28-Fibrous, Fat,Smooth Ms Tumors Flashcards

1
Q

Most

Common location of a keloid

A

Sternal region

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2
Q

Immediate causative factor of the development of a keloid

A

Trauma

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3
Q

What differentiates keloids from hypertrophic scars histologically?

A

Thick hyalinized collagen bundles centrally

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4
Q

How do you administer triamcinolone intralesional injection

A

30 gauge needle on tuberculin syringe
40mg/ml- initial
10-20mg/ml- as lesions soften

6-8 weeks interval

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5
Q

Other forms of treatment for keloid

A

5-FU- higher hyperpigmentation, pain and ulceration risk

Flash lamp pulsed dye laser
Cryosurgery 
Intralesional etanercept
CCB (Verapamil)- decrease IL-6 and VEGF
Excision
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6
Q

Protein Known to be involved in keloid formation

A

TGF-B

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7
Q

Fibromatosis of palmar aponeurosis

Multiple firm nodules in palm

Men 30-50 years old

A

Dupuytren contracture

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8
Q

Most commonly involved part in Dupuytren contracture

A

4th finger

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9
Q

Associated conditions in dupuytren contracture

A

Alcoholic cirrhosis
DM
Muscular dystrophy
Chronic epilepsy

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10
Q

The fibrous nodules in Dupuytren contracture are composed of myofibroblasts that express _____

A

Androgen receptors

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11
Q

This hormone induces an increase in Dupuytren fibroblast proliferation

A

5a- Dihydrotestosterone

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12
Q

Management of Dupuytren contracture

A

Intralesional triamcinolone or collagenase- early disease

Surgical excision of palmar fascia

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13
Q

Slowly enlarging nodules on the soles that cause difficulty walking

Analog of Dupuytren contracture

A

Plantar fibromatosis

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14
Q

Surgical treatment for plantar fibromatosis (Ledderhose disease)

A

Wide excision of plantar fascia

Adjuvant radiotherapy to decrease recurrence

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15
Q

Fibrous infiltration of the intracavernous septum of the penis that leads to its curvature upon erection

Pain may be severe

A

Peyronie disease

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16
Q

Tumor of infancy

Poorly demarcated, slow growing soft tissue mass on an extremity

A

Lipofibromatosis

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17
Q

Associated defects with lipofibromatosis

A

Syndactyly
Cleft lip and palate
ASD
trigonocephaly

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18
Q

Heloderma (knuckle pads) develop in which part of the toes and fingers

A

Extensor aspect of proximal interphalangeal joints of toes and fingers

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19
Q

Pachydermodactyly can be misdiagnosed as

A

Juvenile idiopathic arthritis

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20
Q

Benign fibromatosis of fingers

Fullness of digits proximal to PIP joint

A

Pachydermodactyly

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21
Q

Large deep seated well circumscribed masses from muscular aponeurosis

Recur locally and can kill if they invade vital structures

A

Desmoid tumor

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22
Q

Most common site of desmoid tumor

A

Abdominal wall

Especially in pregnant women

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23
Q

Most dangerous desmoid tumors are located in

A

Root of neck and intraabdominal type

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24
Q

Local recurrence of desmoid tumor may be due to mutations in this gene

A

B-catenin

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25
Q

Treatment of desmoid tumor

A

Wide local excision
RT
hormonal- tamoxifen with sulindac

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26
Q

Most common fibrous tumor of infancy

A

Infantile myofibromatosis

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27
Q

Most common site of infantile myofibromatosis

A

Metaphyseal regions of long bones

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28
Q

Linear constriction around the affected digit that leads to the amputation of the distal part spontaneously

Black African men

Usually unilateral

A

Ainhum

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29
Q

Ainhum usually affects which digit

A

Fifth toe

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30
Q

Treatment of ainhum

A

Cutting constricting band
Amputation-advanced cases
Intralesional betamethasone

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31
Q

May be acquired, congenital or as part of a disease

Abnormal collagen bundles and altered amounts of elastin

Usually on trunk, in lumbosacral area
May be solitary or linear/zosteriform arrangement- slighlty elevated yellow to orange plaques

A

Connective tissue nevus

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32
Q

Benign tumor usually located in subscapular region

Firm, encapsulated and believed to be an unusual response to repeated trauma

Excision is curative

A

Elastofibroma dorsi

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33
Q

Dome shaped sessile skin colored white or reddish papules on or near the nose, may be solitary

Concentric fibrosis surrounding vessels and adnexal structures

Superficial shave biopsy may be mistaken for melanoma

A

Fibrous papule of the nose

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34
Q

Fibrous papule of the nose may be mistaken for:

