10
-579 INTRACRANIAL TUMOURS A Clinical Review By C. WORSTER-DROUGHT, M.A., M.D., F.R.C.P. Phlysician to the IVest Entd Hospital for Diseases of the Nervous System and to the Mctropolitant Hospital From the clinical standpoint intracranial tumours can be divided roughly into two main groups: (a) Those that affect a particular region only, e.g. pituitary region (adenoma, craniopharyngioma, chordoma), acoustic nerve (neurinoma), choroid plexus (papilloma), pineal region (pinealoma). (b) Those that may affect any region of the brain, e.g. various types of glioma, meningioma, angioma, metastatic tumours such as secondary carcinoma, etc. (a) The first, or regional, group may be con- sidered as follows: (i) Tumours of the pituitary region. Tumours of the pituitary body itself are usually adenomata, either chromophil, basophil, chromophobe, or mixed. The chromophil adenoma is almost invariably associated with some degree of acro- megaly if occurring after puberty and if before puberty, with giantism. Adenoma of the basophil cells, according to Cushing, gives rise to a syn- drome characterized by increasing obesity, especially of the face, neck and trunk, sexual dystrophy and, in women, hirsutes of the face and trunk. Chromophobe adenomata may be associated with some degree of hypopituitarism. Other tumours affecting the pituitary region are the group now known as ' craniopharyngio- mata' which comprises all squamous epithelial growths, both cysts and solid formations, arising from the remains of the hypophyseal duct. They include, therefore, the tumours previously known as Rathke's pouch tumours, suprasellar and sub- sellar pituitary cysts, adamantinomata, amelo- blastomata and hypophyseal duct tumours. Their origin is congenital and although symptoms may develop at any age from the first to the seventh decade, they most frequently appear in childhood and adolescence. Finally, a rare tumour known as ' chordoma' may arise from the remains of the primitive noto- chord in the basi-sphenoid (clivus Blumenbachii) and invade the pituitary region. (z) Neurinoma. The acoustic neurinoma or neurofibroma arises from the distal part of the auditory nerve. It is often single and unilateral, but may be bilateral or even associated with a diffuse neurofibromatosis either central or peri- pheral. In diffuse cenitral neurofibromatosis, similar and numerous neurinomata are found on the cranial nerves and spinal nerve roots, especially the cauda equina, and often on cranial nerves sucl as the third, fifth and twelfth (Wishart's syndrome). In addition numerous subcutaneous neurofibro- mata may be present. (3) Choroid plexus papilloma. These rare choroid plexus tumours represent in general a reproduction of the choroid plexus villi and arc said to originate from the latter. Actually they consist of numerous cauliflower-like villi with a central core of loose fibrous tissue and are rich in blood vessels. They occur in the lateral ventricle and in the roof of the third and fourth ventricles. (4) Pinealoma. This tumour grows from the epiphysis or pineal body and contains two types of cell in groups, (i) loose meshes of large spherical cells with large nuclei and a finely-granular cyto- plasm corresponding to the parenchymatous cells of the pineal body, and (ii) smaller lymphocyte- like cells lying in groups in a connective-tissue stroma. When of more primitive type and rapidly- growing, the tumour is termed a ' malignant pinealoma.' Pinealomata as they continue to grow exert pressure on neighbouring structures in the mid- brain and Sylvian aqueduct causing hydrocephalus by obstruction. The third ventricle may become filled with tumour-tissue and lead to compression of the hypothalamus, infundibulum and pituitary body. In addition to the usual results of increased intracranial tension, paralysis of upward conjugate deviation of the eyes and failure of vision may occur. Pineal teratomata-both cystic and solid-may also occur. Some of these teratomata when occurr- ing in boys before puberty are associated with pubertas praecox, in which there is precocity of both mental and physical development-the latter especially of the genitalia and secondary sexual characteristics. (b) The second main group includes the following: Based on an acddress to the Dartford Division of the British Medical Association, July I948. copyright. on May 20, 2020 by guest. Protected by http://pmj.bmj.com/ Postgrad Med J: first published as 10.1136/pgmj.24.277.579 on 1 November 1948. Downloaded from

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-579

INTRACRANIAL TUMOURSA Clinical Review

By C. WORSTER-DROUGHT, M.A., M.D., F.R.C.P.Phlysician to the IVest Entd Hospital for Diseases of the Nervous System and to the Mctropolitant Hospital

From the clinical standpoint intracranial tumourscan be divided roughly into two main groups:(a) Those that affect a particular region only,e.g. pituitary region (adenoma, craniopharyngioma,chordoma), acoustic nerve (neurinoma), choroidplexus (papilloma), pineal region (pinealoma).

