List the (3) joint soft tissue swelling/tumors
Giant cell tumor of tendon sheath
Tenosynovial giant cell tumor
Soft tissue tumors can be divided into 5 categories. List these categories
Tumors of Adipose
Tumors of Muscle
Tumors of Smooth Muscle
Tumors of Uncertain origin
List the adipose soft tissue tumors
List the fibrous soft tissue tumors
Fibromatosis (Superficial and Deep)
List the muscle tumors
List the smooth muscle tumors
List the tumors of uncertain origin
Undifferentiated pleomorphic sarcoma
What pathology is being shown here?
Is this malignant?
Ganglion cysts, not malignant
Small cyst-like spaces with no epithelial lining, containing myxoid mucinous material
What is this called?
Usually located where in body?
What cells are commonly present in these tumors?
Giant Cell Tumor of Tendon Sheath
Usually near joints, can interfere with function
Many lipid laden macrophages in tumor, gives it the yellow color
What do Giant Cell Tumors of Tendon Sheaths look like microscopically? Youve got this
Multinucleated giant cells in a background of histiocytes
List 2 gross pathologic features seen in Diffuse type tenosynovial giant cell tumors?
Joint most commonly involved?
Does this tumor inhibit function of the joint?
Hemosiderin pigment deposition, Villi formation
Commonly involves knee
Causes destruction (and loss of function) of joint
Most common benign soft tissue tumor in adults?
Most common malignant soft tissue tumor in adults
Most common malignant soft tissue tumor in children?
By definition, lipomas are a tumor of ____ ____ tissue?
Common location of lipoma?
superficial extremity or trunk
Are lipomas associated with pain? Name an exception
No, most are painless, except angiolipomas will manifest with local pain
55-75% of lipomas are associated with what feature? (vague question)
molecular rearrangements of HMGA2/HMGIC at 12q13-15
- she said not to memorize the specific rearrangment, just know they are associated with them
Treatment for lipoma?
cut that bitch out. they be good...usually
What is this cell? What is it a hallmark cell for?
Lipoblast, hallmark cell for liposarcoma
Key feature regarding location of liposarcomas within the body?
Found in deep soft tissue of the proximal extremities.
Also can be found in the retroperitoneum
What immunostain(s) can be used to identify liposarcoma?
Molecular testing for liposarcoma?
MDM2 and CDK4
3 histologic subtypes of liposarcoma? Which is worst?
Well differentiated liposarcoma (bad)
Myxoid liposarcoma (worse)
Pleomorphic liposarcoma (you're fucked)
Are fibrous tumors common or rare?
Which one is the exception?
Relatively rare, except nodular fasciits (more common)
What is the key to identifying nodular fasciitis?
Rate of growth in nodular fasciitis?
Benign or malignant?
Nodular fasciitis commonly is associated with a hx of what in patients?
What cells are commonly found in nodular fasciitis
Since its rapid growth,
Rapid proliferation of immature fibroblasts, tons of mitotic figures, highly cellular
Within skin/subcutaneous layers, where is nodular fasciitis commonly found?
What condition is this associated with
Eggshell calficiation seen in Myositis ossificans
Fibromatosis can be divided into what 2 types?
Superficial fibromatosis definition?
infiltratitive fibroblastic proliferation that causes local deformity
Irregular nodular thickening of palmar fascia, 50% bilateral, progressive contracture of 4th and 5th fingers. What is this condition called? What tumor is it associated with (be specific)
Palmar (Dupuytren contracture), part of Superficial fibromatosis
What organ is involved in Peyronie Disease? What occurs? What tumor is it associated with (be specific)?
Palpable induration or mass on the dorsal penis. Eventual curvature of the shaft and/or constriction of the urethra.
Associated with Superficial Fibromatosis
Deep Fibromatosis definition?
Large infiltrative masses that recur but do not metastasize
Epidemiology of Deep Fibromatosis?
What disorder is Deep Fibromatosis associated with?
Bonus question: What other tumor is also associated with this disorder?
Osteomas also associated with Gardner's
What genes are associated with deep fibromatosis?
