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S. Sarcomas
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C hapter Outline
·
Sarcoma
·
Liposarcoma
·
Fibrosarcoma
·
Malignant Fibrous
Histiocytoma
·
Leiomyosarcoma
·
Rhabdomyosarcoma
SARCOMA
Sarcomas can arise from bone or soft tissue. Osteosarcoma
is the commonest of all sarcomas. Soft tissue sarcoma (STS)
which is arising from mesenchyma is the one which will be
discussed in this chapter. STS are aggressive tumours which
needs multimodality therapeutic approach.
Sarcoma occurs in younger age group compared to carcinoma
with fish fleshy gross look with haemorrhage and necrosis. It
shows rapid growth, with tendency to show early blood spread.
Lymphatic spread is rare even though few sarcomas can spread
through lymphatics along with blood spread. Blood spread
commonly occurs to lung but liver, skin, brain can also get involved.
Soft tissue is the tissue that connect, surround and support
the skeletal system which is non-epithelial, extra-skeletal tissue
excluding reticuloendothelial system, glial tissue but it also
includes peripheral nervous tissue by convention. Embryologically it is mainly from mesoderm but few from neuroectoderm.
STS are named based on their tissue which it resembles.
Liposarcoma—fat; fibrosaroma—fibroblast; malignant fibrous
histiocytoma—mesenchyma/histiocytes; leiomyosarcoma—
smooth muscle; rhabdomyosarcoma—skeletal muscle; chondrosarcoma—chondroblast; angiosarcoma—blood vessels.
Features
Sarcomas are much lesser in incidence compared to
carcinomas.
It occurs in younger age group compared to carcinomas.
They can arise from bone (osteosarcoma) or from any soft
tissues (soft tissue sarcomas) (Mesenchymal tissue).
They are much more aggressive compared to carcinomas.
They are rapidly growing tumours with fleshy appearance.
They are not encapsulated but often are having pseudocapsule.
They spread through blood especially to lungs often also to
other organs.
Lymphatic spread is not common with certain exceptions.
Main method of treatment is surgery, i.e. wide excision,
amputation.
·
Chondrosarcoma
·
Haemangiosarcoma
·
Synovial Sarcoma
·
Malignant Peripheral
Nerve Sheath Tumour
·
Kaposi’s Sarcoma
In inoperable cases debulking is the accepted method of
treatment.
Chemotherapy is the adjuvant therapy.
Commonest sarcoma of bone is osteosarcoma
Note:
Commonest malignancy of bone is secondaries.
IMPORTANT FEATURES OF SARCOMA
B
x More aggressive
x Not very much radiosensitive
x Painless soft tissue mass is
the presentation
SOFT TISSUE SARCOMA
B
x 1% of adult malignancy
x 15% of paediatric malignancies
x Incidence:
– 35% occurs in lower limb (commonest site)
– 15% upper limb, 15% retroperitoneum
– 10% trunk, 15% viscera, 10% other areas
x Soft tissue sarcoma (STS) arises from pluripotent mesenchymal
stem cell without in situ changes. Transformation from benign
lesion to sarcoma is not observed/disproved now except MPNST
(Malignant peripheral nerve sheath tumour)
x Ratio of soft tissue sarcoma to bone sarcoma is 3:1
x Soft tissue sarcoma is more common in males (4:1)
x Ratio of benign soft tissue tumour to malignant soft tissue tumour
is 100:1. Most of the sarcomas arise as de novo. Per se benign will
not turn into malignancy, as it is now found that cell of origin to
begin with multiples with anisocytosis in soft tissue sarcoma. In
olden days it was accepted to consider benign (precursor tumour)
turning into malignancy like lipoma turning into liposarcoma
which is disproved now. But this argument does not hold good
for nerve sheath tumour and probably lymphoedema turning into
lymphangiosarcoma (post-mastectomy)
x 50% of STS occurs in extremities called as extremity STS; 35%
lower and 15% upper limb
x 3% of sarcoma spreads to lymph nodes
x Soft tissue tumour >5 cm should be biopsied in suspicious of
sarcoma
Commonest soft tissue sarcoma is liposarcoma / malignant
x Rapidly spreading
x Blood spread
x Very vascular
fibrous histiocytoma (MFH – 25%) overall; in the extremities
both MFH and liposarcoma; in the retroperitoneum it is
liposarcoma.
