Tumor Muskuloskeletal

Soft Tissue Tumors and
Tumor-Like Lesions
• Soft tissue tumors may arise in any location, although approximately
40% occur in the lower extremities, especially the thigh; 20% in the
upper extremities; 10% in the head and neck; and 30% in the trunk
and retroperitoneum. Regarding sarcomas, males are affected more
frequently than females (1.4:1), and the incidence generally
increases with age.
• Fifteen per cent arise in children; they constitute the fourth most
common malignancy in this age group, following brain tumors,
hematopoietic cancers, and Wilms tumor in frequency.
• Specific sarcomas tend to appear in certain age groups (e.g.,
rhabdomyosarcoma in children, synovial sarcoma in young
adulthood, and liposarcoma and malignant fibrous histiocytoma in
mid- to late adult life).
Morphology of Cells in Soft Tissue
Tumors
Cell Type Features Tumor Type
Rod-shaped, long axis twice as Fibrous, fibrohistiocytic, smooth

Spindle cell
great as short axis muscle, Schwann cell
Small round Size of a lymphocyte with little Rhabdomyosarcoma, primitive

cell cytoplasm neuroectodermal tumor
Polyhedral with abundant

Smooth muscle, Schwann cell
Epithelioid cytoplasm, nucleus is centrally
endothelial, epithelioid sarcoma
located
Architectural Patterns in Soft Tissue
Tumors
Pattern Tumor Type

Fascicles of eosinophilic spindle cells intersecting at right angles Smooth muscle
Short fascicles of spindle cells radiating from a central point (like
Fibrohistiocytic
spokes on a wheel)—storiform
Nuclei arranged in columns—palisading Schwann cell
Herringbone Fibrosarcoma
Mixture of fascicles of spindle cells and groups of epithelioid
Synovial sarcoma
cells—biphasic
Tumors of adipose tissue
Lipomas
Liposarcoma
Tumors and tumor-like lesions of fibrous tissue
Nodular fasciitis
Soft Tissue Fibromatoses
Tumors Superficial fibromatoses

Deep fibromatoses
Fibrosarcoma
Fibrohistiocytic tumors
Fibrous histiocytoma
Dermatofibrosarcoma protuberans
Malignant fibrous histiocytoma
Tumors of skeletal muscle
Rhabdomyoma
Rhabdomyosarcoma
Tumors of smooth muscle
Leiomyoma
Smooth muscle tumors of uncertain malignant potential
Leiomyosarcoma
Vascular tumors
Hemangioma
Lymphangioma
Hemangioendothelioma
Hemangiopericytoma
Angiosarcoma
Peripheral nerve tumors
Neurofibroma
Schwannoma
Granular cell tumor
Malignant peripheral nerve sheath tumors
Tumors of uncertain histogenesis
Synovial sarcoma
Alveolar soft part sarcoma
Epithelioid sarcoma
Myxoid liposarcoma with abundant ground substance in which are scattered adult-appearing fat cells and
more primitive cells, some containing small lipid vacuoles (lipoblasts).
Nodular fasciitis with plump, randomly oriented spindle cells surrounded by
myxoid stroma. Note the mitotic activity and extravasated red blood cells.
Fibromatosis infiltrating between
skeletal muscle cells.
These tumors occur as gray-white, firm, poorly demarcated masses varying from 1 to 15 cm in greatest diameter. They
are rubbery and tough and infiltrate surrounding structures. Histologically deep-seated fibromatosis is composed of
plump fibroblasts arranged in broad sweeping fascicles that infiltrate to the adjacent tissue .Mitoses are usually
infrequent. Regenerating muscle cells, when trapped within these lesions, may take on the appearance of
multinucleated giant cells.
Typically, these neoplasms are
unencapsulated, infiltrative, soft, fish-
flesh masses often having areas of
hemorrhage and necrosis.
Better-differentiated lesions may appear
deceptively encapsulated.
Histologic examination discloses all
degrees of differentiation, from slowly
growing tumors that closely resemble
cellular fibromatosis sometimes having
spindled cells growing in a herringbone
fashion to highly cellular neoplasms
dominated by architectural disarray,
pleomorphism, frequent mitoses, and
areas of necrosis
Rhabdomyosarcoma composed of malignant
small round cells. The rhabdomyoblasts are
large and round and have abundant eosinophilic
cytoplasm; no cross-striations are evident.
• Rhabdomyosarcoma is histologically
subclassified into the embryonal,
alveolar, and pleomorphic variants.[
• The rhabdomyoblast—the diagnostic
cell in all types—contains eccentric
eosinophilic granular cytoplasm rich in
thick and thin filaments. The
rhabdomyoblasts may be round or
elongate; the latter are known as
tadpole or strap cells contain
sarcomeres, and
immunohistochemically they stain with
antibodies to the myogenic markers
desmin, MYOD1, and myogenin.
Synovial sarcoma revealing the classic biphasic spindle
cell and glandular-like histologic appearance.
• The histologic hallmark of biphasic

synovial sarcoma is the dual line of
differentiation of the tumor cells (i.e.,
epithelial-like and spindle cells). Despite
the mimicry of synovium, the tumor cells do
not have the features of synoviocytes. The
epithelial cells are cuboidal to columnar and
form glands or grow in solid cords or
aggregates. The spindle cells are arranged in
densely cellular fascicles that surround the
epithelial cells
• Many synovial sarcomas are monophasic in
that they are composed of only spindled cells
or, rarely, epithelial cells.
PIGMENTED VILLONODULAR SYNOVITIS AND GIANT
CELL TUMOR OF TENDON SHEATH
Villonodular synovitis is the term for several closely

related benign neoplasms that develop in the synovial
lining of joints, tendon sheaths, and bursae. They were
previously considered reactive synovial proliferations.
Aggressive tumors erode into adjacent bones and soft
tissues, causing confusion with other types of neoplasia.
In contrast, GCT manifests as a solitary, slow-growing,
painless mass that frequently involves the tendon sheaths
along the wrists and fingers; it is the most common
mesenchymal neoplasm of the hand

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Soft Tissue Tumors and

Rod-shaped, long axis twice as Fibrous, fibrohistiocytic, smooth

Small round Size of a lymphocyte with little Rhabdomyosarcoma, primitive

Polyhedral with abundant

Pattern Tumor Type

Tumors Superficial fibromatoses

• The histologic hallmark of biphasic

Villonodular synovitis is the term for several closely

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