SOFT TISSUE TUMOURS

PART-1
SOFT TISSUE TUMOURS

Mesenchymal proliferations that occur in the extraskeletal,

nonepithelial tissues of the body, excluding the viscera,
coverings of the brain, and lymphoreticular system
FNCLCC GRADING SYSTEM
 BENIGN ADIPOCYTIC TUMORS
Lipoma NOS
Composed of mature adipocytes
Subtype:
Intramuscular lipoma
Chondrolipoma
Site- MC Upper back, proximal extremities,
abdomen
Gross- glistening yellowish-tan
M/E: uniform proliferation of mature
adipose tissue without atypical
hyperchromatic stromal cells
BENIGN ADIPOCYTIC TUMORS

Intramuscular lipoma: Chodrolipoma:

Adipose tissue infiltrating mature skeletal nodules of metaplastic bone or cartilage
muscle
also termed as Osteolipoma
show degenerative changes
 BENIGN ADIPOCYTIC
TUMORS
Lipomatosis:
Subtype:
 Diffuse lipomatosis
 multiple symmetrical lipomatosis
 pelvic lipomatosis
 steroid lipomatosis
 HIV lipodystrophy
Site: trunk/large portion of extremity
Gross: poorly circumscribed, soft, yellow
M/E: All the different types of lipomatosis have identical morphological
features
consisting of lobules and sheets of mature adipocytes that may infiltrate
other structures such as skeletal muscle
BENIGN ADIPOCYTIC TUMORS

Lipoblastoma/Lipoblastomatosis
Benign neoplasm of embryonal white
fat
Subtype: Localized
Diffuse
Site : MC trunk and extremities
Gross: typically 2-5cm exceed upto
10cm
Soft, lobulated, yellow/white/tan
C/S: myxoid nodule, cystic spaces/fat
nodules separated by fine white
fibrous trabeculae
 BENIGN ADIPOCYTIC TUMORS

Lipoblastoma/
Lipoblastomatosis:
M/E :
Sheets of adipocytes separated by
fibrovascular septa
Myxoid areas display plexiform
vascular pattern with primitive
mesenchymal cells
Fat cells show spectrum of
maturation
BENIGN ADIPOCYTIC TUMORS
Angiolipoma:
Subcutaneous tumor consisting of mature fat
cells +small and thin-walled vessels + fibrin
thrombi
Subtype- Cellular
Site- extrimities (forearm)
Gross: encapsulated yellowish/ reddish nodule
M/E: mature adipocytes and branching
capillary-sized vessels with fibrin thrombi
Should be distinguished from Angiosarcoma
and Kaposi sarcoma
BENIGN ADIPOCYTIC TUMORS

Myolipoma :
benign extrauterine tumor, mature adipose
tissue+ well differentiated smooth muscle cells
Site: Retroperitoneum/ abdominal cavity/pelvic
cavity
Gross: large (10-25cm), well circumscribed,
thin capsule C/S – yellowish , tan-whitish
whorled nodule
M/E: mature fat+ spindled cells
Spindled area of smooth muscle cytologically
bland cells having eosinophilic cytoplasm,
cigar-shaped nuclei with perinuclear vacuoles
BENIGN ADIPOCYTIC TUMORS

Chondroid Lipoma:
composed of lipoblasts + mature adipocytes in a
myxohyaline chondroid matrix
Site: deep seated skeletal muscle/ connective
tissue/ subcutaneous fat
Gross: lobulated, circumscribed, yellowish-tan,
gelatinous
M/E: mature adipose tissue + nests and cords of
small round vacuolated cells +myxoid-chondroid
matrix
Eosinophilic + vacuolated cells containing
glycogen
can be mistaken chondrosarcoma or myxoid
liposarcoma
BENIGN ADIPOCYTIC TUMORS

Spindle cell lipoma and Pleomorphic

lipoma:
Composed of mature adipocytes+ bland
spindle cells + ropy collagen
PL- in addition pleomorphic+ multinucleated
floret like giant cells
Site: posterior neck/back/shoulders
Gross: oval/discoid mass C/S- yellow/greyish-
white and myxoid
M/E: triad of- bland spindle cells+ mature
adipocyte+ ropy collagen (thick, refractile,
eosinophilic) in fibromyxoid stroma
BENIGN ADIPOCYTIC TUMORS

