Diagnosis and variants M/F AgeAbdominal fibromatosis F>M YoungAcquired tufted angioma ChildrenAcral myxoinflammatory fibroblastic sarcoma M=F 30-50Adenomatoid tumor 30-60Adult rhabdomyoma M>F >40Aggressive angiomyxoma F>MAlveolar soft part sarcoma F>M 15-35AmyloidomaAngiolipoma M>F 15-50 - Cellular angiolipoma - Spinal angiolipomaAngiomatoid fibrous histiocytoma 10-40Angiomatosis (Diffuse hemangioma) 0-20Angiomyofibroblastoma F>>MAngiomyoma F>M 30-60Angiosarcoma - Cutaneous angiosarcoma without lymphedema M>F 40+ - Angiosarcoma associated with lymphedema F>M 60-70 - Angiosarcoma of the breast F>>M 20-40 - Angiosarcoma of soft tissue M>F Any - Radiation-induced angiosarcomaArterial spidersArteriovenous hemangiomaAtypical fibrous histiocytoma 20-50Atypical fibroxanthoma ElderlyAtypical vascular lesionBacillary angiomatosis M>FCalcifying aponeurotic fibroma M>F 8-14Calcifying fibrous pseudotumor F>M 10-30Cardiac rhabdomyoma M=F InfantsCavernous hemangioma Children - Sinusoidal hemangioma F>MCellular angiofibroma M=FCherry angiomaChondroid lipoma F>M 20-40Clear cell sarcoma of tendon and aponeurosis 20-40Clear cell sarcoma-like tumor of gastrointestinal tractCrystal-storing histiocytosisCutaneous leiomyoma EarlyCutaneous myxoma M>F 20-40Dedifferentiated liposarcoma M=F 50-70Dermatofibrosarcoma protuberans M>F 20-50 - Pigmented DFSP (Bednar tumor)Desmoplastic fibroblastoma M>F 40-70Desmoplastic melanoma OlderDesmoplastic small round cell tumor M>F 15-35Diffuse lipomatosis 0-2Diffuse malignant mesothelioma M>F 45-75Elastofibroma F>M 50-70 - ElastofibrolipomaEBV-associated smooth muscle tumor ChildrenEpithelioid angiomatous noduleEpithelioid hemangioendothelioma M=F 10-90

Epithelioid hemangioma F>M 20-40Epithelioid sarcoma M>F 10-35 - Proximal-type epithelioid sarcomaEpithelioid sarcoma-like hemangioendotheliomaExtra-abdominal fibromatosis F>M 15-40Extra-cranial meningiomaExtra-gastrointestinal stromal tumor AdultsExtra-skeletal chondroma M>F 30-60Extra-skeletal Ewing's sarcoma/PNET M>F 0-30Extra-skeletal mesenchymal chondrosarcoma F>M 15-35Extra-skeletal myxoid chondrosarcoma M>F 35-60Extra-skeletal osteosarcoma M>F 35-80Extra-spinal ependymomaFetal rhabdomyoma - Myxoid fetal rhabdomyoma M>F 0-1 - Intermediate fetal rhabdomyoma M>F Adults - Neural variant of fetal rhabdomyomaFibroma of the tendon sheath M>F 20-50 - Pleomorphic fibroma of the tendon sheathFibrodysplasia ossificans progressiva M=F 0-6Fibromatosis colli M>F NewbornFibrosarcoma, classic type M>F 20-50Fibrous hamartoma of infancy M>F 0-2Fibrous histiocytoma (dermatofibroma) 20-50 - Aneurysmal fibrous histiocytoma - Cellular fibrous histiocytoma - Epithelioid fibrous histiocytoma - Lipidized fibrous histiocytomaFlorid vascular proliferation of the colonGanglion cell choristomaGanglion cyst F>M 25-45Ganglioneuroma M=F 5-40Gardner fibroma M=F YoungGenital rhabdomyoma F>>M YoungGiant cell fibroblastoma M>F 0-10Giant cell tumor of low malignant potentialGiant cell tumor of tendon sheath - Diffuse type F>M 4-76 - Localized type F>M 30-50 - Malignant F>M 12-79Gingival fibromatosis M=F YoungGlial heterotopia InfantsGlomangioma ChildrenGlomangiomatosis ChildrenGlomangiomyomaGlomeruloid hemangiomaGlomus tumor M=F 20-40 - Malignant glomus tumor - Glomus tumor of uncertain malignant potential - Symplastic glomus tumorGranular cell reactionGranular cell tumor F>M 30-60 - Congenital granular cell tumor F>M InfantsHemangioma of peripheral nerves 0-40

