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142 Cards in this Set

  • Front
  • Back
  • 3rd side (hint)

1 List vascular tumors? (6)

1 Angiosarcoma
Glomus tumor
Kaposi sarcoma

1 List extraskeletal osseous and cartilaginous tumors? (3)

1 Extraskeletal chondrosarcoma, extraskeletal osteosarcoma, myositis ossificans

Extraskeletal chondrosarcoma
1 3 variants? (same histo as bone versions)
2 What molecular alteration is associated with which variant?
3 Patient populations and prognosis?

1 well diff, myxoid, mesenchymal
2 t(9;22) with myxoid variant
3 Well diff M>W 50yrs; better than bone counterpart; mesenchymal W>M 25yrs, terrible prognosis

Extraskeletal osteosarcoma: (histologically identical to bone version)
1 patient population?
2 associations?
3 Prognosis?

1 50s +, M=W
2 Radiation for other malignancy, Thorotrast (radioactive thoriam dioxide)
3 Terrible, often present with mets

Myositis ossificans
1 patient population, location, association ?
2 histo?

1 young 2nd 3rd decades active males; upper arms and thighs, trauma
2 classic zonation, central "nodular fasciitis" like fibroblasts surrounded by osteoblasts producing osteoid and outermost layer of mature lamellar bone

1 List fibrohistiocytic tumors? (10)

1 Atypical fibroxanthoma
Fibrous histiocytoma
Giant cell fibroblastoma
Juvenile xanthogranuloma
Malignant giant cell tumor of
tendon sheath
Pleomorphic malignant fibrous
Tenosynovial giant cell tumor,
diffuse type
Tenosynovial giant cell tumor,
localized type

Atypical fibroxanthoma
1 List another name? considered?
2 What patient population? associated with?
3 Histo?

1 Superficial malignant fibrous histiocytoma; superficial form of pleomorphic MFH
2 elderly; sundamaged skin in association with actinic keratoses
3 dermal proliferation of spindled cells with bizarre nuclei arranged in vague fascicles; numerous mits, no necrosis

Dermatofibrosarcoma protuberans
1 histo?
2 Called what if it contains pigmented macrophages?
3 Positive IHC?
4 75% have what molecular alteration?
5 What is a grenz zone?

1 Bland monomorphic spindled cells in distinct storiform pattern involving dermis and subcutis (checkerboard pattern of fat infiltration)
2 Bednar tumor
3 CD34
4 t (17;22) may form ring chromosomes
5 Strip of uninvolved dermis right below epidermis

Fibrous histiocytoma
1 AKA?
2 List three variants? which has translocation?
3 Histo?
4 Positive IHC? Negative IHC?

1 Dermatofibroma
2 Cellular t4;17), epithelioid and aneurysmal
3 spindled cell proliferation in dermis with overlying hyperplastic epidermis (table-topping) with collagen trapping at periphery often with histios or giant cells
4 75% alpha 1 anti-trypsin; - CD34

Giant cell fibroblastoma:
1 patient population and location?
2 The juvenile analoge of ? with same molecular atleration?
3 histo?
4 Recurrence rate?

1 <5yrs male; dermal/subcutis of thigh, inguinal region or chest wall
2 DFSP; ring t(17;22)
3 fibroblastic cells in ill defined fasicles with gaping pseudovascular spaes lined by multinucleated giant cells with mast cells and lymphocytes
4 50%

Jeuvenile xanthogranuloma
1 patient population? Location?
2 histo?
3 IHC?
4 Associations? (syndromes)

1 infants (<6m), head and neck (50%)
2 Sheets of histiocytes with mixed inflammatory background (eos PCs lymphs) often fibrosis and giant cells that cause flattening of the epdermis and infiltrate adnexal structures often with giant cells
3 Negative for S100 and CD1a
4 NF and urticaria pigmentosa

1 Considered soft tissue counterpart of PVNS? aka?
2 Location? Patient pop?
3 microscopic pathology?
* inconsistent terminology see card below

1 GCT of tendon sheath, diffuse type aka malignant GCT of tendon sheath because may represent extra articular extension.
2 distal extremities (80% knee), >40yrs female > male
3 three cell types: mononuclear synovial cells (may be very atypical and grow in sheets giant cells and pigmented macrophages with associated hemorrhage, synovial cells lining pseudoglandular or alveolar spaces


Pleomorphic malignant fibrous histocytoma
1 now called?
2 name 3 subtypes?
3 Patient population and location (with exception)?
4 histo?
5 Prognosis by subtype?

Tenosynovial GCT, diffuse type
1 list 4-5? alternate names?
2 soft tissue counterpart of?
3 Patient population and location?
4 Micro?
5 Prognosis?

1 Proliferative synovitis, florid synovitis, extra-articular PVNS, PVN bursitis, malignant GCT of tendon sheath (as described earlier)
3 <40yrs F>M 80% around knee
4 Sheets of mononuclear cells admixed with xanthoma and spindled cellswith slit like pseudoglandular spaces with chronic inflammation and giant cells
5 50% recurr, locally agressive

Tenosynovial giant cell tumor, localized type
1 alternate names?
2 Patient population, location and size?
3 Micro?
4 prognosis?

1 Nodular tenosynovitis, giant cell tumor of tendon sheath
2 30-50yrs W>M, 85% joints of hands and <4cm
3 circumscribed sheets of mononuclear cells with fibrous septa and cleft like spaces with giant cell sand hemosiderin (same as diffuse)
4 benign, local recurrence 20-30%

1 General location and clinical setting?
2 List 6 variants?
3 histo?
4 All 5 associated with which thype of hyperlipidemia?

1 skin and subcutis, hyperliproproteinemia (PBC or DM)
2 Eruptive, tuberous, tendinous, xanthelasma, plane, verruciform
3 All have foamy histiocytes except eruptive. Tuberous and tendinous have choleserol and giant cells

1 Tendinous xanthoma associated with what AR disease?
2 Plan xanthoma association?

1 cerebrotendinous xanthomatosis
2 PBC and type II hyperlipidemia

List fibroblastic/myofibroblastic tumors ? (13)

1 Calcifying aponeurotic fibroma
Fibroma of tendon sheath
Infantile digital fibromatosis
Inflammatory myofibroblastic
Low grade fibromyxoid sarcoma
Low grade myofobroblastic
Myxoinflammatory fibroblastic
Nodular fasciitis

Calficying aponeurotic fibroma
1. Patient population, location, size?
2 micro?
3 prognosis?

1 kids and teens; hand/feet, <3cm
2 nodules consisting of central calc/cartilage surrounded by palisading fibroblasts between bands of hypocellular collagen that infiltrate fat at edges
3 50% local recurrence

1 Patient pop and location?
2 micro?
3 prognosis?

1 >55yrs W>>M (associated with repetitive manual labor) deep seated on back ribs 6, 7, 8 or scapula R>L
2 hypocellular hyalinzed collagen (types I-III) admixed with thickerecractile elastic fibers that may round up and form globules called chenille bodies; entraps adipose tissue
3 benign reactive process that almost never recurs

/Fibroma of tendon sheath
1 Patient population and location
2 Micro?
3 IHC and prognosis?

