Transcript Dermal and Subcutaneous Tumors

Dermal and Subcutaneous
Tumors
David M. Bracciano, D.O.
Cutaneous Vascular Anomalies
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Hamartomas
Malformations
Dilation of preecisting vessels
Hyperplasias
Benign neoplasms
Malignant neoplasms
Hamartomas
• Characterized by an abnormal arrangement
of tissues normally present.
Phakomatosis
Pigmentovascularis
• Vascular malformations (Hamartomas) and
melanocytic or epidermal nevi.
Phakomatosis
Pigmentovascularis
Phakomatosis
Pigmentovascularis
• Type I: nevus flammeus and an epidermal
nevus
• Type II: nevus flammeus with aberrant
mongolian spots
• Type III: nevus flammeus with nevus spilus
• Type IV: nevus flammeus, ectopic
mongolian spots and nevus spilus
Phakomatosis
Pigmentovascularis
Phakomatosis
Pigmentovascularis
• Nearly all pts are Asian
• Systemic findings may include; intracranial
and visceral anomalies and visceral vascular
anomalies, ocular abnormalities, and hemihypertrophy of the limbs.
• Type II most common
Eccrine Angiomatous
Hamartoma
• Solitary nodular lesion, palms and soles,
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acral areas of extremities
Birth or early childhood
Often pain and hyperhidrosis
Dome-shaped bluish hemangioma
1-2cm, when touched develop characteristic
beads of perspiration
Eccrine Angiomatous
Hamartoma
• Histo: lobules of mature eccrine glands and
ducts with thin-walled blood vessels
• Benign and slow growing
Malformations
• Abnormal structures that result from an
aberration in embryonic development
• Functional: Nevus Anemicus
• Anatomic: capillary, venous, arterial,
lymphatic, or combined
Nevus Anemicus
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Congenital pale macules
Cannot be made red by trauma, cold, heat
Normal amount of melanin
Increased sensitivity of the blood vessels to
catacholamines
• May occur occur in neurofibromatosis,
tubercular sclerosis, phakomatosis
pigmentovascularis
Cutis Marmorata Telangectatica
Congenita
• Purplish, reticulated vascular network
• Phlebectasia, telangiectasia, and at times
ulcerations, extremities
• Associated with varicosities, nevus
flammeus, hypoplasia and hypertrophy of
soft tissue and bone
• No tx required, may regress with time
Cutis Marmorata Telangectatica
Congenita
Nevus Flammeus
• “stork bite” Nevus flammeus nuchae;
congenital capillary malformation of skin
• 25% of newborns
• Persists in 5%
• “salmon patch”; glabellar region or upper
eyelid present in 15% of newborns
Nevus Flammeus
“Salmon Patch”
Nevus Flammeus
• “Port Wine Stain”; 0.3% of births
• Small red macules to large red patches
partially or completely blanched by
diascopic pressure
• Usually unilateral on face and neck
• Mucous membrane of mouth may be
involved
Nevus Flammeus
• Often becomes bluish or purple with age
• Rarely involute
• Sturge-Weber Syndrome;
encephalotrigeminal angiomatosis, occurs
in 10% of patients with CNVI involvement,
epilepsy, hemiplegia, homonymous
hemianopsia, calcifications of cerebral
cortex
Sturge-Weber Syndrome
Nevus Flammeus
• Klippel-Trenaunay Syndrome; port-wine
malformations in association with deep
venous system malformations, superficial
varicosities, bony and soft tissue
hypertrophy
Klippel-Trenaunay Syndrome
Nevus Flammeus
• Beckwith-Wiedemann Syndrome; facial portwine stain, macroglossia, omphalocele, visceral
hyperplasia, hemihypertrophy, and hypoglycemia.
• Cobb Syndrome; (cutaneous meningospinal
angiomatosis) port-wine or other vascular
malformation found in association with a
dermatome supplied by a segment of the spinal
cord, kyphoscoliosis, neurologic, GI, urologic, and
skeletal abnormalities
Cobb Syndrome
Nevus Flammeus
• Proteus Syndrome; facial port-wine,
hemihypertrophy, macrodactyly, verrucous
epidermal nevus, soft-tissue subcutaneous
masses, and cerebriform overgrowth of the
plantar surface.