Hemangiopericytoma-like tumor of nasal passagesHemangiopericytoma / solitary fibrous tumor M=F AdultsHemosiderotic fibrohistiocytic lipomatous lesion AdultsHereditary hemorrhagic telangiectasia ChildrenHibernoma M>F 20-40Histoid leprosyHobnail hemangioma YoungHobnail hemangioendothelioma AnyIdiopathic retroperitoneal fibromatosis M>F 20-70Infantile digital fibromatosis F>M 0-3Infantile fibromatosis (lipofibromatosis) M>F 0-8Infantile fibrosarcoma M>F 0-2 - Infantile rhabdomyofibrosarcoma 0-3Infantile hemangiopericytoma 0-1Inflammatory myofibroblastic tumor F>M 0-45Inflammatory pseudotumor M~F 30-50Intra-abdominal fibromatosis F>M 20-35Intramuscular hemangioma M=F 0-30Intramuscular lipoma / Intermuscular lipoma M>F 30-60Intramuscular myxoma F>M 40-70Intranodal palisaded myofibroblastomaIntravenous leiomyomatosis F Pre-MPIschemic fasciitis F>M 70-90Juvenile hemangioma InfantsJuvenile hyaline fibromatosis M>F 0-5Juvenile xanthogranuloma InfantsJuxta-articular myxoma M>F 20-50Kaposi sarcoma - Classic Kaposi sarcoma M>F 50-70 - Endemic Kaposi sarcoma F>M - Iatrogenic Kaposi sarcoma - AIDS-related Kaposi sarcoma M>F YoungerKaposiform hemangioendothelioma ChildrenKeloid M=F 15-50Knuckle pads M=F 30-60 - PachydermatodactylyLeiomyoma of deep soft tissue M=FLeiomyomatosis peritonealis disseminata F Pre-MPLeiomyosarcoma F>M - Retroperitoneal/abdominal leiomyosarcoma F>M 60-70 - Leiomyosarcoma of somatic soft tissue M=F - Cutaneous leiomyosarcoma M>F 40-70 - Leiomyosarcoma of vascular origin F>F 50Lipoblastoma / Lipoblastomatosis M>F 0-35Lipoma M>F 40-60 - Fibrolipoma, sclerotic lipoma, myxolipoma, chondrolipoma, osteolipomaLipoma of joint (lipoma arborescens) M>F AdultsLipoma of tendon sheath M=F 15-35Localized massive edemaLow-grade fibromyxoid sarcoma M>F Wide - Hyalinizing spindle cell tumor with giant rosettes M>F 20-40Lumbosacral lipoma F>M 0-10Lymphangioma M>F 0-2 - Acquired progressive lymphangioma

- Cystic lymphangioma M>F ChildrenLymphangiomatosis M=F ChildrenMalakoplakiaMalignant extrarenal rhabdoid tumorMalignant fibrous histiocytoma M>F 50-70 - Storiform-pleomorphic MFH - Myxoid MFH - Giant cell MFH - Inflammatory MFHMalignant mesenchymomaMalignant peripheral nerve sheath tumor M~F 20-50Mammary-type myofibroblastoma M>F 60-70Melanotic neuroectodermal tumor of infancy 0-1Melanotic schwannomaMorton's neuroma F>MMucosal neuroma 0-20Multicystic peritoneal mesothelioma F>M YoungMuscular fibrosis 0-3Mycobacterial pseudotumorMyelolipoma >40Myofibroma / myofibromatosis M>F WideMyofibrosarcoma (myofibroblastic sarcoma) M>F 30-40Myolipoma F>M 40-60MyopericytomaMyositis ossificans M>F Young - Fibro-osseous pseudotumor of the digits F>M YoungMyxofibrosarcoma 40-70 M>F - Epithelioid myxofibrosarcomaMyxoid liposarcoma 25-45 - Round cell liposarcomaNasopharyngeal angiofibroma M>>F 10-20Nerve sheath ganglionNeural fibrolipoma (lipofibromatous hamartoma) M>F 0-30Neuroblastoma and ganglioneuroblastoma M>F 0-5Neurofibroma - Localized neurofibroma M=F 20-30 - Plexiform neurofibroma Children - Diffuse neurofibroma - Pigmented neurofibromaNeuromuscular hamartoma ChildrenNeurothekeoma Children - Cellular neurothekeomaNevus flammeusNevus lipomatosus cutaneous superficialis M=F 0-20Nodular fasciitis M=F 20-40 - Cranial fasciitis Infants - Intravascular fasciitis M=F < 30 - Ossifying fasciitisNuchal-type fibroma M>F 20-50Odontogenic myxoma F>M YoungOlfactory neuroblastoma M=F 40-60Ossifying fibromyxoid tumor of soft parts M>F 50Pacinian neuromaPalisaded encapsulated neuroma M=F

Palmar fibromatosis M>F >30Papillary endothelial hyperplasiaParachordoma / mixed tumor / soft tissue myoepithelioma M=F 10-40Paraganglioma - Carotid body paraganglioma M>F 40-60 - Jugulotympanic paraganglioma F>M 40-50 - Vagal paraganglioma F>M - Mediastinal paraganglioma M=F >40 - Retroperitoneal paraganglioma M=F 30-45Paraganglioma-like dermal melanocytic tumorPelvic lipomatosis M>F 20-40Penile fibromatosis M 45-60Perineurioma - Intraneural perineurioma Young - Extraneural perineurioma M=F Adults - Sclerosing perineurioma M>F Young - Reticular perieuriomaPerivascular epithelioid cell neoplasm (PEComa) F>M 30-40 - Angiomyolipoma F>M 40s - Lymphangiomyoma / lymphangiomyomatosis F>>M 40sPhosphaturic mesenchymal tumorPlantar fibromatosis M=F AnyPleomorphic fibroma of the skin F>M 40sPleomorphic hyalinizing angiectatic tumor of soft parts F>M 50sPleomorphic liposarcoma ElderlyPlexiform fibrohistiocytic tumor 0-30Polymorphous hemangioendotheliomaPolyvinylpyrridoline (PVP) granulomaProliferative fasciitis M=F 40-70Proliferative myositis M=F 45-65Proteus syndrome M=FPyogenic granuloma M=F AllReactive nodular fibrous pseudotumorReticulohistiocytoma (solitary) M>F AdultsReticulohistiocytosis (multicentric)Rhabdomyomatous mesenchymal hamartoma M>>F 0-1Rhabdomyosarcoma M>F 0-25 - Embryonal rhabdomyosarcoma (ERMS) 0-10 - ERMS, spindle cell type M>F Young - ERMS, botryoid type - Alveolar rhabdomyosarcoma 10-25 - Pleomorphic rhabdomyosarcoma M>F >45 - Sclerosing rhabdomyosarcoma M~F 18-50Rosai-Dorfman disease (extranodal)Schwannoma M=F 20-50Schwannomatosis M=FSclerosing epithelioid fibrosarcoma M=F WideSilica reactionSpindle cell hemangioma YoungSpindle cell/pleomorphic lipoma M>F 45-60Spindle cell liposarcomaSteroid lipomatosis VariesSymmetric lipomatosis M>F AdultsSynovial chondromatosis