Commonest visceral (GIT) sarcoma is leiomyosarcoma.
In genitourinary system leiomyosarcoma is commonest in
adults and rhabdomyosarcoma in paediatric age group; in
uterus leiomyosarcoma; in myocardium angiosarcoma; in
hand and foot synovial sarcoma; in skin Kaposi’s sarcoma;
in head and neck region angiosarcoma.
Fig. 1.553: Soft tissue sarcoma foot. Note the vascularity.
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Fig. 1.554: Osteosarcoma of the upper end of humerus in an
adolescent boy.
Incidence of STS
In adults
MFH and liposarcoma—35–45%.
Rhabdomyosarcoma—10%.
Leiomyosarcoma—9–15%.
Synovial sarcoma—7%.
Malignant peripheral nerve sheath tumour (MPNST)—6%.
Fibrosarcoma—5%.
In children
Rhabdomyosarcoma, neuroblastoma are common tumours.
AETIOLOGY
B
x Genetic
– von Recklinghausen disease
– Gardner’s syndrome
– Tuberous sclerosis
– Basal cell naevus syndrome
– Li-Fraumeni syndrome
x Chemicals—PVC, tetrachlorodibenzodioxin, arsenic
x Viral—HIV in Kaposi’s sarcoma, cytomegalovirus
x Ionising radiation—malignant fibrous histiocytoma (p53)
x Lymphangiosarcoma in post-mastectomy lymphoedema—
Stewart—Treves syndrome.
x Osteogenic sarcoma in Paget’s disease of bone/exposure to
radium
x Retinoblastoma associated sarcoma
x Gorlin’s syndrome
x Thorotrast, vinyl chloride, arsenic, pesticides
297
CHAPTER 1S General Surgery: Sarcomas
Fig. 1.555: Osteosarcoma upper tibia. Note the size of the tumour
and ulceration. It is very vascular.
A
Figs. 1.556
A and B: X-ray pictures of osteosarcoma in
B
tibia and femur.
Clinical Features of Soft Tissue Sarcoma
Painless swelling of short duration with progressive increase
in size—soft tissue mass. Thirty percent of patients may
present as pain during first evaluation.
Compression of adjacent structures
Smooth, firm/hard, warm and vascular
Features of secondaries in lung—cough, haemoptysis and
chest pain. Lung is the commonest site of secondary.
Secondaries in liver as a principal site especially in visceral
STS.
There are no reliable findings to distinguish benign from
malignant swellings.
One has to maintain a high index of suspicion in any soft
tissue mass deep-to-deep fascia, any soft tissue mass >5
cm, any new enlarging or symptomatic soft tissue mass.
Note:
• Many liposarcomas arise at sites devoid of adipose tissue. Most rhab-
domyosarcomas arise in locations that lack voluntary muscle.
• 40% of soft tissue sarcomas are more than 10 cm in size at the time of
presentation; 30% are 5-10 cm and 30% are less than 5 cm.
A
Figs. 1.557A and B: Soft tissue sarcoma upper chest wall. Note the
All things are difcult before they are easy.
B
vascularity and extent.
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Investigations
B
x To obtain a tissue diagnosis
x To determine the “exact extent” of the tumour
x To evaluate metastatic disease
x Other investigations
Tissue Diagnosis
Incision biopsy is the most reliable method of diagnosis. It
provides adequate tissue sample.
SRB’s Manual of Surgery
B
x It is the ideal tool to conclude sarcoma histologically
x Incision should be placed in such a way that it can be included in
x One should achieve absolute/adequate haemostasis to avoid
x Proper site of incision biopsy should be decided
x One should use shortest possible route to tumour while taking
x Smallest longitudinal incision is used to provide adequate
x Transverse incision is contraindicated in the limbs except over
x Minimal tissue disturbance and avoiding raising of flaps are
x It is better to use cold knife
x One should avoid crushing/distorting the specimen
x Frozen section/Imprint specimen can be used to avoid sampling
x Drains are not used routinely in incision biopsy. If used exit
x Excise the biopsy tract and drain site enbloc during the definitive
x Immunohistochemistry and cytogenetics are possible
PREOPERATIVE EVALUATION OF STS IS DONE:
INCISION BIOPSY FOR SOFT TISSUE SARCOMA
wide tumor excision at a later period—as biopsy track is always
contaminated
haematomas as tumours are vascular
incision biopsy; should not violate more than one compartment;
should avoid neurovascular bundle. (Injury to vessels and nerves
should be avoided)
specimen (Incision should be longitudinal in limbs)
the flexures
crucial (Flaps should not be undermined)
error.