• Robert’s Syndrome; facial port-wine,
hypomelia, hypotrichosis, growth
retardation, cleft lip
Nevus Flammeus
• Wyburn-Mason Syndrome; unilateral
retinal arteriovenous malformation
associated with ipsilateral port-wine stain
near the affected eye.
• Tar Syndrome; congenital
thrombocytopenia, bilateral absence or
hypoplasia, and port-wine stain
Nevus Flammeus
Nevus Flammeus
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Tx: Flashlamp pumped pulsed dye laser
Localizes heat within ectatic vessels
450microsecond pulse
577 or 585nm
Venous Malformation
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Aka: cavernous hemangioma
Congenital malformation of veins
Round, bright red or purple, spongy nodules
Often on head and neck, mucous membranes
Usually a deep component
Recurrent thrombophlebitis, calcified phleboliths
Pressure on surrounding structures (nerves)
Venous Malformation
• Consumptive coagulopathy
• Persistant, not amenable to laser or surgical
tx due to deep component
Venous Malformation
• Bannayan-Riley-Ruvalcaba Syndrome;
cutaneous and visceral venous, capillary, and
lymphatic malformations, macrocephaly,
pseudopapilledema, systemic lipoangiomatosis,
spotted pigmentation of the penis, hamartomatous
intestinal polyps, and rarely trichilemmomas.
• Autosomal dominant
Venous Malformation
• Maffucci’s syndrome; (dyschondroplasia
with hemangiomata) uneven bone growth
with frequent fractures, nodules on small
bones in puberty and later on long bones
• Degeneration of the sacrum in 50%
• Venous malformations of the skin and
mucous membranes
• Nonhereditary
Maffucci’s syndrome
Venous Malformations
• Blue rubber bleb nevus syndrome: cutaneous and
gastrointestinal venous malformations
• Skin lesions have a cyanotic, bluish appearance
with a soft, elevated, nipplelike center
• Emptied by firm pressure, trunk and arms,
nocturnal pain
• GI hemangiomas esp in small bowel may rupture
Blue rubber bleb nevus
syndrome
Venous Malformation
• Gorham’s disease; cutaneous and osseus
venous and lymphatic malformations
• Massive osteolysis, “Disappearing Bones”
usually only one bone involved with
replacement of bone with fibrous tissue
Venous Malformations
• Klippel-Trenaunay Syndrome: nevus
flammeus, varicose veins and venous
malformations, soft-tissue hypertrophy of
the affected extremity
• Involved limb is usually larger and longer
than normal
Klippel-Trenaunay Syndrome
Klippel-Trenaunay Syndrome
Arteriovenous Fistulas
• Route from artery to vein, bypassing the
capillary bed. Congenital or aquired.
• Osler-Weber-Rendu; (hereditary
hemorrhagic telangectasia) internal AV
fistulas
• Acquired; secondary to trauma, made for
hemodialysis access
Osler-Weber-Rendu
Arteriovenous Fistulas
• Skin over fistulas may be warmer,
hypertrichosis, thrills and bruits, stasis,
edema, parasthesias
• Psuedo-Kaposi’s sarcoma; (BluefarbStewart syndrome) reddish purple nodules
or a plaque, 2nd or 3rd decade
• Tx: embolization, surgery
Superficial Lymphatic
Malformation
• Groups of deep-seated, vesicle-like papules
resembling frog spawn. Exude clear lymph
when ruptured
• Abdomen, axillae, mouth and tongue
• Blood and lymph elements may be present
changing color from clear to purple
• Tx; MRI to r/o deep component, CO2 laser
if superficial
Cystic Lymphatic Malformation
• Cystic Hygroma; neck axilla, groin, and
oral cavity
• Deep-seated, multilocular masses
• Cytogenic analysis of children with cystic
hygromas to detect aneuploidy XO
• Tx: tranvaginal US, may reoccur after
surgery due to depth of invasion
Dilation of Preexisting Vessels
• Spider Angioma; ascending central
arteriole, face, neck, upper trunk and upper
extremities
• Young children and pregnant women most
frequent. Childhood lesions usually
involute.
