Dermal and Subcutaneous Tumors
Mastocytosis
Urticaria 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 < 6 mo
* Macules, papules, nodules, plaques, vesicles
* Lesions persist and gradually become chamois- or slate-colored
* Darier’s 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
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
* Generalized eruption, childhood type
* Pseudoxanthomatous mastocytosis
* Diffuse cutaneous mastocytosis
* Generalized eruption, adult type
* Erythrodermic mastocytosis
* Telangiectasia macularis erupta perstans
* Systemic mastocytosis
* Familial urticaria pigmentosa
* 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
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
Abnormalities of neural tissue
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
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
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
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
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
* Occur in many other organs
* excision
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
ganglioneuroma
* Rarely described in the skin as an isolated entity
* Arise most often in von Recklinghausen’s neurofibromatosis
* Occur in childhood
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
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
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
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
Abnormalities of Fat Tissue
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
* Solitary lesions reaching greater than 10 cm should be investigated for malignancy
* Multiple lesion may be painful if growing rapidly
* Madelung’s disease, benign symmetric lipomatosis
* Dercum’s disease, assoc with weakness and psychiatric disturbances
* Familial multiple lipomatosis, AD inheritance
* Bannayan-Riley-Ruvalcaba syndrome
* MEN 1
* Frohlich’s syndrome
* Gardner’s syndrome
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
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
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
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
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
* Nevus lipomatosus superficialis
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
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
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
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`
Benign lipoblastomatosis
* Frequently confused with a liposarcoma
* Affects exclusively infants and young children, 90% < 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
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
* Adult males are mostly affected
* May be well or poorly differentiated
* Tx is adequate radical excision
* For metastatic liposarcomas, radiation therapy may be effective
Abnormalities of smooth muscle
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
* Solitary cutaneous leiomyoma
* Multiple cutaneous leiomyomas
* Solitary genital leiomyoma
* angioleiomyoma
Grouped leiomyomata of the back
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
* Clinically may mimic a mastocytoma, pseudo-Darier’s sign is seen in 80%
* No treatment is necessary
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
Miscellaneous tumors and tumor-associated conditions
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
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
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
* 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
Paraneoplastic syndromes
* Some cancers produce findings in the skin that indicate to the clinician that an underlying internal malignancy may be present
* Bazex’s 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
Bazex’s syndrome
* 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
EGR
Hypertrichosis lanuginosa
* The sign of Lesser-Trelat, the sudden appearance of multiple pruritic seborrheic keratosis, associated with and internal malignancy
* Trousseau’s sign, migratory thrombophlebitis, pancreatic ca
* Pityriasis rotunda
* Tripe palms
* Several others with less frequency
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
* 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)
* 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
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