5. Vascular Pathology Flashcards
(22 cards)
1
Q
Hemangioma
• Most common tumor of ____
• Distinct ____ predilection
• ____ and cavernous most common
• Most common tumor of childhood > hemangioma ○ Benign vascular neoplasm ○ Composed of proliferation of endothelial cells § Vascular channels • Female predilection ○ Like \_\_\_\_ granulomas ○ Suggests \_\_\_\_ can play a role in pathogenesis • Could be result of different histologist all associated with different caliber BV ○ Capillary hemangioma § Tumors composed of \_\_\_\_ (small) ○ Cavernous hemangioma § \_\_\_\_ caliber vessels § Lesions are larger than with capillary • \_\_\_\_ tongue, on the gingiva and on \_\_\_\_ lip ○ Looks like pyogenic granuloma clinically > was a hemangioma • Most common DD on gum > three P's > \_\_\_\_ granuloma, peripheral ossifying or peripheral giant cell ○ Not exclusive; hemangioma and \_\_\_\_ is also an example
A
childhood female capillary pyogenic hormones capillaries larger ventral lower pyogenic metastatic carcinoma
2
Q
Hemangioma
• When macular they look like \_\_\_\_ • Approach to patient: ○ Ask if there's \_\_\_\_ ○ Two week and wait and see if resolves § Bruising resolves \_\_\_\_ in a few days ○ If haven't changed in appearance > warrants a \_\_\_\_
A
ecchymoses
trauma
spontaneously
biopsy
3
Q
Clinical features
- ____, purple, blue
- May feel ____ to touch
- May feel ____ or hear bruit upon auscultation• Similar to the three P’s (red, blue, purple) and an ecchymosis and potentially like a melanoma
• The larger it is > the warmer it is to touch > the tumor contains lots of BV and blood
○ Will feel a pulse > bruit
○ May hear a pulse with a stethoscope
A
red
warm
pulse
4
Q
Diagnosis • \_\_\_\_ • \_\_\_\_ – Identify feeder vessel • ALWAYS \_\_\_\_ before biopsy • Biopsy / excise in \_\_\_\_ setting
• Biopsy is most critical for diagnosis • Suspect hemangioma > don't biopsy in office > a lot of bleeding will occur • Diascopy ○ Quick and easy ○ Take a glass slide and press on the lesion > if the lesion blanches with minimal exertion > implies \_\_\_\_ pathology ○ Not diagnostic, but indicative of what you're dealing with • Send the patient to a radiologist for an angiogram ○ Radiograph ○ Inject dye into \_\_\_\_ (wait 0.5 hour-1 hour) contains radioopaque particles ○ If hemangioma, will see a collection of dye within the \_\_\_\_ itself > will identify the \_\_\_\_ from which it is derived § Not derived \_\_\_\_! • Purpura is extravascular, a bruise is \_\_\_\_ ○ Will not blanch ○ Hemangioma is \_\_\_\_ • Hemangioma in the finger ○ BV are lit up by the dye ○ Derives feeder vessel > surgeron can tie off the BV > strangulate the blood supply and shrink it before surgery, or can inject directly into the lesion with chemical caudery > shrink tumor so it's manageable for surgery
A
diascopy
angiogram
aspirate
controlled
vascular vein mass BV de novo
extravascular
intravascular
5
Q
• Hemangioma in the vestibule
• Microscopically:
○ Tumor filled with BV
○ Will ask us to recognize a cavernous hemangioma
§ Tumor composed of ____ vessels filled with blood
• Any BV can give rise to a hemangioma ○ Tumor composed of \_\_\_\_ - \_\_\_\_ hemangioma
A
large
veins
venous
6
Q
- Mixture of both veins and ____
- Any vascular channel could give rise to a hemangioma
- Have to be removed because they will continue to ____
A
arteries
grow
7
Q
Lymphangioma
• Composed of \_\_\_\_ vessels • Most noticed at \_\_\_\_ – ≈ 90% develop by age 2 – >65% in head and neck region • \_\_\_\_ lesions occur at any age • No \_\_\_\_ predilection
• Benign tumor composed of lymphatic vessels • Considered \_\_\_\_ and most commonly manifest during early childhood: ○ Most common way is lesion in the neck on one side > \_\_\_\_ hygroma § Risk for airway obstruction > especially in the anterior triangle of the neck § Treated very early in life; at least when patients can tolerate general anesthesia ○ Or areas with a lot of \_\_\_\_ (axilla, groin, belly) ○ Variant on the tongue > \_\_\_\_ lymphangioma ○ Occur at any age ○ Take on a lumpy bumpy/\_\_\_\_ appearance ○ Most common location: \_\_\_\_ tongue; but can grow anywhere on the skin ○ Bumps have a \_\_\_\_ coloration > lymphatic fluid (stains pink microscopically, and clinically looks clearish) ○ Run risk of being traumatized because of their locations > they become \_\_\_\_ and blood rushes in as well ○ More commonly take in a reddish, bluish pinkish coloration • If left untreated will expand ○ In H+N area they expand invariably > has a runny nose and the lesion expands in size > presence of \_\_\_\_ ○ Once they grow they do not \_\_\_\_ (they can shrink slightly?) § Young kids get sick frequently > as they grow more risk to impinge on \_\_\_\_ • Completely \_\_\_\_ • \_\_\_\_ pudding and bubble tea > looks like lymphangioma
