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Bone And Soft Tissue > Soft Tissue > Flashcards

Soft Tissue Flashcards

1
Q

Lipoma molecular

A

FISH: absence of MDM2 amplification
Rearrangements in HMGA1 and HMGA2

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2
Q

A 25 year old male came in due to a painful right forearm mass. Mass was excised and microsections show mature adipocytes, branching vascular network and fibrin thrombi.
Dx?
Why painful?
Molecular?

A

Angiolipoma
Fibrin thrombi
PRKD2 mutations

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3
Q

A 50 year old man came in due to a shoulder and back mass. Excision was done. Microsections show mature adipocytes with bland spindle cells and ropey collagen with a variable myxoid background and mast cells.
IHC: (+) CD34
Dx?
Ddx?

A

Spindle cell lipoma
If predominantly spindle cells, may mimic DFSP
Pleomorphic lipoma: + floret cells

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4
Q

A 35 year old female came in due to right upper arm mass. Excision was done. Microsections show nests and cords of lipoblast-like and chondrocyte-like vacuolated cells on a chondromyxoid background. There is no atypia, mitoses or necrosis
Dx?

A

Chondroid lipoma

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5
Q

A 55 year old man came in due to right retroperitoneal mass adjacent to kidney. Resection done. Microsections show mature adipocytes with normal marrow elements and an adjacent adrenal parenchyma.
Dx?
Mc location?

A

Myelolipoma
Adrenal medulla

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6
Q

A 2 year old boy was brought to the clinic due to an incidental finding of a soft tissue mass. Excision was done. Microsections show immature adipocytes searated by connective tissue that has loose myxoid appearance.
Adipocytes are seen at various points of maturation including spindle cells
Dx?

A

Lipoblastoma

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7
Q

A 35 year old man came in due to right thigh mass. Excision done. Microsections show organoid arrangement of adipocytes with granular and vacuolated cytoplasm. There are also delicate branching capillaries
(+) S100
Dx?

A

Hibernoma
Can produce steroids

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8
Q

Mc form of liposarcoma
Subtypes?
Prognosis?
Molecular?

A

Atypical lipomatous tumor (well-differentiated liposarcoma)
Lipoma-like - MC
Sclerosing- MC in paratesticular or retroperitoneal
Inflammatory
Mixed
No metastatic potential but recurs locally and can de-differentiate
FISH: MDM2 and CDK4 amplification (can use real time PCR)

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9
Q

A 50 year old man came in due to a retroperitoneal mass. Resection was done. Microsections show one area with bland spindle cells and an area with pleomorphic cells, spindle cells and MNGCs with entrapped lipoblasts and a BG of delicate branching vessels
Dx?
Prognosis?
Molecular?

A

Dedifferentiated liposarcoma
Better prognosis than other high grade sarcomas
Metastasizes in 15-20% of cases and recurs in 40-50%
MDM2 and CDK4 amplification

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10
Q

A 35 year old man came in due to a thigh mass. Resection was done. Microsections show proliferation of bland monomorphic stellate and fusiform cells in a background of myxoid matrix and chicken-wire vessels. Some lipoblasts are present. In some areas, round cells are also seen.
Dx?
Behavior?
Prognosis?
Molecular

A

Myxoid liposarcoma
Round cells : more primitive, higher grade
Metastasizes in 10-60% of cases
FUS-DDIT3

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11
Q

A 57 year old man came in due to a retroperitoneal mass. Microsections show pleomorphic cells (bizarre, giant), lipoblasts, mitoses, necrosis
Dx?
Molecular?
Variant?

A

Pleomorphic liposarcoma
Complex karyotype (negative MDM2 and CEK4)
Epithelioid variant

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12
Q

A 9 year old girl came in due to neck mass. A subcutaneous mass was seen and excised. Microsections show bland stellate/spindle cells with a tissue culture pattern and extravasated RBCs in stroma.
Ihc: (+) SMA, Calponin
(-) Desmin
Dx?
Behavior?
Molecular?

