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Reprinted from The American Journal of Obstetrics and Diseases OF AVOMKN AND CHILDREN, Vul XVL, Nos. VIII. & IX., 1883. NEW YORK : \VM WOOD & CO., PUBLISHERS, ,50 & 58 LAFAYETTE PLACE. Looo. CANCEl T. JOHNI Consultinj Reprinted , OF W( WM WO PRIMARY CANCER [ENCEPHALOIDJ OF THE KIDNEY DURING CPIILDHOOD. BY T. JOHNSON ALLOWAY, M.D., L.R.C.S. and P., Edinburgh, Consulting Physician Montreal Dispensary; Attending Piiysician Department for Diseases of Women, West End Free Dispensary, Montreal. Reprinted from The American Journal of Obstetrics and Diseases OF Women and Children, Vol. XVI., Nos. VIII. & IX., 1883. NEW YORK : WM WOOD & CO., PUBLISHERS, 56 & 58 LAFAYETTE PLACE. ,1883, PRIMARY CANCER (Encephaloid) OF THE KIDNEY DURING CHILDHOOD. The following case of cancer of the right kidney has been of some interest to me from its extreme rarity in childhood, and it may also be interesting, on that account, to others. Ellen W., aged five years, is of English parentage. Both father and mother are vigorous and have always enjoyed good health. Upon careful inqun-y, however, it appears that a paternal aunt of the patient, when about six years of age, caused con- siderable interest to be manifested in her by several prominent London physicians concerning the growth of a tumor in her right side, and that, although she had visited several iiospitals in tliat great metropolis, the parents could not obtain a definite diag- nostic opinion concerning its precise nature. The tumor, how- ever, is said to have disappeared after lasting about two years, although it had in thai- period of time obtained such a size as to cause quite a deformity, which was apparent in a photograph submitted to me. Further than this, there is a record of an ex- cellent family history on both mother's and father's side. My patient was the second child in a family of three, and has always been in good health, with the exoeption of a rather severe attack of entero-colitis in the hot summer of 1879, and, about a year ago, of rtu attack of remittent fever, which lasted about three weeks, and was accofupaiiied ivith diarrhea. She never had scarlatina. The first manifestation of her recent illness occurred last De- cember (1882), in the appearance of " a lump on her right side," as her father expressed it. He stated that the child was not sick, but that he felt anxious about this lump, as she had fallen down stairs, three months previous, and had struck the very side in question against an upright post during her descent. She com- 4 Alloway : Primavt/ Cancer of the Kidney. l»liiinu(l iit the time of tlic iicciileut, of nothing njorc tlian uhiUlrcn uauiillv do undor such circunistiuices, and it was only recalled to mind "hy the uppearanco of the pivsont tumor in the right side. I'livsiciil examination revealed an ovoid tumor extending from the lower margin of the liver to within an inch of the crest of ilium. This tumor occupied such a position that, if a lateral- median section of the body were made, the line of section would l)as8 half an inch liehind ihe ))osterior margin of the tumor. It was very hard and movable both by the hand, and by the action of the diaphragm during respiration. Gentle manipulation caused no pain whatever. At this time, the tumor could be grasped in its middle with fingers and thumb, and lifted and moved about quite freely without pain or incon- venience to the little patient. Percussion gave a continuous dull note from the upper margin of the liver to the lower point of tumor, and from this it was concluded that close adhesions existed be- tween it and the lower margin of liver. The patient was ap- parently in good health ; the excretory organs performed their respective functions in a normal way. The urine was carefully examined with negative results. Her appetite was good. She Alloway: Pn'mirij Cancer of the Kidneij. B!o|»t well. Her skin was ck'iir, iind hIic liiul tlio appoaruncc of :i plump woll-noiirislit'd cliild. Slio would play and romp altout as usual vvitli oilier children. Towards oveninji;, she would com- plain slightly to her inollior of cramps or " pains in her hcUy." Jan. 11th. 8hw patient again. Tumor increasing in size, chiefly towards the middle line of alxlomen, and downwards towunl ilium. I'atient not (piite so lively. She would cojnplain suddenly of pain at play, making her peevish. Ap|)etite still good, nutrition good, bowels regular, and urine normal. Jan. 