J. clin. Path. (1969), 22, 183-186

Melanoma and pigmentation of the leptomeningesin Ugandan Africans

M. G. LEWISFrom the Pathology Department, Makerere Medical College, Kampala, Uganda

SYNOPSIS A case of leptomeningeal melanoma in an African child of 7 years is presentedtogether with a survey of pigmentation in the normal African brain.

There is a direct relationship between the depth of pigment of the leptomeninges and the skin inUgandan Africans, suggesting that similar factors operate in the control of melanocytes in thesetwo sites.

One of the earliest recorded cases of melanoma ofthe leptomeninges was described in 1861 byRokitansky and these tumours have been thesource of considerable interest and controversy overthe years. Single case reports have appeared in theliterature, and these have been extensively reviewedby Gibson, Burrows, and Weir (1957). Theycollected a total of 66 cases of meningeal melanosis,both benign and malignant, from the world literature.Since this condition appears to be such a relativerarity, it was decided to present the findings of achild seen in Uganda, and to report a study ofpigmentation of the leptomeninges and the relation- 4

ship to skin pigment in Ugandan Africans.FIG. 1. Surface of brain covered with black pigment.

CASE HISTORY

A 7-year-old female Muganda child was admitted witha four-week history of headaches and inability to walkor swallow. There was a history of a similar episode atthe age of 3 from which she apparently recoveredcompletely.On examination the child was comatose, with slight

neck rigidity and papilloedema. Lumbar puncturerevealed normal cerebrospinal fluid.The child did not regain consciousness and died.

POST-MORTEM EXAMINATION

The main abnormalities of interest were confined in thecranial cavity. The brain was oedematous and weighed1.200 g. The entire surface was covered with blackpigment located in the leptomeninges with only smallareas spared (Fig. 1). This pigmentation extended overthe cerebellar hemispheres, the brainstem, and spinalcord. On cutting the meninges with scissors, thick blackpigment, rather like indian ink, characteristic of melanin,was released.Received for publication 20 June 1968.

FIG. 2. Black tumour in anterior part of thecerebellum. Note the dilated fourth ventricle.

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M. G. Lewis

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FIG. 3. Spindle-shaped heavily pigmented cells filling thesubarachnoid spaces and extending into the Virchow-Robinspaces.

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FIG. 4. Pleomorphic cells, many containing melanin, in a

tumour of the cerebellum.

FIG. 6.

FIGS. 5 and 6. Pigmentation of the normal leptomeningesin an African.

184

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Melanoma and pigmentation of the leptomeninges in Ugandan Africans18

On cutting the brain, a single black tumour was seenin the cerebellum (Fig. 2) communicating with the surfacepigmentation. In addition a well marked intemal hydro-cephalus was noted. No other abnormality was foundand no other pigmented tumours of the central nervoussystem were discovered. A careful search was made forsigns of other melanotic tumours in the body but nonewere found.

MICROSCOPICAL EXAMINATION

The subarachnoid spaces and Virchow-Robin spaceswere tilled withi spindle cell and pleomorphic cells ladenwith melanin (Fig. 3).The cerebellar tumour was composed of similar cells,

many containing melanin, arranged in clumps and sheetswith evidence of invasion of adjacent tissues (Fig. 4).

TABLELEPTOMENINGEAL PIGMENT AND SKIN PIGMENTATION IN

UGANDAN AFRICANSBrainPigment

Scanty 14Moderate 8Extensive 1

23

Skin Pigmentation

Light Medium Dark Total

0 E 0 E 0 E

11-72 126-08 55-21 6

23

11-72 16-08 15-21 5

7

3-57 271-85 141-58 12

53 cases

0= Observed; E=Expected.2= 14.41 (4df) P<0-01

Comparison between scanty and moderate x2- 0-44 NS 2 dfComparison between scanty + moderate and extensive =

14-06, 2 df, P<0-00I

PIGMENTATION OF LEPTOMENINGES IN UGANDANAFRICANS

This study was prompted by the case of lepto-meningeal melanoma described and by the con-troversies regarding the possible origins of themelanocyte in this region (Willis, 1960), and theobject was to determine the normal distribution ofvisible pigment in the leptomeninges in UgandanAfricans and to determine if there was any recog-nizable relationship between this and skin pigmen-tation.

METHODS AND MATERIALS

A random selection of brains of all ages and tribes wasexamined in the postmortem room, and the areas ofvisible pigmnent were noted. The tribe, age, sex, anddegrees of skin pigment were also recorded. The skin ofeach case was subjectively graded as light, medium ordark. The degree of involvement of the leptomeningeswith visible pigment was graded as nil or very slight(brainstem only), moderate, with involvement of thebrainstem and optic chiasma, and extensive, involvingthe brainstem, chiasma, and areas of the ventral anddorsal surface of the hemnisphere (Figs. 5 and 6).

