Sarcoma of the Pons, and Glio-Sarcoma ofthe Cerebellnm.

Read before the January 1993, Meeting of the Chicago Academy ofMedicine.

BY

LUDWIG HEKTOEN, M.D.,Oh’ CHICAGO.

Reprinted from the “ Journalof the American Medical Association,’February 11,1893.

CHICAGO:PUBLISHED' AT THE OFFICE OF THE ASSOCIATION.

1893.

SARCOMA OF THE PONS AND GLIO-SARCOMAOF THE CEREBELLUM.

LUDWIG HEKTOEN, M.D.,OF CHICAGO.

The following two instances of tumor of the brainare reported in order to increase the number of suchcases recorded and described in medical literature.

Case 1. Sarcoma of the Pons.—This case occurred in theCook County Hospital, where Dr. E. T. Edgerly ascertainedand wrote down the clinical history as well as the,results ofthe physical examination ; the writer made the post-mortemexamination and examined the tumor microscopically.

Man, single, baker, German by birth, 37 years old, wasadmitted to the Cook County Hospital of Chicago, April 5,1890. He was his parents’ only child; the parents wereboth dead, the cause of death being unknown. He deniedhaving had any venereal disease, said that he drank beer inmoderate quantities, and that he had never been ill beforehis recent sickness, which began with headache threemonths ago, since which time he had been unable to workon account of the pain, which was of a dull persistent char-acter, with nocturnal exacerbations, principally frontal, butoften extending over the entire top of the head. Two daysbefore admission he fell on the sidewalk in a fit of faintness,but did not lose consciousness entirely ; he had been trou-bled with dyspepsia; the bowel and urinary functions hadbeen abjectively normal. The patient on admision is men-tally dull and a clear cut history cannot be obtained.

Present Condition.—Patient is well nourished; he lies inbed groaning and complaining loudly of pain in the head,which he grasps with his hands; the right eye is tightlyclosed, the left eye is half open ; the pupil of the right eye issomewhat contracted, both pupils respond to light. Move-ment of the eyeballs seem to be limited, particularly as

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regards motion upwards and to the left. The tongue is nor-mal and does not deviate on protrusion ; the lines in theface seem better marked on the right side. Physical exam-ination of the chest and abdomen is negative; there is noankle clonus ; the patellar and cremasteric reflexes are nor-mal; muscle reflexes are universally present; rough testsindicate the tactile sensibility to be much impaired, thustwo points three inches apart cannot be distinguished onthe hands or the feet; when half an inch apart on thetongue the two points cannot be separated. Taclffi cere-berale is very distinct.

Pulse 64, respiration 16, temperature 97.6°.Ophthalmoscopic examination by Dr. Mary C. Fowler:

the discs cannot be outlined at all, yet the vessels are notlarge and do not pulsate. This condition is more markedin the left eye than in the right.

April 12. Patient suddenly became unconscious whileeating and unable to swallow the mouthful of food he hadtaken; the extremities became cold, profuse perspirationbroke out on the skin and the pupils dilated. Patient istaking iodide of potassium in increasing doses.

April 14. Lies in stupor; requires catheterization ; refusesfood. Right pupil dilated, does not respond to light. Supra-orbital pressure arouses patient sufficiently to answer ques-tions. The right side of the body seems to respond to stim-ulation more quickly than the left, and often he moves theright side in response to irritation of the opposite half.

At 8 p.m. Dr. Murphy removed a button of bone with thetrephine from the skull on the upper end of the rightRolandic Assure. The dura was dense. An aseptic aspi-rating needle was passed into the brain in all directionsfrom the trephine opening, but no abnormal fluids wereobtained nor resisting body encountered. Replacement ofbutton, suture. 12 p.m. Death.

