Vascular tumors Flashcards
When do vascular malformations occur?
They are present at birth
What is the clinical progression of vascular malformations?
These are capillary, venous, lymphatic or arteriovenous malformations, therefore they do not rapidly enlarge like hemangiomas (not true neoplasms)
They may persist and become more verrucous over time (PWS)
What is the GLUT-1 staining in vascular malformations?
GLUT-1 negative
What disorders can vascular malformations be associated with?
Maffucci syndrome, Klippel-Trenaunay, Sturge-Weber, Blue rubber bleb (cavernous hemangioma), kasabach-merritt syndrome and proteus syndrome
What is the histology of the nevus flammeus?
Dilated capillary-sized vessels
What are the histologic hallmarks of an angiokeratoma?
Hyperkeratosis, acanthosis, ectatic, thin-walled vessels that are in contact w/ the epi.
when you see these think a bloody looking seborrheic keratosis
What are the 5 types of angiokeratoma?
- Angiokeratoma of Mibelli: 10-15 y/o; fingers and toes
- Angiokeratoma of Fordyce: older men (scrotum) or women (vulva)
- Angiokeratoma corporis diffusum: multiple lesions in childhood/adolescence; bathing suite distribution; a/w Fabry disease and other enzyme deficiencies
- Angiokeratoma circumscriptum: In children, F>M, aggregates of lesions forming plaques
- Solitary and multiple angiokeratomas: children and adults; can arise anywhere; can be related to chronic irritation or trauma of superficial dermal vessels
What type of angiokeratomas is a/w Fabry’s disease?
Angiokeratoma corporis diffusum: look for multiple lesions in childhood or adolescence in a bathing suit distribution
What is the clinical description of lymphangioma?
Frog spawn clinically
These are often noted to come and go somewhat
What stains are used for vascular/lymphatic endothelium?
CD31, CD34, PAL-E, factor VIII, Ulex
What stains are used for lymphatic only tumors?
VEGFR-3, poloplanin, LYVE-1, prox-1; D2-40, thrombomodulin
Do vascular malformations like nevus flameus ever go away?
These can fade (forehead and central face), however, are largely permanent
Grows in proportion to the child, does not spontaneous involute
What is the clinical presentation of venous malformations?
These are congenital (cavernous hemangioma). They appear as blue-purple papules on nodules on infants.
They don’t have a proliferative phase and do not regress over time like capillary hemangiomas
What is the histology of venous malformations?
Large dilated vascular channels with flat endothelium in the deep dermis/subcutis. You can see thrombosis and calcifications of the walls.
What conditions are venous malformations associated with?
Blue rubber bleb nevus syndrome and Maffucci’s syndrome (enchondromas of bone, chondrosarcoma risk, spindle-cell hemangioendothelioma, may be due to parathyroid receptor mutation in some cases)
What are the most common locations for glomerulovenous malformation (glomangioma)?
Most commonly located on the extremities and trunk
It presents as painless, blue, or violaceous macules, nodules, or plaques
What is the histology of glomangioma?
Think lots of vessels with one to two layers of glomus cells around the prominent vessels. So when you look the vessels look thicker because they have the glomus cells
As compared to glomus tumors, these are mostly vessels with some glomus cells, whereas the glomus tumors are tumors of glomus cells with some surrounding inconspicuous vessels
What are the most common locations for lymphangioma cicumscriptum?
Proximal limbs, girdle
What is the clinical presentation of deep lymphangiomas?
These are soft swellings int he skin, epidermis should be normal (deep)
Variants included: cavernous lymphangioma, cystic hygroma
What syndromes are deep lymphangiomas associated with?
Cystic hydromas (lymphangiomas) are seen in the neck and axilla area in turner’s syndrome, down syndrome, Noonan’s syndrome
What is the clinical description of verrucous hemangioma?
They occur at birth to childhood
Most commonly located on the legs
They appear as bluish-red soft papules, plaques or nodules that become wart-like, may develop satellite lesions
If removed they frequently recur due to deep component
What is the histology of verrucous hemangioma?
Numerous small and large vessels in dermis and subcutis, irregular papillomatosis and overlying verrucous hyperplasia changes
What is the clinical presentation of venous lakes?
Elderly, occur on the ear, face, lip, neck
What is the histology of a venous lake?
Single large dilated vascular channel in the upper dermis, thin fibrous wall and flat endothelial lining; thrombus; sun-damaged skin