28-Fibrous, Fat,Smooth Ms Tumors Flashcards
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Common location of a keloid
Sternal region
Immediate causative factor of the development of a keloid
Trauma
What differentiates keloids from hypertrophic scars histologically?
Thick hyalinized collagen bundles centrally
How do you administer triamcinolone intralesional injection
30 gauge needle on tuberculin syringe
40mg/ml- initial
10-20mg/ml- as lesions soften
6-8 weeks interval
Other forms of treatment for keloid
5-FU- higher hyperpigmentation, pain and ulceration risk
Flash lamp pulsed dye laser Cryosurgery Intralesional etanercept CCB (Verapamil)- decrease IL-6 and VEGF Excision
Protein Known to be involved in keloid formation
TGF-B
Fibromatosis of palmar aponeurosis
Multiple firm nodules in palm
Men 30-50 years old
Dupuytren contracture
Most commonly involved part in Dupuytren contracture
4th finger
Associated conditions in dupuytren contracture
Alcoholic cirrhosis
DM
Muscular dystrophy
Chronic epilepsy
The fibrous nodules in Dupuytren contracture are composed of myofibroblasts that express _____
Androgen receptors
This hormone induces an increase in Dupuytren fibroblast proliferation
5a- Dihydrotestosterone
Management of Dupuytren contracture
Intralesional triamcinolone or collagenase- early disease
Surgical excision of palmar fascia
Slowly enlarging nodules on the soles that cause difficulty walking
Analog of Dupuytren contracture
Plantar fibromatosis
Surgical treatment for plantar fibromatosis (Ledderhose disease)
Wide excision of plantar fascia
Adjuvant radiotherapy to decrease recurrence
Fibrous infiltration of the intracavernous septum of the penis that leads to its curvature upon erection
Pain may be severe
Peyronie disease
Tumor of infancy
Poorly demarcated, slow growing soft tissue mass on an extremity
Lipofibromatosis
Associated defects with lipofibromatosis
Syndactyly
Cleft lip and palate
ASD
trigonocephaly
Heloderma (knuckle pads) develop in which part of the toes and fingers
Extensor aspect of proximal interphalangeal joints of toes and fingers
Pachydermodactyly can be misdiagnosed as
Juvenile idiopathic arthritis
Benign fibromatosis of fingers
Fullness of digits proximal to PIP joint
Pachydermodactyly
Large deep seated well circumscribed masses from muscular aponeurosis
Recur locally and can kill if they invade vital structures
Desmoid tumor
Most common site of desmoid tumor
Abdominal wall
Especially in pregnant women
Most dangerous desmoid tumors are located in
Root of neck and intraabdominal type
Local recurrence of desmoid tumor may be due to mutations in this gene
B-catenin
Treatment of desmoid tumor
Wide local excision
RT
hormonal- tamoxifen with sulindac
Most common fibrous tumor of infancy
Infantile myofibromatosis
Most common site of infantile myofibromatosis
Metaphyseal regions of long bones
Linear constriction around the affected digit that leads to the amputation of the distal part spontaneously
Black African men
Usually unilateral
Ainhum
Ainhum usually affects which digit
Fifth toe
Treatment of ainhum
Cutting constricting band
Amputation-advanced cases
Intralesional betamethasone
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
Benign tumor usually located in subscapular region
Firm, encapsulated and believed to be an unusual response to repeated trauma
Excision is curative
Elastofibroma dorsi
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
Fibrous papule of the nose may be mistaken for:
Nevocytic nevus
Neurofibroma
Granuloma pyogenicum
BCC
Pearly white dome shaped angiofibromas occurring circumferentially on the coronal margin and sulcus of glans penis
Pearly penile papule
Pearly penile papules should be distinguised from
Papilloma
Hypertrophic sebaceous glands
Condyloma acuminata
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
Acral fibrokeratoma is usually acquired from
Immunosuppressive therapy
Lesions that clinically resemble warts but on biopsy show focal neovascularization and mucin like changes in papillary dermis
Familial myxovascular fibromas
Subungual exostosis is a small pinkish growth beyond the inner free edge of the nail and it occurs usually on
Great toe
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
Treatment for chondrodermatitis nodularis chronica helicis
Topical nitroglycerin
Change sleep position
Shave excision- refractory
Thickening of palate, tonsillar pillars and fauces secondary to dermal and muscular fibrosis
7% of patients-malignant transformation
Oral submucous fibrosis
Oral submucous fibrosis is seen among people who
Diet heavily seasoned with chili
Betel chewing
Oral submucous fibrosis can transform into this type of CA