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Section twenty-four hours post-mortem;The trunk cavities were empty. The heart was of normal

size, the semilunar valves competent to water; the mitraland tricuspid orifices admitted three and four finger tipsrespectively ; the endocardium was smooth and the muscu-lar tissue firm and light greyish-red in color. The fossaovalis presented many small perforations. The lungs con-tained much frothy and bloody fluid. The abdominal organspresented no special change, contained no tumors. In thescalp over the right parietal eminence was a curved incisionand beneath the flap thus outlined was a circular openingin the skull containing an accurately fittingbutton of bone.The dura was free from thickening, the Pacchionian granu-lations were exuberant. The pia was quite universallyadherent to the surface of both the vertex and the base ofthe brain. There were small clots of blood in the rightSylvian fissure at the base ; there was a small clot along thepost communicant artery, but the vessel itself was intact.In the region of the upper third ofjthe right pre- and post-central convolutions, beneath the trephine openings, weremany small punctures in the membrane and in the brainsubstance. There was some clear fluid in each lateral ven-tricle. The brain substance in the hemispheres was macro-scopically normal and free from any focal disease. On theinternal half of the ventricular aspect of each thalamuswere slate-colored, slightly raised areas, and on the leftthalamus was a gelatinous body as large as a flax-seed.

The choroid plexus and the pineal gland were thickenedand the peduncles of the pineal gland contained someslightly ’discolored areas. The slaty areas in the thalmiextended into the substance for about 1 mm. There wereno areas of softening in thalami or in the crura. The ponswas larger than normal, but the enlargement was symmet-rical, at the widest portion it measured 5 cm. across fromright to left. In the central portion of the pons, incliningperhaps to the right, was an oval, firm mass, 8.5 cm. in diam-eter and 5 cm. long, the long diameter lying in a dorso-ven-tral direction. The tumor was but loosely imbedded in thepons; having no organic connection with the pontine sub-stance, and on attempted manipulation it slipped outentirely. It weighed 17 grams; it was of firm consistency,greyish in color on the cut surface, with many small haem-orrhagic areas, old and young, scattered through its sub-stance. The fourth ventricle was empty ; the medulla andcerebellum were normal. The spinal cord was not exam-ined.

Microscopic examination of the tumor, after hardening

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in Mueller’s fluid, shows that it is made up of rather deli-cate, quite small round and spindle shaped cells, with dis-tinct deeply colored nuclei, densely packed together; thereare places consisting principally of round cells, and inother spots mostly spindle shaped cells are found; no dis-tinct stroma can be made out, perhaps on account of thedense crowding together of cells. There are a number ofirregular spaces without any plain wall separate and dis-tinct from the tumor structure; in some places the cellsimmediately surrounding these spaces are, however, muchlike normal connective tissue cells, often these spaces arequite empty; some contain red blood corpuscles only orcorpuscles mixed with tumor cells; there are also foundquite large areas consisting of blood infiltrating with thesubstance of the neoplasm ; in such foci the fibrinous mesh-work is usually plain. At the margin of one of these areasof infiltration is seen a distinct, thin-walled blood-vessel,filled with blood which in one place seems to be breakinginto the tumor. There are also found a few areas of amor-phous, yellowish material. The areas of discoloration ob-served with the naked eye upon the caudal surface of thethalami consist of masses of round cells, irregular in out-line and in size, some being large and some small, and a fewseem to be intravascular ; here the ceils are large and notso closely aggregated as in the pons.

Diagnosis.—Round and spindle-celled sarcoma of the pons ;secondary foci in the thalami.

Remarks.—It will be seen at once that the symp-toms presented by this patient are readily explainedby the nature and the position of the intrapontinetumor. The general symptoms such as headache,attacks of fainting and ophthalmoscopic retinalchanges, occur in tumors of the pons as well as inother parts of the brain. The headache was intense,of rather uncertain localization and well-nigh con-tinuous, In view of the fact that the pain in suchcases is situated in the dura mater, it might perhapsbe more reasonable to speak of the headache in thiscase not as a general but as a focal symptom, due tolocal compression or irritation of some part of thefifth nerve, near its origin, this being the nerve whichsupplies sensation to the dura.1 The varying inten-

i Wernicke, Lehrbnch der Gehirnkrankheiten, Berlin, 1883.

