Malignant cerebral melanoma complicating giant pigmented naevus: a case report.

Abstract

The giant pigmented naevus is the most florid expression of cutaneous hyperpigmentation. Examples may be seen in illustrations of physiognomic works ofhet eightet4-cetury (Fanconi, -1956). To date over 200 cases of extensive cutaneous pigmentation have been recorded in the literature, and the pathogenesis, clinical course and pathology have recently been reviewed (Reed, Becker, Becker, and Nickel, 1965).Rokitansky in 1861 described a patient with a giant pigmented naevus and leptomeningeal hyperpigmentation. Since then some 20 cases of cutaneous and leptomeningeal hyperpigmentation have been reported and recently reviewed (Fox, Emery, Goodbody, and Yates, 1964;Reed et al., 1965). Excluding those cases in which either skin malignancy or metastases elsewhere in the body were present, there exist in the literature seven case reports of primary intracerebral melanoma complicating the condition of extensive cutaneous pigmentation ( Table I). The rarity of this association prompted us to report another case. CASE REPORTThe patient, a 15-year-old Polish girl, a binovular twin, was admitted on 7 November 1966 to a gynaecological unit. She presented with amenorrhoea of two months' duration. In response to further questions to the relatives, a history of increasingly severe bilateral retro-orbital headache and anorexia over the preceding month was obtained. Family history was non-contributary; in particular the patient's binovular twin sister was free of skin blemishes and well in every respect.She was apyrexial, listless, and apathetic. There was marked neck-stiffness and Kernig's sign was positive. There were no other localizing neurological signs. The striking feature was the presence of a giant, hairy, pigmented naevus of the 'cape' variety. It involved the posterior cervical and occipital regions and extended synmnetrically over both shoulders (Fig. 1). In addition, multiple large pigmented naevi were scattered over the rest of the body. protein 210 mg and sugar 36 mg/100 ml.; Pandy test positive, W.R. negative, Lange curve normal. No organisms were seen by Gram staining, and no acid fast bacilli were detected. The blood haemoglobin was 15-2 g/100 ml., white cell count 8,400 c.mm with a normal distribution, ESR 7 mm in the first hour (Westergren). Radiography of the chest and skull were normal. ELECTROENCEPHALOGRAM (9 November 1966): 'The patient was drowsy at the time of recording. The alpha rhythm was very unstable at 7-9 c/s, and appeared less in amount on the right side. Several bisynchronous and symmetrical generalized paroxysms of anteriorly predominant high voltage delta activity at 1-2 c/s were seen. Between the paroxysms the activity over the left hemisphere returned to a lower voltage mixture of theta and faster frequencies, whereas over the right hemisphere very irregular, moderate voltage delta activity persisted and was mixed with theta activity in the lower range. No constant focal features were seen, but the right hemisphere slow wave abnormality was most marked in the temporal region. Arousal response...

Familial aggregation of small congenital nevomelanocytic nevi

“…In 45 pairs of MZ twins [Siemans, 19241, 1,783 pigmented moles were identified; each twin had similar numbers of moles, albeit a nonidentical anatomic distribution. For giant CNN, discordance has been demonstrated for identical twins in three cases [Siemans and Waardenburg, 1927;Cantu et al, 1973;Amir et al, 19821, quintuplets in one case [Macklin, 19481, and nonidentical twins in two cases [Morris and Danta, 1968;Conway, 19391. Discordance in MZ twins, assuming an autosomal dominant mode of inheritance, could have multiple explanations, including pre-and postzygotic events.…”

Neurocutaneous melanoblastosis associated with cerebral sinus venous thrombosis

“…Neurocutaneous melanoblastosis has been reported to present with a variety of neurological complications in-cluding seizures in almost all patients, psychiatric disturbances, increased intracranial pressure with signs of space-occupying lesions, spinal cord involvement and hydrocephalus [1,[4][5][6][7][8][9][10]. The dense perivascular cellular infiltration in the leptomeninges acts as a barrier to complete CSF circulation and absorption [7].…”

Neurocutaneous Melanosis

“…we cannot exclude the possibility of a metastatic origin of the tumour in our patient. However, we favourthe hypothesisofaleptomcningeal origin of the melanoma, and the reasons for this opin ion are based on the following facts: the majority of malig nant transformation of giant pigmented naevi appear dur ing the first decade of life [4,6]; statistically, there is a much greater incidence of malignant transformation of lcptomeningeal melanosis than of cutaneous melanosis [3,7,27]; there was no evidence of a primary source outside the ner vous system, and other organs were normal in our patient; there was no involvement of the lymph nodes; several sam ples of different naevi failed to show any malignant trans formation.…”