near/close to the wound and not away/distant from the incision
biopsy wound.
procedure.
Trucut biopsy/core needle biopsy is an acceptable first
diagnostic step as it is technically easier, not costly, with
fewer complications. But it is not useful in visceral STS. Again
if it is inadequate one should not be hesitant to go for incision
biopsy at the earliest. It is done using 14 gauge needle; often
US/CT guide is used.
Excision biopsy is done only if the tumor size is <3 cm which
is cutaneous or subcutaneous wherein wide local re-excision
is possible. Otherwise excision biopsy should be avoided as it
may contaminate the tumour bed and restricts the therapeutic
options.
Note:
FNAC is of less value in STS. It is significant only if it positive. It is useful in
local or distant recurrences in documented sarcoma patients, or to evaluate
nodal status if enlarged. CT guided FNAC is useful for retroperitoneal/intraabdominal sarcomas.
Assessment of the Extent of Tumour
Imaging in STS provides a 3–dimensional extent of the tumour
and helps in accurate planning of surgical procedure. But it
does not reliably distinguish between a benign and malignant
process. It assesses macroscopic and not microscopic extent of
the disease. Imaging is essential for metastatic work up.
MRI is the investigation of choice as it determines the
vascularity, relation to vessel and fascial planes (extent and
invasion). Advantages of MRI are—imaging of choice in STS,
excellent soft tissue delineation, without radiation, multiplanar
imaging possible. Images of skip metastases are possible.
Disadvantages of MRI are—cost, bone involvement is poorly
delineated, claustrophobia, not possible in presence of metal
implants and pacemakers.
CT scan also can be used to see the extent and invasion but not
equivalent to MRI. CT scan helps in identifying presence and
extent of the soft tissue mass, status of the adjacent structures,
with mandatory contrast enhancement isodense masses,
vasculature are better delineated. SP
reconstruction feasible. Advantages of CT are—easy availability,
ECT—3 dimensional
Fig. 1.558: Incision for biopsy in STS should be longitudinal so that it
can be included in future surgical wide excision. Never place transverse
incision for biopsy.
Fig. 1.559: MRI of STS leg.
Fig. 1.5 60: MRI picture of the soft tissue tumour thigh encasing vessels
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partially and eroding the bone. Patient needed high level, above-knee
amputation.
X-ray of part is not necessary; it is only used in initial phases
to differentiate STS from bony lesion.
US is less sensitive investigation. It is useful in extremity
lesions to assess vascular system. It is initial/first investigation
done in GI leiomyosarcoma/retroperitoneal sarcoma. It is
useful for serial studies, guided trucut biopsy.
Evaluation of Metastatic Disease
Chest X-ray is done to look for secondaries.
CT chest is ideal to see early lung secondaries. It is done in all
deep seated, high grade and tumour more than 5 cm in size.
US abdomen is sufficient to check liver secondaries. But CT
abdomen may be better choice. Often CT pelvis is also added.
PET scan or Integrated PET scan (with CT) is often useful to
identify metastatic diseases.
Other Investigations
Radionuclide scintigraphy (Gallium-67).
-p-MRS (p-Magnetic Resonance Spectroscopy) and FDG
(Fluor-2-Deoxy Glucose) PET are done to assess the
metabolic activity of tumour.
Immunohistochemistry and FISH (fluorescence in situ
hybridization).
Tumour markers.
Haematocrit, peripheral smear, ESR, serum alkaline
phosphatase, serum creatinine.
