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294
Manipal Manual of Surgery
Fig. 33.19: Elephantiasis neuromatosa. (Cour
Shetty, Professor, Department of Surgery, KS Hegde Medical
Academy, Mangalore)
tesy: Dr Balakrishna
pearance of elephant’s leg. The skin is dry and coarse
(Fig. 33.19).
Complications of Neural Tumours (Key Box 33.4)
Key Box 33.4
Complications of Neural Tumours
Atrophy of muscles
Dumb-bell tumours from dorsal spinal nerve root can
cause backache or paralysis
Acoustic neuroma—deafness
Cystic degeneration
Sarcomatous change
5. Pachydermatocoele
This refers to the plexiform lesions mainly found in the
neck as a thickened, coiled single mass.
NEURILEMMOMA (Schwannoma
)
• This is a benign tumour arising from Schwann cells.
• Commonest site is the acoustic nerve. However, vagus
nerve is the most common peripheral site
(Fig. 33.20).
• They can be single or multiple and present with a
fusiform swelling in relationship with the nerves.
Fig. 33.20: Vagal schwannoma at surgery—you can see the
displaced carotid artery and internal jugular vein. Second picture
showing the specimen. (Courtesy: Prof Balakrishnan, Department
of ENT, Kasturba Hospital, Manipal. Initially it was diagnosed as
solitary nodule of the thyroid gland but it was not moving with
deglutition)
• They can also arise from a peripheral nerve. Sensory
branches are affected more frequently (Table 33.2).
• They can also be seen in mediastinum and retro-
peritoneum.
• They are soft, lobulated, well encapsulated tumours.
• They are benign and do not turn into malignancy.
Treatment
Excision of the tumour can be done without sacrificing
the nerves because the tumour is well encapsulated and
displaces the nerve.
HAMARTOMA
• It is a tumour-like developmental malformation of
the tissues of a particular part of the body wherein it
is arranged haphazardly.
• Hamartoma is a Greek word which means fault or
misfire. It is not a clinical diagnosis.
Table 33.2 Comparison between neurofibroma and schwannoma
Neurofibroma Schwannoma
• More common • Less common
• Ectodermal and mesodermal origin • Ectodermal origin
• Subcutaneous (forearm) nerves are the commonest site • Acoustic nerve is the commonest site
• Multiple lesions are common • Very rare
• Feels firm or hard • Soft
• Tender • Nontender
• Can turn into sarcoma • Rarely turns into sarcoma
• Often nerve fibres are entangled with tumour. Hence, excision • Well encapsulated. Hence, enucleation is possible without
Section II • General Surgery
involves sacrificing nerve also
sacrificing nerve
Tumours and Soft Tissue Sarcoma
Table 33.3 Pathology of tumours
Terminology Explanation Examples
1. Well differentiated Cells that resemble very closely their normal Well-differentiated squamous cell
counterparts carcinoma
2. Undifferentiated (anaplasia) Loss of structural and functional differentiation Poorly-differentiated carcinoma
(anaplasia)
3. Dysplasia Loss in the uniformity, loss in the architectural Barrett’s columnar cell lined
orientation oesophagus
4. Carcinoma in situ Dysplastic changes involving entire thickness Cheek, tongue, breast, etc.
of the epithelium
5. Apoptosis Programmed cell death Seen in malignant tumours
295
A Few Examples of Hamartoma
Haemangioma, neurofibroma, glomus tumour, benign
naevus, lymphangioma.
Characteristic Features
• Being a developmental anomaly, they are seen at
birth or in early childhood.
• In adults, there is a long history of swelling.
• Being a malformation (not a tumour), it does not
have a capsule.
• They can be single or multiple.
• Some may regress as in strawberry angioma.
• They are benign lesions.
Treatment
• Excision is not only curative but also gives a correct
diagnosis.
• Care should be taken when it contains vascular tissue
such as haemangioma or neural tissue as in cases of
neurofibroma.
• Facial nerve and its branches may be damaged while
excising hamartomatous lesions over the face.
CHORDOMA
• Rare tumour
• Remnant of notochord (origin)
• Sacrococcygeal region (common site)
• Resection is difficult, chances of neurological deficit
and bleeding are high.
• Radioresistant
MALIGNANT TUMOURS
TYPES OF MALIGNANT TUMOURS
• They are of two types: Carcinoma and sarcoma.
Carcinoma arises from epithelium—ectodermal,
endodermal or mesodermal in origin.