1 20-50 M>W, hand
2 multilobular architecture of hypocellular areas of fibroblasts and stelate cells in hyalinized collagenous stroma and more cellular areas may be fasicular or storiform
3 +factor XIIIa and CD34, 25% recurrence rate

1 Patient population? 2 kinds/locations with variants and alternate names? (3Ps)
2 micro? pertinent negative stains?

1 adults;
Penile (Peyronie dz)
Palmar (dupuytren
Plantar (Ledderhose dz)
deep: aka desmoid tumor
intrabdominal (pelvic &
2 fibroblastic/myofibroblastic bland fasicles with scattered thin walled curvilinear vessels with lymphocytic cuff at advancing infiltrative edge in myxoid or collagenous background (deep has more large sweeping bundles); negative (or focally positive) for myogenic markers

Facts bout fibromatosis?
1 Penile curvature?
2 Plantar/palmar?
3 Abdominal?
4 Mesenteric?
5 IHC? and molecular alteration?
6 prognosis?

1 toward the side of the lesion
2 usually multiple and bilateral; palmar associate with EtOH abuse
3 Pregnant women (or within 1yr
of pregnancy)
4 Associated with Gardner syndrome (FAP) often after Sx
5 beta-catenin nuclear positivity; trisomy 8 25%
6 recurrence extra-abdominal (35-65%) > abdominal desmoids

1 Name 2 types?
2 Patient population for each and location?
3 micro for both?

1 adult and juvenile/infantile
2 50s; 90% w/in 1st year; both in extremities and won't be superficial unless arising in DFSP
3 relatively uniform population of spindled cells in herringone pattern of intersecting fascicles

Infantile fibrosarcoma
1 features unique to this type
2 molecular alteration? also seen in?
3 prognosis?

1 hemagiopericytoma like pattern with dilated vessels and fibrin thrombi and myxoid changes
2 t(12;15), n mesoblastic nephroma
3 excellent (moratlithy <25%) much better than adult type (50% 5 year survival)

Infantile digital fibromatosis
1 aka?
2 Location and patient age?
3 micro?
4 Prognosis?

1 Inclusion body fibromatosis
2 fingers or toes; <1yr with 1/3 present at birth
3 well circumscribed superficial fibro/myofibroblastic proliferation surrounded by dense collagen stroma; cells have eosinophilic inclusions near nuclei made of intermediate filaments
4 50% local recurrence

Inflammatory myofibroblastic tumor
1 aka? (3)
2 location and patient population?
3 micro?
4 molecular alteration/stain?
5 Features of more aggressive tumors?

1 (extrapulmonary) inflammatory pseudotumor, inflammatory fibrosarcoma and plasma cell granuloma
2 usually abdomen (mesentery or omentum) but can occur anywhere
3 mixture of 3 patterns all composed of fibroblasts and myofibroblasts
"nodular faciitis like" with
loose myxoid stroma and
mixed inflammation
(neuts, eos, lymphs
< PCs)
cellular islands with compact
stroma and many
clusters of PCs
almost acellular with
hyalinized stroma
4 clonal aterations in ALK on 2p23 and 40% positive with ALK
5 ganglion like cells, reed sternberg like cells p53 exprfession and -ALK

Low grade fibromyxoid sarcoma
1 patient population and location?
2 name non-classic pattern with other name?
3 histo?
4 molecular?
5 Three positive immunostains?
6 prognosis?

1 young adultss (35) on trunk and proximal extremities
2 LGFS with giant collagen rosettes aka hyalinizing spindle cell tumor with giant rosettes
3 admixture of densly collagenized hypocellular areas with myxoid nodules of bland spindle cells in short whorling fasicles
4 t(7;16) FUS/CREB3L2
5 MUC4, CD99, BCL2
6 extremely good (10% recurrence 6% death)

Low grade myofibroblastic sarcoma
1 patient population and location?
2 histo?
3 prognosis?

1 adults M>F, thigh or H&N especially tongue
2 fascicles of spindled cells in storiform pattern, with pale pink cytoplasm and wavy elongated nuclei, diffusely infiltrative
3 local recurrence common mets rare


1 Patient population and location?
2 micro?
3 Negative prognostic factor?

What the f

Myxoinflammatory fibroblastic sarcoma
1 aka? (2)
2 patient population and location?
3 histo? (3 cell types)
4 prognosis?
5 pertinent negative stains?

1 inflammatory myxohyaline tumor of distal extremities wiht virocytes or reed sternburg like cells and acral MFS
2 40-50, subcutis of hands>feet
3 spindled/epithelioid cells with occasional bizarre ganglionlike cells with viral includion like nucleoli, liboblast like vacuolated cells, giant cells with emperipolesis with backgorund of alternating inflammation and fiborosis or hyalinization
4 very good, recurrence <20%
5 T and B cell markers and CD30

1 patient population and location?
2 Histo?
3 Prognosis?

1 elderly (70-80), superficial > deep extremities lower> upper
2 multinodular growth pattern of plump pleomorphic spindled cells in myxoid background that condensate around curvilinear vessels; scattered pseudolipoblasts and may have higher grade transformation/areas
3 65% recur, 25% metastasize

Nodular fasciitis
1 Patient population, location and history?
2 micro?
3 List 4 variants? which occur in kids?
4 prognosis?

1 young adults (20-50) upper extremities or trunk; rapidly growing but <3cm
2 spindled cells in s or c shaped fascicles with feathered edges and "microcyst" formation of mucin pools, prominent branching vasculature and often lots of normal mits, inflammation and extravasated rbc's; long standing with keloid like collagen
3 intravascular, cranial, ossifying, proliferative; intravascular and cranial (infant <1)
4 Benign, <2% recur

1 List lipomatous tumors? (4)

1 Hibernoma

1 patient population and location
2 histo?
3 list 4 variants/subtypes?
4 molecular alteration? also seen in?
5 EM findings?

1 young adults (25yrs) on shoulder mediastium, thigh, axilla and inguinal region
2 "organoid" arrangment of round to oval cells with central nuclei and grnaular to multivaculated cytoplasm mixed with normal fat usually often lipofuscin in cytoplasm
3 classic, myxoid, lipoma-like, spindle cell
4 translocation/rearrangments 11q13; lipomas and liposarcomas
5 filled with mitochondria

1 if diffuse and infiltrating called?
2 Patient population and location of each kind?
3 micro?
4 molecular alteration?
5 may be indistinguishable from what adult tumor? (different age and this has excellent prognosis)

1 lipoblastomatosis (because usually localized in subcutis)
2 kids <3 (may be congenital) boys 2x> girls; lipoblastoma in extremities and lipoblastomatosis deep skeletal muscle, retroperitoneum or mesentery
3 fat in multiple stages of development (immature stellate spindled cells, multivacuolated, lipoblasts) lobulated by fibrous bands with myxoid areas and a plexiform vascular network; may have hibernoma like areas
4 rearrangment of 8q11; excellent
5 myxoid liposarcoma

1 patient population and general location?
2 3 types (not variants) with locations for less common ones?
3 micro of classic lipoma?
4 list 7 variants?
5 molecular alteration?

