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284
Fig. 32.14: Wound is ready for skin grafting. Observe the red
granulation tissue
Fig. 32.15: Skin graft is applied over the recipient area
Manipal Manual of Surgery
Key Box 32.1
Ideal Requirements for Free Skin Graft
Wound should be free from infections such as strepto-
cocci and pseudomonas
Vascular wounds, e.g. wounds with healthy granulation
Wound should be thoroughly debrided
Haemostasis must be achieved before placing the graft
Close and immobile contact between graft and the
wound
Recipient area should be immobilised with POP slab
CONTRAINDICATIONS FOR SKIN GRAFT
• Infection by beta-haemolytic streptococci. They produce
fibrinolysin which dissolves fibrin.
• Presence of an infected wound with copious dis-
charge in the vicinity.
• Avascular wounds: With exposed bare bone without
periosteum, exposed tendon without paratenon and
exposed cartilage without perichondrium.
Fig. 32.16: Skin graft is taken from thigh using Humby’s knife
2. Full Thickness Graft (Wolfe Graft)
• Consists of epidermis and full thickness of dermis.
• Harvested using ordinary scalpel.
• Needs excellent vascularity of the recipient wound
for graft survival.
• Used for small uncontaminated wounds produced
after excision of skin lesions or after release of skin
contractures (lower eyelids, fingers).
• Donor area needs primary suturing or split skin graft
for healing and hence limits the size of the graft.
• Unlike split skin grafts, full thickness grafts do not
contract and retain their colour. Hence, they are
cosmetically superior.
INDICATIONS OF SKIN GRAFT (Key Box 32.1)
1. Skin loss
• Post-traumatic (e.g. avulsion and degloving injury)
• Post-surgical (e.g. excision of tumours, excision of
burn wound)
• As a result of pathological process (e.g. venous
ulcer, diabetic ulcer)
2. Mucosa loss
• After excision of lesions of oral cavity, tongue.
• For resurfacing reconstructed vagina in cases of
Section II • General Surgery
vaginal agenesis.
HEALING OF THE DONOR AREA
Donor area of split skin graft heals by epithelialisation
from the adnexal remnants of dermis, pilosebaceous
follicles and or sweat gland apparatus. Complete
healing of donor area occurs by 8–10 days.
THE PROCESS OF GRAFT ‘TAKE’
The processes which result in reattachment and
revascularisation of the graft to the bed are collectively
referred to as “take” of graft.
• The graft initially adheres to its new bed by fibrin.
Revascularisation starts by 48 hours and is completed
by 4–5 days. This is achieved by the outgrowth of
capillary buds from the recipient area to unite with
those on the deep surface of graft. For the first 2 days
after grafting, the skin graft derives its nutrition from
the wound by the process of serum imbibition/
plasmatic circulation.
FLAPS
Flap is a block of tissue transferred from donor to
recipient area along with its vascularity.
COMMON INDICATIONS FOR FLAP SURGERY
• To cover defects/wounds where free skin graft
cannot be used, e.g. exposed bare bones, bare
tendons, bare cartilage.
• Wounds with exposed joints, exposed major vessels
and nerves.
Burns and Skin Grafting
285
• Implant exposure following orthopaedic procedures.
• In wounds with soft tissue loss, where future
reconstructive surgery is contemplated.
• Defects which need better contour to improve
cosmesis.
• Breast reconstruction following mastectomy.
CLASSIFICATION OF FLAPS
These are broadly classified into pedicled flaps and free
flaps.
I. Pedicled Flaps
Pedicle or the base remains attached to the donor site
during its transfer to the recipient area (Figs 32.17 to
32.19). Pedicled flaps may be of following types:
. Local flaps, e.g. rotation, transposition, limberg and
1
bilobed.
2. Pectoralis major myocutaneous (PMMC) flap,
deltopectoral (DP) flap for head and neck defects,
transverse abdominis myocutaneous (TRAM) flap for
breast reconstruction.
• Regional flap, e.g. PMMC, DP for head and neck
defects,
• TRAM for breast reconstruction.
Fig. 32.17: PMMC flap
following radical parotidec
tomy
Fig. 32.18: Latissimus dorsi flap
used to cover the defect in the arm
following radical excision of a
sarcoma
. Distant flaps, e.g. groin flap, subaxillary flap for hand
3
defects.
