Alexis Thomson and Alexander Miles - Manual of Surgery

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Among the rarer and more serious lesions of the inherited disease may be
mentioned gummatous disease in the _larynx and trachea_, attended with
ulceration and resulting in stenosis; and lesions of the _nervous
system_ which may result in convulsions, paralysis, or dementia.

In a limited number of cases, about the period of puberty there may
develop _deafness_, which is usually bilateral and may become absolute.

_Changes in the Permanent Teeth._--These affect specially the upper
central incisors, which are dwarfed and stand somewhat apart in the gum,
with their free edges converging towards one another. They are tapering
or peg-shaped, and present at their cutting margin a deep semilunar
notch. These appearances are commonly associated with the name of
Hutchinson, who first described them. Affecting as they do the
permanent teeth, they are not available for diagnosis until the child is
over eight years of age. Henry Moon drew attention to a change in the
first molars; these are reduced in size and dome-shaped through dwarfing
of the central tubercle of each cusp.

#Diagnosis of Inherited Syphilis.#--When there is a typical eruption on
the buttocks and snuffles there is no difficulty in recognising the
disease. When, however, the rash is scanty or is obscured by co-existing
eczema, most reliance should be placed on the distribution of the
eruption, on the brown stains which are left after it has passed off, on
the presence of condylomata, and of fissuring and scarring at the angles
of the mouth. The history of the mother relative to repeated
miscarriages and still-born children may afford confirmatory evidence.
In doubtful cases, the diagnosis may be aided by the Wassermann test and
by noting the therapeutic effects of grey powder, which, in syphilitic
infants, usually effects a marked and rapid improvement both in the
symptoms and in the general health.

While a considerable number of syphilitic children grow up without
showing any trace of their syphilitic inheritance, the majority retain
throughout life one or more of the following characteristics, which may
therefore be described as _permanent signs of the inherited disease_:
Dwarfing of stature from interference with growth at the epiphysial
junctions; the forehead low and vertical, and the parietal and frontal
eminences unduly prominent; the bridge of the nose sunken and rounded;
radiating scars at the angles of the mouth; perforation or destruction
of the hard palate; Hutchinson's teeth; opacities of the cornea from
antecedent keratitis; alterations in the fundus oculi from choroiditis;
deafness; depressed scars or nodes on the bones from previous gummata;
"sabre-blade" or other deformity of the tibiae.

#The Contagiousness of Inherited Syphilis.#--In 1837, Colles of Dublin
stated his belief that, while a syphilitic infant may convey the disease
to a healthy wet nurse, it is incapable of infecting its own mother if
nursed by her, even although she may never have shown symptoms of the
disease. This doctrine, which is known as _Colles' law_, is generally
accepted in spite of the alleged occurrence of occasional exceptions.
The older the child, the less risk there is of its communicating the
disease to others, until eventually the tendency dies out altogether, as
it does in the tertiary period of acquired syphilis. It should be
added, however, that the contagiousness of inherited syphilis is denied
by some observers, who affirm that, when syphilitic infants prove
infective, the disease has been really acquired at or soon after birth.

There is general agreement that the subjects of inherited syphilis
cannot transmit the disease by inheritance to their offspring, and that,
although they very rarely acquire the disease _de novo_, it is possible
for them to do so.

#Prognosis of Inherited Syphilis.#--Although inherited syphilis is
responsible for a large but apparently diminishing mortality in infancy,
the subjects of this disease may grow up to be as strong and healthy as
their neighbours. Hutchinson insisted on the fact that there is little
bad health in the general community that can be attributed to inherited
syphilis.

#Treatment.#--Arsenical injections are as beneficial in the inherited as
in the acquired disease. An infant the subject of inherited syphilis
should, if possible, be nursed by its mother, and failing this it should
be fed by hand. In infants at the breast, the drug may be given to the
mother; in others, it is administered in the same manner as already
described--only in smaller doses. On the first appearance of syphilitic
manifestations it should be given 0.05 grm, novarsenbillon, injected
into the deep subcutaneous tissues every week for six weeks, followed by
one year's mercurial inunction--a piece of mercurial ointment the size
of a pea being inserted under the infant's binder. In older children the
dose is proportionately increased. The general health should be improved
in every possible direction; considerable benefit may be derived from
the use of cod-liver oil, and from preparations containing iron and
calcium. Surgical interference may be required in the destructive
gummatous lesions of the nose, throat, larynx, and bones, either with
the object of arresting the spread of the disease, or of removing or
alleviating the resulting deformities. In children suffering from
keratitis, the eyes should be protected from the light by smoked or
coloured glasses, and the pupils should be dilated with atropin from
time to time, especially in cases complicated with iritis.