Synovial hemangioma YoungSynovial sarcoma M>F 15-40Traumatic neuromaTumoral calcinosis M=F YoungVascular transformation of lymph nodesVenous hemangiomaWell-differentiated liposarcoma M=F 50-70 - Lipoma-like well differentiated liposarcoma - Sclerosing well differentiated liposarcoma - Inflammatory well differentiated liposarcoma - LipoleiomyosarcomaWell-differentiated papillary mesothelioma F>M 20-40Xanthoma

Location Positive IHC and special stainsAbdominal wall

Fingers, hands, toes, feet, ankles Vimentin; +/- CD68Genital tract, rarely others CK, calretinin, D2-40, WT1, CK5/6Head/neck (pharynx, oral cavity) Desmin, MSA, +/- focal SMAVulva, inguinal region, scrotum Vimentin, desmin, MSA, SMALower extremities, head/neck TFE3, +/- S-100; PAS-D (crystals)Anywhere Congo redForearm, trunk, upper arm CD31/CD34 (vascular component)

Arms, legs +/- CD68, desmin, CD99

Vulva, vagina Desmin, ER, PRExtremities, head, trunk, oralSkin, superficial soft tissue, otherHead/neck (scalp, forehead), leg CD31, CD34, thrombomodulinExtremities (arm)BreastLeg, trunk, arm, head/neck CD31, vWF, +/- keratinAbdomen, breast skin

Arms, legsHead/neck (nose, cheek, ear)BreastSkin, organs, soft tissue Warthin-Starry (organisms)Palm, fingers, feetExtremities, trunk +/- SMA, MSA, desmin, CD34Heart ventricles MSA, desmin, +/- HMB-45Upper portion of body

Inguino-scrotal, vulvo-vaginal CD34, +/- SMA, desmin, ER, PRTrunk, extremitiesExtremities, limb girdle PAS, Oil red O, S-100, CD68Foot, ankle, knee, thigh, hand S-100, HMB-45, Melan-A, NSESmall bowel, stomach, colon

CD68Genital region/nipples; elsewhere SMATrunk, legs, head/neck Vimentin, CD34; Alcian blueRetroperitoneumTrunk, legs, arms CD34, apolipoprotein D

Shoulder, arm Focal SMA and MSAHead, neck S-100, clusterin; +/- MiTFAbdominal cavity, pelvis CK, vimentin, desmin, carboxy WT1Extremities, trunk, head/neckPleura, peritoneum, pericardium CK5/6, calretinin, WT1, D2-40Subscapular area Elastic stainsMediastinumSolid organs EBER, SMASkinSoft tissue, lung, liver, bone, skin CD31, CD34, vWF

Head/neck, earHand, fingers, forearm, wrist, knee CK, EMA, vimentin; +/- CD34Pelvis, perineum, genital tract +/- desmin, SMAExtremities CK, vimentin, CD31, FLI1Shoulder, chest/back, thigh B-catenin, SMA, MSAScalp, vertebral axisOmentum, mesentery CKIT, CD34, +/- SMAFingers, hands, toes, feetUpper thigh, buttock, arm, shoulder CD99, FLI1, S-100, synaptophysinOrbit, head/neck, thigh, legs S-100 (cartilage); NSE, CD99, Sox9Thigh, popliteal fossa, limb girdle Vimentin; +/- focal, weak S-100Thigh, pelvis, shoulder, RP OsteocalcinSacrococcygeal region

Desmin, MSAHead/neck (auricular area)Head/neck (orbit, tongue, palate)

Fingers, hands SMA, MSA, CD68

Spreads throughout bodySternocleidomastoid muscleThigh, knee, arms, trunk Vimentin; maybe focal SMA, MSAAxilla, upper arm, thigh Actin, +/- CD34Arms, legs Factor XIIIa; +/- desmin, SMMS

Ankle

SkinDorsal wrist, fingers, foot, toesPosterior mediastinum, RPHead/neck, other CD34, nuclear B-catenin, +/- CD99Vagina, vulva, cervix, male tract Desmin, MSAThigh, inguinal region, chest CD34

