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Dermal and Subcutaneous Tumors – Part II. Michael Hohnadel March 2004. Kaposi’s Sarcoma. Five Presentations: 1. Classic Middle aged, European men Red, violaceous nodules on toes or soles. Coalesce to form plaques. Brawny edema

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Dermal and subcutaneous tumors part ii l.jpg

Dermal and Subcutaneous Tumors – Part II

Michael Hohnadel

March 2004

Kaposi s sarcoma l.jpg

Kaposi’s Sarcoma

Five Presentations:

1. Classic

  • Middle aged, European men

  • Red, violaceous nodules on toes or soles.

  • Coalesce to form plaques. Brawny edema

  • Later more widespread involvement esp soft palate. Periods of remission/involution.

    2. African Cutaneous

  • Nodular infiltrating vascular masses of extremities

  • Men 20-50 yrs. Endemic to Africa.

  • Locally aggressive.

  • Massive edema of legs and bone involvement.

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Kaposi’s Sarcoma

3. African Lymphadenopathic

  • Lymph nodes with/without skin in children <10.

  • Fatal in < 2years.

    4. AIDS

  • Violaceous macules progress to papules, nodules and plaques.

  • Head, neck, trunk and mucous membranes.

    5. Lymphoma / immunosuppressive TX.

    Resembles classic but more variable presentation.

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Kaposi’s Sarcoma

Classic Variety.

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Kaposi’s Sarcoma

HIV Associated.

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Kaposi’s Sarcoma

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Kaposi’s Sarcoma

  • Internal Involvement

    • G.I. Tract is site of internal involvement esp the small intestine.

    • Skeletal changes are diagnostic

      • Rarefaction, cyst, cortical erosion

    • In AIDS:

      • 25% cutaneous alone,

      • 29% viscous alone.

      • Ultimately, 70% will have viscous involvement of G.I. (50%), lungs (37%) or Lymph nodes (50%)

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Kaposi’s Sarcoma

  • Etiopathogenesis

    • Proliferation of abnormal vascular endothelial cells.

    • Muliticentric origin

    • HHV-8 is strongly assoc. and predictive of K.S. in HIV infected individuals.

  • Histology

    • Large endothelial cells of capillaries protrude into lumen like buds.

    • Lesions with proliferation of capillaries and fibrosarcoma like tissue in varying proportions.

    • Late lesions: spindle cell proliferation with sarcoma like properties.

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Kaposi’s Sarcoma

Early lesion with dilated thin walled vascular vessels with protruding endothelial cells.

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Kaposi’s Sarcoma

Later, ‘hemangioma- like’ lesion.

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Kaposi’s Sarcoma

  • Treatment:

    • Radiation. (all types are responsive)

    • For individual lesions: cryotherapy, vincristine, excision, laser ablation. Alitretinoin applied 2-4 times daily.

    • Systemic chemotherapy if >10 lesions / month or symptomatic visceral involvement.

    • Resistance to single chemo agents over time so combo with other modalities.

  • Course: Usually progresses slowly and is rarely the cause of death. (except African cutaneous variety.

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Epithelioid Hemangioendothelioma

  • Solitary, slow growing papule on the extremities. Intermediate between angiosarcoma and hemangioma.

  • M>F. Frequently before age of 25 years.

  • Rare.

  • Histology: Dilated vascular channels, spindle cells.

  • TX: Wide excision with evaluation of regional nodes. 20-30% 2 year mortality.

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Spindle cell Hemangioendothelioma

  • Child or young adult with Multifocal occurance of firm, blue nodules on extremity

  • Rare. May recur when excised.

    Retiform Hemangioendothelioma

  • Low grade angiosarcoma. Slow growing exophytic mass sub-Q nodule or even a plaque.

  • Rare

  • Extremities of young adults

  • Wide excision. Nodes may be affected locally but no deaths reported due to Mets.

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4 Clinical settings:

1.) Head and neck tumor of elderly

  • F:M = ratio 2:1

  • Ill defined bluish nodule resembles a bruise often with an erythematous ring. Satellite nodules, bleeding are common.

  • TX: Complete excision with radiation.

  • Multicentric nature and rapid metastasis usually results in death in two years.

    2.) In area of chronic lyphedema.

  • Classically after mastectomy (Stewart-Treves)

  • Appears 10 years after surgery in 0.45 % of pts.

  • Mets to lungs = death in 19-31 months. (6% -5 year)

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3.) Previously irradiated sites

  • Interval of tumor development depends on nature of lesion for which radiation was given:

    • Benign – 23 years to develop.

    • Malignant – 12 years.