#Acquired Syphilis in Infants and Young Children.#--When syphilis is met
with in infants and young children, it is apt to be taken for granted
that the disease has been inherited. It is possible, however, for them
to acquire the disease--as, for example, while passing through the
maternal passages during birth, through being nursed or kissed by
infected women, or through the rite of circumcision. The risk of
infection which formerly existed by the arm-to-arm method of
vaccination has been abolished by the use of calf lymph.

The clinical features of the acquired disease in infants and young
children are similar to those observed in the adult, with a tendency,
however, to be more severe, probably because the disease is often late
in being recognised and treated.

CHAPTER X

TUMOURS[2]

Definition--Etiology--General characters of innocent and malignant
tumours. CLASSIFICATION OF TUMOURS: I. Connective-tissue tumours:
(1) _Innocent_: _Lipoma_, _Xanthoma_, _Chondroma_, _Osteoma_,
_Odontoma_, _Fibroma_, _Myxoma_, _Endothelioma_, etc.; (2)
_Malignant_: _Sarcoma_--II. Epithelial tumours: (1) _Innocent_:
_Papilloma_, _Adenoma_, _Cystic Adenoma_; (2) _Malignant_:
_Epithelioma_, _Glandular Cancer_, _Rodent Cancer_, _Melanotic
Cancer_--III. Dermoids--IV. Teratoma. Cysts: _Retention_,
_Exudation_, _Implantation_, _Parasitic_, _Lymphatic or Serous_.
Ganglion.

[2] For the histology of tumours the reader is referred to a text-book
of pathology.

A tumour or neoplasm is a localised swelling composed of newly formed
tissue which fulfils no physiological function. Tumours increase in size
quite independently of the growth of the body, and there is no natural
termination to their growth. They are to be distinguished from such
over-growths as are of the nature of simple hypertrophy or local
giantism, and also from inflammatory swellings, which usually develop
under the influence of a definite cause, have a natural termination, and
tend to disappear when the cause ceases to act.

The _etiology of tumours_ is imperfectly understood. Various factors,
acting either singly or in combination, may be concerned in their
development. Certain tumours, for example, are the result of some
congenital malformation of the particular tissue from which they take
origin. This would appear to be the case in many tumours of blood
vessels (angioma), of cartilage (chondroma), of bone (osteoma), and of
secreting gland tissue (adenoma). The theory that tumours originate from
foetal residues or "rests," is associated with the name of Cohnheim.
These rests are supposed to be undifferentiated embryonic cells which
remain embedded amongst fully formed tissue elements, and lie dormant
until they are excited into active growth and give rise to a tumour.
This mode of origin is illustrated by the development of dermoids from
sequestrated portions of epidermis.

Among the local factors concerned in the development of tumours,
reference must be made to the influence of irritation. This is probably
an important agent in the causation of many of the tumours met with in
the skin and in mucous membranes--for example, cancer of the skin, of
the lip, and of the tongue. The part played by injury is doubtful. It
not infrequently happens that the development of a tumour is preceded by
an injury of the part in which it grows, but it does not necessarily
follow that the injury and the tumour are related as cause and effect.
It is possible that an injury may stimulate into active growth
undifferentiated tissue elements or "rests," and so determine the growth
of a tumour, or that it may alter the characters of a tumour which
already exists, causing it to grow more rapidly.

The popular belief that there is some constitutional peculiarity
concerned in the causation of tumours is largely based on the fact that
certain forms of new growth--for example, cancer--are known to occur
with undue frequency in certain families. The same influence is more
striking in the case of certain innocent tumours--particularly multiple
osteomas and lipomas--which are hereditary in the same sense as
supernumerary or webbed fingers, and appear in members of the same
family through several generations.

INNOCENT AND MALIGNANT TUMOURS

For clinical purposes, tumours are arbitrarily divided into two
classes--the innocent and the malignant. The outstanding difference
between them is, that while the evil effects of innocent tumours are
entirely local and depend for their severity on the environment of the
growth, malignant tumours wherever situated, in addition to producing
similar local effects, injure the general health and ultimately cause
death.