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CHAPTER 1S General Surgery: Sarcomas
Fig. 1.561: CT picture of retroperitoneal leiomyosarcoma.
relatively cost effective, best to demonstrate bony involvement,
useful for guided biopsies.
Disadvantages of CT are—cross-
sectional imaging, ionizing radiation, inferior soft tissue detail.
Angiogram is traditionally used to delineate adjacent
vasculature. It is upstaged by CECT/MRA. It is not necessary
to do regularly in all cases but when there is a need for
accurate assessment of vasculature it is ideal. It is used in
intra-arterial chemotherapy for unresectable tumours.
CT abdomen is better in GI/retroperitoneal sarcomas.
Staging
Staging of the soft tissue tumour is done depending on the
tumour size, nodal status, metastasis and histological grading
of the tumour (GTNM staging).
Note:
• STS is an aggressive, invasive, destructive growth with high recurrence
and distant metastases rate. Tumour shows fish flesh cut appearance.
• Basis used for classification of STS—STS being highly heterogeneous
group of tumour is classified based on adult tissue it resembles; i.e the
type of tissue formed rather than from the type of origin.
• Grade is the single most important factor in staging. It denotes the
“biological aggressiveness” of the sarcoma. It predicts the likelihood
of metastases.
• Nodal metastases are rare in STS (3%). It has the poor prognosis.
• M1 is – Distant metastases.
A B C
Figs. 1.562A to C: (A) Recurrent sarcoma buttock; (B) sarcoma thigh; (C) sarcoma right proximal thigh.
Knowledge is re and it is antidote to fear.
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(G) TNM STAGING OF SOFT TISSUE SARCOMA (OF TRUNK AND EXTREMITIES) (AJCC, 8TH EDITION, 2018)
B
T – Tumour (T)
TX - Primary tumor cannot be assessed
T0 - No evidence of primary tumor
T1 - Tumor 5 cm or less in greatest dimension
T2 - Tumor more than 5 cm and less than or equal to 10 cm in greatest dimension
T3 - Tumor more than 10 cm and less than or equal to 15 cm in greatest dimension
T4 -Tumor more than 15 cm in greatest dimension
T Suffix (m) Select if synchronous primary tumors are found in single organ
N - Nodes (N)
N0 - No regional lymph node metastases or unknown lymph node status
N1 - Regional lymph node metastasis
SRB’s Manual of Surgery
N Suffix (sn) if regional lymph node metastasis identified by SLN biopsy only
(f) if regional lymph node metastasis identified by FNA or core needle biopsy only
M - Distant Metastasis (M)
M0: No distant metastases
M1: Presence of disatant metastases
The terms pM0 and MX are not valid categories in the TNM system.
Assignment of the M category for clinical classification may be cM0, cM1, or pM1
cM0 - No distant metastasis
cM1- Distant metastasis
pM1- Distant metastasis, microscopically confirmed
Definition of FNCLCC (Fédération Nationale des Centres de Lutte Contre le Cancer)
Histologic Grade (G)
The FNCLCC grade is determined by three parameters: Differentiation, mitotic activity, and extent of necrosis.
Each parameter is scored as follows differentiation (1–3), mitotic activity (1–3), and necrosis (0 –2)
The scores are added to determine the grade
GX - Grade cannot be assessed
G 1 – Total differentiation, mitotic count and necrosis score of 2 or 3
G 2 – Total differentiation, mitotic count and necrosis score of 4 or 5
G 3 – Total differentiation, mitotic count and necrosis score of 6, 7, or 8
Tumor Differentiation score
1 - Sarcomas closely resembling normal adult mesenchymal tissue (e.g. low grade leiomyosarcoma)
2 - Sarcomas for which histologic typing is certain (e.g. myxoid/round cell liposarcoma)
3 - Embryonal and undifferentiated sarcomas, sarcomas of doubtful type, synovial sarcomas, soft tissue osteosarcoma, Ewing sarcoma/
primitive neuroectodermal tumor (PNET) of soft tissue
Mitotic Count (Score)
In the most mitotically active area of the sarcoma, 10 successive high-power fields (HPF; one HPF at 400 × magnification = 0.1734 mm
assessed using a 40 × objective.
