Transcript Dermal and Subcutaneous Tumors – Part II

Dermal and
Subcutaneous Tumors
– Part II
Adam Wray, D.O.
June 28, 2005
Kaposi’s Sarcoma
Five Presentations:
1. Classic
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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
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Nodular infiltrating vascular masses of extremities
Men 20-50 yrs. Endemic to Africa.
Locally aggressive.
Massive edema of legs and bone involvement.
Kaposi’s Sarcoma
3. African Lymphadenopathic
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Lymph nodes with/without skin in children
<10.
Fatal in < 2years.
4. AIDS
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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
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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,
 In
cyst, cortical erosion
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
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Proliferation of abnormal vascular endothelial cells.
Muliticentric origin
HHV-8 is strongly assoc. and predictive of K.S. in HIV
infected individuals.
Histology
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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.
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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.
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Course: Usually progresses slowly and is
rarely the cause of death. (except African
cutaneous variety.
Epithelioid Hemangioendothelioma
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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;
cords and nests of epithelioid endothelial cells in a
myxoid or hyalinized background
30% mets to regional nodes, lung, liver, or bone
TX: Wide excision with evaluation of regional
nodes. 20-30% 2 year mortality.
Spindle cell Hemangioendothelioma
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AKA spindle cell hemangioma
Child or young adult with Multifocal occurance of firm,
blue nodules on extremity
Rare. May recur when excised.
Retiform Hemangioendothelioma
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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.
Spindle Cell Hemangioendothelioma
Angiosarcoma
4 Clinical settings:
1.) Head and neck tumor of elderly
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Most common clinical setting
M:F = 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.
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Appears 4-27 years following duration of lympedema
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
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Interval of tumor development depends on nature
of lesion for which radiation was given:
Benign – 23 years to develop.
 Malignant – 12 years.
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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
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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
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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.
Dupuytren’s Contracture
Knuckle Pads
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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
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Fibrous infiltration of intercavernous septum of
the penis results in nodules and plaques. A
fibrous chordee is produced with curvature of
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
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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.
Radiotherapy and hormonal manipulation are other
options
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Desmoid Tumor
5 subtypes
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Abdominal wall
Extrabdominal
Intrabdominal
Multiple
Gardner’s syndrome
Aponeourotic Fibroma
Juvenile aponeurotic fibroma.
 Slow growing cyst-like masses occurring on the
limbs.
 X-ray reveals Stippled calcification.
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Congenital Generalized Fibromatosis
Multiple firm dermal and subdermal nodules
presenting at near birth.
 2 types of involvement:
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Dermal and Skeletal (metaphyseal) – resolution 2 yrs.
Involvement of viscera - 80% mortality. If live past 4 mo. then
regression.
Juvenile aponeurotic fibroma
Aponeourotic Fibroma
Juvenile aponeurotic fibroma.
 Slow growing cyst-like masses occurring on the
limbs.
 X-ray reveals Stippled calcification.
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Congenital Generalized Fibromatosis
Infantile myofibromatosis
 Multiple firm dermal and subdermal nodules
presenting at near birth.
 2 types of involvement:
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Dermal and Skeletal (metaphyseal) – resolution 2 yrs.
Involvement of viscera - 80% mortality. If live past 4 mo. then
regression.
Infantile Digital Fibromatosis
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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
Whorled fascicles of spindle cells
Eosinophilic inclusion bodies
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Fibrous Harmartoma of infancy
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Fibomatosis Colli
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Single dermal or sub-q nodule of upper trunk present at
birth.
Excision.
Fibrous proliferation infiltration sternocleidomastoid m.
at birth.
Spontaneous remission in a few months.
Diffuse Infantile Fibromatosis
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Multicentric fibrous infiltration of m. of arms, neck and
shoulder area.
Giant Cell Tumor of Tendon Sheath
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Firm 1-3 cm. nodule attaches to tendons of
fingers hand and wrist (esp flexor).
Histopathology:
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Lobules of densely hyalinized collagen. Giant cells
with eosinophilic cytoplasm and variable nuclei.
TX
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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.
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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
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Multiple or solitary plaques 1-15 cm in diameter,
yellow to light orange with a shagreen leather
like surface texture.
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Predilection for lumbosacral area.
Acquired type: eruptive collagenoma, isolated
collagenoma and isolated elastoma
Congenital Types:
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1.) Buschke – Ollendorf: AD. Widespread
asymmetrically distributed plaques. Osteopoikilosis
of long bones is diagnostic.
Connective Tissue Nevi
(Continued)
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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.
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½ of T.S. cases are new mutations so any pt with
connective tissue nevi should be evaluated for
tuberous sclerosis.
Connective Tissue Nevi
Angiofibromas
Fibrous and vascular proliferation of upper
dermis
Common types:
1.
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3.
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
Angiofibromas
Fibrous papule of the nose
Pearly Penile Papule
Acral Fibrokeratoma
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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
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Solitary, fibrous and bony nodule protruding
from the distal edge of the nail, most commonly
of the great toe.
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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
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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).
Chondrodermati
ts Nodularis
Chronica Helicis
Achrocordon
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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,
cryosurgery
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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 ?
CD34 neg; Factor XIII pos; Stromolysin pos
Histology: Dermal mass or whorled fibrous tissue.
Numerous cells with large nuclei and spindle cells.
Some very atypical cells referred to as ‘Monster
cells’. Respects subQ fat
TX: Reassurance. Progressive enlargement warrants
excision.
Dermatofibroma
Dermatofibroma
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
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.
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Early lesions resemble keloids or large DF.
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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.
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TX: Mohs (2% recurrence) or wide excision (1150%) 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
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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.
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Variants: Dermal, intravascular and proliferating.
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Histology: myxoid, fibroblastic and capillary
proliferation. Lymphocytic-histiocytic infiltration.
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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
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Small, firm nodule often with eroded surface.
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Usually occurs on sun exposed locations on the head
and neck, Caucasians >50 yrs. old.
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A subset occurs in 25% of patients where the tumor is located on
covered area in person approx 40 yrs old.
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Histology: Bizarre spindle cells (vesicular nucleus) and
atypical histiocytes with mitotic cells, eosinophilic nuclei
and biphasic cell population.
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TX: Surgical excision. Recurrence is frequent and
MOHS offers best cure rate. Possible metastasis in
rare cases.
Atypical Fibroxanthoma
Atypical Fibroxanthoma
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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
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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
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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
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Tumor of the extremities (half on hands) of young
men (2/3 of cases), ages 20-40 years.
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Slow growing tumor among fascial structures and
tendons with nodules and overlying ulceration.
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DDX: G.A. fibroma, EIC, ganglion, SSC.
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HX: Acidophilic polygonal cells merging with
spindle cells and hyalinized collagen.
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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.
2 types:
Myxomas
1.) Digital Mucous cyst.
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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.
Myxomas
2.) Cutaneous Myxomas
 Solitary of multiple flesh colored nodules
of the trunk, face or extremities.
 Syndromes: Carney (Also called: NAME, LAMB ect.)
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Autosomal dominate inheritance
Carney syndrome (2+ of following)
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7.
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%)