1 50-60, M>W any location but usually upper half of body on trunk or neck
2 subcutanous>> intramuscular (trunk), intermuscular (abdominal wall)
3 mature adipose tissue (NO atypia) with thin fibrous septa and inconspicuous vasculature
4 Fibrolipoma
Chondroid lipoma
Spindle cell lipoma
Pleomorphic lipoma
5 50-80% have 12q13-15 rearrangements involving HMGA2/HMGIC


1.describe histolog including differrentiaton from spindle cell lipoma?

1. prominent bundles of mature bland fibrous tissue traversing mature adipose lobules, does not have the vascular pattern and thick collen bundles of spindle cell lipoma.

Chondroid lipoma
1 80% in?
2 micro?

1 women
2 nests and cords mature adipocytes, lipoblasts (resembles brown fat) and myxochondroid stroma

spindle cell lipoma
1 CLASSIC location/patient population?
2 histo?
3 Positive for what stain along with what other lipoma variant?
4 these two also share what unique (among lipomas) molecular alteration?

1 shoulder and posterior neck of older men (>40)
2 mature fat mixed with uniform spindle cells in mucinous background with rope like collagen bundles
3 CD34; pleomorphoic lipoma
4 16q13 alterations (70%)

Pleomorphic lipoma
1 on a continuum with ? so shares location, patient pop, molecular and staining pattern!!!!!
2 micro?

1 spindle cell lipoma
2 adipose tissue with fibrous septae with multiple hyperchromatic multinucleated "floret cells" bizarre pleomorphism

1 Multiple lipomas are associated with what 4 syndromes? describe each, multiple lipomas + ?

hemangiomas and
Cowden: hemangiomas, goiter,
lichenoid, papular and
papillomatous lesions of skin
and mucosa
Frohlich: obesity & sexual
Proteus: fibroplasia of hands
and feet, skeletal hypertophy
bony exostoses, scoliosis, and
linear verrucus epidermal

1 Patient population and location
2 List 4/5 variants?

1 40-60 yrs M>W, lower extremities and retroperitoneum
2 Well differentiated/atypical
lipomatous tumor, dedifferentiated,
round cell/myxoid,

Well differentiated/atypical lipomatous tumor
1 micro?
2 list 4 morphologic subtypes?
3 when to use each name?
4 molecular alterations?

1 variably sized mature adipocytes separated by fibrous bands with atypical, multinucleated hyperchromatic stromal cells
2 adipocytic (lipoma-like)
Spindle cell
3 well diff liposarc when central or retroperitoneal, ALT when on extremities
4 ringed chromosome 12 (80%) which MDM2 is on so --> amplification of it

1 patient population and location?
2 micro?

1 younger adults than other liposarcs; myxoid on extremities almost never retroperitoneal
2 proliferating lipoblasts in mycoid matrix with cystic cpaces and classic prominent "chicken wire" vasculature

Round cell
1 location/prognosis
2 micro?
3 molecular alteration shared with myxoid liposarc?

1 extremities with frequent mets to retroperitoneum and lungs, very agressive
2 sheets of small round tumor cells with high n/c ratio and much less prominent musculature
3 t(12;16) FUS/DDIT3

Pleomorphic liposarcoma
1 Associated with?
2 Micro?
3 Histo that makes this variant stand out from the rest?
4 Prognosis?

1 Radiation therapy or NF
2 Large bizarre spindled cells and adpocytic cells with vacuoles and scalloped nuclei, intra and extra cellular hyaline globules often round cell component and necrosis +/- neutrophilic infiltrate
3 Abundant atypical mitotic activity
4 Aggressive 40% recur 40% metastasize

Dedifferentiated liposarcoma
1 Most common locations?
2 Micro?
3 Dedifferentiation is a function of?
4 molecular alterations? (also stains)

1 retroperitoneum and groin
2 Well diff with abrupt transition to high grade pleomorphic sarcoma
3 time! longer bad
4 MDM2 (12q13-15 ring) and CDK4 amplification

1 List lymphatic tumors? (3)

1 Lymphangioma(tosis)

1 patient population?
2 List two types?
3 When the second becomes very dilated its called?
4 micro?
5 Main histologic differences between types?
6 aka ? in skin

1 50% congenital, 90% <2yrs
2 Cutaneous and Cavernous
3 Cystic hygroma
4 anastomosing thin walled variably thick lymphatic channels lined by bland endothelium filled with proteinaceous fluid lymphs blood and fat
5 Cutaneous is superficial and has smaller channels while cavernous/cystic hygroma has much more dilatation and larger walled channels
6 lymphangioma circumscriptum

1 Cystic hygromas associated with? (5)
2 Lymphangiomas + hemangiomas = what syndrome?

1 Turner syndrome
Hydrops fetalis
Noonan syndrome
Familial pterygium colli
Fetal alcohol syndrome
2 Maffucci syndrome

1 involves?
2 especially?
3 Patient population?
4 50-75% of patients also have?
5 prognosis?

1 visceral organs diffuse or multifocally
2 lung
3 infants and kids
4 multiple asymptomatic osteolytic bone lesions
5 only skeletal disease has excellent prognosis; involvement of liver spleen lung thoracic duct has poor prognosis because not surgically resectable

1 difference between 2 types of disease?
2 very specific patient population and location?
3 often present with?
4 micro?
5 treatment?

1 one is local one is diffuse
2 exclusively reproductive age women (often previous birth control use)l mediastinum, retroperitnonum and lungs
3 Chylous pleural effusion
4 disorganized proliferation of smooth muscle (short fascicles) involving lymphatics (ramifying network) and lymph nodes
5 progesterone and/or oopherectomy

1 lymphangioma(tosis) is associated with?
2 These lesions belong to PEComa family which also includes? (3)
3 IHC? why?

1 Tuberous sclerosis
2 angiomyolipoma, clear cell "sugar" tumor of lung, clear cell myomelanocytic tumor of falciform ligament/ligamentum teres (and unusual clear cell tumors anywhere else)
3 HMB-45 positive, related to PEComas

1 Arises in what clinical settings?
2 This is called ? syndrome?
3 Histo?
4 essentially indistinguishable from?

1 long standing chronic lymphedema, s/p axillary node dissection/radiation (with mastectomy)
2 Stewart Treves syndrome
3 anastomosing capillary sized vascular channels lined by atypical endothelial cells
4 Angiosarcoma

1 List myogenic tumors ? (4)

1 Leiomyoma

1 List 6 variants/locations with alternate names if any?

1 Cutaneous (leiomyoma cutis)
Genital leiomyomas (nipple, areola,
scrotum, labia, penis)
Angioleiomyomas (vascular
Of deep soft tissue
Intravenous leiomyomatosis
Leiomyomatosis peritonealis

Cutaneous leiomyoma
1 origin?
2 painful especially when?
3 Separated from overlying atrophic epidermis by?
4 Multiple cutaneous leiomyomas associated with ? (5)

1 erector pili muscle
2 cold exposure
3 grenz zone
4 Dermatitis herpetiformis, HLA-B8, premature uterine lieomyomas, increased EPO, MEN1

Vascular leiomyoma (angioleiomyoma)
1 Origin?
2 Pain worse with?

1 outer muscular wall of arteries
2 cold, pregancy or menses

Intravenous leiomyomatosis
1 location?
2 patient population?
3 origin?

1 uterine or pelvic veins even the heart
2 premenopausal middle age women
3 Either vascular invasion of uterine leiomyoma or arise de novo in a vessel

Leiomyomatosis peritonealis disseminata
1 location and patient population?
2 unique micro?
3 Prognosis?

