CANCER AND LARDACEOUS DEGENERATION OF THE INTESTINES.
BY I. E. ATKINSON, M.D.
The term cancer of the intestines is used here in a clinical sense to designate new formations in the intestinal tract the tendency of which is to destroy life, and has no reference to the histological characters of the tumors, inasmuch as these are, during life, for the most part, concealed from the eye of the pathologist. It so happens, however, that in a histological as well as in a clinical sense the term is appropriately applied to all but a very few of the malignant new growths that develop in the parts under consideration, if we adopt, as seems proper, the opinion of most modern pathologists, that cancer or carcinoma should only include those tumors "consisting of cells of an epithelial type, without any intercellular substance, grouped together irregularly without the alveoli of a more or less dense fibroid stroma."
Carcinoma of the intestines appears either as cylindrical-cell cancer, as scirrhus, or as gelatinous or colloid cancer. Scirrhous cancer of the intestines may resemble in appearance and texture the ordinary medullary cancer, degrees of hardness or of softness depending upon the predominance of the stroma or of the cellular elements in the constitution of the tumor. Rarely, and in a purely clinical sense, cancer of the bowels may exist as a lympho-sarcoma in the small intestine, and then through progression from the glands of the mesentery or elsewhere. Primary intestinal sarcoma has, however, been observed. Similarly, melano-sarcoma has been detected in the intestine as secondary to this form of sarcoma, originating in the skin or in the eye.
Of the forms of carcinoma, cylinder-cell cancer is the most frequent. Carcinoma gelatinosum or colloid cancer is of great relative frequency, but it is altogether probable that here, as elsewhere, this represents a degenerative form of ordinary carcinoma. At all events, it is certain that it may be detected in many cases where the essential changes reveal the ordinary glandular or cylinder-cell variety. These forms of cancer may affect the bowel primarily or secondarily by extension from adjacent organs and textures, or by metastasis. Primary cancer occurs most frequently, metastatic cancer with great rarity. The relative frequency of the different forms of cancer is not definitely known. In the article on carcinoma in the Dictionnaire Encyclopédique des Sciences médicales (xii. pp. 576, 577) cancer of the bowels is said to constitute about 8 per centum of all carcinomatous new growths. Sibley1 found that primary carcinoma occurred in the alimentary canal (exclusive of the mouth, tongue, and the annexed organs) in 6 per centum of the cases collated by him. Tauchou's compilations of nine thousand fatal cases of carcinoma show that intestinal cancer was present in 4 per centum.2 These computations include cancer of the rectum. If statistics of cancer of the bowels exclusive of rectal cancer were available, they would show, doubtless, a much smaller proportion.
1 Medico-Chir. Transact., xlii., 1859.
2 Leube, Ziemssen's Cyclop., vii. p. 432.
Any portion of the intestinal tract is liable to be attacked by cancer, though undoubtedly some parts of it with much greater frequency than others. Köhler3 reported that in thirty-four cases the cancer was situated twenty-two times in the large intestine (the rectum excluded) and twelve times in the small intestine (nine times in the duodenum). It is not unlikely that in the cases of duodenal cancer the new growth extended from the pylorus. At all events, primary cancer is seated with far greater frequency in the large intestine, and, not including the rectum, usually in either the sigmoid flexure or the cæcum. Grisolle4 declares the large intestine to be four times more often affected with cancer than the small intestine; that the sigmoid flexure is attacked as often as all the rest of the colon taken together; and that the cæcum is still more often affected. Where the intestinal new growth is secondary to carcinoma elsewhere, it is usually so by extension from neighboring parts; thus, the ileum may become implicated by contact with uterine cancer, etc., and cancer of the stomach, liver, kidney, etc. may invade the colon.
3 Ibid., vii. p. 431.
4 Pathologie int., 1865, ii.
Cancer of the intestines usually begins after the middle period of life, and apparently irrespective of sex. Nevertheless, young persons are occasionally affected, and children sometimes develop malignant new growths of the bowels (usually sarcomatous), either primarily, which is rare, or secondarily, by extension from other parts. The influence of heredity seems not to be well established. There can be no doubt that chronic irritation may act as an exciting cause of cancer of the bowels, as it may in cancer of other parts. It has been impossible to recognize any specific influence from especial forms of irritation, and it is not likely that such exist. Indeed, the etiological relations of intestinal cancer remain exceedingly obscure.
