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 »  Introduction
 »  Case report
 »  Discussion
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Year : 2001  |  Volume : 49  |  Issue : 2  |  Page : 200-2

Primary cerebello-pontine angle malignant melanoma : a case report.

Departments of Neurosurgery and Neuropathology, K.E.M. Hospital and Seth G.S. Medical College, Parel, Mumbai, 400 012, India.

Correspondence Address:
Departments of Neurosurgery and Neuropathology, K.E.M. Hospital and Seth G.S. Medical College, Parel, Mumbai, 400 012, India.

  »  Abstract

A rare case of primary malignant melanoma in the cerebello-pontine angle, in a 17 year old girl is presented. The patient presented with one month history of headache, diplopia, facial asymmetry and ataxia. The computerised tomography (CT) scan and magnetic resonance imaging (MRI) revealed a large cerebello-pontine angle mass with features suggestive of a melanoma. The typical black coloured, solid and vascular melanoma was excised completely. Cerebello-pontine angle melanoma are extremely rare tumours with dismal long term outcome in majority of these cases.

How to cite this article:
Desai K, Dindorkar K, Goel A, Shenoy A. Primary cerebello-pontine angle malignant melanoma : a case report. Neurol India 2001;49:200

How to cite this URL:
Desai K, Dindorkar K, Goel A, Shenoy A. Primary cerebello-pontine angle malignant melanoma : a case report. Neurol India [serial online] 2001 [cited 2022 May 16];49:200. Available from: https://www.neurologyindia.com/text.asp?2001/49/2/200/1262

   »   Introduction Top

Primary intracranial malignant melanomas are distinctly rare intracranial lesions.[1] We located eight cases of primary cerebello-pontine angle malignant melanoma in English literature. The subject is briefly reviewed, focussing on imaging character and management options.

   »   Case report Top

A 17 year old girl presented with one month history of rapidly worsening symptoms of headache, vomiting, diplopia and ataxia. On examination, she had right third and fifth to eighth cranial nerve paresis and right cerebellar signs. The computerised tomography (CT) scan showed a large, hyperdense, minimally enhancing lesion in the right cerebello-pontine angle, which extended into the Meckel's cave. The magnetic resonance imaging (MRI) showed the mass to be hyperintense on T1WI and relatively hypointense on T2WI (Fig. 1 and 2). Right internal carotid artery angiogram showed a faint tumour blush. The tumour was fed mainly by the meningeal branches of the cavernous part of carotid artery and the ascending pharyngeal artery. The lesion was completely excised by a lateral basal subtemporal approach.[2] The tumour was pitch black in colour. The cottonoid and saline irrigation used during surgery were stained black. The tumour was relatively firm, elastic and vascular. Except for some of the fifth cranial nerve fibres which were damaged during dissection, rest of the neural structures were preserved. The patient showed recovery in her neurological function following surgery, including the fifth nerve function. Clinical and radiological search for any other site of melanoma in the body was negative. Investigations included chest radiograph, ocular examination, ultrasonography of abdomen and barium enema. Postoperative scan confirmed total tumour resection. Histological examination revealed highly cellular tumour arranged in sheets. The cells were pleomorphic with abundant cytoplasm and dark black pigmentation. Bleaching confirmed the nature of pigment to be melanin. There was evidence of nuclear pleomorphism, mitosis and prominent nucleoli. Masson Fontana staining revealed positivity for melanin pigment . Subsequently, the patient received focal irradiation of 60CO in the dose of 6000 cGy (14 fractions) over 28 days. She was well at 12 month follow up.