1 throughout peritoneum, pregnant black women
2 will have scattered decidual cells
3 Will regress with estrogen and progesterone tx but recur with subsequent pregnancy

1 Patient population?
2 Locations more common to women and stimulated by?
3 General micro?
4 List 3 variants?
5 positive stains?

1 adult W>M
2 retroperitoneum, vena cava or large leg veins; by pregnancy and estrogen
3 90 degree fascicles of spindled tumor cells with pleiomorphic blunt ended nuclei and cytoplasmic vacuoles that merge with blood vessel walls
4 Epithelioid, myxoid, cutaneous,
5 muscle specific actin, smooth muscle actin, desmin (50-100%)

Epitheloid leiomyosarcoma
1 most common location? alternate name?
2 histo?

1 uterus; malignant leiomyoblastoma (spp in stomach)
2 atypical rounded cells weth clear cytoplasm, primitive muscle cells lacking desmin or collagen

Myxoid leiomyosarcoma
1 patient population and common location?
2 histo?

1 >75% in women, uterus
2 by definition >50% myxoid stroma with more low grade spindled cells than seen in typical leiomyosarc's

Sub-/cutaneous leiomyosarcomas
1 patient population?
2 Location?
3 Associations?
4 list 3 other leiomyosarcoma "variants" based on origin/location?

1 Middle aged men 2-3x > W
2 superficial, arise from erector pili in dermis or subcutanous arising from vessel wall
3 Radiation at that site
4 Retroperitoneal, deep soft tissue and LMS of vascular origin

1 Much less common than malignant counterpart called?
2 List 4 kinds?

1 Rhabdomyosarcoma
2 Of the heart, adult, fetal and genital

Cardiac Rhabdomyoma
1 Patient population and location?
2 Considered a ?
3 Histo?
4 Associated syndrome?

1 pediatric, usually multifocal (ventricles and septum), represents 50-90% of primary heart tumors in kids
2 hamartoma
3 large vaculoated (glycogen) cells with little cytoplasm and scattered "spider cells" (rim of cytoplasm with radial myofibers)
4 Tuberous sclerosis

Adult rhabdomyoma
1 patient population and location?
2 histo?
3 Molecular alteration?

1 M>W >40yrs, head and neck, 65% occur in mucosa of oropharynx, nasopharynx or larynx
2 Lobules of uniform large pink polyognal cells with variable lipid and glycogen; frequent cross striations and intracytoplasmic rod-like crystalline inclusions "jackstraws
3 15;17 translocation (like APL)

Fetal type rhabdomyoma
1 List two types?
2 patient population and location?
3 Special variant associated with peripheral nerve?
4 multicentric tumors associated with ?

1 myxoid and cellular
2 both in head and neck region, face, oropharynx, palate, posterior auricular; myxoid in boys <3yrs and cellular in older patients
3 Benign Triton tumor
4 Gorlin syndrome (nevoid BCC syndrome)

Myxoid fetal rhabdomyoma
1 histo?

1 bundles or fascicles of immature skeletal muscle and found/oval mesenchymal cells with myxoid stroma; skeletal component matures toward peripheray; may have pseudocambium layer of plasma cells beneath epithelium.

Cellular fetal rhabdomyoma
1 histo?

1 fasicles or plexiform arrangment of muscle cells at various stages of maturation with sparse collagenous or myxoid stroma; may have ganglion like rhabdomyomblasts with prominent nucleoli or strap cells

Genital type rhabdomyoma
1 Patient population and location?
2 Histo?

1 middle aged women vagina and vulva < cervix
2 polypoid, mature haphazard striated and straplike skeletal muscle cell in firous stroma with dilated vesels

1 The most common sarcoma of kids and adolescents?
2 Locations?
3 Three variants (most to least common)?
4 List subtypes (4) of most common variant?
5 + IHC and pattern?

1 Rhabdomyosarcoma
2 H&N (<9yrs) = trunk > paratesticular (last two usually teens)
3 Embryonal>>alveolar>> pleomorphic
4 NOS, botryoid, spindle cell and anaplastic
5 desmin, muscle specific actin and myoglobin, more differentiated tumor = more positivity

Embryonal NOS
1 list other common site besides H&N?
2 histo?
3 Prognosis?
4 Molecular?

1 GU
2 infiltrative undifferentiated small round blue cells (rhabdomyomblasts) with relatively uniform nuclei and inconspicuous nucleoli, occasional spindled or strap cells with more skeletal muscle differentiation with scant myxoid or collagenous stroma; may form cellular "cambium layer" beneath epithelium.
3 intermediate
4 Trisomy 8 and loss of heterozygosity at 11p 55(or 15 on PO)

Botryoid (embryonal) rhabdomyosarcoma
1 named for?
2 Location?
3 histo?
4 prognosis?

Botryoid (embryonal) rhabdomyosarcoma
1 named for?
2 Location?
3 histo?
4 prognosis?

1 "grape like" gross appearance
2 Hollow organs (80% GU !!!!)
3 Polypoid, superficial (submucosal) cellular layer of rounded or spindled rhabdomyoblasts with less cellularity deeper and more loose myxoid stroma
4 Very good

Spindle cell (embryonal) rhabdomyosarcoma
1 Most common location?
2 Histo?
3 Prognosis?

1 paratesticular
2 highly differentiated elongated fusiform spindled (>50%) rhabdomyomlasts with pink fibrillary cytoplasm arranged in fascicles with hyalinized stroma
3 Good

Anaplastic (embryonal) rhabdomyosarcoma
1 Most common location?
2 histo?
3 prognosis?

1 lower extremities
2 have bizarre hyperchromatic nuclei 3x larger than surrounding rhabdomyoblasts and bizarre mitotic figures with rare skeletal muscle differentiation
3 Poor

Alveolar rhabdomyosarcoma
1 patient population and location compared with embryonal?
2 histo?
3 prognosis?
4 have to look out for what histologic pattern?
5 molecular alterations (not present in embryonal!)

1 older (teens) and occurs on extremities more than embryonal
2 aggregates of poorly differentiated small round/oval pink tumor cells in nests surrounded by fibrous septae, cells in center of nest "float" loosely cohesive
3 poor
4 solid variant, no alveolar pattern
5 t(2;13) > t(1;13) (13= FKHR (FOXO1, 1=PAX7, 2=PAX3)

Pleomorphic rhabdomyoscarcoma
1 patient population (unique for this variant) and location?
2 histo?
3 prognosis?
4 list 3 types?

1 adults men >40yrs in deep lower extremity
2 infiltrative mixture of small primitive rhabdomyoblasts and large pleomorphic hyperchromatic cells with scant collagen
3 terrible,(70% die <2 yrs), the most common RMS to metastazsize (especially to lungs)
4 classic, round cell, spindle cell

1 List neuroectodermal tumors? (14)

1 Clear cell sarcoma
Extraskeletal ewing sarcoma
Granular cell tumor
Melanotic neuroectodermal
tumor of infancy
Melanotic schwannoma
Merkel cell carcinoma
Desmoplastic melanoma

Clear cell sarcoma
1 aka?
2 Patient population and location?
3 Histo?
4 Three positive IHC?
5 Molecular alteration with genes?
6 prognosis?