MitoticCountScore:1:0–9mitosesper10HPF.2:10–19mitosesper10HPF.3:≥20mitosesper10HPF.
Tumor Necrosis Score
0
–Nonecrosis.1–<50%tumornecrosis.2–≥50%tumornecrosis.
Staging
IA: T0, N0, M0, GX/G1.
IB: T1,2,3; N0, M0, GX/G1.
II: T1, N0, M0, G2/G3.
IIIA: T2, N0, M0, G2/G3.
IIIB: T3,4; N0, M0, G2/G3.
IV: Any T, Any N, M0, Any G; Any T, Any N, M1, Any G.
Note: Staging of STS differs in thorax, abdomen and heads and neck.
2
) are
DIFFERENTIAL DIAGNOSIS FOR SOFT TISSUE SARCOMA
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B
x Haematoma
x Aneurysm
x Abscess
x Myositis
Treatment
Principles of Treatment
Surgery is the main treatment modality. Amputation
rate for STS has come down drastically from 50% in
1960 to 5% at present. It is also because of proper
adjuvant radiotherapy following function/limb sparing
complete excision, application of microvascular surgeries.
Neoadjuvant chemotherapy, perioperative/postoperative RT
also play a major role.
In low grade tumour without any spread—functional/limb
sparing complete wide excision is sufficient without any
adjuvant therapy. If microscopic margin is positive for tumour
then postoperative External Beam RT (EBRT) is given.
In high grade tumour <5 cm size, function sparing wide
excision with more than 1 cm clearance margin is sufficient.
If clearance margin is less than 1 cm or shows microscopic
positive margin, then perioperative brachytherapy OR
postoperative EBRT is given.
In high grade tumour which is between 5–10 cm size, function/
limb sparing complete wide excision with perioperative
brachytherapy OR postoperative EBRT is given.
In high grade tumour more than 10 cm in size, initially
neoadjuvant chemotherapy; then functional/limb sparing
complete wide excision with postoperative brachytherapy
and EBRT should be given.
All limbs should be conserved if possible but with curative intent.
Surgery
Enneking classification of surgical procedures
Intralesional excision—done inside pseudocapsule, very high
recurrence, 100%—not used.
Marginal excision—en bloc resections through the reactive
zone—high recurrence rate 70%
Wide excision means en bloc resection done through normal
tissues beyond the reactive zone; it means if the margin is
less than 5 cm; tumour is never visualised during surgery; it
has local recurrence rate of 30%. Wide margin is classified
as adequate if margin is at least beyond 1cm outside the
reactive zone or inadequate if margin is within 1 cm.
Radical excision—if the margin is more than 5 cm outside
the reactive zone. It is like compartment excision with very
low recurrence rate.
Other procedures
Compartmental excision; function/limb sparing.
Vascular resections with vascular reconstruction.
Amputation.
FIg. 1.563: Different surgical approaches for STS.
Note:
Limb sparing; function preserving; margin free wide excision—is the new
trend.
A thin barrier is considered to be a 2 cm thickness of normal tissue;
a thick barrier is 3 cm thickness; and joint cartilage is said to be
equivalent to a 5 cm thickness margin. A surgical margin that
is outside a barrier, with normal tissue between the barrier and
the reactive zone of the tumour, is considered to be curative.
Wide local excision with clearance of 2 cm (minimum need is
1 cm) or more with preservation of function is needed. Depth
clearance is also important. 3–5 cm clearance even though
was practiced in olden days, is not necessary.
Compartment resection is a radical limb saving procedure.
Here muscle group of one compartment (anterior, posterior or
medial) is resected entirely from its origin to insertion with the
tumour. It is done only when tumour is intracompartmental.
It is not suitable when tumour is extracompartmental or
many compartments are involved or encased to major
neurovascular bundle. Basis for compartmental excision
is—STS rarely penetrate anatomical barriers unless it is
very advanced.
Amputation is done in large tumours of upper or lower limbs.
¾
Radical amputation is done as disease has not spread
systemically which should be confirmed by CT chest,
abdomen and pelvis. In metastatic disease there is no need
to do amputation as long-term survival is not possible
except if primary is fungating and distressing.
