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105 Cards in this Set
- Front
- Back
SMA/MSA, focal CD34 in fibroblasts
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Fibroblastic differentiation
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S100 (fat, lipoblasts), CD34 (spindled areas)
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Lipomatous differentiation
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CD31, CD34, SMA (pericytes), HHV8 (KS)
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Vascular differentiation
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S100, CD34, NFP, GFAP, EMA
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Nerve sheath differentiation
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Desmin, MyoD1 (nuclear), myogenin, myoglobin
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Skeletal muscle differentiation
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SMA, MSA, desmin
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Smooth muscle differentiation
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PAS crystals, no consistent IHC
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Alveolar soft part sarcoma
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Keratin, CD34, EMA
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Epitheliloid sarcoma
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Keratin, CK7, CK19, CD99, Bcl-2, EMA
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Synovial sarcoma
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HMB45 > S100, melan A, MITF (microphtalmia transcription factor), CD57, c-kit
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Clear cell sarcoma
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Translocation(s) in Ewing sarcoma?
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t(11;22)(q24;q11.2-12)
11 = FLI-1 22 = EWS t(21;22) 21 = ERG 22 = EWS |
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t(11;22)? (2)
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Ewing sarcoma
t(11;22)(q24;q11.2-12) 11 = FLI-1 22 = EWS Desmoplastic small round cell tumor t(11;22)(p13;q12) 11 = WT1 22 = EWS |
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t(21;22)?
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Ewing sarcoma
21 = ERG 22 = EWS |
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t(12;22)? (2)
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Clear cell sarcoma
12 = ATF1 22 = EWS Myxoid/round cell liposarcoma 12 = CHOP = DDIT3 22 = EWS Note: ATF1 and CHOP both map to 12q13 |
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Translocation in desmoplastic small round cell tumor?
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t(11;22)(p13;q12)
11 = WT1 22 = EWS |
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Translocation in clear cell sarcoma?
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t(12;22)
12 = ATF1 22 = EWS |
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t(12;16)?
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Myxoid/round cell liposarcoma
12 = CHOP = DDIT3 16 = FUS = TLS |
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Translocation(s) in myxoid/round cell liposarcoma?
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t(12;16)
12 = CHOP = DDIT3 16 = FUS = TLS t(12;22) 12 = CHOP = DDIT3 22 = EWS |
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Translocation in alveolar soft part sarcoma?
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t(X;17)
X = TFE3 17 = ASPL |
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t(X;17)?
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Alveolar soft part sarcoma
X = TFE3 17 = ASPL |
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Translocation in synovial sarcoma?
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t(X;18)
X = SSX1, SSX2, or SSX4 18 = SYT |
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t(X;18)?
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Synovial sarcoma
X = SSX1, SSX2, or SSX4 18 = SYT |
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Translocation(s) in rhabdomyosarcoma, alveolar type?
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t(1;13)
1 = PAX7 13 = FKHR t(2;13) 2 = PAX3 13 = FKHR |
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t(1;13)?
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Rhabdomyosarcoma, Alveolar type
1 = PAX7 13 = FKHR |
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t(2;13)?
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Rhabdomyosarcoma, Alveolar type
2 = PAX3 13 = FKHR |
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Translocation in dermatofibrosarcoma protuberans?
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t(17;22)
17 = COL1A1 (collagen type 1) 22 = PDGF-beta (platelet derived growth factor) |
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t(17;22)
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Dermatofibrosarcoma proturberans
17 = COL1A1 (collagen type 1) 22 = PDGF-beta (platelet derived growth factor) |
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Translocation in low grade fibromyxoid sarcoma?
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t(7;16)
7 = CREB 16 = FUS |
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t(7;16)?
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low grade fibromyxoid sarcoma
7 = CREB 16 = FUS |
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This sarcoma typically presents as single or multiple nodular, sometimes ulcerated, lesions involving the fingers, hands, and forearms of young adults (especially males). Histologically, it is recognized by its characteristic nodular pattern of growth, central cystic degeneration, and epithelioid cells with eosinophilic cytoplasm.
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Epithelioid sarcoma
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Typically affecting the shoulder or axillary region of male infants, this lesion displays 3 components: adipose tissue, some of which has tiny cytoplasmic droplets (brown fat); haphazardly placed paucicellular fascicles of spindle cells with abundant collagen; primitive-appearing rounded to spindled cells set in pale amphophilic stroma. There is no necrosis or mitoses.