SYMPTOMATOLOGY.—Up to a certain period of development cancer of the bowels will give no sign of its presence; indeed, cases have been observed where, death having occurred from other causes, the existence of the malady became apparent only at the necropsy. In all cases the symptoms are, at first, of an indefinite character and very inconstant. Vague abdominal pains are experienced; these gradually tend to become referable to a certain locality and to become associated with irregular action of the bowels. Constipation, alternating with short intervals of diarrhoea, supervenes, and a varying amount of meteorism is developed. These symptoms may be attended by the signs of failing nutrition. The body gradually shows the effects of chronic imperfect assimilation, and becomes emaciated. The complexion slowly assumes the peculiar hue of chloasma cachecticorum. Long before this occurs, however, the cancerous new formation usually becomes perceptible as a more or less distinct abdominal tumor, movable or fixed, as the part affected permits of free movement or is bound down to the neighboring parts either by normal attachments or by adhesions resulting from inflammatory processes or from the extension of the cancerous growth. When the tumor is movable, it is generally situated in the small intestine or transverse colon or sigmoid flexure, the other portions of the intestinal canal being comparatively fixed. It should be mentioned, however, that portions of the intestines normally freely movable may become adherent to contiguous parts, as the transverse colon, with the gall-bladder, liver, stomach, spleen, etc. etc.; the transverse colon and small intestine, drawn down by the weight of the new growth, with the pelvic organs, the bladder, uterus, uterine appendages, etc.; and that, finally, different portions of the bowels may become involved in one mass.
When the duodenum is the portion implicated the tumor may escape observation or may be indistinguishable from cancer of the pylorus. It occasionally happens that no tumor can be discovered until the malady is far advanced whatever part of the bowel is affected. In nearly all cases, however, before very long the tumor will be detected wherever situated, but it will often remain difficult, owing to its situation, to arrive at exact conclusions as to its precise character. Usually, it offers considerable resistance to the touch, but its features may readily be obscured by the fecal accumulation that forms above the constricted portion of the gut and by the gaseous distension of the bowel. This tumor will be slightly painful to pressure, and the patient will refer to it a spontaneous pain, usually of a dull aching, sometimes of a stabbing, character. Percussion yields a sound of muffled resonance, due to the tubular nature of the tumor. Cancerous neoplasms of the bowel, and of the duodenum especially, are apt to be associated with a distinct pulsation caused by the subjacent abdominal aorta. This may readily be distinguished from aneurismal pulsation by the absence of an expansile character, by the disappearance of the impulse that may sometimes be observed when the patient is made to kneel upon all fours, and by the occasional mobility of the cancerous tumor. By extension and by inflammatory infiltration the tumor frequently becomes converted into a conglomerate mass where all determination of locality becomes conjectural. The tumor is, with very rare exceptions, single.
The symptoms that accompany the development of these growths depend mostly upon their position in the alimentary tract. Pain alone seems independent of this, but is at best a most uncertain concomitant. When the duodenum is the part affected by extension from the pylorus, the symptoms are indistinguishable from ordinary pyloric cancer. Even primary cancer of this part may exactly simulate pyloric cancer. The localized pain and tumor, the vomiting after meals, the frequent presence of blood in the vomited matters, the progressive emaciation from starvation, the absence of abdominal distension (a result of the constriction of the gut at its upper extremity), the gastric dilatation,—all combine to make the diagnosis difficult.
