Atormac
brintellex
Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 3042  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Search
 
  » Next article
  » Previous article 
  » Table of Contents
  
 Resource Links
  »  Similar in PUBMED
 »  Search Pubmed for
 »  Search in Google Scholar for
 »Related articles
  »  Article in PDF (2,064 KB)
  »  Citation Manager
  »  Access Statistics
  »  Reader Comments
  »  Email Alert *
  »  Add to My List *
* Registration required (free)  

 
  In this Article
 »  Abstract
 »  Introduction
 »  Case Report
 »  Discussion
 »  References
 »  Article Figures

 Article Access Statistics
    Viewed4700    
    Printed169    
    Emailed1    
    PDF Downloaded61    
    Comments [Add]    
    Cited by others 6    

Recommend this journal

 


 
CASE REPORT
Year : 2010  |  Volume : 58  |  Issue : 5  |  Page : 781-783

Primary spinal melanoma of the cervical leptomeninges: Report of a case with brief review of literature


1 Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
2 Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Date of Acceptance22-Jul-2010
Date of Web Publication28-Oct-2010

Correspondence Address:
Mukul Vij
Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareilli Road, Lucknow - 226 014, UP
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.72209

Rights and Permissions

 » Abstract 

Central nervous system primary malignant melanoma accounts for approximately 1% of all melanomas. Primary spinal melanomas are even more unusual. We report a patient with primary spinal melanoma of the cervical leptomeninges. The histology of the tumor showed tumor cells arranged in sheets, ill-defined fascicles and nests and displayed a moderate grade of cellular and nuclear pleomorphism and mitoses with abundant pigment in the cytoplasm. The tumor cells were immunoreactive for HMB-45, and for S-100.


Keywords: Central nervous system, leptomeninges, primary melanoma, spinal


How to cite this article:
Vij M, Jaiswal S, Jaiswal A, Behari S. Primary spinal melanoma of the cervical leptomeninges: Report of a case with brief review of literature. Neurol India 2010;58:781-3

How to cite this URL:
Vij M, Jaiswal S, Jaiswal A, Behari S. Primary spinal melanoma of the cervical leptomeninges: Report of a case with brief review of literature. Neurol India [serial online] 2010 [cited 2020 Nov 28];58:781-3. Available from: https://www.neurologyindia.com/text.asp?2010/58/5/781/72209



 » Introduction Top


Central nervous system (CNS) primary malignant melanoma accounts for approximately 1% of all melanomas. Primary spinal melanomas are even more unusual. Since the initial report by Hirschberg in 1906, only 40 cases have been reported. These include intramedullary or extramedullary, leptomeningeal and extradural lesions. [1],[2],[3],[4],[5] This also includes 14 cases with dissemination from primary CNS malignant melanoma. The prognosis is poor, however, primary spinal melanoma has a better prognosis than cutaneous melanoma. We present a patient with primary spinal melanoma of the cervical leptomeninges.


 » Case Report Top


A 40-year-old man presented with complaints of sudden-onset progressive pain in right shoulder and neck of six months duration. Three months later, he developed gradual-onset progressive weakness of right upper limb; followed by weakness of right lower limb since 2 months. He also complained of right upper limb heaviness since 2 months. There was no history of bladder or bowel complaints. Neurological examination showed motor power of 4/5 grade on the right side and normal power on the left side. Deep tendon reflexes were brisk on the right side. Right plantar was extensor and left plantar flexor. Spinal tenderness against C2-C3 was noticed. Magnetic resonance imaging (MRI) spine revealed a well-defined round intradural extramedullary lesion measuring 2.1×1.9×1.5 cm against C1-C2. The lesion was hyperintense on T1 [Figure 1]a and heterointense on T2 [Figure 1]b images. There was intense contrast enhancement [Figure 1]c. C1-C3 laminectomy with removal of posterior rim of foramen magnum and excision of lesion was performed. The tumor was attached to dura and had to be sharply incised to separate it. Duroplasty was done. Postoperatively, the patient did not experience any neck or shoulder pain and remains neurologically intact. He has not received adjuvant chemotherapy or radiation therapy. Subsequent evaluations over a period of 1 year by oncologists failed to find any other melanotic lesions or primary melanoma. Repeated magnetic resonance imaging of the craniospinal axis has provided normal results.
Figure 1: (a) MRI scans of spinal melanoma: Sagittal section T1 image showing intradural hyperintense lesion at C2 level; (b) T2 image showing hypointense lesion; and (c) T1 contrast image showingintense contrast enhancement in the lesion