(b) Those that may affect any region ofthe brain,e.g. various types of glioma, meningioma, angioma,metastatic tumours such as secondary carcinoma,etc.

(a) The first, or regional, group may be con-sidered as follows:

(i) Tumours of the pituitary region. Tumours ofthe pituitary body itself are usually adenomata,either chromophil, basophil, chromophobe, ormixed. The chromophil adenoma is almostinvariably associated with some degree of acro-megaly if occurring after puberty and if beforepuberty, with giantism. Adenoma of the basophilcells, according to Cushing, gives rise to a syn-drome characterized by increasing obesity,especially of the face, neck and trunk, sexualdystrophy and, in women, hirsutes of the face andtrunk. Chromophobe adenomata may be associatedwith some degree of hypopituitarism.

Other tumours affecting the pituitary regionare the group now known as ' craniopharyngio-mata' which comprises all squamous epithelialgrowths, both cysts and solid formations, arisingfrom the remains of the hypophyseal duct. Theyinclude, therefore, the tumours previously knownas Rathke's pouch tumours, suprasellar and sub-sellar pituitary cysts, adamantinomata, amelo-blastomata and hypophyseal duct tumours. Theirorigin is congenital and although symptoms maydevelop at any age from the first to the seventhdecade, they most frequently appear in childhoodand adolescence.

Finally, a rare tumour known as ' chordoma'may arise from the remains of the primitive noto-chord in the basi-sphenoid (clivus Blumenbachii)and invade the pituitary region.

(z) Neurinoma. The acoustic neurinoma orneurofibroma arises from the distal part of theauditory nerve. It is often single and unilateral,but may be bilateral or even associated with adiffuse neurofibromatosis either central or peri-

pheral. In diffuse cenitral neurofibromatosis,similar and numerous neurinomata are found onthe cranial nerves and spinal nerve roots, especiallythe cauda equina, and often on cranial nerves suclas the third, fifth and twelfth (Wishart's syndrome).In addition numerous subcutaneous neurofibro-mata may be present.

(3) Choroid plexus papilloma. These rarechoroid plexus tumours represent in general areproduction of the choroid plexus villi and arcsaid to originate from the latter. Actually theyconsist of numerous cauliflower-like villi with acentral core of loose fibrous tissue and are rich inblood vessels. They occur in the lateral ventricleand in the roof of the third and fourth ventricles.

(4) Pinealoma. This tumour grows from theepiphysis or pineal body and contains two typesof cell in groups, (i) loose meshes of large sphericalcells with large nuclei and a finely-granular cyto-plasm corresponding to the parenchymatous cellsof the pineal body, and (ii) smaller lymphocyte-like cells lying in groups in a connective-tissuestroma. When of more primitive type and rapidly-growing, the tumour is termed a ' malignantpinealoma.'

Pinealomata as they continue to grow exertpressure on neighbouring structures in the mid-brain and Sylvian aqueduct causing hydrocephalusby obstruction. The third ventricle may becomefilled with tumour-tissue and lead to compressionof the hypothalamus, infundibulum and pituitarybody. In addition to the usual results of increasedintracranial tension, paralysis of upward conjugatedeviation of the eyes and failure of vision mayoccur.

Pineal teratomata-both cystic and solid-mayalso occur. Some of these teratomata when occurr-ing in boys before puberty are associated withpubertas praecox, in which there is precocity ofboth mental and physical development-thelatter especially of the genitalia and secondarysexual characteristics.

(b) The second main group includes thefollowing:

Based on an acddress to the Dartford Division of theBritish Medical Association, July I948.

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(i) Glioma-various types (see classificationbelow).

(2) Meningioma, sometimes termed endotheli-oma and fibroblastoma which arises from themesothelial cells of the meninges. The cells areoval and epithelial-like and are arranged-in theform of islands separated from one another bystrands of connective tissue. The tumour oftenpossesses a capsule and does not invade butmerely depresses the brain tissue. Of all intra-cranial tumours they are the most favourable forsurgical removal.