A

Nodular fasciitis
Benign, self-limiting, rapid growth
MYH9-USP6

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13
Q

A 51 year old male came in due to a rapidly growing forearm mass. Microsections show a tissue culture pattern with ganglion-like cells. There are extravasated RBCs and arborizing vascular network.
IHC: (+) SMA
GANGLION CELLS —> vimentin
dx?
Behavior?

A

Proliferative fasciitis
If in muscle, proliferative myositis
Benign

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14
Q

A 60 year old bedridden man had a coccygeal mass. Excision was done: microsections show fibroblasts with degenerative changes (ganglion-like) with areas of zonal fibrinoid necrosis and prominent myxoid areas. Also seen are ectatic, thin-walled vessels.
Dx?
RF?

A

Ischemic fasciitis
In immobilized individuals

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15
Q

A 67 year old previous tennis player came in due to a subscapular mass. Microsections show thickened and fragmented eosinophilic fibers, collagenous stroma and variable fat
(+) elastin stain
Dx?
RF?

A

Elastofibroma
Repetitive physical movements

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16
Q

A 36 year old man came in due to a palpated nape mass. Microsections show paucicellular fibrocollagenous proliferation composed of patternless collagen bundles separated by clefts (artifactual separation). Also seen are entrapped nerves, adenxal structures, vessels and fat.
Ihc: (+) CD34
(-) SMA, Desmin, Beta catenin
Dx?

A

Nuchal-type fibroma

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17
Q

A 9 year old boy came in due to back mass. Hx revealed that patient also has multiple colonic polyps and previous epidermoid cysts and osteomas. The mass was excised. Microsections show paucicellular fibrocollagenous proliferation composed of patternless collagen bundles separated by clefts (artifactual separation). Also seen are entrapped nerves, adenxal structures, vessels and fat.
Ihc: (+) Beta Catenin
Dx?
Molecular?
Syndrome?

A

Gardner-type fibroma
(+) beta catenin vs nuchal type fibroma
APC gene in FAP
Gardner syndrome

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18
Q

A 55 year old man came in due to a finger mass. Microsections show proliferation of fibroblasts and myofibroblasts with abundant granular eosinophilic to amphophilic cytoplasm. Also seen are slit-like spaces at the peripheral edge lined by flat endothelial cells.
(+) focal SMA
(-) desmin
Dx?
Behavior?
Molecular?

A

Fibroma of tendon sheath
Benign
USP6

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19
Q

A 7 year old bay was brought to the clinic due to a palmar mass. Microsections show bland ovoid to spindled fibroblasts in a BG of collagenous stroma with variable cellularity. Also seen are scattered calcifications and cartilaginous foci rimmed by OLGCs
Dx?
Prognosis?

A

Calcifying aponeurotic fibroma
50% recurrence

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20
Q

A 29 year old male came in due to a plantar mass. He has a hx of colonic polyps. Microsections show infiltrative proliferation of myofibroblasts in sweeping fascicles in a BG of collagen with small vessels
IHC: (+) nuclear beta catenin, SMA
(-) CK and desmin
Dx?
Subtypes
Molecular

A

Fibromatosis - plantar
Palmar
Plantar
Penile (peyronie)
Deep (Desmoid tumor)
Familial/FAP: APC
Sporadic: CTNNB1

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21
Q

A 27 year old female came in due to a soft tissue mass. Microsections show ovoid to spindled/stellate myofibroblastic cells in a loose myxoid background. Also seen are inflammatory cells, predominantly lymphoplasmacytic.
IHC (+) ALK
DX?
molecular?