  • Prognosis: death in 6-24 months.

    4.) Miscellaneous subset.

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Infiltration of the dermis by ill-defined vascular spaces and hyperchromatic, atypical endothelial cells. Factor VIII staining is positive.

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Fibrous Tissue Abnormalities


  • Firm, irregularly shaped, fibrous excrescence usually at a site of previous trauma. Claw-like projections overgrow wound boundary.

  • May occur at any body site. Most common site - sternal.

  • Histopathology: Growth of myofibroblast and collagen in the dermis with a whirl like arrangement of hyalinized bundles of collagen. There is a paucity of elastic tissue and thinning of the overlying papillary dermis.

  • DDX: Hypertrophic scar- No claw like extensions. Stays in wound boundaries. Often improves in 6 mo.

  • TX: Intralesional kenalog 20-40 mg/cc. Lasers. Excision with intralesional injections. Silicone sheeting.

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Hypertrophic Scar at 5 months and 1 year

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Spontaneous Keloids

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Hypertrophic Scar



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Dupuytren’s Contracture

  • Fibromatosis of the palmar aponeurosis. Plantar Fibromatosis is seen on soles.

  • Men 30-50 years. Multiple firm nodules in the palm, 1 cm in diameter, proximal to the 4th finger. Contractures develop with time.

  • Associations: Alcoholic cirrhosis, DM, epilepsy, plantar fibromatosis, Peyronies dx. Familiar predispositions.

  • TX: Early intralesional Triamcinolone, Surgery.

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Dupuytren’s Contracture

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Knuckle Pads

  • Well defined, round, plaque-like fibrous thickenings that develop over the proximal interphalangeal joints on toes and fingers.

  • May become 10-15mm in diameter and persist permanently. Remain freely mobile.

  • Assoc with Dupuytren’s contractures and some autosomal dominate familial cases have been reported.

  • Histology: Fibromas

  • TX: intralesional steroids.

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Knuckle Pads

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Peyronie’s Disease

  • Fibrous infiltration of intercavernous septum of the penis results in nodules and plaques. A fibrous chordee is produced with curvature of the on erection.

  • Assoc with Dupuytren’s contractor

  • TX: Intralesional Triamcinolone may be curative.

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Peyronie’s Disease

As Dr. Ladd would say: ‘Something just ain’t right…..’

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Desmoid Tumor

  • Deep seated well circumscribed mass arising from muscular aponeurosis.

  • Most common on abdominal wall esp. in women during or after pregnancy.

  • May be fatal if invade or compress vital structures. Most dangerous are those of neck and intra-abdominal

  • TX: MRI to evaluate extent of tumor. Excision.

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Aponeourotic Fibroma

  • Juvenile aponeurotic fibroma.

  • Slow growing cyst-like masses occurring on the limbs.

  • X-ray reveals Stippled calcification.

    Congenital Generalized Fibromatosis

  • Multiple firm dermal and subdermal nodules presenting at near birth.

  • 2 types of involvement:

    • Dermal and Skeletal (metaphyseal) – resolution 2 yrs.

    • Involvement of viscera - 80% mortality. If live past 4 mo. then regression.

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Infantile Digital Fibromatosis

  • Asymptomatic, firm, red, 1 cm. diameter nodules on DIP of fingers or toes during the first year of life. No metastasis.

  • Whorled fascicles of spindle cells eosinophilic inclusion bodies

  • Surgical excision has high recurrence.

  • TX: Observation, hope for spontaneous resolution.

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Infantile Digital Fibromatosis

Whorled fascicles of spindle cells

Eosinophilic inclusion bodies

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  • Fibrous Harmartoma of infancy

    • Single dermal or sub-q nodule of upper trunk present at birth.

    • Excision.

  • Fibomatosis Colli

    • Fibrous proliferation infiltration sternocleidomastoid m. at birth.

    • Spontaneous remission in a few months.

  • Diffuse Infantile Fibromatosis

    • Multicentric fibrous infiltration of m. of arms, neck and shoulder area.

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Giant Cell Tumor of Tendon Sheath

  • Firm 1-3 cm. nodule attaches to tendons of fingers hand and wrist (esp flexor).

  • Histopathology:

    • Lobules of densely hyalinized collagen. Giant cells with eosinophilic cytoplasm and variable nuclei.

  • TX

    • Excision. Recurs in 25%.

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Giant Cell Tumor of Tendon Sheath

Sheets of epithelioid histiocytes with a

variable number of the characteristic

multinucleated osteoclast-like giant cells. Some of the histiocytes may have pale foamy cytoplasm.

