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65 Cards in this Set
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types of soft tissue lesions?
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*Mass-forming reactive processes
*Neoplasms -Benign (-oma) -Malignant (-sarcoma) |
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Prefixes:
Fibrous tissue neoplasms (fibro-) Fatty tissue neoplasms (lipo-) Smooth muscle (leiomyo-) Skeletal muscle (rhabdomyo-) |
Fibrous tissue neoplasms (fibro-)
Fatty tissue neoplasms (lipo-) Smooth muscle (leiomyo-) Skeletal muscle (rhabdomyo-) |
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Analyzing soft tissue tumors: information to consider:
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*Age of patient
-Rhabdomyosarcoma in kids -Synovial sarcoma in young adults -Liposarcoma and MFH in older adults *Location of lesion -40% in lower extremity, especially in thigh -Deep lesions generally worse than superficial *What is the cell of origin? *If malignant (sarcoma): what is the grade? -Grade relates to prognosis Grade I, low, well-differentiated Grade II, moderate, moderately differentiated Grade III, high, poorly differentiated -Mitotic rate, necrosis especially important |
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tl: adipose
tr: fibrous bl: smooth muscle br: skeletal muscle |
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Significance of staging in soft tissue tumors:
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*Staging is important in prognosis
*Size is most important factor: <5 cm sarcoma: 30% of cases will have metastasis >20 cm sarcoma: 80% of cases will have metastasis *Overall 10yr survival rate for sarcoma: 40% |
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Fibrous tumor-like proliferations--list types:
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*Reactive processes which are not aggressive
Nodular fasciitis Myositis ossificans *Locally aggressive – ‘fibromatoses’ Superficial Deep |
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Nodular fasciitis:
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*Self-limited fibroblastic proliferation
*Presentation: -Adults; volar forearm, chest, back -Several weeks of solitary, rapidly growing, sometimes painful mass -Preceding trauma noted in 10-15% *Over time may spontaneously regress *Rarely recur after excision |
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What does Nodular fasciitis look like:
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*Gross: several cm in diameter, nodular, poorly defined margins
*Histology: -very cellular with plump, immature fibroblasts (‘tissue-culture’ appearance) -Myxoid stroma -Often conspicuous nucleoli, mitoses present |
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*note triangular fibroblast nucleus upper center
*background is myxoid, blue--a tipoff that this is NODULAR FASCIITIS |
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Myositis ossificans:
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*Post-traumatic bony metaplasia
*Presentation: -Athletic adolescents/young adults -Proximal muscles of extremities -Initially swollen/painful, evolves to painless, hard, well demarcated mass, bony on Xray *Simple excision is curative *Differential diagnosis: osteosarcoma |
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Fibromatoses
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*Infiltrative fibroblastic proliferations
-May be locally aggressive -May recur after excision *Disfiguring, disturb function, +/- painful *Two clinicopathologic groups 1) superficial and 2) deep Histologically similar |
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What do fibromatoses look like:
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*Gross: 1-15 cm size, gray-white, firm, rubbery, poorly demarcated
*Histology: cell of origin is myofibroblast -Plump cells, broad sweeping fascicles -Penetrate adjacent tissue -Infrequent mitoses -Early lesions cellular, later are less cellular with abundant collagen (esp. superficial ones) |
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Fibromatoses!
*Histology: cell of origin is myofibroblast -Plump cells, broad sweeping fascicles -Penetrate adjacent tissue -Infrequent mitoses -Early lesions cellular, later are less cellular with abundant collagen (esp. superficial ones) |
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superficial Fibromatoses:
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*Presentation:
M>F, present early, deformation of hand (often bilateral), foot, penis *Eponyms Palmar fibromatosis – Dupuytren’s contracture Penile fibromatosis – Peyronie’s disease *May stabilize, or resect +/- recurrence *May show trisomy 3 or trisomy 8 |
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Palmar fibromatosis, aka Dupuytren’s contracture
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deep Fibromatoses:
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*Any age, but most frequent teens-30s
*Muscles of trunk or extremity -Shoulder, thigh, chest wall, back *Abdominal wall and abdominal cavity -In abdomen, often called “desmoid tumor” -Present with abdominal signs (abd or flank pain, bowel obstruction, hydronephrosis, etc.) *APC gene (5q21-q22) mutation *Beta-catenin gene overexpression *Component of Gardner syndrome -Autosomal dominant -Germline mutation of APC gene -Fibromatoses, intestinal adenomas, osteomas |
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Do fibromatoses metastasize? How do you treat them?
