but corresponds with the generally-understood principles.
DURATION.--It is very difficult to determine accurately the duration of
malignant diseases of the mediastinum, since for a long time the
patient may be quite free from any local subjective symptom, even
though a growth may have attained to a considerable size. Moreover,
intra-thoracic malignant disease, especially in the non-cancerous
varieties and if the digestive tract be normal, may be unattended by
any of those symptoms commonly associated with malignant process, such
as a peculiar tint of skin, progressive and great emaciation, or the
aspect of suffering. Sarcomatous tumors usually grow rapidly, as in a
case related by Jaccoud, in which death occurred within eight days
after admission to the hospital. Prior to this time the patient had
suffered from no objective symptoms whatever, although when admitted
there was physical evidence of a large growth extending from the
clavicle to the nipple.[26] West records a fatal case at two and a half
months; Horstman, one in which the disease originated on the right of
the sternum, as evidenced by a very small area of dulness; the entire
right side of the thorax was invaded within five weeks.[27] Berevidge
reports a case of sudden death from hæmoptysis in a man aged sixty-four
years, who up to that time had appeared healthy, and only a few days
before had complained of a slight cough and a feeling of oppression in
the chest. At the autopsy two cancerous masses the size of a hazelnut
were found, one of which overlaid a bronchus which was ulcerated to a
considerable extent. The bronchi were filled with blood. Virchow
mentions a case the duration of which was only two months. Walsh,
speaking of malignant growths in general, assigns three and a half
months as the minimum duration of these cases.
[Footnote 26: _Leçon de Clin. méd._, p. 636, Paris, 1867.]
[Footnote 27: _Trans. Path. Soc. London_, 1883.]
Undoubtedly, the duration will depend on the freedom from pressure upon
the oesophagus, or from interference with digestion due to
pneumogastric irritation, or from malignant processes in the stomach or
intestines. Pain, and consequent loss of sleep, will also accelerate
the termination of any case. Lebert assumes an average duration of
thirteen months, and Walsh states the maximum duration in
intra-thoracic malignant processes at twenty-seven months. The soft
secondary malignant sarcomata or carcinomata grow more quickly, and
have a relatively shorter course, than the harder forms of the same
species. Lymphadenoma may persist a long time, and appear for a while
to be stationary and unattended by any serious impairment of the
general {876} health, but the cases are exceptional. The persistence of
fibrous, fatty, or cystic tumors depends chiefly on the mechanical
inconvenience occasioned by them. All forms of malignant intra-thoracic
disease, however, are steadily progressive to a fatal termination.
Death commonly arises from the gradual increase in seriousness of the
pressure symptoms. Inability to lie down, harassing cough, want of
sleep, all tend to induce fatigue which may prove fatal. Deficient
aëration of the blood may occasion stupor, or sudden simultaneous
pleural and pericardial effusion or general pulmonary oedema may
terminate the scene. In exceptional instances death has resulted from
laryngeal spasm or from acute hypertrophy of the thyroid gland with
tracheal occlusion. In a remarkable case reported by Bennett paroxysmal
dyspnoea had been the only symptom of intra-thoracic disease for a few
months, when suddenly a severe seizure occurred which persisted
uninterruptedly for three days, till weakness and exhaustion terminated
in death by asphyxia. In this case the thyroid gland was found enlarged
to the size of a double fist, but the enlargement was mainly below the
sternum and along the sides of the trachea, which was literally
surrounded by the greatly-enlarged and firm lateral lobes of the
thyroid, so as to be completely flattened laterally. The structure of
the thyroid appeared healthy, but very firm, and the enlargement was
due solely to hypertrophy, and not to cystic or other disease, nor was
there any exophthalmos.[28] Death is possible from sudden asthmatic
attack, or, more rarely of all, by hæmoptysis.
[Footnote 28: See "Cancerous and Other Intra-thoracic Growths,"
Bennett, _The Lumleian Lect._, 1872, p. 169.]
PROGNOSIS.--The prognosis is invariably unfavorable, and must continue
so unless the more recent attempts for removal of primary growths in
the anterior thoracic regions yield grounds for a more hopeful outlook.