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  • Linear constriction occurs around the toes (esp 5th at PIP) eventually resulting spontaneous amputation in 5-10 years. Begins as grove on flexor surface and joins over time.

  • African Men.

  • Etiology: Unknown. (Trauma?)

  • TX: Surgery sometimes helpful. Intralesional steroids.

  • Pseudo-Ainhum: Hereditary and nonhereditary diseases associated with annular constriction.

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Connective Tissue Nevi

  • Multiple or solitary plaques 1-15 cm in diameter, yellow to light orange with a shagreen leather like surface texture.

    • Predilection for lumbosacral area.

  • Acquired type: eruptive collagenoma, isolated collagenoma and isolated elastoma

  • Congenital Types:

    • 1.) Buschke – Ollendorf: AD. Widespread asymmetrically distributed plaques. Osteopoikilosis of long bones is diagnostic.

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Connective Tissue Nevi


  • 2.) Familial cutaneous collagenomas: Numerous symmetrical asymptomatic nodules on back. Onset in teens. MEN-I assoc.

  • In tuberous sclerosis, connective tissue nevi are shagreen patches.

    • ½ of T.S. cases are new mutations so any pt with connective tissue nevi should be evaluated for tuberous sclerosis.

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    Connective Tissue Nevi

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    Fibrous and vascular proliferation of upper dermis

    Common types:

    • Fibrous papule of the nose – 3-6 mm diameter, dome shaped sessile papule with white to reddish color. Usually solitary.

    • Pearly Penile Papule – Pearly white papules appearing on the coronal margin and sometimes on penile shaft. No TX required

    • Multiple hereditary forms - adenoma sebaceum (Tuberous Sclerosis) and in MEN-I

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    Fibrous papule of the nose

    Pearly Penile Papule

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    Acral Fibrokeratoma

    • Pink, hyperkeratotic, hornlike projection on fingers (most common) toes or palms. Emerges from a collarette of scale.

    • Average age 40.

    • TX: excision. Laser ablation.

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    Acral Fibrokeratoma

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    Subungal Exostosis

    • Solitary, fibrous and bony nodule protruding from the distal edge of the nail, most commonly of the great toe.

      • Begins as a pink papule which destroys overlying nail and grows to a maximum diameter of 8 mm.

    • Pressure causes great pain.

    • X-ray is diagnostic

    • TX: excision and curettage.

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    Subungal Exostosis

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    Chondrodermatits Nodularis Chronica Helicis

    • Small, tender, inflammatory nodule with gently sloping sides located on the outer helix. Common in older men. No malignant potential.

    • Often hx of chronic trauma. Lesions are very painful. Pt often complains of pain when sleeps on lesion.

    • Histology: Degeneration of collagen with acanthosis and hyperkeratosis and thinning of epidermis.

    • TX: Excision. Often a spicule of cartilage is discovered. (Transdermal elimination).

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    Chondrodermatits Nodularis Chronica Helicis

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    • Flesh colored, pigmented sessile or pedunculated papillomas.

    • Areas: eyelids, axilla, neck, groin.

    • 60 % incidence by the age of 69.

    • Treatment: excision.

    Dermatofibroma l.jpg


    • Lesion which grows slowly to a firm, 4-20 mm diameter papule or nodule, yellow or reddish brown in color and then stops expanding. Sharply demarcated. Chiefly located on lower extremities.

    • ‘Dimple sign’. Seldom seen in children. May grow to 5 cm in size.

    • Etiology: Trauma, bites ?

    • Histology: Dermal mass or whorled fibrous tissue. Numerous cells with large nuclei and spindle cells. Some very atypical cells referred to as ‘Monster cells’.

    • TX: Reassurance. Progressive enlargement warrants excision.

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    Nodular proliferation of spindled fibroblasts and histiocytes in the reticular dermis, with hyperplasia and hyperpigmentation of the overlying epidermis. There is extension into the subcutaneous tissue in a radial pattern (arrow).

    The fibroblasts are arranged in broad intersecting fascicles with entrapment of thick collagen bundles

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    Monster Cells

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    Dermatofibrosarcoma Protuberans

    • On a middle aged PT, a slowly enlarging, erythematous, firm nodule or plaque often with purulence appears on the trunk (60%). Pain is more prominent with as lesion grows and may be severe.

      • Early lesions resemble keloids or large DF.

    • Histology: Subepidermal fibrotic plaque with uniform spindle cells. Cartwheel pattern of spindle cells surrounding collagen. Pigment cells = Bednar tumor in dark skinned individuals. CD 34 positive.

    • TX: Mohs (2% recurrence) or wide excision (11-50%) recurrence.

