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Dermal and Subcutaneous Tumors

Dermal and Subcutaneous Tumors. Erik Austin, D.O., M.P.H. Cutaneous Vascular Anomalies. Hamartomas Malformations Dilation of preexisting vessels Hyperplasias Benign neoplasms Malignant neoplasms. Hamartomas. Characterized by an abnormal arrangement of tissues normally present.

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Dermal and Subcutaneous Tumors

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  1. Dermal and Subcutaneous Tumors Erik Austin, D.O., M.P.H.

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

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

  4. Phakomatosis Pigmentovascularis – presenting as vascular malformations (hamartomas) + melanocytic or epidermal nevi.

  5. Phakomatosis Pigmentovascularis • 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

  6. Phakomatosis Pigmentovascularis • Typically, affects Asians • Systemic findings may include: intracranial and visceral anomalies, visceral vascular anomalies, ocular abnormalities, and hemi-hypertrophy of the limbs. • Type II = most common

  7. Eccrine Angiomatous Hamartoma • 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

  8. Malformations • Definition: abnormal structures that result from an aberration in embryonic development. • Functional: Nevus Anemicus • Anatomic: capillary, venous, arterial, lymphatic, or combined

  9. Nevus Anemicus • 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

  10. Cutis Marmorata Telangiectatica Congenita • 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

  11. Cutis Marmorata Telangectatica Congenita

  12. Nevus Flammeus (Port Wine Stain) • 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

  13. Nevus Flammeus “Salmon Patch”

  14. Nevus Flammeus • Sturge-Weber Syndrome: Nevus flammeus associated with seizures, trigeminal angiomatosis, ipsilateral intracranial calcifications, contralateral hemiparesis and ocular abnormalities. Tx: Neuro, Ophthalmo, Dental consults.

  15. Sturge-Weber Syndrome

  16. Nevus Flammeus • Klippel-Trenaunay Syndrome: Nevus Flammeus associated with venous varicosities and overgrowth of soft tissue and bone. Tx: support stockings; ortho consult.

  17. Klippel-Trenaunay Syndrome

  18. Nevus Flammeus • 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

  19. Cobb Syndrome

  20. Nevus Flammeus • 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

  21. 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

  22. Nevus Flammeus

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

  24. 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 • Associated with recurrent thrombophlebitis and calcified phleboliths • Pressure on surrounding structures (nerves) • Tx: U/S, MRI studies; vascular sx consult

  25. 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

  26. Venous Malformation • 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

  27. Maffucci’s syndrome

  28. 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 • Affects trunk and arms; associated w/nocturnal pain • GI hemangiomas in small bowel may rupture

  29. Blue rubber bleb nevus syndrome

  30. Venous Malformation • Gorham’s disease: cutaneous and osseus venous and lymphatic malformations • Massive Osteolysis ~ “Disappearing Bones”

  31. Arteriovenous Fistulas • Route from artery to vein that bypasses the capillary bed. Congenital or aquired. • Osler-Weber-Rendu: (hereditary hemorrhagic telangectasia) internal AV fistulas • Acquired orsecondary to trauma (HD access)

  32. Osler-Weber-Rendu

  33. 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

  34. 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

  35. 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: transvaginal U/S; may reoccur after surgery due to depth of invasion

  36. Dilation of Preexisting Vessels • 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)

  37. Spider Angioma

  38. 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)

  39. Capillary Aneurysms • 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

  40. Telangiectasia • 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

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

  42. Generalized Essential Telangiectasia • 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

  43. Angiokeratomas • Telangiectasias that have an overlying hyperkeratotic surface. • Dilatations of preexisting papillary dermal vessels.

  44. Angiokeratomas

  45. Angiokeratoma of Mibeli • 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

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