1 Malignant Melanoma of soft parts
2 Young adultss (30yrs) deep seated extremities, pelvic girdle arising near tendons or aponeuroses
3 distinct nests of round or spindled cells with clear (glycogenated) cytoplasm separated by fibrous septae (usually delicate), cells often pigmented, melanin (2/3) and have prominent basophilic nucleoli
4 S100, HMB45. MelanA/MART1
5 t(12;22), ATF1 and EWS
6 50% metastasize <5yrs

Extraskeletal Ewing Sarcoma/PNET
1 Patient population and location?
2 Histo?
4 Molecular?
5 Factors with worse prognosis?

1 Young adults (M>W), paravertebral and chest wall
2 sheets of uniform small cells with small finely granular nuclei and scant sometimes clear (glycogen) cytoplasm, richly vascularized with periepithelioumatous pattern often with necrosis and hemorrhage (*morphologically indistinguisable from bone counterpart)
3 CD99 (membranous), BCL2 positive, S100 chromogranin negative???
4 t(11;22) FLI1/EWS (also 7, 17, and 21 with 22)
5 Large size, extensive necrosis (70% 5yr survival)

Granular cell tumor
1 patient population and location?
2 cell of origin? micro?
3 IHC? cause of granular cytoplasm?
4 Reaction in superficial skin lesions?
5 prognosis?
6 Newborn gingival version?

1 W>M 4-6th decades, dermis, or submucosa, tongue isclassic location but can occur anywhere
2 schwann cell, sheets or nests of large monomorphous polygonal cells with central nuclei, granular cytoplasm and distinct cell borders
3 S100+, lysosomes!
4 pseudoepithelioumatous hyperplasia
5 benign
6 congenital epulis

Malignant peripheral nerve sheath tumor
1 Associated with? (2)
2 patient population(s) and location?
3 what tumor will these arise from?
4 histo?
5 name two histologic "variants" with alternate name

1 von Recklinghausen disease aka NF1, and radiation
2 if associated with vonR dz 30yr M>>W; if sporadic 40 M>W; trunk>extremities> H&N in association with a major nerve trunk
3 Neurofibromas (rare in schwannomas gangioneuromas ect)
4 elongated serpentine cells in hyper and hypo cellular arrangements with geographic palizading necrosis with sparing around vessels, highly variable with numerous patterns
5 Epithelioid and with rhabdomyoblastic diff (malignant Triton tumor)

Melanocytic neuroectodermal tumor of infancy
1 aka? (3)
2 patient population and location?
3 histo?
4 IHC?
5 prognosis?

1 Retinal analage tumor, melanotic progonoma, pigmented NED tumor of infancy
2 <1yr, jaw
3 Biphasic tumor with large melanin containing cells and small neuroblast like cells with nests and tubules, may have neuropil like stroma
4 Large cells (with melanin) are HMB45 and melanA positive, both types variably NSE positive
5 benign

Melanotic schwanomma
1 Location and patient population?
2 Histo
3 What is present in 50%?
4 Associated syndrome and %?
5 positive IHC?
6 other name for schwanomma?

1 spinal/midline 4th decade
2 Spindled to epithelioid cells with indistinct cytoplasm almost completely obscured by granular pigment, usually have cytoplasmic inclusions
3 Psammomma bodies (then called psammomatous melanotic schwanomma)
4 Carney syndrome, 50%
5 S100 and HMB45
6 neurilemmoma

Merkel cell carcinoma
1 what is it?
2 patient population and location?
3 Histo?
4 IHC?
5 associated tumors?

1 a neuroendocrine carcinoma of the skin
2 elderly H&N or extremities
3 dermal proliferation of uniform small round cells with fine dusty chromatin and scant cytoplasm, lots of mitoses and apoptoses
4 Positive for LMW cytokeratins (like CK20) in a perinuclear dot like pattern
5 invasive SCC, insitu SCC
(Bowen dz), BCC, adnexal carcinoma,

1 List 5 clinical variants of neurofibroma?
2 which 3 are associated with NF1?
3 Name 2 unusual microscopic variants?

1 Localized cutaneous, diffuse cutaneous, localized intraneural, plexiform, massif soft tissue
2 Diffuse cutaneous, plexiform and massive soft tissue
3 epithelioid and pigmented

1 Micro common to all neurofibromas?
2 Histo, IHC and prognosis unique to plexiform NF?
3 structures found in the 3 NF's associated with NF1?

1 spindeled schwann cells with serpentine/ comma shaped nuclei and fibroblasts in fascicles whorls or storiform pattern in its own mucopolysacharide rich matrix mixed wtih strands of "shredded carrot" collagen
2 prominent mucoid matrix, may have schwann cells forming verocay bodies; EMA positive (others are not), more likely to undergo malignant transfromation
3 Tactoid structures resembling pacinian corpuscles or Wagner Meissner bodies

1 Neurofibromatosis type 1, list diagnostic criteria?
2 AKA ?
3 chromosome and protein product involved?

1 >2 of any of the following:
cafe-au-lait spots (>6 5mm)
neurofibromas (or one plexiform
axillary/inguinal freckles
optic glioma
Lisch nodules ((>2 pigmented
iris hamartomas, 90%)
osseous lesions (non-ossifying
fibromas, skeletal erosion)
1 first degree relative with NF1
2 peripheral form or von Recklinghausen
3 17q11, neurofibromin

1 Neurofibromatosis type 2, list criteria?
2 AKA?
3 Cutaneous lesions?
4 Spinal cord lesions?
5 Chromosome and protein product?

1 bilateral 8th cranial nerve
1st degree relative with NF2 +
any two of the following:
meningioma, glioma,
schwannoma juvenile
cortical cataract
2 Central form
3 small and few cafe-au-lait spots, cutaneous schwannomas >70%, rare cutaneous NFs
4 70% ependymomas> schwannomas or meningiomas
5 22q12, merlin/schwannomin protein

Wagner Meissner bodies, fequently seen in the NFs below
1 list 5 variants of neurofibromas seen in NF-1?
2 which one is diagnostic?
3 distinct gross description and associated type?

1 localized and diffuse cutaneous
localized intraneural
massive soft tissue
2 massive soft tissue
3 "bag of worms", plexiform

1 List 5 variants of neuroma?
2 which 3 are associated with trauma?

1 Traumatic
palisaded encapsulated
1 traumatic, morton and pacinian

Traumatic neuroma
1 histo?

1 tangled web of random nerve bundles

Morton's neuroma
1 aka?
2 patient population and location?
3 histo?

1 Morton's metatarsalgia and localized interdigital neuritis
2 women between 3-4th toes
3 fusiform enlargment with extensive perineural fibrosis

mucosa neuroma
1 associated with? list features?
2 histo?
3 perineurium positive for?

1 MEN2b
b/l pheos, c0cell hyperplasia,
medullary thyroid carcinoma
parathyroid hyperplasia
2 irregular tortuous nerves with prominent perineurium

Palisaded encapsulated neuroma
1 aka?
2 patient population and location?
3 histo?

1 solitary circumscribed neuroma
2 face of middle aged adults
3 solid proliferation of schwann cells and axons surrounded by thin capsule of perineurium.

Neurothekeoma (myxoid)
1 patient population and location?
2 histo?
3 IHC?