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Fibrous hamartoma of infancy
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This benign tumor of infancy occurs exclusively on the fingers and toes. It consists of a dermal infiltrate of uniform, plump, spindled cells in a collagenous stroma. The spindled cells have a fascicular growth pattern. Mitoses can be prominent. There are numerous small round eosinophilic cytoplasmic inclusions within the spindle cells which stain deep red on Masson trichrome.
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Infantile digital fibrosis
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Which sarcoma tends to metastasize to other soft tissue sites rather than to lungs?
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Myxoid liposarcoma
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What is the classical location of spindle cell lipoma?
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Neck or shoulder
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t(9;22)(q22-31; q11-12)?
Note: this is not BCR-ABL |
Extraskeletal chondrosarcoma
9 = CHN 22 = EWS Note: skeletal chondrosarcoma doesn't have this translocation |
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Translocation in extraskeletal chondrosarcoma?
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t(9;22)
9 = CHN 22 = EWS |
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Occurring most commonly in the upper back, this benign tumor is composed of three types of cells: mulberry cell (multivacuolated adipocyte with a centrally placed round nucleus); cells with granular (rather than vacuolated) cytoplasm, and mature adipocytes.
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hibernoma
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This painful skin nodule tends to present in the legs and feet of females. Histologically, the tumor is composed of smooth muscle bundles that appear to originate from medium sized vessels.
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Vascular leiomyoma
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What does ANGEL stand for?
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Angiolipoma
Neuroma (traumatic) Glomus tumor Eccrine spiradenoma Leiomyoma (vascular) ANGEL is the acronym for painful skin nodules |
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In the setting of HIV, what virus is implicated in leiomyosarcoma?
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EBV
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What are the most common demographic and sites for clear cell sarcoma of soft parts?
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Demographic: 2nd-4th decades
Sites: Foot and ankle |
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What's the most common demographic and location for desmoplastic small round cell tumor?
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Demographic: Young men (average age =22)
Site: Abdominal |
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What is the proliferative cell in nodular fasciitis?
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Myofibroblasts
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What is the typical demographic and location for nodular fasciitis?
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Demographic: 2nd to 5th decades
Site: Forearm Other sites include upper extremity (shoulder), head and neck, and thigh. |
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What is the most common site of nodular fasciitis in children and infants?
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Head and neck
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Is nodular fasciitis usually deep or superficial?
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Superficial (subcutaneous)
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After local excision, is nodular fasciitis prone to recur?
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No, local excision is usually curative though recurrences occur extremely rarely.
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Is nodular fasciitis encapsulated?
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No
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This lesion has a "tissue culture" appearance, numerous mitoses (but no atypical forms), extravasated RBC in the stroma, mononuclear inflammatory cells and sparce multinucleated giant cells. The proliferative cells are positive for SMA with rare desmin positive cells. CD68 highlight histiocytes.
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Nodular fasciitis
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This lesion which tends to occur between the 3rd and 5th decades involving the extremities, is slowly growing, presents as slightly raised and pigmented cutaneous lesion centered in the dermis with associated epidermal hyperplasia. Histologically, the lesion is arranged in a storiform pattern or in short fascicles and is composed of spindled to oval fibroblasts and round to oval histiocytoid cells. The lesion also contains multinucleated, osteoclast-like giant cells; xanthoma cells may be seen and may be prominent. The stroma can be myxoid or densely fibrotic with a sparse chronic inflammatory infiltrate as well as focal hemosiderin pigment.
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Fibrous histiocytoma
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Is extraskeletal myxoid chondrosarcoma usually deep or superficial?
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Deep
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Is extraskeletal myxoid chondrosarcoma usually well-circumscribed or infiltrative? Is there a capsule?
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Extraskeletal myxoid chondrosarcoma is usually well-circumscribed surrounded by a fibrous pseudocapsule.
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Histologically, this tumor which is most commonly located in skeletal muscle, is multinodular with abundant myxoid matrix; the tumor cells are arranged in cords and clusters, frequently oriented radially. The tumor cells are round to stellate with eosinophilic cytoplasm, round and hyperchromatic nuclei, and sparse mitotic activity.
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Extraskeletal myxoid chondrosarcoma
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What demographic is most commonly affected with dermatofibrosarcoma protuberans?