A few examples of the pedicled flap
• Skin flap
• Fasciocutaneous flap
• Muscle flap
• Myocutaneous flap
• Adipofascial flap
• Osteocutaneous flap
II. Free Flaps
These are completely detached from the donor area
before being transferred to the recipient area. The
vascularity of the flap at the recipient site is immediately
restored by anastomosing the vessels of the flap with
the vessels at the recipient area using microvascular
techniques.
SOME OF THE COMMONLY PERFORMED FLAPS
• Forehead flap: Entire forehead skin can be raised
based on anterior branch of superficial temporal
artery. It bears an unsightly scar of the donor site.
Median (Indian) forehead flap based on supratrochlear vessels is a very useful flap in reconstructing
defects over nose.
• Deltopectoral (DP) flap: It is supplied by upper
4 perforating branches of internal mammary artery
and is used to reconstruct defects of neck and lower
face. After about 4 weeks, the flap is divided and the
base is returned to the chest wall.
• Pectoralis major myocutaneous (PMMC) flap:
Pectoral branch of thoracoacromial artery is the
pedicle of this flap. It is the ideal pedicled flap for
reconstruction of head and neck defects following
ablative surgeries for various head and neck cancers.
Hence, it is described as a workhorse among the flaps.
Osteomyocutaneous PMMC flap by including 5th or
6th rib can be used for mandibular reconstruction.
• Latissimus dorsi flap (LDF): As a myocutaneous flap,
based on thoracodorsal vessels can be used for
reconstruction of the lower half of face, neck, breast,
chest wall, axilla, and upper arm.
Fig. 32.19: Bedsore covered with local advancement flap
FREE (MICROVASCULAR) FLAPS
Using operating microscope and microvascular techniques, it is possible to do a free tissue transfer of tissues
such as skin, muscle, bone, intestine, omentum, etc. The
procedure involves anastomosis of vessels of the flap
to vessels at the donor site, e.g. latissimus dorsi muscle
myocutaneous flap, radical artery forearm flap, gracilis
flap and free fibula flap.
Section II • General Surgery
286
Manipal Manual of Surgery
MISCELLANEOUS
Skin Substitutes
• This is one of the important requirements wherein
large surface area of burns and skin is lost.
• An ideal substitute must be affordable, permanent,
provide normal pigmentation, resist scar formation
and grow with developing children.
• A few examples of skin substitutes are:
Dermal substitutes: They allow for creation of a
–
‘neodermis’. They are formed from patient’s own
mesenchymal cells. Once ‘neo-dermis’ is formed,
split thickness skin graft is applied. Thus, the burns
site is closed quickly with less scarring.
– Cultured epithelial autograft is another example
of dermal substitute. They are cultured from
patient’s own full thickness biopsy. It will require
3 weeks to grow.
– Another cultured skin is a biologic dressing from
cultured neonatal keratinocytes and fibroblasts.
They are all very expensive.
Hydrotherapy in Burns
It refers to usage of external water (may be very cold)
to run over the burned part for a few minutes.
Methods used are:
Immersion hydrotherapy: It is performed in tubs
1.
called hydrotanks or burns tanks. Tanks should be
disinfected after usage. Water used is sterile.
2. Shower hydrotherapy: Depending upon severity of
burns, the shower can be either in supine or sitting
position.
Showering immediately rinses away dead skin and
bacteria.
How does it work?
• Cleans the surface of wound and remove debris
• Removes pus
• Prevents loss of fluid through skin
• Minimises scar formation
• Provides moist environment for wound healing
• Minimises risk of infection
Duration
• It may vary from 10 to 20 minutes
Sedation
• Sedation or general anaesthesia is required as it can
be very painful.
SU4.3: Discuss the medicolegal aspects in burn injuries.
1. Antemortem or postmortem burns: Firstly, it has to be
determined whether the burns are antemortem or postmortem in origin.
Features of antemortem burns are:
Presence of soot (carbonaceous particle) in airways
Line of redness surrounding the burn injury
Blister with serous fluid rich in protein and chloride
2. Manner of sustaining burns: It can be accidental,
suicidal or homicidal
Accidental burns: It is the commonest variety
encountered. It may happen during cooking, careless
burning of various forms of waste, fire cracker
accidents or any other industrial/domestic accidents.