Atypical Spindle cell/ pleomorphic lipomatous tumour :

variable proportions of atypical spindle
cells, adipocytes
univacuolated / bivacuolated to
multivacuolated lipoblasts
 pleomorphic (multinucleated) cells
 myxoid to collagenous extracellular
matrix
BENIGN ADIPOCYTIC TUMORS

Hibernoma:
rare, showing brown fat differentiation
Site: interscapular region/thigh/axilla
Gross: well circumscribed, lobular ,
brown to yellow
M/E: rich capillary network of
eosinophilic pale, polygonal,
multivacuolated, granular, brown fat
cells+ univacuolated white fat
MALIGNANT ADIPOCYTIC
TUMOUR
Atypical lipomatous tumour / well-
differentiated liposarcoma
(ALT/WDLPS):
 locally aggressive mesenchymal
neoplasm composed of an adipocytic
proliferation showing at least focal
nuclear atypia in both adipocytes and
stromal cells

Gross: well-circumscribed, lobulated

mass
 MALIGNANT ADIPOCYTIC
TUMOUR
Liposarcoma, well differentiated, NOS
Lipoma-like subtype:
• MC subtype
• Scattered atypical cells
• Frequently contain lipoblasts
• Grossly can be indistinguishable from lipoma
Sclerosing subtype:
• Retroperitoneal or paratesticular location
• Collagenous fibrous tissue +scattered adipocytes+
atypical multinucleated stromal cells
MALIGNANT ADIPOCYTIC
TUMOUR
Liposarcoma, well differentiated, NOS
Inflammatory subtype:
 rare
 retroperitoneum where it is confused for
nonlipogenic tumors
 Chronic inflammatory cells + lymphoid
follicles scattered in a cellular F/C stroma
 May obscure adipocytes
MALIGNANT ADIPOCYTIC
TUMOUR

Dedifferentiated liposarcoma:
Site: Retroperitoneum
Gross: large multinodular yellow
masses + tan-grey non-lipomatous
(dedifferentiated) areas
M/E: histological hallmark-
transition from ALT/ WDLPS to
non-lipogenic sarcoma (high grade)
MALIGNANT ADIPOCYTIC
TUMOUR
Myxoid Liposarcoma:
uniform, round to ovoid cells + small
lipoblasts in a myxoid stroma with a
branching capillary vasculature
Site: Thigh
Gross: large ,circumscribed, multinodular
intramuscular C/S- smooth, gelatinous, and
glistening
M/E: numerous vacuolated lipoblasts in
loose myxoid stroma, prominent
anastomosing (chicken-wire)
MALIGNANT ADIPOCYTIC
TUMOUR

Pleomorphic Liposarcoma:
high-grade sarcoma containing pleomorphic
lipoblasts
Subtype: Epithelioid Liposarcoma
Essential diagnostic features:

