Soft Tissue Tumors dan Bone

Tumors

Dr Faison SpB (K)Onk

Bedah Onkologi RS Doris Sylvanus
Palangkaraya
 SOFT TISSUE TUMORS
Soft tissue tumors are defined as
mesenchymal proliferations that occur in the
extraskeletal, nonepithelial tissues of the
body.

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 SOFT TISSUE TUMORS

They are classified according to the tissue

they originate such as muscle, fat, fibrous
tissue, vessels, and nerves.

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 SOFT TISSUE TUMORS
• The cause of most soft tissue tumors is unknown.
• The majority of soft tissue tumors occur
sporadically, but a small minority is associated with
genetic syndromes
1. neurofibromatosis type 1 (neurofibroma, malignant
schwannoma),
2. Gardner syndrome (fibromatosis),
3. Li-Fraumeni syndrome (soft tissue sarcoma),
4. Osler-Weber-Rendu syndrome (telangiectasia)

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 SOFT TISSUE TUMORS
There are documented associations, between
radiation therapy and rare instances in which
chemical burns, thermal burns, or trauma
were associated with subsequent
development of a sarcoma.

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 SOFT TISSUE TUMORS
• Exposure to phenoxyherbicides and
chlorophenols has also been implicated in
some cases.
• Kaposi sarcoma is causally associated with the
human herpesvirus 8; (viruses are probably
not important in the pathogenesis of most
sarcomas)

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 SOFT TISSUE TUMORS
Soft tissue tumors may arise in any location,
• 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 , and the incidence generally
increases with age.

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 SOFT TISSUE TUMORS
• Tumors of adipose tissue
Lipomas
Liposarcoma
• Tumors and tumor-like lesions of
fibrous tissue
Nodular fasciitis,
Fibromatoses
Superficial fibromatoses
Deep fibromatoses
Fibrosarcoma
• Fibrohistiocytic tumors
Fibrous histiocytoma
Dermatofibrosarcoma protuberans
Malignant fibrous histiocytoma
• Tumors of skeletal muscle
Rhabdomyoma
Rhabdomyosarcoma
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• 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
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 LIPOMA
LIPOMAS
• Benign tumors of fat, known as lipomas, are the
most common soft tissue tumor of adulthood.
• They are subclassified according to particular
morphologic features as conventional lipoma,
fibrolipoma, angiolipoma, spindle cell lipoma,
myelolipoma, and pleomorphic lipoma.
• Lipomas are soft, mobile, and painless (except
angiolipoma) and are usually cured by simple
excision

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 LIPOMA
Morphology
• The conventional lipoma, the most common
subtype, is a well-encapsulated mass of
mature adipocytes that varies considerably in
size.
• It arises in the subcutis of the proximal
extremities and trunk, most frequently during
mid-adulthood.
• Histologically, they consist of mature white fat
cells with no pleomorphism.
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 LIPOSARCOMA
LIPOSARCOMA
• Liposarcomas are one of the most common sarcomas
of adulthood and appear in those in their forties to
sixties,
• they are uncommon in children.
• They usually arise in the deep soft tissues of the
proximal extremities and retroperitoneum and
developing into large tumors.
• All types of liposarcoma recur locally and often
repeatedly unless adequately excised.

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 LIPOSARCOMA
Morphology
• Histologically, liposarcomas can be divided into(
atypical lipomatous tumor) well-differentiated,
myxoid, round cell, and pleomorphic variants.
• Myxoid type is the most common.
• The cells in well-differentiated liposarcomas are
readily recognized as lipocytes.
• In the other variants, most of the tumor cells are not
obviously adipogenic, but some cells indicative of
fatty differentiation are almost always present.
• These cells are known as lipoblasts; they mimic fetal
fat cells and contain round clear cytoplasmic
vacuoles of lipid that scallop the nucleus.
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 Myxoid liposarcoma

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 TUMORS OF SKELETAL MUSCLE
• Skeletal muscle neoplasms, in contrast to
other groups of tumors, are almost all
malignant.
• The benign variant, rhabdomyoma, is
distinctly rare.