Click here to view


Grossly the surgical specimen submitted for histology measured 2.2×2.1 cm. It was blakish in colour. The tissue was fixed in 10% buffered formalin for 12 hours and processed for routine histology. Paraffin sections were stained with hematoxylin and eosin, Masson Fontana for melanin, and potassium permanganate bleach for melanin. Based on light microscopic examination, representative sections were selected for immunohistochemistry for HMB-45, S-100 and glial fibrillary acidic protein (GFAP). The histology of the tumor showed a solid growth [Figure 2]a. The tumor cells were arranged in sheets, ill-defined fascicles and nests and displayed a moderate grade of cellular and nuclear pleomorphism and mitoses with abundant pigment in the cytoplasm [Figure 2]b. The nuclear features were better appreciated after melanin bleach [Figure 2]c. The pigment was positively stained with Masson Fontana [Figure 2]d. The tumor cells were positive for HMB-45 [Figure 3]a, and for S-100 [Figure 3]b.
Figure 2: (a) Light microscopy of the tumor: Low power demonstrating tumor cells displaying solid growth pattern, H and E, ×100. (b) High power displaying fascicular arrangement of tumor cells with abundant cytoplasmic melanin, H and E, ×200. (c) Tumor cells after melanin bleach, ×200. (d) Masson Fontana positivity for melanin in the tumor cells, ×200

Click here to view
Figure 3: HMB-45 immunopositivity of the tumor cells, ×200 (a). S100 immunopositivity of the tumor cells, ×200 (b)

Click here to view



 » Discussion Top


Primary melanocytic neoplasms of the CNS are uncommon. They can be diffuse or circumscribed, benign or malignant. Diffuse lesions such as melanocytosis and melanomatosis generally occur in the setting of dermatologic syndromes (neurocutaneous melanosis, nevus of Ota). Localized lesions present as leptomeningeal masses and consist of a spectrum ranging from 'well-differentiated' melanocytomas to lesions of intermediate malignancy and overtly malignant melanomas. According to the Hayward classification, [6] the diagnosis of primary spinal cord melanoma is based on the absence of melanoma outside the CNS, the absence of this lesion at other sites in the CNS, and histologic confirmation of melanoma. According to these classification criteria, our patient had a primary malignant melanoma of the spinal cord. This lesion occurs most often at middle age and can affect both genders equally. [1],[2],[3],[4],[5] The presenting symptoms of this lesion are predominantly those of spinal cord compression. [5],[7]

Radiological studies may not be specific, especially in the absence of primary disease. MRI may offer some indication of a melanotic lesion, but the rarity of a primary melanotic lesion in the CNS most often precludes the preoperative radiological diagnosis of this lesion. According to most descriptions, the MR imaging pattern of spinal melanoma includes signal hyperintensity on T1-weighted images and signal isointensity or hypointensity on T2-weighted images. [1],[8] Pattern of melanoma on MRI depends on content of melanin, as well as acute or chronic intratumoral hemorrhages and fat deposits. [8]

Pathological examination and immunohistochemistry of the primary spinal melanoma are critical to diagnosis and distinguish it from other different nervous system tumors undergoing melanization, such as meningioma, schwannoma, medulloblastoma and gliomas. [8] There is little evidence to support the existence of true melanotic meningioma, although rare melanotic colonization of meningiomas has been reported. From a combined pathological and clinical point of view, the principle differential is between melanocytoma and melanoma. [8] Melanomas are more pleomorphic, have more anaplastic nuclei than melanocytoma and often demonstrate tissue invasion and necrosis. [8] It is obviously important to determine if the melanoma is primary or secondary. Absolute determination is only approached by a thorough examination at autopsy, including sectioning of the eyes. Even then, the possibility of previous regression of a primary site cannot be eliminated entirely.

In the present case, the cytological features were quite typical of malignant melanoma. Immunoreactivity for the HMB-45 melanoma antigen and S-100 further supports the diagnosis. HMB-45 is a useful marker for melanocytic differentiation in a neoplasm, because it indicates active melanosome formation. [9] S-100 is expressed by cells with melanocytic differentiation but is also found in other neuroectodermal cell types. As anticipated, GAFP was nonreactive.

