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61 Cards in this Set
- Front
- Back
Dx?
Distribution? Features? |
Spindle cell / pleomorphic lipoma
Cape distribution -Mature fat -small uniform spindle cells -THICK ROPY COLLAGEN -Mast cells |
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genetics of spindle cell / pleomorphic lipoma?
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13q & 16q
(vs LS, 12q) |
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Pleomorphic lipoma
FLORET GIANT CELLS |
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Painful
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Angiolipoma
-Small capillaries - FIBRIN THROMBI |
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Hibernoma
Brown fat tumor |
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Dx?
IHC? |
Angiomyolipoma
-Thick walled bvs - Immature spindled cells - fat IHC: HMB45+ Desmin+ |
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Types of liposarcoma
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Well-diff / ALT
Myxoid / Round cell Pleomorphic De-diff |
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Myxoid liposarcoma
- CHICKEN WIRE BVs - Myxoid matrix - Adjacent ROUND CELL component |
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Pleomorphic liposarcoma
Hard to tell that it is fatty - Large atypical malignant cells |
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mass in retroperitoneum
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Well-diff liposarcoma
• Widened septa • Low power “busy appearance” • Heterogeneous cell size and shape • Lipoblasts may be seen. • Hallmark finding: lipocytic atypia |
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Difference between WD-LS vs. ALT?
IHC? |
SITE!
WD-LS if groin, retroperitoneum, intramuscular ALT everywhere else S100+ in fat CD34+ spindled cells |
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True or pseudo-lipoblast
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Left: Pseudo. histiocyte, multinucleated, eosinophilic cytoplasm
Right: TRUE! Large single nuc, little cytoplasm Above: True. SCALLOPING of nuclei by fat |
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Silicone granuloma of the breast
Characteristic cystic spaces |
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Dx?
IHC? |
Neurofibroma
-Tapered buckled nuclei -Shredded carrot collagen S100 + (Schwann cells) CD34+ (intraneural fibroblasts) |
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Plexiform neurofibroma
-bag of worms, tortuous DIAGNOSTIC of NF1! CAN become malignant! |
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Diffuse NF
WAGNER-MEISSNER bodies (highlighted with S100) • Ill-defined subcutaneous mass • Honeycomb pattern, reminiscent of DFSP • Wagner-Meissner bodies are the hallmark. • Typically does not undergo malignant transformation by itself *usually only if associated with plexiform. |
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NF1
-name? - inheritance? - genetics? |
Von Recklinghausen's disease
AD Mutation tumor suppressor gene NF1 on chromosome 17 5% get MPNST |
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NF1 clinical features?
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• Café-au-lait macules
• Neurofibromas can be plexiform type • Axillary/inguinal freckling • Optic glioma (pilocytic astrocytoma) • Lisch nodules (iris hamartomas) • Bone changes osteoporosis, kyphoscoliosis, pseudoarthrosis |
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Dx and main differential?
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MPNST
-Must arise from nerve, PNST, or in NF1 patient - Increased cellularity, fascicular growth, mitoses |
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Must r/o DESMOPLASTIC MELANOMA in ddx of MPNST...
How? |
Both are S100+ but DM is strong and MPNST patchy. Also MPNST is negative for MART, MelanA, HMB45... but DM often is too! MPNST can show some GFAP+ (DM-)
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What is a malignant Triton tumor?
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MPNST + RMS
Desmin + rhabdomyoblasts |
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Dx?
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Schwannoma
-Encapsulated - Verocay bodies - Antoni A (cellular) &B (hypocellular) areas -Hyalinized bvs -Pericapsular lymphocytic cuffing |
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Schwannoma IHC?
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++S100
+EMA in capsule -ckit -CD34 (can be + in antoniB area) |
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Bilateral acoustic schwannomas
Syndrome? Genetics? |
NF2
Merlin gene chrom 22 |
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features NF2?
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Bilateral acoustic schwannomas
Glial hamartomas Cataracts Meningiomas |
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rapid growth
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Nodular fasciitis
-Well-circumscribed, not encapsulated -STROMAL MUCIN -monomorphic -Tissue-culture like pattern -storiform -EXTRAVASATED RBCs but NO HEME -Can see KELOIDAL collagen -Reactive atypia & mits Benign |
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Proliferative fasciitis
vs NF: Both benign, solitary NF: Younger patients (15-40) PF: Older patients 40-60, less often trauma related areas with Ganglion-cell-like myofibroblasts (Actin+, S100-, desmin-) Must r/o RMS (desmin) Ganglioneuroblastoma (S100+, GFAP+) |
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Dermatofibroma / fibrous histiocytoma
-PUSHING border -Storiform pattern -can have MNGCs -can have hemosiderin |
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Do DFs recur?
IHC? |
CELLULAR form 25% recur
Classic form no - 1% + a1-antitrypsin + F8a - CD68 |
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Dx?