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    Dermatofibrosarcoma Protuberans

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    Dermatofibrosarcoma Protuberans

    Characteristic multilayered pattern of infiltration into the subcutaneous tissue

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    Dermatofibrosarcoma Protuberans

    Spindle-shaped cells are arranged in a ‘storiform’ pattern.

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    Nodular Fasciitis

    • Deep, firm, solitary, sometimes tender nodule of deep fascia that rapidly grows to 1-4 cm in diameter over several weeks. Pt is otherwise healthy and average age is 40 years.

    • Variants: Dermal, intravascular and proliferating.

    • Histology: myxoid, fibroblastic and capillary proliferation. Lymphocytic-histiocytic infiltration.

    • TX: complete excision. Intralesional steroids.

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    Nodular Fasciitis

    Spindle-shaped and stellate fibroblasts are loosely arranged in a myxomatous stroma, with some cells in mitosis

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    Atypical Fibroxanthoma

    • Small, firm nodule often with eroded surface.

    • Usually occurs on sun exposed locations on the head and neck, Caucasians >50 yrs. old.

      • A subset occurs in 25% of patients where the tumor is located on covered area in person approx 40 yrs old.

    • Histology: Bizarre spindle cells (vesicular nucleus) and atypical histiocytes with mitotic cells, eosinophilic nuclei and biphasic cell population.

    • TX: Surgical excision. Recurrence is frequent and MOHS offers best cure rate. Possible metastasis in rare cases.

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    Atypical Fibroxanthoma

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    Atypical Fibroxanthoma

    • The spindle cells have pale foamy cytoplasm and hyperchromatic nuclei with small nucleoli. There is also a large atypical giant cell with darker nuclear chromatin as well as a cell in atypical mitosis.

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    Malignant Fibrous Histiocytoma

    • Most common soft tissue sarcoma of middle age and late adulthood. Resembles DFSP.

    • Progressively enlarging, 1-3 cm diameter, protruding, tumor with a reddish or dusky

      • 1/3 on thigh or buttocks. Peak incidence in 7th decade.

      • Association with radiodermatitis and chronic ulcers.

    • Histology: Polygonal and spindle cells with large bizarre multinucleated types. Pleomorphic cellular elements and bizarre mitotic figures.

    • TX: Excision. Recurrence in 25 % of cases. Metastasis in 35%. Overall survival of 50%

    • Prognosis: Deeper and proximal = poorer prognosis. Assoc with radiodermatitis = esp poor prognosis

    Malignant fibrous histiocytoma65 l.jpg

    Malignant Fibrous Histiocytoma

    Epithelioid sarcoma l.jpg

    Epithelioid Sarcoma

    • Tumor of the extremities (half on hands) of young men (2/3 of cases), ages 20-40 years.

    • Slow growing tumor among fascial structures and tendons with nodules and overlying ulceration.

      • DDX: G.A. fibroma, EIC, ganglion, SSC.

    • HX: Acidophilic polygonal cells merging with spindle cells and hyalinized collagen.

    • TX: Wide local excision in early disease. Recurrence in 3 of 4 cases. Late mets in 45%

    Epithelioid sarcoma67 l.jpg

    Epithelioid Sarcoma

    Epithelioid cells are palisaded around an area of necrosis.

    Myxomas l.jpg


    2 types:

    1.) Digital Mucous cyst.

    • Taut, shiny translucent white or pink dome shaped lesions typically located distal to the DIP joint. Often with accompanying grooving and dystrophy of the associated nail.

    • Focal accumulations of mucin without a true lining.

    • Etiology: Assoc with joint space and forms by extrusion of jt. space mucin? Independent of jt. space?

    • TX: Drain, Excision. Intralesional steroids.

    Digital mucous cyst l.jpg

    Digital Mucous cyst.

    Myxomas70 l.jpg


    2.) Cutaneous Myxomas

    • Solitary of multiple flesh colored nodules of the trunk, face or extremities.

    • Syndromes: Carney (Also called: NAME, LAMB ect.)

      • Autosomal dominate inheritance

    • Carney syndrome (2+ of following)

      • Cardiac Atrial Myxoma (79%) Can be life threatening.

      • Cutaneous myxomas (45%) <1 cm flesh colored papules which develop by the age of 18 and occur on ears, eyelids and nipples.

      • Mammary myxoid fibromas (30%)

      • Spotty mucocutaneous pigmentation (blue nevi) (65%)

      • Prim. Pig. Nodular adrenocortical disease. (45%)

      • Testicular tumors (56%)

      • Pituitary G.H. secreting tumors. (10%)

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