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 TUMORS OF SKELETAL MUSCLE

RHABDOMYOSARCOMA
• Rhabdomyosarcoma, the most common soft tissue
sarcoma of childhood and adolescence, usually
appears before age 20.
• They may arise in any anatomic location, but mostly
the head and neck or genitourinary tract.
• Only in the extremities they appear in relation to
skeletal muscle.

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 Rhabdomyosarcoma Age

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Head-neck 40%
Genitourinery 20%
Extremities 20%
Sites
7% Orbit
28% Head & Neck
11% Trunk
2% Intrathoracic
2% GI-Hepatic
6% Retroperitoneum
18% GU
2% Perineum-Anus
24% Extremities

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 Common Histiotypes of Rhabdomyosarcoma

60% HN-GU 20% Extremities

Botryoid 10% Vagina-Grapelike
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 TUMORS OF SKELETAL MUSCLE

Morphology
• 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 and may contain cross-
striations visible by light microscopy.
• Ultrastructurally, rhabdomyoblasts contain sarcomeres, and
immunohistochemically they stain with antibodies to the
myogenic markers desmin, MYOD1, and myogenin.

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 Rhabdomyosarcoma composed of malignant small round cells. The
rhabdomyoblasts are large and round and have abundant eosinophilic
cytoplasm; no cross-striations are evident

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 Myogenin Ab-1

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 TUMORS OF SKELETAL MUSCLE
• Embryonal rhabdomyosarcoma is the most
common type, accounting for 66% of
rhabdomyosarcomas.
• It includes the sarcoma botryoides described
in and spindle cell variants.
• The tumor occurs in children under age 10
years and typically arises in the nasal cavity,
orbit, middle ear, prostate, and paratesticular
region.

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 Embryonal rhabdomyosarcoma

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 Embryonal rhabdomyosarcoma

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 TUMORS OF SKELETAL MUSCLE
• Alveolar rhabdomyosarcoma is most common
in early to mid-adolescence and usually arises
in the deep musculature of the extremities.
• Histologically the tumor is traversed by a
network of fibrous septae that divide the cells
into clusters or aggregates; as the central cells
degenerate and drop out, with resemblance to
pulmonary alveolae.

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 Alveolar rhabdomyosarcoma

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 Alveolar rhabdomyosarcoma with
numerous spaces lined by tumor cells

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 TUMORS OF SKELETAL MUSCLE
• Pleomorphic rhabdomyosarcoma is
characterized by numerous large, sometimes
multinucleated, bizarre eosinophilic tumor
cells.
• This variant is rare, has a tendency to arise in
the deep soft tissue of adults and, as noted
earlier, can resemble malignant fibrous
histiocytoma histologically.

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 TUMORS OF SKELETAL MUSCLE
• Rhabdomyosarcomas are aggressive neoplasms and
are usually treated with a combination of surgery
and chemotherapy with or without radiation.
• The histologic variant and location of the tumor
influence survival.
• The botryoid subtype has the best prognosis,
followed by the embryonal, pleomorphic, and
alveolar variants.
• Overall, approximately 65% of children are cured of
their disease, but adults fare less well.

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 TUMORS OF SMOOTH MUSCLE

LEIOMYOMA
• Leiomyomas, the benign smooth muscle tumors,
often arise in the uterus where they represent the
most common neoplasm in women
• Leiomyomas may also arise in the erector pili
muscles found in the skin, nipples, scrotum, and
labia (genital leiomyomas) and less frequently
develop in the deep soft tissues.

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 TUMORS OF SMOOTH MUSCLE
• They are usually not larger than 1 to 2 cm in greatest
dimension and are composed of fascicles of spindle
cells that tend to intersect each other at right angles.
• The tumor cells have blunt-ended, elongated nuclei
and show minimal atypia and few mitotic figures.
• Solitary lesions are easily cured; however, they may
be so numerous that complete surgical removal is
impractical.

