Dermal and Subcutaneous Tumors

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Dermal and Subcutaneous Tumors
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Mastocytosisurticaria pigmentosa

• Local and systemic accumulations of mast cells
• Persistent pigmented itchy skin lesions
• Urticate on mechanical or chemical irritation
• c-KIT mutation
• Birth to middle age, ½ lt 6 mo

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• Macules, papules, nodules, plaques, vesicles
• Lesions persist and gradually become chamois- or
  slate-colored
• Dariers sign, pruritis
• Severe symptoms may result from massive
  liberation of histamine from mast cells after
  ingestion of known mast cell degranulators
• Spontaneous resolution is likely in those pts
  whose disease began in childhood

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Urticaria pigmentosa
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Solitary mastocytoma

• May be present at birth, may develop during the
  first weeks of life
• Brown macule that urticates upon stroking
• Smooth or peau d orange
• Dorsum of the hand near the wrist
• Edema, urtication, vesiculation may be observed

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• Generalized eruption, childhood type
• Pseudoxanthomatous mastocytosis
• Diffuse cutaneous mastocytosis
• Generalized eruption, adult type
• Erythrodermic mastocytosis
• Telangiectasia macularis erupta perstans
• Systemic mastocytosis
• Familial urticaria pigmentosa

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• Giemsa, azure A, or polychrome toluidine blue
• Local anesthetic adjacent to the lesion, without
  epi
• Dx is bx confirmed
• Histamine metabolites methylhistamine and
  methylimidazole acetic acid

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Prognosis and treatment

• In all forms without systemic involvement the
  prognosis is good
• Solitary lesions usually involute within 3 years
• H1 and H2 blockers
• PUVA
• Intralesional and topical steroids
• Avoid physical stimuli

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Abnormalities of neural tissue
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Solitary neurofibroma

• Soft, flaccid, pinkish white, 2-20 mm
• Invaginates on pressure, buttonholing
• Solitary or multiple
• Distinctive histopathologic findings, fibrils,
  cellular proliferation, and degenerative changes
• Sx excision

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Granular cell tumor

• Well-circumscribed, solitary firm nodule, with a
  brownish red or flesh tint
• Usually solitary, 10-15 multiple
• 1/3 of cases have occurred on the tongue
• May occur anywhere on the body
• Grows slowly
• Cells stain positively with vimentin,
  neuron-specific enolase, S-100, and myelin
  protein
• Malignant granular cell tumor is rare

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Neuroma cutis

• Three true neuromas exist in the skin and mucous
  membranes traumatic neuroma, multiple mucosal
  neuromas, and solitary palisaded encapsulated
  neuromas
• Traumatic neuromas occur commonly on the fingers,
  tender and painful
• Multiple mucosal neuromas occur as part of
  multiple mucosal neuroma syndrome
• solitary palisaded encapsulated neuromas occur
  commonly on the face, resembles BCC

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neurothekeoma

• Nerve sheath myxoma
• Benign tumor of nerve sheath
• Mitotic figures and nuclear atypia are sometimes
  observed
• Intradermal or subcutaneous
• Histologically are divided into two subtypes
  myxoid and more common cellular variant

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schwannoma

• neurilemmoma
• Usually a solitary nerve sheath tumor
• Most often seen in women
• Occur almost exclusively along the main nerve
  trunks of the extremities
• Soft or firm nodules, may be painful
• May be multiple
• May be assoc. with NF-1 or NF-2

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• Occur in many other organs
• excision

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Infantile neuroblastoma

• The most common malignant tumor of childhood
• Cutaneous nodule are most often seen in the
  younger patients
• Blue nodules the when rubbed form a halo of
  erythema
• Periorbital ecchymoses and heterechromia
• Good prognosis for patients with skin
  involvement, spontaneous remission

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ganglioneuroma

• Rarely described in the skin as an isolated
  entity
• Arise most often in von Recklinghausens
  neurofibromatosis
• Occur in childhood

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Nasal glioma

• Cephalic brainlike heterotopias
• Rare, benign congenital tumors
• Easily confused with hemangiomas
• Firm, reddish blue lesion on the nasal bridge
• No connection with the subarachnoid space
• Radiography and neurosurgical consultation
• Does not involute spontaneously

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Cutaneous memingioma

• Psammoma
• Results from the presence of meningocytes outside
  the calvarium
• Small, hard, fibrous, calcified nodules occurring
  along the spine, in the scalp, and on the
  forehead
• Usually seen within the first year
• No distinctive appearance, dx by histo