_Innocent_, benign, or simple tumours present a close structural
resemblance to the normal tissues of the body. They grow slowly, and are
usually definitely circumscribed by a fibrous capsule, from which they
are easily enucleated, and they do not tend to recur after removal. In
their growth they merely push aside and compress adjacent parts, and
they present no tendency to ulcerate and bleed unless the overlying skin
or mucous membrane is injured. Although usually solitary, some are
multiple from the outset--for example, fatty, fibrous, and bony tumours,
warts, and fibroid tumours of the uterus. They produce no constitutional
disturbance. They only threaten life when growing in the vicinity of
vital organs, and then only in virtue of their situation--for example,
death may result from an innocent tumour in the air-passage causing
suffocation, in the intestine causing obstruction of the bowels, or in
the vertebral canal causing pressure on the spinal medulla.

_Malignant tumours_ usually show a marked departure from the structure
and arrangement of the normal tissues of the body. Although the cells of
which they are composed are derived from normal tissue cells, they tend
to take on a lower, more vegetative form; they may be regarded as
parasites living at the expense of the organism, multiplying
indefinitely and destroying everything with which they come in contact.

Malignant tumours grow more rapidly than innocent tumours, and tend to
infiltrate their surroundings by sending out prolongations or offshoots;
they are therefore liable to recur after an operation which is
restricted to the removal of the main tumour. They are not encapsulated,
although they may appear to be circumscribed by condensation of the
surrounding tissues; they are rarely multiple at the outset, but show a
marked tendency to spread to other parts of the body. Fragments of the
parent tumour may become separated and be carried off in the lymph or
blood-stream and deposited in other parts of the body, where they give
rise to secondary growths. Malignant tumours tend to invade and destroy
the overlying skin or mucous membrane, and thus give rise to bleeding
ulcers; if the tumour tissue protrudes through the gap in the skin, it
is said to _fungate_. In course of time they give rise to a condition of
ill-health or _cachexia_, the patient becoming pale, sallow, feverish,
and emaciated, probably as a result of chronic poisoning from the
absorption of toxic products from the tumour. They ultimately destroy
life, it may be by their local effects, such as ulceration and
haemorrhage, by favouring the entrance of septic infection, by
interfering with the function of organs which are essential to life, by
cachexia, or by a combination of these effects.

The situation of a malignant tumour exercises considerable influence on
the rapidity, as well as on the mode, in which it causes death. Some
cancers, such as that known as "rodent," show malignant features which
are entirely local, while others, such as melanotic cancer, exhibit a
malignancy characterised by rapid generalisation of growths throughout
the body. Tumours that are structurally alike may show variations in
malignancy, according to their situation and to the age of the patient,
as well as to other factors which are as yet unknown.

In attempting to arrive at a conclusion as to the innocence or
malignancy of any tumour, too much reliance must not be placed on its
histological features; its situation, rate of growth, and other clinical
features must also be taken into consideration. It cannot be too
emphatically stated that there is no hard-and-fast line between innocent
and malignant growths; there is an indefinite transition from one to the
other. The possibility of the transformation of a benign into a
malignant tumour must be admitted. Such a transformation implies a
change in the structure of the growth, and has been observed especially
in fibrous and cartilaginous tumours, in tumours of the thyreoid gland,
and in uterine fibroids. The alteration in character may take place
under the influence of injury, prolonged or repeated irritation,
incomplete removal of the benign tumour by operation, or the altered
physiological conditions of the tissues which attend upon advancing
years.

After a tumour has been removed by operation it should as a routine
measure be subjected to microscopical examination; the results are often
instructive and sometimes other than what was expected.

#Varieties of Tumours.#--In the following description, tumours are
classified on an anatomical basis, taking in order first the
connective-tissue group and subsequently those that originate in
epithelium.

INNOCENT CONNECTIVE-TISSUE TUMOURS

#Lipoma.#--A lipoma is composed of fat resembling that normally present
in the body. The commonest variety is the _subcutaneous lipoma_, which
grows from the subcutaneous fat, and forms a soft, irregularly lobulated
tumour (Fig. 45). The fat is arranged in lobules separated by
connective-tissue septa, which are continuous with the capsule
surrounding the tumour and with the overlying skin, which becomes
dimpled or puckered when an attempt is made to pinch it up. As the fat
is almost fluid at the body temperature, fluctuation can usually be
detected. These tumours vary greatly in size, occur at all ages, grow
slowly, and, while generally solitary, are sometimes multiple. They are
most commonly met with on the shoulder, buttock, or back. In certain
situations, such as the thigh and perineum, they tend to become
pedunculated (Fig. 46).