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sity of many of the symptoms was due in all prob-ability to the occurrence of haemorrhages into thetumor, which would cause sudden increase in theintracranial pressure. Thus, for instance, the at-tacks of fainting would result from increased pressureor sudden irritation of the nerve tracts passingthrough the pons due to the haemorrhagic extrava-sations into the neoplasm. The examination afterdeath showed the presence of old and recent, largeand small, foci of haemorrhage into the tumor. Infact the variations in all the symptoms was undoubt-edly caused either by varying intracranial pressureor by varying direct compression of important struc-tures in the vicinity of the tumor dependent uponthe repeated congestions and haemorrhages Therather slight evidences of motor and sensory dis-turbances during the time the patient was observedin the hospital are attributable to the nature andthe position of the growth; it was circumscribed, notin any way infiltrating but rather displacing, andconsequently its effects upon the surrounding struc-tures would be due entirely to pressure. The ratherindefinite ocular paralysis or paresis and the equallyimperfect left hemiplegia was due to the graduallyincreasing pressure of the tumor upon the tissuesaround it. The paresis of left side of the bodymight, for instance, be caused by pressure on partof the tumor in the right upper third of the ponsbefore the decussation of the pyramidal tracts or inthe lower left third after decussation. The symp-toms were not distinct enough to decide this from aclinical standpoint, and the position of the tumor inthe central part of the pons gives no clue as to whichpart of the tracts would be most compressed unless,indeed, it be those in the right half.

It will be observed from the temperature chartthat the occurrence of haemorrhage into the substance,of the tumor was not followed by fall of temperature.

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Gerhard observed that haemorrhage into gliomas didnot, like genuine cerebral haemorrhage, cause persis-tent lowering of temperature, and proposed to utilizethis observation in the diagnosis of this form ofbrain tumor; vascular sarcoma consequently appearsto agree with glioma with respect to the point men-tioned. The sudden death may have been due to theoperation of trephining; it might also have resultedfrom lesions of the vagus centres due to the tumors.

The varieties of tumors occurring in the pons arepractically two, glioma and sarcoma; a third isformed by a combination of these two into glio-sarcoma. Gummata, tubercular foci, parasitic cystsgive rise to symptoms identical with those producedby true tumors. The glioma is more frequently metwith than sarcoma. Starr collected thirty-eightcases of tumors of the pons in children and youth ;of these nineteen were tubercular, ten gliomatous,five sarcomatous, two glio-sarcomatous, one cysticand one unknown. Glioma of the pons and thepons medulla transition usually take the form knownas gliomatous hypertrophy; a name which Hun3 sayswas first proposed by Kiimmel 4 because the growth isnot circumscribed butappears in the form of a diffusehyperplasia of the parts affected, resulting in greatincrease in size and but little distortion of normalconfiguration. This name, gliomatous hypertrophy,has been used by quite a number of writers report-ing cases, and among them may be mentioned Hun3and Spitzka. 5

Gee and Percy Kidd6 termed the same conditiongelatiniform enlargement of the pons in their de-scription of cases in the St. Bartholomew HospitalReports. Glioma is nearly always an infiltrating

2 M. Allen Starr, Medical News, July 12,1889.3 Hun, Medical News, October 1,1887.* Kiimmel, Zeitschrift fur Klin. Med., B. 2, Aeft. 2, Berlin, 1888.5 Spitzka, N. Y. Medical Journal,Marcii 27,1886.6 Angel Money, London Lancet, May 12,1888.

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tumor. Histologically it consists of branched neu-roglia cells many of which may be multinucleated;it takes its origin from the neuroglia and grows byproliferation of its cells; as it grows it infiltratesthe brain substance, and nerve fibres and ganglia,cells swell up and disintegrate as they become en-croached upon, though both these elements may per-sist surprisingly long.7 It is consequently a reallydestructive growth in many instances.

Gliomata are frequently telangiectic. Klebs 8 main-tains that the nerve fibres and ganglion cells takepart in the proliferation, and he consequently callsthe tumor neuroglioma. Spitzka9 describes an excel-lent instance of neurogliomatous hypertrophy ofthe pons medulla transition which in parts showedactual destruction of nuclei and nerve fibres; com-pact nerve strands seemed to present resistance toits destructive efforts, while ganglionic and capillarydistricts favored its extension.