• Vascular spiders: cirrhosis, HepC, liver
dysfunction, (elevated estrogen levels)
Spider Angioma
Venous Lakes
• (phlebectases) small, dark, blue, slightly elevated
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blebs, easily compressed
Face, ears, lips, neck, forarms, back of hands
Manifestations of actinic damage
Markedly dilated, blood-filled spaces that are
lined with thin, elongated endothelial cells
Tx: cautery, Liquid Nitrogen, laser ablation
(532nm laser)
Capillary Aneurysms
• Flesh-colored solitary lesions, resemble an
intradermal nevus
• May suddenly enlarge and become blueblack, surrounded by zone of erythema,
resembling a melanoma
• Histo: thrombotic, dilated capilaries below
the epidermis
• Tx: excision
Telangectasia
• Dilated cutaneous blood vessel; venule,
capillary, or arteriole
• Fine linear vessels coursing on the surface
of the skin
• Normal skin at any age, increased in areas
of actinic of weather exposure
Telangectasia
• Radiodermatitis
• Xeroderma
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pigmentosum
Lupus erythematosus
Dermatomyositis
Scleroderma
CREST
rosacea
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Liver disease
Poikiloderma
BCC
Sarcoid
SLE
Pregenacy
Osler-Weber-Rendu
Etc.
Generalized Essential
Telangectasia
• Women in forties, not associated with
systemic disease
• Dilation of veins and capillaries over a large
segment of the body without other skin
lesions: legs, arms, trunk, entire body
• Dilations persist indefinitely
• Tx: unsuccessful
Angiokeratomas
• Telangectasias that have an overlying
hyperkeratotic surface. Dilations of
preexisting papillary dermal vessels.
Angiokeratomas
Angiokeratoma of Mibeli
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1-5 mm red vascular papules
Become hyperkeratotic
Dorsum of fingers, toes, elbows, and knees
Surface becomes hyperkeratotic and
verrucous aka; “telangectatic warts”
• Patients often have cold, cyanotic hands and
feet
Angiokeratoma of Mibeli
• Rare genodermatosis, autosomal dominant,
family history of chilblains
• Ddx: APACHE (acral
pseudolymphomatous angiokeratoma in
children); unilateral, spontaneous, no cold
sensitivity, lymphohistiocytic inflitrate
• Tx: electrocautery, CO2laser, cryotx
Angiokeratoma of the Scrotum
(Fordyce)
• Small vascular papules that stud the
scrotum, middle-aged or elderly, urethra,
clitoral, and vulvar lesions
• Tx: Laser, fulguration, reassurance
Solitary Angiokeratoma
• Single small, bluish black, warty papule,
mainly on lower extremities
• Probably follows trauma.
• Tx: removal
Lymphangiectasis
• Acquired dilations of lymph vessels that result
from destruction or obstruction of lymphatic
drainage
• Arms, axillae, chest, and back after node
dissection and RadTx for breast CA
• Scrotum, penis, thighs after tx for prostate CA
• May be presenting sign of CA (obstruction of
lymphatic in an extremity)
Lymphangiectasis
• Also seen in benign disease which scar
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lymphatics ie; scrofula, erysipelas
Chronic high potency steroid use can induce
lymphagiectases
Lesions are thick-walled, translucent 25mm white vesicles
May have chylous discharge
Tx; underlying cause
Hyperplasias
• Angiolymphoid Hyperplasia with
Eosinophilia
• Pyogenic Granuloma
Angiolymphoid Hyperplasia with
Eosinophilia (AHLE)
• Pink to red-brown, dome-shaped, dermal
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papules or nodules of the head or neck
Also mouth trunk, extremities, penis, and
vulva
Grouped lesion form plaques or clusters
May occur after trauma, arteriovenous shunt
Tx; excision, pulsed dye laser
AHLE
• Ddx: Kimura’s disease
• Kimura’s inflammatory disorder seen in
young Asian men. Massive subcutaneous
swelling in the periauricular and
submandibular region
• Histo; eosinophils in lesions
• Lymphadenopathy, elevated IgE
Pyogenic Granuloma
• Small, solitary, sessile or pedunculated,
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rasberry-like vegitation of exuberant
granulation tissue; “proud flesh”
Exposed surface, due to trauma
Granuloma Gravidarum; gingiva
Tx: fulguration, dermal curette, laser
May be due to Isotretinoin or indinavir
Pyogenic Granuloma
Pyogenic Granuloma
Intravascular Papillary
Endothelial Hyperplasia
• Reactive hyperplasia of endothelial cells
may occur in the dermis, subcutis, or
intramuscularly.
• Mimics angiosarcoma, red or purpulish
5mm-5cm papules or nodules on the head,
neck, or upper extremities.