(3) Psammoma. A meningeal tumour containingdeposits of lime salts. Bruns classifies as psam-momata only those tumours which arise in situa-tionis where lime salts are normally present, viz.the pineal body and choroid plexus.

(4) Cholesteatoma. Benign and avasculartumours arising mainly from the pia mater at thebase of the brain and in the lateral and fourthventricles. They also occur in the middle ear.'Their appearance is that of mother of pearl witha smooth surface and a connective tissue capsule.

(5) Angioma including haemangioma and haem-angioblastoma. Vascular tumours which may bearterial, venous or mixed. A haemangioblastomaconsists of proliferated blood-vessel cells or angio-blasts and occurs chiefly in the cerebellum. It isoften associated with cystic formations, whichmay also be found in the kidneys, pancreas andsuprarenal body (Lindau's disease).

(6) Congenital tumours (dermoid and teratoma).The dermoid is a sharply defined tumour or cystarising from displaced embryonic skin cells.Intracranial teratomata are rare and mav beassociated with other congenital malformations ofthe brain, spinal cord (spina bifida) or of other parts.

(7) Granulomata (tuberculoma and gumma orsyphiloma). Tuberculomata may calcify butoccasionally they terminate in tuberculous menin-gitis.

(8) Metastatic and invasive tumours (carcinoma,sarcoma, hypernephroma, myeloma).

During recent years there appears to have beena considerable increase in the case incidence ofprimary bronchial carcinoma. Multiple cerebralnietastases are especially liable to follow this formof the disease ; in fact the patient frequentlycomes under observation for the first time onaccount of symptoms of cerebral tumour. Theprimary growth in the lungs is often so small as toescape detection on clinical and X-ray examinationand is revealed only at autopsy. The secondarycerebral tumours are often multiple and occur mostfrequently in the temporo-sphenoidal and frontallobes and in the cerebellum.

Metastases of the brain are fairly common incarcinoma of the breast but infrequent in car-

cinoma of the bowel. When the latter occurs thelungs also show numerous secondary deposits.Melanotic sarcoma of cutaneous origin mayinvolve both the brain and the bones of the skull,and is usually rapidly fatal.

(9) Primary sarcoma may appear both in thebrain and meninges.

(io) Lipoma. This type of tumour occasionallyoccurs within the brain, usually at the base of theskull or above the corpus callosum.

(iI) Osteoma and osteochondroma arisinig fromthe bones of the skull.

(12) Various cysts. Subarachnoid cysts, hydatidand cysticercus cysts. Also in the lateral and thirdventricle, cysts may originate from the pia materand choroid plexus.

Classification of GliomataBailey and Cushing, in 1926, classified the

gliomata as follows:(i) Astrocytoma. The' glioma durum 'of earlier

writers, constituting about 40 per cent. of classifiedgliomata. The tumour is relatively avascular,hard to the touch and tends to become cystic.Microscopically it consists of cytoplasmic andfibrillary 'astrocytes, the processes of which forma network. As the tumour grows very slowly,it is relatively benign and patients may survivefor long periods even when surgical removal is in-complete. Astrocytomata may develop in almostany part of the central nervous system and theyoccasionally undergo calcification.

(2) Glioblastoma multiforme or spongioblastomamultiforme. A very malignant tumour occurringalmost without exception in the cerebral hemi-spheres of adults and representing the gliosarcomaof older writers. It grows very rapidly, and itsaverage course is seldom longer than twelvemonths. As the tumour tends to undergo extensivedegenerative changes it sometimes is difficult toclassify histologically. Microscopically, it is com-posed of polymorphic neuroglial cells; most ofthe cells are of. bipolar, fusiform shape whichcause the growth to resemble a spindle-celledsarcoma.

(3) Medulloblastoma. This is a soft vasculartumour usually occurring in children, and espec-ially originating in the anterior part of the roof ofthe fourth ventricle in the region of the vermis ofthe cerebellum. In this situation it is rapidlygrowing and highly malignant. The tumour hasalso been found in the cerebrum both of childrenand adults; in the latter it is sometimes well

1 'A Classification of Tumours of the Glioma Groupon a histogenic basis' by Percival Bailey and HarveyCushing, 1926 and 'Intracranial Tumours' by P. Bailey,I933.