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Encephalocele and Meningocele

• Primary defect in the neural tube
• Present in infancy along the midline
• Compressible masses that may transilluminate or
  enlarge with crying
• Midline masses require intensive radiologic and
  neurosurgical evaluation before biopsy

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chordomas

• Slow-growing, locally invasive
• Firm, smooth nodules in the sacralcoccygeal
  region or at the base of the skull
• Arise from notochord remnants
• May metastasize late in their course
• Wide excision and postoperative radiation therapy

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Abnormalities of Fat Tissue
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lipomas

• Subcutaneous tumors composed of fat tissue
• Most commonly found on the trunk
• Also neck, forearms and axillae
• Soft, single or multiple, lobulated and
  compressible
• Growth to size and remain stationary
• again be careful of sacrococcygeal lipomas
• Lesion may be left untreated or excised

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• Solitary lesions reaching greater than 10 cm
  should be investigated for malignancy
• Multiple lesion may be painful if growing rapidly
• Madelungs disease, benign symmetric lipomatosis
• Dercums disease, assoc with weakness and
  psychiatric disturbances

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• Familial multiple lipomatosis, AD inheritance
• Bannayan-Riley-Ruvalcaba syndrome
• MEN 1
• Frohlichs syndrome
• Gardners syndrome

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angiolipoma

• A painful subcutaneous nodule just slightly above
  the level of the skin
• Has all other typical features of a lipoma
• Seen in young adults who have multiple painful
  lumps in the skin
• Multiple subcutaneous angiolipomas have no
  invasive or metastatic potential

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Neural fibrolipoma

• Overgrowth of fibro-fatty tissue
• Occurs along a nerve trunk and often leads to
  compression
• Slowly enlarging subcutaneous mass with
  tenderness and decreased sensation or parasthesia
• Median nerve is most commonly involved
• MRI, no effective treatment

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Spindle-cell lipoma

• Asymptomatic, slow growing subcutaeneous tumor
• Predilection for the back and neck and shoulders
  of older men
• Consists of lobulated masses of mature adipose
  tissue

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Painful Piezogenic pedal papules

• Transitory, soft, sometimes painful papules on
  the sides of the heels
• Elicited by weight-bearing and disappearing when
  this is stopped
• Occur in at least 75 of normal individuals
• Suitable supportive shoes may alleviate
  discomfort
• May occur on the wrist

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Nevus lipomatosus superficialis

• Soft, yellowish papule or ceribriform plaques,
  usually of the buttock or thigh, less often the
  ear or scalp
• A wrinkled surface characterizes this tumor
• Onset prior to age of 20

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• Nevus lipomatosus superficialis

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Folded skin with scarring

• Rare, aka Michelin Tire Baby Syndrome
• There are numerous deep, conspicuous,
  symmetrical, ringed creases around the
  extremities
• The underlying skin may manifest a smooth muscle
  hamartoma, a nevus lipomatosis, or elastic tissue
  abnormalities
• AD, sporadic or an isolated finding assoc with
  congenital facial and limb abnormalities

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Diffuse lipomatosis

• Characterized by an early age of onset, by the
  age of 2, diffuse infiltration of muscle by and
  encapsulated mass of mature lipocytes
• Progressive enlargement and extension
• Usually involves a large portion of the trunk or
  extremity

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Hibernoma(lipoma of brown fat)

• A form of lipoma composed of finely vacuolated
  fat cells of embryonic type
• Have a distinctive brownish color and a firm
  consistency
• Benign and usually occur singly
• Chiefly in the mediastinum and the interscapular
  region
• Onset usually in adult life

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Pleomorphic lipoma

• Occur for the most part on the backs and necks of
  elderly men
• Occasional lipoblast-like cells and atypical
  mitotic figures may require differentiation from
  a liposarcoma
• Behave in a perfectly benign manner

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Benign lipoblastomatosis

• Frequently confused with a liposarcoma
• Affects exclusively infants and young children,
  90 lt age 3
• Commonly involves the soft tissues of the upper
  or lower extremity
• A circumscribed and a diffuse form can be
  distinguished
• TOC- complete local excision

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liposarcoma

• One of the less common mesenchymal neoplasms of
  the soft tissue
• Usually arise from intermuscular fascia
• Do not arise from preexisting lipomas
• Usual course is an inconspicuous swelling of the
  soft tissue with gradual enlargement
• When a fatty tumor becomes greater than 10 cm DX
  should be considered
• Upper thigh is the most common site