'Z'ZA, Found a distinct systolic; bruit, heard loudest over maximum elevation of tumor, gradually fading in intensity poste- riorly toward the spine, and anteriorly toward the epigastrium. In both of these situations, it was j)lainly audible, but had a distiinb character, while over point of greatest elevation of growth it ap- ])eared as if quite near the surface, 'i'hc heart was now slightly displaced ujiward and to the left. Pulse increased slightly iu rapidity. Temperature nornnd. Urine still normal. Jan. 26th. Dr. Kaddicli saw the patient with me to-day. 'i'lio growth was much increased in size since last visit, and a decided feeling of fluctuation was now ((uite evident throughout the entire growth. At this time, there was a general enlargement of the whole abdomen becoming apparent. The arterial bruit so distinctly heard the week previous is no less distinct, and is now distant in character. The patii-nt is beginning to show signs of emaciation. There is now noticed a prominence or bulging on the left side of the vertebral column at about the level of the lirst, second, and third b.nnbar vertebrre. This was thought to be due to involvement of other postiieritoneal glands of that side. Feb. 1st. Dr. I{. P. Howard saw the case with me, and after a careful examination agreed with us in inclining towards the diag- nosis of encephaloid cancer, as far as it was possible to judge at the present existing stage of the disease. The tumor now seemed to increase in almost every direction, and es[)ecially so toward the epigastrium, where a decided ])romincnce with skin tension was apparent. The whole abdominal contents seemed as if in one great mass and moved together. There was no tympanitic note on percussion to bo obtained, except along the anterior axillary line of leftside; all to the right of this was of wooden dul- ness. There is very little pain or annoyance caused by examina- tions. She can stand up with assistance, ])ut cannot walk any longer with comfort. She has to be carried about. Heart is con- siderably displaced upwards, and to the left side, pulse very rai)id. Prominence of left side behind is increasing. Bowels move once a day, and urine normal. The bruit in tumor can no longer be heard at any point. February 5tli. Respiration, 34; pulse, l"-iO; temperature nor- mal. Measurements of abdomen were as follows: At nipple 3o^ inches. Xi))hoid cartilage 22 " Eighth cartilage 23^ " Maximum elevation 24 " 6 Alloway : Prlimo'ij Cancel' of the Kiiil)l)le 21 J iiiclios. Xiplioid cartilage 'Z'.\ " Eighth rib 24 " Maximum elevation 2(il '' Unil)ili(:uH 2(; '• Apex heart-beat one incii outside left nipple and oiu'-f|iuirtpr inch below level of it. Kespii'ation auilible behind no lower thiiii oiu'-(pi;uier of an inch below iiifei'ior angle of scapula. Patient becoming very restless at night, and complains of sore- ness in front when lifted. Pulse rapid; tcmperatuie nornnil. Bowels very much constipated, moving only once every four or live days. No jaunilice. March 5th. J'atient unable tol eave cot. She is much emaci- ated. J'ulse lo-lr. Diarrhea set in last night. Urinates very fre(|uently, passing but a small (|uantity at a lime. Apex heart- beat now one and a half inches outside left nipple, and on a level with it. She has not vomited since beginning of December. There is no edema of extremities or other parts of body, and has never had any during illness. lias never exhibited uremic symptoms. \[arch 12th. Fearfidly emaciated. Can only rest on back, Avith both artns extended backwards over her head, her hands grasping the iron bars at head of the bed. In this position, she seems to obtain the largest jiossible abdominal and thoracic area by protlucing traction upon the chest-walls frotn without. Under these circumstances, the heart, and lungs have more freedom of action, and the patient consequently feels more comfort than in any other ])osition No further (dumge took i)lace until the Dith, Avlien death occurred from asthenia. Antopuji. — Dr. Osier's Keport: The body Avas greatly emaci- ated; abdomen distended; superficial veins not much dilated. When opened, a large tumor was seen tilling the greater part of the abdominal cavity, aiul closely attached to the right side. The omentuu was adherent, the cecum attached to the lower end, and the ascending colon |)assed along the left border. The pylorus lay upon the upper end, and the duodenum was flattened u])on the left side of the mass. The tumor was retroperitoneal, and peeled out easily, bringing with it aorta and inferior cava, which were deeply imbedded in the hiiuler ])art. The mass had a rounded outline, broad above, with a concave left border, which presented several very soft lobulated i)ortions overhanging the groove in which the cecum and colon lay. The right border was more solid, and at the lower part showed a small remnant of the kidney surface. The growth has perforated the capsule, and pro- Alloway: Primary Cancer of tJic Kidney. 