• Sarcomas arise from soft tissues or bone and are
derived from mesoblast or mesenchymal tissues.
• It may be observed that mesoderm can give rise to
carcinoma and mesenchymal sarcoma also.
Pathology (Table 33.3)
Spread
Local spread: Generally, local spread occurs into
1.
adjacent structures. A few examples are given:
• Carcinoma cheek—fixity to mandible
Significance: May necessitate removal of mandible
along with wide excision (Fig. 33.21A).
• Squamous cell carcinoma—fixed to tibia may
necessitate an amputation.
2. Lymphatic spread: It is one of the most important
features of carcinoma (Key Box 33.5). As you
complete reading this book, you will come across
many cases and many examples of lymphatic spread
of malignant tumours. A few sarcomas also spread
by lymphatics (see page 301). Different types of
lymphatic spread are given below:
• Embolisation: More aggressive tumour means
more aggressive spread—by embolisation wherein
nodes can be enlarged in a far away station, e.g.
malignant melanoma (Fig. 33.21B).
Key Box 33.5
Carcinoma
Origin
Ectodermal—skin cancer
Endodermal—gut cancer
Mesodermal—renal carcinoma
Types
Squamous cell carcinoma
Basal cell carcinoma
Glandular
Section II • General Surgery
296
Fig. 33.21: Spread of malignant tumour (see text)
Manipal Manual of Surgery
• Permeation: Refers to tumour cells travelling along
the lymphatic vessel, e.g. carcinoma tongue with
sub-mandibular node enlargement (Fig. 33.21C).
• Retrograde lymphatic spread: When main
lymphatic pathway is blocked retrograde spread
can occur and a node in an unusual location may
get enlarged, e.g. Irish node (left axillary node
enlargement in carcinoma stomach).
3. Haematogenous spread: This is most important
method of spread of sarcomas. Also, a few malignancies such as renal cell carcinoma, follicular
carcinoma thyroid, carcinoma prostate, carcinoma
breast (Fig. 33.21D) and malignant melanoma
commonly spread by blood. Bone metastasis and
lung metastasis result from blood spread. Bone
metastasis can vary from mild form with only bone
pain to severe form with quadriplegia/pathological
fracture.
4. Transcoelomic spread: Spread through peritoneal
cavity by dislodgment of malignant cells, e.g. Ca
stomach with Krukenberg’s tumour—bilateral bulky
ovarian metastasis, commonly seen in premenopausal
patients (Fig. 33.21E).
5. Seeding (Fig. 33.21F): A few examples are given below:
• Cancer of lower lip spreading to upper lip, also
called kiss cancer. Other example: Cancer of vulva.
• Incision and ‘port’ site metastasis (port refers to
laparoscopic port).
Comparison of benign and malignant tumours is
given in Table 33.4.
Table 33.4 Comparison of benign tumours and malignant tumours
Feature Benign Malignant
Growth Very slow Rapid
Duration Long Short
Pain Usually not a feature Pain can be present due to local infiltration
Mobility Present Restricted
Fixity No Can be present
Consistency Firm/soft Hard, irregular
Spread No Spreads
Capsule Capsulated Uncapsulated
Recurrence after surgery Does not occur Can occur if wide excision is not done
Section II • General Surgery
Tumours and Soft Tissue Sarcoma
297
PARANEOPLASTIC SYNDROMES (PNS)
These are interesting syndromes and are listed in
Table 33.5.
Certain cancers produce some specific clinical
syndromes (symptom complexes other than cachexia)
which cannot be explained by their local or distant
spread or by the hormones produced by the tissue of
origin of these tumours. These are called paraneoplastic
syndromes (Key Box 33.6).
Just to give an example, hypercalcaemia due to
skeletal metastasis from carcinoma breast is not
considered as PNS, but if it occurs without skeletal
metastasis, it is considered as PNS.
Key Box 33.6
Important Featurs of PNS
Incidence: 10–15% of patients with cancer
It may be the earliest manifestation (primary can be
occult)
Bronchogenic cancer and breast cancer are most
commonly associated with PNS.
Hypercalcaemia and Cushing’s syndromes are the
most common clinical syndromes associated with
PNS.
PNS can be a major clinical problem and can be
treated.
AETIOLOGY OF CARCINOMA IN GENERAL
1. Tobacco is the most important factor in the develop-
ment of lung cancer, upper respiratory tract cancer,
gastrointestinal tract and genitourinary tract cancer.
