Soft tissue pic

Tumors of fat
• Hibernoma.
• Lipoma.
1. convenstional
2. Angiolipoma Note:
diagnosis of lipoma requires
3. Fibrolipoma
presence of a mass
4. Myelolipoma
5. Spindle cells
6. Pleomorphic
7. Chondroid
8. Myxolipomas
9. Intramuscular lipoma

• lipoblastomas.
• Liposarcoma.
1. differentiated/ dedifferentiated Liposarcoma
2. Myxoid Liposarcoma
3. pleomorphic Liposarcoma
 Tumors of fat
• Hibernoma: a lipoma of brown fat.
 Tumors of fat
• Lipoblastoma: benign pediatric tumor of mature fat and
benign lipoblasts.

lipoblastoma is composed of more mature-appearing fat cells arranged in multiple lobules

Low power view of a lipoblastoma showing lobules of immature fat separated by connective tissue septa.

The lobules are composed of lipoblasts in various stages of development.

Some lobules show extensive myxoid change.

Lipoblastomas may be circumscribed (benign lipoblastoma) or diffuse (diffuse lipoblastomatosis).

 immature fat cells in witch the nucleus is star shaped or
• LIPOBLASTS scalloped, due to being indented on multiple sides by
small bubbles of fat.

Lipoblasts come in a variety of sizes

and shapes.
The cytoplasm is filled with lipid that
appears as 1 or multiple clean,
punched-out vacuoles that indent
and scallop the nucleus.
Malignant lipoblasts are required to diagnose
pleomorphic liposarcoma.
This lipoblast has a large hyperchromatic
nucleus sharply indented by multiple lipid-
filled vacuoles
lipoma
 Spindle cell / pleomorphic lipoma
• Usually found on back or neck of elderly patient.

• Both are stain for CD34 and show loss of RB-1 staining.

• Spindle cell lipoma: Triad of mature adipocytes, bland spindle cells and
hyalinized rope-like collagen fibers.

• Pleomorphic lipoma: contains pleomorphic and Multinucleated stromal giant

cells and floret cells in pleomorphic lipoma.
 Remind you of a

Spindle cell lipoma nerve sheath tumor

Mature adipocytes with bland spindle cells Cellular spindle cell neoplasm with
collagen fibers and few adipocyte
 Pleomorphic lipoma

Pleomorphic lipoma with multiple floret cells, some with bizarre

features
 Spindle cell lipoma
Soft tissue, back, excision:
Spindle cell lipoma. (see comment).

Comment:
Sections show a benign adipocytic neoplasm with bland spindle cells and eosinophilic collagen
bundles in a myxoid background with scattered mast cells.
No necrosis, marked cytologic atypia or significant mitotic activity is identified.
Immunohistochemical stains show that the spindle cells are diffusely positive for CD34, while
demonstrating loss of RB1. These results support the above diagnosis
 Atypical Lipomatous Tumor / Well-differentiated
Liposarcoma

• Looks similar to a lipoma except for

an increase in fibrous (interstitium)
between fat cells and fibrous bands.

• A close examination of fibrous areas

reveals hyperchromatic , irregular
shaped nuclei.

• finding a lipoblast is a bonus.

• Differently sized fat cells, unlike

monomorphic benign lipoma, but can also
seen in begin entities as well.
Atypical Lipomatous Tumor / Well-differentiated
Liposarcoma
 Atypical Lipomatous Tumor / Well-differentiated
Liposarcoma
• Atypical Lipomatous Tumor: occurs on an extremities.
• Well-differentiated Liposarcoma: occurs in non-resectable location.

Atypical Lipomatous Tumor

• Dedifferentiated Liposarcoma:
Well-differentiated Liposarcoma area + transition to high grade tumor
 Dedifferentiated Liposarcoma
• Key to diagnosis : is recognizing adjacent WDLS.

• Because a retroperitoneal sarcoma is a dedifferentiated Liposarcoma until proven

otherwise, if you are grossing such a tumor , be sure to sample anything near the tumor
that looks like normal fat : it may be well differentiated Liposarcoma.
Myxoid Liposarcoma
Myxoid Liposarcoma
Myxoid and Round Cell Liposarcoma
Pleomorphic Liposarcoma
 Fibrous and myxoid tumor
• Inflammatory myofibroblastic tumor
• Nodular fasciitis.
• Proliferative fasciitis.
• Proliferative myositis.
• Fibromatosis.
– infantile
– superficial
– desmoid
• Solitary fibrous tumor.
• Low grade fibromyxoid sarcoma
• Fibrosarcoma
• Myxofibrosarcoma.
 inflammatory myofibroblastic tumor
• Mainly in young people.
• ALK+.
• Arising in the abdominal cavity (visceral location) Not seen in
• Prominent plasma cells nodular fasciitis
Inflammatory myofibroblastic tumor
Inflammatory myofibroblastic tumor
 • Translocation neoplasm.
• Nodular fasciitis • Rapidly growing lesion.
• Circumscribed but unencapsulated .
Nodular fasciitis
 Nodular fasciitis

The morphology of nodular fasciitis varies according to the age of the lesion.
Earlier lesions often contain scant numbers of stellate or spindle cells in copious myxoid matrix composed
of Alcian blue-positive mucopolysaccharides.
 Nodular fasciitis

• Fibroblasts show (( tissue culture appearance)), floating in Myxoid background.