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Middle-aged males, African American predominance
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What are the clinical characteristics of dermatofibrosarcoma protuberans?
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Most cases are slow and progressive with a plaque-like phase followed by a nodular phase where the overlying epidermis becomes thinned and ulcerated.
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The cut surface of this tumor is gray-white and the tumor involves the dermis and subcutis, sometimes extending to the underlying muscle. There is entrapped dermal collagen and appendages. The overlying epithelium is thinned or ulcerated. The tumor is cellular and composed of uniform, mildly atypical fibroblasts arranged in a monotonous, tight and repetitive storiform pattern.
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Dermatofibrosarcoma protuberans
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What is a dermatofibrosarcoma protuberans with malanocytic differentiation (i.e. S100+, melan A+, HMB-45+) called?
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Bednar tumor
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What is the juvenile variant of dermatofibrosarcoma protuberans called?
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Giant cell fibroblastoma
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What is the IHC for dermatofibrosarcoma protuberans?
CD34 S-100 SMA Desmin Keratins EMA CD99 Factor 13A c-kit |
Dermatofibrosarcoma protuberans
CD34: + S-100: - SMA: - Desmin: - Keratins: - EMA: - CD99: + Factor 13A: - c-kit: - |
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What is the age of peak incidence for well-differentiated liposarcoma?
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6th to 7th decades
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What location is most commonly affected by well-differentiated liposarcoma?
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Most common: deep muscles of extremities.
Less common: retroperitoeneum and contiguous areas (inguinal canal and paratesticular region). |
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What are the proliferative cells in malignant fibrous histiocytoma?
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fibroblastic and myofibroblastic cells
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IHC of malignant fibrous histiocytoma?
Vimentin SMA CK S100 CD45 CD68 |
Malignant Fibrous Histiocytoma:
Vimentin: + SMA: + CK: - S100: - CD45: - CD68: + |
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What is the peak age-range for malignant fibrous histiocytoma?
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6th to 7th decades.
Note: MFH is rare before age 40. |
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What is the most commonly affected site of malignant fibrous histiocytoma?
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Thighs
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What is the most common sarcoma arising after radiation therapy?
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Malignant fibrous histiocytoma
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What are the 4 types of malignant fibrous histiocytoma? What are the 2 most common?
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Most to least common:
1. pelomorphic-storiform type 2. myxoid variant (>50% myxoid) Less common: - Inflammaotry: when inflammatory component becomes prominent, including abundant neutrophils - Giant cell variant: when the giant cells become a predominant element |
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Name the 4 most common sarcomas.
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1. MFH
2. Liposarcoma 3. Rhabdomyoarcoma 4. Synovial sarcoma |
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What's the typical demographic affected by synovial sarcoma?
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Young adults (median age 30-35), but can occur in children and older people.
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What is the most common site of involvement with synovial sarcoma?
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Synovial Sarcoma
Most common: Extremities (60% lower, 20% upper), typically arises in the proximity of large joints Less common: - Trunk - head and neck |
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Is synovial sarcoma typically fast or slow growing?
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Synovial sarcoma presents as a slow growing swelling, sometimes present for years. Poorly differentiated cases may present directly with metastases though.
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What are the 3 microscopic types of synovial sarcoma list from most to least common.
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1. Monophasic (~66%)
2. Biphasic (20-30%) 3. Poorly differentiated (least common) |
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What are the 3 types of rhabdomyosarcoma?
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- Embryonal: includes botryoid, spindle cell, and anaplastic variants
- Alveolar: only type with a translocation - Pleomorphic |
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Cambium layer, "strap cells", and rhabdomyoblasts characterize what lesion?
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Embryonal rhabdomyosarcoma, botryoid variant
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What are the first and second most common malignant soft tissue tumor of children?
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1. Rhabdomyosarcoma
2. Ewings/PNET |
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What is the most common variant of rahbdomyosarcoma?
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Embryonal rhabdomyosarcoma
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What are the most common sites for embyronal rhabdomyosarcoma? (3)
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Genitogurinary, retroperitoneal, head and neck
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What is the most common site for embryonal rhabdomyosarcoma, spindle cell variant?
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paratesticular
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What demographic is affected by alveolar rhabdomyosarcoma and what are the 2 most common site of involvement?
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Demographic: Older children (than embryonal rhabdomyosarcoma)
Sites: extremities, head/neck |
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What demographic is most commonly affected with pleomorphic rhabdomyosarcoma? What are the most common sites?
