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91 Cards in this Set
- Front
- Back
good ihc markers for nerve sheath tumors (general)
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S100, CD34, NFP, GFAP, EMA
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what histochemical stain can be used for alveolar soft parts sarcoma
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pas for crystals
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stains for epithelioid sarcoma
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Keratin, EMA, Vimentin, CD34
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ihc for synovial sarcoma
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Keratins, CK7, EMA
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ihc for clear cell sarcoma
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HMB45 >S100 protein
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ihc fo4 Angiomatoid (Malignant) Fibrous
Histocytoma |
50% desmin
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three fx for grading sarcomas
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Differentiation
Mitotic activity Necrosis |
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examples of some grade 2 sarcomas
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MFH, myxoid sarcomas, MPNST, and
angiosarcoma |
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of soft tissue sarcomas which are generally hematogenous
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i hope i got this right
epitheloid and melanocytic (most others go to LNs)? |
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which sarcomas are not staged
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Angiosarcoma, DFSP, IMFT, desmoid, and
sarcoma of parenchymal organs |
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primary stage determinant in sarcoma
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tumor size
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for treatment, when do more than just surgery
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i believe usually when you get to intermediate, then employ rad/chemo
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ihc for sarcomatoid scca
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AE1/AE3, Keratin (CK) 5/6, p63
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IHC for spindle cell adenoca
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AE1/AE3, CK18 alone
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if see dirty necrosis and cherry red nuclei, these are fx of what 2 general categories
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carcinoma, melanoma
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ihc for DLBL
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CD20, CD79a, CD10, Bcl6, Bcl2
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ihc for anaplastic large cell lymphoma
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CD30, ALK-1, EMA
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what
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analplastic large cell lymphoma: dishesive and hallmark cells
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what in lymphoma
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dot and membrane staining with CD30 in anaplastic large cell lymphoma
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ihc of MFH:
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vimentin, focal S MA or MSA only,
diagnosis of exclusion |
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ihc of Liposarcoma:
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S100 in lipoblasts, CD34 in
spindled cells |
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ihc of Rhabdomyosarcoma:
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Desmin, MSA, MyoD1, myf4 (skeletal muscle specific
myogenin) |
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ihc of Leiomyosarcoma
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: Desmin, SMA, MSA
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ihc of Epithelioid Malignant Peripheral Nerve
Sheath Tumor |
S100 protein, GFAP
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five patterns of MFH
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• Pleomorphic Sarcoma NOS (Storiform-
Pleomorphic MFH) • Myxofibrosarcoma (Myxoid MFH) • Giant Cell Rich • Angiomatoid (Malignant) Fibrous Histiocytoma |
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most common type of MFH
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storiform/pleomorphic (90% are deep, intramuscular)
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in whom does angiomatoid MFH occur
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kids
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what
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pleomorphic/storiform MFH
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if subcutaneous MFH usually what type
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myxoid MFH
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role of CD68 and CD163 in MFH
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CD68 stains lots of mesenchymal cells, relatively nonspecific, stains cells with lysosomes
CD163 specific histiocyte marker, MFH cells are negative |
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what, where located
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50% myxoid histology, extremities, superficial location
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what
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myxofibrosarcoma - Prominent ropy vasculature “dripping” with tumor cells
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another name for low grade fibromyxoid sarcoma
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EVANS TUMOR, ON SPECTRUM
WITH HYALINIZING SPINDLE CELL TUMOR WITH GIANT ROSETTES) |
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cytogenetic findings in fibromyxoid sarcoma
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t(7;16)(q33;p11) FUS/CREB3L2
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what
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low grade fibromyxoid sarcoma
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what
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Hyalinizing Spindle Cell
Tumor with Giant Rosettes (on spectrum with low grade fibromyxoid sarcoma) |
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what
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GC rich MFH
=storiform/pleomorphic MFH+ osteoclast-like GCs |
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what, ddx
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inflammatory MFH: retroperitoneal, CD30+, CD15-
sheets of polys and xanthoma cells clinically: systemic fx b sx, peripheral eos. ddx: dediff liposarcoma, Hodgkin dz |
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how do sarcomas usually met
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hematogenously
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what
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atypical fibroxanthoma
Solar Elastosis Small size <1-2 cm and Superficial dermal location: |
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cf pleomorphic liposarcoma from dedifferentiated liposarcoma
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pleomorphic is just that - crazy cells
dedifferentiated floret-like GCs |
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ST leiomyosarcoma - mits
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low tolerance for mits; cyto atypia with 1 mit/50 malignant involving cutaneous/dermis
pilar, angio or genital are exceptions |
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cytogenetics with pleomorphic rhabdomyosarcoma
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CHOP
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age group for pleomorphic rhabdomyosarcoma, ihc
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>40 years, if mismatch between smooth muscle tumors, think skeletal
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two pleomorphic sarcomas with worst prognoses
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Pleomorphic Leiomyosarcoma
AND Pleomorphic Rhabdomyosarcoma |
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ddx for fascicular malignant spindle cell tumors
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“Fibrosarcoma”
Malignant peripheral nerve sheath tumor (MPNST) Synovial sarcoma Kaposi sarcoma |
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if see a "fibrosarcoma" close to dermis, what is it
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DFSP
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"fibrosarcomas" have been stratified into what four tumors
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Sclerosing epithelioid fibrosarcoma
Myxofibrosarcoma Low grade fibromyxoid sarcoma (Evans Tumor) Fibrosarcoma arising in Dermatofibrosarcoma Protuberans |
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DERMATOFIBROSARCOMA PROTUBERANS - in whom, where found, marker
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males, Old Fashioned Bathing-Suit Distribution, pdgf-beta
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some growth patterns for dfsp
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honeycomb, storiform
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ihc for dfsp
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cd34 - goes away if portion develops into Fibrosarcoma (which is present portends a worse prognosis)
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cytogenetics of DFSP
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Ring chromosome: COL1A1-PDGFB fusion t(17;22)(q22;q13)
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S100 and MPNST
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if spindled (which most are), only focal S100. If epitheloid very S100.