• A response to intravascular thrombosis.
• Tx: excision
Benign Neoplasms
• Angioma Serpiginosum
• Infantile Hemangioma (Strawberry
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Hemangioma)
Cherry Angioma
Targetoid Hemosiderotic Hemangioma
Microvenular Hemangioma
Tufted Angioma
Benign Neoplasms
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Glomeruloid Hemangioma
Kasabach-Merritt Syndrome
Acquired Progressive Lymphangioma
Glomus Tumor
Hemangiopericytoma
Proliferating Angioendotheliomatosis
Angioma Serpiginosum
• Minute, copper-colored to red angiomatous
puncta that have a tendency to become
papular. Occur in groups. New lesions
occur at periphery with central clearing.
• Lower extremities most common, may
affect any area except palms and soles
• 90% in girls under 16 years
• Tx: pulsed dye laser
Angioma Serpiginosum
• Ddx: Progressive pigmentary disease of
Schamburg; cayenne pepper spots coalesce
• Purpura annularis telangiectodes; bilateral,
acute outbreaks of telangectatic points that
spread peripherally
• Histo: dilated and tortuous capillaries in the
dermal papillae. No inflammatory infiltrate
Infantile Hemangioma
(Strawberry Hemangioma)
• Most common benign tumor of childhood
• Present at birth in 30%
• Remainder appear rapidly in an
inconspicuous macule at 2 weeks to 2
months
• 60% on the head and neck
• Dome-shaped lesion, dull red, white streaks
when involution occurs
Infantile Hemangioma
• Lesions have sharp borders, they are soft
and easily compressed.
• Tend to grow over the first year, remain
stable, and then involute over months to
years. 10% involution rate per year.
• Skin may appear normal after involution
• Commonly atrophy, telangectasia, or
anetoderma-type redundancy
Infantile Hemangioma
• 7% may be associated with structural
malformations
• PHACE syndrome: Posterior fossa brain
malformations (Dandy-Walker),
Hemangiomas, Arterial anomalies,
Coarctation of aorta, Eye abnormalities
• Hemangiomas tend to be large, facial in
PHACE
Infantile Hemangiomas
• “Strawberry Marks”; composed of
primitive endothelial cells, proliferate
intraluminally, fibrosis becomes pronounced
as involution progresses.
• Tx: in most cases intervention detracts
from the quality of the ultimate cosmetic
result.
Infantile Hemangiomas
Treatment
• Pulsed dye laser can help the residual
involuted lesions with residual
telanfiectasias
• The depth of infantile hemangiomas does
not allow the lasers to be effective in
growing or stable childhood hemangiomas.
• “Cyrano Effect”; bulbous nasal tip
hemangioma, may be treated surgically
Infantile Hemangiomas
Treatment
• Indications for Tx:
• Severe hemorrhage, thrombocytopenia, high
output cardiac failure
• Nasal, laryngeal, oral, auditory, anal,
urinary, and pulmonary obstruction
• Limb dysfunction, occlusion amblyoplia,
astigmatism
Infantile Hemangiomas
Treatment
• Intralesional or oral steroids (2-3mg/kg/d)
• 30% respond, with growth arrested in 3 to
21 days, may require retreatment or chronic
treatment
• 40% will respond later
• 30% will have no response; interferon alfa2a or –2b good response in 80%
Cherry Angiomas
(Senile Angiomas, DeMorgan
Spots)
• Most common Vascular anomaly
• Oval, slightly elevated, 0.5mm, ruby-red
papules
• 30 yrs onset, increase with age, most on the
trunk
• If surrounded by purpuric halo suspect
Amyloidosis
Cherry Angiomas
• Tx: Laser, electrodesication
Targetoid Hemosiderotic
Hemangioma
• Acquired hemangiomas in young to middle
age, trunk, extremities
• Central brown or violaceous papule
surrounded by an exxhymotic halo
• Likely represent trauma to a preexisting
hemangioma with thrombosis and
subsequent recanlization
Microvenular Hemangioma
• Asymptomatic, slowly growing 0.5-2cm
reddish lesion on the forearms or other sites
in young to middle-age adults.
