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Table of Contents    
LETTERS TO EDITOR
Year : 2019  |  Volume : 67  |  Issue : 2  |  Page : 591-595

Conus melanocytoma: A rare spinal tumor


1 Department of Neurosurgery, Postgraduate Institute of Medical Education and Research and Dr. Ram Manohar Lohia Hospital, New Delhi, India
2 Department of Pathology, Postgraduate Institute of Medical Education and Research and Dr. Ram Manohar Lohia Hospital, New Delhi, India

Date of Web Publication13-May-2019

Correspondence Address:
Dr. Vikas Sharma
Department of Neurosurgery, PGIMER and Dr. Ram Manohar Lohia Hospital, New Delhi - 110 001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.258008

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How to cite this article:
Sharma V, Bhaskar S, Kumar A, Bhardwaj M. Conus melanocytoma: A rare spinal tumor. Neurol India 2019;67:591-5

How to cite this URL:
Sharma V, Bhaskar S, Kumar A, Bhardwaj M. Conus melanocytoma: A rare spinal tumor. Neurol India [serial online] 2019 [cited 2019 May 24];67:591-5. Available from: http://www.neurologyindia.com/text.asp?2019/67/2/591/258008




Sir,

Melanocytoma is a rare tumor that arises from the leptomeninges, with an annual incidence of 1 per 10 million population. It accounts for less than 0.1% of all central nervous system (CNS) tumors.[1] The first description of this tumor was given by Limas and Tio in 1972.[2] It usually has an intracranial localization but may also involve the spinal column, where it is most often intradural and extramedullary. Intramedullary melanocytomas, such as the one we describe here, are extremely rare.

The histological differential diagnosis includes a melanotic schwannoma, malignant melanoma, melanotic meningioma, and melanocytosis. Intramedullary spinal melanocytoma is a rare entity. We present a case of a primary spinal melanocytoma in the conus region, and describe its symptoms, radiological features, histological findings, treatment, along with the current management options and literature review. Till now, 30 cases have been reported in the English literature. The details of the previously reported cases have been summarized in [Table 1].
Table 1: Previous published cases

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A 26-year old female patient presented with a history of low-back pain for 3 years, radicular pain to the left buttock and posterior aspect of the left thigh for 2 years, and weakness in the left lower limb (LL) for 1 year. Systemic examination was within normal limits. There were no neurocutaneous markers. On motor system examination, bilateral upper limbs were normal, and there was hypertonia in both LLs with exaggerated knee and ankle reflexes and upgoing plantars. The power in the right and left LLs was 4+/5 and 3/5, respectively. The abdominal reflex was present in the upper quadrant and was absent in the lower quadrant. Sensory loss was present for all modalities in the left LL below the L3 dermatome. Bowel and bladder functions were normal.

CEMRI (contrast enhanced magnetic resonance imaging) of the dorso-lumbar spine showed a relatively well-defined soft tissue mass lesion of size 7.4 × 1.4 cm, involving the distal dorsal spine and conus medullaris at the level of T10–L1 vertebrae. It was iso- to hyperintense on T1-weighted (T1W) images and iso- to hypointense on T2W images with a mild heterogenous contrast enhancement. An area of cerebrospinal fluid (CSF) intensity was present proximal to the lesion that showed a mild peripheral enhancement. These findings were suggestive of an intramedullary lesion at the conus [Figure 1]. A T9–T12 laminotomy was done and a black-colored lesion arising from the conus, with the nerve roots of cauda equina entangled in it, was seen. The tumor was firm and was highly vascular. At some places, the tumor was adherent to the cauda equina nerve roots. Near-total excision of the tumor was done [Figure 2].
Figure 1: Magnetic resonance imaging of the dorso-lumbar spine: (a) Sagittal T1 weighted image shows an iso- to hyperintense, well-defined, soft tissue mass lesion of size 7.4×1.4 cm, involving the distal dorsal spine and conus medullaris at the level of D10-L1, (b) T2 weighted sagittal image shows the iso- to hypointense lesion, (c) T1 sagittal image with contrast shows a mild heterogenous enhancement, (d) T2 axial image shows the tumor displacing the cord

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Figure 2: Intraoperative picture showing durotomy with black pigmented tumor

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The macroscopic examination of the specimen was suggestive of a pigmented tumor. Histologic analysis revealed a highly cellular tumor comprising spindle to ovoid cells arranged in fascicles, sheets, and nests. Most of the cells were heavily pigmented with dark brown, coarse, granular pigment, obscuring the nuclear details. No mitosis was noted and occasional foci of necrosis were seen. Focal glial infiltration was noted [Figure 3], [Figure 4], [Figure 5]. Immunohistochemistry (IHC) analysis was positive for HMB-45 (human melanoma black-45) as well as S-100, and was negative for epithelial membrane antigen (EMA) and glial fibrillary acid protein (GFAP). The MIB-1 index was <1%. These features were suggestive of an intermediate grade melanocytoma.
Figure 3: Microscopic section showing sheets and fascicles of pigmented tumor cells (H and E; ×4)

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Figure 4: Microscopic section showing pigment obscuring the cellular details. (H and E; ×10)

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Figure 5: Immunohistochemical analysis revealing positive expression by HMB-45 and S-100

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The patient had an increase in her paraparesis in the immediate postoperative period (right LL 2/5, left LL 0/5) with bladder involvement. She made a gradual recovery over 6 months. The patient received 50 Gy dose of radiotherapy in 25 fractions as an adjuvant therapy.