7 tectoil in largo, soft muHHOs, some of which wore hemorrliagic. The lust inch of tho iibdomiiiiil iiortii wiis surrou.idwl hy tho tumor, tlio loft iliao was pervioiis, tiio riglit wan lillod with tiiromhi, and tho wall oaten away. Tho inferior cava eould be traced in the inasH for about an iiicli and a half, and just above the bifurcation was oblileratcul, tlie walls in close contact. Soction of tho nmss showed it to bo niado of a soft cerobri- fortn tissue intcrs|)orHeil with cxtravasated blood. At tho right border liieru was a trace of kidney substanco in tho form of a thin shell. The ureter was occluded. The pelvic and renal vessels wore infiltrated with the neoplasm. On niicros(;o|)ie examination, the soft, grayish-whito substance was found to be composed of small round cells with but little stroma. Towards the right border of the nmss where it was firmer, the soft cerebriform substance was inclosed in denser strands. liemarJcs. In consifloring lomo of tho more interesting details in con- nection with the above case, I think it wonld bo m^oU to dis- cuss them, as points of interest, in a short review of the litera- ture of the subject as recorded up to the present time. • Nothing of any value was written upon cancer of the kidney, with the excej)tion of G. Konig's " Treatise upon Diseases of the Kidney " in 1826, and that of Wilson, of London, in 1817, until after tjie year 1 830. Cruveilhior was probably the tirst writer of note, closely followed by Rayer, who put tho disease on a sound foundation. After this came the classic works of Walshe, and then of Lebert, who distinguislied so clearly between primary and secondary forms of cancer of the kidney. Ebstein and Roberts are also well-known authors on this subject. Rowe, of Cincinnati, records a very interesting case in the American Journal ok Oustktrios, April, 1881. Of the different species of cancer found in the human body, encephaloid (fungus homatodes) is the one almost invariably •found in the kidney. Its chief physical characters as a neo- plasm consists in its soft, pulpy, vascular condition ; it is fre- quently the site of extensive hemorrhages, cavities are formed ■ in the mass, containing large quantities of blood mixed with cancerous detritus which flows freely from the cavities when opened or broken into during the post-mortem examination. The whole organ may become infiltrated uniformly, and when it does so, the enlargement is regular. But we also have the •formation of nodules of disease growing from a particular part 8 Alloway : Primary Cancer of the Kidney. or end of the kidney, and encroacliing upon the ground of a neighboring organ, which gives on palpation tlie idea of an irregular shaped tumor, and adds very materially to the diffi- culty of diagnosis. This disease always begins in the cortical substance, and gradually inv^olves the pyramids. The whole growth is surrounded by and contained within a strong fibrous membraL In about sixty (60) per cent of all reported cases, there have been found secondary deposits elsewhere. The seats of these deposits have been the lymphatic glands in the hilus of the kidneys, the vertebral, and mesenteric glands. The liver and lungs were also affected, but not so often. In considering the etiology of the disease it is a remarkable faet that among traumatisms an injury, such as a blow or fall, is often the starting-point in the history. Chamel, in 1829, -•ers to a case originating from a blow, in which the anterior - 11 of the abdomen was destroyed by tlie disease. Bright gives the history of a young woman who, several months pre- vious to her death from cancer, had experienced a fall down- stairs, and dated her disease from that time. x\nother case, of a boy, who dated his suffering from a kick in the left side ; he afterwards died from medullary cancer of the left kidney. A very interesting case is