Dermal and Subcutaneous Tumors – Part II

Michael Hohnadel

March 2004

Kaposi’s SarcomaFive 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.

Kaposi’s Sarcoma3. 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.

Kaposi’s Sarcoma

Classic Variety.

Kaposi’s Sarcoma

HIV Associated.

Kaposi’s Sarcoma

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%)

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.

Kaposi’s Sarcoma

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

Kaposi’s Sarcoma

Later, ‘hemangioma- like’ lesion.

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.

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.

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.

Angiosarcoma4 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)

Angiosarcoma

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.

Angiosarcoma

Angiosarcoma

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

Fibrous Tissue Abnormalities

Keloids• 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.

Keloids

Hypertrophic Scar at 5 months and 1 year

Spontaneous Keloids

Keloids

Hypertrophic Scar Keloid Keloid

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.

Dupuytren’s Contracture

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.

Knuckle Pads

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.

Peyronie’s Disease

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

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.

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.

• 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.

Infantile Digital Fibromatosis

Infantile Digital Fibromatosis

Whorled fascicles of spindle cells Eosinophilic inclusion bodies

• 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.

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%.

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.

Ainhum• 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.

Ainhum

Ainhum

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.

Connective Tissue Nevi

(Continued)– 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.

Connective Tissue Nevi

AngiofibromasFibrous and vascular proliferation of upper

dermisCommon types:

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

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

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

Angiofibromas

Fibrous papule of the nose

Pearly Penile Papule

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.

Acral Fibrokeratoma

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.

Subungal Exostosis

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).

Chondrodermatits Nodularis

Chronica Helicis

Acrocordon

• Flesh colored, pigmented sessile or pedunculated papillomas.

• Areas: eyelids, axilla, neck, groin.

• 60 % incidence by the age of 69.

• Treatment: excision.

Dermatofibroma• 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.

Dermatofibroma

DermatofibromaNodular 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

Dermatofibroma

Monster Cells

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.

Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma Protuberans

Characteristic multilayered pattern of infiltration into the subcutaneous tissue

Dermatofibrosarcoma Protuberans

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

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.

Nodular Fasciitis

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

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.

Atypical Fibroxanthoma

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.

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 Histiocytoma

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 Sarcoma

Epithelioid cells are palisaded around an area of necrosis.

Myxomas2 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.

Myxomas2.) 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)1. Cardiac Atrial Myxoma (79%) Can be life threatening.

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

3. Mammary myxoid fibromas (30%)

4. Spotty mucocutaneous pigmentation (blue nevi) (65%)

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

6. Testicular tumors (56%)

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