1. Dermal and Subcutaneous Tumors

2. 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

3. 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

4. Urticaria pigmentosa

6. 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

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

10. 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

11. 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

12. Abnormalities of neural tissue

13. 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

14. Granular cell tumor
Well-circumscribed, solitary firm nodule, with a brownish red or flesh tint
Usually solitary, % 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

16. 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

17. 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

19. 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

20. Occur in many other organs
excision

21. 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

23. ganglioneuroma Rarely described in the skin as an isolated entity
Arise most often in von Recklinghausen’s neurofibromatosis
Occur in childhood

24. 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

26. 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

27. 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

28. 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

29. Abnormalities of Fat Tissue

30. 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

32. 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

33. Familial multiple lipomatosis, AD inheritance
Bannayan-Riley-Ruvalcaba syndrome
MEN 1
Frohlich’s syndrome
Gardner’s syndrome

35. 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

36. 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

37. 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

38. 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

39. 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

40. Nevus lipomatosus superficialis

42. 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

44. 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

45. 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

46. 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`

47. 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

48. 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

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

51. Abnormalities of smooth muscle

52. 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

53. Solitary cutaneous leiomyoma
Multiple cutaneous leiomyomas
Solitary genital leiomyoma
angioleiomyoma

55. Grouped leiomyomata of the back

56. 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

59. Clinically may mimic a mastocytoma, pseudo-Darier’s sign is seen in 80%
No treatment is necessary

60. 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

61. Miscellaneous tumors and tumor-associated conditions

62. 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

63. 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

64. 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

65. 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

66. 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

67. Bazex’s syndrome

68. 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

69. EGR

70. Erythema gyratum repens

71. Hypertrichosis lanuginosa

72. 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

74. 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

75. 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)

76. 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