A

Inflammatory myofibroblastic tumor (IMT)
ALK rearrangments
If ALK negative, test for ROS1, PDGFRB, NTRK3, RET fusions

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22
Q

A 45 year old man due to cough. CT reveal
Multiple lung lesions. Biopsy was done. Microsections show uniform and hyperchromatic fibroblast-like spindle cells with elongated nuclei and variable cytoplasm in a herringbone pattern and intervening collagen fibers.
IHC (+) SMA
(-) s100, cd34, desmin, ema, keratins
Dx
Behavior
Ddx
Ihc

A

Fibrosarcoma (adult type)
Metastasizes most to LUNGS, bone and liver
If subcutis: must be DFSP with fibrosarcomatous transformation
If pleomorphic: must be UPS
Ihc nonspecific

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23
Q

A 9 month old baby boy was brought to the clinic due to a rapidly growing forearm mass. Biopsy was done which showed infiltrative monomorphic spindle cells in sheets and sweeping fascicles. Also seen are hemangipericytoma-like vessels
Most likely dx?
Molecular?
Prognosis?

A

Infantile/congenital fibrosarcoma
ETV6-NTRK3 t(12;15)
Favorable prognosis but recurrence is 50%

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24
Q

MC sarcoma in the elderly population
Prognosis
Ihc molecular

A

Myxofibrosarcoma
Depends on grade
Nonspecific IHC
Complex karyotypes

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25
Q

Microsections of a ST tumor show bland spindle cells with indistinct cytoplasm in small fascicles and whorled patterns and fibrocollagenous and myxoid component. Myxoid foci have arcades of vessels.
(+) MUC4
Dx?
Behavior?
Molecular?

A

Low-grade fibromyxoid sarcoma (LGFS)
Malignant but indolent
FUS-CREB3L2 (MC)

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26
Q

A 55 year old man came in due to right lower leg mass. Microsections show small to Medium-sized epithelioid cells with variable clear to eosinophilic cytoplasm and bland ovoid nuclei in cords surrounded by dense hyalinized and sclerotic matrix with thin collagen strands
(+) MUC4
Dx?
Prognosis
Molecular

A

Sclerosing epithelioid fibrosarcoma (SEF)
Poor, mets to CNS, bone, lung
EWSR1-CREB3L1 fusion

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27
Q

Schwannoma (neurilemmoma)
IHC
Syndromes
Variants

A

(+) s100, sox10, H3K27me
Epithelioid variant: loss of INI1
Retroperironeal, posternior mediastinum, GI: GFAP and CK
MC: multiple meningiomas
NF2, schwannomatosis
Ancient, cellular, plexiform, epithelioid, paeudoglandular, neuroblastoma-like, reticular

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28
Q

MC peripheral nerve sheath tumor
3 components and ihc
Predisposing factors to malignant transformation

A

Neurofibroma
Perineural-like cells: EMA
fibroblasts: CD34
Schwann cells: s100, sox10
With interspersed mast cell
Intact H3K27me
NF1 patients, plexiform and intraneural (MC preursor to MPNST)

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29
Q

Rare benign neoplasm arising from perineural cells
Variants
Ih

A

Perineurioma
Intraneural - onion skinning
Sclerosing
Reticular
Plexiform
(+) GLUT1, claudin, EMA, variable CD34
(-) sma, desmin, s100, ck

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30
Q

A 45 year old black woman came in due to a neck mass. Microsections show polygonal cells with granular eosinophilic cytoplasm arranged in cords, nests, and sheets in a collagenous stroma. The overlying epithelium shows pseudoepitheliomatous hyperplasia.
Ihc: (+) s100, sox10, cd68, TFE3
Dx?
Origin?
Behavior
Em findings?

A

Granular cell tumor
Schwannian
Benign but if malignant, 50% metastasize
EM: rudimentary ICJs; pleomorphic secondary lysosomes

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31
Q

A benign tumor from neural crest composed of monotonous spindled schwann cells with elongated/wavy nuclei arranged in fascicles. Also seen are ganglion cells arranged singly and in small nests.
(+) s100, nf (both), synaptophysin (ganglion)
Dx?
MC location?

A

Ganglioneuroma
Retroperitoneum
Posterior mediastinum

32
Q

A 41 year old amputee came in due to a painful mass on his stump. Microsections of the mass show hyperplastic proliferation of cytologically bland spindled schwann cells. Also seen are unencapsulated nerve fibers arranged in irregular fascicles in cicatrix
Dx?