1 kids or young adults, in dermis of head/neck or shoulders
2 plump or round spindled cells with whorled multilobular architecture srounded by thin collagen and delicate blood vessels with prominent myxoid matrix.
3 MITF positive S100 negative

1 list 2 sets of variants?
2 histo? (general kind)
3 IHC?
4 Molecular alteration

1 intra or extra neural
sclerosing and reticular
2 elongated cells in bundles or fasicles resembling perneurium may be storiform (intraneural has concentric "onion bulb" growth"
3 EMA +, S100-
4 monosomy 22

Primitive neuroectodermal tumor
1 aka?
2 On a spectrum with? difference being?
3 patient population and location?
4 micro? 2 types of rosettes?
5 positive IHC
6 molecular
7 called ? if in thoracopulmonary location in kids?

1 peripheral neuroepithelioma
2 Ewing sarcoma; obvious neural differentiation in PNETs (rossettes)
3 teens/young adults; paravertebral > extremities
4 vaguley lobularsheets of uniform round cells with scant cytoplasm, mits necrosis and crushed areas; homer wright and Flexner Wintersteiner (needed for dx)
5 CD99 (aka HBA71) and bcl2
6 t(11;22) EWS/FLI1
7 askin tumor

1 aka?
2 location and patient population?
3 variants? (8)
4 general histo?
5 molecular alteration?

1 neurilemmoma
2 adults (20-50), head and neck or flexor surfaces of extremities
3 cellular, plexiform, ancient, epithelioid/malignant change, psammomatous melanocytic, microcystic reticular (GI), pigmented, plexiform
4 encapuslated with spindled schawn cells and biphasic, nuclear palisading (verocay bodies) cellular with collagen (antoni A) and hypocellular myxoid (antoni B)
5 loss of NF2 product (merlin) on chromosome 22

ancient schwannoma
1 due to?
2 histology?

1 longstanding duration
2 degenerative changes including cyst, hemorrhage, hyalinization, calcification, marked nuclear atypia with hyperchromasia and multilobation NO mits!

Cellular schwannoma
1 histo?
2 important diff dx?
3 location?

1 composed exclusively of Antoni A areas with pleomorphism, 10% have necrosis
3 retroperitoneum, mediastinum, pelvis

1 List tumors of uncertain histiogenesis ? (12)

1 Alveolar soft part sarcoma
Angiomatoid fibrous histiocytoma
Desmoplastic small round cell
Epithelioid sarcoma
Fibrous hamartoma of infancy
Giant cell angiofibroma
Malignant extrarenal rhabdoid
Malignant mesenchymoma
Ossifying fibromyxoid tumor
Synovial sarcoma

Alveolar soft part sarcoma
1 patient population and location?
2 histo?
3 EM/unique histo?
4 pertinent negative IHC?

1 young adults F>M (15-35), deep soft tissue lower extremities, oral cavity mediastium
2 large uniform polygonal pink cells with distinct cell borders and prominent nucleoli arranged in uniform nests (alveolar if discohesive) separated by delicate fibrous septa. Larger vessels and septa at periphery
3 Rhomboid rod shaped crystals (PASD +)
4 keratin negative

Angiomatoid fibrous histocytoma
1 location and patient population?
2 symptoms?
3 histo?
4 pertinent negative IHC?
5 molecular alteration FACT CHECK
6 prognosis?

1 ares of normal lymph nodes, in young adults and kids
2 systemic such as anemia fever weight loss and parproteinemia
3 multinodular proliferaton of uniform pink histiocytoid or myoid cells with areas of hemorrhagic cysts or pseudoangiomatoid spaces, with peripheral chronic inflammatory aggregates surrounded by thick fibrous capsule
4 vascular markers
5 t(12;16)(q13;p11) creating ATF1-FUS
6 good, 10% recurrence >1% mets

desmoplastic small round cell tumor
1 patient population and location?
2 histo?
3 positive IHC?
4 molecular alteration
5 prognosis?

1 young adults (15-35) abdominal, pelvic or peritnoeal
2 small luniform round/oval cells with indistinct scant cytoplasm in large nests "trapped" in desmoplastic stroma with cystic necrotic spaces
3 keratin, desmin, EMA, WT1
4 t(11;22), EWS/WT1
5 terrible, highly aggressive 75% mortality within 2.5 yrs.


Epithelioid sarcoma
1 patient population and location?
2 histo?
3 positive IHC?
4 prognosis?
5 describe "variant"

1 young adults, distal extremities especially hands
2 nodular round or polygonal epithelioid cells imperceptibly mixed with spindled cells often with central necrosis which tumor cells "palisade" around creating granuloma like appearence
3 cytokeratins and 50-60% CD34+
4 80% recurrence at 10yrs and 40% metastasize
5 proximal variant occuring in pelvis perineum or genital tract, marked cytologic atypia may have rhabdoid features

Fibrous hamartoma of infancy
1 patient population and location?
2 histo?
3 prognosis?

1 <2yrs or ~20% at birth, B>G, usually in axilla also extremities inguinal and trunk but NEVER hannds or feet
2 triphasic organoid pattern composed of spindled cells with collagen, rounded immature stellate cells in mucoid matrix and fat
3 totally benign

Giant cell angiofibroma

1 Patient population and location?

2 histo?

3 positive IHC? which cells?

4 Prognosis?

1 middle aged adults, eyelid or orbit

2 admixture of round/spindled cells with indistinct cytoplasm and myxocollagenous stroma with small/medium ectatic blood vessels lined by variable giant cells

3 CD34 and CD99 in stroma

4 good, excision curative

Hemangiopericytoma (on spectrum or same as Solitary Fibrous Tumor)

1 Patient population and location?

2 histo?

3 positive/negative IHC?

4 Molecular alteration?

1 middle aged adults; deep seated in thigh, pelvic retroperitoneum and orbit

2 uniform round/spindled cells with scattered ectatic "staghorn" vessels

3 CD99 and CD34 (variable)/ CD31 neg

4 12q13-15 alterations

1 List 7 tumors with a "hemangiopericytoma-like vascular pattern"

1 synovial sarcoma

fibrous histocytoma



Extrapleural SFT



Endometrial stromal


Malignant extrarenal rhabdoid tumor

1 patient population and location?

2 histo?

3 prognosis?

4 IHC/molecular alteration?

1 infants and kids, deep soft tissue of trunk, abdomen, pelvis, retroperitoneum (anywhere really)

2 sheets of discohesive round ro polygonal celsl with exccentric nuclei and prominent nucleoli with eoinophilic cytoplasm containing globular cytoplasmic inclusions near nucleus

3 really horrible, very aggressive

4 loss of INI1, called SMARCB1

1 List 4 variants of myxoma with alternate names

2. All have a pretty good prognosis but which two have a higher recurrence rate and what is it?

1 intramuscular, digital, juxta-articular (digital mucous cyst), cutaneous (superficial angiomyxoma)

2. juxta-articular and superficial angiomyxomas have 30% local recurrence rates

intramuscular myxoma

1. patient population and location?

2. Histology ? molecular alteration and %?

3. gross appearance, location and patient population for digital myxoma? other name?