Cancer of the duodenum in its descending part may be suspected when signs of hepatic and pancreatic obstructive difficulties point to implication of the ducts, through which are produced jaundice upon the one hand, and evidences of imperfect pancreatic digestion, in the presence of undigested fat in the stools, upon the other. In the lower portions of the intestines the cancer becomes more and more associated with meteorism and fecal accumulations. Constipation becomes steadily more obstinate, but there are occasional fluid evacuations containing blood, pus, and mucus, often stinking abominably. When the tumor is toward the end of the large intestine—in the sigmoid flexure, for example—fluid discharges occur with very great frequency at times; but these are scanty in amount and but slightly fecal in character. In these cases one does not usually observe the compressed, ribbon-like stools that are seen in rectal cancer. These symptoms may precede the appearance of the tumor, when the diagnosis will be less readily made. The constipation will at first be more amenable to the use of purgatives. (It is said to be due more to a loss of contractility of the bowel than to the narrowing of its lumen.) Gradually these will lose their efficacy, and finally complete obstruction of the lumen of the gut is effected; in which event the symptoms of ileus will develop, with cramps and vomiting, finally of a fecal character, and the fatal issue quickly follow. Not unfrequently peritonitis is developed, and may be of a chronic character or may destroy life within a day or two, or the patient may die from exhaustion before the obstruction becomes complete.
It may happen that the integument will become involved in the malignant process, or may become continuous with the tumor by adhesive inflammation. In such cases an opening may be formed by suppuration, or the lancet may secure the passage of feces through an artificial anus, and temporary respite be obtained. Sometimes a sudden disappearance of the symptoms of obstruction—a result due to the softening and breaking down of the cancerous mass, restoring temporarily the integrity of the intestinal tube—may give an unjustifiable hope to the patient; or the same effect may follow the establishment of a communication, by ulceration, between the bowel above the tumor and some portion nearer to the anal orifice. The progress of the new growth soon annuls the benefits thus gained.
Not uncommonly, particles of the cancerous mass may become detached, and, if diligently searched for, may be discovered in the feces. Microscopic examination may then definitely determine the nature of the disease. It has been claimed that colloid cancer may be diagnosticated in this manner even before the appearance of other symptoms.5 Death may be hastened by the occurrence of metastatic deposits in other and vital organs. Oedema of the lower extremities (of the left extremity in cancer of the sigmoid flexure) will often be observed as a result of the interference of the cancerous mass with the return of blood from the extremities by pressure upon the large veins. The combination of pain, tumor, constipation, tympanitis, progressive wasting, and the cachexia that sooner or later supervenes, stamps eventually most cases with unmistakable characters.
5 Charon and Ledegank, Journ. de Med.-Chir. et de Pharm., v. lxviii., 1879, p. 493.
The duration of intestinal cancer may extend from several months to one, rarely two, years, the latter age sometimes being attained by colloid cancer, the most chronic and least malignant form.
MORBID ANATOMY.—By far the most frequently encountered malignant new growth of the bowel is carcinoma, in one or another of its forms. The cylinder-cell epithelioma is probably the most common of these, and, as seen in the intestine, offers many naked-eye points of resemblance with ordinary encephaloid carcinoma. It is soft, filled with a milky juice, and may attain considerable size. The tumors appear as discoid prominences of varying size and number. Later, these may become fungoid and ulceration ensue. The growths early involve the whole intestinal wall, and by their increase tend to obstruct the passage of the intestinal contents. When ulcerated they present a nodular, uneven surface, situated upon a thickened base consisting of the infiltrated coats of the bowel. Villous prolongations (villous cancer; the undestroyed connective-tissue stroma) may project into the lumen of the bowel and give a peculiar tufted appearance to the part implicated. One or more points may be invaded by cancerous growth, and above each will be developed a dilatation of the gut (the result of distension) containing uncertain quantities of fecal matter, upon the removal of which the tumor will appear much smaller than it appeared during life.
Scirrhus usually implicates the gut in its entire circumference, so that a high degree of constriction may result from a small amount of cancerous infiltration. It begins as small nodules or plates upon the mucous membrane. As commonly observed, the lumen of the intestine is narrowed by an annular band of gristly hardness. All the coats of the bowel, with the peritoneum, become involved, and frequently the contiguous parts are included in the cancerous infiltration, forming an undefinable mass through which the contracted channel of the bowel may be traced, though often impervious to any but the smallest articles (a crow-quill, for example). The surface of the gut is generally ulcerated, irregular, and nodular. The walls of the ulcer are irregular and infiltrated. It will sometimes happen that the autopsy reveals permeability of the bowel where total obstruction prevailed during the latter days of life. This may be probably accounted for by the disappearance of the hyperæmia that doubtless existed during life and caused more or less turgidness of the growth. Sometimes the connective-tissue element is less predominant, and gives place to a more or less luxuriant cell-development; in a word, scirrhous carcinoma is replaced by soft or encephaloid cancer. This difference is simply one of degree, but is associated with greater rapidity and extent of growth. Ulceration is extensive, and one may here also often discover the villous, tufted appearance of villous cancer, caused by the fringe-like shreds of stroma entangling cellular elements not yet detached from the mass.