A

Traumatic neuroma

33
Q

A 9 year old boy was brought to the clinic due to a right thigh mass. Microsections show proliferation of variably cellular hyperchromatic uniform spindled cells with pale cytoplasm arranged in fascicles, whorls and herringbone with a collagenous to myxoid vascular background.
IHC: (+) s100, sox10, GFAP
(-) H3K27me
Dx?
MC site
Morpho
RF
Adverse factors
Variant

A

Malignant peripheral nerve sheath tumor (MPNST)
Sciatic nerve
“Marbled appearance due to variations in celularity
Exhibits “divergent diffetentiation” due to focal areas that exhibit other lines of differentiation (glandular, cartilaginous, osseous, rhabdomyoblastic - triton)
NF1 (younger age, male), plexiform NF sometimes diffuse NF
Central location, >5 cm, high grade

MPNST with rhabdomyoblastic differentiation (malignant triton tumor) - assoc with NF1, with rhabdomyoblasts

34
Q

Tumor with lobular proliferation of epithelioid to spindle cells and suspended in a myxoid matrix. Lobules separated by dense fibrocollagenous septae
(+) s100 and GFAP
MC in hands and fingers

A

Nerve sheath myxoma

35
Q

A 65 year old man came in due to a forehead mass. Microsections show plump bland spindle cells arranged in interlacing fascicles with a collagenous stromal BG and clefting
Ihc (+) s100
Dx?
Mc sites

A

Solitary circumscribed neuroma (palisaded encapsulated neuroma)
Lips, nose, forehead, oral mucosa

36
Q

A 21 year old female came in due to a subungual mass which is painful when it’s cold. Microsections show proliferation of small nests of cells with round nuclei and small nucleoli organized around vascularized hyalinized to myxoid stroma
Ihc (+) SMA, caldesmon
Dx?
Molecular
Types

A

Glomus tumor
NOTCH1 gene rearrangements
Solid, angiomatous and myxoid

37
Q

A 53 year old man came in due to a painless growing mass on left lower leg. Mocrosections show uniform, plump ovoid to spindle blnd cells with CONCENTRIC growth pattern around thin-walled BVs
(+) caldesmon
Dx?
Mc site
Molecular

A

Myopericytoma
Lower legs
PDGFRB

38
Q

A 50 year old female came in due to right forearm mass. Microsections show epithelioid to spindled cells with round to oval nuclei and lightly eosinophilic cytoplasm arranged in nests, sheets, trabeculae and radiate outward from vessels
Ihc (+) HMB45, SMA, MelanA, caldesmon, desmin
Dx?
Variants
Pathogenesis
Molecular
Folpe criteria

A

Perivascular epithelioid cell tumor (PEComa)
Fibroma-like and sclerosing variants
TSC2 deletion - mTOR pathway
Non-TSC2 related - TFE3 mutations or SFPQ-TFE3 fusion

  • HR: >5 cm, cellular, high nuclear grade, mitoses, necrosis, vascular invasion, infiltrative growth pattern

Likely benign: <5 cm and <2 HRFs
UMP: >/= 5 cm, no other HRF or MNGC or pleomorphism only
Malignant : >/= 2 HRFs

39
Q

Leiomyoma
Mitotic counts in deep tissues, retroperitoneal/pelvic in women and men
Molecular

A

Deep tissues/retroperitoneal, pelvic in MEN: benign(<1/50), STUMP (1-5/50)
R/P in women: benign (<5/50), STUMP (5-10/50)
PLAG1 or HMGA2
Retroperitoneal: EWSR1- PBX3 or KAT6B-KANSL1 fusions

40
Q

Leiomyosarcoma
MC site of metastasis
Variants
Molecular

A

Lung
Pleomorphic, myxoid, epithelioid
Complex karyotypes

41
Q

A 62 year old renal transplant patient complained of hoarseness. Laryngoscopy revealed a true vocal cord mass. Excision done.
Microsections show round to spindled to tumor cells, forming storiform fascicles, there is prominent vascularity
(+) SMA, p53, h caldesmon
Dx?
Pathogenesis

A

EBV-associated smooth muscle tumor
(+) EBER-ISH

42
Q

A 55 year old men presented with a painless, slow growing mass in the neck. Microscopic sections show large polygonal tumor cells with central or eccentric nuclei, finely granular, eosinophilic, cytoplasm, arranged in small nests and sheets. The cytoplasm often has cross striations. There is also delicate capillary net work.
IHC (+) MYOD1, desmin, myogenin
Dx?

