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Year : 2018  |  Volume : 13  |  Issue : 1  |  Page : 168-171

A unique case of primary intracranial melanoma

Department of Neurosurgery, Sher-I-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India

Date of Web Publication6-Feb-2018

Correspondence Address:
Arif Hussain Sarmast
Dalipora, Kawadara, Srinagar - 190 002, Jammu and Kashmir
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1793-5482.224832

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Primary intracranial melanoma is an uncommon entity and only case reports have been published in the literature. We report a case of an elderly male who was operated with a preliminary diagnosis of meningioma, but it proved to be a histological surprise as it came out to be melanoma with no primary anywhere in the body.

Keywords: Central nervous system, melanocytes, melanoma

How to cite this article:
Sarmast AH, Mujtaba B, Bhat AR, Kirmani AR, Tanki HN. A unique case of primary intracranial melanoma. Asian J Neurosurg 2018;13:168-71

How to cite this URL:
Sarmast AH, Mujtaba B, Bhat AR, Kirmani AR, Tanki HN. A unique case of primary intracranial melanoma. Asian J Neurosurg [serial online] 2018 [cited 2022 May 18];13:168-71. Available from:

  Introduction Top

Primary intracranial melanoma is uncommon and accounts for only approximately 1% of all cases of melanoma.[1] Intracranial melanomas are often complicated to diagnose with differential diagnosis of other pigmented lesions such as pigmented meningioma, schwannoma, medulloblastoma, choroid plexus papilloma, astrocytoma, and pituitary tumors.[2] In this report, we present a case of primary intracranial melanoma in the left frontal area; the clinical, neuroradiological, and histological findings are discussed along with a review of the literature.

  Case Report Top

The patient is a 48-year-old man who presented to us with the history of rapidly worsening symptoms of headache and nausea. A left frontal lesion with variegated appearance was detected on noncontrast computed tomography (CT) head [Figure 1]. A contrast-enhanced magnetic resonance imaging brain was done demonstrated a heterogeneous mass lesion, (hyperintense on T1-weighted image [T1-WI] and relatively hypointense on T2-WI) [Figure 2] with mass effect with perilesional edema was seen in the left frontal area. A provisional diagnosis of meningioma was made preoperatively. Patient underwent gross total resection of this lesion. The lesion was large about 7 by 5 cm, blackish, vascular with no dural attachment, and confined to the left frontal lobe. Histopathological examination revealed that the tumor consisted of epithelioid cells, arrayed in sheets, with abundant melanin pigment deposits in the cytoplasm. The tumor was hypercellular with some signs of pleomorphism. Mitoses were present. Foci of hemorrhage and necrosis were seen [Figure 3] and [Figure 4]. Both melanin and hemosiderin pigment were identified by Masson-Fontana stain and iron stain, respectively. The neoplastic cells were stained positively with human melanin black-45 (HMB-45) antibody. It was reported as malignant melanoma. Clinical and radiological search for any other site of melanoma in the body was negative. Investigations included chest radiograph, ocular examination, and ultrasonography of abdomen. Postoperative period proved to be uneventful and patient was discharged on the seventh postoperative day with complete excion confirmed by post opertative NCCT Head and MRI Brain [Figure 5] and [Figure 6].
Figure 1: Noncontrast computed tomography head showing a left frontal lesion with variegated appearance, mass effect, edema, and midline shift

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Figure 2: Contrast-enhanced magnetic resonance imaging brain showing a left frontal contrast enhancing mass lesion

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Figure 3: Histopathological examination showing melanoma infiltration in the form of nests with pigment deposition (melanin) and surrounding brain tissue (H and E, ×40)

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Figure 4: Histopathological examination showing infiltration of melanoma cells with high nucleo-cytoplasmic ratio, prominent nucleoli, and melanin pigment (H and E, ×400)

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Figure 5: Postoperative noncontrast computed tomography head of patient showing complete excision of mass lesion

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Figure 6: Fluid attenuation inversion recovery sequence of postoperative magnetic resonance imaging showing mild edema with no mass lesion

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  Discussion Top

Primary malignant melanoma is very rare, and the other sites of possible primary melanoma in the body should be excluded by clinical and radiological examination.[1] Primary melanocytic tumors of the central nervous system (CNS) form a rare entity which is histologically and clinically distinct from metastatic cutaneous or retinal malignant melanoma. These account for only approximately 1% of all cases of melanoma. During embryonic development, precursor melanocytes originate in the neural crest and migrate actively to peripheral sites. In humans, melanocytes can be found in skin, mucous membranes, parenchyma, and uvea.[3],[4],[5] In most instances, melanomas involving the CNS represent metastatic disease. Melanoma is the most common tumor, after lung and breast cancers, which produces brain metastases.[6],[7]