those that arise in NF1 are spindled |
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what
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MPNST - note nerve on side
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what, ddx
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MPNST,
can see donut growth (geographic necrosis/not dirty though) in syn sarc and PNETs too |
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what
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MPNST+rhabdo=malignant triton tumor
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what
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syn sarcoma (biphasic)
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what, CD7+, ema+, CD34-, czome?
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monphasic synovial sarcoma t(x;18), SYT-SSX fusion
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four clinical settings for kaposi sarcoma
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Classic (Chronic) - middle age, men, meditteran
AIDS related Post-Transplant Lymphadenopathic (african) same histol. etc |
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what
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kaposi
fascicles of spindle cells, separated by slit-like vascular spaces containing erythrocytes, PAS positive diastase resistant hyaline globules, extravasated RBCs, scattered plasma cells, and hemosiderin deposits breakdown of rbcs, hyaline globules, like pink grapes, think mediterrean |
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name 4 benign fascicular tumors
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Fibrous Histiocytoma
Fibromatosis Neurofibroma Schwannoma |
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what
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benign fibrous histiocytoma - note pushing stellate border
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ihc for deep fibromatosis, syndromic assoc, gene alterations
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nuclear beta catenin, apc, gardners (and sporadic)
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what, ihc
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Neurofibroma, note how central portion is more cellular, S100, CD34
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name, dz
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wagner meisser corposcle
diffuse nf |
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skin findings of NF1
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Café-au-lait macules appear
shortly after birth lisch nodules, axillary freckle and neurofibromas occur in childhood or at puberty |
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findings in NF1 (not skin)
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Neurofibromas
Optic glioma (pilocytic astrocytoma) Bone changes: osteoporosis, kyphoscoliosis, and pseudoarthrosis (tibia) Vasculopathy: arterial dysplasia, spontaneous rupture (renal, cerebral vessels) |
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mutation in NF1
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loss of neurofibromin Nf1 (generally
suppresses Ras), chromosome 17, Tumor Suppressor Gene |
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what
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schwannoma
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what
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hyaline vascularization in antoni B (myxoid area) of Schwannoma
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what
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pericapsular lymphoid cuffing in cellular schwannoma
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ihc for schwannoma
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- S100 protein diffuse
– CD34 only in Antoni B – EMA at periphery in capsule (perineurium) – Cellular schwannoma has diffuse S100 protein, GFAP, and occasionally keratin reactivity |
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what
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Madelung Lipomatosis, posterior shoulder, can be assoc with etoh
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cytogenetic changes in spindle cell lipoma/pleomorphic lipoma
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13q changes and 16q
losses with partial monosomy |
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what
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chondroid lipoma
|
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kidney, ihc, syndrome
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histo: thick walled vessels, 2) immature specialised smooth muscle cells which may be pleomorphic , 3)mature fat
ihc: desmin, HMB45 syndrome: TS |
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what
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myxoid liposarcoma, plexiform vessels, t(12;16)
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what, cytogenetics
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round cell liposarcoma, t(12;16), CHOP by FISH
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Mesenchymal chondrosarcoma ihc
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SOX9
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Evans low grade fibromyxoid sarcoma FISH
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FUS
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ihc for ewings
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vimentin, keratins, CD99, glycogen
pnet same but add in neural markers or EM |
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name 4 ish tumors that have the t(12;22)(q13;q12)
EWS-ATF1 fusion finding |
Ewings/PNET
angiomatoid MFH clear cell sarcoma |
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name 4 ish tumors with the same t(12;15)(p13;q25) ETV6-NTRK3 fusion
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Infantile fibrosarcoma
Congenital mesoblastic nephroma Acute myeloid leukemia Secretory breast carcinoma |
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name two tumors with t(X;17)(p11.2;q25)ASPL-TFE3 fusion
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Alveolar soft part sarcoma
Papillary renal cell carcinoma |
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two mutations in syn sarcoma
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t(2;13)(q35;q14)
– PAX3-FKRH t(1;13)(p36;q14) – PAX7-FKRH |
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what
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Nodular fasciitis;
MYXOID DEGENERATION EXTRAVASATED ERYTHROCYTES AND LYMPHOCYTES |
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honeycomb pattern in subcutis
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DFSP
Neurofibroma |
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cf diffuse NF vs. DFSP
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cd34 in both
s100 negat in dfsp, S100 pos in nf look for weigner-m. corpusles in diffuse nf |
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see keloidal collagen think:
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Keloid
nodular fasciitis fibromatosis, |
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see central necrosis think:
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Epithelioid Sarcoma
Necrobiotic Granuloma (CD163, CD68 positive, negative for keratin, EMA) Diabetic Lipoidica, Rheumatoid Nodule, Infection, Granuloma Annulare |
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see extravascated rbcs, think:
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Nodular fasciitis
Myxoid desmoid Kaposi Sarcoma |