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Understand the classification and types of soft tissue tumours

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Title: Understand the classification and types of soft tissue tumours


1
SOFT TISSUE TUMOURS By Dr. Fahd Al-Mulla
  • Understand the classification and types of soft
    tissue tumours
  • Understand the necessity for a team-approach
  • Correlate Pathological findings with clinical
    presentation (Clinico-pathological correlation)
  • Know the most relevant information and know it
    well
  • ALL INFORMATION IS ON WEBSIT
  • http//www.tumours.com

2
Soft Tissue Tumours Definition
Mesenchymal proliferations that occur in the
extraskeletal, nonepithelial tissues of the body,
excluding the viscera, coverings of the brain,
and lymphoreticular system.
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4
The cause of most soft tissue tumors is unknown.
There are documented associations, however,
between radiation therapy and rare instances in
which chemical burns, heat burns, or trauma were
associated with subsequent development of a
sarcoma. Exposure to phenoxyherbicides and
chlorophenols has also been implicated in some
cases. Kaposi sarcoma in patients with AIDS and
in immunosuppressed patients is related to
viruses and defective immunocompetence. Most soft
tissue tumors occur sporadically, but a small
minority are associated with genetic syndromes,
the most notable of which are neurofibromatosis
type 1 (neurofibroma, malignant schwannoma),
Gardner syndrome (fibromatosis), Li-Fraumeni
syndrome (soft tissue sarcoma), and
Osler-Weber-Rendu syndrome (telangiectasia).
5
  • More recently, Enzinger and Weiss proposed a
    classification system based on histogenic origin.
  • Problems
  • It is very difficult for competent pathologists
    to agree on the histogenesis of these tumors.
    Some sarcomas have multiple cell types present in
    different areas of the tumor.
  • Many tumors are so undifferentiated that to
    subclassify them into their histogenic type is
    close to impossible, even with specialized
    techniques such as electron microscopy and
    immunohistochemistry However, the major drawback
    of this classification system is that it does not
    really take into account the grade of the tumor
    and its implications for prognosis.

6
After the histologic type of soft-tissue sarcoma
has been determined, the tumor is graded 1 to 4,
depending on its degree of differentiation (How
similar it is to the original tissue) The
majority of histologic types can be low-,
intermediate-, or high-grade (grade 1, 2, or 3,
respectively). However, some soft-tissue sarcomas
such as well-differentiated liposarcomas and
myxoid liposarcomas are always low-grade, whereas
others such as rhabdomyosarcoma, synovial
sarcoma, mesenchymal chondrosarcoma, and
extraskeletal Ewings and osteosarcomas are
always high-grade The American Joint Committee
on Cancer (AJCC) has developed a
clinicopathologic staging system that depends
primarily on the grade and size of soft-tissue
sarcomas
7
American Joint Committee on Cancer AJCC of soft
tissue sarcomas classification
8
FATTY TUMOURS
Benign tumors of fat, known as lipomas, are the
most common soft tissue tumor of
adulthood. 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. Infrequently,
lipomas are large, intramuscular, and
circumscribed. Histologically, they consist of
mature fat cells with no evidence of pleomorphism
or abnormal growth. Lipomas are soft, mobile, and
painless (except angiolipoma) and are usually
cured by simple excision. conventional lipomas
often show rearrangements of 12q14-15, 6p, and
13q, and spindle cell and pleomorphic lipomas
have rearrangements of 16q and 13q .
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Liposarcoma Liposarcomas are one of the most
common sarcomas of adulthood and appear in the
forties to sixties they are uncommon in
children. They usually arise in the deep soft
tissues of the proximal extremities and
retroperitoneum and are notorious for developing
into large tumors. MORPHOLOGY. Histologically,
liposarcomas can be divided into
well-differentiated, myxoid, round cell, and
pleomorphic variants. The cells liposarcomas are
readily recognized as lipoblasts, which mimic
fetal fat cells
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Fibrous tumours and Fibrohistiocytic tumours
Fibromatoses 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 fascicles of
mature-appearing fibroblasts surrounded by
abundant dense collagen. Immunohistochemical and
ultrastructural studies indicate that many of
these cells are myofibroblasts Examples
Dupuytren contracture, plantar fibromatosis
DEEP-SEATED FIBROMATOSIS (DESMOID
TUMORS) Biologically, deep-seated fibromatoses
lie in the interface between exuberant fibrous
proliferations and low-grade fibrosarcomas. On
the one hand, they present frequently as large,
infiltrative masses that may recur after
incomplete excision, and on the other, they are
composed of banal well-differentiated fibroblasts
that do not metastasize.
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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. 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 discloses all degrees of
differentiation, from slowly growing tumors that
closely resemble cellular fibromas 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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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.
Rhabdomyosarcoma Rhabdomyosarcomas, the most
common soft tissue sarcomas of childhood and
adolescence, usually appear before age 20. They
may arise in any anatomic location, but most
occur in the head and neck or genitourinary
tract. 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
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Cytogenetics play an important role in confirming
the diagnosis rhabdomyosarcoma. Alveolar
rhabdomyosarcoma is associated with a specific
translocation, t(213)(q37q14) or its variant
t(113)(p36q14). Embryonal rhabdomyosarcoma
often shows loss of heterozygosity for 11p, but
there is no specific cytogenetic or molecular
marker comparable to those for alveolar RMS.
20
TUMORS OF SMOOTH MUSCLE Leiomyomas 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 skin and subcutis from the arrector pili
muscles found in the skin, nipples, scrotum, and
labia (genital leiomyomas) and less frequently
develop in the deep soft tissues. 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. Leiomyosarcoma Leiomyosarcomas
account for 10 to 20 of soft tissue sarcomas.
Most develop in the skin and deep soft tissues of
the extremities and retroperitoneum.
Microscopically, the lesion is composed of
interlacing fascicles of mildly pleomorphic,
spindle cells with blunt-ended nuclei and
eosinophilic cytoplasm. Average mitotic rate was
3 per 10 hpf. Geographic areas of necrosis is
present
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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. MORPHOLOGY. The histologic
hallmark of synovial sarcoma is the biphasic
morphology of the tumor cells (i.e.,
epithelial-like and spindle cells). Immunohistoch
emistry is helpful in identifying these tumors,
since the epithelioid and spindle cell portions
yield positive reactions for keratin and
epithelial membrane antigen, differentiating
these tumors from most other sarcomas.
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24
  • In Summary
  • Soft tissue benign tumours outnumber malignant
  • Tumours 1001
  • -They are aggressive if malignant
  • -Be a good clinician and always correlate
    clinical
  • with pathological findings.
  • -Work together as a multi-disciplinary team
  • Any Questions??