Sarcoma of the pons may occur as an independentgrowth, unattended by neuroglia proliferation, andit is thought to be developed from the sheaths, or theactual walls, of blood-vessels. As in the presentinstance they usually present rounded, extremelyloosely connected, sharply defined masses of varioussizes; sometimes they possess a distinct vascularmembrane. The histological structure does not inany way differ from that of sarcoma elsewhere, andwhen haemorrhage foci are present in great numberthe vessel wall will usually be found to consist ofembryonal connective tissue. When some glioma-tous structure is present, then the tumor is usuallyspoken of as a glio-sarcoma, and it may manifestthe infiltration tendency usually observed in the gli-oma. Thus Simon 10 reported a case of glio-sarcoma

i Zeigler. Text-hook, 1887.- s Klehs, Vierteljahreschrift fur prakt. Heilkunde, 125, 133.

9 Spitzka, Loc. Cit.

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of the pons which consisted of small round cells,neuroglia cells, and in places large cells, with a man-ifest tendency to infiltrate surrounding structures.Most authors expressly state that sarcoma of thepons is not infiltrating as a general rule (Zeigler,Wernicke, Eichhorst); Henoch, 11 however, details anintra-pontine sarcoma which presented no sharpboundary, but which seemed to extend diffusely intothe substance of the pons from a number of minutefoci secondary to the principal mass.

While glioma and neuro-glioma of the pons, as wellas of other parts of the brain, usually appear single,sarcoma tends to give rise to vascular metastasis,which is also shown in the present instance in thesecondary foci in the thalami, some of which arequite distinctly intra-vascular.

Case 2. Glio-sarcoma of the Cerebellum.—The followingcase was in the Cook County Hospital in a homoeopathicward in the spring of 1890, but there is no record extant ofthe case from that time, and consequently I can only givesuch clinical facts as have been gathered from family andfriends after the patient’s death.

Man, age 45; was in good health up to May, 1885, weigh-ing 200 pounds, and of imposing physique. At about thattime he was engaged in raising an old building, and his soncrawled under the building; fearing some injury, heattempted to drag him out, and during his efforts, thebuilding fell, striking him on the back of the head and theneck, and driving his face into the sand. Owing to hisgreat strength, he was able to extract himself, producingin so doing lacerated scalp wounds. He recovered from hiswounds, and did not complain of any pain until the follow-ing winter, when he suffered for several months with severeheadache, which was diagnosed by his attending physicianas neuralgia. The summer following he did his usual work,but he never seemed quite well. The second winter thepain in the head returned, was principally occipital, andthis time it was worse than before; he also had vertigo andnausea. The remissions in the headache grew shorter andthe attacks longer, and from this time (1888) until his death

io Simon, Zur Casuistik,der Tmnoren der Pons, 1887.n Henoch, CharitAAnnalen, V, 1880, p. 461.

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(July 5, 1890) the pain in the head steadily increased, untilhe at last died from exhaustion. For a short time beforedeath he was totally blind, lie was treated from time totime in hospitals, by ordinary practitioners and by authori-tative specialists, under various diagnoses, a favorite onebeing spinal meningitis; he was blistered and burnt, andreceived all the anodynes in the pharmacopoeia. The abovefacts were obtained principally from Dr. Charles Caldwell,of Chicago, to whom I am greatly indebted for the requestto make the post-mortem examination. The doctor adds : Iknew him personally for many years, but I never had himunder my professional care. I have never, in a practice oftwenty-five years’duration, seen any one suffer such terrificpain as did this man.

The post-mortem examination was made twenty-sevenhours after death. The neck and head only were examined.

Examination of the cervical spine, of the cervical portionof the spinal cord and of the organs of the neck was nega-tive. The calvarium was free from any change; the durawas very tense, and could not be pinched up into a foldanteriorly near the crista galli; there was no fluid in thesubdural space; the brain filled the cavity completely, theconvolutions were much flattened, the sulci apparentlyobliterated. The pia showed uniform injection of its ves-sels, large and small, and at a little distance it presented arosy or pink color. It was not adherent to the dura, but itpeeled from the cerebral surface with difficulty. On remov-ing the brain, clear fluid spouted like a fountain streamfrom the vicinity of the base beneath the hypophysis. In-spection of the brain shows uniform pinkish color, due to-vascular injection ; this is the case both in the dorsal andthe ventral portions. The brain substance is very firm ; theencephalic cavities contain much clear, almost colorlessfluid, and they appear enlarged, dilated; the velum overthe third ventricle is distended, and appears like a greyishband; the fourth ventricle is empty; the oblongata seems;compressed. Underneath the tentorium is seen a hard,,nodular projection from the left cerebellar hemisphere ; itis covered by a thin stratum of cerebellar substance and bythickened pia, in which run large, tortuous vessels. To bemore accurate, it is found that the postero-superior lobe ofthe left hemisphere is occupied by an oval tumor, present-ing a projection beneath the tentorium. The long or dorso-ventral diameter of this tumor is 2, the short or verted, l% yand the transverse, 1% inches; it encroaches some on the-vermes, i. e., beyond the median line ; it is everywhere sur-rounded by cerebellar substance which, dorsally, is very