   »   Discussion Top

Primary solitary intracranial melanoma is a rare tumour and its occurrence in the cerebello-pontine angle is extremely uncommon. Eight primary solitary cerebello-pontine angle melanomas have been reported in the literature till 1992.[3] Primary intracranial melanomas are either solitary or of diffuse variety.[4] Diffuse leptomeningeal melanomas preferentially affect children and may be part of neuro-cutaneous melanosis complex or phakomas.[5] Such melanomas usually present with features of raised intracranial pressure, cranial nerve palsies and meningism.[3] Focal melanomas present as leptomeningeal or dural based neoplasms and are more common in adults. Melanomas originate from melanocyte cell rest which are of neural crest origin. These cells are commonly distributed in the leptomeninges with a maximum density in the region of the caudal medulla and the high cervical cord.[6] The melanocytic tumours range between the relatively indolent melanocytomas to the aggressive malignant melanomas.[7] The prognosis of the patient with solitary primary intracranial melanomas depends upon the degree of mitosis, leptomeningeal dissemination, extent of surgical excision and location of the tumour.[3] Yamane et al reported mean survival of 20.7 months in patients with solitary primary intracranial melanomas, while the survival was only 6.7 months in patients with diffuse leptomeningeal spread.[8] Metastasis from primary intracranial melanomas to lungs, spleen, pancreas and kidneys although rare, have been reported in the literature.[9]
Metastasis from the extracranial primary melanoma is more prone to occur in the brain and is the third most common site of intracranial metastasis after carcinoma of breast and lung.[3] Intracranial melanomas is often confused with other pigmented lesions like pigmented meningioma, schwannoma, medulloblastoma, choroid plexus papilloma, astrocytoma and pituitary tumours. Primary malignant melanoma is exceptionally rare and the other sites of possible primary melanoma in the body should be excluded by clinical and radiological examination.[3] Cerebrospinal fluid examination can be positive in diffuse leptomeningeal variety of melanomas and spinal melanomas.[10] The melanomas have characteristic CT and MRI findings. The lesions are hyperdense on CT and on contrast administration there is only marginal enhancement. MRI shows a hyperintense lesion on T1WI and hypointense on T2WI. The hyperintensity of these lesions on T1WI is a result of the paramagnetic phenomenon exhibited by the melanin due to the presence of free radicals with unpaired electrons. The combination of characters on CT scan and MRI are unique for melanomas.[11],[12]
Radical tumour resection and use of aggressive whole brain irradiation have been recommended.[3],[8] The prognosis and long term survival interval is longer (19.6+2.3 months) in patients who had undergone a radical tumour resection and shorter (9.3+2.4 months) in patients where only a biopsy or partial tumour excision was done.[3] To conclude, primary solitary cerebello-pontine angle melanomas are rare tumours. Radical surgical excision is recommended to improve the prognosis and long term disease free interval.


  »   References Top

1.Rubinstein LJ : Neurocutaneous melanosis and primary meningeal melanoma. In : Tumours of the central nervous system. Atlas of tumor pathology, 2nd series, fasc 6. Washington D.C. Armed forces Institute of pathology, 1972; 309-311.   Back to cited text no. 1    
2.Goel A, Nadkarni T : Basal lateral subtemporal approach for trigeminal neurinomas : report of an experience with 18 cases. Acta Neurochir (wein) 1999; 141 : 711-719.   Back to cited text no. 2    
3.Baena RR, Gaetani P, Danova M et al : Primary solitary intracranial melanoma : case report and review of literature. Surg Neurol 1992; 38 : 26-37.   Back to cited text no. 3    
4.Kiel FW, Starr LB, Hansen JL : Primary melanoma of the spinal cord. J Neurosurg1961; 18 : 616-629.   Back to cited text no. 4    
5.Hoffan HJ, Freeman A : Primary malignant leptomeningeal melanoma in association with giant hairy nevi. Report of two cases. J Neurosurg 1967; 26 : 62-71.   Back to cited text no. 5    
6.Desai AP, Bhatt AD, Parab MH et al : Primary melanoma of the leptomeninges. A report of two cases. Indian Journal of Cancer 1982; 19 : 161-164.   Back to cited text no. 6    
7.Akelaitis AJE : Primary melanosarcoma of the leptomeninges. Am J Pathol 1935; 11 : 591-610.   Back to cited text no. 7    
8.Yamane K, Shima T, Okada Y et al : Primary pineal melanoma with long-term survival: case report. Surg Neurol 1994; 4 : 2433-2437.   Back to cited text no. 8    
9.Glasauer FE, Yuan RHP : Intracranial tumors with extracranial metastasis. Case report and review of the literature. J Neurosurg1963; 20 : 474-493.   Back to cited text no. 9    
10.Aichner F, Schuler G : Primary leptomeningeal melanoma: diagnosis by ultrastructural cytology of cerebrospinal fluid and cranial computed tomography. Cancer1982; 50 : 1751-1756.   Back to cited text no. 10    
11.Ginaldi S, Wallace S, Shalen P et al : Cranial computed tomography of malignant melanoma. Am J Roentgenol 1981; 136 : 145-149.   Back to cited text no. 11    
12.Yamashita H, Toba T, Yasunaga A et al : A case of primary intracranial malignant melanoma with characteristic magnetic resonance imaging. No Shinkei Geka 1989; 17 : 887-891.   Back to cited text no. 12    


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