CD68, SMA, TRAP

Knee, ankle/foot, wrist, fingersFingers, toes, ankle/foot, knee CD68, HAM56; CD45 (giant cells)

GingivaScalp, other GFAPHand, forearm

Finger, palm, wrist, forearm, organs Actin, +/- desmin; type IV collagenActin, type IV collagen

AFBDermis, subcitus, muscle, organs PAS (granules), S-100, NSE, CD68Gingiva negative for S-100Nerves

Nasal cavity SMA, MSA; +/- desmin, CD34Thigh, pelvic fossa, RP, pleura CD34, CD99; +/- bcl2, EMA, actinFoot, ankleFace, lips, oral mucosa, tongueThigh, shoulder, back, neck, chest S-100, Oil red OSubcutis, dermis Fite-FaracoExtremities CD31, VEGFR3, D2-40Distal extremities CD34, CD31, vWF, VEGFR3RetroperitoneumFingers, toes Actin; trichrome for inclusion bodiesHead/neck, shoulder, upper armFoot, ankle, hand, wrist +/- SMA, MSA

SMA, desmin; - myoglobinOral cavity, subcutisLung, mesentery, omentum SMA, MSA; +/- desmin, ALKMostly GU organs SMA, MSA, desmin, CK, +/- ALKPelvis, mesentery B-cateninMuscles of lower extremity (thigh)Thigh, shoulder, upper armThigh, shoulder, buttocks, upper arm Vimentin; Alcian blue (background)Groin, submandibular Actin, vimentinUterus, can reach heartShoulder, chest Focal actin, CD68Head/neck, parotid VEGF, CD31, vWF, SMA, GLUT1Head/neck, fingers, trunk Hyaline material: PAS, Alcian blueHead/neck, trunk CD68, CD31, factor XIIIa, A1ATKnee, shoulder, elbow, hip, ankle

CD31, LANA1 (HHV8), VEGFR3Extremities (legs, arms), organs

Lines of cleavage, mucosa, organsSuperficial and deep soft tissue CD31, CD34, FLI1, D2-40Face, shoulders VimentinKnuckles

Extremities; retroperitoneum ER, PR (gynecologic type)Peritoneum

SMA, MSA, +/- desmin, caldesmonRetroperitoneum, abdomenLower extremitiesExtremities (extensor surfaces)IVC, pulmonary artery, otherExtremities, head/neck, trunkUpper back, neck, extremities

Knee, shoulder, hip, elbowWrist, hand, ankle, footMedial extremitiesLegs/thigh, chest wall Focal SMA, MSA; +/- desmin, CKLegs/thigh Rounded cells: S-100, NSELumbosacralHead, neck, axilla, pectoral, other VEGFR3, D2-40

+/- Factor VIII-related antigenMultiple CD31GU organs, retroperitoneum PAS-DParaspinal region, neck, others Vimentin, EMA, CK, INI1 lossLegs, thigh, arms, retroperitoneum +/- focal keratin, desmin, NF

Retroperitoneum +/- CD68

Sciatic nerve, brachial plexus +/- S-100 (focal), nestin, Leu-7, p53Breast, other Desmin, actin, CD34Jaw, others CK, HMB-45; NSE, synaptophysinMidline/autonomic nerves S-100, HMB-45Metatarsals (3rd-4th, 2nd-3rd)Lips, mouth, eyelid, intestinesUterus, cul-de-sac, bladder surface CK, EMA, calretinin, CK 5/6QuadricepsLymph nodes, other AFB, CD68, S-100; +/- desminAdrenal, rarely otherHead/neck, trunk ActinHead/neck SMA, calponin, +/- desmin, MSARetroperitoneum, abdomen SMA, desmin, trichromeLower extremities SMA, h-caldesmon; +/- desminLimbs, head, neckDigits Vimentin, actinExtremities +/- SMA, MSA

Extremities, thigh, popliteal area

Nasopharynx CD31, CD34, SMA, AR, B-cateninExternal popliteal nerveHand (left > right), wrist, forearmSympathetic ganglia, incl. adrenal NSE, NF, S-100, synapto, chromo

No site predilection S-100 (focal)

Head/neckMelanin markers (pigmented cells)

Brachial and sciatic nerve trunksHead, neck, shoulder S-100, PGP9.5

negative for S-100Mid-forehead, eyelid, nape of neckPelvic girdle (in multifocal cases)Arm, trunk SMA, MSAScalp/skullArm, head/neck

Nuchal area CD34, nuclear B-catenin, +/- CD99Tissue around maxilla or mandible CK19Sinonasal (nasal vault) NSE, CD56, synapto/chromoExtremities, trunk, head/neck Vimentin, S-1000; +/- Leu-7, NSEDigitsFace

Palms Focal B-catenin; +/- SMA, MSAHead, neck, fingers, trunkThigh, calf, upper arm, forearm CK8/18, EMA, S-100

Carotid artery bifurcation NSE, NF, synapto/chromo; S-100Temporal bone/middle earHead/neck (around vagus nerve)Pulmonary artery, aortic archRetroperitoneum

Perirectal, perivesicalPenis

Upper extremity EMATrunk, extremities, deep tissue EMA, claudin-1, GLUT1Hand EMA, GLUT1; +/- SMA, MSA

Uterus, soft tissue, viscera, bone HMB-45, Melan-A, SMA, +/- desminKidney, soft tissue Actin, desmin, HMB-45, Melan ALung, mediastinum, retroperitoneum HMB-45