Suicidal: Self-inflicted burns. It may be related in
dowry (Section 304 (B) IPC). It may also be a form of
protest against government (self-immolation)
Homicidal: It may be done sometimes related to dowry
or any other crime related to women. Sometimes a
person may be killed by others using fire or any other
inflammable substance.
Scalds: May be accidental commonly. It may be also
seen in child abuse cases where it is done deliberately
on the child by the adult.
Electrical burns: It is mainly accidental. Sometimes it
may be suicidal which is however rare.
Lightening burns: It is accidental in nature.
1. What fluid is used for IV fluid resuscitation in burns
according to Parkland’s formula?
A. Crystalloids
. Colloids
B
C. Both crystalloids and colloids
D. None of the above
2. Scald burns are caused by:
A. Chemicals B. Dry heat
Section II • General Surgery
C
. Hot water D. Hot metal
3. Commonly used fluid resuscitation formula for
burns is:
A. Evans B. Brooke’s
C
. Muir and Barclay D. Parkland’s
4. Adequacy of fluid resuscitation in burns is assessed
by:
A. Hourly urine output B. Volume of IV fluids
C
. 24-hour urine volume D. Colour of urine
Burns and Skin Grafting
287
5. Which of the following is an example of biologic
dressing?
A. Human amnion B. Paraffin gauze
.
Burn mesh D. Acticoat
C
6. Which formula is used for calculating energy
requirement in burns?
A. Curreri formula B. Brooke’s formula
.
Rule of 9 D. None of the above
C
7. Advantages of meshed skin grafts are:
A. Wider area of coverage
.
Lets out blood and serum from beneath the graft
B
C. Better graft take
D. All of the above
8. Fasciotomy is indicated in:
A. Scald burns B. Flame burns
C.
Chemical burns D. Electrical burns
9. Antidote for hydrofluoric acid is:
A. Calcium chloride B. Calcium gluconate
C.
Sodium chloride D. Sodium gluconate
10. Human skin allografts are usually obtained from:
A. Cadavers B. Live donors
C.
Infected persons D. Newborn babies
11. Following factors contribute to burns shock except:
A Increased capillary permeability
B.
Increased plasma oncotic pressure
C. Increased capillary hydrostatic pressure
D. Depressed myocardial function
12. Bacterial translocation can occur in the following
conditions except:
A Burns
Subacute bacterial endocarditis
.
B
C. Intestinal obstruction
D. Myocardial infarction
13. Acute tubular necrosis can occur in burns due to
the following factors except:
A. Hypovolaemia B. Toxins
.
Myoglobin D. Aldosterone
C
14. Following metabolic responses to burns occur except:
A. Gluconeogenesis is increased
Lipolysis is increased
B.
C. Catecholamines are increased
D. Proteolysis is decreased
15. Tangential excision refers to:
A. Excision and leaving the wound open immediately
after admission
Excision within 6 hours and primary closure
B.
C. Excision after 2–3 days and skin grafting
D. Excision after 10 days
16. Following are true for split skin graft except:
A. It is partial thickness graft
B.
It is called Thiersch graft
C. Humby’s knife is used often
D. Cosmetically it is superior to full thickness graft
17. Following are true for full thickness graft except:
A. It is called Wolfe graft
.
It consists of epidermis and dermis
B
C. Cosmetically it is superior to partial thickness graft
D. Problem with this graft is that it contracts often
18. Following heals by serum imbibition process:
A. Split skin graft B. Musculocutaneous flaps
C. Fracture of bones D. Mesh healing
19. Following are true for pectoralis major myocutaneous flap except:
A. It is the flap for head and neck reconstruction
Based on pectoral branch of thoracoacromial artery
B.
C. It is an example of pedicle flap
D. It is a free flap
20. Following is true for hydrotherapy in burns
except:
A. It can be done by immersion
Shower can also be used
B.