We may also hope that some remedy may influence or control the
development of lymphoma. Considerable relief may be obtained by rest,
suitable feeding, careful regulation of the digestive system, and such
hygienic measures as may seem most available.
DIAGNOSIS.--From Aneurism.--When we consider that in the diagnosis of
aneurism of the aorta every sign and symptom has in turn been found
fallacious in the ever-varying conditions under which aneurisms appear,
and that one is forced to say that aneurism has no pathognomonic signs
or symptoms, the difficulties in the way of the diagnosis of
intra-thoracic morbid growths may be recognized. Moreover, the
diversity in the peculiarities of each case, the multifarious character
of the pressure symptoms and physical signs, and the absence of a
precise order of phenomena peculiar to tumors in this situation, may
render a positive diagnosis in the early stages very difficult.
Aneurism in the absence of unequivocal signs of its existence may be
excluded on the following grounds: the absence of conditions which
predispose to disease of the coats of the arteries--_i.e._ syphilis,
alcoholism, Bright's disease, rheumatism, laborious avocations, violent
exercise. Aneurism may occur at any age, but it is rare before the age
of thirty years, and most prevalent between the ages of forty and fifty
years. Aneurism is also less frequent in the female sex. The distal
pressure symptoms of aneurism are more variable than in other morbid
growths of the mediastinum, and especially dysphagia is less constant.
Great emaciation without intense pain is adverse to the diagnosis of
aneurism, while severe pain with occasional exacerbations is favorable
to this diagnosis. However, instances of morbid growths are recorded in
which intercosto-humeral neuralgia was an initial symptom.
"An extensive area of dulness must in aneurism mean a large sac, and
with such a large tumor we should almost invariably get marked
expansive pulsation. Again, aneurismal sacs, before they produce
extensive dulness in {877} any portion of the parietes of the chest,
point, as it were, in some particular direction, becoming distinctly
prominent and producing an eccentric motion around them in consequence
of the thoracic parietes being absorbed or yielding at the point of
greatest pressure" (Graves). Hæmoptysis may occur not only from
aneurismal leakage, but from the effects of pressure of morbid growths
upon a bronchus or the invasion of the same by the malignant process.
Blood-spitting cannot therefore be regarded as an important
differential symptom. Unless valvular disease be associated with
aneurism, the displacement of the heart is less frequent in aneurism
than in morbid growths.
From Abscess.--The etiological relation in this process is traumatic,
or mediastinal abscess occurs in connection with caries or fracture or
after an operation in the neighborhood of the throat or neck, or of
suppurative disease elsewhere in the thorax, as abscess of the lung or
empyema. The pain in cases of abscess is deep-seated, constant, slowly
increasing, rather than the paroxysmal pain of aneurism or solid tumor.
The febrile movement may afford decided aid in the diagnosis, but it is
also true that high temperature may mark the progress of
lymphadenomata, as in Bennett and Sutton's case, in which from Jan.
11th to Feb. 28th the thermometrical wave vibrated between 103.5°
maximum, with a pulse of 148 per minute, to 100.5° minimum, with a
pulse of 108. In this remarkable case sweating was also a prominent
feature; and a somewhat similar example has been recorded by Murchison.
In corresponding circumstances the existence of secondary processes in
the lungs or elsewhere, with enlarged glands in the neck, may prevent
error. In mediastinal abscess there will probably be a tendency to
point, with the appearance of a fluctuating, circumscribed, superficial
tumor at the sternal border or adjacent to this bone. There may also be
tenderness on pressure associated with the pain, and an oedematous
condition of the tissues of that portion of the sternal region covering
the tumor, although this symptom sometimes attends malignant new
formations. Pulsation may accompany abscess, but will be of the
transmitted variety. In suspicious cases the sternal bone can be
drilled and an exploratory needle introduced into the tumor.
The general diagnosis of mediastinal tumor can be more easily made upon
the basis of regional invasion. But in any suspicious case an elaborate
and thorough clinical history is an essential prerequisite. In
proportion as one completes the natural history of a case of obscure
intra-thoracic disease the more likely one is to approach by exclusion
a correct interpretation of the existing physical signs and symptoms.