variable number of pleomorphic lipoblasts

myxofibrosarcoma-like morphology with
pleomorphic lipoblasts
epithelioid subtype with sheets of carcinoma-
like epithelioid cells with pleomorphic lipoblast
 ADIPOCYTIC TUMORS
Tumor
Lipoblastomatosis
Myolipoma
Chondroid lipoma
Spindle cell lipoma
Atypical spindle
cell/pleomorphic lipomatous
tumor
Liposarcoma
Myxoid
Pleomorphic liposarcoma
IHC Molecular genetics
S100, CD34, CD56 PLAG1 rearrangement
Desmin, SMA -
+:S100, Cyclin D1, PAS t(11;16)
-: CD34, SMA, EMA
CD34, Vimentin Loss of RB1 locus
CD34, S100, Desmin Absence of MDM2 or CDK4
MDM2 or CDK4 MDM2 or CDK4 gene
amplification
Vimentin, S100 t(12;16)(q13;p11.2) FUS-
DDIT3
Vimentin, S100, SMA Absence of amplification of
MDM2/CDK4
MAJOR UPDATES IN WHO 2020
CLASSIFICATION
•Adipocytic tumors
2 new entities:
Benign
• Atypical spindle cell / pleomorphic lipomatous tumor (ASPLT)
Malignant
• Myxoid pleomorphic liposarcoma
• Epithelioid liposarcoma is classified as a distinctive variant of pleomorphic
liposarcoma
• Extrarenal angiomyolipoma was deleted in this category and reclassified in the
category of tumors of uncertain differentiation-- now referred to as
angiomyolipoma
• Extra-adrenal myelolipoma was removed
FIBROBLASTIC &
MYOFIBROBLASTIC TUMOURS
Benign
Nodular fasciitis:
self-limiting mesenchymal neoplasm in
subcutaneous tissue
Subtype:
Intravascular- arteries and veins
Cranial –infants, skull involvement
Site: upper extremities/trunk/neck
Gross: round/oval, nodular well-circumscribed,
C/S- myxoid/fibrous, central cystic change
M/E:
• Spindle stellate cells with a loose fascicular to
storiform pattern (so called "tissue culture-like"
and "feathery" growth)
FIBROBLASTIC &
MYOFIBROBLASTIC TUMOURS
Myositis Ossificans and fibro-osseous
pseudotumour of digits :
self-limited, spindle cells and osteoblasts
Site: anywhere, elbow/thigh/buttock/shoulder
Gross: well circumscribed, tan hemorrhagic
with gritty area
M/E: hypercellular
fascicles of uniform spindle cells
woven bone with zonation
 most mature at the periphery
FIBROBLASTIC &
MYOFIBROBLASTIC TUMOURS
Fibroma of tendon sheath:
fibroblastic/myofibroblastic nodular proliferation
(attached to a tendon sheath)
Site: finger tendons (thumb/index finger/ middle
finger)
Gross: circumscribed, firm, grey-white, lobulated
nodule attached to tendon
M/E:
 low cellularity
bland spindle cells in collagenous background
Thin walled slit-like vessels
FIBROBLASTIC &
MYOFIBROBLASTIC TUMOURS
Myofibroblastoma:
Site: inguinal/groin area
Gross: white/yellow, well circumscribed,
mobile, rubbery/ gelatinous
M/E:
 haphazardly intersecting short fascicles
of bland
Short/elongated spindle cells
bands of hyalinized collagen
variable adipocytic component
FIBROBLASTIC &
MYOFIBROBLASTIC TUMOURS

Calcifying aponeurotic fibroma:

slow growing, painless mass, primarily in
children
Site: palmar surfaces hands/fingers,
plantar surface feet/toes
Gross: ill-defined, greyish-white to gritty
soft tissue mass
M/E: bland, spindle cells lying in
parallel within a collagenous matrix
 islands of calcified matrix surrounded
by palisading epithelioid fibroblasts
FIBROBLASTIC &
MYOFIBROBLASTIC TUMOURS
Cellular angiofibroma:
Site: vulvovaginal/inguinoscrotal region
Gross: well -circumscribed
round/oval/lobulated, soft to rubbery and
the C/S- solid greyish pink
M/E:
 bland spindle cells
bundles of collagen fibres
Small/ medium-sized thick-walled
vessels +/- adipose tissue
FIBROBLASTIC &
MYOFIBROBLASTIC TUMOURS
Angiofibroma NOS:
Site: extremities (legs), adjacent to large
joints
Gross: well-demarcated,
nodular/multinodular white to yellow,
glistening
M/E:
 bland and uniform short spindle cells
with alternating myxoid and collagenous
areas
 small thin-walled branching blood vessels
FIBROBLASTIC &
MYOFIBROBLASTIC TUMOURS
Solitary fibrous tumour:
haphazardly arranged spindled to ovoid
cells, prominent staghorn vasculature
and NAB2-STAT6 gene rearrangement
Site: superficial and deep soft tissues,
visceral organs and bone
Gross: well circumscribed mass, C/S- tan
to reddish brown

M/E: Ovoid to fusiform spindle cells,

branching, hyalinized staghorn-like
vasculature, collagenous stroma
 FIBROBLASTIC &
MYOFIBROBLASTIC TUMOURS
Dermatofibromasarcoma protuberans NOS:
superficial, locally aggressive fibroblastic neoplasm
Subtypes:
 Pigmented DFSP
 DFSP fibrosarcomatous
 Myxoid DFSP
 DFSP with myoid differentiation
 Plaque-like DFSP
Site: trunk /proximal extremities
Gross: indurated plaques, one/multiple nodules ill-
defined, firm, C/S- greyish-white gelatinous area
FIBROBLASTIC &
MYOFIBROBLASTIC TUMOURS
Dermatofibromasarcoma protuberans NOS:
M/E: Classical DFSP
diffuse infiltration dermis/subcutis
neoplastic cells infiltrate SC fat
(honeycomb appearance)
tumour cells encase skin appendages
without destroying them
spindled tumour cells containing
elongated wavy nuclei
 arranged in storiform/
whorled/cartwheel patterns
FIBROBLASTIC &
MYOFIBROBLASTIC TUMOURS