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November 1948 WORSTER-DROUGHT: Intracranial Tumours 58I

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FIG. i.-Calcifying Menin-gioma. Superficially sit-uated in lower part ofright parietal region anderoding the overlyingbone. The patient was awoman, aged 22, withfocal epilepsy of threeyears' duration and thetumour was successfullyremoved by operation.

FIG. 2.-Calcifying Astro-cytoma in left front-parietal region. The lin-ear shadows seen in theregion indicated suggestthat the lesion is a cal-cified angioma but thisdiagnosis was excludedby arteriography. Thepatient was a man, aced29, with right-sidedhemiparesis and focalepilepsy of four years'duration. The greaterpart of the tumour wasremoved but recurredtwo years later.

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FIG. 3.- Craniopharyngiomnia (Supxa,sellat Cyst). Air-ventriculogram in Townes-Twiiig'. po:sition. ''The largesuprasellar cyst is seen Vprojed.ing'upwards and to theright and both lateral'ventrile.s are. considerably dilated.Sixtv-four cc. of yelo.w fluid containing numerouscholesterol crystals Were removed from the cyst bytrans-ventricular. tappihg. .under local anaesthesia, thecraniopharyn.gioma being successfullv removed at asecond-stage operation'. The patient was a man, aged40, with headache and failing vision of seven years'duration.

FIG. 4. - Medulloblastomainvolving the CorpusCallosum. Air-ventricu-logram showing a largetumour situated in themidline. The dilatedanterior horn of thelateral ventricle is seenin front of the tumourand the somewhat dilatedbody of the ventriclebehind. The patient wasa man, aged 48, with atwo years' history ofepileptiform attacks andmore recent mental con-fusion; the case provedfatal, the histologicaldiagnosis of the tumourbeingmade post-mortem.

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November I948 WORSTER-DROUGHT: Intracranial Tumours 583

FIG. 5.-Spongioblastoma Multiforme involving right thala-mus. Air-ventriculogram showing displacement ofventricular system to left with elevation of floor of rightlateral ventricle. In addition, the descending hom of theright lateral ventricle was obliterated and there was anirregular filling defect of third ventricle indicating anextension of the tumour into the hypothalamus. Thecase was that of a man, aged 45, and the condition provedfatal.

FIG. 6.-Spongioblastoma Multiforme involving basal gangliaon right side and obliterating the right lateral ventricle.Air-encephalogram showing obliteration of right lateralventricle by tumour with displacement of the dilated leftlateral ventricle and the upper part of the third ventricleto the left. The case was that of a man, aged 69, whichproved fatal, operation not being advised.

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584 POST GRADUATE MEDICAL JOURNAL November I94S

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FIG. 7.-Arterio-Venous An-gioma of left parieto-occipital region. Skia-gram showing density ofaffected area owing topartial calcification ofthe angioma. Thepatient, a man aged 33,had suffered from right-sided hemiparesis andhemianopia since the ageof four years and epilep-tiform attacks for twoyears.

FIG. 8.-Angiogram ofArterio-Venous Angi-oma. The same case asillustrated in Fig. 7.The left common carotidartery was ligatured tominimize the risk ofintracranial haemorrhagefrom the angioma ; theoperation resulted inconsiderable reduction infrequency of the epilepti-form attacks.

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encapsulated and may attain an enormous size.Microscopically the growth is composed of smallcells, poor in cytoplasm and with hyperchromaticnuclei; the cells tend to group themselves inthe form of rosettes.

(4) Astroblastoma usually occurs in the cerebralhemispheres of adults. It is relatively slowlygrowing and tends to become cystic. The tumouris rich in blood vessels around which the cells,precursors of astrocytes, are grouped in the formof palisades.From the point of view of classification, the

astroblastomata do not constitute a very welldefined group of tumours, as they represent atumour intermediate between the astrocytoma andthe glioblastoma; consequently, some examplesshow characteristics of glioblastoma multiforme.In general, however, they are more slowly growingand less malignant than the glioblastomata.

(5) Sponglioblastoma or glioblastoma polare. Aneoplasm composed mainly of unipolar spongio-blasts extending in parallel rows. It is relativelyavascular, slowly growing and benign but is liableto occur in comparatively inaccessible parts ofthe brain, especially in the mid-line of the brainfrom the optic chiasma in front to the medullaposteriorly.

