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Table of Contents    
Year : 2017  |  Volume : 65  |  Issue : 1  |  Page : 110-112

Coexistent intracerebral metastatic melanoma and meningioma

1 Department of Pathology, BYL Nair Hospital, Mumbai, Maharashtra, India
2 Department of Neurosurgery, BYL Nair Hospital, Mumbai, Maharashtra, India

Date of Web Publication12-Jan-2017

Correspondence Address:
Dr. Sweety V Shinde
Building 1, Flat 27, Hajiali Doctor's Quarters, Hajiali, Mahalaxmi, Mumbai - 400 034, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.198212

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 » Abstract 

Coexistence of multifocal neural crest tumors, namely meningioma, melanoma, and nerve sheath tumors, is termed as neurocristopathy. Neurofibromatosis is the commonest form of neurocristopathy. We report a rare case of frontal lobe metastatic melanoma coexistent with a parietal lobe meningioma, in the absence of any stigmata of neurofibromatosis.

Keywords: Coexistent, intracerebral, melanoma, meningioma, metastatic, neurocristopathy,
Key Messages:
A melanoma and a meningioma in the brain or spinal canal can coexist in the form of a neurocristopathy and can mimic each other on magnetic resonance imaging. Establishing their simultaneous coexistence and confirming their histological identity is important as they have different management protocols and prognosis.

How to cite this article:
Shinde SV, Shenoy AS, Savant HV, Balasubramaniam SB. Coexistent intracerebral metastatic melanoma and meningioma. Neurol India 2017;65:110-2

How to cite this URL:
Shinde SV, Shenoy AS, Savant HV, Balasubramaniam SB. Coexistent intracerebral metastatic melanoma and meningioma. Neurol India [serial online] 2017 [cited 2021 Aug 2];65:110-2. Available from:

Melanomas and meningiomas are tumors of neural crest origin. Their coexistence is termed as neurocristopathy.[1] We report a case of parietal lobe meningioma existing with a metastatic melanoma in the frontal lobe. Multifocal neural crest tumors necessitate a thorough radiological examination for investigating the presence of a neurocristopathy.

 » Case Report Top

A 50-year-old woman complained of headache and slurred speech since the past 1 month. She had chronic hypertension and was on anti-hypertensive therapy.

Her magnetic resonance imaging (MRI) showed two distinct extra-axial lesions [Figure 1]a and [Figure 1]b. The frontal lobe mass measured 3.3 × 3.3 × 3.8 cm, and the parietal lobe mass measured 3.8 × 5.6 × 4.4 cm. Both masses were reported on radiology as meningiomas. No significant family history or stigmata of neurofibromatosis was found. At surgery, the frontal mass was observed to have brownish black color, and hence, sent for intraoperative diagnosis. The frozen section showed atypical cells with cytoplasmic brownish granules suggestive of a melanocytic lesion.
Figure 1: (a) Magnetic resonance imaging shows T1-weighted images. The frontal lobe mass (star) is hyperintense peripherally and isointense centrally, while the parietal lobe mass (arrow) is hypointense. Both were isointense on T2-weighted images. (b) MRI with contrast shows a homogeneous enhancement of both the masses

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Subsequently, the frontal mass was resected in toto without any vascular damage [Figure 2]a. Histopathology showed a highly cellular tumor. Cells showed abundant brownish pigment, nuclear pleomorphism, brisk mitoses, and tumor necrosis [Figure 3]a, [Figure 3]b and [Figure 4]a. Thus, a diagnosis of malignant melanoma was made. Immunohistochemistry (IHC) revealed that the tumor cells were positive for melanocytic markers S-100 and human melanoma black (HMB 45) [Figure 4]a inset] but negative for epithelial membrane antigen (EMA), which is a marker for meningothelial cells and carcinoma. Proliferative index by methylation-inhibited binding protein 1 (MIB-1) was 40%–50%.
Figure 1: (a) Magnetic resonance imaging shows T1-weighted images. The frontal lobe mass (star) is hyperintense peripherally and isointense centrally, while the parietal lobe mass (arrow) is hypointense. Both were isointense on T2-weighted images. (b) MRI with contrast shows a homogeneous enhancement of both the masses