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 TUMORS OF SMOOTH MUSCLE
LEIOMYOSARCOMA
• Leiomyosarcomas account for 10% to 20% of
soft tissue sarcomas.
• They occur in adults and afflict women more
frequently than men.
• Most develop in the skin and deep soft tissues
of the extremities and retroperitoneum.

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 TUMORS OF SMOOTH MUSCLE
Morphology
• Leiomyosarcomas present as pain-less firm masses.
• Retroperitoneal tumors may be large and bulky and
cause abdominal symptoms.
• Histologically, they are characterized by malignant
spindle cells that have cigar-shaped nuclei arranged
in interweaving fascicles. Morphologic variants
include tumors with a prominent myxoid stroma and
others with epithelioid cells.
• Immunohistochemically, they stain with antibodies to
vimentin, actin, smooth muscle actin, and desmin.

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 TUMORS OF SMOOTH MUSCLE
• Treatment depends on the size, location, and
grade of the tumor.
• Superficial or cutaneous leiomyosarcomas are
usually small and have a good prognosis,
• large, retroperitoneal ones cannot be entirely
excised, and cause death by both local
extension and metastatic spread.

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 FIBROHISTIOCYTIC TUMORS
• Fibrohistiocytic tumors contain cellular
elements that resemble both fibroblasts and
histiocytes.

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 FIBROHISTIOCYTIC TUMORS
BENIGN FIBROUS HISTIOCYTOMA
(DERMATOFIBROMA)
• Benign fibrous histiocytoma is a relatively
common lesion that usually occurs in the
dermis and subcutis.
• It is painless and slow growing and most often
presents in mid-adult life as a firm, small (up
to 1 cm) mobile nodule.

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 FIBROHISTIOCYTIC TUMORS
Morphology
• Most benign fibrous histiocytomas consist of
a proliferation of bland spindle cells arranged
in a storiform pattern.
• These tumors have infiltrative margins;
common secondary findings include the
presence of foam cells, hemosiderin deposits,
multinucleated giant cells, and hyperplasia of
the overlying epidermis

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 FIBROHISTIOCYTIC TUMORS
MALIGNANT FIBROUS HISTIOCYTOMA
• Malignant fibrous histiocytoma refers to a group of
related soft tissue sarcomas characterized by
considerable cytologic pleomorphism, the presence
of bizarre multinucleate cells, and storiform
architecture.
• Malignant fibrous histiocytoma usually arises in the
musculature of the proximal extremities and the
retroperitoneum.
• Cutaneous variants have also been called atypical
fibroxanthomas.
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 FIBROHISTIOCYTIC TUMORS
Morphology.
• These tumors are usually large (5 to 20 cm), gray-
white unencapsulated masses but often appear
deceptively circumscribed.
• Malignant fibrous histiocytomas have been
categorized into storiform-pleomorphic, myxoid,
inflammatory, giant cell, and angiomatoid variants
based on their histologic features.
• The storiform-pleomorphic type is the most
common and as the name indicates is composed of
malignant spindle cells oriented in a storiform
pattern with scattered, large round pleomorphic cells
.
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 FIBROHISTIOCYTIC TUMORS
• Most variants of malignant fibrous histiocytoma,
except for the angiomatoid type, are aggressive,
recur unless widely excised, and have a metastatic
rate of 30% to 50%.
• However, cutaneous tumors rarely disseminate; the
angiomatoid variant is also indolent and in contrast
to the other types occurs in adolescents and young
adults.

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Malignant fibrous histiocytoma revealing fascicles of plump
spindle cells in a swirling (storiform) pattern, typical but not
pathognomonic of this neoplasm

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 REACTIVE PSEUDOSARCOMATOUS
PROLIFERATIONS
• Reactive pseudosarcomatous proliferations are non-
neoplastic lesions that either develop in response to some
form of local trauma (physical or ischemic) or are idiopathic.
• They are composed of plump reactive fibroblasts or related
mesenchymal cells.
• They are alarming because they develop suddenly and grow
rapidly;
• histologically, they cause concern because they mimic
sarcomas owing to their hyper cellularity, mitotic activity, and
a primitive appearance.
• Representative of this family of lesions are nodular fasciitis
and myositis ossificans.