1. W>M 50-70 in large skeletal muscles thigh, buttock, upper ext.

2. few bland spindled to stellate fibroblast cells with scant eosinophilic tapering cytoplasm in myxoid stroma; infiltrative at edges of lesion but no atypia or mits; 61 % have GNAS1 mutations

3. 2x as common in women a dome shaped nodule on finger usually solitary and pinful with a verucous surface; same histology; superficial acral fibromyxoma

Juxtarticular myxoma

1. patient population and location?

2. Describe patient populatoin and location for cutaneous myxoma? what is associated with multiple, pathognomonic?

3. Histology of cuteous mysomas?

1. 4-6th decares M>W, 90% around knee, 25% meniscus of large joint shoulder elbow hip ankle

2. M=W 4-6 decades, usually sucuranous on trunc, genitalia, H&N; multiple associated with Carney complex while an external ear lesion is pathognomonic for Carney

3. dermal pfoliferation of bland plunp spindled or stellate cells in multilobulated pattern, no atypia or mits bug may entrap epithelial or adpiocytic elements

1. Which two myxomas have syndrome associations?

2. List and describe each?

3. Which myxoma is NOT associated with any syndroms?

1. intramuscular and cutenous myxomas


McCune albright: mono or poly ostotic fibrous dysplasia, melanotic pigmentation of skin, endocrine abnormalities (precocious puberty) GI polyps, hypophosphatemic osteomalacia and bone tumors (b9 and malignant)

Mazabrauds: fibrous dysplasia

CUTANEOUS myxomas:

Carney syndrome: cardiac and breast myxomas, spotty pigmentation, endocrine tumors (adrenal or pituitary), large cell calcifying sertoli cell tumor of testis, psammomatous melanotic schwanomma

3. Juxta-articular, its all on its own :)

Ossifying fibromyxoid tumor

1. patient population and location?

2. histology?

3. Features suggesting an typical or malignant?

4. What positive stain suggests cell of origin and what is it?

5. Prognosis?

1. m=w over 50yrs with 70% on the extremities> trunk and H&N

2. Nests of uniform round to polygonal cells with pale to clear eosinophilic cytoplasm ill defined cell borders and uniform nuclei in abundant fibromyoid collagneous stroma; stoma may have thrombosed vessels, 80% incomplete shell of lamellar bone

3. central rather than peripheral osteoid, increased cellularity and high mitotic rate

4. 75-80% are s100 positive suggesting schwannian origin

5. Benign!!

Synovial sarcoma

1. Patient population and location ?

2. Two types and histology? which is more common?

3. List positive stains? which one is actually useful/specific?

4. Molecular alteration?

5. List features portending a better and worse prognosis?

1. younger patients (15-40 yrs) M>F; usually deep seated mass 80% around knee or ankle!

2. Monophasic and biphasic (more common); spindled cells are plump wiht scant cytoplasm adn oval hyperchromatic nuclei that are arranged in sheets and/or herringbone fascicles with alternating hyper and hypocellular areas; in biphasic an epithelial component is added and makes nests cords glandular structures or papillae

3. cytokeratins EMA, CD99 and BCL2 are all positive but TLE1 is the bomb stain for SSs!

4. t(X;18) translocates SYT with SSX1 or SSX2

4. worse: extensive necrosis, rhabdoid cells, high mits, high brade nuclei, monophasic

better: biphasic wiht >50% epithelial component, calcs, osseous metaplasia, youger (<15yrs), distal ext lesions, small lesions, low mitotic rate and low necrosis

1. List 4 presentations of angiosarcoma and describe each?

1. cuteaneous w/o lymphedema often on scalp or forhead looking like a bruise, 50% multifocal

Cutenous WITH longstanding lymphedema, 90% after mastectomy called Stewart Treves syndrome

Breast primary, rapidly enlarging with blue discoloration after radiation therapy

Radiation induced Deep soft tissue of ext or abdominal cavity with extensive hemorrhage and necrosis


1. Histology?

2. Liver angiosarcomas assocated with?

1. ill defined infiltrative with wide range of differentiation, irregular anastomosing vascular channels that dissect tissue along facial planes and collagen bundles with atypical endothelial cells that may be tufted or form signet ring like single cell vascular channels

2. Thorotrast (thorium dioxide), vinyl cloride (PVC), silver oxide containing insectisides, arsenic poisoning, androgenic anabolic steroids

Glomus tumor

1. patient population and location?

2. Histology? List and describe variants?

3. Positive IHC?

1. equal sex distribution exept for subungal which is more common in women (and the most common site), 3-5 decades and usually a solitary dermal or subcutaneous mass, usually painful and sensitive to cold and touch

2. classic: nests or sheets of round uniform cells with pink cytoplasm and hyprchromatic nuclei surrounding capillaries and peripherally encircled by rim of collagen

glomangioma: cavernous hemangioma like arrangement of clusters of glomus cells around gaping blood vessels, +/- secondary thrombosis

Glomangiomyoma: cells transitioning between glomus and smooth muscle cells

Symplastic: marked cytologic atypia but no other bad features

Malignant glomus tumor (glomangiosarcoma) can be spindled or round cell but must be >2cm subfascial or visceral and have lots of atypical mites or marked nuclear atypia

3. Smooth muscle actin

1. List variants (5)?

2. What is a hemagioendothelioma?

1. Epithelioid, retiform, spindle cell, malignant endovascular papillary angioendothelioma (dabska tumor) , kaposiform

2. A vascular tumor with intermediate behavior between angiosarc and hemangioma

epithelioid HE

1. Patient population and location? Caveat?

2. Histology?

3. Unique treatment for what location?

4. Location with worst prognosis?

1. M=W, distal ext of young adults, 50% arising in wall of vein; women more likely to have parenchymal (lung liver bone) lesions that are multifocal

2. epithelioid or histiocyte like tumor cells with abundant eosinophilic cytoplasm arranged in short strands or nests with bland nuclei and intracytoplasmic lumina (vacuoles) containing rbs, tumor cells may arise from vessel (50%) extending out into soft tissue, and has either myxoid or hyalinized stroma f variable content

3. Liver primary can be treated with transplant, 75% 5 year survival

4. pulmonary primary has 65% mortality rate, liver 35%

Retiform HE

1. Location and patient population?

2. Histology?

3. This tumor is closely related to ?

4. IHC positive?

1. plaque like nodule of skin and subcutaneous tissue of distal extremities of young people

2. diffuse dermal involvment charactherized by elongated narrow arborizing vascular channels (looks like rete testis) lined by hyperchromatic endothelial cells hobnailing into lumina, half have prominent stromal and pervascular lymphocytic infiltrate

3. Dabska tumor

4. CD31, CD34, von willebrand factor

spindle cell HE

1. Location and patient population?

2. Histology?

3. IHC positive? shared with?

4. Prognosis?

5. Associated with 2 syndromes and one congenital condition?

1. distal extremities (especially hand) of young M>F (50% <25yrs)

2. Combo of cavernous hemangioma and kaposi sarcoma features; bland spindled tumor cells in multinodular pattern with peripheral gaping vascular channels at periphery and scattered itractyoplasmic vaculones like epithelioid HE

3. Factor VII positive (spp not in the spindled part though but he vascular spaces and epithelioid areas) as is epithelioid HE, and dabscka tumor (factor VII NOT positive retiform and not kaposiform)

4. 60% local recurrence rate

5. Maffucci syndrome, Klippel Trenaunay syndrome and congenital lymphedema

Malignant endovascular papillary angioendothelioma

1. AKA? patient population and location?

2. Histology?

3. Prognosis?

1. dabska tumor, skin and soft tissue of children and infants, RARE in adults!