A

Rhabdomyoma
Types.
Cardiac type - seen in hearts of infants and children and has spiderweb cells, assoc with TSC
Fetal type - head and neck

43
Q

MC sarcoma in children in the first decade composed of tumor cells with variable Morphology and hyperchromasia. Described as marbled appearance with alternating cellular and hypocellular foci. Tumor cells may show rhabdomyoblastic differentiation, with eccentric, nuclei, and prominent eosinophilic cytoplasm with or without striations. May have strap or tadpole cells.
Ihc: (+) MyoD1, myogenin (nuclear), desmin
Dx?
Variants?

A

Embryonal rhabdomyosarcoma
Botryoid and anaplastic variants

44
Q

Alveolar rhabdomyosarcoma
Molecular
Prognosis

A

MC: PAX3-FOXO1
worse than embryonal type

45
Q

Tumor composed of significantly pleomorphic, rhabdomyoblasts which have a variety of morphology. Unlikely to see cross-striations.
(+) MYOD1
Dx
3 patterns

A

Pleomorphic rhabdomyosarcoma
Round cell, classic and spindle cell patterns

46
Q

Benign tumor MC found in the knee composed of large epitheioid cells with the nuclei that are around ovoid and excentrically, located with conspicuous nucleoli and ample eosinophilic cytoplasm. OLGC, macrophages and hemosiderin pigment present.
dx?
Molecular?

A

TGCT, diffuse type
COL6A3-CSF1

47
Q

Dermatofibroma
Ihc

A

(+) CD34 and FXIIIA

48
Q

A tumor composed of bland uniform, spindled to sellate cells with oval nuclei and fibrillary or tapered cytoplasm, suspended in a myxoid matrix. There is sparse, vasculature and spindle cells lack condensation around vessels.
IHC: (-) MUC4
Variable CD34 and SMA
Dx
Types
Molecular

A

Myxoma
Intramuscular, juxta-articular, cardiac (sporadic or familial)
May be assoc with MAZZABRAUD syndrome (myxabraudysplasia)
Most important IHC is negative MUC4

Sporadic, cellular and noncelular, mazabraud - Activating mutation in GNAS
Familial cardiac- PRKAR1A
sporadic cardiac - none of those two above

49
Q

Tumor in middle aged adults with stress fracture in thigh composed of bland spindled and stellate cells in a background of chondromyxoid or hyalinized matrix material with scattered flocculent calcifications
(+) FGF23, SATB2, ERG, CD56
Dx
Clinical

A

Phosphaturic mesenchymal tumor
Hypophosphatemia and hyperphosphaturia with normal calcium

50
Q

A 25 year old man came in due to a right foot mass. Microsections show monotnonous who void the spindle cells with nuclei that have conspicuous nucleolus and clear cytoplasm and arranged in fascicles and nests. Some cells contain melanin. There are scattered MNGCs
IHC: (+) HMB45, melanA
Dx?
Molecular?