FGF23Plantar aponeurosis SMAExtremities, trunk MSA, +/- CD34 and CD99Ankle/foot, lower leg, thigh, perineum Vimentin, CD34, factor XIIIaRetoperotineum, extremities +/- keratin, actin, desmin, S-100Arms, legs CD68, SMA

Liver, spleen, lymph nodes Congo red, mucicarmine, ironArm, trunk SMA, MSATrunk, shoulderVariesGingiva, fingers, lips, face, tongueMesentery Actin, desmin, CD117Anywhere CD68, SMA; +/- A1AT, lysozyme

CD68, A1At, lysozyme, PAS-DFace/neck (chin)Head/neck, GU tract, RP MSA, desmin, myogenin, myoD1Head/neck (orbit, parameninges)Paratesticular, head/neck MSA, desmin, titin, troponin DVagina, bladder, nasopharynxExtremities, head/neck MyogeninExtremities (thigh), RP, chest MSA, desmin, myoglobin, myoD1

MyoD1; focal myogenin, desminS-100; +/- CD1a

Head, neck, flexor surfaces S-100, Leu-7, GFAP

Legs, limb girdle, trunk EMA; rarely S-100, NSEInguinal region, abdominal wall PAS-DDistal extremities (hand)Posterior neck, shoulder, back CD34, Bcl-2

Neck, cheeks, breast, arm, axillaJoints (knee, hip, elbow, shoulder)

Para-articular regions of extremities CK7, CK19, EMA, TLE1, CD99, bcl2

Near joints: hip, shoulder, elbowLymph nodes (axilla)

Extremities, retroperitoneum MDM2, CDK4

Groin, retroperitoneumRetroperitoneum

Omentum, mesentery, pelvisDepends on type

Molecular

Inheritance linked to EDR, ENG, FLT4?

12q13-15 (HMGA2) mutationt(X;17) TFE3-ASPL

t(12;16) ATF1-FUS or t(12;22) ATF1-EWSR1

Possible link to CMC1 on 5q

t(11;16)t(12;16) EWS-ATF1t(12;16) EWS-ATF1

Germline 1q43 mutation (link to HLRCC)PRKAR1A mutation (Carney complex patients)MDM2, CDK4t(17;22) COL1A1-PDGFB

t(11;22) EWS-WT1

Rearrangements of 22q11 (INI1), 8q, 18q11Aberration of 22q11 (INI1)

KIT, PDGFRA

t(11;22) EWS-FLI1; t(21;22) EWS-ERG; others

t(9;22) EWS-NOR1 or t(9;17) RBP56-NOR1

PTCH

Possible mutations at 4q27-31 or 17q21-22

t(17;22) COL1A1-PDGFB

t(1;2) COL6A3-CSF1t(1;2) COL6A3-CSF1

Truncating glomulin mutation at 1p21-22

HHT1 on 9q, HHT2 on 12q11q13-21 (GARP?) rearrangement

t(12;15); + 8, 11, 17, 20

+/- ALK+/- ALK

GNAS1 mutations

Nonrandom X-inactivation patterns

8q11-13 (PLAG1) rearrangement12q13-15 (HMGIC) aberrations, others

t(7;16) FUS-CREB3L2t(7;16) FUS-CREB3L2

Aberration of 22q11 (INI1)various gains, losses

NF1, other gains/losses

t(12;16) FUS-CHOP; rarely t(12;22); p53

B-catenin

del(1p); MYCN amplification in some

NF1

CMC1 on 5q in some lesions11 deletion (in Michelin tire baby syndrome)

Germline mutation in SDH

Can have TSC1 and TSC2 deletions

PTEN in some patients

LOH at 11p15.5; trisomy 8

t(2;13) PAX3-FKHR; t(1;13) PAX7-FKHR

Mutated gene on chromosome 22+/- FUS

loss of 16q, 13q

t(X;18) SYT-SSX1, SYT-SSX2, or SYT-SSX4

GALNT3 or FGF23 mutation (familial cases)

VMCM1 mutation on chromosome 9Giant and ring chromosomes (12q13-15)

Behavior / NotesBenign; linked to pregnancy, FAP, or trauma; typically 3-10 cm; can recur locallyBenign; may be same lesion as kaposiform hemangioendotheliomaIntermediate; has a predilection to recur; metastases rareBenign; usually < 2 cm; usually solitaryBenign; can be multifocal; does not regress; can recurBenign but locally aggressive; can exceed 20 cm; recurs oftenMalignant; grows slowly; poor prognosis; metastasizes early; age and tumor size prognosticBenign; can be associated with hematopoietic neoplasms, other conditionsBenign; subcutaneous; painful to palpation; usually multiple; rarely > 2 cm