Squamous cell carcinoma
Treatment for oral submucous fibrosis
Intralesional triamcinolone
Surgical excision and grafting
Flesh colored to dark brown pedunculated/ sessile papillomas usually in the neck
Often in association with seborrheic keratosis
Acrochordon (skin tag)
Skin tags often occur when there is weight gain, DM or pregnancy because of the growth hormone like activity of ____
Insulin
Management of acrochordon
Small lesions- clipped off at base, light electrodessication
Larger lesions- snip excision
Biopsy should be performed on children with skin tags because it might be associated with this disease
NBCCS
Nevoid basal cell carcinoma syndrome
Single round or ovoid papule or nodule, reddish brown
May be elecated or slightly depressed
“Dimple sign”
Dermatofibroma
Most common sites of dermatofibroma
Lower extremities
Above elbows
Sides of trunk
Presence of this in histology is pathognomonic of dermatofibroma
Touton giant cells containing hemosiderin
Treatment of dermatofibroma
None
Involution may occur after several years
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
Pigmented DFSP- cells contain melanin
Usually occurs in persons of color
Bednar tumors
These tumor markers distinguish DFSP from dermatofibroma
(+) CD34
(+) Nestin
Treatment of choice for Dermatofibrosarcoma protuberans
Mohs surgery
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
Staining that differentiates atypical fibroxanthoma from SCC
S100- Dendritic cells but not tumor cells
Prekeratin staining negative
Treatment for atypical fibroxanthoma (AFX)
Complete surgical excision
Mohs- fewer recurrences and smaller defects
Most common soft tissue sarcoma of middle and late adulthood
Undifferentiated pleomorphic sarcoma
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
Mastocytosis has revealed mutations in this proto-oncogene in many adult cases
c-KIT
Most common type of cutaneous mastocytosis
Urticaria pigmentosa
“Maculopapular “ cutaneous mastocytosis
Patients with this type of mastocytosis usually lack skin lesions
Aggressive systemic mastocytosis
What substances are known mast cell degranulators?
Alcohol
Morphine
Codeine
Extended rubbing of skin
Mast cells also produce this, which may result in hematemesis, epistaxis, melena and ecchymoses
Heparin
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
Difference of urticaria pigmentosa and urticaria
Urticaria pigmentosa lesions are not evanescent
Systemic mastocytosis is diagnosed by fulfilling how many major and minor criteria?
1 major and 1 minor or
3 minor
Major criterion of systemic mastocytosis
dense infiltrates of mast cells in bone marrow or other extracutaneous tissues
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
Most common type of systemic mastocytosis in adults
Indolent systemic mastocytosis
Preferred laboratory test to demonstrate evidence of increased mast cell burden
Serum tryptase
Confirmatory diagnosis of cutaneous mastocytosis
Biopsy of lesion- increased mast cells
Once the diagnosis of mastocytosis is made with biopsy, this is the next step
Assess for bone marrow involvement
(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
Symptomatic relief of lesions in mastocytosis
H1 blockers
H2 blockers-ex. cimetidine
Antiserotonin- cyproheptadine
PUVA
Intralesional triamcinolone/ topical steroids
Avoid physical stimuli
What class of drugs given for HIV disease may induce lipomas?
Protease inhibitors
Ex. indinavir
Lipomas are most often found in this area
Trunk
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
Multiple lipoma seen in obese or menopausal women who develop symmetric, tender, circumscribed fatty lesions
Accompanied by weakness and psychiatric conditions
Dercum’s disease
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
[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
[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
Most common nerve involved in neural fibrolipoma
Median nerve
[Subtypes of Lipoma]
Deep seated, firm, yellow tumors that characteristically occur on legs of women
Chondroid lipoma
[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)
Most common soft tissue sarcoma
Liposarcoma
Liposarcomas usually arise from what tissue
Intermuscular fascia
A fatty tumor must be considered as a liposarcoma when the size becomes
Larger than 10cm
Most common site of liposarcoma
Upper thigh
Treatment for liposarcoma
Radical excision
Radiotherapy-metastatic liposarcoma
Skin colored or lightly pigmented patch or plaque with hypertrichosis usually on the trunk (lumbosacral)
Congenital smooth muscle hamartoma
Congenital smooth muscle hamartoma is clinically and histologically overlapping with this condition
Becker nevus
