1. Dermal and Subcutaneous Tumors
David M. Bracciano, D.O.

2. Cutaneous Vascular Anomalies
Hamartomas
Malformations
Dilation of preecisting vessels
Hyperplasias
Benign neoplasms
Malignant neoplasms

3. Hamartomas
Characterized by an abnormal arrangement of tissues normally present.

4. Phakomatosis Pigmentovascularis
Vascular malformations (Hamartomas) and melanocytic or epidermal nevi.

5. Phakomatosis Pigmentovascularis

6. 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

7. Phakomatosis Pigmentovascularis

8. 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

9. 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

10. Eccrine Angiomatous Hamartoma
Histo: lobules of mature eccrine glands and ducts with thin-walled blood vessels
Benign and slow growing

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

12. 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

15. 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

16. Cutis Marmorata Telangectatica Congenita

17. 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

18. Nevus Flammeus “Salmon Patch”

19. 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

20. 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

21. Sturge-Weber Syndrome

22. Nevus Flammeus
Klippel-Trenaunay Syndrome; port-wine malformations in association with deep venous system malformations, superficial varicosities, bony and soft tissue hypertrophy

23. Klippel-Trenaunay Syndrome

24. 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

25. Cobb Syndrome

26. 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

28. 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

29. Nevus Flammeus

30. Nevus Flammeus Tx: Flashlamp pumped pulsed dye laser
Localizes heat within ectatic vessels
450microsecond pulse
577 or 585nm

31. 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)

34. Venous Malformation Consumptive coagulopathy
Persistant, not amenable to laser or surgical tx due to deep component

35. 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

36. 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

37. Maffucci’s syndrome

38. 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

39. Blue rubber bleb nevus syndrome

40. 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

41. 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

42. Klippel-Trenaunay Syndrome

43. Klippel-Trenaunay Syndrome

44. 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

45. Osler-Weber-Rendu

46. 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

47. 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

48. 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

49. 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)

50. Spider Angioma

51. 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)

53. 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

54. 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

55. Telangectasia Radiodermatitis Xeroderma pigmentosum
Lupus erythematosus
Dermatomyositis
Scleroderma
CREST
rosacea
Liver disease
Poikiloderma
BCC
Sarcoid
SLE
Pregenacy
Osler-Weber-Rendu
Etc.

56. 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

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

58. Angiokeratomas

59. 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

60. 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

61. 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

62. Solitary Angiokeratoma
Single small, bluish black, warty papule, mainly on lower extremities
Probably follows trauma.
Tx: removal

63. 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)

64. 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

65. Hyperplasias Angiolymphoid Hyperplasia with Eosinophilia
Pyogenic Granuloma

66. 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

67. 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

68. 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

69. Pyogenic Granuloma

70. Pyogenic Granuloma

71. 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

72. Benign Neoplasms Angioma Serpiginosum
Infantile Hemangioma (Strawberry Hemangioma)
Cherry Angioma
Targetoid Hemosiderotic Hemangioma
Microvenular Hemangioma
Tufted Angioma

73. Benign Neoplasms Glomeruloid Hemangioma Kasabach-Merritt Syndrome
Acquired Progressive Lymphangioma
Glomus Tumor
Hemangiopericytoma
Proliferating Angioendotheliomatosis

74. 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

75. 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

76. 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

79. 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

80. 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

81. 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.

82. 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

83. 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

84. 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%

85. 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

86. Cherry Angiomas
Tx: Laser, electrodesication

87. 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

88. 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

89. 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

90. Tufted Angioma

91. 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)

92. 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)

93. 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

94. 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.

95. 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

96. 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

97. 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.

98. 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

99. 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

100. Glomus Tumor

101. 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

102. 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.

103. Hemangiopericytoma

104. 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.

105. Proliferating Angioendotheliomatosis

106. 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