recorded of a lady who died of car- cinoma of the kidney eighteen years after a severe fall on the stairs. Immediately after the accident, she had a severe attack of hematuria, and in the course of six months a tumor became evident as a swelling under the margin of the right ribs. In my own oase was experienced a severe fall down-stairs, three months before the first appearance of the growth, but there was no hematuria or other evidence of internal injury. Trau- matism may play a certain role as an exciting cause in theso cases ; yet, why should it not be constant, and do so in all cases ; instead of which we have such injury followed in one case by nephritis, in another by perinephritis, and another by malignant disease. These are pure clinical facts, inexplicable unless we fall back on the doctrines of individual predisposi- tion. The earlier writers looked upon cancer of the kidney during childhood as a curiosity from its rarity, but more extended re- search has taught us that it is found chiefly in the extremes of life — earlv childhood and old ajje — \yhilft the periods of life \ Alloway : Primary Cancer of the Kidney. 9 between these points enjoy a remarkable immunity from it. It is said that males are more prone than females — of twenty- four children fifteen were boys and nine girls, and the dis- parity is still greater with adults. This disproportion may be accounted for by tlie marked preference shown by cancer for the organs of generation in the female. In cancer of the kidney, tliere are two distinct symptoms almost always met with — an aldominal tumor and hematuria. Roberts asserts that in all fatal cases one or both of these symptoms are invariably present. Of sixty-four cases sixty- one had a large abdominal tumor, and tlie remaining three had hematuria ; and it is noteworthy that in all children who have died from this disease there was an enormous abdominal tumor present. These tumors, as a rule, represent the largest, as a variety, which are met witli in children. They begin in the loins between the ribs and the crest of the ilium, they increase Yi upwards and downwards, and to the front towards the navel. Percussion within this area elicits a dull note, provided there is not part of the intestine between tlie tumor and the abdominal wall. But, as a rule, this is the case ; and in the case of the right kidney being affected, the ascending colon and cecum are generally found on the outer side of the tumor, and as the growth enlarges, the ascending colon is pushed forward, and is found running obliquely across the growth from right to left. Here, of course, we would get a clear tympanitic note. In my case, the bowel ran along on the left side of the growth in its entire length, and at no place crossed it. There- fore, there was an important diagnostic sign wanting, though, towards the end, such a displacement of the bowel was, how- ever, considered possible, and would account for the uninter- rupted dull note obtained. In the case of the left kidney when it is affected, the same condition obtains. Here the descending colon, and often part of the small intestines, cross or lie right in .front of the tumor, separating it from the abdominal wall. It is quite true, however, that the intestines can cross the tumor and still elicit a dull note on percussion, from the tact of it being so compressed that it becomes a mere flattened band, and in some cases has been felt as such through the abdominal walls. These tumors are genernlly lirmly flxed by adhesion, and are not intinenced by the movement of the diapliragm. 10 Alloway : Primarij Cancer of the Kidney. In my case, however, the tnrnorwas remarkably movable, both by palpation and the action of the diaphragm during respira- tion, without causing the patient pain or inconvenience. This point is important, from the fact that there is a case reported in the London Lancet (March 18th, 1865), in which the dis- eased kidney was so movable or wandering that it was taken for an ovarian tumor. The usual elasticity of these growths on palpation, amounting sometimes to a sense of fluctuation, carries with it a deception for which we cannot be too much " upon our guard " in making a diagnosis. It is probably con- veyed to the hand by the general softening process taking place in the growth as it enlarges, and by the formation of secondary cysts or cavities, chiefly on the surface of the principal growth, filled with a semi