28-Fibrous, Fat,Smooth Ms Tumors Flashcards
1
Q
Most
Common location of a keloid
A
Sternal region
2
Q
Immediate causative factor of the development of a keloid
A
Trauma
3
Q
What differentiates keloids from hypertrophic scars histologically?
A
Thick hyalinized collagen bundles centrally
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
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
6
Q
Protein Known to be involved in keloid formation
A
TGF-B
7
Q
Fibromatosis of palmar aponeurosis
Multiple firm nodules in palm
Men 30-50 years old
A
Dupuytren contracture
8
Q
Most commonly involved part in Dupuytren contracture
A
4th finger
9
Q
Associated conditions in dupuytren contracture
A
Alcoholic cirrhosis
DM
Muscular dystrophy
Chronic epilepsy
10
Q
The fibrous nodules in Dupuytren contracture are composed of myofibroblasts that express _____
A
Androgen receptors
11
Q
This hormone induces an increase in Dupuytren fibroblast proliferation
A
5a- Dihydrotestosterone
12
Q
Management of Dupuytren contracture
A
Intralesional triamcinolone or collagenase- early disease
Surgical excision of palmar fascia
13
Q
Slowly enlarging nodules on the soles that cause difficulty walking
Analog of Dupuytren contracture
A
Plantar fibromatosis
14
Q
Surgical treatment for plantar fibromatosis (Ledderhose disease)
A
Wide excision of plantar fascia
Adjuvant radiotherapy to decrease recurrence
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
16
Q
Tumor of infancy
Poorly demarcated, slow growing soft tissue mass on an extremity
A
Lipofibromatosis
17
Q
Associated defects with lipofibromatosis
A
Syndactyly
Cleft lip and palate
ASD
trigonocephaly
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
19
Q
Pachydermodactyly can be misdiagnosed as
A
Juvenile idiopathic arthritis
20
Q
Benign fibromatosis of fingers
Fullness of digits proximal to PIP joint
A
Pachydermodactyly
21
Q
Large deep seated well circumscribed masses from muscular aponeurosis
Recur locally and can kill if they invade vital structures
A
Desmoid tumor
22
Q
Most common site of desmoid tumor
A
Abdominal wall
Especially in pregnant women
23
Q
Most dangerous desmoid tumors are located in
A
Root of neck and intraabdominal type
24
Q
Local recurrence of desmoid tumor may be due to mutations in this gene
A
B-catenin
25
Treatment of desmoid tumor
Wide local excision
RT
hormonal- tamoxifen with sulindac
26
Most common fibrous tumor of infancy
Infantile myofibromatosis
27
Most common site of infantile myofibromatosis
Metaphyseal regions of long bones
28
Linear constriction around the affected digit that leads to the amputation of the distal part spontaneously
Black African men
Usually unilateral
Ainhum
29
Ainhum usually affects which digit
Fifth toe
30
Treatment of ainhum
Cutting constricting band
Amputation-advanced cases
Intralesional betamethasone
31
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
Connective tissue nevus
32
Benign tumor usually located in subscapular region
Firm, encapsulated and believed to be an unusual response to repeated trauma
Excision is curative
Elastofibroma dorsi
33
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
Fibrous papule of the nose
34
Fibrous papule of the nose may be mistaken for:
Nevocytic nevus
Neurofibroma
Granuloma pyogenicum
BCC
35
Pearly white dome shaped angiofibromas occurring circumferentially on the coronal margin and sulcus of glans penis
Pearly penile papule
36
Pearly penile papules should be distinguised from
Papilloma
Hypertrophic sebaceous glands
Condyloma acuminata
37
Pinkish, hyperkeratotic, hornlike projection on finger, toe, palm or sole
Emerges from collarette of eleveted skin
Resembles a supernumerary digit, cutaneous horn or neuroma
Acral fibrokeratoma
38
Acral fibrokeratoma is usually acquired from
Immunosuppressive therapy
39
Lesions that clinically resemble warts but on biopsy show focal neovascularization and mucin like changes in papillary dermis
Familial myxovascular fibromas
40
Subungual exostosis is a small pinkish growth beyond the inner free edge of the nail and it occurs usually on
Great toe
41
Nodules on helix
Extremely tender and covered by adherent scale
No tendency for malignant change
Occurs on side the px favors during sleep
Chondrodermatitis nodularis chronica helicis
42
Treatment for chondrodermatitis nodularis chronica helicis
Topical nitroglycerin
Change sleep position
Shave excision- refractory
43
Thickening of palate, tonsillar pillars and fauces secondary to dermal and muscular fibrosis
7% of patients-malignant transformation
Oral submucous fibrosis
44
Oral submucous fibrosis is seen among people who
Diet heavily seasoned with chili
| Betel chewing
45
Oral submucous fibrosis can transform into this type of CA
Squamous cell carcinoma
46
Treatment for oral submucous fibrosis
Intralesional triamcinolone
| Surgical excision and grafting
47
Flesh colored to dark brown pedunculated/ sessile papillomas usually in the neck
Often in association with seborrheic keratosis
Acrochordon (skin tag)
48
Skin tags often occur when there is weight gain, DM or pregnancy because of the growth hormone like activity of ____