A

Nevocytic nevus
Neurofibroma
Granuloma pyogenicum
BCC

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35
Q

Pearly white dome shaped angiofibromas occurring circumferentially on the coronal margin and sulcus of glans penis

A

Pearly penile papule

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36
Q

Pearly penile papules should be distinguised from

A

Papilloma
Hypertrophic sebaceous glands
Condyloma acuminata

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37
Q

Pinkish, hyperkeratotic, hornlike projection on finger, toe, palm or sole

Emerges from collarette of eleveted skin
Resembles a supernumerary digit, cutaneous horn or neuroma

A

Acral fibrokeratoma

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38
Q

Acral fibrokeratoma is usually acquired from

A

Immunosuppressive therapy

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39
Q

Lesions that clinically resemble warts but on biopsy show focal neovascularization and mucin like changes in papillary dermis

A

Familial myxovascular fibromas

40
Q

Subungual exostosis is a small pinkish growth beyond the inner free edge of the nail and it occurs usually on

A

Great toe

41
Q

Nodules on helix

Extremely tender and covered by adherent scale
No tendency for malignant change
Occurs on side the px favors during sleep

A

Chondrodermatitis nodularis chronica helicis

42
Q

Treatment for chondrodermatitis nodularis chronica helicis

A

Topical nitroglycerin
Change sleep position
Shave excision- refractory

43
Q

Thickening of palate, tonsillar pillars and fauces secondary to dermal and muscular fibrosis

7% of patients-malignant transformation

A

Oral submucous fibrosis

44
Q

Oral submucous fibrosis is seen among people who

A

Diet heavily seasoned with chili

Betel chewing

45
Q

Oral submucous fibrosis can transform into this type of CA

A

Squamous cell carcinoma

46
Q

Treatment for oral submucous fibrosis

A

Intralesional triamcinolone

Surgical excision and grafting

47
Q

Flesh colored to dark brown pedunculated/ sessile papillomas usually in the neck

Often in association with seborrheic keratosis

A

Acrochordon (skin tag)

48
Q

Skin tags often occur when there is weight gain, DM or pregnancy because of the growth hormone like activity of ____

A

Insulin

49
Q

Management of acrochordon

A

Small lesions- clipped off at base, light electrodessication

Larger lesions- snip excision

50
Q

Biopsy should be performed on children with skin tags because it might be associated with this disease

A

NBCCS

Nevoid basal cell carcinoma syndrome

51
Q

Single round or ovoid papule or nodule, reddish brown

May be elecated or slightly depressed
β€œDimple sign”

A

Dermatofibroma

52
Q

Most common sites of dermatofibroma

A

Lower extremities
Above elbows
Sides of trunk

53
Q

Presence of this in histology is pathognomonic of dermatofibroma

A

Touton giant cells containing hemosiderin

54
Q

Treatment of dermatofibroma

A

None

Involution may occur after several years

55
Q

Bulky protuberant neopastic masses that usually occurs in the trunk

Erythematous firm nodules or plaques often with purulent exudate or ulceration
Slowly growing and with pain

A

Dermatofibrosarcoma protruberans

DFSP

56
Q

Pigmented DFSP- cells contain melanin

Usually occurs in persons of color

A

Bednar tumors

57
Q

These tumor markers distinguish DFSP from dermatofibroma

A

(+) CD34

(+) Nestin

58
Q

Treatment of choice for Dermatofibrosarcoma protuberans

A

Mohs surgery

59
Q

Low grade malignancy that occurs on sun exposed parts of the head and neck in white persons over age 50

Small firm nodule with an eroded or crusted surface

A

Atypical fibroxanthoma

60
Q

Staining that differentiates atypical fibroxanthoma from SCC

A

S100- Dendritic cells but not tumor cells

Prekeratin staining negative

61
Q

Treatment for atypical fibroxanthoma (AFX)

A

Complete surgical excision

Mohs- fewer recurrences and smaller defects

62
Q

Most common soft tissue sarcoma of middle and late adulthood

A

Undifferentiated pleomorphic sarcoma

63
Q

Sarcoma that is usually seen in young adults (2/3 men)

Found on extremities (50% hands and wrist)
Tumor grows slowly among fascial structures and tendons
often with central necrosis of tumor nodules and skin ulceration

A

Epitheloid sarcoma

64
Q

Mastocytosis has revealed mutations in this proto-oncogene in many adult cases

A

c-KIT

65
Q

Most common type of cutaneous mastocytosis

A

Urticaria pigmentosa

β€œMaculopapular β€œ cutaneous mastocytosis

66
Q

Patients with this type of mastocytosis usually lack skin lesions

A

Aggressive systemic mastocytosis

67
Q

What substances are known mast cell degranulators?