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• Adult males are mostly affected
• May be well or poorly differentiated
• Tx is adequate radical excision
• For metastatic liposarcomas, radiation therapy
  may be effective

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Abnormalities of smooth muscle
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leiomyoma

• Smooth muscle tumors
• Characterized by painful nodules
• Singly or multiple
• Benign
• Treatment is directed toward the removal of the
  pain source
• Simple excision is best

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• Solitary cutaneous leiomyoma
• Multiple cutaneous leiomyomas
• Solitary genital leiomyoma
• angioleiomyoma

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Grouped leiomyomata of the back
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Congenital smooth muscle hamartoma

• Typically a skin colored or slightly pigmented
  patch or plaque with hypertrichosis
• Often present at birth
• Usually seen on the trunk, lumbosacral area in
  2/3
• Michelin tire baby syndrome may result from a
  diffuse smooth muscle hamartoma

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• Clinically may mimic a mastocytoma,
  pseudo-Dariers sign is seen in 80
• No treatment is necessary

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leiomyosarcoma

• Of soft tissue origin are extremely rare
• May occur as metastasis from internal source
• Appears in the dermis as a solitary nodule, good
  prognosis
• Subcutaneous lesions have a guarded prognosis,
  with fatal hematogenous metastases in 1/3
• WLE or Mohs

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Miscellaneous tumors and tumor-associated
conditions
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Cutaneous endometriosis

• Brownish papules in the umbilicus or lower
  abdominal scars after gynecologic surgery
• Tender or painful lesions
• Bluish black from cyclic bleeding
• Usually misdiagnosed as malignant metastases
• Surgical excision
• Preoperative tx with danazol or leuprolide may
  reduce size

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teratoma

• May develop in the skin but are most common in
  the ovaries or testes
• No characteristic clinical features
• Tissue representing all three germ layers are
  present
• Occasionally malignancy may occur

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Metastatic carcinoma

• 5 to 10 of patients with cancer develop skin
  metastases
• Usually present as numerous firm, hard, or
  rubbery masses
• Predilection for chest, abdomen or scalp
• Sister Mary Joseph nodule, metastatic tumor
  localized to the umbilicus, most common primary
  sites include the stomach, large bowel, ovary and
  pancreas

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• A poor prognosis is usually the rule
• The involvement of the skin is likely to be near
  the area of the primary tumor
• Breast cancer is the type most commonly
  metastatic to the skin in women and melanoma
  followed by lung cancer in men
• Metastatic lesions are uncommon in children

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Paraneoplastic syndromes

• Some cancers produce findings in the skin that
  indicate to the clinician that an underlying
  internal malignancy may be present
• Bazexs syndrome, characterized by violaceous
  erythema and scaling of the fingers, toes, nose,
  and aural helices.
• Secondary to a primary malignant neoplasm of the
  upper aerodigestive tract

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Bazexs syndrome
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• Necrolytic migratory erythema, seen with
  glucagon-secreting tumors of the pancreas
• Erythema gyratum repens, erythema with
  characteristic wood-grain-pattern scales, is
  almost always associated with and underlying
  malignancy
• Hypertrichosis lanuginosa aquisata, most common
  with lung and colon ca

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EGR
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Erythema gyratum repens
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Hypertrichosis lanuginosa
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• The sign of Lesser-Trelat, the sudden appearance
  of multiple pruritic seborrheic keratosis,
  associated with and internal malignancy
• Trousseaus sign, migratory thrombophlebitis,
  pancreatic ca
• Pityriasis rotunda
• Tripe palms
• Several others with less frequency

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carcinoid

• Characterized by distinctive involvement of the
  lungs, heart, gastrointestinal tract and the skin
• Cutaneous flushing lasting 5-10 minutes
• Involves the head and neck producing a scarlet
  color
• Cyanosis may be present
• Episodic flushing continues for months or years

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• The release of excessive amounts of serotonin and
  bradykinen into circulation produces attacks of
  flushing of the skin, weakness, abdominal pain,
  nausea and vomiting, sweating, palpitation,
  diarrhea and collapse
• Tumor arises from the argentaffin Kulchitsky
  chromaffin cells of the appendix or terminal
  ileum (gi, lungs, ovaries, testes)

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• The diagnosis may be established by finding high
  levels of 5-hydroxyindolacetic acid (5-HIAA) in
  the urine
• Tx- primary tumor should be removed, and excision
  of metastatic lesion should be considered
• Chemotherapy