Dermatofibromasarcoma protuberans
NOS:
M/E: Pigmented DFSP (Bednar tumour)
pigmented dendritic melanocytic cells
Myxoid DFSP
prominent myxoid stroma+ numerous
vessels +fibrotic vessel walls
 FIBROBLASTIC &
MYOFIBROBLASTIC TUMOURS
Fibrosarcoma:
rare sarcoma, monomorphic fibroblastic tumour cells
with variable collagen production and herringbone
architecture
Site: deep tissues of extremities/ trunk/ head and neck
Gross: circumscribed, firm, white / tan mass
M/E:
 Spindle cells arranged in sheets and intersecting
fascicles creating a diffuse herringbone appearance
Mild to moderate nuclear atypia
Mitotic activity > 4/10 HPF is most important criteria
for diagnosing fibrosarcoma
FIBROBLASTIC AND
MYOFIBROBLASTIC TUMORS
Tumor IHC Molecular genetics
Nodular fascitis SMA, MSA, Calponin MYH9-USP6 fusion genes,
USP6 rearrangement

Myofibroblastoma Desmin, CD4 Loss of RB1

Calcifying aponeurotic fibroma SMA, MSA, CD99, S10 FN1-EGF gene fusion

Solitary fibrous tumor STAT6, CD34 NAB2-STAT6 gene fusion

DFSP CD34, Vimentin COL1A1-PDGFB gene fusion
MAJOR UPDATES IN WHO 2020
CLASSIFICATION
Fibroblastic and myofibroblastic tumors:
Benign
• Angiofibroma of soft tissue
• EWSR1-SMAD3 fibroblastic tumor
• Superficial CD34 positive fibroblastic tumor
 SO-CALLED
FIBROHISTIOCYTIC TUMOURS
Tenosynovial giant cell tumour NOS:
arising from the synovium of joints/bursae/ tendon sheaths, showing
synovial differentiation
 Subtype: Tenosynovial GCT diffuse
 malignant tenosynovial GCT
Site: hand
Gross: well circumscribed, lobulated, white/grey +
yellowish/brown areas
M/E: mononuclear cells, multinucleated giant cells, foamy
macrophages, inflammatory cells, and haemosiderin
mononuclear components:
 Small histiocyte-like cells: pale cytoplasm+ round /reniform nuclei
 Large epithelioid cells: amphophilic cytoplasm, rounded vesicular
nuclei + containing a peripheral rim of hemosiderin granules
 SO-CALLED
FIBROHISTIOCYTIC TUMOURS
Malignant tenosynovial giant cell tumour:

 Composed of sheets/nodules of enlarged

mononuclear cells
 increased mitotic count+ atypical mitoses +necrosis
+enlarged nuclei with nucleoli, spindling of
mononucleated cells and myxoid changes
 contain areas that resemble undifferentiated
pleomorphic sarcoma or myxofibrosarcoma
 MAJOR UPDATES IN WHO 2020
CLASSIFICATION
The family of so called malignant fibrous histiocytoma (MFH)
•Undifferentiated pleomorphic sarcoma currently represents the correct
designation for the storiform and pleomorphic variant of MFH
•Giant cell MFH is currently replaced by 3 distinct tumor types: giant cell tumor
of soft tissues, extraskeletal osteosarcoma and giant cell rich osteosarcoma
•Myxoid MFH is currently recognized as a purely fibroblastic tumor, identified
with the original name myxofibrosarcoma
•Inflammatory MFH overlaps entirely with the inflammatory variant of
dedifferentiated liposarcomas
•Angiomatoid MFH (an indolent lesion most often harboring a EWSR1-
CREB1 fusion gene and more rarely a EWSR1-ATF1 or FUS-ATF1 fusion gene) is
currently listed within the group of soft tissue lesion of unknown differentiation
 SO-CALLED
FIBROHISTIOCYTIC TUMOURS
Tumor IHC Molecular genetics

Tenosynovial giant cell tumor Clusterin, desmin, CD68 Translocation CSF1 gene at 1p13

Malignant tenosynovial GCT Clusterin, D2-40 Loss of CDK2A/B

Thank
You