Dermal and Subcutaneous Tumors

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Dermal and Subcutaneous Tumors

• David M. Bracciano, D.O.

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Cutaneous Vascular Anomalies

• Hamartomas
• Malformations
• Dilation of preecisting vessels
• Hyperplasias
• Benign neoplasms
• Malignant neoplasms

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Hamartomas

• Characterized by an abnormal arrangement of
  tissues normally present.

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Phakomatosis Pigmentovascularis

• Vascular malformations (Hamartomas) and
  melanocytic or epidermal nevi.

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Phakomatosis Pigmentovascularis
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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

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Phakomatosis Pigmentovascularis
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Phakomatosis Pigmentovascularis

• Nearly all pts are Asian
• Systemic findings may include intracranial and
  visceral anomalies and visceral vascular
  anomalies, ocular abnormalities, and
  hemi-hypertrophy of the limbs.
• Type II most common

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Eccrine Angiomatous Hamartoma

• Solitary nodular lesion, palms and soles, 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

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Eccrine Angiomatous Hamartoma

• Histo lobules of mature eccrine glands and
  ducts with thin-walled blood vessels
• Benign and slow growing

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Malformations

• Abnormal structures that result from an
  aberration in embryonic development
• Functional Nevus Anemicus
• Anatomic capillary, venous, arterial,
  lymphatic, or combined

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Nevus Anemicus

• 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

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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

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Cutis Marmorata Telangectatica Congenita
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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

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Nevus Flammeus Salmon Patch
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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

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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

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Sturge-Weber Syndrome
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Nevus Flammeus

• Klippel-Trenaunay Syndrome port-wine
  malformations in association with deep venous
  system malformations, superficial varicosities,
  bony and soft tissue hypertrophy

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Klippel-Trenaunay Syndrome
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Nevus Flammeus

• Beckwith-Wiedemann Syndrome facial port-wine
  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

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Cobb Syndrome
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Nevus Flammeus

• Proteus Syndrome facial port-wine,
  hemihypertrophy, macrodactyly, verrucous
  epidermal nevus, soft-tissue subcutaneous masses,
  and cerebriform overgrowth of the plantar
  surface.
• Roberts Syndrome facial port-wine, hypomelia,
  hypotrichosis, growth retardation, cleft lip

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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

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Nevus Flammeus
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Nevus Flammeus

• Tx Flashlamp pumped pulsed dye laser
• Localizes heat within ectatic vessels
• 450microsecond pulse
• 577 or 585nm

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Venous Malformation

• 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)

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Venous Malformation

• Consumptive coagulopathy
• Persistant, not amenable to laser or surgical tx
  due to deep component

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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

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Venous Malformation

• Maffuccis 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

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Maffuccis syndrome
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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

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Blue rubber bleb nevus syndrome
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Venous Malformation

• Gorhams disease cutaneous and osseus venous
  and lymphatic malformations
• Massive osteolysis, Disappearing Bones usually
  only one bone involved with replacement of bone
  with fibrous tissue

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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

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Klippel-Trenaunay Syndrome
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Klippel-Trenaunay Syndrome
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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

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Osler-Weber-Rendu
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Arteriovenous Fistulas

• Skin over fistulas may be warmer,
  hypertrichosis, thrills and bruits, stasis,
  edema, parasthesias
• Psuedo-Kaposis sarcoma (Bluefarb-Stewart
  syndrome) reddish purple nodules or a plaque,
  2nd or 3rd decade
• Tx embolization, surgery

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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

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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

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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)

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Spider Angioma
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Venous Lakes

• (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)

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Capillary Aneurysms

• Flesh-colored solitary lesions, resemble an
  intradermal nevus
• May suddenly enlarge and become blue-black,
  surrounded by zone of erythema, resembling a
  melanoma
• Histo thrombotic, dilated capilaries below the
  epidermis
• Tx excision

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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

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Telangectasia

• Radiodermatitis
• Xeroderma pigmentosum
• Lupus erythematosus
• Dermatomyositis
• Scleroderma
• CREST
• rosacea

• Liver disease
• Poikiloderma
• BCC
• Sarcoid
• SLE
• Pregenacy
• Osler-Weber-Rendu
• Etc.

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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

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Angiokeratomas

• Telangectasias that have an overlying
  hyperkeratotic surface. Dilations of preexisting
  papillary dermal vessels.

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Angiokeratomas
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Angiokeratoma of Mibeli

• 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

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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

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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

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Solitary Angiokeratoma

• Single small, bluish black, warty papule, mainly
  on lower extremities
• Probably follows trauma.
• Tx removal

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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)

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Lymphangiectasis

• Also seen in benign disease which scar lymphatics
  ie scrofula, 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

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Hyperplasias

• Angiolymphoid Hyperplasia with Eosinophilia
• Pyogenic Granuloma

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Angiolymphoid Hyperplasia with Eosinophilia (AHLE)

• 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

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AHLE

• Ddx Kimuras disease
• Kimuras inflammatory disorder seen in young
  Asian men. Massive subcutaneous swelling in the
  periauricular and submandibular region
• Histo eosinophils in lesions
• Lymphadenopathy, elevated IgE

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Pyogenic Granuloma

• Small, solitary, sessile or pedunculated,
  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

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Pyogenic Granuloma
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Pyogenic Granuloma
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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

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Benign Neoplasms

• Angioma Serpiginosum
• Infantile Hemangioma (Strawberry Hemangioma)
• Cherry Angioma
• Targetoid Hemosiderotic Hemangioma
• Microvenular Hemangioma
• Tufted Angioma

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Benign Neoplasms

• Glomeruloid Hemangioma
• Kasabach-Merritt Syndrome
• Acquired Progressive Lymphangioma
• Glomus Tumor
• Hemangiopericytoma
• Proliferating Angioendotheliomatosis

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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

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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

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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

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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

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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

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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.

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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

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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

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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 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

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Cherry Angiomas

• Tx Laser, electrodesication

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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

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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 Kaposis sarcoma

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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

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Tufted Angioma
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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)

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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)

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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

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Kaposiform Hemangioendothelioma

• KHE is locally aggressive, may be complicated by
  platlet trapping and consumptive coagulopathy
  (Kasabach-Merritt syndrome)
• Histo combined features of cellular infantile
  hemangioma and Kaposis sarcoma.

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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

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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

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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.

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Glomus Tumor

• 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

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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

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Glomus Tumor
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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

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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.

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Hemangiopericytoma
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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.

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Proliferating Angioendotheliomatosis
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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