A
lymphatic
superficial
sex
neoplastic cystic fat papillomatous dorsum translucent red lymph shirnk airway painless tapioca
8
Q
Lymphangioma
• Often affects ____ tongue – May induce ____
• Clusters of small translucent nodules – May be red, if ____
• ____
A
anterior
macroglossia
traumatized
asymptomatic
9
Q
Cystic hygroma • Develop in loose, \_\_\_\_ areas • Usually \_\_\_\_ neck triangle – Anterior neck lesions may cause \_\_\_\_ obstruction • \_\_\_\_ over time
• Development tumor in areas with lots of space and fat ○ H+N § Anterior neck triangle > airway obstruction ○ Buccal mucosa ○ Axilla ○ Groin • Variant of a \_\_\_\_
A
spacious posterior airway wax and wane lymphangioma
10
Q
Angiosarcoma
• ____
• Not common • Very deadly ○ Has one of the \_\_\_\_ survival rates of any cancer known ○ Even worse than a melanoma • Other vascular cancer (malignant): Kaposi sarcoma ○ Caused by \_\_\_\_ ○ Not as aggressive as angiosarcoma
A
malignant vascular neoplasm
poorest
herpes 8
11
Q
Kaposi sarcoma
- KSV (herpes type 8)
- ____
- ____
- ____• True malignancy, where there are three clinical subtypes:
○ Classic
§ Occur in ____ Mediterranean mean
§ Occurs in ____ primarily; ____ almost exclusively
§ ____ disease
□ Slow-growing
□ Chances will die of ____ instead of cancer
○ Endemic
§ ____ endemic
□ Africa, Haiti
§ Different variations where there are indolent and aggressive
○ AIDS-related
§ Most aggressive
• Each area on face is a discrete KS
• Treated with ____ FIRST
• AIDS defining illness
○ Decreasing in prevalence bc of treatment of ____-disease; treatments are equally effective for ____
A
classic endemic AIDS-related elderly extremities lower indolent old age
virus
chemotherapy
HIV
KS
12
Q
Kaposi’s sarcoma
* Emphasize that you cannot use appearance of a lesion to commit yourself that it's nothing bad * No known illness of HIV until diagnosis of \_\_\_\_ * In this area of world > diagnose KS > \_\_\_\_ until proven otherwise * Can be mistaken for \_\_\_\_, hemangioma or an \_\_\_\_ * All examples of KS * Some lesions were thought to be pigmented lesions and rule out \_\_\_\_
A
KS AIDS-related pyogenic granuloma ecchymoses melanoma
13
Q
Varix / Varicosity
• Acquired ____ malformation
• ____ tongue and lower ____ – May be related to ____ sun damage
• Can be born with hemangioma that may resolve on it's own • May just be malformations, not true tumors ○ Can be acquired: varicositiies/varix § Varicous veins □ With reduced walking/exercise the blood starts pooling in the lower extremeties > veins engorge bc the blood cannot go back up to the heart § Can seen in oral cavity > floor of mouth and ventral tongue, and lower lip □ Two large veins course ventral tongue > can engorge with \_\_\_\_ □ Unknown cause of ventral tongue □ For lower lip, people think it may be due to sun damage • Clinical variation of normal > not \_\_\_\_ • Biopsy in a \_\_\_\_ environment > blood filled lesion
A
vascular
ventral
lip
chronic
blood
pathologic
controlled
14
Q
Congenital vascular malformation
• CN \_\_\_\_ (V1, 2, and 3) ○ During development, as developing on both sides > fills nerves with BV and vascular channels > some kids are born with pronounced and overt \_\_\_\_ malformations that track along V1, V2 and/or V3 • Know the chart • Vascular malformation manifests at the time of \_\_\_\_ ○ Can affect the H+N, but more commonly found elsewhere ○ Do not spontaneously resolve > require \_\_\_\_ • \_\_\_\_ hemangiomas develop shortly after birth ○ Tend to manifest in the H+N primarily • V1 and V2 involvement, in addition to involvement of \_\_\_\_ and leg area
A
V vascular birth surgery strawberry (juvenile)
15
Q
Congenital vascular malformation
• Not \_\_\_\_, and not lesions comprised of \_\_\_\_ • Abnormal \_\_\_\_ between vascular channels ○ Arterial meets venous side > anastomosis ○ In malformations > irregular anastomosing of these vessels > manifests in a gross section > each circular structure is an abnormal anastomosis of BV > triggers the clinical pathology • Will not go away by itself, requires \_\_\_\_