C. It is painless
D. It rinses away dead skin and bacteria
Answers
1. A 2. C 3. D 4. A 5. A 6. A 7. D 8. D 9. B 10. A
11. B 12. D 13. D 14. D 15. C 16. D 17. D 18. A 19. D 20. C
Section II • General Surgery
33
Tumours and Soft Tissue Sarcoma
Benign tumours
Papilloma
Fibroma
Lipoma
Neural tumours
Neuroma
Neurofibroma
Neurilemmoma
Chordoma
Malignant tumours
Paraneoplastic syndromes
INTRODUCTION
A tumour is a new growth consisting of cells of
independent growth arranged atypically and serves no
function. Broadly classified into:
• Benign
• Malignant
BENIGN TUMOURS
PAPILLOMA
This is a benign tumour arising from skin or mucous
membrane. It is characterised by finger-like projections
with a central core of connective tissue, blood vessels,
lymphatics and lining epithelium (Fig. 33.1). It can be
called hamartoma or a skin tag. It is an example of
overgrowth of fibrous tissue (Key Box 33.1). It can be
pedunculated with narrow base or broad base.
Soft tissue sarcomas
Aetiology/Epidemiology of STS
Role of chemotherapy
Differential diagnosis of soft tissue sarcoma
Liposarcoma
Malignant fibrous histiocytoma
Synovial sarcoma
Angiosarcoma
Rhabdomyosarcoma
Kaposi’s sarcoma
Dermatofibrosarcoma protuberans
Acrochordons (skin tags) are fleshy, pedunculated masses
located on the axillae, trunk and eyelids.
Types
1. Skin Papilloma
a. Squamous papilloma occurs in the skin, cheek,
tongue, etc. (Figs 33.2 and 33.3)
• Soft papillomas are squamous papillomas. They
are seen in elderly patients on the eyelid as small,
soft, brownish swellings.
• Squamous papilloma can also be congenital,
sometimes multiple in number and can be sessile
or pedunculated.
b. Basal cell papilloma (seborrhoeic keratosis) is seen
on the trunk of elderly patients as brownish elevated
patch of skin and gives a semitransparent, oily
appearance.
Key Box 33.1
Overgrowth of Fibrous Tissue
Keloid : Page 106
Desmoid tumour : Page 975
Hypertrophic scar : Page 106
2. Arising from Mucous Membrane of
Visceral Or
gans
a. Transitional cell papilloma in the urinary bladder
as a cause of haematuria.
b. Columnar cell papilloma in the rectum as a cause of
mucous diarrhoea.
288
Tumours and Soft Tissue Sarcoma
289
Fig. 33.1: Structure of papilloma Fig. 33.2: Pedunculated papilloma
thigh—broad base
c. Cuboidal cell papilloma in the gallbladder.
d. Squamous papilloma in the larynx can cause respira-
tory obstruction.
e. Papilloma of breast (duct papilloma)
causes bleeding
per nipple.
LIPOMA: UNIVERSAL TUMOUR
Lipoma is a benign tumour arising from fat cells of adult
type. It is also called ‘universal tumour’ because it
can occur anywhere in the body where there is fat
(Key Box 33.2).
Treatment
Excision, only if papilloma causes discomfort, or if it is
symptomatic.
Subcutaneous—commonest type
Complications
1. Skin papilloma can get secondarily infected resulting
in pain and swelling.
. Ulceration and bleeding (trauma).
2
Soft to firm lobular swelling
‘Slip’ sign positive—a pathognomonic sign
Semifluctuant swelling
‘Smart’ dimple sign on movement of the skin
3. Papilloma in the breast, rectum, tongue and gallbladder can undergo malignant change.
Types
1. Single Encapsulated Lipoma
FIBROMA
Fibroma is a benign tumour, consisting of connective
tissue fibres only. Clinically, it presents as a firm,
subcutaneous swelling. However, a true fibroma is rare.
They are combined with neural elements, muscle tissue
1
• This is a single, soft, slow-growing, painless and
semifluctuant swelling (Fig. 33.4).
• The swelling is soft, may feel cystic with fluctua-
tion. This is also called pseudofluctuation because
fat at body temperature behaves like fluid.
or fatty tissue.
Fig. 33.3: Papilloma thigh with a narrow
base—easy to remove it. Ulceration can
be a problem here
Key Box 33.2
Diagnostic Features of Lipoma
Types
1. Soft fibromas: Less fibrous tissue
. Hard fibromas: More fibrous tissue
2
• Neurofibroma: Fibroma mixed with nerve fibres
• Fibrolipoma: Fibroma mixed with fat
• Myofibroma: Fibroma mixed with muscle fibres
• Angiofibroma: Fibroma mixed with blood vessels
Treatment
They are treated by excision because of the possibility
of developing into a sarcoma.