(6) Oligodendroglioma. This tumour occursalmost exclusivelv in the sub-cortical white matterof the cerebral hemisphere of adults, especiallyin the fifth decade of life. It is very cellular, slowlygrowing and consists of cells with little cytoplasmwith eosinophilic granules. Areas of calcificationmay develop which show on X-ray examination.The tumour resembles a medulloblastoma butdiffers from it in that the nuclei are spherical andrich in chromatin.

(7) Ependymoma is a comparatively benigntumour which occurs along the walls of the ven-tricles, especially the fourth ventricle. In thelatter situation it extends into the cerebellum andis usually non-cystic. As the neoplasm growsonly very slowly, the patient may survive longafter decompression. It originates from ependy-mal cells and, microscopically, is composed ofependymoblasts.

(8) Ganglioneuroma or neurocytoma. A patho-logical curiosity. The tumour is very cellular,consists of numerous ganglion and glial cells andis closely related to the oligodendroglioma. Itoccurs principally in the tuber cinereum, medullaand cerebellum and may undergo calcification.These tumours are relatively benign.

(g) Neuro-epithelioma or medullo-epithelioma.A highly malignant tumour composed of embry-onic nerve cells. They are rare in the brain itselfbut more frequent in the spinal cord and in theretina. In the brain they occur more specially

in children, arising from the roof and floor-platesof the third and fourth ventricles. The character-istic microscopical feature is the presence of canalssurrounded by primitive spongioblasts in the formof rosettes.

Clinical AspectsFrom the clinical standpoint we may consider

the symptoms of intracranial tumour also undertwo headings:

(a) General symptoms and signs owing to thepresence of a tumour within the cranial cavity.

(b) Localizing signs according to the situationof the tumour.

(a) General symptoms and signs: These includethe symptoms of headache; occasional vomitingwithout relation to meals; papilloedema orswelling of the optic disc; and occasionallyepileptiform attacks. Headache is by -no meansconstant and may sometimes be absent in pituitarytumours, frontal tumours, etc. Vomiting is alsovariable and more liable to occur as a latersymptomwith increasing intracranial tension. Papilloedemais most frequent in cerebellar tumours, next infrontal and basal tumours; it may be absent intumours of the parietal and mid-brain regions.Papilloedema of more than four dioptres is anindication for decompression in order to savevision. Epileptiform attacks are an occasionalsymptom and are more liable to be met with inassociation with cortical and subcortical tumoursespecially of the parietal and frontal lobes or inthe moLre severe degrees of intracranial tension.

(b) Localizing signs: These vary according tothe situation of the neoplasm. In parietal lobetumour there is a gradually increasing contra-lateral hemiplegia, and if the lesion is on the leftside, probably aphasia in addition. Focal epilepsyaffecting an arm or a leg may also occur. Occipitallobe tumours will give rise to visual field defects-contralateral homonymous hemianopia, etc.Frontal lobe tumours are often associated withconfusion, amnesia, a grasp-reflex, and possiblywith some aphasia and contralateral hemiplegicsigns. Mid-brain tumours cause pupillary changesand oculomotor defects, occasionally emotionaldisturbances and euphoria. Cerebellar neoplasmsare indicated by nystagmus, ataxia, incoordinationof the homolateral limbs and often by dysarthria.

Tumours of the pituitary region result in dis-turbances of the visual fields (e.g. bilateral tem-poral hemianopia) and occasionally dystrophicendocrine syndromes.

Acrontegaly always indicates an eosinophiladenoma of the anterior pituitary lobe. The sameobservation may be made with regard to mostcases of giantism, especially those occurring beforepuberty.

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Craniopharyngioma enlarging before pubertyusually gives rise to some degree of dyspituitarism.This is sometimes of the Frohlich type-dwarfism,obesity and genital dystrophy. If the hypothalamusis also involved, the hypopituitarism may be ofthe Levi-Lorain type (infantilism, etc. withoutobesity). When symptoms of craniopharyngiomado not appear until the third to sixth decade,growth and development may have been normal;the initial symptom is then usually some visualdisturbance such as hemianopia, papilloedema,or optic atrophy.