Carcinoma pancreas is found more commonly in
smokers. Passive smokers also have increased
incidence of development of cancers.
2. Alcohol: Smoking with alcohol increases the
permeability of the upper digestive tract mucosa and
respiratory mucosa to the carcinogens. Thus, they
increase the incidence of cancer. Hepatocellular
cancer is commonly found in alcoholic cirrhotic liver.
3. Ionising radiation: Atomic bomb blasts in Japan
have definitely resulted in increased number of
cases of breast cancer in premenopausal women and
leukaemia in children.
4. Ultraviolet radiation: Causes all types of skin cancers.
5. Genetic causes (Key Box 33.7)
Key Box 33.7
Genetic/Defective DNA Repair
1. Xeroderma pigmentosa : Skin cancer
2. Bloom’s syndrome : Acute leukaemia, various
cancers
3. Fanconi’s anaemia : Acute leukaemia, squamous
cell carcinoma, hepatoma
4. Ataxia-telangiectasia : Acute leukaemia, lymphoma, breast cancer
Table 33.5 Paraneoplastic syndromes
Clinical syndrome Underlying major cancer Mechanism
1. Endocrinopathies
• Cushing’s syndrome Small cell Ca lung, Ca pancreas ACTH or ACTH-like substance
• Hyponatraemia Small cell Ca lung ASH or atrial natriuretic peptide
• Hypoglycaemia Hepatoma Insulin or insulin-like subst
• Hypercalcaemia Squamous cell Ca lung
Ca breast, Ca kidney
• Carcinoid syndrome Bronchial carcinoid, Ca pancreas, Serotonin, bradykinin
Ca stomach
• Polycythaemia Renal cell carcinoma, cerebellar Erythropoietin
haemangioma
2
. Muscle syndrome
Myasthenia gravis Thymoma Immunologic
3. Bone and soft tissues
Hypertrophic osteoarthropathy Adenocarcinoma lung Unknown
and clubbing of fingers
4. Vascular
• Venous thrombosis Carcinoma p
• Disseminated intravascular coagulation Ca pancreas, lung, prostate Tumour products
ancreas Hypercoagulability
PTH-like substance
Ca: Carcinoma
ance
Section II • General Surgery
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Manipal Manual of Surgery
6. Hereditary causes
• MEN syndrome: Medullary carcinoma thyroid
(Multiple Endocrine Neoplasia)
• FPC: Colonic cancer (multiple)
(Familial Polyposis Coli)
• Li-Fraumeni syndrome: Familial breast cancer.
• Retinoblastoma.
7. Dietary factors
• Red meat: Carcinoma colon, carcinoma breast.
• Fat: Carcinoma breast, carcinoma colon.
• Smoked, charred fish: Carcinoma oesophagus,
carcinoma stomach.
8. Chemicals
• Benzanthracenes: Skin cancer when painted on the
skin
• Benzopyrenes: Lung cancer
• β-naphthylamine: Bladder cancer
• Nitrosamines and amides: Cancer stomach
• Aflatoxin B: Hepatocellular carcinoma
• Asbestos: Lung cancer
9. Viral factors
• Human T cell leukaemia virus type 1 (HTLV-1):
T cell leukaemia/lymphoma (RNA virus)
• Human papillomavirus (HPV)
Cancer cervix, cancer urogenital region
• Epstein-Barr virus: Burkitt’s lymphoma.
SOFT TISSUE SARCOMAS (STS)
These are malignant tumours arising from soft tissues.
Thus, they can occur in any part of the body. Examples
are given in Table 33.6.
Table 33.6 Soft tissue sarcoma
Tissue of origin Name
• Mesenchymal tissue Malignant fibrous histiocytoma (MFH)
• Adipose tissue Liposarcoma
• Smooth muscle Leiomyosarcoma (GIST)
• Striated muscle Rhabdomyosarcoma
• Synovial tissue Synovial sarcoma
• Neural tissue Malignant schwannoma
• Uncertain Epithelioid sarcoma
• Blood vessels Angiosarcoma
• Lymph vessels L
ymphangiosarcoma
INTRODUCTION
• Sarcomas are relatively uncommon tumours account-
Section II • General Surgery
ing for approximately 1% of all adult cancers. These
are heterogeneous group of tumours of mesenchymal
cell origin, occurring more commonly in young
patients with not a favourable outcome. The overall
survival is approximately 40
–50% at 5 years.