INDICATIONS FOR AMPUTATIONS IN SOFT TISSUE
B
SARCOMA
x Major neurovascular encasement
x Bone involvement
x Multiple compartment involvement
x Limb itself is diseased like lymphoedema
x Recurrence with multicentricity
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CHAPTER 1S General Surgery: Sarcomas
People can be divided into three groups: (1) Those who make things happen, (2) Those who watch things happen, (3) Those
who wonder what’s happening.
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A
B
C
SRB’s Manual of Surgery
D
Figs. 1.564A to E: Surgical approach to a deep-seated sarcoma in leg—deep to gastrocnemius and soleus.
Tumor site Level of amputation
Foot Below knee
Leg Above knee
Thigh-middle and lower third Hip disarticulation
Thigh proximal third Hemipelvectomy
Buttock Hemipelvectomy
Hand and wrist Below elbow
Forearm Above elbow
Distal arm and elbow Shoulder disarticulation
Axilla and shoulder girdle Forequarter amputation
E
Primary radiotherapy alone (radical) is of less beneficial in soft
tissue sarcoma, but now it is used with more favorable results.
RADIOTHERAPY IN SOFT TISSUE SARCOMA
B
x Brachytherapy is given in high grade tumour
x External beam radiotherapy is used in low grade tumour
x All tumours more than 5 cm need adjuvant radiotherapy (external
beam)
x Preoperative radiotherapy is also beneficial
x Deep seated tumour; high grade; size more than 5 cm needs
chemoradiation
Debulking surgery is useful in large advanced tumours like
retroperitoneal sarcomas.
Radiotherapy
Preoperative radiotherapy followed by wide excision—
neoadjuvant RT.
Postoperative radiotherapy is commonly used because of
less tumour burden and less wound problems. Titanium clips
are placed during surgery at high-risk areas to identify the
site to concentrate proper RT.
¾
Brachytherapy is very effective in local control of the
tumour. Initially precise mapping of the area is done in the
operation theatre. Loading catheters are placed in surgical
field peroperatively. Later these catheters are loaded with
iridium 192. Dose is 45 Gy to tumour bed for 6 days.
¾
Permanent radioactive sources can also be placed to the
area.
¾
Postoperative external beam radiotherapy (EBRT)—it is
quiet effective and used in high grade tumour more than
5 cm often with brachytherapy. Dose is 70 Gy-25 fractions.
Palliative external radiotherapy can be given to prevent
bleeding, fungation and to reduce pain in advanced cases. It
is also used in secondaries in brain, bone.
Chemotherapy
Chemotherapy drugs—VAC (Vincristine, Adriamycin,
Cyclophosphamide) are commonly used. Other drugs
ifosfamide, dacarbazine are used in combination with above
drugs. Mesna is used as a protection for haemorrhagic
cystitis. MESNA is 2 Mercapto Ethane Sulfonate NA (sodium).
Chemotherapy is used when tumour is more than 5 cm or
high grade.
Usually postoperative chemotherapy is given. MAID
regime is used especially in recurrent STS. Mesna,
Adriamycin, Ifosfamide, Dacarbazine drugs (MAID
regime) are used. Its response rate is 50%. But survival
benefit is controversial.
Neoadjuvant chemotherapy is used to make the primary
tumour better operable. It makes eventual surgery better;
provides early treatment for micrometastasis; gives idea about
the response for chemotherapy. Drugs used are adriamycin
and ifosfamide.
Isolated limb perfusion using cytotoxic drugs and tumour
necrosis factor with hyperthermia is also often used.
Chemoradiation is a good alternate adjuvant therapy used.
Distant Spread
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Pulmonary metastasis can be treated with wedge resection,
segmentectomy, lobectomy, pneumonec tomy. Surgery is
done only when primary is well-controlled. Radiotherapy
and chemotherapy are also tried. More than three number
metastases in lung signify poor prognosis.
PROGNOSTIC FACTORS IN STS
B
x Size >5 cm—important factor
x More than one compartment
involvement
x Neurovascular invasion
x Clearance margin
x High grade
x Deep tumours and multi-
centric
x Lung secondaries
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CHAPTER 1S General Surgery: Sarcomas
A B
Figs. 1.566A and B: Recurrent soft tissue sarcoma over scapular
region and forearm in two different patients. Old surgical scar is seen.