According to the literature regarding primary melanoma of the nervous system, the origin of melanin cells is not fully understood, although several histogenetic theories have been proposed: (1) The mesodermal theory: The mesoderm gives rise to pigment cells which reach the brain or 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. (3) The neurogenic theory: The pigment cells originate from the neural crest, which develop into mesodermal and neural elements.[8] Primary intracranial melanomas are either solitary or of diffuse variety.[9] Diffuse leptomeningeal melanomas preferentially affect children and may be part of neurocutaneous melanosis complex or phakomas.[10] Such melanomas usually present with features of raised intracranial pressure, cranial nerve palsies, and meningism.[11] Focal melanomas present as leptomeningeal or dural based neoplasms and are more common in adults. The melanocytic tumors range between the relatively indolent melanocytomas to the aggressive malignant melanomas.[11] The prognosis of the patient with solitary primary intracranial melanomas depends on the degree of mitosis, leptomeningeal dissemination, extent of surgical excision, and location of the tumor.[11] The differential diagnoses of leptomeningeal tumors include meningioma, meningeal melanocytoma, leptomeningeal melanosis, metastases, and sarcoma.[4],[5] It is difficult to distinguish primary CNS melanoma from metastatic melanoma on neuroimages alone.[12] Hayward [13] proposed the following factors for establishing a diagnosis of a primary CNS melanoma: (a) No malignant melanoma outside the CNS, (b) leptomeningeal involvement, (c) intramedullary spinal lesions, (d) hydrocephalus, (e) tumor location in the pituitary or pineal gland, and (f) a single intracerebral lesion. In this case, the preoperative diagnostic consideration was a meningioma. Meningioma has similar neuroradiologic findings, such as enhanced hyperdense lesion on CT scan, and contact with the skull and dura mater. HMB-45 is an antibody with a higher specificity for melanocytic tumors. According to the literature, 86–97% of melanocytic tumors are positive for HMB-45 antigen.[3],[14] Tumor bleeding can be detected by positive Prussian blue stains for iron. Radiotherapy, chemotherapy, and immunotherapy may be considered nonsurgical therapy options for primary melanoma of the CNS, but their effects have not been clearly established. Various authors suggested that higher total doses of radiation (>40 Gy) should be irradiated to the tumor area for patients with good general conditions and absence of or controlled extracranial disease.[13],[14],[15] As for chemotherapy, dimethyl-triazeno-imidazole-carboxamide is the most commonly employed a chemotherapeutic agent.[16],[17] Unfortunately, a standardized therapy concept is still lacking. Literature search showed 95% mortality in case of metastasis of melanoma to the brain. Despite treatment, the median survival is less than a year.[18] The clinical outcome of patients with primary CNS melanoma is reported to be better than that of patients with metastatic disease.[19]

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Conflicts of interest

There are no conflicts of interest.

  References Top

Rubinstein LJ. Neurocutaneous melanosis and primary meningeal melanoma. In: Tumours of the Central Nervous System. Atlas of Tumor Pathology, 2nd Series, Fasc. 6. Washington, DC: Armed Forces Institute of Pathology; 1972. p. 309-11.  Back to cited text no. 1
Önal C, Ozy G, Akyürek S, Selek U, Ozyar E, Atahan L. Primary intracranial solitary melanoma: A rare case with long survival. Turk J Cancer 2006;36:185-7.  Back to cited text no. 2
Greco Crasto S, Soffietti R, Bradac GB, Boldorini R. Primitive cerebral melanoma: Case report and review of the literature. Surg Neurol 2001;55:163-8.  Back to cited text no. 3
Copeland DD, Sink JD, Seigler HF. Primary intracranial melanoma presenting as a suprasellar tumor. Neurosurgery 1980;6:542-5.  Back to cited text no. 4
Salpietro FM, Alafaci C, Gervasio O, La Rosa G, Baio A, Francolini DC, et al. Primary cervical melanoma with brain metastases. Case report and review of the literature. J Neurosurg 1998;89:659-66.  Back to cited text no. 5
McGann GM, Platts A. Computed tomography of cranial metastatic malignant melanoma: Features, early detection and unusual cases. Br J Radiol 1991;64:310-3.  Back to cited text no. 6
Gupta G, Robertson AG, MacKie RM. Cerebral metastases of cutaneous melanoma. Br J Cancer 1997;76:256-9.  Back to cited text no. 7
Pappenheim E, Bhattacharji SK. Primary melanoma of the central nervous system. Clinical-pathological report of a case, with survey and discussion of the literature. Arch Neurol 1962;7:101-13.  Back to cited text no. 8
Kiel FW, Starr LB, Hansen JL. Primary melanoma of the spinal cord. J Neurosurg 1961;18:616-29.  Back to cited text no. 9
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. 10
Rodriguez y Baena R, Gaetani P, Danova M, Bosi F, Zappoli F. Primary solitary intracranial melanoma: Case report and review of the literature. Surg Neurol 1992;38:26-37.  Back to cited text no. 11
Wadasadawala T, Trivedi S, Gupta T, Epari S, Jalali R. The diagnostic dilemma of primary central nervous system melanoma. J Clin Neurosci 2010;17:1014-7.  Back to cited text no. 12
Hayward RD. Malignant melanoma and the central nervous system. A guide for classification based on the clinical findings. J Neurol Neurosurg Psychiatry 1976;39:526-30.  Back to cited text no. 13
Brat DJ, Giannini C, Scheithauer BW, Burger PC. Primary melanocytic neoplasms of the central nervous systems. Am J Surg Pathol 1999;23:745-54.  Back to cited text no. 14
Isokangas OP, Muhonen T, Kajanti M, Pyrhönen S. Radiation therapy of intracranial malignant melanoma. Radiother Oncol 1996;38:139-44.  Back to cited text no. 15
Nakagawa H, Hayakawa T, Niiyama K, Nii Y, Yoshimine T, Mori S. Long-term survival after removal of primary intracranial malignant melanoma. Case report. Acta Neurochir (Wien) 1989;101:84-8.  Back to cited text no. 16
Ozden B, Barlas O, Hacihanefioglu U. Primary dural melanomas: Report of two cases and review of the literature. Neurosurgery 1984;15:104-7.  Back to cited text no. 17
Goulart CR, Mattei TA, Ramina R. Cerebral melanoma metastases: A critical review on diagnostic methods and therapeutic options. ISRN Surg 2011;2011:276908.  Back to cited text no. 18
Saha S, Meyer M, Krementz ET, Hoda S, Carter RD, Muchmore J, et al. Prognostic evaluation of intracranial metastasis in malignant melanoma. Ann Surg Oncol 1994;1:38-44.  Back to cited text no. 19


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]

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