25
Bone Tumours WHAT SHOULD YOU KNOW
  • Understand the clinical algorithm
  • Correlate clinical presentation with radiological
    features
  • Understand the classification and types of bone
    tumours
  • Comprehend the management of bone tumours
  • Understand the necessity for a team-approach
  • Correlate Pathological findings with clinical
    presentation (Clinico-pathological correlation)

26
CLASSIFICATIONS OF PRIMARY TUMOURS INVOLVING
BONES Metastatic cancers are the most frequent
malignant tumors found in bone
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SITE OF LONG BONE INVOLVEMENT (most primary bone
tumors have favored sites within long bones this
may provide a clue to diagnosis).
Diaphyseal lesions centered in the
cortexAdamantinoma, osteoid osteoma
Diaphyseal intramedullary lesionsFavored
location for Ewing's sarcoma, lymphoma, myeloma.
Common for fibrous dysplasia and enchondroma
Metaphyseal intramedullary lesionsOsteosarcoma
is usually centered in the metaphysis.
Chondrosarcoma and fibrosarcoma often present as
metaphyseal lesions. Osteoblastoma, enchondroma,
fibrous dysplasia, simple bone cyst, and
aneurysmal bone cyst are common in this location.

Metaphyseal lesions centered in the
cortexClassic location for a non-ossifying
fibroma (NOF). Also, a common site for osteoid
osteoma.
Epiphyseal lesionsChondroblastoma (Ch) and
Giant Cell Tumor (GCT) are almost invariably
centered in the epiphysis. Chondroblastoma is a
rare tumor seen in children and adolescents with
open growth plates. GCT is the most common tumor
of epiphyses in skeletally mature individuals
with closed growth plates. GCT often shows
metaphyseal extension.
Metaphyseal exostosisOsteochondroma
29
  • But remember
  • Listen to your patients (Is the lesion painful,
    What is the age of the patient)
  • Listen to the Radiologist (patterns of growth and
    ask to see the films)
  • Listen to the surgeons (rapid growth, involve the
    periosteum, soft tissue)