A
Fig. 1.567: Recurrent soft tissue tumour thigh.
Note the scar of old surgery.
LIPOSARCOMA
It is the commonest type of soft tissue sarcoma arising from
the fat cells (of primitive mesenchymal cells).
Lipoma as a precursor tumour for liposarcoma which was
thought earlier is disproved/not accepted now.
It is 20% of all soft tissue sarcomas; spread is to lungs.
Common Sites: Thigh—commonest site; Retroperitoneum;
B
Figs. 1.565A and B: Recurrent chest wall tumour, could be secondaries,
as patient was earlier amputated for soft tissue tumour hand (forearm
amputation). X-ray of the same patient shows large tumour mass.
REMEMBER ABOUT STS
B
x Specific and nonspecific genetic alterations with change tumour suppressor genes p53 and RB1 are noted in STS
x Type is identified based on tissue formed or resembled rather than of tissue origin
x Liposarcoma (20%) and MFH (18-20%) are the commonest STS
x Extremity STS spreads principally to lungs; visceral STS to liver
x Low grade has <15% chance of distant spread; but high grade has got >50% chances
x Immunohistochemistry is a must using different markers like CD 31, CD 34, CD 59, CD 99, desmin, vimentin, S 100, cytokeratin etc.
x Expansive pathological type growth shows pseudocapsule, reactive zone (neovascularisation with oedema) and compression zone. Infiltra-
tive type shows satellite finger like infiltrations into the pseudocapsule
x Tumour growth initially contained by fascia, adventitia of vessel, periosteum and nerve sheaths
Back; Shoulder.
Contd...
Small minds are rst to condemn great ideas.
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Contd...
x Incision or core tissue biopsy is a must; MRI/CT imaging of primary is needed. CT chest is done to see metastases. All high grade STS
needs chest CT. MRI of spine may need in selected types like round cell type. CNS CT scan is done in suspected cases, alveolar STS and
in angiosarcoma. All visceral STS needs abdominal CT/MRI
x PET scan is done when targeted biopsy is needed; to predict prognosis; to assess response to chemotherapy. MR spectroscopy may be
useful in grading
x Limb sparing, function preserve, margin free wide excision with 2 cm clearance should be done—present concept. Limb salvage is the priority
x Amputation will not reduce the chances of regional and distant spread and will not improve disease specific survival
x Radiotherapy is mainly as postoperative as brachytherapy or EBRT. Intraoperative RT (IORT) is also tried often mainly in visceral/retrop-
eritoneal STS. RT is a must as an adjuvant in high grade/recurrent/ low grade > 5cm/margin positive/margin < 1cm
x Preoperative RT often used to reduce seedling during surgery; to make pseudocapsule thick and acellular; given 6 weeks prior to planned
surgery usually as 50Gy dose
x All high grade tumours are also treated with chemotherapy either single agent or combination (MAID). Preoperative chemoradiation is a
SRB’s Manual of Surgery
good additional alternative in stage II and III
x Unresectable primary tumour is treated with RT/chemoradiation/isolated limb chemoinfusion → if resectable surgery → adjuvant RT/
chemotherapy
x Stage IV STS treatment—Single organ spread by metastatectomy, management of primary, RT, chemotherapy. Disseminated spread—RT,
palliative surgery, chemotherapy
x Good prognostic factors are—Low grade tumor, size <5 cm. Poor prognostic factors are—High grade, tumor size >5 cm.
x Staging in relation to prognosis (MSKCC/Memorial Sloan Kattering Cancer Centre): Stage 0—no poor prognostic factors; Stage 1—one
poor prognostic factors; Stage 2—two poor prognostic factors; Stage 3—three poor prognostic factors; Stage 4—with metastases
TYPES
B
1. Well-differentiated—common between 50–70 years age group;
common in extremities (75%); metastasis is rare.
2. Dedifferentiated—common between 50–70 years age group;
common in retroperitoneum (75%); metastasis is high.
3. Myxoid—common at 25- 45 years age; common in extremities
(75%); high rate of metastasis.
4 Round cell type—common at 25–45 years age; common in
extremities (75%); high rate of metastasis.