A
tumors
BV
anastomoses
surgery
16
Q
Sturge-Weber angiomatosis
• Non-____
• Activating mutation in ____ gene
– G protein coupled receptor mutation results in ____ stimulation of downstream signaling pathway
• Manifestations – \_\_\_\_ (port-wine stain) – Hemangiomas of leptomeninges – “\_\_\_\_” calcifications of brain – Seizures and mental retardation
• Port-wine stain or a nevus flamius • Port-wine stain > common in population ○ Forehead: \_\_\_\_ ○ V2 on the right patient ○ V2 and V3 in the man on the left ○ Can occur sporadically, or in context of development disorder: SWA § Not a genetic/inherited disease > but a \_\_\_\_ disease caused by a known gene defect occuring after \_\_\_\_ □ GNAQ > const activation of molecular pathways that trigger formation of \_\_\_\_ endothelium □ Not \_\_\_\_ □ Children will not get the \_\_\_\_ mutation ® Postzygotic mutation § Occurring earlier in development > the \_\_\_\_ the phenotype > several areas and will typically be unilaterally § Occuring later in development > more limited in development □ Ex: half face affected □ Would also develop true hemangiomas of the \_\_\_\_ ® Risk for seizures and mental retardation
A
hereditary GNAQ constitutive nevus flammeus tramline V1 developmental gametogenesis hyperplastic neoplastic genetic worse brain
17
Q
• First glance though AA > she’s caucasian > such severe/overt sturge weber occuring at such a young age > ____ and half body is affected
A
full face
18
Q
Sturge-Weber angiomatosis
• Upon mutation of GNAQ > const signalling to lead to manifestations • Pathognomonic appearance on a skull film: ○ Tram-line \_\_\_\_ § \_\_\_\_ lines in their brain § Represent calcified \_\_\_\_ □ Contributes to the \_\_\_\_ and retardation • Not a genetic disease • No \_\_\_\_ for this disorder
A
calcifications parallel BV seizures treatment
19
Q
Hereditary hemorrhagic telangiectasia
- ____ syndrome
- Autosomal ____
- Genetically ____
- Mutations disrupt ____ between pro- and anti-angiogenic signaling• Genetic disease
• Autosomal dominant
• Mutation in gene that triggers array of complications that focus on the vasculature
○ Results in disruption in homeostasis between processes that grow BV and processes that suppress BV formation
○ Homeostasis is balanced; and patients with this disorder > become imbalanced
○ Have telangiectasias > blood ____ develop everywhere
§ Primarily in skin and mucosa
□ Oral and GI mucosa
® Rectal bleeding
• Not ____
○ Prone to ____ also (true tumors of endothelium)
○ Prone to vascular malformations
○ Prone to a whole spectrum of lesions derived from BV
§ ____ and non-neoplastic combined
• How to test vascular in nature
○ ____
§ Blanching test - if blanches it’s a vascular proliferation and not a petechiae or purpura
• Patients may become ____ because of the loss of blood through the fecal matter
• No ____ for this disease
○ Monitor patients closely
○ And ____ for whatever they’re anemic for
A
rendu-osler-weber
dominant
heterogeneous
balance
blisters tumors hemangiomas neoplastic diascopy anemic treatment
supplement
20
Q
Hereditary hemorrhagic telangiectasia
- ____
- Hemangiomas
- ____ malformations
- Spontaneous ____
A
telangiectasias
vascular
bleeding
21
Q
CREST syndrome (Limited scleroderma)
- ____ cutis
- ____ phenomenon
- Esophageal ____
- ____
- ____• Not genetic disease, but similar clinically
○ ____ disease
• May mimic HHT bc of presence of telangiectasias
○ Very different processes
○ CREST > composed of array of findings that go beyond the vascular spectrum
§ Do not get ____ or malformations
§ They get:
□ Calcifications
□ Raynoud phenomenon
® BV constrict in extremities and toes/fingers turn ____
□ Esophageal strictures
® Difficulty ____ over time
□ Scleodactyly
® Fibrosis of the soft tissue and as it becomes more fibrotic may cause resorption of the ____
□ Telangiectasias
§ Also called ____ scleroderma
□ Includes the other five manifestations
□ Systemic sclerosis is associated with ____ risk for fibrosis
• ____ predilection; can run in families (not a genetic link!)
A
calcinosis raynaud dysfunction sclerodactyly telangiectasias
autoimmune hemangiomas white eating bone limited
full-body
female
22
Q
CREST syndrome (limited scleroderma)
• Calcifications subcutaneously ○ Very painful ○ Dermis starts \_\_\_\_
A
calcifying