Intermediate; anemia, pyrexia, weight loss; rarely recurs or metastasizesBenign; causes pain and swelling; almost always recursBenign; overlaps with other benign genital stromal tumors; can rarely be malignantBenign; usually < 2 cm; half of cases cause pain; recurrence very rareMalignant; behavior varies based on site and etiology; linked to many possible factorsMalignant; most common angiosarcoma subtype; metastasizes often; size is prognosticMalignant; usually in post-mastectomy patients; metastasizes oftenMalignant; rapidly growing; often metastatic at time of presentation; grade is prognosticMalignant; often syndrome-associated; can be large; often recurs or metastasizesMalignant; develop 3-5 years after radiation; often recurs or metastasizesBenign; acquired in altered physiologic statesBenign; more a clinical than a pathologic diagnosisIntermediate; < 2 cm; low rates of recurrence, metastasisBenign/intermediate; solitary; < 2 cm; secondary to radiation; recurrence rareBenign; < 1 cm; usually develop within 3 years of radiationBenign; caused by Bartonella infection in immunocompromised patientsBenign; slow-growing, < 3 cm, gritty; high recurrence rateBenign; 3-5 cm; gritty; can recurBenign; often multiple; can cause cardiac symptoms; regress; linked to tuberous sclerosisBenign; large, can be deep; do not regress; linked to Kasabach-Merritt, other syndromesBenign; solitary, well-demarcatedBenign; overlaps with angiomyofibroblastoma-like tumor of male genital tractBenign; few millimeters in size; may be linked to infection or chemical exposureBenign; slow-growing; 1-11 cmMalignant; 2-6 cm; can be grossly pigmented; size prognostic; often recur or metastasizeMalignant; often metastasizeBenign; linked to monoclonal immunoglobulin productionBenign; pilar type 1-2 cm, painful, can be multifocal; genital type solitary and rarely painfulBenign; linked to Carney's complex; usually 1-5 cm; often recurMalignant; often recurs or metastasizes; site is most important prognostic factorIntermediate; slow, persistent growth; high rate of recurrence; metastases uncommon

Benign; slow-growing, < 4 cmMalignant; nodal spread rare; often recurs; may have better prognosis than typical melanomaMalignant; poor prognosis; often metastasizes widelyBenign; linked to osseous hypertrophy, tuberous sclerosis; tends to recurMalignant; symptoms depend on location; linked to asbestos, SV40; dismal prognosisBenign; often history of manual labor; often bilateral (subclinically?)1 reported caseBenign(?); seen in immunocompromised patients; half of patients have multiple lesionsBenign; may be variant of epithelioid hemangiomaIntermediate; can arise in vessels; rarely multifocal; low rate of recurrence or metastasis

Benign; reactive?; patients can have peripheral eosinophilia; can recur; rarely regressMalignant; typically 3-6 cm; possible trauma link; often recurs multiple times or metastasizesMalignant; possibly linked to malignant extrarenal rhabdoid tumorIntermediate; can recur or metastasizeBenign; 5% multicentric; usually 5-10 cmBenign; two types depending on origin and siteBenign or malignant (criteria: cellularity, mitoses, necrosis); linked to Carney triad, NF1Benign; slow-growing; painless; solitary; < 3 cm; can recurMalignant; typically in Caucasians; usually 5-10 cm; often metastasizes; prognosis improvingMalignant; grows rapidly; metastasizes oftenMalignant; slow-growing; late recurrence and metastasis commonMalignant; 5-10 cm; related to radiation, potentially trauma; recurs and metastasizes oftenIntermediate; link to spina bifida and similar abnormalities; can metastasizeBenign; associated with nevoid basal cell carcinoma syndrome; does not regress

Benign; grows slowly over several years, < 2 cm, can recur

Benign but progressive,debilitating, and typically fatal within 10-15 years; can be familialBenign; causes torticollis; 1-3 cm; grows rapidly; may spontaneously regress; does not recurMalignant; slow-growing, painless, 3-8 cm; recurs, metastasizes; possibly injury-relatedBenign; solitary; grows rapidly (3-5 cm); does not regress; may recurBenign, possibly reactive; 1/3 multiple; can recur; metastases extremely rareBenign; can evolve rapidly; higher risk of local recurrenceBenign; higher risk of local recurrence

Benign; larger than normal fibrous histiocytomaBenign; secondary to intussusception and prolapseBenign; incidental findingBenign; half of cases linked to trauma; typically 1.5-2.5 cm; recurrence rareBenign; ganglioneuromatous polyposis can occur in NF1 and MEN IibBenign; linked to Gardner syndrome; may recur; see nuchal-type fibromaBenign; usually < 3 cm; does not regressIntermediate; painless nodule; 1-8 cm; can recur; not known to metastasize; link to DFSPIntermediate; can recur; not known to metastasize

Benign; cause pain, tenderness, joint symptoms; unencapsulated; often recursBenign; slow-growing; circumscribed; may be reactive or neoplastic; can recurMalignant; can recur or metastasizeBenign; many forms, some linked to syndromes; tends to recur; can be familialBenignBenign; malformation; not painfulBenign; deep, extensive, painful lesionsBenignBenign; seen in POEMS syndrome and multicentric Castleman's diseaseBenign; usually solitary; usually < 1 cm; causes sudden, radiating pain; infrequently recurMalignant; can metastasizeIndeterminate; follow-up lacking on known examplesBenignBenign; linked to surgical traumaBenign; typically < 3 cm; poorly circumscribed; can be multifocal; very rarely malignantBenign; typically 1-2 cm; often regressBenign; often painful

Benign; can cause nasal obstruction or epistaxis; can be locally aggressiveIntermediate; can cause hypoglycemia; malignant examples can recur, metastasizeBenign; can recur; possible link to pleomorphic hyalinizing angiectatic tumor of soft partsBenign; lesions frequently hemorrhageBenign; slow-growing; 5-15 cm; has prominent vascularity; does not recurBenign; linked to sulfone-treated lepromatous leprosy; expansive subcutaneous nodule