Growths in the Anterior Mediastinum.--Tumors located in the anterior
mediastinal space overlie the heart and aorta, and consequently the
heart-sounds, especially the second, may be indistinct or muffled; or
the second sounds may be audible in some new situation, owing to
displacement of the heart. The sternal region may be distinctly
prominent or bulged, and at the notch the bone may appear thickened.
The resonance in the interscapular regions remains unimpaired, but over
the sternum percussion should yield a very dull sound if the growth be
large, but when a comparatively small tumor exists the sternal
resonance will be hardened and high-pitched. An additional explanation
of this modification exists in instances where the growth is not
adherent to the sternum and the bone is arched over the tumor.
The respiration may be whistling or stridulous if the stethoscope is
placed over the trachea, and over one or other apex anteriorly the
respiratory murmur may be feeble or blowing, in proportion to the
volume of air which is permitted to enter the chest. Posteriorly, the
respiratory murmur may be unaffected at first, although as the growth
advances evidence of pressure on the bronchial tubes may be detected
over the interscapular region. The superficial veins of the chest may
be enlarged, especially those below the level of the upper segment of
the sternum. Dysphagia is usually slight in {878} proportion to the
other pressure symptoms or entirely absent. It may be simply a symptom
of irritation of the intra-thoracic nerves or due to enlargement of the
glands of the mediastinum.
Mediastinal growths usually develop in the middle line; they spread in
all directions, especially laterally, but avoid at first the roots of
the lungs. Pressure is rather exercised upon the parts in the mesial
line. They reach a large size and grow with great rapidity, producing
symptoms rather as a consequence of their size than by virtue of
contractile properties.
From Pericarditis.--A possible pericarditis may be mistaken for a tumor
of the anterior mediastinum. The diagnosis of pericarditis must be
sustained by evidence showing the dependence of this process upon
rheumatism, syphilis, nephritis, or propagated inflammation. The
distension of the pericardial sac due to pericarditis exhibits a
definite outline. The dulness of a tumor is irregular, with a tier of
dulness upon a higher level than in effusion. The absence of various
pressure signs is marked in pericarditis, while disturbance of the
heart's rhythm is more frequent. Kussmaul states that there are two
signs characteristic of chronic pericardial inflammation with
thickening and adhesion--viz.: a "complete or almost complete failure
of the radial pulse during inspiration, and simultaneously visible
swelling of the great veins of the neck, instead of the collapse that
usually takes place during this portion of the expiratory act. Adhesion
of the great vessels to the sternum, either directly or through the
medium of the pericardium, is supposed to account for these phenomena."
Febrile movement is usually present in pericarditis, and, while a
possible temporary feature in new growths, is not persistent unless
complicated by inflammation in the pulmonary tissues. Finally, the
progress of the case will often decide the question.
Growths in the Posterior Mediastinum.--In growths located in the
posterior mediastinum one or the other bronchus is one of the earliest
structures implicated by the pressure, because in these cases the chief
mass of tumor is found at the root of the lung. Secondary lesions in
the lungs directly traceable to pressure are frequent, but unilateral,
although secondary cancer from malignant lesions elsewhere than in the
lungs may be bilateral. Pressure symptoms as a class occur early, are
grave, constant, and progressive. Percussion according to directions of
Guéneau de Mussy may be made available. Abolition or great impairment
of breath and voice sounds over one or other posterior aspect of the
chest is the rule, since these tumors are prone to contraction.
Sometimes the respiratory murmur is whistling or blowing if the
bronchial pressure is less decided. Progressive emaciation and cachexia
are commonly present, not only from the inherent tendencies of the
disease, but also depending upon the disturbance of the functions of
many important organs which have been encroached upon by the tumor. The
exclusion of a malignant disease of the oesophagus is very difficult.
The passage of a bougie might determine the seat of obstruction, and
thus assist in the diagnosis, but great caution must be observed lest
penetration of the softened tissues occur. (See Cancer of Oesophagus.)
From Pleural Effusion.--The greatest difficulty may be experienced in
deciding between uncomplicated pleurisy and effusion complicated by
morbid growth.