Basophil adenoma (Cushing's syndrome) isassociated clinically with rapidly developing andoften painful obesity especially of the trunk andface, a tendency to kyphosis, amenorrhoea inwomen and functional impotence in men, hyper-trichosis of the face and trunk in females and adusky appearance of the skin with lineae atrophicaeand a raised blood pressure.

Adult hypopituitarism may result from a pituitaryadenoma of purely chromophobe type.

The solitary acoustic neurinoma or neurofibromagives rise to a characteristic clinical syndrome.This consists in nerve deafness on the affectedside and often tinnitus. Vertigo, owing to involve-ment of the vestibular portion of the eighth nerve,may also occur and is occasionally the initial symp-tom. Vestibular tests (caloric and rotatory) willindicate a lesion of the eighth nerve. Neighbouringcranial nerves are next affected-usually the fifth asshown by paraesthesia or anaesthesia of the cheek.The seventh nerve often escapes paralysis butmay suffer irritation as indicated by occasionalfacial twitching; the sixth nerve may also beaffected. Pressure of the tumour upon the neigh-bouring lobe of the cerebellum leads to nystagmustowards the affected side and ataxia of the homo-lateral arm and leg. As the tumour enlarges thepons may be compressed giving rise to motorweakness and increased deep reflexes in the contra-lateral limbs. With increasing intracranial tension,papilloedema usually develops.

DiagnosisA provisional diagnosis of intracranial neo-

plasm is made by careful attention to the symptomsand physical signs mentioned above, especiallyto the possible presence of papilloedema. Forconfirmation of the diagnosis we have the followingadditional investigations:

(i) Cerebrospinalfluid examination. On perform-ing lumbar puncture for the withdrawal of cere-brospinal fluid, the pressure of the latter is oftenfound to be increased (over i8o mm.). Examina-tion of the fluid will usually show no increasefrom the normal number of cells but an increasedprotein content, eqg. o.o6 to o. 15 per cent. or more

(normal 0.025 to 0.04 per cent.). Occasionallythe cerebrospinal fluid is slightly yellowish incolour (Froin syndrome) and may contain fibrinin addition to increased protein. The other con-stituents remain normal. A gumma is excluded bya negative Wassermann reaction in the blood andcerebrospinal fluiid.

(2) X-ray examination. A general thinning ofthe skull-bones due to increased intracranial ten-sion may be demonstrated, or the bone sometimesshows the appearance of beaten copper owing topressure of the convolutions producing localizedthinning. If the tumour is near the surface of thebrain, as in the case of some meningiomas, wemay see local erosion of the bone adjacent to thetumour (Fig. i). Also a tumour that has undergonecalcification (e.g. meningioma, astrocytoma, oligo-dendroglioma or tuberculoma) may reveal itspresence in the radiogram (Fig. 2).

In acoustic neurinoma, it is possible occasionallyto demonstrate erosion of the petrous portion ofthe temporal bone and dilatation of the internalauditory meatus.

Displacement of the pineal body may assistdiagnosis and lateralization of the tumour. Inabout 6o per cent. of normal individuals thepineal body is calcified and can be demonstratedon X-ray examination; in an antero-posteriorview, its displacement to one side suggests neo-plasm on the opposite side. Similarly, a calcifiedfalx cerebri or a calcified choroid plexus mayalso be displaced by a cerebral tumour.

In the diagnosis of pituitary tumour radiographyis of inestimable value. When the tumour ariseswithin the sella turcica (e.g. chromophil adenoma)the pituitary fossa is seen to be uniformly expandedor ' ballooned.' This finding is the rule in acro-megaly. Suprapituitary tumours (craniopharyn-gioma) tend to depress the sella turcica leading toflattening of the pituitary fossa and often toerosion of the clinoid processes. Extensive des-truction of bone in the pituitary region suggestschordoma or a basal meningioma.

Areas of calcification above the sella turcicaare sometimes seen in cases of craniopharyngioma.

(3) Air-encephalography and ventriculography.These radiographic methods are of the greatestassistance in the diagnosis and localization ofcerebral tumours.