Intermediate; can recur; lymph node metastases uncommonBenign; obstructs ureters or aorta; sometimes due to drugs or malignancyBenign; < 2 cm; many recur; regresses; X-linked dominant inheritance?Benign; usually asymptomatic; can become large; may recur; possible trauma linkMalignant, can recur/metastasize; good prognosis; 1/3 present at birthMalignant, can metastasize; very few reported casesBenign; rarely multiple; may regress; can recur or rarely metastasizeUsually benign; 5-10 cm; can be multiple; can cause anemia, elevated ESRBenign; often history of trauma; relationship to IMT?Benign; often > 10 cm; some mesenteric cases linked to Gardner's syndromeBenign; often painful; can impair local function; can recurBenign; can be greater than 20 cmBenign; painless, fluctuant; linked to Mazabraud syndrome; typically 5-10 cmBenignBenign; associated with recent pregnancy, enlarged uterus, pelvic pain; can recurBenign; mass overlying bone in debilitated patients (< 6 months)Benign; type of capillary hemangioma; one-fifth of cases multiple; enlarge, then regressBenign; multiple; can be up to 10 cm; do not regressBenign; often multiple; usually regress spontaneously; can occur in adulthoodBenign; usually 2-6 cm; recurrence not uncommonMalignant; caused by HHV8; can be aggressive; prognosis depends on numerous factorsMalignant; prevalent in Poland, Russia, Italy, parts of Africa; linked to immunocompromiseMalignant; heterogeneous disease; seen in children with bulky lymphadenopathyMalignant; typically develops several months after a renal transplantMalignantIntermediate; associated with Kasabach-Merritt phenomenon; rarely metastasizesBenign; induced by injury, trauma, or infection in dark-skinned patientsBenign; often asymptomatic; linked to palmar and plantar fibromatosis

Benign; often calcified; can be > 10 cm; must be distinguished from leiomyosarcomaBenign; linked to pregnancy; nodules can be several cm; can regress or recurMalignant; prognosis varies by siteMalignant; usually > 5 cm; aggressive local extension and metastasesMalignant; often vessel-associated; can recur and metastasizeMalignant; usually < 2 cm; excellent prognosis; can recur but infrequently metastasizeMalignant; causes site-based symptoms; worse prognosis in vena cava lesionsBenign; may grow slowly or quickly; usually 3-5 cm; can recur in diffuse diseaseBenign; slow-growing, soft mass; rarely > 10 cm; can be multiple or familial; may recur

Benign; causes effusions, joint swelling; possibly a reactive processBenign; often bilateral; can cause local symptomsBenign; patients morbidly obese; overlying skin thickened, hyperkeratotic; can recurMalignant; slow-growing, painless, 1-18 cm; recurs often; can metastasize after decades

Benign; causes myelopathy or radiculopathy in 2/3 of cases; imaging is essentialBenign; can wax and wane; in utero cases linked to Turner, other syndromesBenign; spontaneous in children, follows injury/irradiation in adults; may recur

Benign; patients can have multiple bone lesions; prognosis depends on disease extent

Malignant; typically < 5 cm; poor prognosis; often metastasizes widelyMalignant; can grow slowly or quickly; 5-10 cm; recurs and metastasizes oftenMost common subtypeBetter prognosis than storiform-pleomorphic type; recurs more than metastasizes

Fever, leukocytosis; grossly yellow; may be dedifferentiated liposarcomaMalignant; term is variably applied and does not refer to a lone entity; not a WHO diagnosisMalignant; roughly half of cases associated with NF1; large; often recurs, metastasizesBenign; can be bilateralIntermediate; locally destructive; can increase VMA; often recurs; can metastasizeIntermediate; link to Carney syndrome; heavily pigmented; can metastasizeBenign; non-tumorous fibrosing process secondary to wearing high heelsBenign; link to MEN Iib; usually multipleBenign; causes nonspecific symptoms; can recur; can uncommonly lead to patient deathBenign; congenital or acquired (injection-related); severely affects movementBenign; immunocompromised patientsBenign; usually 3-7 cm, but can be giantBenign but may cause organ failure; multicentricity more common; regressMalignant; slow-growing, painless, 1-12 cm; recurs, rarely metastasizesBenign; can be up to 15 cm if deepBenign; solitary; painless; can be intravascular; very rarely recur or metastasizeBenign; self-limiting; may regress; usually related to traumaBenign; painful, localized; often related to traumaMalignant; often subcutaneous; grade influences behavior; recurs more than metastasizes

Malignant; often metastasizes; age, round cell areas, and necrosis worsen prognosisMalignant; significant round cell areas imparts worse prognosis in myxoid liposarcomasBenign; facial deformity, epistaxis; may recur destructively; linked to FAP?Benign; degenerative processBenign; slow-growing; causes compression neuropathy; can be seen with macrodactylyMalignant; symptoms vary; urine catecholamines increase; detailed classification systems

Benign; not associated with neurofibromatosis 1; slow-growing, painlessBenign but can have malignant transformation; pathognomonic of neurofibromatosis 1

Benign; causes neurologic symptomsBenignEntity contestedBenign; very common; typically regress; can be indicative of many syndromesBenign; can be multiple or solitary; typically does not recurBenign; reactive; rapid growth (1-2 weeks), slight pain, solitary, < 2 cmRelated to birth trauma?

