Transcript Dermal and Subcutaneous Tumors

Dermal and Subcutaneous
Tumors
Erik Austin, D.O., M.P.H.
Cutaneous Vascular Anomalies
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Hamartomas
Malformations
Dilation of preexisting vessels
Hyperplasias
Benign neoplasms
Malignant neoplasms
Hamartomas
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Characterized by an abnormal arrangement of
tissues normally present.
Phakomatosis Pigmentovascularis – presenting as
vascular malformations (hamartomas) + melanocytic
or epidermal nevi.
Phakomatosis
Pigmentovascularis
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Type I: nevus flammeus + epidermal nevus
Type II: nevus flammeus + aberrant mongolian
spots
Type III: nevus flammeus + nevus spilus
Type IV: nevus flammeus + nevus spilus +
ectopic mongolian spots
Phakomatosis
Pigmentovascularis
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Typically, affects Asians
Systemic findings may include: intracranial
and visceral anomalies, visceral vascular
anomalies, ocular abnormalities, and hemihypertrophy of the limbs.
Type II = most common
Eccrine Angiomatous
Hamartoma
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Benign, slow growing, solitary, bluish nodule
on the palms, soles or extremities
Presents at birth or in early childhood
Often painful – when touched may develop
beads of perspiration (hyperhidrosis)
Histo: lobules of mature eccrine glands and
ducts with thin-walled blood vessels
Malformations
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Definition: 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 or heat
Normal amount of melanin
Occur due to increased sensitivity of the blood
vessels to catecholamines
Associations: neurofibromatosis, tubercular
sclerosis, phakomatosis pigmentovascularis
Cutis Marmorata Telangiectatica
Congenita
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Presents as a purplish, reticulated vascular
network pattern (referred to as livedo
reticularis) – extremities, trunk, face, scalp
Telangiectasis and superficial ulcerations
occur, but improve with age
Associations: varicosities, nevus flammeus,
hypoplasia and hypertrophy of soft tissue and
bone
Tx: none; regress with time
Cutis Marmorata Telangectatica
Congenita
Nevus Flammeus (Port Wine Stain)
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Pink, red, or wine colored macules or patches
Congenital malformation of skin
Histo: dilated capillaries in dermis
“stork bite” = Nevus flammeus nuchae
“salmon patch” = glabellar region or upper
eyelid
Rarely involutes
Nevus Flammeus “Salmon
Patch”
Nevus Flammeus
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Sturge-Weber Syndrome:
Nevus flammeus associated with seizures,
trigeminal angiomatosis, ipsilateral intracranial
calcifications, contralateral hemiparesis and
ocular abnormalities. Tx: Neuro, Ophthalmo,
Dental consults.
Sturge-Weber Syndrome
Nevus Flammeus
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Klippel-Trenaunay Syndrome:
Nevus Flammeus associated with venous
varicosities and overgrowth of soft tissue and
bone. Tx: support stockings; ortho consult.
Klippel-Trenaunay Syndrome
Nevus Flammeus
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Beckwith-Wiedemann Syndrome:
port-wine stain associated with macrosomia,
macroglossia, and omphalocele
Cobb Syndrome: (cutaneous meningospinal
angiomatosis) port-wine stain associated with
a dermatome supplied by a segment of the
spinal cord, kyphoscoliosis, neurologic, GI,
urologic, and skeletal abnormalities
Cobb Syndrome
Nevus Flammeus
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Proteus Syndrome: facial port-wine stain,
hemihypertrophy, macrodactyly, verrucous
epidermal nevus, soft-tissue subcutaneous
masses, and cerebriform overgrowth of the
plantar surface
Robert’s Syndrome: facial port-wine stain,
hypomelia, hypotrichosis, growth retardation,
cleft lip
Nevus Flammeus
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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
Associated with recurrent thrombophlebitis and
calcified phleboliths
Pressure on surrounding structures (nerves)
Tx: U/S, MRI studies; vascular sx consult
Venous Malformation
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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
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Maffucci’s syndrome: (dyschondroplasia
with hemangiomata) uneven bone growth,
frequent fractures, nodules on small bones in
puberty and later on long bones
Degeneration of the sacrum in 50%
Nonhereditary
Maffucci’s syndrome
Venous Malformations
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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
Affects trunk and arms; associated w/nocturnal
pain
GI hemangiomas in small bowel may rupture
Blue rubber bleb nevus syndrome
Venous Malformation
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Gorham’s disease: cutaneous and osseus
venous and lymphatic malformations
Massive Osteolysis ~ “Disappearing Bones”
Arteriovenous Fistulas
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Route from artery to vein that bypasses the
capillary bed. Congenital or aquired.
Osler-Weber-Rendu: (hereditary hemorrhagic
telangectasia) internal AV fistulas
Acquired or secondary to trauma (HD access)
Osler-Weber-Rendu
Arteriovenous Fistulas
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Skin over fistulas may be warmer,
hypertrichosis, thrills and bruits, stasis, edema,
parasthesias
Psuedo-Kaposi’s sarcoma: (Bluefarb-Stewart
syndrome) reddish purple nodules or a plaque,
2nd or 3rd decade
Tx: embolization, surgery
Superficial Lymphatic
Malformation
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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
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Cystic Hygroma: neck axilla, groin, and oral
cavity
Deep-seated, multilocular masses
Cytogenic analysis of children with cystic
hygromas to detect aneuploidy XO
Tx: transvaginal U/S; may reoccur after
surgery due to depth of invasion
Dilation of Preexisting Vessels
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Spider Angioma: ascending central arteriole
on the face, neck, upper trunk and upper
extremities
Young children and pregnant women most
frequent.