A

Clear cell sarcoma of soft tissue
EWSR1-ATF1

51
Q

A 56 year old man came in with a left lower leg mass. Microsections show monotonous, proliferation of round to spindle shaped cells with minimal eosinophilic, cytoplasm, arranged in cords, clusters and nests with a handholding type of look. The background has abundant chondromyxoid matrix. Necrosis, hemorrhage and hemosiderin are common.
(+) s100 and EMA (nonspecific)
Dx?
Molecular

A

Extraskeletal myxoid chondrosarcoma
EWSR1-NR4A3

52
Q

Synovial sarcoma
Unfavorable characteristics
Ihc
Molecular

A

Unfavorable factors: large size, age >40, poorly diff in >/=20% of tumor, high stage at dx
Ihc (+) ssx, ss18, TLE1
SS18-SSX1 fusion

53
Q

A 55 year old male came in due to right upper arm mass and cough. CT scan also showed multiple lung nodules. Biopsy of mass shows sheet like growth of large pleomorphic, rhabdoid-appearing cells with similar nuclear features the spindles cells
(+) EMA, CK, CD34, ERG
(-) INI1
Dx?
Types?
Prognosis
Molecular?
Adverse prognostic factors?

A

Epithelioid sarcoma
Classic/conventional and proximal- young adult males
Proximal - worse prognosis, middle-age or older male adults
Deletion of SMARCB1 (INI1)

Proximal or deep sites, male sex, >5 cm

54
Q

A 9 year old girl came in with a 4 cm buttock mass. Microsections show large,
Uniform epithelioid cells with round nuclei and conspicuous nucleolus and abundant granular eosinophilic cytoplasm in pseudoalveolar pattern due to tumor cel fall out. And these are arranged in lobules, separated by fibrocollagenous septa. There are rod to rhomboid-shaped intracytoplasmic inclusions
(+) TFE3
(+) PAS inclusions
Dx
Prognosis
Prognostic factors
Molecular

A

Alveolar soft part sarcoma
Poor prognosis
Favorable: age less than 10 diagnosis, small size of less than 5 cm, no metastasis
ASPSCR1-TFE3 fusion

55
Q

A tumor with proliferation of small round cells with moderate nuclear pleomorphism, coarse, chromatin, conspicuous nucleoli, and indistinct cytoplasm
(+) WT1, CD99, ETV4
Dx
Molecular

A

Undifferentiated round cell sarcoma with CIC-DUX4 translocation

56
Q

A tumor with small round plump, spindle cells with the monomorphic around Nuclei find chromatin and scant eosinophilic cytoplasm. These are arranged in solids sheets with the rich vascular stroma.
Dx
Prognosis
Molecular

A

BCOR-CCNB3 fusion-positive sarcoma
Better than CIC-rearranged
Ihc (+) BCOR

57
Q

Solitary fibrous tumor
Variants
Risk stratification
Ihc
Molecular

A

Lipomatous - >male
Superficial - > females
Based on tumor size, age, mitotic activity, and necrosis
(+) STAT6, CD34
NAB2-STAT6 gene fusion

58
Q

A 70 year old man dies. He was noted to have multiple nodules in the body. At autopsy, a large mass was seen in the pulmonary artery. Microsections show poorly differentiated spindle cells with moderate to severe pleomorphism
(+) MDM2
(-) CD31, CD34, ERG
Dx
Pathogenesis
Prognosis
Molecular

A

Intimal sarcoma
Arises in the wall of large BVs usually in the pulmonary trunk or artery
Mortality >80% in 1 year
Chromosomal gains and amplifications (MDM2, EGFR, PDGFRA)

59
Q

Intravascular, exuberant proliferation of endothelial cells with fibrin. Small Papillae, covered by a single layer of endothelium with a collagenous fibrin core. Papillae can fuse, forming anastomotic channels.

A

Papillary endothelial hyperplasia (aka masson tumor)

60
Q

It is a pseudo neoplastic vascular, proliferation cost by Bartonella henselae, or Bartonella Quintana. It is associated with neutrophilic infiltrates. It is positive for warthin-starry

A

Bacillary angiomatosis

61
Q

Lymphangioma
Assoc
Syndrome
Ihc

A

Turner
(+) D2-40c prox1 and cd31

62
Q

Tumor with large epithelioid endothelial cells with densely eosinophilic, cytoplasm distinct vasoformation, love either architecture and can have solid areas. Often have many eosinophils
Dx
Ihc
Molecular

A

Epithelioid hemangioma
Vascular markers
Rearrangements of FOS or FOSB

63
Q

A 9 month old boy suddenly had painful violaceous plaques. CBC showed thrombocytopenia. Biopsy of the plaques showed infiltration of tumor cells into soft tissue in a cannon-ball fashion. There are also areas with glomeruloid-like structures which may have thrombi(+) CD31, CD34, D2-40, prox1
(-) HHV8
Dx?
Association?
Behavior?

