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32 Cards in this Set

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  • Back
Dermis, subQ
Adult mid-life
painless, slow-growing, mobile, up to 1 cm
Bland spindle cells in storiform pattern
Acanthosis of overlying epidermis
Benign fibrous histiocytoma (involves langerhan's cells)
aka Dermatofibroma

BENIGN
Develops suddenly: amazingly fast growing (wks)
Volar aspect of forearm>chest>back
Deep dermis, subQ, muscle: 2-3cm
Plump fibroblasts growing in short fasciciles
Looks like tissue culture
Prominent nucleoli, many mitoses
Nodular (Pseudosarcomatous) Fasciitis

BENIGN
Affects more males than females
Fibroblasts with abundant collagen formation
Many fibroblasts are myofibroblasts (contractile -> scar/bunch up)
Superficial Fibromatoses

BENIGN
Palmer (Dupuytren contracture)
Planter (Ledderhose disease)
Penile (Peronie disease)
What type of benign tumors are these??
Superficial Fibromatoses:

BENIGN
Bilateral sometimes
4th and 5th fingers
Evolving contractures
requires surgery
Dupuytren Contracture

BENIGN
Usually 45-65
Often hx of injury - concomitant with Dupuytron or Ledderhose in 50%
Fibrotic thickening of areas of tunica albuginea
Consequences during erection (pain, abnormal bend/shortened, constriction with flail affect)
Normal flow proximal and distal to lesion... not a vascular process
Peronie Disease

BENIGN
Abdominal wall - following pregnancy
Extra-abdominal (trunk/thigh) - white rubbery masses, deep in muscle
Intra-abdominal (mesentery +/- Gardner syndrome)
Fascicles of plump fibroblasts infiltrating muscle
locally agressive/highly invasive
Triple therapy
Desmoid Tumors

BENIGN
Most common soft tissue tumor of adults
May have chromosomal abnormalities!
Soft, mobile, painless
Trunk, proximal extermities
Mature fat cells
Lipoma

BENIGN
Most common human tumor
1/4 of fertile women (more black)
May be genetic abnormalities!
Mostly in myometrium of corpus
Leiomyoma

BENIGN
Which two benign tumors have chromosomal/genetic abnormalities?
Leiomyoma
Lipoma
Where does leiomyoma typically metastasize to?
Uterine leiomyoma erodes into bvs, commonly travels to the lung
Soft Tissue Tumors: Benign
Benign fibrous ________
Nodular ______
Superficial ________
_______ tumors
Lipoma
Leiomyoma
histiocytoma
fasciitis
fibromatosis
Desmoid tumors
Most common sarcoma of adulthood (40-60)
Deep in proximal extremities and retroperitoneum
If well-differentiated - indolent
Aggressive, may metabolize
t(12;16) in 2/3 variants
Surgery - usually recur unless adequately excised
Liposarcoma

MALIGNANT
Which malignant soft tissue tumor has a t(12;16)?
Liposarcoma
Distinguishable lipoblast in Liposarcoma by the nucleus placed where?
Nucleus travels centrally - clear lipid stuff is broken up into little fatt packets

Lipocyte has nucleus at the perimeter always
Adults (more females)
Skin, deep soft tissue of extermities, retroperitoneum
Interweaving fascicles of malignant spindle cells
Prognosis related to location and size
Leimyosarcoma

MALIGNANT
Origin is fibroblastic
Mimics other soft tissue sarcomas (dx by exclusion)
Muscle of proximal extremities and retroperitoneal (deceptively circumscribed - like benign)
Spindle cells (storiform), round cells
Aggressive - 30-50% met unless excised fully
Malignant fibrous histiocytoma

MALIGNANT
Extremities, retroperitoneum. Very rare
Invasive, fish-flesh appearance. Foci of hemorrhage, necrosis
Increased mitoses, indolent vs highly pleomorphic fibroblasts
With or without herringbone pattern
Aggressive: recurrence 50%, metastasis > 25%
Fibrosarcoma

MALIGNANT
Older adults: skin, soft tissue, breast, liver
Breast: especially if radiated
Arm: 5-10 yrs post radical mastectomy: lymphangiectasia, may be associated with lymphedema, and ultimately with lymphangiosarcoma
Angiosarcoma

MALIGNANT
Gross: early, small red; late, large grey-white. Margins blend imperceptibly. Necrosis, hemorrhage
Micro: vascular channels vs solid spindle (CD31, CD34, vwf +)
Grim, few survive more than 5 yrs
Angiosarcoma: Breast

MALIGNANT
What's another term for CD31?

CD34?
PCAM - platelet endothelial cell adhesion molecule (neutrophils and endothelial cells diapedesis)

CD34 = primitive hematopoeitic cell
20-50 years
Vicinity of large joints (knee) - NOT IN THEM
Uncertain cellular origin but EMA and kertin +
Monophasic - spindle cells
Biphasic - spindle cells and glandular structures
Synovia Sarcoma

MALIGNANT
Most common STS of childhood/adolescence
Most occur in head, neck or GU - little/no sk mus component
Elsewhere (arms/legs) - related to sk mus
Most t(2;13) with PAX3/FKHR fusion
Aggressive neoplasms
65% cured with triple tx
Rhabdomyosarcoma

MALIGNANT
Which malignancy is associated with t(2;13) with PAX3/FKHR fusion
Rhabdomyosarcoma
Failure to differentiate and ultimately may turn into sarcoma
Mostly children under 10 (orbit, nose, middle ear, prostate, paratesticular region, vagina)
Commonly allelic loss of 11p15.5
Embyronal Rhabdomyosarcoma

MALIGNANT
Allelic loss of 11p15.5 creates what condition?
Embryonal Rhabdomyosarcoma
Looks like a cluster of grapes protruding into hollow, mucosa-lined structured, bladder, vagina, common bile duct, nasopharynx
Forms cambium layer (zone of hypercellularity)
Rhabditiform cells may be present
Botryoid Rhabdomyosarcoma

MALIGNANT
most common in mid-adolescence
Usually in deep musculature of extremities
Either t(1;13) or t(2;13)
Tumor traversed by fibrous septae
Cells moderate in size, many with little cytoplasm
Cross striations in about 25% of cases (Synonym...Rhabditiform)
Alveolar Rhabdomyosarcoma

MALIGNANT
Rhabditiform cells appear how?
Cross-striations
t(1;13) or t(2;13)
Alveolar Rhabdomyosarcoma
Adult variant - very rare
Tends to arise in deep soft tissues
Numerous large, often multinucleate, eosinophilic cells (tadpole, tennis-racquet, watch strap)
X-striations are diagnostic
Prognosis fair to poor with triple Rx
Pleomorphic Rhabdomyosarcoma

MALIGNANT
Soft Tissue Tumors: Malignant
Liposarcoma
Leiomyosarcoma
Malignant fibrous _______
Fibrosarcoma
Angiosarcoma
Synovial ______
Rhabdomyosarcoma
histiocytoma

sarcoma