• These are malignant tumours which are fatal if
untreated or mistreated. Most of them occur in young
patients as painless lumps. CT scan, MRI and incision
biopsy (details later) are key investigations. Early
diagnosis and curative resection have a major role in
the management of soft tissue sarcomas. In addition
to TNM staging, pathological grading of the tumour
has been included GTNM staging.
AETIOLOGY/EPIDEMIOLOGY OF STS (Key Box 33.8)
1. Genetic factors: Genetic mutations/gene rearrange-
ments have been implicated in the pathogenesis of
STS. Two genes that are most relevant to soft tissue
tumours are retinoblastoma (Rb) tumour suppressor
gene and p53 tumour suppressor gene. Various
familial syndromes are associated with STS such as
Li-Fraumeni syndrome, Gardner’s syndrome and von
Recklinghausen’s disease. Several oncogenes have
been identified.
2. Exposure to radiation: 8–50-fold increase in the
incidence of STS is reported in patients who are treated
for cancer of the breast, cervix and ovary by radiation.
3. Neurofibromatosis type I: Specially mentioned here
because it is the only benign tumour progressing to STS.
100% lifetime risk of malignant peripheral nerve
sheath tumour, in patients with familial neurofibromatosis, caused by mutations in the NF1 gene.
4. Exposure to chemicals: Thorium oxide, vinyl
chloride, arsenic have been implicated in hepatic
angiosarcomas.
5. Trauma: Chronic tissue trauma is blamed as a trigger-
ing factor for development of STS. Others believe that
trauma draws attention of the patient to the STS.
6. Immunosuppression: Kaposi’s sarcoma occurs in
/AIDS patients.
HIV
Key Box 33.8
Aetiology of STS—Summary
Genetic factors
Exposure to radiation
Neurofibromatosis (NF-1)
Exposure to chemicals
Trauma
Immunodeficiency
Chronic lymphoedema
Remember as GENETIC
Tumours and Soft Tissue Sarcoma
299
7. Chronic lymphoedema can give rise to lymphangio-
sarcoma. Postmastectomy lymphangiosarcoma is
called Stewart-Treves syndrome.
TNM STAGING Soft tissue sarcomas of (A) trunk and
extremity and (B) retroperitoneum
Definition of Primary Tumour (T) AJCC, 8th Edition
TX Primary tumour cannot be assessed
T0 No evidence of primary tumour
T1 Tumour 5 cm or less in greatest dimension
T2 Tumour more than 5 cm and 10 cm in greatest
dimension
T3 Tumour more than 10 cm and 15 cm in greatest
dimension
T4 Tumour more than 15 cm in greatest dimension
Definition of Regional Lymph Node (N)
N0 No regional lymph node metastasis or unknown
lymph node status
N1 Regional lymph node metastasis
Definition of Distant Metastasis (M)
M0 No distant metastasis
M1 Distant metastasis
Definition of Grade (G): FNCLCC = Fédération Nationale
des Centres de Lutte Contre Le Cancer;
GX Grade cannot be assessed
G1 Total differentiation, mitotic count and necrosis
score of 2 or 3
G2 Total differentiation, mitotic count and necrosis
score of 4 or 5
G3 Total differentiation, mitotic count and necrosis
score of 6, 7 or 8.
FNCLCC: Fédération Nationale des Centres de Lutte Contre Le Cancer
Table 33.7 AJCC prognostic stage groups for soft tissue
sarcoma in the trunk and extremity and
retroperitoneum
Primary Regional Distant Histologic
tumour lymph metastasis grade
Stage (T) node (N) (M) (G)
IA T1 N0 M0 G1, GX
IB T2, T3, T4 N0 M0 G1, GX
II T1 N0 M0 G2, G3
IIIA T2 N0 M0 G2, G3
IIIB T3, T4 N0 M0 G2, G3
Any T N1 M0 Any G
IV Any T Any N M1 Any G
Clinical Features
Sarcomas are rapidly growing vascular growth
(Table 33.8).
• Typically patients present with painless lump/
swelling in the thigh or forearm.
• When the tumour is increasing in size, size more than
5 cm, deep fascia and painful—suspect STS.
INVESTIGATIONS/DIAGNOSTIC IMAGING
• Few blood tests, cardiac evaluation is done and ECOG
performance score is calculated (page 681).
• Chest radiography: Presence of cannonball metastasis
alters the staging, treatment policy and prognosis.