Benign; linked to diabetes; may recur; see Gardner fibromaBenign but can be locally destructive; recurrence commonMalignant; causes local symptoms; late recurrence and metastasis commonIntermediate; typically 3-5 cm; usually benign, but can recur or metastasizeBenign; linked to trauma; typically painful; Pacinian structures are typically > 1.5 mmBenign; not linked to syndromes

Benign; 50% bilateral, can impair use of hand; linked to diabetes, alcoholBenign; small (average 2 cm); exuberant form of organizing thrombus; sometimes recursIntermediate; can be histologically malignant; can recur, sometimes late; metastases rareUsually benign; cause hypertension if functional; can be familial; uncommonly metastasizes

Occasionally multifocal or metastaticCan occur in patients with Carney's triad; higher rate of recurrence, metastasis, and deathCan be multiple and/or occur in patients with Carney's triadBenignBenign; causes local genitourinary symptoms; can lead to recurrent DVTBenign; causes penile curvature; linked to palmar and plantar fibromatosisBenignBenign; causes muscle weaknessBenign; circumscribed; can reach 20 cm; rarely recursBenignBenignIntermediate; rarely linked to tuberous sclerosis complex; can recur or metastasizeBenign; often associated with tuberous sclerosis complex; can involve lymph nodes benignlyBenign; associated with tuberous sclerosis complex; can cause severe pulmonary symptomsIntermediate; causes osteomalacia, phosphaturia, hyperphosphatemia; can metastasizeBenign; 33% bilateral; linked to palmar fibromatosis, diabetes, alcoholBenign; 0.5-2 cmIntermediate; grows slowly; recurrences common; does not metastasizeMalignant; high recurrence and metastasis rate; age, size, and location affect outcomeIntermediate; 1-3 cm, ill-defined; recurrences frequent; metastases rareNo longer a distinct entity per the WHO

BenignBenign; rapid growth (3 weeks), usually < 3 cmBenign; sporadic; wide range of manifestationsBenign; type of capillary hemangioma; reactive?; one-third occur after trauma; can recur

Benign; rarely multiple; recurrence uncommonBenign; waxes and wanes; can be paraneoplastic; weight loss, crippling arthritisBenign; usually < 2 cm; does not regress; linked to congenital anomalies

Malignant; intermediate prognosis among rhabdomyosarcomaMalignant; superior prognosis among rhabdomyosarcomasMalignant; superior prognosis among rhabdomyosarcomasMalignant; poor prognosis among rhabdomyosarcomasMalignant; rapidly growing, large (> 10 cm), painless; metastasizes early; poor prognosis

Benign; may be associated with lymphadenopathy; can resolve spontaneouslyBenign; typically sporadic, but linked to NF2; rarely undergo malignant transformationBenign; patients have multiple painful schwannomasMalignant; 5-10 cm; may recur or metastasize; link to LGFMS?Benign; exuberant reaction to large amount of injected silica (polarizable)Benign; may be multifocal; usually asymptomatic; often recur; linked to Mafucci syndromeBenign; slow-growing; rarely multiple or familial; can reach 14 cm

Benign; may be due to endogenous or exogenous steroidsBenign; linked to alcoholism and liver disease; causes neuropathy; can be familialBenign

Benign; may be diffuse; may be trauma-relatedMalignant; often recurs or metastasizes, though rates lowering due to better treatmentBenign; non-neoplastic response to injury; typically < 5 cm; can be painfulBenign; often multifocal; can be familial; slow-growing; 5-15 cm; calclium level normalBenignBenign; malformation; can be superficial or deep; may be linked to cavernous hemangiomaMalignant; slow-growing, often large; recur but do not metastasize; can dedifferentiate

Benign if localized; aggressive if widespread (can metastasize); possible link to asbestosBenign; reactive, seen in hyperlipidemic patients; asymptomatic in and of themselves

Subtypes

verrucous hemangioma

Pilar, genital

Epithelial, sarcomatoid, mixed

Usual type, fibroma-like

Fibrochondroma, osteochondroma, myxochondroma, chondroblastoma-like

Oncocytic

Lipomatous, meningeal, with giant cells

Typical; myxoid; lipoma-like; spindle cell

Retiform, Dabska-type

Pelvic, mesentericCapillary type, cavernous type

Somatic, gynecologic

Usual, myxoid, inflammatory, granular cell

Subcutaneous, deep; sometimes termed based on site

Head/neck type, intra-abdominal type, cutaneous type

Malignant Triton tumor, glandular, epithelioid, superficial epithelioid

Epithelioid neurofibroma

Port-wine stain

Subcutaneous, intramuscular, fascial types

Nasopharyngeal, laryngeal, orbital, gangliocytic, of cauda equina, cardiac

Epithelioid

Traditional, small-cell/epithelioid (not official terms)

Pregnancy-related (granuloma gravidarum) ; intravenous

Embryonal rhabdomyosarcoma with anaplasia

Clear cell rhabdomyosarcoma

Ancient, cellular, plexiform, epithelioid

Biphasic, monophasic fibrous, monophasic epithelial

Eruptive, tuberous, tendinous, xanthelasma, plane