APC gene or Beta-Catenin, both lead to increased WNT signaling
Severity of deep fibromatosis?
The fibromatosis itself is not severe (does not metastize) but its the damage they can cause on other organ function that is severe. (i.e. can cause bowel obstructions etc)
Immunostain + for what in deep fibromatosis?
Beta catenin + on immunostain
Microscopy of deep fibromatosis?
DENSE collagen, spindle cell proliferation, no mitosis, no necrosis
What is being shown here? What tumor is it associated with?
malignant neoplasm of fibroblasts in a herringbone pattern
Seen in Fibrosarcoma
Over 25% of fibrosarcomas spread where? via what route?
hematogenous spread to lungs
Most frequent primary tumor of pediatric heart?
Tubular sclerosis is associated with what tumor?
Rhabdomyomas, seen in 50%
Mutations associated with Rhabdomyoma?
What is this cell? What is it associated with?
Spider cell, Rhabdomyoma
Prognosis of rhabdomyoma?
Most spontaneously regress
Sarcoma means what?
Malignant mesenchymal tumor
Rhabdomyo means what?
Rhabdomyosarcoma means what?
Malignant mesenychmal tumor with skeletal muscle differentiation
3 types of rhabdomyosarcoma?
Which 2 are more common in people < 20 years old?
Which is more common in adults?
<20 years old: alveolar, embryonal
Pediatric forms of rhabdomyosarcoma commonly arise where in body?
sinuses, head and neck, GU tract
What are these cells?
What tumor are they associated with?
Rhabdomyoblasts (eosinophilic inclusions)
associated with Rhabdomyosarcoma
Rhabdomyoblasts contain what muscle components? As a result, what stains can you use to identify them?
Contain sarcomes (thick and thin filaments) and Z-bands
Can use actin and myosins stains
Besides rhabdomyoblasts, what other cells are associated with rhabdomyosarcoma?
Elongated "strap" cells
Dx of this gross picture?
Variant of what? (be specific)
What is another differential dx?
Variant of embryonal rhabdomyosarcoma
Teratoma looks kinda similar, but isnt coming out of vagina
Translocations associated with alveolar rhabdomyosarcoma?
Which has a worse prognosis?
t(2:13) PAX3-FKHR = worse prognosis
Benign tumor of smooth muscle?
Most common neoplasm in women?
Key feature of leiomyomas on histology?
Fascicles of densely eosinophilc spindle cells that often intersect at right angles
Loss of function mutation in fumerate hydratase is associated with what 2 disorders?
Hereditary leiomyomatosis, renal cell cancer syndrome
Most common site of leiomyoma?
Uterus most common (wall of uterus is made up of smooth muscle)
However, they can be found all over body
What type of tumor is known to wrap around the esophagus?
Describe gross pathology of leiomyoma
Well-circumscribed, cut surface shows homogeneity, "white and whorled" appearance, degeneration in middle
If you see hemorrhage or necrosis, start thinking leiomyosarcoma
Account for what % of soft tissue sarcomas?
More common in men or women?
Painful or painless?
Deadly form can arise if what involved?
10-20% of soft tissue sarcomas
Typicall painless (can be large and bulky)
Deadly form can arise from the great vessels
What gross feature of leiomyosarcomas can distinguish them from leiomyomas?
will have hemorrhage, necrosis
In general, sarcomas tend to spread via what route?
Commonly spread to what organ?
hematogenous spread, as opposed to carcinomas that generally spread through lymphatics
Commonly spread to lungs
Chromosomal translocation t(X,18) asocciated with what tumor?
Are synovial sarcomas common?
Yes, 10% of all soft tissue sarcomes, ranked #4
Why is synovial sarcoma a misnomer?
B/c it was thought to start in synovium (first recognized in knee synovium), but now its known that they can present in areas without synovium
Synovial sarcoma subtypes?
Monophasic or biphasic described as uniform spindle cells with scant cytoplasm?
Biphasic has spindle cells AND gland like structures
What is considered the malignant high grade waste basket of sarcomas?
Undifferentiated pleomorphic sarcoma
- cannot be identified based on histology, immunohistochemical profile, ultrastructure, or molecular genetics