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thin, and in the thickened pia are numerous large, tortuousvessels, especially around periphery of the tumor projec-tion. The cut surfaces of the tumor show areas of haemor-rhage of irregular size and age, large and small districts ofhomogeneous, greyish-yellow,gelatinous substance in abedof firm, whitish-grey tissue. The tumor seems simply im-bedded in the cerebellum; it has no distinct capsule, andyet it has no distinct connection with the cerebellum exceptthe vessels seem to pass into it on all sides —especially,however, from above. It cannot be said that there is muchsoftening around the tumor.

Microscopical examination shows this tumor to consist ofvarious kinds of tissue. In places, especially at the periphe-ral portions, it is composed of branching cells like the neu-roglia cells; the fibrils are delicate and the cells small,with large nuclei; in an occasional cell more than onenucleus is seen. In the central portions there are areas ofpure round cells, without projections, and in every way likethe cells in round-celled sarcomas; then, again, there aremore or less homogeneous areas without any definite cellu-lar structure; there are also foci, composed of granularbrownish pigment and blood corpuscles, as well as areas ofrecent haemorrhagic infiltration. The vessels are numerousand large, comparatively speaking, with many sacculardilatations, and the vessel walls are all very thin, and, inplaces, are simply canals, containing blood, without distinctwalls; in two places blood is seen in the act of passing intothe tumor through the embryonal wall.

Diagnosis.—Telangiectatic glio-sarcoma.

Remarks.—In this case one interesting feature isthe apparent casual connection between the injuryand the development of the tumor. Quite a fewcases presenting just as distinct, apparent traumaticorigin, if not more so, are found in the literature.Eichhorst records two cases in which a sarcoma ofthe cerebellum and a glioma of the pons followedInjuries to the head in two boys, resulting in deathone and one-half and eight months respectively afterthe trauma. Curschman, cited by the same author,observed a sarcoma develop at the site of an injury•causing a fracture of the skull. Starr remarks thatthere seems to be no doubt that blows and falls onthe head may cause intra-cranial tumors, and in fact

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many writers attribute the frequent falls of childrenon the back of the head as explaining in part thegreater numbers of tumors in the posterior cranialfossae in early life.

The fact that the tumor has developed immediatelyafter and is apparently the result of injury is thoughtby Bramwell to suggest that it is either a syphiliticgrowth, a sarcoma, a glioma, or a scrofulous tumor.

The length of time intervening between the receiptof the trauma and the appearance of symptomsin the case described was about six or eight months.During this time the tumor may have been latent,the general symptoms being due to the graduallyincreasing intra-cranial pressure and the encroach-ment of the growth upon the vermes, becauseit is only when this portion becomes affected thatvertigo, nausea and vomiting and other symptoms,more or less characteristic of cellular growths,appear.

Death in this case undoubtedly occurred in coma,in consequence of the continually increasing intra-cranial pressure.

Anatomically the tumor appears to be a quitetypical example of glio-sarcoma with large and em-bryonal blood-vessels, and consequently presentinga favorable condition for the occurrence of haemor-rhages, congestions and areas of softening, with thesudden exacerbation of symptoms usually observedon this account in similar growths; exacerbationswhich by some authors are utilized in the differen-tial diagnosis between the various kinds of tumors,because they can only occur in vascular or telangi-ectatic growths, such as sarcoma and especially thesoft varieties of glioma. This tumor illustrates wellalso the production of internal acquired hydroceph-alus due to pressure at the base of the brain uponthe veins of Galen, and perhaps upon the passagebetween the third and fourth ventricles.

119 Loomis St.

Sarcoma of the pons, and glio-sarcoma of the cerebellum /FRONTCover page

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