fluid cellular substance which is capable of conveying a wave-like impression on palpation. We come now to a very interesting, and I may say almost unique, symptom in the case ; a loud blowing systolic murrmir heard all over the tumor. This phenomenon first attracted my attention about the end of the third week of attendance, when the tumor was still hard. It continued to be audible, though gradually fading in intensity, until the tumor became soft and fluctuating, when it disappeared altogether. Its disappearance at this time was probably due to the non-transmission of sound through so extensive a semi-fluid mass, as there was certainly no evidence of the complete occlusion of the main vessel. This symptom we must regard as one of peculiar interest. To judge it from first impressions occurring to an observer not over cautious in giving an opinion, it would very probably be diagnosticated as an aneurism of one of the abdominal vessels, and I think the error would bear some fairly pardonable fea- tures in it. Ballard reports a case in Transactions of Patho- logical Society, 1859, in which the murmur was so loud that it was diagnosed as an aneurism of the renal artery. Jiristow also reports a case {Med. 2'imes and Gaz.^ 1854, ii., 395) in which a similar phenomenon was present. It may be remarked, however, that in each of the above instances there was also a distinct pulsation, which could not be obtained in this case. The direct cause of this murmur is ditticult to decide upon. It may be duo to direct pressure upon the aorta, or from the blood current acting upon a partially occluding thrombus situated on ALLOWA.Y : Primary Cancer of the Kidney. 1 1 the wall of the vessel. If these views are correct one would expect to have this symptom more often recorded than it has heen, and probably it would have been were it more carefully looked for at the beginning of tiie disease. "We now come to the consideration of the other distinctive symptom, hematuria. It will be remembered that, although the most careful investigation was made in regard to this symptom, it never occurred from first to last. This is important from the fact that the patient's illness dated from a severe fall with di- rect injury to the right loin; and that in about fifty per cent of all cases reported, hematuria was noticed at some period of the illness. In the cases which received direct violence to the part, the hemorrhage was generally noticed at the time of the accident, and lasted in some cases but a short while, and not reappearing before death. Then again it may be intermittent, appearing for a few days, disappearing, and then in a few days reappearing again. In other cases again, it does not appear until within a few weeks of death. When hematuria is noticed in the early stages, it is undoubtedly a sign of great value, and should give us food for thought; but it must not be forgotten that it is also present in other diseases of tiie urinary tract. It may also be present with a tumor in the left side and not be due to renal cancer, for profuse hematuria often occurs in leukfBinic patients. When hematuria is absent it is tiionght to be due to complete occlusion of the ureter of affected kid- neys, either by pressure or by extension of the disease into it. In Van Denburg's case (Am. Journal of Obst., October, 1881, 593), the urine was free from blood and other abnormal ingre- dients throughout the entire period of disease. This case of Van Denburg's, I may remark in passing, teaches us a very important lesson in another respect, namely, the necessity for giving very guarded opinions in respect to these abdominal tumors in children. His first consultant declared emphatically that it was a case of hepatitis, "just as sure as if he had the liver in his hand." Further council decided it was a case of nonmaligiiant tumor of a cystic nature. Paracentesis was per- foriuod, withdrawing six to eiglit ounces of albuminoid fluid. llooklets of the ecchinococcus could not he found. Again fresh council was obtained, and a larger needle used under chloro- form, which resulted in the diagnosis of " multiple cystic ta- 12 Alloway: Primary Cancer of the Kidney. raor.'' Tlie patient died, and at the autopsy it turned out that the liver, which was accused of being tlie cause of all the trouble in so many different ways, and by so many experienced ^\^s^no%t\^i\M^&,wa8 perfectly healthy, and that that little deceiv- ing