A fatty tumour is to be diagnosed from a cold abscess and from a cyst.
The distinguishing features of the lipoma are the tacking down and
dimpling of the overlying skin, the lobulation of the tumour, which is
recognised when it is pressed upon with the flat of the hand, and, more
reliable than either of these, the mobility, the tumour slipping away
when pressed upon at its margin.

[Illustration: FIG. 45.--Subcutaneous Lipoma showing lobulation.]

The prognosis is more favourable than in any other tumour as it never
changes its characters; the only reasons for its removal by operation
are its unsightliness and its probable increase in size in the course of
years. The operation consists in dividing the skin and capsule over the
tumour and shelling it out. Care must be taken that none of the outlying
lobules are left behind. If the overlying skin is damaged or closely
adherent, it should be removed along with the tumour.

[Illustration: FIG. 46.--Pedunculated Lipoma of Buttock of forty years'
duration in a woman aet. 68.]

_Multiple subcutaneous lipomas_ are frequently symmetrical, and in a
certain group of cases, met with chiefly in women, pain is a prominent
symptom, hence the term _adiposis dolorosa_ (Dercum). These multiple
tumours show little or no tendency to increase in size, and the pain
which attends their development does not persist.

In the neck, axilla, and pubes a diffuse overgrowth of the subcutaneous
fat is sometimes met with, forming symmetrical tumour-like masses, known
as _diffuse lipoma_. As this is not, strictly speaking, a tumour, the
term _diffuse lipomatosis_ is to be preferred. A similar condition was
described by Jonathan Hutchinson as being met with in the domestic
animals. If causing disfigurement, the mass of fat may be removed by
operation.

[Illustration: FIG. 47.--Diffuse Lipomatosis of Neck.]

_Lipoma in other Situations._--The _periosteal lipoma_ is usually
congenital, and is most often met with in the hand; it forms a
projecting lobulated tumour, which, when situated in the palm, resembles
an angioma or a lymphangioma. The _subserous lipoma_ arises from the
extra-peritoneal fat in the posterior abdominal wall, in which case it
tends to grow forwards between the layers of the mesentery and to give
rise to an abdominal tumour; or it may grow from the extra-peritoneal
fat in the anterior abdominal wall and protrude from one of the hernial
openings or through an abnormal opening in the parietes, constituting a
_fatty hernia_. A _subsynovial lipoma_ grows from the fat surrounding
the synovial membrane of a joint, and projects into its interior, giving
rise to the symptoms of loose body. Lipomas are also met with growing
from the adipose connective tissue _between or in the substance of
muscles_, and, when situated beneath the deep fascia, such as the fascia
lata of the thigh, the characteristic signs are obscured and a
differential diagnosis is difficult. It may be differentiated from a
cold abscess by puncture with an exploring needle.

[Illustration: FIG. 48.--Zanthoma of Hands in a girl aet. 14, showing
multiple subcutaneous tumours (cf. Fig. 49).

(Sir H. J. Stiles' case.)]

#Zanthoma# is a rare but interesting form of tumour, composed of a
fibrous and fatty tissue, containing a granular orange-yellow pigment,
resembling that of the corpus luteum. It originates in the corium and
presents two clinical varieties. In the first of these, it occurs in the
form of raised yellow patches, usually in the skin of the eyelids of
persons after middle life, and in many instances is associated with
chronic jaundice; the patches are often symmetrical, and as they
increase in size they tend to fuse with another.

The second form occurs in children and adolescents; it may affect
several generations of the same family, and is often multiple, there
being a combination of thickened yellow patches of skin and projecting
tumours, some of which may attain a considerable size (Figs. 48 and 49).
On section, the tumour tissue presents a brilliant orange or saffron
colour.

There is no indication for removing the tumours unless for the deformity
which they cause; exposure to the X-rays is to be preferred to
operation.

[Illustration: FIG. 49.--Zanthoma showing Subcutaneous Tumours on
Buttocks. From same patient as Fig. 48.]