2. plumph endothelial cells forming papillay tufts within dilated well formed vascular spaces (glomeruloid) and also hobnailing often with intraluminal rbc's lymphocytes and perivascular lymphocytes

3. Good prognosis despite reginal lymph node metastases

Kaposiform hemangioendothelioma

1. Patient population and location?

2. Histology?

3. Associations? (one syndrome to explain)

1. exclusively in kids and adolescents in superficial (75% skin) or deep soft tissue (20% reroperitoneum)

2. combines features of capillary hemangioma and kaposi sarcoma; intersecting fascicles of bland spindle cells mixed with capilaries; fasicles may be compact or have slit like or cresent vascular lumens and scattered glomeruloid nests are seen

3. lymphangiomatosis, Kasabach Merrit syndrome (consumptive coagulopathy and thrombocytopenia especially with deep tumors like retroperitoneum)

1. List 16 types of hemangiomas with alternate names?

1. capillary, acquired tufted angioma, hobnail, verrucous, cherry angioma (senile angioma), cavernous, arteriovenous, venous, spindle cell, epithelioid (angiolymphoid hyperplasia with eosinophilia), pyogenic granuloma (granulation tissue type capillary), intramuscular, synovial, perineural (hemangioma of peripheral nerve), diffuse (angiomatosis), papillary enodthelial hyperplasia (intravascular HE of Masson)

(lobular) Capillary hemangioma

1. list 3 variants of capillary hemangioma with most common first?

2. Patient population, location and natural history? tx?

3. Histology?

4. If congenital or perinatal these are all considered inflantile hemangiomas and are positive for ?

1. Cellular hemangioma of infancy, pyogenic granuloma (aka lobular capillary hemangioma) and epithelioid hemangioma

2. children especially H&N locations, appears shortly after birth and grows rapidly but will spontaneously regress; corticosteroids or interferon alpha

3. Plump to flat endothelial cells with variable pericytes arranged in multilobular pattern with branching small vascular channels, fibrotic stroma increases with age of lesions (as they involute)

3. GLUT 1

Targetoid hemosiderotic hemangioma

1. aka?

2. patient population and histology?

1. hobnail hemangioma

2. Exophytic skin lesion young adults, with superficial dilated vessels lined by hobnnail endothelial cells with slit like capillaries at deeper portion

Cherry angioma

1. aka?

2. Patient population, gross appearance and histology?

1. Senile angioma

2. older people, most common angioma that increases with age, has red papule with pale halo on trunk and extremities of adults, made of thin walled dilated capillaries covered wtih atrophic epidermis

Cavernous hemangioma

1. patient population and location?

2. histology?

3. Treatment?

4. Associations? describe?

1. kids upper body, larger, deeper less well circumscribed than capillary hemangioma, may destroy other structures

2. medium to large blood vessels with flattened endothelium arranged in lobular or diffuse pattern
+/- distrophic calcs

3. recombinant interferon alpha2a and pentoxifylline

4. kasabach merrit syndrome (consumptive coagulopathy) blue rubber bleb nevus syndrome (AD, cutaneous and GI hemangiomas)

Maffuci syndrome (enchondromas multiple hemangiomas, cavernous and or spindle)

Spindle cell hemangioendothelioma

1. gross appearance?

2. Histology?

3. Associated with what?

4. Prognosis?

1. often multiple noduels in same anatomic area

2. thin walled cavernous vessels lined by flat endothelium separated by spindled areas containing epithelioid cells with cytoplasmic vacuoles/lumens

3. Mafucci syndrome (multiple enchondromas and soft tissue hemangiomas, spindled or cavernous)

Klippel Trenaunay syndrome

early onset varicosities, congenital lymphedema and epithelioid hemangioendothelioma

4. 60% recurrence rate

Epithelioid hemangioma

1. aka?

2. patient population and location?

3. histology?

4. treatment and prognosis?

1. angiolymphoid hyperplasia with eosinophilia

2. 30-50 y/o women in superficial H&N region often periauricular

3. epithelioid endothelial cells with vacuolated eosinophilic cytoplasm in multilobular pattern ususally with large central vessel surrounded by smaller vessels with a mixed inflammatory infiltrate with eos

4. superficial radiotherapy gives 80% response rate

Papillary endothelial hyperplasia

1. aka?

2. what is it? location?

3. histology?

1. Intravascular hemangioendothelioma of Masson

2. an organizing thrombus in a blood vessel wiht occasional soft tissue extension, often in association with b9 vascular lesions; often H&N location fingers or trunk (hemorrhoids)

3. well defined interatastomosing channels and papillary structures line by benign endothelium often associated with residual orgnaizing thrombus

Weibel palade body

1. What is it?

1. rod shaped storage granules composed of parallel tubules typically found in endothelial cells that contain P-selectin and von Willebrand Factor

Kaposi sarcoma

1. List and describe 4 types?

1. classic: indolent, 90% in elderly men of Mediterranean/ east european/ Jewish descent, indolent corse with death in 8-10 yreas, 25-30% have secondary malignancy of which half are heme origin, red cell aplasia, autoimmune hemolitic anemia

lymphadenpathic: African, LAD in children or LE dz in men, kids have rapid progression due to internal organ involvement, not so for adults

transplant associated: 1% incidene, 100% death rate unless immunosupression tx is decreased by at least 50% which --> marked response rate of 100%, balancing game, associated with Castlemans dz and angioimmunoblastic LAD

AIDS related: 30% develop, highest incidence in homosexual men, widespread skin and oral lesions, 50% LN involvement, 30% GI involvement, mortality rate of 40%,

Kaposi sarcoma patch

1. Histology?

2. Charactheristic "sign"?
3. Nuclei in all forms of kaposi are positive for?

1. earliest stage, composed of subtle small thin walled vessels dissecting though collagen

2. Promontory sign: when existing vessels and adnexal structures are trapped and protrude into the neoplastic luimina

3. HHV-8

Kaposi sarcoma plaque

1. histology?

2. Characteristic hitologic finding?

1. more prominent anastomosing irregular vessels + spindled cell component and appearance of intra/extra cytoplasmic hyaline globules and extravasated RBCs

2. autolumination: paranuclear vacuoles containing RBCs

Kaposi sarcoma nodule

1. Histology?

2. buzzword?

3. Staining of globules?

1. same as plaque only more prominent everything especially consolidation of spindled areas into curvilinear fascicles resembling fibrosarcoma and clusters of hyaline globules

2. Seive-like pattern

3. PAS positive diasetase resistant


1. patient population and location?

2. Histology?

3. pertinent negative stain?

1. subcutaneous nodule on distal extremities of middle aged adults with = sex predilection

2. well circumscribed nonencapsulated lesion composed of monomophic spindle to oval eosinophlic or amphophilic cells growing in multilayered concentric fashion around lesional blood vessels and occasional whorled or fasicular with myxoid stroma often with subendothelial proliferation (making luminal mass)

4. desmin negative despite myoid appearance