Click here to view
Figure 3: (a) Photomicrograph (hematoxylin and eosin, 10×) showing the papillary pattern of the frontal lobe tumor. Tumor cells show cytoplasmic brown pigment (red arrows). (b) Photomicrograph (hematoxylin and eosin, 40×) showing pleomorphic melanoma cells with vesicular nuclei and prominent nucleolus (black arrows)

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Figure 4: (a) Photomicrograph (hematoxylin and eosin, 40×) showing coarse brown cytoplasmic pigment within the melanoma cells. Inset shows cytoplasmic positivity of HMB-45 immunostain. (b) Photomicrograph (hematoxylin and eosin, 40×) of the parietal lobe mass showing meningothelial whorls and fascicles of meningioma. Inset shows membranous positivity of epithelial membrane antigen (EMA) immunostain

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The parietal mass was firm and grey-white. It was resected en bloc. Histopathology showed the typical features of a meningioma. On immunohistochemistry (IHC), tumor cells were positive for epithelial membrane antigen (EMA) [Figure 4]b and vimentin but negative for melanocytic markers HMB-45, thus confirming the diagnosis of meningioma.

Within 2 weeks after surgery, the patient complained of loose motions and abdominal distention. Ultrasonography showed multiple, isoechoic hepatic nodules suggestive of metastases. Chest radiographs showed similar metastatic nodules within the left lung. Hepatic nodule biopsy was attempted; however, the tissue was inadequate to give a definite opinion. A repeat biopsy was not possible owing to the deteriorating clinical condition.

On questioning, the patient revealed that a blackish skin lesion in the neck region was biopsied in a private hospital a few months ago [Figure 2]b. The histopathology report showed a cutaneous melanoma. In view of the histopathology report, we considered the cutaneous neck lesion as the primary melanoma, with subsequent metastases to the brain, liver, and lung. The patient expired 1 month after surgery.

 » Discussion Top

Primary central nervous system (CNS) melanoma forms 1% of all primary melanomas;[2],[3] CNS is the third most common site of metastases from a melanoma; and, metastatic melanoma form 9% of CNS metastases.[4] Primary CNS melanocytic lesions include neurocutaneous melanosis, melanocytoma, primary leptomeningeal melanomatosis, and malignant melanoma.[2]

Neurocutaneous melanosis and melanomatosis present as diffuse, discolored thickening of leptomeninges. The former is a phakomatosis associated with Dandy–Walker or Sturge– Weber syndrome More Details. Melanocytoma is a benign, solitary tumor, while primary melanoma is an aggressive tumor. Neurocutaneous melanosis and melanocytoma have malignant potential.[2]

Melanocytic lesions on MRI are usually hyperintense on T1- and hypointense on T2-weighted images, and show homogenous contrast enhancement. These findings are due to indole quinolone and semiquinolone free radicals of melanin. Amelanotic melanoma is hypointense on T1- and hyperintense on T2-weighted images. Lesions with at least 10% melanin will show typical imaging characteristics of melanotic melanoma.[2]

Kan et al.,[4] reported two coexistent cerebral tumors that were misdiagnosed as meningioma on MRI and later diagnosed as melanoma on histopathology. The frontal lobe mass in our case was misdiagnosed as meningioma.

MRI cannot distinguish between primary and metastatic CNS melanomas. Both will be positive for melanocytic markers like S-100 and HMB-45. However, EMA positivity is absent in the former and present in the latter.[2],[5]

It is important to distinguish between primary and metastatic CNS melanoma. Both can be surgically resected. If the tumor is in vital areas of the brain, it is resected subtotally. Primary CNS melanoma does not respond well to radiotherapy and chemotherapy, unlike metastatic CNS melanoma. The drugs used for chemotherapy include dacarbazine, temozolomide, and ipulimumab.[3],[4] Our patient had a primary cutaneous melanoma, which later metastasized to the CNS, liver, and lungs.