If STS is more than 5 cm (T2), computed tomography (CT) of
the chest should be considered.
Table 33.8 Comparison of carcinoma and sarcoma
Carcinoma Sarcoma
1. Cell of origin Ectodermal or endodermal (epithelial) Mesodermal (mesenchymal)
2. Age group Elderly, 40–60 years Young, 10–30 years
3. Rate of growth Slow Fast
4. Presentation Nonhealing ulcer, cauliflower-like growth Fleshy mass, red and vascular, dilated veins
with everted edges and induration. Fixity over the surface, local rise of temperature.
is a late feature Ulceration is a late feature
5. Spread Lymphatic spread is very common, both Blood spread occurs very early and results in
by emboli and permeation. Blood spread cannonball secondaries in lung. Rhabdomyosarcoma,
does occur as in renal cell carcinoma, synovial sarcoma, epithelioid sarcoma. Malignant
thyroid carcinoma, breast carcinoma, etc. fibrous histiocytoma, angiosarcoma spread by lymphatics
6. Microscopy Cell nests or epithelial pearls are Malignant cells resemble their cell of origin. Thus,
seen in well-differentiated cancers spindle-shaped cells are found in fibrosarcoma
7. Treatment Surgery is the main treatment of ectodermal Surgery is the main modality of treatment. Radiotherapy
cancers, surgery or radiotherapy for ectodermal and chemotherapy are also beneficial when the tumour
lesions. Chemotherapy is not very useful is more than 5 cm
Section II • General Surgery
300
Manipal Manual of Surgery
Imaging
• CT scan is useful in evaluating retroperitoneal
sarcomas. It can define structures, infiltration into
neighbouring structures, hydronephrosis, etc. CT can
guide a core biopsy also. It is also used to image chest
and pelvis to stage the disease.
• MRI is the investigation of choice when STS occurs
in extremities to delineate muscle groups, bones,
vascular structures, etc. (Figs 33.22 and 33.23).
Fig. 33.22: Giant lipoma thigh Fig. 33.23: MRI-giant lipoma
• FNAC
– Useful to detect metastatic disease
–
To detect local recurrence
– Ideal for superficial lesions
– Disadvantage: Cannot assess tumour grading.
Tissue is not sufficient for diagnostic tests.
• Core needle biopsy: It is the investigation of choice
to confirm or rule out the diagnosis. It can give not
only the diagnosis but also the histological type and
grade also. Several scores are added to TNM staging
such as tumor differentiation score, mitotic score and
tumor necrosis score.
Safe and accurate
–
– Tissue is sufficient for grading, electron microscopy
and flow cytometry.
– With CT guidance, core biopsy can be taken from
deeper structures also.
• Incisional biopsy
– When core biopsy tissue is not adequate, incisional
biopsy is indicated (Key Box 33.9). Strict guideline
to be followed to avoid risk of tumour seeding.
• PET-CT scan is indicated in high grade tumors,
Section II • General Surgery
metastatic disease or before an amputation.
Key Box 33.9
Guidelines while Doing an Incisional Biopsy
Incision should be oriented longitudinally in STS of
extremities to facilitate removal of biopsy site track, scar
and tumour en bloc. Do not put transverse incision.
Flaps should not be raised
Perfect haemostasis should be achieved
Prevent dissemination, avoid crushing of the tissues
Avoid drain
TREATMENT (see next page)
• The aim is to achieve local control and to treat meta-
stasis including subclinical metastasis, thus trying for
a cure.
• Adult sarcomas are best treated by wide excision with
negative margin (R–O) resection. However, near the
major and minor arteries, margin need not be wide
but it may be microscopically positive margin.
• Surgery is the first line of treatment varying from a
wide local excision to amputation or disarticulation
(5%), when it occurs in the extremities. Low-grade
tumours can be treated on 1 cm wide excision of
surrounding normal tissue and high-grade tumours
by 2 cm margin (Key Box 33.10).
• Small tumours less than 5 cm have not been
associated with recurrence. Hence, radiotherapy may
not be required but if grade is high, RT is required
(Key Box 33.11).
Key Box 33.10
Surgery for Soft Tissue Sarcoma
Surgery is the primary and most effective therapy
Local wide excision with 2 cm of surrounding normal
tissue should be removed. 1 cm for low-grade and
2 cm for high-grade tumour.
Not to dissect along pseudocapsule which is composed
of tumour cells.