Malignant melanomas are considered to be highly aggressive and radio-resistant tumors with poor prognosis. Beresford reported only 1 of 37 patients with intracranial melanoma responding to radiation therapy. [10] It has been suggested that primary spinal melanoma exhibits slow progression, and the tumor is less aggressive than the more common melanoma of the skin with metastases to the CNS. Metastatic melanomas grow rapidly and usually lead to fatal outcome in less than 6 months.

 
 » References Top

1.Nishihara M, Sasayama T, Kondoh T, Tanaka K, Kohmura E, Kudo H. Long-term survival after surgical resection of primary spinal malignant melanoma. Neurol Med Chir 2009;49:546-8.  Back to cited text no. 1
    
2.Farrokh D, Fransen P, Faverly D. MR findings of a primary intramedullary malignant melanoma: Case report and literature review. AJNR Am J Neuroradiol 1866;22:1864-6.  Back to cited text no. 2
    
3.Francois P, Lioret E, Jan M. Primary spinal melanoma: Case report. Br J Neurosurg 1998;12:179-82.  Back to cited text no. 3
    
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 1988;89:659-66.  Back to cited text no. 4
    
5.Yamasaki T, Kikuchi H, Yamashita J, Asato R, Fujita M. Primary spinal intramedullary malignant melanoma: Case report. Neurosurgery 1989;25:117-21.  Back to cited text no. 5
[PUBMED]    
6.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. 6
[PUBMED]  [FULLTEXT]  
7.Salame K, Merimsky O, Yosipov J, Reider-Groswasser I, Chaitchik S, Ouaknine GE. Primary intramedullaryspinal melanoma: Diagnostic and treatment problems. J Neurooncol 1998;36:79-83.  Back to cited text no. 7
[PUBMED]  [FULLTEXT]  
8.Brat DJ. Perry A Melanocytic lesions. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. WHO Classification of Tumours of the Central Nervous System. 4th ed. Lyon: IARC Press; 2007. p. 181-3.  Back to cited text no. 8
    
9.Skelton HG 3rd, Smith KJ, Barrett TL, Lupton GP, Graham JH. HMB-45 staining in benign and malignant melanocytic lesions: A reflection of cellular activation. Am J Dermatopathol 1991;13:543-50.  Back to cited text no. 9
[PUBMED]    
10.Beresford HR. Melanoma of the nervous system. Treatment with corticosteroids and radiation. Neurology 1969;19:59-65.  Back to cited text no. 10
[PUBMED]    


    Figures

  [Figure 1], [Figure 2], [Figure 3]

This article has been cited by
1 Primary spinal melanoma of extramedullary origin: a report of three cases and systematic review of the literature
Q-Y Liu,A-M Liu,H-G Li,Y-B Guan
Spinal Cord Series and Cases. 2015; 2: 15003
[Pubmed] | [DOI]
2 Primary extramedullary spinal melanoma mimicking spinal meningioma: A case report and literature review
YU-PING LI,HENG-ZHU ZHANG,LEI SHE,XIAO-DONG WANG,LUN DONG,ENXI XU,XING-DONG WANG
Oncology Letters. 2014; 8(1): 339
[Pubmed] | [DOI]
3 Easily misdiagnosed delayed metastatic intraspinal extradural melanoma of the lumbar spine: A case report and review of the literature
LIN SUN,YUEMING SONG,QUAN GONG
Oncology Letters. 2013; 5(6): 1799
[Pubmed] | [DOI]
4 Easily misdiagnosed delayed metastatic intraspinal extradural melanoma of the lumbar spine: A case report and review of the literature
Sun, L. and Song, Y. and Gong, Q.
Oncology Letters. 2013; 5(6): 1799-1802
[Pubmed]
5 Primary melanoma of the cervical spine with cerebral metastases: Case report and review of the literature
Yu, J. and Zhao, D.-D. and Chen, S. and Zhang, J.-M. and Xu, J.
Journal of International Medical Research. 2012; 40(3): 1207-1215
[Pubmed]
6 Primary Melanoma of the Cervical Spine with Cerebral Metastases: Case Report and Review of the Literature
J Yu,D-D Zhao,S Chen,J-M Zhang,J Xu
Journal of International Medical Research. 2012; 40(3): 1207
[Pubmed] | [DOI]



 

Top
Print this article  Email this article
Previous article Next article
Online since 20th March '04
Published by Wolters Kluwer - Medknow