1
Students should not give the diagnosis of fibroma because in majority of cases, it is neurofibroma or fibrolipoma.
Fig. 33.4: Lipoma in the flank—commonest site of lipoma
Section II • General Surgery
290
Manipal Manual of Surgery
• Surface is lobular. Lobulations are better appre-
ciated with firm palpation of the swelling. Due to
the pressure, lobules bulge out between the fibrous
tissue strands.
• The edge slips under the palpating finger which is
a pathognomonic sign of lipoma.
• Commonly present as a subcutaneous swelling. It
is freely mobile. The flank is the commonest site.
Shoulder region, neck, back, upper limbs are the
other common sites. (For various locations, refer
to Table 33.1, Figs 33.5 and 33.6.) Some lipomas
from the chest wall can be of large size.
• Dimpling sign: Fibrous bands connect a lipoma
to the skin. When the skin moved, a dimple
appears on the skin.
2. Multiple Lipomatosis (Fig. 33.7)
• Such lipomas are multiple and very often tender
because of nerve elements mixed with them. Hence,
they are called multiple neurolipomatosis. Dercum’s
disease is one example of this variety (Adiposis dolorosa)
wherein tender, lipomatous swellings are present in
the body, mainly the trunk.
Fig. 33.5: Mediastinal lipoma. The
swelling was deep to pretracheal
fascia and partly substernal
soft and lobular—confused for
compressible swelling. Candidate
offered haemangioma as diagnosis.
[Courtesy: Dr Salim, Head, Department of Surgery, Trivandrum Medical
College, Kerala (PG exam case
2009)]
. It was
Fig. 33.6: Lipoma forehead.
An undergraduate gave
the diagnosis as dermoid
cyst. You see the location.
It is not the classical site of
dermoid cyst and the ‘slip
sign’ was positive. Even
though face is not a
common site, lipoma can
occur in this location.
Another differential diagnosis for this swelling is
sebaceous cyst
3. Uncapsulated Lipoma (Diffuse)
• Diffuse variety is a rare type of lipoma. It is called
pseudolipoma. It is an overgrowth of fat without a
capsule.
Table 33.1 Various types of lipoma
Location Presentation Differential diagnosis Significance
1. Subcutaneous Mobile Neurofibroma The most common variety
Shoulder Lobular
Flank Edge slips under palpating fingers
2. Subfascial Difficult to appreciate the edge and lobulation Implantation dermoid, Subfascial lipoma of the scalp—
Limbs, palm, sole TB tenosynovitis erodes bone
3. Subsynovial, Swelling in relation to knee joint, elbow joint Bursa Intra-articular lipomas are rare
intra-articular (Figs 33.10 and 33.11) Baker’s cyst
4. Intermuscular Swelling of the thigh. On contraction Fibrosarcoma Chances of developing
Thigh of the muscles, it becomes more Haematoma liposarcoma are more
Shoulder region firm due to transmitted pressure
5. Parosteal Under the periosteum of bone, feels hard Bony tumour Very, very rare
6. Submucous Asymptomatic or stridor or Intestinal tumour Intussusception
Intestines, larynx pain abdomen Laryngeal tumour
7. Subserosal Retroperitoneal swelling Hydronephrosis Liposarcoma
Retroperitoneum Retroperitoneal cyst
8. Extradural Very rare – –
9. Intraglandular Breast, pancreas Cystic lesions Very rare
Section II • General Surgery
Fig. 33.7: Multiple lipomas—being subcutaneous in location,
they become prominent on contraction of muscles
Tumours and Soft Tissue Sarcoma
291
Histological Types of Lipoma
1. Fibrolipoma: Since fibrous tissue is mixed with fat,
lipoma feels hard.
2. Neurolipoma: Painful lipoma, because of presence of
nerve elements.
3. Naevolipoma: Lipoma is usually relatively avascular
but this variety is vascular.
Treatment
• An incision is given over the swelling. Dissection is
carried out all around, separating it from underlying
tissues and it is excised (Fig. 33.8).
• Small lipoma can be removed by incising the skin
followed by squeezing the lipoma out (no dissection
method).
Complications
. Liposarcoma: The current view is that lipomas are
1
benign and do not turn into malignancy. However,
atypical retroperitoneal lipomas and lipoma in the
thigh can turn into liposarcoma after many years of
growth. Malignancy should be suspected when:
• The swelling grows rapidly (Fig. 33.9).
