Dermal and Subcutaneous Tumors

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Dermal and Subcutaneous Tumors Part II

• Adam Wray, D.O.
• June 28, 2005

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Kaposis 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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Kaposis Sarcoma

• 3. African Lymphadenopathic
• Lymph nodes with/without skin in children lt10.
• Fatal in lt 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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Kaposis Sarcoma
Classic Variety.
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Kaposis Sarcoma
HIV Associated.
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Kaposis Sarcoma
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Kaposis 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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Kaposis 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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Kaposis Sarcoma
Early lesion with dilated thin walled vascular
vessels with protruding endothelial cells.
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Kaposis Sarcoma
Later, hemangioma- like lesion.
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Kaposis Sarcoma

• Treatment
• Radiation. (all types are responsive)
• For individual lesions cryotherapy, vincristine,
  excision, laser ablation. Alitretinoin applied
  2-4 times daily.
• Systemic chemotherapy if gt10 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.
• MgtF. 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.

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• Spindle cell Hemangioendothelioma
• AKA spindle cell hemangioma
• 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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Spindle Cell Hemangioendothelioma
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Angiosarcoma

• 4 Clinical settings
• 1.) Head and neck tumor of elderly
• Most common clinical setting
• MF ratio 21
• 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.
• 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)

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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.

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Angiosarcoma
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Angiosarcoma
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

• 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.

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Keloids
Hypertrophic Scar at 5 months and 1 year
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Spontaneous Keloids
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Keloids
Hypertrophic Scar
Keloid
Keloid
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Dupuytrens 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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Dupuytrens 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 Dupuytrens 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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Peyronies Disease

• 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 Dupuytrens contractor
• TX Intralesional Triamcinolone may be curative.

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Peyronies Disease
As Dr. Ladd would say Something just aint
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.
  Radiotherapy and hormonal manipulation are other
  options

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• Desmoid Tumor
• 5 subtypes
• Abdominal wall
• Extrabdominal
• Intrabdominal
• Multiple
• Gardners syndrome

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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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Juvenile aponeurotic fibroma
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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
• Infantile myofibromatosis
• 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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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.

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Ainhum
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Ainhum
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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

• (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.

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Connective Tissue Nevi
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Angiofibromas

• 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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Angiofibromas
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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Achrocordon

• 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.

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Dermatofibroma
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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
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Dermatofibroma
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 gt50 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

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Malignant Fibrous Histiocytoma
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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

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Epithelioid Sarcoma
Epithelioid cells are palisaded around an area of
necrosis.
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Myxomas

• 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.

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Digital Mucous cyst.
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Myxomas

• 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) lt1 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)