fusion and increase in PDGFbeta chain)
391
translocation 17, 22
dermatofibrosarcoma protuberans (Col11A1-PDGFbeta fusion and increase in PDGFbeta chain)
392
Col11A1-PDGFbeta
increase in PDGFbeta chain, dermatofibrosarcoma protuberans, translocation 17, 22
393
increase in PDGFbeta chain,
dermatofibrosarcoma protuberans, translocation 17, 22; Col11A1-PDGFbeta fusion
394
dermatofibrosarcoma distribution
bathing suit
395
dermatofibrosarcoma protuberans histo and IHC
monotonous haphazard storiform arrangement of spindle and stellate cells that infiltrate and entrap adnexa and adipose; low mitoses; IHC: Cd34+, factor XIIIa neg (vs BFH, CD34-, XIIIa+)
396
pigmented dermatofibrosarcoma protuberans
Bednar's tumor
397
Bednar's tumor
pigmented dermatofibrosarcoma protuberans
398
giant cell fibroblastoma
juvenile form of dermatofibrosarcoma protuberans
399
juvenile form of dermatofibrosarcoma protuberans
giant cell fibroblastoma
400
malignant fibrous histiocytoma age and pattern
older patients, storiform pattern
401
most commo soft tissue sarcoma (%, age?)
liposarcoma - 20% of all malignancies in adults
402
liposarcoma location
most in thigh, retroperitoneum, inguinal
403
liposarcoma types
well diff (most common in oral cavity), myxoid/round cell, pleomorphic, dedifferentiated
404
most common liposarcoma in mouth
well diff
405
worst prognosis of liposarcoma
pleomorphic
406
malignant peripheral nerve sheath tumor vs NF1
50% of MPNSTs are in NF1 pts; pts with MPNST and NF1 are 10y younget (35 vw 45) and worse prognosis than without NF1
407
MPNST location
proximal extremities and trunk
408
MPNST xray
widening of MD canal or mental forament
409
malignant triton tumor
MPNST + malignant skeletal muscle
410
MPNST + malignant skeletal muscle
malignant triton tumor
411
MPNST histo and IHC
low-power: distinctive perivascular tumor cells surrounded by necrosis: S100+ in only 50% of caes
412
angiosarcoma location and better prognosis
most in scalp and forehear; early lesion resembles a bruise; better prognosis for oral and salivary gland tumors
413
hemangioendothelioma
intermedite between hemangioma and angiosarcoma
414
angiosarcoma IHC
CD31+, factor VIII+; CD34 less consistnet
415
angiosarcoma environmental risk factors
PVC vinyl chloride carcinogen; radiation (increased incidence in women with history of breast cancer
416
stewart-treves syndrome
angiosarc with chronic lymphedema
417
angiosarc with chronic lymphedema
stewart-treves syndrome
418
kaposi sarcoma types
classic, endemic (benign, aggresive, florid, lymphadenopathic), iatrogenic, AIDS related
419
kaposi stages
patch, plaque, nodular
420
patch stage of kaposi
normal structures admixed with tumor proliferation, promontory sign
421
leiomyosarcoma location
most in uterine wall and GI tract
422
epithelioid leiomyosarcoma histo
entirely of rounded cells
423
leiomyosarcoma mutation
some associated with RBI mutation
424
RBU mutation
leiomyosarcoma (some)
425
leiomyosarcoma special stain
PAS shows glycogen within the cells; cell cytoplasm red on Masson trichrome; MSA (HHF35), SMA, desmin, H-caldesmon, SMMS (smooth muscle myosin)
426
rhandomyosarcoma frequency
60% of soft tissue sarcomas in children
427
types of rhabdomyosarcomas and ages
embryonal (NOS, botryoid, spindle) - <10 yo; alveolar (10-25yo), pleomorphic >40yo
428
most common sarcoma in children
rhabdomyosarcoma
429
embryonal rhabdomyosarc histo
round, spindle, and strap cells
430
rhabdomyosarcoma location