Other CNS tumors with melanocytic differentiation include a schwannoma, medulloblastoma, teratoma, gliosarcoma, and craniopharyngioma. All of them can be distinguished by their typical histopathology and immunohistochemistry findings.[2]

Melanomas, meningiomas, and peripheral nerve sheath tumors are of neural crest origin. Complex neurocristopathy is a multifocal aberrant proliferation of neural crest tissues.[1] Neurofibromatosis type 1 is its most common example, manifesting with a coexistent meningioma, glioma, schwannoma, neurofibroma, and cutaneous melanoma. Concurrent tumors of neural crest origin as reported in literature [6],[7],[8],[9] are tabulated in [Table 1].
Table 1: Concurrent tumors of neural crest origin involving the central nervous system

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Melanoma can also coexist with tumors of non-neural crest origin, such as renal cell carcinoma, bilateral breast carcinoma, uterine fibromatosis, and intestinal adenomatous polyposis.[9] Our patient showed a meningioma coexistent with cutaneous melanoma along with CNS metastases, and thus can be classified under the broad category of neurocristopathy.

Rahmah et al.,[5] reported a pigmented atypical meningioma masquerading as a metastatic melanoma owing to the presence of brown pigment within it. Han et al.,[10] also reported the diagnostic difficulty in differentiating a pigmented meningioma during performance of an intraoperative diagnosis. Special stains such as Prussian blue can identify the brown hemosiderin pigment. In our patient, using Masson Fontana stain and bleach technique, the brown cytoplasmic pigment was proven to be melanin.

 » Conclusion Top

A melanoma and a meningioma in the brain or spinal canal can coexist in the form of a neurocristopathy, can mimic each other on MRI, can show metaplastic transformation into each other, and can also show tumor-to-tumor metastases. Identification of multiple neural crest tumors should alert the clinician toward the presence of a neurocristopathy.

All efforts to distinguish between a primary and metastatic melanoma of the CNS should be made on the basis of detailed clinical history and a full-body clinical examination. MRI with contrast study should be done preoperatively, and the diagnosis should be confirmed on histopathology. This is important because both primary and metastatic melanoma of the CNS have a different prognosis and require different treatment protocols.

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Conflicts of interest

There are no conflicts of interest.

 » References Top

Bolande RP. Neurocristopathy: Its growth and development in 20 years. Pediatr Pathol Lab Med 1997;17:1-25.  Back to cited text no. 1
Smith AB, Rushing EJ, Smirniotopoulos JG. Pigmented lesions of the central nervous system: Radiologic-pathologic correlation. Radiographics 2009;29:1503-24.  Back to cited text no. 2
Li YP, Zhang HZ, She L, Wang XD, Dong L, Xu E, et al. Primary extramedullary spinal melanoma mimicking spinal meningioma: A case report and literature review. Oncol Lett 2014;8:339-44.  Back to cited text no. 3
Kan P, Shelton C, Townsend J, Jensen R. Primary malignant cerebellopontine angle melanoma presenting as a presumed meningioma: Case report and review of the literature. Skull Base 2003;13:159-66.  Back to cited text no. 4
Rahmah NN, Horiuchi T, Nakayama J, Nitta J, Hongo K. Cutaneous malignant melanoma “recurred as” or “in coexistence” with meningioma? Surg Neurol Int 2010;1:60.  Back to cited text no. 5
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Warwar RE, Bullock JD, Shields JA, Eagle RC Jr. Coexistence of 3 tumors of neural crest origin neurofibroma, meningioma and uveal malignant melanoma. Arch Ophthalmol 1998;116:1241-3.  Back to cited text no. 6
Mehta P, Agrawal P, Luthert P, Durrani OM. Optic nerve sheath meningioma in a patient with choroidal malignant melanoma: A case report of a complex neurocristopathy. Orbit 2009;28:436-8.  Back to cited text no. 7
Pal D, Bhargava D, Bucur SD, Shivane A, Chakrabarty A, Van Hille P. Metastatic malignant melanoma with meningioma with intratumoral infarct: Report of an unusual case and literature review. Clin Neuropathol 2010;29:105-8.  Back to cited text no. 8
Sini G, Colombino M, Lissia A, Maxia S, Gulino M, Paliogiannis P, et al. Primary dermal melanoma in a patient with a history of multiple malignancies: A case report with molecular characterization. Case Rep Dermatol 2013;18:192-7.  Back to cited text no. 9
Han SH, Joo M, Lee BH, Park SH. Cytologic features of pigmented atypical meningioma mimicking melanoma on intraoperative crush preparations. Diagn Cytopathol 2015;43:149-52.  Back to cited text no. 10


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

  [Table 1]


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