Aside from the history of the case and state of nutrition, paracentesis
may aid the diagnosis, since, if the fluid is turbid, highly
albuminous, with a large proportion of coagulable fibrin, it is an
evidence of inflammatory origin; but if it is clear, limpid, and on
standing gives but a delicate veil of pseudo-fibrin, it indicates a
passive or mechanical cause. Hemorrhagic exudation is only of relative
importance. The recognition of pleural friction râles over parts flat
on percussion will be an evidence of tumor. Hæmoptysis in this {879}
association would negative the idea of simple effusion. The presence of
signs of pressure on central parts is indicative of tumor (Walsh), but
Powell has recorded an instance of simple pleural effusion accompanied
by husky voice and laryngeal cough; and also an instance in which, from
a similar cause, there was increased size, tortuosity, and throbbing of
the radial and brachial arteries on the affected side without oedema of
the limb, yet probably attributable to obstruction of the return
circulation.
Enlarged glands in the neck, or enlarged veins with evidence of
thrombosis of the descending vena cava, would indicate tumor. Dulness
from a tumor itself might resemble sacculated effusion, yet there might
be retraction in place of distension of the chest, and particularly
characteristic dulness in the mediastinal region as compared with the
circumferential regions, or peripheral patches of resonance may be
suggestive and lead to critical revision of the symptoms.
From Chronic Pneumonia.--Mediastinal growth invading the lung from its
root has been mistaken for chronic pneumonia. Walsh lays stress on the
following signs as distinguishing tumor: 1. A tendency to increase
instead of diminution of bulk of the affected side. 2. Implication of
the mediastinum, with dyspnoea out of proportion to the extent of
consolidation. 3. Different characters of respiration in the two
diseases. To these may be added pressure symptoms in general in cases
of tumor, with displacement of the heart toward the side unaffected by
the pulmonary process. Hæmoptysis is very often a concomitant of
bronchial pressure, but occurs so frequently in basic pneumonia,
especially in the syphilitic, that it is devoid of importance except
from the standpoint of relative investigation. With reference to
symptoms of bronchial irritation without assignable cause, we should
always do well to remember the observation of Stokes, that they may be
characteristic of disseminated morbid process.
Differentiation of Malignant Growths.--The younger the patient the more
probable the existence of lymphoma or sarcoma. The majority of primary
tumors of the mediastinum are lymphomatous, and when the growths
originate in the anterior space they are almost certainly
lympho-sarcoma or sarcoma. Widespread enlargement of the lymphatic
glands, with or without enlargement of the spleen, indicates a
lymphadenoma.
Finally, primary lympho-sarcoma or sarcoma tends to spread by extension
of the process by continuity of structure, although secondary forms of
the process present lesions distributed through the lungs.
The evidence in favor of sarcoma may be drawn from exclusion of the
other forms of morbid process, from the rapidity of the growth, and
from the history of previous operative interference for the removal of
foreign growth, especially if the previous disease were sarcomatous.
Carcinomata may be suspected in cases in which there has been an
hereditary predisposition to carcinomatous disease or the previous or
concomitant existence of cancerous disease in the mammæ or elsewhere,
particularly if the period of life is relatively advanced. The
development of the tumor may be more slow than other forms of growth,
and is associated with tendency to progressive emaciation in the
absence of evidences of direct pressure on the oesophagus and the
existence of cachexia. Carcinomatous disease is more commonly
coincident with the presence of hard, nodular, immovable masses in the
neck.
Cystic tumors present signs of fluctuation. Syphilitic gummata must be
diagnosticated by exclusion and the existence of the syphilitic
history. The possibility of substernal thickening due to syphilis, with
reflex disturbances, particularly oesophageal spasm, must be borne in
mind.
Those rare forms of disease due to hyperplasia or caseous deposit in
the thoracic glands, independent of pulmonary disease, must be
recognized by {880} exclusion. The fact must be remembered that with
great enlargement of glands in the neck and elsewhere the bronchial
glands may remain constantly unaffected.