If necessary, excision should include nerves and
vessels followed by nerve grafts and arterial reconstruction—when they are directly infiltrated.
Otherwise preserve them.
STS rarely involve bones and skin. Hence, wide resec-
tions of these structures are infrequently necessary.
It should also include previous scars.
If amputation can be avoided by giving preoperative
radiotherapy, it is preferred first. It is then followed by
wide excision/compartmental excision and postoperative radiotherapy. Dose of pre- and postoperative
radiotherapy is 50–60 Gy given in 25 fractions.
Brachytherapy is also given.
Margin negative resection should be the aim.
Tumours and Soft Tissue Sarcoma
301
Key Box 33.11
Radiotherapy
It is not the first modality of treatment in STS.
Radiotherapy is given after margin negative resection.
Surgery + RT has resulted in local control rates up to
or more than 90%.
External beam radiation therapy (electrons, protons or
neutrons) is given with a margin of 5 to 7 cm or even
more depending on size of tumour. Dose is 50 Gy
given in 25 fractions—as preoperative dose.
Dose is 60 to 70 Gy for postoperative treatment.
In brachytherapy, multiple catheters are placed in
tumour resection bed. Catheters are loaded with seeds
containing iridium 192.
Dose of brachytherapy is 42–45 Gy to tumour bed
over 4–6 days.
Short period of treatment time and less systemic
toxicity are advantages of brachytherapy.
• Tumours do respond to radiotherapy and chemo-
therapy.
Enneking classification of surgical procedures: Once the
specimen is subjected for pathology request should be
made to look specifically into the margin status. This is
very important. Details are given below. Four types of
margin have been recognised. If it is intralesional, means
tumour remains—margin runs through tumour. If it is
marginal, means surgical plane passes through pseudocapsule. Pseudocapsule is a reactive zone, hence high
chances of local recurrence because tumour satellites
may be present. When the margin is wide, means say
2 cm in all dimensions, recurrence rate is low, because
surgical plane is in the normal plane. Radical, means
tumour is removed including affected compartments
and there is a minimal risk of local recurrence.
ROLE OF CHEMOTHERAPY
• High-grade tumours have high potential of meta-
stasis. Hence, combination chemotherapy is to be
considered before or after surgery.
• The most favoured combination chemotherapy drugs
include Mesna, Adriamycin, Ifosfamide and
Dacarbazine (MAID).
• The success rate is around 10–20%. Round cell
sarcomas respond better to chemotherapy than
spindle cell sarcoma.
Not all sarcomas are chemosensitive. A few details are
given below.
Relative chemosensitivity
i. Ewing’s sarcoma
Embryonal and alveolar rhabdomyosarcoma
ii.
Chemosensitive
i. Synovial sarcoma
ii.
Myxoid/round cell liposarcoma
Moderately chemosensitive
i. Pleomorphic liposarcoma
Epithelioid sarcoma
ii.
iii. Pleomorphic rhabdomyosarcoma
iv. Leiomyosarcoma
v. Malignant peripheral nerve sheath tumour
vi. Angiosarcoma
Chemo-insensitive
i. Dedifferentiated liposarcoma
ii.
Alveolar soft part sarcoma
Sarcomas that Metastasise to Lymph Nodes*
• Rhabdomyosarcoma
• Angiosarcoma
• Clear cell carcinoma
• Epithelioid sarcoma
• Synovial sarcoma
*Remember as RACES
Isolated limb perfusion (like melanoma) with high dose
chemotherapy (melphalan) and tumour necrosis factor
alpha (TNF-α) with hyperthermia to the links can be
used by cannulating artery and vein with tourniquet.
PROGNOSIS
• Almost 80% of STS metastasise to lungs within
2–3 years of the diagnosis.
• Prognosis depends on metastatic STS, grade of the
tumour, size of the tumour, margins after resection
and anatomical location.
• If pulmonary metastasis is resectable, 30% survival
may be expected at 3 years.
DIFFERENTIAL DIAGNOSIS OF
SOFT TISSUE SARCOMA
LIPOSARCOMA
• It is a malignant fatty tumour (see page 749).
• Common sites: Proximal extremity (Figs 3
3.24 to 33.26
MRI of liposarcoma of thigh), trunk or retroperitoneum (Fig. 33.27).
• They are generally large at the time of diagnosis, e.g.
retroperitoneum. It results in gross swelling, which
is firm to hard (more than 50% will be of
>20 cm size)
(Key Box 33.12).