• Elongated blood vessels with small lumina
involve the entire reticular dermis
• Ddx: Kaposi’s sarcoma
Tufted Angioma
(Angioblastoma)
• Develops in infancy or early childhood on
the neck or upper trunk
• Dull-red macules with a mottled appearance
• Histo: clusters of angiomatous tufts and
lobules scattered in the dermis in a so-called
“cannonball” pattern
Tufted Angioma
Glomeruloid Hemangioma
• Benign vascular neoplasm reported with
POEMS syndrome.
• POEMS syndrome: polyneuropathy,
organomegaly (heart, spleen, kidneys),
endocrinopathy, m- protein, skin changes
(hyperpigmentation, hypertrichosis,
thickening, sweating, clubbed nails,
leukonychia, and angiomas)
Glomeruloid Hemangioma
• Histo: microvenular hemangiomas, cherry
angiomas, or glomeruloid hemangiomas:
( ectatic vascular structures with aggregates
of capillary loops within a dilated lumina,
simulating the appearance of a renal
glomerulus)
Kaposiform
Hemangioendothelioma
• KHE; is an uncommon vascular tumor that
affects infants and young children
• Frequently occurs in the retroperitoneum
• May present as mulinodular soft tissue
masses, purpuric macules, plaques, and
multiple telangiectatic papules
• Lesions extend locally and usually involve
the skin, soft tissues, and even bone
Kaposiform
Hemangioendothelioma
• KHE is locally aggressive, may be
complicated by platlet trapping and
consumptive coagulopathy (KasabachMerritt syndrome)
• Histo: combined features of cellular
infantile hemangioma and Kaposi’s
sarcoma.
Kaposiform
Hemangioendothelioma
• Prognosis depends on the depth and
location of the lesion.
• Localized lesions may be excised.
• Excision is usually not possible due to depth
and infiltration.
• Tx: steroids
Kasabach-Merritt Syndrome
(Hemangioma with
Thrombocytopenia)
• Infants, reddish or blue plaque or tumor on
the limb or trunk, lymphatic component
• Infant suddenly develops a painful
violaceous mass in association with purpura
and thrombocytopenia
• Bleeding into the hemangioma or into the
chest or abdominal cavities
Kasabach-Merritt Syndrome
(Hemangioma with
Thrombocytopenia)
• Spleenomegaly, consumptive coagulopathy
with decrease in Hgb, platlets, fibrinogen,
Factors II, V, and VII. Increased pt/ptt.
• Mortality 30%
• Usually a self-limited disorder.
Glomus Tumor
• Skin-colored or slightly dusky blue firm
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nodule 1 to 20mm in diameter
Subungual tumor shows a bluish tinge
through the nail plate
Usually tender, radiating pain when touched
Also on fingers and arms
Diag: MRI
Glomus Tumor
• Histo: numerous vascular lumina lined by a
single layer of flattened endothelial cells.
Peripheral to the endothelial cells are a few
to many layers of glomus cells (smooth
muscle cells that stain with vimentin)
• Tx: complete excision
Glomus Tumor
Hemangiopericytoma
• Non-tender, bluish red tumor that occurs on the
skin or in the subcutaneous tissues on any part of
the body.
• Firm, solitary nodule up to 10cm
• Histo: endolithelium-lined tubes and sprouts filled
with blood and surrounded by cells with oval or
spindle-shaped nuclei (pericytes)
• Tx: WLE
Hemangiopericytoma
• Malignant Hemangiopericytomas:
– 50% of soft tissue masses have Mets
– 20% of skin masses have Mets
– Pulmonary Mets are most common cause of
death
– Exception is tumors in infants, almost always
cutaneous or subcutaneous, and do not
metastasize.
Hemangiopericytoma
Proliferating
Angioendotheliomatosis
• Historically divided into a Reactive involuting
type and a Malignant, rapidly fatal type.
• Malignant type is actually a lymphoma;
Intravascular lymphoma.
• Reactive type is uncommon, occurs in SBE,
Chagas’ disease, pulmonary TB, ASHD. Red to
purple patches/plaques, nodules, ecchymosis of
lower extremities. Involution over 1-2 years.
Proliferating
Angioendotheliomatosis
Proliferating
Angioendotheliomatosis
• Intravascular Lymphoma: rapidly progressive,
death within 10 months. Mean age 55 yrs.
• Reddish, purple plaques, nodules or patches.
Multisystem involvement common (CNS).
Kidney, heart, lung, GI.
• Histo: atypical cells fill the lumen of cutaneous
vessels. Usually B-cell some cases of T-cell
lineage.
• Tx: Doxorubicin