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 Nodular Fasciitis
• Nodular fasciitis, also known as infiltrative or
pseudosarcomatous fasciitis, is the most common of
the reactive pseudosarcomas.
• It most often occurs in adults on the volar aspect of
the forearm, followed in order of frequency by the
chest and back.
• Patients typically present with a several-week history
of a solitary, rapidly growing, and sometimes painful
mass.
• Trauma is noted in only 10% to 15% of cases.

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 Nodular Fasciitis

Morphology
• Nodular fasciitis lesions arise in the deep dermis,
subcutis, or muscle.
• Grossly the lesion is several centimeters in greatest
dimension, is nodular in configuration, and has
poorly defined margins.
• Nodular fasciitis is richly cellular and consists of
plump, immature-appearing fibroblasts arranged
randomly (simulating cells growing in tissue culture)
or in short intersecting fascicles
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 Nodular fasciitis with plump, randomly oriented
spindle cells surrounded by myxoid stroma

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 Myositis Ossificans
• Myositis ossificans is distinguished from the other fibroblastic
proliferations by the presence of metaplastic bone.
• It usually develops in athletic adolescents and young adults
and follows an episode of trauma in more than 50% of cases.
• The lesion typically arises in the musculature of the proximal
extremities.
• The clinical findings are related to its stage of development; in
the early phase, the involved area is swollen and painful, and
within several weeks, it becomes more circumscribed and
firm.
• Eventually, it evolves into a painless, hard, well-demarcated
mass.

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Morphology
• Grossly, the usual lesions are 3 to 6 cm in greatest dimension.
• Most are well delineated and have soft, glistening centers and
a firm, gritty periphery.
• The microscopic findings vary according to the age of the
lesion; in the earliest phase, the lesion is the most cellular and
consists of plump, elongated fibroblast-like cells simulating
nodular fasciitis
• Morphologic zonation begins within 3 weeks; the center
retains its population of fibroblasts; however, it merges with
an adjacent intermediate zone that contains osteoblasts,
which deposit ill-defined trabeculae of woven bone.
• The most peripheral zone contains well-formed, mineralized
trabeculae that closely resemble cancellous bone.

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 FIBROMATOSES

• Superficial Fibromatosis (Palmar, Plantar, and

Penile Fibromatoses)
• Deep-Seated Fibromatosis (Desmoid Tumors)

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 Superficial Fibromatosis (Palmar, Plantar,
and Penile Fibromatoses)
• Palmar, plantar, and penile fibromatoses, more bothersome
than serious lesions, constitute a small group of superficial
fibromatoses.
• They are characterized by nodular or poorly defined broad
fascicles of mature-appearing fibroblasts surrounded by
abundant dense collagen.
• Immunohistochemical and ultrastructural studies indicate that
many of these cells are myofibroblasts.
• All forms of superficial fibromatosis affect males more
frequently than females.
• In about 20% to 25% of cases, the palmar and plantar
fibromatoses stabilize and do not progress, in some instances
resolving spontaneously. Some recur after excision,
particularly the plantar variant.

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 Superficial Fibromatosis (Palmar, Plantar,
and Penile Fibromatoses)
• In the palmar variant (Dupuytren contracture), there is
irregular or nodular thickening of the palmar fascia either
unilaterally or bilaterally (50%).
• Over a span of years, attachment to the overlying skin causes
puckering and dimpling. At the same time, a slowly
progressive flexion contracture develops, mainly of the fourth
and fifth fingers of the hand.
• Essentially similar changes are seen with plantar fibromatosis,
except that flexion contractures are uncommon and bilateral
involvement is infrequent.

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 Superficial Fibromatosis (Palmar, Plantar,
and Penile Fibromatoses)
• In penile fibromatosis (Peyronie disease), a
palpable induration or mass appears usually
on the dorsolateral aspect of the penis. It may
cause eventually abnormal curvature of the
shaft or constriction of the urethra, or both.