A

Kaposiform hemangioendothelioma
Kasabach-merritt phenomenon
Locally aggressive

64
Q

A tumor with cords and nests of epithelioid endothelial cells within myxohyaline stroma. There are larger cells with abundant eosinophilic cytoplasm some have intra-cytoplasmic lumen with red blood cells called blister cells.
(+) vascular markers
Dx
Molecular

A

Epithelioid hemangioendothelioma
WWTR1-CAMTA1 fusion

65
Q

A tumor with proliferation of bland, spindle cells with slit like vascular spaces containing erythrocytes.
(+) LANA1, erg, cd31, cd34
Dx
Stages
Pathogenesis

A

Kaposi sarcoma
Patch, plaque and tumor stage
>Classic/endemic: assoc with podoconiosis (foot lymphedema from soil exposure)
>AIDS assoc: most ggtessive and presents at PATCH stage
- coinfection with HIV1 and HHV8
>iatrogenic

66
Q

Angiosarcoma
RF
Molecular

A

Postradiation - 5 years after
Can occur in post surgial
Lymphedema: stewart- treves syndrome
High level of MYC amplification

67
Q

In sarcomas with simple karyotype, whatbis the usual microscopic appearance and which individuals are more affected? Karyotype? %?

A

Monomorphic microscopic apppearance (e.g. ewing)
Younger individuals
Usually euploid
20%

68
Q

In sarcomas with complex karyotype, whatbis the usual microscopic appearance and which individuals are more affected? Karyotype? %?

A

Diverse appearance
Adults
Aneuploid or polyploid
80%

69
Q

MC sarcomas of adulthood

A

Liposarcomas

70
Q

3 types of liposarcomas and their molecular aberrations

A

Well-differentiated - MDM2 amp
Myxoid - t(12;16) —> arrest adipocyte differentiation —> unregulated proliferation of primitive cells
Pleomorphic - complex karyotype (no reproducible genetic abnormalities)

71
Q

4 types of rhabdomyosarcoma

A

Embryonal - child and teens
Alveolar- child and teens (PAX3-FOXO1)
Pleomorphic-adults
Spindle cell- all ages

72
Q

MT or F: soft tissue tumors arise from
Pluripotent mesenchymal stem cells rather than mature cells

A

True

73
Q

Components of a neurofibroma

A

Perineurial-like cells
Fibroblasts
CD34+ spindle cells
Mast cells

74
Q

Types of neurofibroma

A

Superficial cutaneous neurofibromas- sporadic or NF-1 assoc (multiple)
Diffuse neurofibromas- plaque-like, typically NF1 assoc
Plexiform neurofibromas - deep or superficial location in assoc with nerve roots or large nerves and are unifomrly associated with NF1

75
Q

This tumor produces focal collections of cells mimicking the appearance of Meissner corpuscles
Dx?
What are these called

A

Diffuse neurofibroma
Wagner-Meissner corpuscles or Pseudo-Meissner corpuscles or tactile-like bodies

76
Q

Neurofibromatosis type 1
Protein product
Tumors
Clinical

A

AD LOFm in NF1 gene
Neurofibromin
Neurofibromas, MONST, gliomas of optic nerve, other glial tumors and hamartomatous lesions, pheochromocytoma
Intellectual disability or seizures, cafe au lait spots

77
Q

NF type 2
Gene product
Tumors
Clinical

A

AD
Merlin
Bilateral
Eight nerve schwannoma, multiple meningiomas, ependymomas of SC
Schwannosis, meningiomatosis, glial hamartia

Bone And Soft Tissue (2 decks)

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