Dermal and Subcutaneous Tumors hemiplegia

					Dermal and Subcutaneous
        Tumors

   David M. Bracciano, D.O.
    Cutaneous Vascular Anomalies
•   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 hemi-
  hypertrophy of the limbs.
• Type II most common
         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
        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
• 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 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
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
•   Tx: Flashlamp pumped pulsed dye laser
•   Localizes heat within ectatic vessels
•   450microsecond pulse
•   577 or 585nm
          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)
        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; (Bluefarb-
  Stewart 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
    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 blue-
  black, 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         •   Liver disease
• Xeroderma               •   Poikiloderma
    pigmentosum           •   BCC
•   Lupus erythematosus   •   Sarcoid
•   Dermatomyositis       •   SLE
•   Scleroderma           •   Pregenacy
•   CREST                 •   Osler-Weber-Rendu
•   rosacea               •   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
• 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
    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
            Hyperplasias
• Angiolymphoid Hyperplasia with
  Eosinophilia
• Pyogenic Granuloma
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
                 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,
    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
    Hemangioma)
•   Cherry Angioma
•   Targetoid Hemosiderotic Hemangioma
•   Microvenular Hemangioma
•   Tufted Angioma
           Benign Neoplasms
•   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 alfa-
  2a 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 (Kasabach-
  Merritt 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
    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

				
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