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Do not metastasize
Treatment: Some superficial ones may stabilize Resection, but often recur, repeatedly Some respond to tamoxifen Others insensitive to chemo/radiation |
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Fibrous/fibrohistiocytic tumors: types:
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*Benign
Fibroma Benign fibrous histiocytoma (aka dermatofibroma) *Malignant Fibrosarcoma Malignant fibrous histiocytoma *‘histiocytic’ does not describe cell origin! |
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Fibroma:
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*Rare, most often a small ovarian mass
*Gross: well-demarcated, round, firm, white nodule *Histology: mature fibroblasts, copious collagen |
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Benign fibrous histiocytoma:
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*Common benign lesion
*Presentation: Adults, dermis or subcutis, firm nodule, <1 cm *Histology: Bland, mature fibroblasts with interlacing histiocyte-like cells *Cured by simple excision |
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Fibrosarcoma:
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*Presentation:
-Adults -Deep tissue of thigh, knee, retroperitoneum -Slow growth, present years once diagnosed >50% recur locally after excision Metastasize hematogenously (lungs) |
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What do fibrosarcomas look like:
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Gross:
Soft, unencapsulated, infiltrative Areas of hemorrhage and necrosis Histology: varies, may be: Bland, like a fibromatosis Cellular, with herringbone pattern Pleomorphic with many mitoses and necrosis |
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think????
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fibrosarcoma- herringbone pattern
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fibrosarcoma
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fibrosarcoma
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Malignant fibrous histiocytoma:
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*Historically represented variety of sarcomas
*Histology: Pleomorphic, bizarre giant cells Storiform (swirling) architecture Necrosis, hemorrhage, many mitoses *Very aggressive behavior |
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Malignant fibrous histiocytoma
-very pleomorphic and cellular |
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Malignant fibrous histiocytoma:
pleomorphic, unspecific looking |
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Diagnosing Malignant fibrous histiocytoma:
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*Currently MFH is a diagnosis of exclusion
aka pleomorphic sarcoma, NOS *Immunohistochemistry: -Doesn’t stain for: S-100 (lipo) Desmin (rhabdo, leio) Smooth muscle actin (leio) Myogenin or myoD1 (rhabdo) Cytokeratin or EMA (synovial sarcoma) |
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Tumors of fat (adipocytes)- types:
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Benign: Lipoma
Malignant: Liposarcoma Immunohistochemistry: Usually positive for S-100 |
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Lipoma:
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*Most common soft tissue tumor of adults
*Presentation: Adults, usually solitary, slowly enlarging, mobile, painless mass *Complete excision usually curative |
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what do lipomas look like?
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Gross: soft, yellow, encapsulated
Histology: mature adipocytes *May be subclassified by: Other histologic features: conventional, myolipoma, spindle cell, myelolipoma, angiolipoma, pleomorphic characteristic chromosomal rearrangements (don’t worry about these) |
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lipoma
-there would be a thin fibous capsule around the edge of the lesion |
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Liposarcoma:
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*Presentation:
Adult, 5th and 6th decades, mass or functional changes, in deep soft tissues or viscera *Gross: Fatty, may be TAN-WHITE, have hemorrhage or necrosis *Histology: Varies, type is prognostically important Diagnostic cell is the lipoblast |
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*Liposarcoma
*scalloped nucleus (LIPOBLAST) compressed by multiple little fat globs |
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*Liposarcoma
*scalloped nucleus (LIPOBLAST) compressed by multiple little fat globs |
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*Liposarcoma
*scalloped nucleus (LIPOBLAST) compressed by multiple little fat globs |
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Liposarcoma, histology:
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*Well-differentiated:
Mature adipocytes with some lipoblasts *Myxoid: As above, but with a bluish, mucoid matrix, and chicken-wire vascular pattern *Round cell: Lipoblasts present, but majority are small, round cells *Pleomorphic: Significant pleomorphism |
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Myxoid liposarcoma
Chicken wire vascular pattern |
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Myxoid liposarcoma
Chicken wire vascular pattern Note lipoblast on left center |
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Why is important to be able to differentiate b/t types of liposarcoma based on their appearance?