TREATMENT.--From the inaccessible location of these growths but little
assistance can be rendered by surgery. The progress of this branch of
science has of recent years included resection or excision of the
sternum or some of the ribs for the removal of growths involving the
mediastinum or pleura. Küster[29] has successfully made partial
resections of the sternum for the removal of mediastinal tumors, and
the entire bone has been excised by König[30] in a case of sarcoma. The
pericardial and both pleural cavities were opened in the course of the
dissection; the wound became gangrenous, and the heart was afterward
surrounded with pus: notwithstanding this, the wound slowly healed and
the patient ultimately recovered. In cases treated by this method
pleural adhesions usually prevent double pneumothorax; portions of the
ribs have been resected with the sternum, and have been succeeded by
unilateral pneumothorax, and recovery has ensued. (See Fig. 55.)
[Footnote 29: _Berliner klinische Wochenschrift_, No. 20, 1883, pp.
127, 136, 274.]
[Footnote 30: _Centralblatt f. Chir._, No. 42, 1882.]
[Illustration: FIG. 55. From a case of Kolaczek's, in which the
resection of the third to the sixth ribs, with a portion of the
sternum, was practised for the removal of an enchondroma. Diagram
exhibits the aperture in the thoracic wall which permitted the exposure
of the pericardium. Pneumothorax occurred, but patient recovered
(_Deutsches Archiv für klinische Medicin_, Bd. xxx. 1882).]
Paracentesis must sometimes be practised to relieve accumulation of
fluid in the pleural sacs in instances in which dyspnoea is serious,
and life may be prolonged by repeatedly practising this operation.
Reflex laryngeal irritation, or paroxysmal dyspnoea with stridulous
breathing, requires the use of inhalations or atomization of
antispasmodics, and among the most useful of these are ether and
chloroform. This group of neural symptoms can sometimes be markedly
palliated by hypodermic use of morphia with atropia. But too often the
symptoms are caused by actual pressure, and not by nerve-irritation,
and this mode of treatment is futile, and therefore these measures
should be employed with caution.
{881} Sleeplessness, cough, bronchial or other pulmonary complications,
must be managed upon general principles. The local pains may be met by
local treatment, such as mustard sinapisms or soothing lotions; even
blisters may secure temporary relief. The digestive system should be
carefully studied, and assimilable and appropriate food should be
selected. In lymphadenoma combinations of iodine with arsenic, as in
Donovan's solution, may be tried, but, unfortunately, the utmost aid
from present resources consists in a palliative and expectant policy.
{882}
DISEASES OF THE BLOOD AND BLOOD-GLANDULAR SYSTEM.
BY WILLIAM OSLER, M.D.
INTRODUCTION.
The blood is a fluid tissue composed of cells floating in an albuminous
plasma, and it differs from other tissues not less in the arrangement
of its elements than in the activity of the changes which go on in it.
It is the mart into which is poured from the alimentary canal the
commodities needed in nutrition, and the elements of the body select
from it the various materials which they require, giving in exchange
those chemical combinations which result from the metabolism of the
tissues. In spite of ceaseless changes, a uniformity of composition is
one of the most striking features of the blood in health. This is
maintained, as regards the constituents of the plasma, by the activity
of the organs which regulate income and expenditure--the alimentary
canal and liver on the one hand, and the kidneys, lungs, and skin on
the other; while histological uniformity is maintained by the adenoid
or cytogenous tissue throughout the body, the function of which is to
replace the wornout blood-corpuscles.
The corpuscles form rather less than one-half by weight of the blood.
The plasma contains about 90 per cent. of water, which holds in
solution proteids in the form of serum, albumen, and the fibrin-forming
factors; sugar in traces; creatin, hypoxanthin, and urea; various fatty
bodies in small amount; salts, chiefly sodium; and gases. The
corpuscles (red) consist of hæmoglobin (90 per cent.), proteid bodies,
and traces of lecithin and cholesterin.
So far as we know at present of the function of these two portions of
the blood, the plasma ministers to the general nutrition of the
tissues, while the corpuscles (red) are chiefly concerned with
respiratory processes, acting as the carriers of oxygen and carbonic
oxide.
We shall first give a brief account of the histological characters of
the blood, and of the relation of the groups of adenoid or cytogenous
tissue to the corpuscles.