A

Alcohol
Morphine
Codeine
Extended rubbing of skin

68
Q

Mast cells also produce this, which may result in hematemesis, epistaxis, melena and ecchymoses

A

Heparin

69
Q

Solitary mastocytoma usually appears as a brown macule that urticates on stroking

It usually appears on which body part

A

Dorsum of the hand near the wrist

70
Q

Difference of urticaria pigmentosa and urticaria

A

Urticaria pigmentosa lesions are not evanescent

71
Q

Systemic mastocytosis is diagnosed by fulfilling how many major and minor criteria?

A

1 major and 1 minor or

3 minor

72
Q

Major criterion of systemic mastocytosis

A

dense infiltrates of mast cells in bone marrow or other extracutaneous tissues

73
Q

4 minor criteria in systemic mastocytosis

A

Atypical mast cell morphology
Aberrant mast cell surface phenotype (CD25/ CD2)
Serum tryptase >20 ng/mL
Codon 816 c-KIT mutation in peripheral blood, BM, lesional tissue

74
Q

Most common type of systemic mastocytosis in adults

A

Indolent systemic mastocytosis

75
Q

Preferred laboratory test to demonstrate evidence of increased mast cell burden

A

Serum tryptase

76
Q

Confirmatory diagnosis of cutaneous mastocytosis

A

Biopsy of lesion- increased mast cells

77
Q

Once the diagnosis of mastocytosis is made with biopsy, this is the next step

A

Assess for bone marrow involvement

78
Q

(Mastocytosis)

For asymptomatic adults whose sign is only skin lesion and who choose not to have a bone marrow exam, these tests shoud be done

A

Serum tryptase and CBC yearly

79
Q

Symptomatic relief of lesions in mastocytosis

A

H1 blockers
H2 blockers-ex. cimetidine
Antiserotonin- cyproheptadine

PUVA
Intralesional triamcinolone/ topical steroids
Avoid physical stimuli

80
Q

What class of drugs given for HIV disease may induce lipomas?

A

Protease inhibitors

Ex. indinavir

81
Q

Lipomas are most often found in this area

A

Trunk

82
Q

Multiple lipoma that most often occurs in middle age men that may develop painless coalescent lipomas around the neck, shoulders and upper arms

A

Madelung’s disease

83
Q

Multiple lipoma seen in obese or menopausal women who develop symmetric, tender, circumscribed fatty lesions

Accompanied by weakness and psychiatric conditions

A

Dercum’s disease

84
Q

This syndrome consists of multiple osteomas, fibromas, desmoid tumors, lipomas, fibrosarcomas, epidermal inclusion cysts and leiomyomas

Associated with intestinal polyposis exclusively in the colon and rectum

A

Gardner syndrome

85
Q

[Subtypes of Lipoma]
Present as painful subcutaneous nodules

Have no invasive or metastatic potential
May be associated with capillary malformation and protease inhibitor use

A

Angiolipomas

86
Q

[Subtypes of Lipoma]
Overgrowth of fibrofatty tissue along a nerve trunk that often leads to a nerve compression

Patients are usually age 30 or younger

A

Neural fibrolipoma

87
Q

Most common nerve involved in neural fibrolipoma

A

Median nerve

88
Q

[Subtypes of Lipoma]

Deep seated, firm, yellow tumors that characteristically occur on legs of women

A

Chondroid lipoma

89
Q

[Subtypes of Lipoma]
Composed of finely vacuolated fat cells of embryonic type

Dostinctive brownish color and firm consistency
Usually occurs singly; benign tumor
Mediastinum is and interscapular region

A

Hibernoma (lipoma of brown fat)

90
Q

Most common soft tissue sarcoma

A

Liposarcoma

91
Q

Liposarcomas usually arise from what tissue

A

Intermuscular fascia

92
Q

A fatty tumor must be considered as a liposarcoma when the size becomes

A

Larger than 10cm

93
Q

Most common site of liposarcoma

A

Upper thigh

94
Q

Treatment for liposarcoma

A

Radical excision

Radiotherapy-metastatic liposarcoma

95
Q

Skin colored or lightly pigmented patch or plaque with hypertrichosis usually on the trunk (lumbosacral)

A

Congenital smooth muscle hamartoma

96
Q

Congenital smooth muscle hamartoma is clinically and histologically overlapping with this condition

A

Becker nevus