Air-encephalography consists in the removal ofcerebrospinal fluid and the introduction of airinto the subarachnoid space by means of ordinarylumbar puncture. By regulating the position ofthe patient's head the air rises in the subarach-noid space and fills the cisterna magna and thecortical subarachnoid space ; if the foramina ofMagendie and Lushka are patent, the air will alsopenetrate into the fourth ventricle and lateral

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ventricles. The air is injected only very slowly-about i cc. per minute-the total amount injectedvarving between 30 and 6S cc. This procedurecan be carried out only if the cerebrospinal fluidpressure is within normal limits, that is, notabove i8o mm. If the cerebrospinal fluid pressureis above this level, air-encephalography by lumbarpuncture is unsafe and one has to resort to air-ventriculography.

Alir-ventriculography. The lateral ventricle ispunctured over Keen's point; cerebrospinal fluidis then withdrawn and replaced by an equalquantity of air by means of a two-way tap joiningthe needle and syringe. It is preferable to punctureboth ventricles as if only one is entered, the cere-brospinal fluid in the other ventricle cannot becompletely replaced by air and considerable mani-pulation of the head is required to enable any airto pass from the punctured lateral ventricle intothat of the opposite side. According to thechanges in symmetry of the ventricles on sub-sequent X-ray examination, one can often lateralizethe tumour and occasionally localize it (Figs. 3,4 and 5).

V'entricular estimation' may occasionallyassist. The quantities of fluid removed from eachventricle on puncture are carefully measured andcompared. Normally, the amounts are approxi-mately equal on the two sides. Any pronounceddifference indicates partial obliteration of thatventricle yielding the smaller quantity of fluidand this is usually on the side the tumour is situ-ated. Not infrequently, it is found impossible toobtain any cerebrospinal fluid from the lateralventricle of the affected side owing to its oblitera-tion by the tumour (Fig. 6).

(4) Angiography. This method of investigationconsists in the injection of a radiographicallyopaque substance thorotrast-directly into thecarotid artery and at once (within 2 seconds) takingan X-ray photograph of the head from the lateralaspect, the patient having previously been placedin position. The arteries are then seen outlinedin the developed X-ray film (arteriogram). Thus,the presence or otherwise of a suspected cerebralangioma may be confirmed (Figs. 7 and 8).Arteriographv is also of value in the demonstrationof aneurisms, especially those on or in the neigh-bourhood of the arterial circle of Willis. By meansof accurate timing (4 seconds) the outline of theveins may also be obtained on a subsequent film(venogram) and a purely venous angioma, ifpresent, demonstrated. Also, cerebral tumoursother than angiomata can sometimes be outlined.The usual method of angiography is to expose

the common carotid artery in the neck in order toensure accurate injection. More recently, a per-cutaneous technique has been developed, the

injection being made into the carotid arterythrough the skin.

(4) Electro-encephalography. The cells of thenormal resting cortex produce delicate spontan-eous oscillations of bioelectrical potential. Thesecan be detected through the intact skull and scalpby suitable electrodes, valve-amplifiers and asimple ink-writing oscillograph. The normal orso-called' alpha' rhythm has a frequency of aboutIo oscillations per second with an amplitude of6o and I00 microvolts.

It is rarely that any single method of clinicalinvestigation alone can be expected to establish acomplete diagnosis, and this observation appliesequally to electro-encephalography. The resultsof the latter must be correlated with those obtainedfrom physical, pathological and radiologicalexaminations. Nevertheless, with the aid of theelectro-encephalogram it is often possible toassist in localizing a cerebral tumour. The cells ofthe latter are electrically inactive, but at theperiphery of some tumours, e.g. a meningioma,or in a cortex infiltrated by a glioma, abnormallyslow or ' delta' waves, as they are termed, frdmI to 5 per second may be recorded and have apathological .and localizing significance.

Differential DiagnosisOther conditions which may simulate intra-

cranial tumour are:(i) Renal disease with uraemia. Headache and

vomiting are frequent symptoms, and even opticneuritis, monoplegia or hemiplegia may be present.The diagnosis is made by careful attention to theexamination of the urine, especially to the presenceof casts, the blood pressure, and the urea contentof the blood and the cerebrospinal fluid.

(2) Meningitis (various forms). The presence ofneck rigidity, Kernig's sign and great increase inthe number of cells in the cerebrospinal fluid(which may be turbid) usually give the diagnosis.

(3) General paresis (Dementia paralytica, G.P.I.).Cerebral tumour may be simulated by this con-dition, especially if mental confusion, vomiting,optic neuritis, and some form of muscular weak-ness is present. The diagnosis is made on thepositive W.R. in the blood and cerebrospinalfluid and in the latter a Lange curve of ' paretic'type.