• It becomes painful due to infiltration of nerves.
• The swelling becomes vascular and red coloured
with dilated veins over the surface.
• Surface is warm due to increased vascularity.
• Skin fungation or fixation occurs later
• Mobility gets restricted because of infiltration into
deeper planes such as muscle.
• Liposarcoma spreads via blood. It rarely spreads
via lymphatics. Metastasis in the lung can rarely
occur from liposarcoma producing multiple chest
secondaries.
• Liposarcoma is treated by wide excision followed
by reconstruction either by split skin graft or by
flaps. In the thigh, sometimes radical surgery may
amount to compartmental excision. Chemotherapy
and radiotherapy
can also be used but the benefit
is doubtful.
2. Calcification
A 32-year-old man presented with gross swelling of the
right leg. He had seen two surgeons earlier who had told
him that he had deep vein thrombosis but no treatment
was offered. Examination revealed an obvious mass which
was palpable in anteromedial and posterior compartment.
MRI revealed an intermuscular mass. At exploration,
an intermuscular lipoma weighing 700 g was excised
(Figs 33.10 and 33.11).
3. Myxomatous degeneration: Occurs only in retro-
peritoneal lipoma.
4. Intussusception—due to submucosal lipoma of
terminal ileum is an abdominal emergency.
5. Saponification (see Key Box 33.3 and Figs 33.10 and
33.11).
Key Box 33.3
Some ‘Rare’ Facts in Lipoma
It is rare in children
Rarely gives rise to transillumination (if size is big)
Rarely gets infected (because it is relatively avascular)
Diffuse variety is rare
Neurolipomas are rare
Rarely they turn into malignancy
Fig. 33.8: Excised specimen
of lipoma—see the lobularity
Since 3 months, it is rapidly
growing, observe dilated
veins and shiny skin. There
is local rise of temperature.
These features are suggestive of sarcoma.
Fig. 33.9: Pedunculated lipoma of the back of 15 years duration
with features suggestive of sarcomatous change
Fig. 33.10: Compare both
the legs. Local gigantism
can also be caused by
extensive lipomatosis involving the leg. This was treated
initially as filariasis
Fig. 33.11: Soleus muscle is cut,
lipoma is seen coming out of the
deeper plane
neurovascular bundle was involved. The patient had a smooth
recovery
. Luckily no major
Section II • General Surgery
292
Manipal Manual of Surgery
NEURAL TUMOURS
NEUROMA
They are uncommon benign tumours which arise
from sympathetic nervous system or spinal cord.
They can be classified into true neuromas and false
neuromas.
True Neuroma
. Ganglioneuroma: It consists of ganglion cells and
1
nerve fibres of sympathetic chain. They are slowgrowing tumours. When present in the neck as a
parapharyngeal mass, it can cause dysphagia. These
tumours can occur in the neck, retroperitoneum or
mediastinum. Excision of the tumour is the treatment
(see clinical notes).
2. Neuroblastoma: It consists of poorly differentiated
cells. It occurs in young children. It is interesting to
know that this tumour can undergo spontaneous
regression.
3. Myelinic neuroma: It is very rare. It arises in relation-
ship with spinal cord made up of myelinic fibres.
• Does not contain any ganglion cells.
All these three tumours are called true neuromas.
False Neuroma
These tumours arise from the connective tissue of the
sheath of nerve endings.
They occur following nerve injuries, lacerations or
after amputation. They are of two types:
An 18-year-old engineering student who had backache
was examined by an orthopaedician and referred to
general surgery. CT scan of the abdomen revealed mass
in the paraspinal region in the retroperitoneum.
Laparotomy and excision of the mass was done. It was a
ganglioneuroma. Paraspinal region is one of the common
sites of ganglioneuroma.
1. End-neuroma occurs after amputation due to
proliferation of nerve fibres from the distal cut end
of the nerve. This produces a bulbous swelling. If it
is caught in the suture line or due to pressure of the
prosthesis, it produces severe neuralgic pain. To
avoid this, when an amputation is being done, the
nerve is pulled downwards and cut as high as
possible so that it retracts upwards (Fig.
33.12).
2. Lateral neuromas occur due to partial injury to the
Section II • General Surgery
nerve on the lateral aspect (Fig. 33.13).