Childhood lesions usually involute.
Vascular spiders associated w/cirrhosis, HepC,
liver dysfunction, (elevated estrogen levels)
Spider Angioma
Venous Lakes
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(phlebectases) small, dark, blue, slightly
elevated 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
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Flesh-colored, solitary lesions resembling an
intradermal nevus
May suddenly enlarge and become blue-black,
surrounded by a zone of erythema
Histo: thrombotic, dilated capillaries below
the epidermis
Tx: excision
Telangiectasia
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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 damage
Telangiectasia
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Radiodermatitis
Xeroderma
pigmentosum
Lupus erythematosus
Dermatomyositis
Scleroderma
CREST
rosacea
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Liver disease
Poikiloderma
BCC
Sarcoid
SLE
Pregenacy
Osler-Weber-Rendu
Etc.
Generalized Essential
Telangiectasia
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Women in forties; not associated with systemic
disease
Dilation of veins and capillaries over large
area of body
Legs, arms, trunk, entire body
Dilations persist indefinitely
Tx: unsuccessful
Angiokeratomas
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Telangiectasias that have an overlying
hyperkeratotic surface.
Dilatations 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“telangiectatic warts”
Patients often have cold, cyanotic hands and
feet
Angiokeratoma of Mibeli
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Rare genodermatosis, autosomal dominant,
family history of chilblains
DDx: APACHE (acral pseudolymphomatous
angiokeratoma in children); unilateral,
spontaneous, no cold sensitivity,
lymphohistiocytic inflitrate
Tx: electrocautery, CO2 laser, cryotx
Angiokeratoma of the Scrotum
(Fordyce)
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Small vascular papules that stud the scrotum,
middle-aged or elderly, urethra, clitoral, and
vulvar lesions
Tx: Laser, fulguration, reassurance
Solitary Angiokeratoma
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Single small, bluish black, warty papule,
mainly on lower extremities
Probably follows trauma
Tx: removal
Lymphangiectasis
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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
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Also, seen in benign disease which scar
lymphatics, i.e., scrofula and erysipelas
Chronic high potency steroid use can induce
lymphagiectases
Lesions are thick-walled, translucent 2-5mm
white vesicles
May have chylous discharge
Tx: underlying cause
Hyperplasias
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Angiolymphoid Hyperplasia with Eosinophilia
Pyogenic Granuloma
Angiolymphoid Hyperplasia with
Eosinophilia (AHLE)
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Pink to red-brown, dome-shaped, dermal
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
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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
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Small, solitary, sessile or pedunculated,
rasberry-like vegitation of exuberant
granulation tissue
Exposed surface secondary 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
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Reactive hyperplasia of endothelial cells may
occur in the dermis, subcutis, or
intramuscularly.
Mimics angiosarcoma, red or purplish 5mm5cm papules or nodules on the head, neck, or
upper extremities.
A response to intravascular thrombosis.
Tx: excision
Benign Neoplasms
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Angioma Serpiginosum
Infantile Hemangioma (Strawberry
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
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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
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DDx: Progressive pigmentary disease of
Schamburg; cayenne pepper spots coalesce
Purpura annularis telangiectoides: bilateral,
acute outbreaks of telangiectatic points that
spread peripherally
Histo: dilated and tortuous capillaries in the
dermal papillae. No inflammatory infiltrate.
Infantile Hemangioma
(Strawberry Hemangioma)
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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
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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, telangiectasia, or
anetoderma-type redundancy
Infantile Hemangioma
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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
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“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
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Pulsed dye laser can help the residual
involuted lesions with residual telangiectasias
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
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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
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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 alfa-2a
or –2b good response in 80%
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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
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Tx: Laser, electrodessication
Targetoid Hemosiderotic
Hemangioma
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Acquired hemangiomas in young to middle
age, trunk, extremities
Central brown or violaceous papule
surrounded by an ecchymotic halo
Likely represent trauma to a preexisting
hemangioma with thrombosis and subsequent
recanalization
Microvenular Hemangioma
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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)
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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
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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
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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
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KHE; is an uncommon vascular tumor that
affects infants and young children
Frequently occurs in the retroperitoneum
May present as multinodular 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
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KHE is locally aggressive, may be
complicated by platelet trapping and
consumptive coagulopathy (Kasabach-Merritt
syndrome)
Histo: combined features of cellular infantile
hemangioma and Kaposi’s sarcoma.
Kaposiform
Hemangioendothelioma
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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)
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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)
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Spleenomegaly, consumptive coagulopathy
with decrease in Hgb, platelets, fibrinogen,
Factors II, V, and VII. Increased pt/ptt.
Mortality 30%
Usually a self-limited disorder.
Glomus Tumor
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Skin-colored or slightly dusky blue firm
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
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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
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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: endothelium-lined tubes and sprouts filled
with blood and surrounded by cells with oval or
spindle-shaped nuclei (pericytes)
Tx: wide local excision
Hemangiopericytoma
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Malignant Hemangiopericytomas:
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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
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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
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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