• Red blood cells and lymphocytes.
• No nuclear atypia , but you will see mitosis .
 Proliferative Fasciitis
• The distinguishing feature of proliferative fasciitis is proliferation of large
ganglion-like myofibroblasts with large vesicular nuclei and prominent nucleoli
 Proliferative myositis
• Microscopically, proliferative myositis
consists of proliferation of
fibroblasts and myofibroblasts in a
background of myxoid stroma.

• A characteristic finding in proliferative

myositis is the presence of large
basophilic cells resembling ganglion
cells or rhabdomyoblasts.

• They have enlarged vesicular nuclei with

prominent nucleoli.
• Ultrastructurally, they have features of
myofibroblasts.

• There is increased mitotic activity;

however, atypical mitoses are not found.

• Areas of metaplastic bone or

 Proliferative myositis
• Desmin is usually positive in proliferative myositis; however, MyoD1 and
myogenin are negative.
 Fibromatosis
Infantile fibromatosis:
• showing immature fibroblasts/myofibroblasts in a myxoid background.
• Infantile fibromatosis does not metastasize but frequently tends to recur locally,
usually a result of inadequate excision.
• The risk of recurrence decreases significantly if resection margins are negative
 Fibromatosis
Desmoid Fibromatosis:
• Cytologic atypia is absent and mitotic activity is not increased
• Some cases of fibromatosis show myxoid matrix.
• Most soft tissue fibromatoses arise from the muscular fascia. Given their relationship, the
term musculoaponeurotic fibromatosis is preferred term over the obsolete term Desmoid
tumor.

• The image shows infiltrative margins entrapping skeletal muscle fibers at the periphery of the
lesion
 Desmoid Fibromatosis
• The entrapped skeletal muscle fibers at the periphery of fibromatoses undergo
atrophy and may mimic multinucleated giant cells.
herringbone pattern

MPNST
 Extraskeletal myxoid chondrosarcoma
• t(9:22) CHN
• t(11:22) FLI1 Ewing sarcoma

Desmoplastic small round cell tumor

• t(11:22) WT1
• t(12:22) ATF1 Clear cell sarcoma

• t(17:22) COL1A1-PDGFB Dermatofibrosarcoma protuberans

 t(9:22) CHN
• Extraskeletal myxoid chondrosarcoma
a malignant mesenchymal neoplasm of uncertain differentiation with abundant myxoid matrix,
multilobular architecture, uniform cells arranged in cords, clusters and reticular networks
and NR4A3 rearrangement.
 t(11:22) FLI1
• Ewing sarcoma: NKX2.2 high specific Desmin -

FLI1

Necrosis

CD99
t(21:22)
(strong, diffuse membranous expression in
90%)
 t(11:22) WT1 Keratin AE1 / AE3

• Desmoplastic small round cell tumor

Desmin +

WT1 +
 t(12:22) ATF1
• Clear cell sarcoma:
• is a malignant soft tissue sarcoma composed of monotonous epithelioid/plasmacytoid and
spindle cells with clear to eosinophilic cytoplasm
• characterized by melanocytic differentiation (S100 / SOX10+, Melan A+, HMB45+)
• EWSR1-ATF1 / CREB1 rearrangement
 t(17:22)
COL1A1-PDGFB
Dermatofibrosarcoma protuberans
• DFSP negative
for perineural
markers EMA-
• t(X:17) TFE3
• t(X:18) SSX1, SSX2, SSX4
 t(X:17) TFE3
• Alveolar soft part sarcoma:
-deep soft tissue of young patient.
characterized by well defined nests of cells separated by fibrous stroma.
 t(X:17) TFE3
Alveolar soft part sarcoma
Diagnosis
• Eosinophilic polygonal cells with a nested growth pattern, rich capillary network and
intracytoplasmic rod shaped crystals
• TFE3 nuclear expression by immunohistochemistry
• Confirmation of TFE3 gene rearrangement or ASPSCR1-TFE3 gene fusion.

metastases to the brain are much more

common in ASPS than any other sarcoma

Special stain for PAS highlights

intracytoplasmic crystals
 t(X:18)
• Synovial sarcoma:
• Monotonous spindle cells with vesicular, plump and
overlapping nuclei with hemangiopericytic vessels.
TLE1+, cytokeratin+, EMA+, BCL2+, CD99+

staghorn, dilated vessels

 TLE1 + CD99 +

CK +

Weak, patchy EMA+ BCL2 +

immunohistochemical staining for CD99 (right panel) demonstrates diffuse and strong
membranous expression of CD99 (brown) by synovial sarcoma cells in a biopsy
• t(12:15)
• t(12:16)
 t(12:15) ETV6-NTRK3
• Infantile sarcoma
 t(12:16) FUS-DDIT3
• Liposarcoma – myxoid and round cell type.
 t(22:17) MYH9-USP6
• Nodular fasciitis
Extravasated erythrocytes
fibromatosis
Endomysial connective tissue increased
between muscle fibers.
McArdle disease is an inherited disease. It results from changes
(mutations) in the gene for the enzyme muscle phosphorylase

• McArdle Disease: Vacuoles Often subsarcolemma

• McArdle disease: Phosphorylase deficiency