Colloid cancer, or carcinoma gelatinosum, may be associated with either of the above-described forms as a degenerative form, or may, apparently, develop as such from the beginning. It is a very frequent variety of the malady. In 27 cases of intestinal cancer, colloid cancer was present in 5, as reported by Lebert. It is most often observed in the sigmoid flexure and cæcum, as are the other forms of carcinoma. It is composed of a considerable mass extending around the bowel. Ulceration is less often found here than in the other forms, nor is there the same tendency to secondary infiltrations. By the unaided eye an alveolar structure may be detected, and when the mass is extensive a soft, jelly-like consistency is presented, together with "a bright, honey-yellow color." Small deposits of the colloid matter may be seen upon the surface. These have been described as resembling wheals of urticaria or herpetic or eczematous vesicles (Bristowe). The glairy fluid of colloid carcinoma oozes from the cut surface of the tumor, bathes it, and is to be found in the intestine.
These different forms of cancer sooner or later invade neighboring parts, as the peritoneum, mesenteric and retro-peritoneal glands, and adjacent organs. On the other hand, the intestines may become invaded by cancer of the peritoneum and other parts. It has even been observed, reversing the usual order of things, as secondary to cancer of the liver (Wilks and Moxon). Under these conditions the symptoms of intestinal cancer will have been associated with those due to the primary affection. Lympho-sarcoma will rarely be found as an extension from the lymphatic glands and involving the small intestine. Melanotic sarcoma may occur as metastatic from an original melano-sarcomatous tumor of the skin or eyeball.
DIAGNOSIS.—In its earlier stages it is impossible to recognize cancer of the intestines. After its symptoms have become established they may resemble those of several disorders. Cancer of the duodenum cannot be distinguished from that of the pylorus unless evidences of pancreatic or biliary disturbances indicate obstruction to the passage of the bile and pancreatic secretions. Previous to the appearance of a tumor one must often remain in doubt. The alternations of constipation and diarrhoea, the signs of partial obstruction, the localized pain usually present, the gradual wasting, will arouse suspicions of cancer, though chronic inflammatory affections of the bowels may induce symptoms not altogether unlike these. The presence of a tumor will supply the additional evidence necessary for a definite diagnosis. It will be necessary to exclude fecal enlargements of the bowels. The cancerous tumor will be somewhat painful, hard, nodulated. A tumor due to fecal accumulation may closely simulate it, and is, indeed, usually associated with it. By manipulation the fecal mass may be moulded, and even displaced, and by appropriate purgative treatment may be caused to entirely disappear. Foreign bodies, mesenteric tumors, and other abdominal enlargements may offer physical resemblances to intestinal cancer, but their symptomatology is usually so different that doubt may be easily dispelled. Syphilitic gummy infiltration, with resulting stricture, is more apt to occur in the rectum than in other parts of the alimentary tract.
The presence of fragments of the new growths may sometimes be detected in the stools, when microscopic examination will determine their nature. With cylinder-cell epithelioma and glandular cancer this is not common, but with colloid cancer much information may be gained by examining the evacuations. According to Charon and Ledegank,6 colloid cancer of the intestine may be detected before symptoms develop, by the presence of colloid matter in the feces. In the later stages, however, the gelatinous change of all the histological elements may occasion embarrassment, as at this stage the peculiarities of the cellular structure will have been destroyed.
6 Journ. de Med.-Chir. et de Pharm., lxviii., 1879.
PROGNOSIS.—Intestinal cancer always proves fatal. Death may result from the debility resulting from the cancerous cachexia or from intestinal occlusion or from peritonitis. The duration of the malady is usually not long. It runs its course in from several months to one, rarely to two, years.