Fig. 33.12: End-neuroma Fig. 33.13: Lateral neuroma
Treatment
Excision of the neuroma.
NEUROFIBROMA
It is a benign tumour arising from the connective tissue
of the nerve sheath. Typically, it produces a fusiform
swelling in the direction of the nerve fibres. The tumour
contains both neural (ectodermal) and fibrous
(mesodermal) elements.
Clinical Types
. Single Subcutaneous Neurofibroma (Local)
1
Commonly affects the peripheral nerves such as ulnar
nerve, median nerve or cutaneous nerves. Occurs in
adults.
Clinical features
• Presents as a painful, subcutaneous nodule.
• Tingling and numbness, paraesthesia in the distribution
of the nerve, specially when the nodule is compressed.
• Round to oval swelling in the direction of nerve fibre.
• Smooth surface, with round border. The swelling
moves at right angles to the direction of nerve fibres.
Vertical mobility is absent.
• Consistency is firm. Sometimes, it is hard.
• Being a subcutaneous swelling, the skin can be lifted
up.
Treatment
• It is treated by excision.
• In most of the cases, excision is easy as the tumour is
well encapsulated.
2. Generalised Neurofibromatosis: von Recklin-
ghausen
’s (vR) Disease (Type I) (Figs 33.14 to 33.16)
• This is an autosomal dominant disorder transmitted
by both sexes. The whole body is studded with
cutaneous nodules of varying sizes. They are soft and
nontender.
• Coffee brown pigmentation is characteristic of this
condition (c
afé au lait spots, Fig. 33.17). Café au lait
Tumours and Soft Tissue Sarcoma
293
Fig. 33.14: von Recklinghausen’s disease
with plexiform neurofibromatosis.
(Courtesy: Dr Prashanth Shetty, Professor,
Department of Surgery, KMC, Manipal) Fig. 33.16: von Recklinghausen’s disease—
Fig. 33.15: Case of vR disease with
multiple nodules, pigmentation and
schwannoma of vagus nerve. (Courtesy:
Dr Siddarth Bhandary, Professor, Department
of Surgery, KMC, Manipal)
tumours are multiple
, congenital, familial
Bilateral vestibular schwannoma or acoustic
neuromas are pathognomonic of neurofibromatosis
type 2, a syndrome resulting from chromosome 22
mutation. It is also associated with increased incidence
of meningiomas and gliomas.
3. Plexiform Neurofibromatosis (Trigeminal) (Fig. 33.18)
• In this condition, the
branches of 5th cranial
nerve are commonly
affected. It can also in-
volve the peripheries.
Fig. 33.17: The café au lait spots. More than five such spots will
appear by early life. (Courtesy: Dr Prashanth Shetty, Professor,
Department of Surgergy, KMC, Manipal)
• The affected part is grossly
thickened due to fibro-
myxomatous degenera-
tion.
spots can be associated with involvement of cranial
nerves
—VIIIth nerve (auditory nerve) acoustic
neuroma—a cerebellopontine angle tumour. Popularly
called vestibular schwannoma.
• Fibroepithelial skin tags are often present.
• Type I is caused by gene mu
tation on chromosome 17.
• The presence of skin pigmentation is an indication
of the common neuroectodermal origin of nerve
sheath cells and melanocytes.
• Skeletal deformities such as kyphoscoliosis or osteo-
porosis are common.
• It may be associated with phaeochromocytoma (high
blood pressure).
• Sarcomatous changes do occur.
• When it involves the
branches of trigeminal
nerve, following problems
can occur:
– Tingling paraesthesia
in the distribution of
Vth nerve, especially
ophthalmic division.
–
When it attains a huge size, it can obstruct the
Fig. 33.18: Plexiform neuro-
fibromatosis. (Courtesy: Dr
Rohit Jain, Assistant Professor,
Department of Surgery, KMC,
Manipal)
vision. As it grows bigger in size, it hangs in front
of the neck, as a grossly thickened pendulous fold
of skin.
• Treatment: Very difficult. Excision can be attempted
with plastic surgery repair.
Record the blood pressure in all cases of von Recklinghausen’s
disease because it may be associated with phaeochromocytoma.
4. Elephantiasis Neuromatosa
• This condition affects the limbs. It represents
an advanced stage of plexiform variety. Gross
thickening of subcutaneous tissue gives the ap-
Section II • General Surgery