#Chondroma.#--A chondroma is mainly composed of cartilage. Processes of
vascular connective tissue pass in between the nodules of cartilage
composing the tumour from the fibrous capsule which surrounds it. On
section it is of a greyish-blue colour and semi-translucent. The tumour
is firm and elastic in consistence, but certain portions may be densely
hard from calcification or ossification, while other portions may be
soft and fluctuating as a result of myxomatous degeneration and
liquefaction. These tumours grow slowly and painlessly, and may surround
nerves and arteries without injuring them. They may cause a deep hollow
in the bone from which they originate. All intermediate forms between
the innocent chondroma and the malignant chondro-sarcoma are met with.
Chondroma may occur in a multiple form, especially in relation to the
phalanges and metacarpal bones. When growing in the interior of a bone
it causes a spindle-shaped enlargement of the shaft, which in the case
of a phalanx or metacarpal bone may resemble the dactylitis resulting
from tubercle or syphilis. A chondroma appears as a clear area in a
skiagram.

A _skiagram_ of a bone in which there is a chondroma shows a clear
rounded area in the position of the tumour, which must be differentiated
from similar clear areas due to other kinds of tumour, especially the
myeloma; when it has undergone calcification or ossification, it gives a
shadow as dark as bone.

[Illustration: FIG. 50.--Chondroma growing from infraspinous fossa of
Scapula.]

[Illustration: FIG. 51.--Chondroma of Metacarpal Bone of Thumb.]

_Treatment._--In view of the unstable quality of the chondroma,
especially of its liability to become malignant, it should be removed as
soon as it is recognised. In those projecting from the surface of a
bone, both the tumour and its capsule should be removed. If in the
interior, a sufficient amount of the cortex should be removed to allow
of the tumour being scraped out, and care must be taken that no nodules
of cartilage are left behind. In multiple chondromas of the hand, when
the fingers are crippled and useless, exposure to the X-rays should be
given a trial, and in extreme cases the question of amputation may have
to be considered. When a cartilaginous tumour takes on active growth, it
must be treated as malignant.

The chondromas that are met with at the ends of the long bones in
children and young adults form a group by themselves. They are usually
related to the epiphysial cartilage, and it was suggested by Virchow
that they take origin from islands of cartilage which have not been used
up in the process of ossification. They are believed to occur more
frequently in those who have suffered from rickets. They have no
malignant tendencies and tend to undergo ossification concurrently with
the epiphysial cartilage from which they take origin, and constitute
what are known as _cartilaginous exostoses_. These are sometimes met
with in a multiple form, and may occur in several generations of the
same family. They are considered in greater detail in the chapter
dealing with tumours of bone.

Minute nodules of cartilage sometimes form in the synovial membrane of
joints and lining of tendon sheaths and bursae: they tend to become
detached from the membrane and constitute loose bodies; they also
undergo a variable amount of calcification and ossification, so as to be
visible in skiagrams. They are further considered with loose bodies in
joints.

Cartilaginous tumours in the parotid, submaxillary gland, and testicle
belong to a class of "mixed tumours" that will be referred to later.

#Osteoma.#--The true osteoma is composed of bony tissue, and originates
from the skeleton. Two varieties are recognised--the spongy or
cancellous, and the ivory or compact. The _spongy_ or _cancellous
osteoma_ is really an ossified chondroma, and is met with at the ends of
the long bones (Fig. 52). From the fact that it projects from the
surface of the bone it is often spoken of as an _exostosis_. It grows
slowly, and rarely causes any discomfort unless it presses upon a
nerve-trunk or upon a bursa which has developed over it. The Rontgen
rays show a dark shadow corresponding to the ossified portion of the
tumour, and continuous with that of the bone from which it is growing
(Fig. 138). Operative interference is only indicated when the tumour is
giving rise to inconvenience. It is then removed, its base or neck being
divided by means of the chisel. The multiple variety of osteoma is
considered with the diseases of bone.

The bony outgrowth from the terminal phalanx of the great toe--known as
the _subungual exostosis_--is described and figured on p. 404. Bony
projections or "spurs" sometimes occur on the under surface of the
calcaneus, and, projecting downwards and forwards from the greater
process, cause pain on putting the heel to the ground.

[Illustration: FIG. 52.--Cancellous Osteoma of lower end of Femur.]