Section II • General Surgery
302
Figs 33.24 to 33.26: T1- and T2-weighted (W) axial MRI. Isointense
to hyperintense mass lesion noted in the anterior compartment
of right thigh
the lesion
. Sagittal T2-W MRI shows supero-inferior extent of
Fig. 33.27: Retroperitoneal liposarcoma
Key Box 33.12
Retroperitoneal Sarcoma (RPS)
Most common RPS is liposarcoma or leiomyosarcoma.
They constitute 15% of adult soft tissue sarcomas.
May present as large masses of more than 20 cm at the
time of presentation.
Local recurrence and intra-abdominal spread is more
common.
Progressive abdominal distension, pedal oedema, young
age and firm to hard retroperitoneal mass clinches the
diagnosis.
CT scan of chest and abdomen followed by FNAC /true-
cut biopsy for histology/grade of the tumour.
Margin negative—complete surgical resection is the
treatment of choice.
Chemotherapy has not been effective against RPS.
Section II • General Surgery
Manipal Manual of Surgery
• The compression on blood vessels may result in
oedema of the limbs when it occurs in retroperitoneum.
• Well-differentiated myxoid liposarcomas are
notoriously known to recur many times before
spreading to lungs. Hence, prognosis is good
(Fig. 33.34).
• Dedifferentiated liposarcoma can be distinguished
from other poorly differentiated STS subtypes on
the basis of MDM2 and CDK-4 immunohistochemistry.
• Pleomorphic and lipoblastic liposarcomas tend to be
of higher grade and often present with metastasis.
• MRI (Fig. 33.24) followed by wide excision/surgery
is the primary treatment.
• They do respond to radiotherapy.
MALIGNANT FIBROUS HISTIOCYTOMA (MFH)
• It is a malignant tumour of mesenchymal tissue
(fibrous tissue). This is the recent nomenclature of
sarcoma. Fibrosarcoma or pleomorphic rhabdomyosarcomas are included under this. Most of the so-
called fibrosarcomas are presently included under
MFH.
• These are high-grade tumours that lack differentia-
tion.
• It can also arise from bone.
• The MFH: Superficial type rarely metastasises and
carries good prognosis.
• Locations: Retroperitoneum, trunks and limbs (inter-
muscular septae of adductors, scapulohumeral and
pectoral muscles).
Clinical Features and Treatment
• Common in elderly patients (50 years) but can occur
at any age.
• Slow-growing, firm to hard mass with restricted
mobility.
• As the tumour is locally invasive, it infiltrates the
muscles and adjacent structures. Thus, it can cause
muscle weakness or pain, etc.
• Spread: Local spread is common. Distant metastasis
by blood is late (lungs). Lymph node metastasis is
rare.
• Like other sarcomas, dilated veins, local rise of
temperature, restricted mobility and hardness will
clinch the diagnosis.
• MRI is the investigation of choice to know the
extent of the disease.
• Margin negative surgery should be the aim.
Tumours and Soft Tissue Sarcoma
Figures 33.28 to 33.41 depict types of common sarcomas, wide excision and histological features.
303
Fig. 33.28: Early stage
of liposarcoma—this
swelling had local rise of
temperature and restricted mobility
Fig. 33.32: Synovial sarcoma left shoulder region. See the
secondary varicosity due to pressure effects. Nonextremity
sarcomas have poor prognosis
Fig. 33.29: Advanced case of
liposarcoma—the swelling was
hard and fixed
over the surface and location
were characteristic
. Dilated veins
Fig. 33.30: Swelling in the
elbow region for two years
duration presented to the
hospital with bleeding and
fungation
epithelioid sarcoma
. Advanced case of
Fig. 33.33: Bleeding vascular lesion in the ankle region—
angiosarcoma. (Courtesy: Dr Mallikarjuna Desai, Head,
Department of Surgery, SDMC, Dharwar, Karnataka)
Fig. 33.31: Ulcerated lesion in
the elbow region of two years
duration presented to the
hospital with bleeding. A case of
dermatofibrosarcoma protuberans
Fig. 33.34: Six times recurrent myxoid liposarcoma since 8 years.
It has good prognosis. (Courtesy: Dr Shashi, Head, Department
of Surgery, Calicut Medical College, Calicut, India)
Fig. 33.35: Spindle cell sarcoma. (Courtesy: Dr Laxmi Rao, Head,
Department of Pathology, KMC, Manipal)
Section II • General Surgery