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Pleomorphic Rhabdomyosarcoma:
Demographic: Elderly Sites: trunk, extremities |
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Which is the most common type of rhabdomyosarcoma?
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Embryonal RMS (75% of cases)
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Alveolar rhabdomyosarcoma tends to have what type of giant cells?
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wreath-like giant cells
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IHC for rhabdomyosarcoma:
Desmin Actin Myoglobin Myogenin Myo D1 (nuclear) CK NFP NSE Leu7 S100 CD99 CD10 CD19 CD20 |
Rhabdomyosarcoma:
Desmin: + Actin: + Myoglobin: + Myogenin: + Myo D1 (nuclear): + CK: +/- NFP: +/- NSE: +/- Leu7: +/- S100: +/- CD99: +/- CD10: +/- CD19: +/- CD20: +/- |
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This rare variant of schwannoma contains melanin pigment and can be associated with Carney complex.
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Psammomatous melanotic schwannoma
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What is the EM finding in clear cell sarcoma?
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pre-mlanosomes
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The histology of this tumor consists of uniform cells arranged in small clusters, nests, and fascicles surrounded by delicate fibrous septa. The tumor cells have clear to eosinophilic cytoplasm, and uniform round to oval nuclei with vesicular chromatin and large nucleoli.
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Clear cell sarcoma
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What is the demographic affected by granular cell tumor?
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Middle-aged (4th-6th decade), predilection for African-Americans
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What % of granular cell tumors are malignant?
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2%
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What is the most common soft tissue site affected by granular cell tumor?
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Hand
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How do the granules in granular cell tumor stain with PAS-D?
What are the granules made of? |
PAS-D: +
Granules: phagolysosomes |
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Typical age group affected by Ewing/PNET?
Which ethnic group is usually spared of Ewing/PNET? |
Affected group:
Adolescents, young adults Spared group: African Americans |
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Typical soft tissue sites for Ewing/PNET are? (4)
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1. Paravertebral area
2. Retroperitonum 3. Chest wall 4. Extremities |
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What is the demographic which alveolar soft part sarcoma affects?
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Children and young adults
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Is alveolar soft part sarcoma fast or slow growing?
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Slow
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Dermatofibrosarcoma protuberans is to t(17;22)
as Giant cell fibroblastoma is to _______? |
ring chromosomes 17 and 22
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The most common type of angiosarcoma is???
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Cutaneous angiosarcoma not associated with lymphedema
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The typical demographic affected by angiosarcoma is???
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Elderly with sun-damage
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3 positive IHC for angiosarcoma?
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CD31, CD34, Factor 8
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IHC for epithelioid hemangioendothelioma:
CD31 CD34 Factor 8 CK7 CK8 CK18 CK19 |
Epithelioid Hemangioendothelioma:
CD31: + (diffuse) CD34: +/- Factor 8: +/- CK7: +/- (focal) CK8: +/- (focal) CK18: +/- (focal) CK19: - |
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Are soft tissue epithelioid hemangioendotheliomas usually painful or not?
Solitary or multiple? Superficial or deep? |
Painful
Solitary Both superficial and deep |
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IHC for desmoplastic small round cell tumor?
Desmin CK EMA WT1 CD99 NSE |
Desmoplastic small round cell tumor:
Desmin + CK + EMA + WT1 + CD99 + NSE + |
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Inflammatory myofibroblastic tumor is associated with what mutation?
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ALK-1 on chromosome 2.
Note: 60% of IMT are ALK+ by IHC. |
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This lesion can affect people of all ages but has a predilection for young children (average age 10) and may present with systemic symptoms. Abnormal lab values include anemia, elevated ESR, thrombocytosis, and hypergammaglobulinemia. Microscopically, the lesion is characterized by 3 possible patterns: myxoid spindle cell lesion w/ tissue-culture-like appearance; cellular with fascicular-storiform archiecture; or spindle cells in a dense fibrous stromal background. The spindle cells may express ALK-1 by IHC.
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Inflammatory myofibroblastic tumor
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Solitary fibrous tumor IHC? (3)
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CD34, CD99, Bcl2
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What is Doege-Potter syndrome?
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Hypoglycemia associated with solitary fibrous tumor.
Insulin-like growth factor is implicated. This only occurs in 4% of SFT. This is usually associated with larger tumors with higher mitotic rates. |