30
An 11-year-old male was seen in consultation for
an increasingly painful distal femoral lesion
associated with a soft tissue mass.
Plain radiograph shows an ill-defined destructive
tumor in the distal femur. Fluffy radiodense
infiltrates represent malignant tumor osteoid.
Biopsy material shows two major components of
this neoplasm highly pleomorphic cells and
haphazard deposits of osteoid. Note that the
malignant cells fill the spaces between osteoid
deposits. Lace-like osteoid deposition is very
characteristic of this neoplasm.
31
The tan-white tumor fills most of the medullary
cavity of the metaphysis and proximal diaphysis.
It has infiltrated through the cortex, lifted the
periosteum, and formed soft tissue masses on both
sides of the bone.
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34
A 17-year-old male presented with increasing pain
in the left upper arm of approximately 3 months'
duration and a recent onset of low-grade fever.
On physical examination, there was some local
tenderness and soft tissue swelling over the
proximal and mid thirds of the left humerus.
Most important here is the patient's age and
short duration of symptoms.
Plain radiograph shows a large ill-defined,
destructive, diaphyseal intramedullary lesion
with permeative pattern of bone destruction and
periosteal reaction of a "hair-on-end" type. The
lesion is associated with a soft tissue mass.
Biopsy material showed a highly cellular,
infiltrative neoplasm consisting of sheets of
tightly packed, round cells with very scant
cytoplasm ("round blue cell tumor"). Occasional
Homer-Wright rosettes were identified. Other
fields showed extensive necrosis.
The cell population consisted of two distinct
cell types the larger round cells with a high
N/C ratio, fine chromatin pattern and occasional
small, inconspicuous nucleoli, and the smaller
and darker cells with eosinophilic cytoplasm and
hyperchromatic, "shrunken" nuclei (degenerated
cells, a typical finding in this entity). Mitotic
rate averaged 2 per 10 hpf.
35
The following studies are required to support
the diagnosis of ES and PNET Demonstration of
t(1122) or EWS-FLI-1 fusion transcript (present
in both ES and PNET) Immunostains(both ES and
PNET are positive for CD99/O13. In addition, PNET
shows positive staining with neural markers)EM
(ES cells are undifferentiated and show prominent
glycogen deposits PNET shows neural
differentiation)
36
A 16-year-old boy was seen in consultation for
increasing pain in the mid upper arm.
Characteristically, the pain intensified at night
and subsided with aspirin.
Plain film shows a small, intracortical,
radiolucent focus (nidus), surrounded by dense
reactive periosteal bone. The lesion is located
in the mid portion of the humeral shaft.
If the nidus is removed intact, it appears as a
circumscribed portion of red, trabecular bone,
usually less than 1cm in size.
Low-power view shows the lesional tissue
("nidus"), well demarcated from the surrounding
sclerotic bone.
The lesion is composed of thin, often
interconnected spicules of osteoid and woven bone
rimmed by osteoblasts. Osteoclast-like giant
cells can be seen. Intervening fibrous stroma
shows prominent vascularity.
37
A 39-year-old female gave a 2-month history of
increasing pain in her knee. There was no
evidence of joint effusion. Laboratory work-up
showed normal serum levels of calcium, phosphate
and alkaline phosphatase
Plain radiograph demonstrated a well defined,
lytic lesion eccentrically located in the distal
femoral epiphysis with subchondral and
metaphyseal extension. There was associated focal
thinning of the cortex
Curettage specimen consisted of fragments of
soft, hemorrhagic, tan-brown tissue with some
firm areas and yellowish speckles. Microscopic
examination showed a cellular lesion composed of
numerous multinucleated giant cells in a
background of small, ovoid, mononuclear stromal
cells
Stromal cells had poorly defined cytoplasmic
borders and bland nuclei resembling those of
giant cells. Mitoses were easily found averaging
4 per 10 hpf. However, no atypical mitoses were
identified.
38
A 45-year old female presented with increasing
pain and swelling around the knee. She mentioned
that the symptoms had progressed over a 4-month
period. Age of the patient is an important
diagnostic clue. If a pathologic fracture is
excluded, pain and swelling imply active growth
of the lesion.
Plain film demonstrates a large, lobulated,
ill-defined lesion centered in the distal femoral
metaphysis. There is endosteal scalloping and
periosteal thickening. Central stippled and "ring
and arc" calcifications are apparent and are