TREATMENT.—Treatment must be directed to the alleviation of the distress caused by the disease. No curative treatment is known. When the cancer is situated in the colon, especially in the sigmoid flexure, the operation for artificial anus often affords great though temporary relief. The diet should consist of such articles in the digestion of which a large residue is not formed. Milk, eggs, soups, etc. should compose the principal articles of food. Mild laxatives will be required to secure the proper evacuation of the bowels, and to relieve pain and discomfort opium is invaluable and should be freely used. When obstruction is imminent nutrient enemata afford the most efficient means of administering nourishment.
Lardaceous Degeneration of the Intestines.
SYNONYMS.—Albuminoid degeneration, Waxy degeneration, Amyloid degeneration, etc.
Lardaceous degeneration of the intestines is an affection of quite frequent occurrence in those persons who are the subjects of a like change elsewhere; for although it has been asserted that it may be present as a primary affection, it almost always succeeds the same form of degeneration in other organs. Since, for the most part, it only makes itself manifest at an advanced stage of the disease, its importance is usually masked by the grave constitutional condition of the patient, whose vital forces are wellnigh exhausted by the already advanced degenerations present elsewhere. The extensive implication of other organs and tissues in the same degenerative process also creates great obscurity in the symptomatology of intestinal lardaceous disease, and is doubtless the cause of the existing dearth of definite knowledge upon the subject.
That the intestines are comparatively frequently involved in lardaceous disease is shown by dead-house statistics. Thus, Charlewood Turner7 reported from the London Hospital that in 58 cases of lardaceous disease the intestines were affected 10 times; and Goodhart8 in 150 consecutive necropsies of lardaceous disease at Guy's Hospital reported implication of the intestines 63 times.
7 Transactions Path. Soc. London, 1879, p. 517.
8 Ibid., p. 533.
Although the bowels do not become affected as early as several other parts, they will almost certainly become involved should the patient's life be prolonged; and in those cases where death is a direct result of the degeneration the intestines share with the kidneys the chief responsibility. It is not, however, until an advanced stage of lardaceous degeneration that its presence in the alimentary canal is revealed by symptoms; indeed, many cases do not, throughout life, betray evidences of the pronounced alterations that are to be discovered after death. Even in extreme cases there are no symptoms that would, even with probability, be referred to lardaceous disease of the bowels in the absence of the same degeneration in other organs and parts. There are, then, no specific symptoms following lardaceous degeneration of the bowels.
Where the normal functions of the intestines can no longer be properly performed in consequences of the changes that have taken place in them, there results a moderate diarrhoea. At first the number of movements may not be increased; the evacuated matters are fluid and of a greenish or pale color. Usually, little or no pain is experienced, though at times and in certain individuals this may be severe and colicky. The diarrhoea is not always steadily progressive, but may from time to time disappear. With the progress of the disease it may become more free and persistent, and in the later stages hemorrhage from the bowels may be superadded. This may vary in amount, and where, as is often the case, the stomach participates in the degeneration, hæmatemesis may also appear. These hemorrhages may be insignificant, or may at once assume alarming proportions, and even bring to an unexpected termination the life of the individual. Already, at the outset of the intestinal symptoms, the general health will have shown evidences of profound alteration, but upon the supervention of the diarrhoea more rapid progress will be observed, consequent upon the increased nutritive disturbance. The use of remedies in temporarily controlling this diarrhoea may prolong for months the life they are powerless to save.
So far as concerns the intestinal affection, there is no special tendency toward febrile excitement. It must not be forgotten, however, that acute inflammatory attacks of various tissues and organs frequently arise in the course of lardaceous disease.
Though there seems to be reason to believe that mild degrees of lardaceous degeneration may sometimes be cured, especially when dependent on syphilis, there is but little hope of arresting its progress at the late stage when the bowels become implicated. Indeed, when pronounced degeneration of the bowels takes place the disease is usually nearing the end of its course; for it is a well-settled fact that in this degeneration extensive implications of organs may occur without markedly reducing the patient's general condition, so long as the kidneys and intestines remain unaffected. The cause of death is usually to be traced to these organs. Dickinson9 found that in 35 cases where death was apparently due to renal lardaceous disorder, the immediate result was brought about by diarrhoea in 13 cases. Presumably, in a large proportion of these lardaceous disease of the bowel was present.