(4) Encephalitis and encephalo-myelitis. Changesin the fundus are usually absent and after the firstfew days the cerebrospinal fluid is usually foundto be normal, that is, with no increase in cells orin the protein content. In doubtful cases, X-rayexamination and ventriculography will assist inexcluding intracranial tumour.

(5) Cerebral arteriopathies. The differentialdiagnosis from cerebral tumour is often difficult

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especially if signs of a focal lesion exist (e.g.hemiparesis or hemianopia). The difficulty isincreased by the facts that Xi) in cerebral arterio-pathy some increase in the protein content (up too.o8 per cent. or more) of the cerebrospinal fluidis often found and (ii) a cerebral tumour maycoexist with arteriosclerosis. Although an opticneuritis may occur in the latter, changes in thedisc itself are usually absent. Retinal arterio--sclerosis is nearly always present, the state of theretinal vessels being the best guide to the conditionof the cerebral arteries. The presence of a highblood pressure favours arteriosclerosis; on theother hand, in the presence of extensive atheromaof the cerebral vessels the blood pressure mayoften be quite low. A history of transient palsiesor of a sudden onset is more indicative Qf arterio-pathy. In some cases it is necessary to keep thepatient under observation for some time in orderto exclude cerebral tumour.

(6) Cerebral abscess. The diagnosis is mademainly on the presence or history of dischargefrom the ear, sinusitis or other source of infection.The commonest sites are the temporo-sphenoidallobe and the cerebellum. Papilloedema if presentat all is usually slight. The cerebrospinal fluidmay show a slight increase in the number oflymphocytes-even a few polymorphonuclearcells-as well as in the protein content. Thepresence of polymorphs in the cerebrospinal fluiddoes not necessarily indicate abscess as they mayoccur in association with some gliomas (e.g.glioblastoma). Also, a leucocytosis in the blood

may occasionally be found in cerebral tumour aswell as in abscess. In some cases a definitedifferential diagnosis can only be made by explora-tion.

TreatmentIn general, if the tumour can be localized,

operation should be performed with a view to itspossible removal. If extirpation is not practicable,as much as possible of the tumour can be removedand the remainder subjected to diathermy. Sub-sequently deep X-ray treatment can be applied.In deep-seated tumours, radon seeds have beenplaced around the tumour with occasional benefit.If the tumour cannot be localized and papilloedemais approaching 4 dioptres, decompression shouldbe carried out, either a right-sided subtemporaldecompression or one over the suspected site ofthe tumour.

In cases of pituitary tumour it is a safe rule notto operate for purely endocrine symptoms.

If operation is impracticable or undesirable forany reason the only resort is deep X-ray treatment.As a temporary measure for reducing increased

intracranial tension, the intravenous injection of50 to ioo cc. of a 50 per cent. solution of sucroseis often successful and has superseded the hyper-tonic saline (15 to 30 per cent.) formerly used.A more slowly acting method of reducing intra-cranial tension is by the rectal administration ofa 25 per cent. solution of magnesium sulphate,according to the quantity the patient will retain,up to a total of 250 cc.

RECURRENT UMBILICAL PAIN IN CHILDHOODWith special reference to non-specific mesenteric adenitis

By JOHN APLEY, M.D., M.R.C.P.Department of Child Health, Bristol University

Partisanship incurs both rewards and penalties.Of its rewards, the most obvious is increasedopportunity for exploitation; of its penalties, theoutstanding one is the hindrance of progress inother directions. In many branches of medicinethe results of over-emphasis have proved in thisway detrimental, and among them is the subjectunder consideration. The wide variety of causesto which 'umbilical colic' has been attributed,will be illustrated during this discussion. Theyrange between such extremes as '.neuropathicconstitution ' and ' chronic constipation,' accord-

ing to the bias of the observer due to variationsboth in opportunity and in thB method of approach.The main purpose of this paper is to survey the

existing extensive contributions in the light ofinformation derived from cases investigated per-sonally.

Causes of chronic or recurrent abdominalpain in childhoodAs a first step it will be helpful to set out a com-

prehensive list of possible causes of pain whichmay be localized to the umbilical region. It is

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