typical of cartilaginous matrix. Small
radiolucent areas are seen at the periphery of
the lesion.
Low magnification shows a moderately cellular,
lobulated cartilaginous tumor.
High-power view shows scattered plump, moderately
pleomorphic chondrocytes. Binucleated cells are
present. Mitotic rate averaged 1 per 10 hpf.
39
The aggressiveness of chondrosarcomas can be
predicted by their histologic grade. Grading
system is based on three parameters cellularity,
degree of nuclear atypia and mitotic activity.
Grade 1 (low-grade)Very similar to enchondroma.
However, the cellularity is higher, and there is
mild cellular pleomorphism. The nuclei are small
but often show open chromatin pattern and small
nucleoli. Binucleated cells are frequent. Mitoses
are very rare. Grade 1 chondrosarcomas are
locally aggressive and prone to recurrences, but
usually do not metastasize.
Grade 2 (low-grade)The cellularity is higher
than in Grade 1 tumors. Characteristic findings
are moderate cellular pleomorphism, plump nuclei,
frequent bi-nucleated cells, and occasional
bizarre cells. Mitoses are rare. Foci of myxoid
change may be seen. Unlike Grade 1 tumors, about
10 to 15 of Grade 2 chondrosarcomas produce
metastases.
Grade 3 (high-grade)Characteristic findings are
high cellularity, marked cellular pleomorphism,
high N/C ratio, many bizarre cells and frequent
mitoses (more than 1 per hpf). These are high
grade tumors with significant metastatic
potential.
40
A 14-year-old female was seen in consultation for
an increasingly painful left humeral lesion
associated with mild joint effusion. Pay
attention to the patient's age, skeletal
location, and the presence of joint effusion,
which may complicate epiphyseal lesions.
Plain radiograph showed an irregular, but
circumscribed, lytic epiphyseal lesion surrounded
by reactive bone sclerosis. There was no evidence
of bone expansion, and the cortex was intact. The
growth plates were open.
The cytoplasmic borders were very distinct with
multiple foci of "chicken-wire" calcification
(calcified reticulin network around individual
tumor cells).
41
A 20-year-old male presented with a painless,
hard subcutaneous mass in the popliteal fossa. He
stated that the mass had been present for several
years and did not change in size. Two words,
"painless" and "non-growing" (or very slow
growing), suggest that the lesion described here
is probably benign.
Plain radiograph demonstrated a pedunculated bony
outgrowth at the proximal tibial metaphysis. The
lesion had a uniform, cartilagenous cap with
stippled calcifications. The tibial cortex and
medulla were continuous with those of the lesion.
The specimen consisted of a pedunculated lesion,
3 x 3 x 2cm, with a lobulated cartilage cap
measuring up to 0.9cm in thickness
Osteochondroma, the most common benign bone
tumor, is not a neoplasm but a hamartoma. It is
thought to arise from a portion of growth plate
cartilage entrapped beneath the periosteum during
skeletal growth. These entrapped pieces continue
to grow and ossify at the same rate as the
adjacent bone. When skeletal maturity is reached,
osteochondromas usually stop growing.
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43
An incidental finding of a bone lesion in the
distal femur of a 38-year old female. The lesion
was completely asymptomatic.
Plain radiograph showed an intarmedullary zone of
stippled and ring-shaped calcifications in the
distal femoral metaphysis. This mineralization
pattern with radiodense stipples and rings is
characteristic of mature hyaline cartilage.
Low-power microscopic examination of the biopsy
specimen shows three characteristic features of
this lesion a) vague lobularity b) abundant
cartilaginous matrix, which can be focally
calcified c) low cellularity.
High-power view shows clustered and scattered
chondrocytes with small, uniform, darkly stained
nuclei. Occasional bi-nucleated chondrocytes are
present. Importantly, there were no mitotic
figures.
44
A 17-year-old male presented with a slowly
enlarging, painful lesion of the right clavicle.
Plain radiograph reveals a circumscribed,
loculated, radiolucent lesion producing blowout
expansion of the bone.
Gross photograph shows a spongy, expansile lesion
containing multiple, blood-filled cavities of
varying sizes.
Low-power view demonstrates blood-filled cystic
spaces without recognizable epithelial lining.
45
Audit
Nursing And Support staff
Radiologist
psychiatrist
Cytopathologist
Oncologist
BoneTumours Diagnosis Treatment
Surgeon
Histopathologist
Molecular Pathologist Geneticist
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