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 Deep-Seated Fibromatosis (Desmoid
Tumors)
• Deep-seated fibromatoses lie in the borderland
between nonaggressive fibrous tumors and low-
grade fibrosarcomas.
• They commonly present as large, infiltrative masses
that frequently recur after incomplete excision;
• They are composed of banal well-differentiated
fibroblasts that do not metastasize.
• They may occur at any age but are most frequent in
the teens to thirties.

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 Deep-Seated Fibromatosis (Desmoid
Tumors)

Desmoids are divided into extra-abdominal, abdominal, and

intra-abdominal, but all have essentially similar gross and
microscopic features.
• Extra-abdominal desmoids occur in men and women with
equal frequency and arise principally in the musculature of
the shoulder, chest wall, back, and thigh.
• Abdominal desmoids generally arise in the
musculoaponeurotic structures of the anterior abdominal wall
in women during or after pregnancy.
• Intra-abdominal desmoids tend to occur in the mesentery or
pelvic walls, often in patients having familial adenomatous
polyposis (Gardner syndrome)
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 Deep-Seated Fibromatosis (Desmoid
Tumors)
Morphology.
• 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.
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 Fibromatosis infiltrating between skeletal
muscle cells

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 FIBROSARCOMA
• Fibrosarcomas are rare but may occur anywhere in
the body, most commonly in the retroperitoneum,
the thigh, the knee, and the distal extremities.
• Many tumors previously considered fibrosarcoma
have been reclassified as aggressive fibromatosis
(desmoid), malignant fibrous histiocytoma,
malignant peripheral nerve sheath tumors, or
synovial sarcomas.

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 FIBROSARCOMA

Morphology
• 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 shows 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.
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 FIBROSARCOMA
• They are aggressive tumors, however,
recurring in more than 50% of the cases and
metastasizing in more than 25%.

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 Fibrosarcoma composed of malignant spindle
cells arranged in a herringbone pattern

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 SYNOVIAL SARCOMA
• Synovial sarcoma is so named because it was once
believed to recapitulate synovium, but the cell of
origin is still unclear.
• In addition, although the term synovial sarcoma
implies an origin from the joint linings, less than 10%
are intra-articular.
• Synovial sarcomas account for approximately 10% of
all soft tissue sarcomas and rank as the fourth most
common sarcoma.

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 SYNOVIAL SARCOMA

• Most occur in patients in their twenties to forties.

• The majority develop in the deep soft tissue in the
vicinity of the large joints of the extremities, and
about 60% to 70% involve the lower extremities,
especially around the knee and thigh.
• Patients usually present with a deep-seated mass
that has been noted for several years.
• Uncommonly, these tumors occur in the head and
neck or the different viscera.

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 SYNOVIAL SARCOMA
Morphology
• 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.

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 SYNOVIAL SARCOMA
• 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.

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 SYNOVIAL SARCOMA
• Lesions composed of spindled cells only, are
easily mistaken for fibrosarcomas or malignant
peripheral nerve sheath tumors.
• Immunohistochemistry is helpful in identifying
these tumors, since the tumor cells yield
positive reactions for keratin and epithelial
membrane antigen, differentiating these
tumors from most other sarcomas.

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 SUMMARY
• The most common soft tissue tumor is lipoma.
• The most common soft tissue sarcoma (malignant
soft tissue tumor) in the retroperitoneal region is
liposarcoma.
• Malignant fibrous histiocytoma is the most common
sarcoma of adulthood.
• Malignant fibrous histiocytoma is the most common
sarcoma that arises after radiation therapy.
• The most common malignant soft tissue tumor of
chidhood is rhabdomyosarcoma.

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 SUMMARY
• Embryonal rhabdomyosarcoma is the most
common type of RMS and has the best clinical
outcome.
• Alveolar RMS has the worst prognosis and is
likely to metastatize to LN.
• Alveolar type RMS is the most common RMS
encountered in adulthood.
• Dermatofibrosarcoma protuberans is the
borderline fibrohistiocytic tumor.
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