neighbor of it, the kidney, was tlie organ at fault. The reaction of tiie urine in my case, it will be remembered,, was normal, as it generally is in these cases, except when ad- mixed with blood, and then, as a matter of course, we got albu- men. But albuminuria without hetnaturia is rare, pyuria and nephritis being excluded. Uremic symptoms are also exceed- ingly rare, for the obvious reason that, so long as the other kidney remains healthy, there can be no retention in the blood of the poisonous constituents of the urine, which it is the function of the kidneys to excrete. Tlie urine in this case was not exam- ined microscopically, it being well known that as an aid in the diagnosis of cancer it is of uncertain and questionable re- liability. Moore records a case {Medico- Ohirg. Trans., xxxv.),. in which he found roundish caudate cells, and in many other recorded instances of the discovery of cancer cells there is no proof of the supposed cancer elements being other than epithe- lial cells from the renal pelvis and ureters. Halle states that in a few instances of renal cancer, he has found deposits of uric acid as well upon discharged flocculi of cancer tissue, as up- on the waste substances obtained at the autopsy. But the dis- covery of certain cell-forms in the urine is of no value in the diagnosis of cancer. The symptom which can only be recorded as signitieant is tlie discovery of cancer particles with an al- veolar strxicture. Roberts says : " It must be remembered that cancer cells which would find their way into the urine must have come from broken down and degenerated parts of the growth, and to identify them in their changed condition is more than he has ever been able to accomplish." A symptom very variable in its degree of intensity is pain. In this case it never amounted to more than that which v/ould cause the patient to say " my belly is sore." In some cases, hovvever, it is described as being agonizing; while in others again it has been entirely wanting until near the end. Its ab- sence, however, is not of sufHcient significance to exclude the existence of renal cancer. Gastric symptoms are generally prominent. In this case Alloway: Primary Cancer of the Kidney. 13 there had been nausea and vomiting in the beginning of the disease, b\it after it ceased it did not return. In some cases tlie appetite is voracious, accompanied with increased thirst. In this case tliere was anorexia from tiie beginning to the end. Tiiere was no jaundice or tinting of the skin of any nature.* No anasarca which might be expected witii thrombosis of the vena cava; probably the compensatory circulation of there- turn blood tlirougli the extensive anastomosis formed by the superficial cutaneous veins prevented its occurrence. In re- marking upon the duration of the disease, it may be said that it is much shorter in children tlian in adults. But it is exceed- ingly difficult to be definite upon this point, as the beginning of the disease cannot be fixed with certainty. In very debili- tated children a few weeks may see the end, while in others, six to thirteen months. Ebstein says he has seen but one case of cancer of the kidney in a child which lasted as long as two jears. It is, however, erroneous, as maintained by Walshe and Lebert, that the renal cancer runs a more rapid course than other visceral cancers. The contrary, in fact, is established, namely, that as a rule death is longer delayed in renal cancer than in primary cancer of any otiier internal organ. The mean duration of cancer of the pylorus, of the liver, lung, or brain is under thirteen months, or thereabout. This tolerance on the part of the kidney may be accounted for by the dupli- cation of the organ ; when one kidney becomes disabled, the other takes on a compensatory hypertrophy, and does tlie work of its diseased fellow. The advantage of much room being af- forded the enlarging organ, in virtue of its situation, is also obvious. As the prognosis of tliis disease is decidedly bad, invariably fatal, the treatment consists in the employment of sucli thera. peutic measures as will tend to relieve distressing symptoms.' I may say that the removal of cancerous kidney is a procedure which I don't think is regarded as sound by scientific surgeons. Walcott extirpated a cancerous kidney which he had taken for a hepatic cyst; the growth vveighed two and a half pounds, and the patient survived the operation just two weeks.