head and neck: face/orbit; and GU tract; oral rhabdomyosarc - palate and maxillary sinus
431
rhabdomyoblasts histo
small cells with dark nucleo and depply pink cytoplasm
432
botryoid rhabdomyosarc
exophytic polypoid growth in a cavity (mouth, vag(
433
exophytic polypoid rhabdomyosarc in a cavity (mouth, vag)
botryoid, type of embryonal
434
cambium layers in rhabdomyosarc
zone of increased cellularity just below mucosa in botryoid
435
zone of increased cellularity just below mucosa in botryoid rhabdomyosarc
cambium layers
436
alveolar rhabdomyosarc genetics
PAX3-FKHR and PAX7-FKHR translocation
437
PAX3-FKHR
alveolar rhabdomyosarc
438
PAX7-FKHR
alveolar rhabdomyosarc
439
embryonal rhabdomyosarc genetics
11p15 LOH
440
11p15 LOH
embryonal rhabdomyosarc
441
rhabdomyosarc IHC
myogenin, myoD1, HHF-35
442
alveolar soft part sarcoma geneics
X;17 translocation, ASPL-TFE3 fusion protein
443
ASPL-TFE3 fusion protein
alveolar soft part sarcoma, X;17 translocation
444
X;17 translocation
alveolar soft part sarcoma, ASPL-TFE3 fusion protein
445
alveolar soft part sarcoma age sex location
young patients -- orbit and tongue, more females; adults -- lower extremities, more males
446
alveolar soft part sarcoma histo and IHC
discohesive cells in nests, minimal atypia, vascular inastion; TFE3+ only IHC marker;; CRYSTALS! crystals are PAS+, diastase resistant; latticework pattern on EM
447
crystals in alveolar soft part sarcoma
crystals are PAS+, diastase resistant; latticework pattern on EM
448
synovial sarcoma genetics
X;18 translocation - SYT gene chr 18 and SSX1/SSX2 on gene X; --> SYT/SSX fusion mRNA can be detected by RT-PCR or FISH
449
X:18 translocation
synovial sarcoma; SYT gene chr 18 and SSX1/SSX2 on gene X; --> SYT/SSX fusion mRNA can be detected by RT-PCR or FISH
450
SYT-SSX fusion mRNA
X;18 translocation; synovial sarcoma
451
synovial sarcoma location
most near large joints and bursae of extremities; head and neck: paravertebral and parapharyngeal mass
452
synovial sarcoma histo and IHC
classically biphasic: spindle cells ~ fibrosarcoma + epithelial cells around glandlike spaces or in nests/cords/whorls.
"slit-oma" calcifications in 30%, staghorn vessels;;; CK+, EMA+, CD99+
453
follicular dendritic cell sarcoma origin and location
arises from dendritic (antigen-presenting) cells; predilection for LN of neck, axilla, mediastinum
454
follicular dendritic cells histo, IHC
syncytial appearing spindly cells in fascicles or whorls; CD21, CD35+
455
ewing sarcoma aka
primitive neuroectodermal tumor
456
primitive neuroectodermal tumor aka
ewing sarcoma
457
ewing sarcoma demographics and presentation
usually whites <20yo, mandible. fever, increased ESR, leukocytosis
458
ewing sarcoma genetics
11,22 translocation. MIC2 gene product. CD99+. FISH against translocation more specific
459
11, 22 translocation
Ewing sarcoma, MIC2 gene product (CD99+ but FISH against translocation more specific)
460
MIC2 gene product
Ewing sarcoma, 11, 22 translocation. (CD99+ but FISH against translocation more specific)
461
periosteal reaction in ewing
onionskin -- long bones, not common in jaws
462
atypical ewing sarcoma
larger cells
463
ewing sarcoma histo
small undifferentiated round blue cells, probably neuroectodermal. 75% contain glycogen granules in cytoplasm.
464
ewing sarcoma locations with poorer prognosis
pelvic and proximal lesions
465
most common locations for mets to oral soft tissues
gingiva ~50% (looks like PG) and tongue 25%
