Dermal and Subcutaneous Tumors

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

Cutaneous Vascular Anomalies
* Hamartomas
* Malformations
* Dilation of preexisting vessels
* Hyperplasias
* Benign neoplasms
* Malignant neoplasms
Hamartomas
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
* 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

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
Malformations
* Definition: 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 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
* 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)
* 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”
Sturge-Weber Syndrome
Klippel-Trenaunay Syndrome
Beckwith-Wiedemann Syndrome
Cobb Syndrome

* Proteus Syndrome:
* Robert’s Syndrome
* Wyburn-Mason Syndrome:
* Tar Syndrome
* 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
* Associated with recurrent thrombophlebitis and calcified phleboliths
* Pressure on surrounding structures (nerves)
* Tx: U/S, MRI studies; vascular sx consult
* 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
* 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

* 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
* Gorham’s disease: cutaneous and osseus venous and lymphatic malformations
* Massive Osteolysis ~ “Disappearing Bones”
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 or secondary to trauma (HD access)
Osler-Weber-Rendu
Arteriovenous Fistulas
Superficial Lymphatic Malformation
Cystic Lymphatic Malformation
Dilation of Preexisting Vessels
Spider Angioma
Venous Lakes
Capillary Aneurysms
Telangiectasia
* Radiodermatitis
* Xeroderma pigmentosum
* Lupus erythematosus
* Dermatomyositis
* Scleroderma
* CREST
* rosacea
* Liver disease
* Poikiloderma
* BCC
* Sarcoid
* SLE
* Pregenacy
* Osler-Weber-Rendu
* Etc.

Generalized Essential Telangiectasia
Angiokeratomas
Angiokeratoma of Mibeli
Angiokeratoma of the Scrotum (Fordyce)
Solitary Angiokeratoma
Lymphangiectasis
Hyperplasias
Angiolymphoid Hyperplasia with Eosinophilia (AHLE)
Pyogenic Granuloma
Intravascular Papillary Endothelial Hyperplasia
Benign Neoplasms
Angioma Serpiginosum
Infantile Hemangioma (Strawberry Hemangioma)
Infantile Hemangiomas Treatment
Cherry Angiomas (Senile Angiomas, DeMorgan Spots)
Targetoid Hemosiderotic Hemangioma
Microvenular Hemangioma
Tufted Angioma (Angioblastoma)
Glomeruloid Hemangioma
Kaposiform Hemangioendothelioma
Kasabach-Merritt Syndrome (Hemangioma with Thrombocytopenia)
Glomus Tumor
Hemangiopericytoma
Proliferating Angioendotheliomatosis

Dermal and Subcutaneous Tumors.ppt