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|Year : 2016 | Volume
| Issue : 1 | Page : 193-194
Black brain caused by primary intracranial malignant melanoma
Harish Naik, Hrushikesh Kharosekar, Vernon Velho
Department of Neurosurgery, Grant Medical College, Mumbai, Maharashtra, India
|Date of Web Publication||11-Jan-2016|
Department of Neurosurgery, Grant Medical College and Jamshedjee Jeejeebhoy Group of Hospitals, Byculla, Mumbai - 400 008, Maharashtra
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Naik H, Kharosekar H, Velho V. Black brain caused by primary intracranial malignant melanoma. Neurol India 2016;64:193-4
Primary intracranial melanoma is uncommon and occurs in approximately 1% of all cases having a melanoma. The clinical and radiological patterns of these tumors can mimic the presence of a meningioma. As melanocytes are considered to originate from neural crest cells and are found in normal leptomeningeal tissues, it is not surprising that primary melanomas also grow within the central nervous system (CNS). However, primary CNS melanomas have rarely (less than 25) been reported.
A 35-year old woman presented with complaints of headache, vomiting, and neck pain since 1 month. Her neurological examination was normal. Computed tomography (CT) scan revealed a well-defined, extra-axial, dural-based, hyperdense lesion measuring 3 × 3.5 × 3.7 cm with homogenous enhancement. CT scans of the chest, abdomen, and pelvis showed no abnormalities. Brain magnetic resonance imaging (MRI) with contrast showed a lesion in the left parietal area that was hyperintense on T1- and hypointense on T2-weighted images. There was heterogeneous enhancement after administration of gadolinium. Clinically, and based on imaging characteristics, we suspected the lesion to be a hemorrhagic metastasis, or a meningioma with intratumoral bleeding [Figure 1]a and [Figure 1]b.
|Figure 1: (a and b) Preoperative brain MRI with contrast showing the intensely enhancing lesion|
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Intraoperatively, a large, blackish, vascular, firm mass was seen in the left parietal area extending up to the temporal lobe along the sylvian fissure, with the brain acquiring a dark black coloration [Figure 2]a and [Figure 2]b. Postoperative scans showed complete excision of the lesion [Figure 3]. Cerebrospinal fluid examination showed no evidence of atypical or malignant cells. Histopathological examination of the tumor tissue was suggestive of a malignant melanoma. Dihydroxyphenyalanine (DOPA) oxidase reaction was positive. The tumor cells exhibited positivity for HMB-45, S-100, and vimentin [Figure 4]. In view of a strong clinical suspicion of metastasis and rarity of a primary malignant melanoma, a search for a primary lesion was made. However, all these investigations turned out to be normal, suggesting the presence of a primary intracranial malignant melanoma in our patient.
|Figure 2: (a and b) Intraoperative picture showing the black brain and the specimen|
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|Figure 3: Postoperative brain CT with contrast showing complete excision|
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Primary melanoma of the CNS arises from melanocytes, which are derived from precursor cells (melanoblasts) originating in the neural crest cells. In the craniospinal axis, melanocytes are predominantly located in the leptomeninges at the anterior and lateral surfaces of the spinal cord, the brain stem, and the base of the brain., According to the criteria set forth by Hayward, a CNS melanoma is more likely to be primary if there is no evidence of melanoma outside the CNS. Our patient had a solitary lesion with leptomeningeal involvement, without any systemic disease. Regarding primary melanomas of the nervous system, the origin of the melanin cells is not fully understood, although several histogenetic theories have been proposed that include: (1) The mesodermal theory: The mesoderm gives rise to pigment cells that reach the brain or the spinal cord via the pial blood vessels; (2) the ectodermal theory: Some epithelial cells produce pigment, and therefore, CNS melanomas are derived from the aberrant embryonic ectodermal cells; and, (3) the neurogenic theory: The pigment cells originate from the neural crest, which develop into mesodermal and neural elements.
Primary melanoma of the CNS can be divided into four different categories: (a) Diffuse leptomeningeal melanomatosis,
(b) discrete spinal cord melanomas, (c) primary intracranial isolated melanomas, and (d) melanosis of the meninges of the brain and/or the spinal cord associated with cutaneous pigmentation (the so-called neurocutaneous melanosis or phakomatosis).
Radiotherapy, chemotherapy, and immunotherapy may be considered as nonsurgical options for managing primary melanomas of the CNS, but their effects have not been clearly established. Olli-Pekka et al., suggested that higher total doses of radiation (>40 Gy) to the tumor bed in patients in a good general condition may result in the eradication or control of the extracranial disease. Dimethyl triazeno imidazole carboxamide (DTIC) is the most commonly used chemotherapeutic agent.
A CNS melanoma without any cutaneous manifestations is difficult to diagnose preoperatively. The prognosis is good in cases of totally excised melanomas. Therefore, an aggressive surgical resection is recommended. The role of adjuvant therapy has not been well-established.
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Conflicts of interest
There are no conflicts of interest.
| » References|| |
Liao WC, Ho JT, Lai PH, Tseng HH. Primary intracranial melanoma: Report of a case and review of the literature. Mid Taiwan J Med 2002;7:118-23.1.
Suranagi VV, Maste P, Malur PR. Primary intracranial malignant melanoma: A rare case with review of literature. Asian J Neurosurg 2015;10:39-41.2.
Shah I, Imran M, Akram R, Rafat S, Zia K, Emaduddin M. Primary intracranial malignant melanoma. J Coll Physicians Surg Pak 2013;23:157-9.3.
Somers KE, Almast J, Biemiller RA, Silberstein HJ, Johnson MD, Mohile NA. Diagnosis of primary CNS melanoma with neuroimaging. J Clin Oncol 2013;31:e9-11.4.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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