9 Diseases of Kidney, Part ii., 1877, p. 496.
The degeneration usually affects the lower portion of the small and the upper part of the large intestine. Occasionally it will be found to have invaded the whole alimentary tract. As in lardaceous degeneration generally, the process begins in the small arteries and capillaries and veins, affecting primarily the arterial and venous muscular coats—not, however, according to the latest authorities, the muscular fibres themselves, but their perimysium and the cement substance, the degeneration being one limited to the connective tissues.10 In the mildest cases only some of the small vessels of the mucous membrane are involved, and no naked-eye changes can be detected. In more advanced stages the mucous membrane is pale and shows evidence of catarrh. Thickening occurs, and as the process advances a peculiar appearance is revealed which has been compared to that of wet wash-leather (Wilks). The iodine test now gives the mahogany-colored reaction of lardaceous matter, with the tissues affected, or, if the methyl-aniline-violet test of Cornil be employed, the lardaceous material will display a red-violet color, while normal structures will be tinged blue-violet. It is said to be better to make the test near a Peyer's patch, since the latter is seldom affected by the degeneration, and brings out, by contrast, the surrounding lardaceous material.11 This distribution of the material cannot be considered as constant, however, since Hayem found the patches of Peyer most frequently affected.
10 Cohnheim, Allgem. Path., 1882, p. 667.
11 Wilks and Moxon, Path. Anat., p. 404; Kyber, Virchow's Archiv, Bd. 81, H. 1 and 2.
In more advanced stages the surface may become irregular from glandular enlargement, and ulceration may occur. Microscopic examination shows the lardaceous material in the vessels, and also in the stroma of the mucous membrane and villi.12 The epithelium is not involved. The degeneration, at first confined to the mucous membrane, extends to the submucous tissue, the proper muscular coat of the intestines being often implicated—so far, at least, as concerns its connective tissue. In the more severe cases Hayem found the agminated and solitary glands extensively involved. Fine branches from affected vessels penetrate to the interior of the glands. In such cases the mesenteric glands will be found implicated. The degeneration of the vessels running through the gland structure causes disappearance of this substance by fatty degeneration, and occasions a reticulated arrangement of the lardaceous material, and, secondarily, ulceration. In a similar manner ulcers may arise in any part of the affected tract. Finally, the lardaceous material may involve the whole thickness of the gut.
12 Eberth, Virchow's Archiv, 80, S. 138.
The diagnosis of lardaceous disease of the bowels can only be made with certainty in the presence of pronounced albuminoid disease of other parts in association with the symptoms of intestinal disorder. It possesses no characteristic symptoms.
Inasmuch as the disorder invades the bowels only at a late stage of its existence, the prognosis acquires additional gravity. It is probable that advanced albuminoid disease is never cured; so much the more hopeless is it when affecting this tract. If unchecked, the diarrhoea rapidly saps the powers of life; if temporarily alleviated, the approach of death is more gradual.
Whatever attempts are to be made to cure the disease, they must be through the general system, and are identical with those directed toward the cure of lardaceous disease generally. Treatment directed to the intestines must be palliative. The diarrhoea must be combated by appropriate diet and the administration of such remedies as protect the surface of the mucous membrane and control the intestinal movement. Bismuth subnitrate in large doses is therefore indicated. Various astringents may be employed, while the use of opium often secures most gratifying relief. It should be given in generous doses. Preparations of the crude drug seem to answer better than its salts. The necessity of keeping the gut free from undigestible matters that may irritate the already badly-damaged mucous membrane is apparent. Patients with this form of lardaceous degeneration usually show the cachexia resulting from profound modifications of nutrition, and their intestinal symptoms can only be regarded as links in a long pathological chain. Hemorrhage will call for remedies that under ordinary circumstances are employed to control bleeding from the bowels.