Types? IHC? Syndrome? |
Fibromatosis
-Benign myofibroblasts, parallel arrangements, elongated BVs, infiltrative -From fascia Superficial vs. deep fibromatosis NUCLEAR B-catenin inactivation of APC t.s. gene also +SMA, MSA, vimentin, calponin; (-)CD34 |
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GARDNER SYNDROME
gene? features? |
5q (APC in WNT pathway)
AD Deep abdominal fibromatosis / mesenteric fibromatosis (prominent myxoid change) Fibromas Osteomas GI adenomas Epidermoid cysts |
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Fun facts fibromatosis
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Intermediate category
Treated with wide local excision Infantile fibromatosis - <2y; H&N, UE, thigh. Plantar fibromatosis is called LEDDERHOSE DISEASE Abdominal fibromatosis occurs in pregnancy Juvenile hyaline fibromatosis is a rare AR disorder; in skin, oral, bone, joints Fibromatosis Colli - seen at birth, sternocleiodmastoid muscle, associated with breech & forceps |
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Dx?
IHC? |
DFSP
- Intermediate category - Dermis and subq - Parallel to surface - STORIFORM cartwheeling pattern - HONEYCOMB infiltration of fat IHC: CD34+ (vs DF-) |
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DX?
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DFSP (honeycomb infilt of fat)
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Pigmented DFSP = BEDNAR TUMOR
DFSP with melanin |
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This area was demarcated from a DFSP in the deeper subq
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"DFSP with fibrosarcomatous transformation"
- needs >5% of area - plump spindled cells - high nuclear grade - 7-10mits/10hpf - REDUCED CD34 STAINING! (one of the only remaining lesions called fibrosarcoma...) |
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Tumor scapula
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Elastofibroma
Rare, benign, elderly |
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Kasabach-Merritt syndrome
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Cavernous hemangioma with thrombocytopenia
rare, kids |
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Cystic hygroma
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= lymphangioma in Turner Syndrome (XO)
D240+ |
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Clinical scenarios?
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Angiosarcoma
Skin, liver, bone, spleen Post-mastectomy with lymphedema (lymphangiosarcoma = STEWART TREVES SYNDROME) Post-radiation ~ 5y out + CD31, CD34 |
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Hemangioendothelioma
INTERMEDIATE GRADE! ... many types... ** (+)FLI-1, CD31 |
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Dx?
IHC? |
Glomus tumor
(arises from glomus body - A-V anastomosis involved in heat regulation) Usually red-blue nodule in deep dermis or subcutaneous tissues of upper and lower extremities. Can have solid areas Most commonly subungual region of the finger +SMA, vimentin, Type4collagen CD34 in only 30% -S100 |
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Kaposi sarcoma
AIDS, HHV8 Spindle cell fascicles EXTRAVASATED RBCs PAS+ HYALINE GLOBULES |
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SMA+
Desmin+ |
Leiomyosarcoma
Intersecting fascicles CIGAR shaped nuc PARANUCLEAR vacuoles (-) ckit, CD34 |
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buzzwords?
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Botryoid / grape like clusters
CAMBIUM layer = Embryonal rhabdomyosarcoma |
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#1 stain for embryonal rhabdo?
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MYOGENIN! (nuclear)(spec)
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Dx?
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Rhabdomyoblasts of embryonal RMS
- #1 sarcoma kids & teens - H&N, GU -EXCELLENT px -tadpole tapered cells -Cross-striations |
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Embyronal rhabdo mets to?
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LUNG > LN > Liver > brain
Alveolar: Lung > LN > bone > brain |
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Px?
Site? Genetics? Metastatic pattern? |
Alveolar rhabdomyosarcoma
"A"wful px Extremities, H&N (GU rare) Mets to Lung > LN > BONE > brain cellular clusters with fibrous septae t(2;13) t(1;13) |
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Stomach mass, CD34+
IHC? Treatment? |
GIST
ckit CD34 DOG1 Gleevec |
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Dx?
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Alveolar soft part sarcoma
Rare. teens. thigh. -organoid pattern - PAS+ crystals - TFE3+ |
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rhomboid crystals of alveolar soft part sarcoma
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What tumor is classically vimentin negative?
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Alveolar soft part sarcoma
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Limb-girdle male 15-30y.
Dx? IHC? |
Synovial sarcoma
(NOT related to synovium!) Monophasic, biphasic, or poorly diff Ovoid nuc with abundant cyt EMA+ CK7 & 19+ bcl2+ 60% + CD99 |
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20-40y
Extremity Aponeurotic lesion |
Clear cell sarcoma
Nested growth Clear cyt SCATTERED GIANT CELLS LOW PLEOMORPHISM poor px |
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Clear cell sarcoma
Classic stains? Genetics? |
CONTAINS MELANIN!
S100, HMB45 t(12;22) |
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Abdominal mass
classic translocation? |
Desmoplastic small round cell tumor
Always in abdomen Kids & young adults widespread serosal involvement Nests of neoplastic cells can have necrosis i/cytoplasmic pink rhabdoid inclusions poor px t(11;22) Classic IHC? |
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IHC Desmoplastic small round cell tumor?
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EMA
NSE Desmin WT1 |
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Kids
Diaphysis |
Ewing sarcoma / PNET
Uniform small round blue cells Homer-Wright rosettes (surround neuropil) |
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Ewings
IHC? Genetics? |
CD99
t(11;22) |
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Immobile patient over bony prominence
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Ischemic fasciitis
coagulative necrosis fibrosis myxoid change vascular proliferation |