5. Pleomorphic (5%)—poor prognosis.
Features
Microscopically, it contains lipoblasts with ‘signet ring’
malignant cells. It is low grade type:
Treatment is wide excision or radiotherapy with surgical
debulking in places where complete removal of tumour is
not possible like in retroperitoneal liposarcoma.
FIBROSARCOMA (11%)
It can arise from the bone or from soft tissues.
It is common between 30-55 years; common in deep soft
tissues of lower extremities with intact overlying skin.
It is arising from fibroblasts. Intramuscular and inter-
muscular fibrous tissue, fascial envelops, aponeurosis and
tendons are common origin.
Commonest site is thigh.
Spindle fibroblasts with ‘herring bone’ pattern are typical on
microscopy.
Types
1. Well-differentiated.
2.
Poorly differentiated.
Dermatofibrosarcoma protuberans. It is common in trunk. DFSP
3.
is an intermediate grade fibrohistiocytic tumor with nodular
A B C
Figs. 1.568A to C: (A) Recurrent dermatofibrosarcoma protuberans, (B) Malignant dermatofibrosarcoma
left side of the chest wall, (C) Dermatofibrosarcoma.
cutaneous mass; common in adult male; common in trunk and
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proximal extremities having slow growth without deep muscle
invasion. Skin is taut, nodular and commonly ulcerated.
4.
Aggressive fibromatoses are variant of fibrosarcoma which are
locally malignant in which desmoid tumour is also included.
Features
Fibrosarcoma is slow growing tumour which attains large
size.
Clinically, it is smooth, hard, warm, localised tumour.
It compresses or infiltrates the adjacent structures like
neurovascular bundle.
MALIGNANT FIBROUS HISTIOCYTOMA (MFH)
It is group of malignant soft tissue tumours with a
fibrohistiocytic appearance.
MFH is one of the most common STS in adult (18–20%).
70% occur in skeletal muscles.
Lower extremity is the common site.
It presents as solitary, multilobular lesion.
It spreads along the fascial planes or between muscle fibres
which is the probable reason for local recurrence.
Myxoid/giant cell/inflammatory/angiomatoid/pleomorphic are
the types. It is common in adults and elderly.
RHABDOMYOSARCOMA
It arises from striated muscle.
It is common in head and neck, upper thigh and arm.
It is commonest sarcoma in children.
It can occur in retroperitoneum, pelvis and genitourinary tract.
It is common in males.
TYPES
B
1. Pleomorphic—most common type of rhabdomyo sarcoma. It is
common in adult, aggressive with poor prognosis.
Embryonal—common in infants and children—is seen in viscera
2.
like urinary bladder.
3.
Botryoidal—slow growing polypoidal—respond well to chemo-
therapy and radiotherapy.
4. Alveolar—chromosomal translocation is common.
Feature: It is more aggressive tumour with poorer prognosis
(High grade); It also metastasises to lymph nodes.
CHONDROSARCOMA
It arises from chondroblasts.
It attains large size with slow growing nature.
Common sites are ribs, flat bones.
305
CHAPTER 1S General Surgery: Sarcomas
Fig. 1.5 69: Large soft tissue tumour over gluteal region with ulceration
and vascularity. It was confirmed as malignant fibrous histiocytoma.
Patient was successfully operated.
LEIOMYOSARCOMA
It arises from smooth muscle. Cut section shows whorled
appearance.
It constitutes (10–17%) of STS; common after 60 years.
Two third occurs in women.
It is undetermined grade—aggressive.
It is common in retroperitoneum and viscera, but can occur
in limbs and skin. Uterus is also common site.
Recurrence is common. It has got poor prognosis.
It can occur in the piloerector muscle of skin; inferior vena
cava; pulmonary artery.
Desmin and actin are the most common immunohistochemical
stains.
A
Figs. 1.570A and B: Chondrosarcoma leg lateral aspect. X-ray shows
calcified tumour. It was successfully removed as limb salvage procedure.
HAEMANGIOSARCOMA
It originates from blood vessel endothelium.
Fig. 1.571: Angiosarcoma.
When you get angry, you lose more than your temper.
B
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