Insulin
49
Management of acrochordon
Small lesions- clipped off at base, light electrodessication
Larger lesions- snip excision
50
Biopsy should be performed on children with skin tags because it might be associated with this disease
NBCCS
| Nevoid basal cell carcinoma syndrome
51
Single round or ovoid papule or nodule, reddish brown
May be elecated or slightly depressed
“Dimple sign”
Dermatofibroma
52
Most common sites of dermatofibroma
Lower extremities
Above elbows
Sides of trunk
53
Presence of this in histology is pathognomonic of dermatofibroma
Touton giant cells containing hemosiderin
54
Treatment of dermatofibroma
None
| Involution may occur after several years
55
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
Dermatofibrosarcoma protruberans
DFSP
56
Pigmented DFSP- cells contain melanin
Usually occurs in persons of color
Bednar tumors
57
These tumor markers distinguish DFSP from dermatofibroma
(+) CD34
| (+) Nestin
58
Treatment of choice for Dermatofibrosarcoma protuberans
Mohs surgery
59
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
Atypical fibroxanthoma
60
Staining that differentiates atypical fibroxanthoma from SCC
S100- Dendritic cells but not tumor cells
| Prekeratin staining negative
61
Treatment for atypical fibroxanthoma (AFX)
Complete surgical excision
| Mohs- fewer recurrences and smaller defects
62
Most common soft tissue sarcoma of middle and late adulthood
Undifferentiated pleomorphic sarcoma
63
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
Epitheloid sarcoma
64
Mastocytosis has revealed mutations in this proto-oncogene in many adult cases
c-KIT
65
Most common type of cutaneous mastocytosis
Urticaria pigmentosa
| “Maculopapular “ cutaneous mastocytosis
66
Patients with this type of mastocytosis usually lack skin lesions
Aggressive systemic mastocytosis
67
What substances are known mast cell degranulators?
Alcohol
Morphine
Codeine
Extended rubbing of skin
68
Mast cells also produce this, which may result in hematemesis, epistaxis, melena and ecchymoses
Heparin
69
Solitary mastocytoma usually appears as a brown macule that urticates on stroking
It usually appears on which body part
Dorsum of the hand near the wrist
70
Difference of urticaria pigmentosa and urticaria
Urticaria pigmentosa lesions are not evanescent
71
Systemic mastocytosis is diagnosed by fulfilling how many major and minor criteria?
1 major and 1 minor or
| 3 minor
72
Major criterion of systemic mastocytosis
dense infiltrates of mast cells in bone marrow or other extracutaneous tissues
73
4 minor criteria in systemic mastocytosis
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
Most common type of systemic mastocytosis in adults
Indolent systemic mastocytosis
75
Preferred laboratory test to demonstrate evidence of increased mast cell burden
Serum tryptase
76
Confirmatory diagnosis of cutaneous mastocytosis
Biopsy of lesion- increased mast cells
77
Once the diagnosis of mastocytosis is made with biopsy, this is the next step
Assess for bone marrow involvement
78
(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
Serum tryptase and CBC yearly
79
Symptomatic relief of lesions in mastocytosis
H1 blockers
H2 blockers-ex. cimetidine
Antiserotonin- cyproheptadine
PUVA
Intralesional triamcinolone/ topical steroids
Avoid physical stimuli
80
What class of drugs given for HIV disease may induce lipomas?
Protease inhibitors
| Ex. indinavir
81
Lipomas are most often found in this area
Trunk
82
Multiple lipoma that most often occurs in middle age men that may develop painless coalescent lipomas around the neck, shoulders and upper arms
Madelung’s disease
83
Multiple lipoma seen in obese or menopausal women who develop symmetric, tender, circumscribed fatty lesions
Accompanied by weakness and psychiatric conditions
Dercum’s disease
84
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
Gardner syndrome
85
[Subtypes of Lipoma]
Present as painful subcutaneous nodules
Have no invasive or metastatic potential
May be associated with capillary malformation and protease inhibitor use
Angiolipomas
86
[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
Neural fibrolipoma
87
Most common nerve involved in neural fibrolipoma
Median nerve
88
[Subtypes of Lipoma]
| Deep seated, firm, yellow tumors that characteristically occur on legs of women
Chondroid lipoma
89
[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
Hibernoma (lipoma of brown fat)
90
Most common soft tissue sarcoma
Liposarcoma
91
Liposarcomas usually arise from what tissue
Intermuscular fascia
92
A fatty tumor must be considered as a liposarcoma when the size becomes
Larger than 10cm
93
Most common site of liposarcoma
Upper thigh
94
Treatment for liposarcoma
Radical excision
| Radiotherapy-metastatic liposarcoma
95
Skin colored or lightly pigmented patch or plaque with hypertrichosis usually on the trunk (lumbosacral)
Congenital smooth muscle hamartoma
96
Congenital smooth muscle hamartoma is clinically and histologically overlapping with this condition
Becker nevus