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Well-differentiated and myxoid types are indolent
Round cell and pleomorphic types are aggressive, recur after excision, metastasize to lungs |
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mutations associated with Liposarcomas:
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*Well-differentiated: amp 12q
-Increased transcription of MDM2 gene -Product binds, inactivates p53 (tumor suppressor) *All myxoid (and some round cell): t(12;16) -CHOP gene (adipocyte differentiation) on 12q13 -FUS gene (transcription factor) on 16p |
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Smooth muscle tumors:
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Benign: leiomyoma
Malignant: leiomyosarcoma Immunohistochemistry: Usually positive for vimentin, desmin, smooth muscle actin (SMA) |
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Leiomyoma:
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*Most common presentation: uterine “fibroids”
Adult female with pelvic fullness, discomfort Pregnancy and delivery complications *Gross: Very well-circumscribed masses Tan-to-white WHORLED cut surfaces *Histology: Bands of smooth muscle cells with no mitotic activity |
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-Leiomyoma in the uterus
-tan-white fibroids -whorled pattern |
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Low power of Leiomyoma
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High power of Leiomyoma
-bands of smooth muscle |
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high power of Leiomyoma
-rounded nuclei -kind of bland looking |
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Leiomyosarcoma:
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*Presentation:
-Adults, female>male -Skin: firm painless masses -Deep tissue of extremities: mass, impaired function -Retroperitoneum: abdominal symptoms *Histology: -spindle cells with cigar-shaped nuclei, +mitoses interweaving fascicles |
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Leiomyosarcoma
mitotic figure elongated nucleoli |
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Leiomyosarcoma
mitotic figure elongated nucleoli |
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treatment and prognosis in Leiomyosarcoma:
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Treatment: excision or radiation, or both
Prognosis: Cutaneous/superficial: small, easy to treat Retroperitoneal: large, difficult to excise, death by local extension and metastases |
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Skeletal muscle tumors- types:
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Benign: rare: rhabdomyoma
Malignant: rhabdomyosarcoma Immunohistochemistry: Usually positive for vimentin, desmin, myogenin, MyoD1 |
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Rhabdomyosarcoma:
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*Kids and adolescents
-Most common soft tissue neoplasm -Head/neck or genitourinary tract *Gross: depends on location -Hollow organs of GU: may be gelatinous, grape-like (sarcoma botryoides) -In solid tissue: infiltrating mass |
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*Rhabdomyosarcoma in a splayed open bladder.
*Sarcoma botryoides |
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histological appearance of Rhabdomyosarcoma:
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*Histology: diagnostic cell: rhabdomyoblast
-Rich in thick and thin filaments -May be elongated (tadpole cell or strap cell, cross striations may be visible) or round -If very poorly differentiated, may look like small round blue cell, so immunohistochemistry needed |
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Rhabdomyosarcoma
-rhabdomyoblasts full of thick/thin filaments |
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Rhabdomyosarcoma
-rhabdomyoblasts full of thick/thin filaments -"strap cells" are elongated rhabdomyoblasts |
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Rhabdomyosarcoma- how does histology relate to prognosis?
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Histology: pattern variants are prognostic
Embryonic, pleomorphic, alveolar (better mid worse prognosis) |
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Mutations in Rhabdomyosarcoma:
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*Most cases have t(2;13)
-Chromosome 2: PAX3 gene, upstream of skeletal muscle differentiation gene -Chromosome 13: FKHR gene -Chimeric protein likely disregulates skeletal muscle differentiation |
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synovial sarcoma:
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*"Other"
*Not comprised of synovial cells *Presentation: -Young adults; 20-40 y.o. -Deep soft tissue around joints of extremities (60-70% around knee) |
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appearance of synovial sarcoma:
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*Histology:
-Biphasic; spindle cells and epithelial-like cells -Monophasic: entirely spindle cells *May require immunohistochemistry *Send for genetic studies! *Immunohistochemistry: Both epithelial and spindle cells stain for cytokeratin |
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*synovial sarcoma
*note the spindle cell in middle |
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mutations in synovial sarcoma:
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*Most are t(X;18)
-leads to fusion product SSX/SYT -Combines SYT gene (transcription factor) with SSX1 or SSX2 gene (transcription inhibitors) *Specific translocation relates to prognosis -SSX1 involvement yields poorer prognosis |
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treatment and prognosis in synovial sarcoma:
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*Treatment is aggressive
Limb-sparing surgery Chemotherapy *Metastasizes to lung, bone, lymph nodes *Survival: 5 year: 25-62% 10 year: 10-30% |