RESULTS

The results of these observations can be seen in theTable.Although these figures are subjective, they show

a definite trend: the light-skinned Africans havelittle or no pigment of the meninges while the verydark-skinned people show more widespread pig-mentation. The relatively smaller numbers of dark-skinned Africans is largely due to the fact that mostof the patients seen in the postmortem room arederived from the tribes close to Kampala, who areBantu and in general paler than the northern tribes

of Hamitic or Sudanic origin. There appears to bea direct relationship between the depth and extentof pigmentation of the meninges and the skin.

DISCUSSION

Melanoma and melanosis of the leptomeninges havebeen reviewed by Gibson et a! (1957) who found66 cases in the world literature up to that time andclassified them into two groups: (1) diffuse tumourof the meninges, especially at the base of the brainand around the spinal cord; (2) discrete foci ofmelanoma cells with diffuse meningeal distributionalso present.

Discrete single tumours were the exception, andthe main sites were frontal, parietal, and occipitallobes (nine authors), basal ganglia and cerebralpeduncles (one author). Fowler and Simpson (1962)reported a malignant cerebellar tumour in a21-year-old male Caucasian, which they interpretedas a medulloblastoma with neuroepithelium con-taining large amounts of melanin. Salm (1967) hasrecently reported a malignant melanoma of thecerebellum with extension into the meninges and ametastasis to the lungs in a 43-year-old Caucasianwoman.The present case shows well marked lepto-

meningeal melanosis and a malignant melanoma inthe cerebellum. The age of the child and the absenceof any other melanotic tumours makes this areasonably convincing case. Regarding the histo-genesis of primary malignant melanoma of theleptomeninges, it has been stated that the origin ofthe melanin-containing cells is uncertain (Willis,1960)., The source of diffuse or local melanotictumours in this site is certainly the elongated andbranched pigment cells normally seen in the piamater, especially in dark-skinned and Mongolianpeople (Willis, 1960). Willis (1965) considers thatthere may well be a common underlying anomaly of

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186 M. G. Lewis

melanogenesis in both skin and leptomeninges. Thisdoes not, of course, imply that both are derived fromthe neural crest since melanocytes in both thesesites could equally have been derived from amesenchymal source. In support of this concept of apossible connexion between pigmentation of theskin and leptomeninges, several authors have quotedexamples of pigmented skin naevi in associationwith leptomeningeal melanosis (Grahl, 1906;Schopper, 1913; Lua, 1914; MacLachlan, 1914;Berblinger, 1915; Hellmann, 1931; Bjomeboe, 1934;Lecoutourier, Ley, Titeca, and Van Bogaert, 1939;Touraine, 1949), showing that the neurocutaneouspigmentation was a familiar condition transmittedas a Mendelian dominant. Several such familial caseshave been added since by Ketels-Harken (1963) andTventson (1965).The results show an association between the

depth and extent of pigmentation in the normalmeninges and the normal skin. This is unlikely to betrue for Africans only, and the less easily recognizedmelanocyte in Caucasian leptomeninges must be the

cell of origin of this rare condition of leptomeningealmelanosis and malignant melanoma.

1 am grateful to Professor R. S. F. Schilling, oftheLondonSchool of Hygiene and Tropical Medicine, for his adviceand to Miss Walford who did the statistical test incor-porated in the Table.

REFERENCES

Berblinger, W. (1915). Virchows Arch. path. Anat., 219, 328.Bjorneboe, M. (1934). Frankfurt Z. Path., 47, 363.Fowler, M., and Simpson, D. A. (1962). J. Path. Bact., 84, 307.Gibson, J. B., Burrows, D., and Weir, W. P. (1957). Ibid., 74, 419.Grahl, F. (1906). Beitr. path. Anat., 39, 66.Hellmann, P. (1931). Zbl. Allg. Path. path. Anat., 52, 369.Ketels-Harken, H. (1963). Ibid., 104, 396.Lecoutourier, R., Ley, J., Titeca, J., and van Bogaert, L. (1939).

J. belge Neurol. Psychiat., 39, 103.Lua, M. (1914). Arch. Psychiat., 53, 895.Maclachian, W. G. (1914). J. med. Res., 24, 433.Rokitansky, S. (1861). Allg. wien. med. Ztg., 6, 113.Saim, R. (1967). J. Path. Bact., 94, 196.Schopper, K. S. (1913). Frankfurt. Z. Path., 13, 77.Touraine, A. (1949). Ann. Derm. Syph. (Paris), 9,489.Tventson (1965). Acta path. microbiol. scand., 63, 1.Willis, R. A. (1960). Pathology of Tumours, 3rd ed., p. 917. Butter-

worths London.(1965). Med. J. Aust., 1, 827.

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Africansthe leptomeninges in Ugandan Melanoma and pigmentation of

M. G. Lewis

doi: 10.1136/jcp.22.2.1831969 22: 183-186 J Clin Pathol

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