Postoperative MR imaging showed postoperative changes in the posterior elements from T9 to T10 levels. Hyperintensity in the dorsal cord and in the region of caudae quina at the region of T11 vertebra with a syrinx formation proximal to it were also seen. There was no evidence of enhancement seen in the operative bed [Figure 6].
Figure 6: Post operative magnetic resonance imaging scan of the dorso-lumbar spine-(a) T1 weighted sagittal image shows postoperative changes in the posterior elements from D9 to D10 levels, (b)T1 weighted contrast image shows no enhancement in the operated bed , (c and d) T2 weighted image with short TI inversion recovery sequence shows hyperintensity in the dorsal cord and in the region of cauda equina at the level of D11 vertebra with syrinx formation proximal to it

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The patient's power improved to 4/5 in the right LL and between 0/5 to 2/5 in the left LL (hip movements 2/5, knee flexion 3/5, knee extension 0/5, ankle movements 0/5, and toe movements 2/5) at a 1-year follow-up. Her bowel and bladder functions remained normal. She had a persistent and minimal residual sensory impairment.

The leptomeningeal melanocytes are derived from the neural crest and are found in highest density underneath the medulla and along the cervical spinal cord.[10] Primary melanocytic neoplasms of the central nervous system (CNS) have been divided by World Health Organization into (1) diffuse melanocytosis and melanomatosis, (2) melanocytoma, and (3) malignant melanoma.[11] The age of the patients harboring these tumors lies in the fourth to fifth decade, with their incidence being more in female as compared to male patients.[12]

The gross appearance of a meningeal melanocytoma, as seen during surgery or at autopsy, is that of a well-encapsulated, nodular, dark brown or black lesion that is firmly attached to the underlying leptomeninges. These tumors have a positive immunohistochemical reaction to antimelanosomal antibody (HMB45), S-100 protein, and vimentin antibodies and a negative reaction to EMA, GFAP, cytokeratins.[13],[14] Primary melanomas have more Ki-67 labeling indices (an average of 8%) than melanocytomas (<1–2%).[15] The other differential diagnosis are melanocytic meningioma and melanocytic schwannoma [Table 2].[16]
Table 2: Immunohistochemical features of pigmented tumors of the meninges

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On MR imaging, these tumors are isointense or hyperintense onT1W images and hypointense on T2W images and show a homogeneous enhancement on postcontrast images.[17]

The goal of treatment should be complete/gross total resection with preservation of neurological function. Radiotherapy is provided when resection is incomplete.[18] Local recurrences up to 12 years after surgery have been reported.[19] In case of local recurrence, resurgery can be attempted, provided the neurological function can be preserved. The other alternative is radiotherapy.

Rades and Schild[20] in their study concluded that complete tumor removal is superior to incomplete tumor removal with regard to local control and survival. In case of incomplete tumor removal, the outcome is improved by radiotherapy.

Gupta et al., have concluded that very little data is present regarding radiosurgery, and chemotherapy has no role.[13] The optimum treatment at present includes a safe surgical excision and adjuvant therapy. The high incidence of local recurrence in incompletely excised lesions mandates a long-term close follow-up.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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Park SH, Park HR, Ko Y. Spinal meningeal melanocytoma. J Korean Med Sci 1992;7:364-8.  Back to cited text no. 1
    
2.
Limas C, Tio FO. Meningeal melanocytoma (“melanotic menigioma). Its melanocytic origin as revealed by electron microscopy. Cancer 1972;30:1286-94.  Back to cited text no. 2
    
3.
Wagner F, Berezowska S, Wiest R, Gralla J, Beck J, Verma RK, et al. Primary intramedullary melanocytoma in the cervical spinal cord: Case report and literature review. Radiol Case Rep 2015;10:1010-8.  Back to cited text no. 3
    
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Merciadri P, Secci F, Sbaffi PF, Zona G. Multifocal meningeal melanocytoma of the conus medullaris. Acta Neurochir 2011;153:2283-5.  Back to cited text no. 5
    
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Trinh V, Medina Flores R, Taylor CL, Yonas H, Chohan MO. Primary melanocytic tumors of the central nervous system: Report of two cases and review of literature. Surg Neurol Int2014;5:147-53.  Back to cited text no. 7
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Dorwal P, Mohapatra I, Gautam D, Gupta A. Intramedullary melanocytoma of thoracic spine: A rare case report. Asian J Neurosurg 2014;9:36-9.  Back to cited text no. 8
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Ruggeri A, Mancarella C, Dugoni D, Landi A, Delfini R. An extremely rare case of thoracic intramedullary melanocytoma: Case report and review of literature. Brain Spinal Cancer 2014;1:1-6.  Back to cited text no. 9
    
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Goldgeier MH, Klein LE, Klein-Angerer S, Moellmann G, Nordlund JJ. The distribution of melanocytes in the leptomeninges of the human brain. J Invest Dermatol 1984;82:235-8.  Back to cited text no. 10
    
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Gupta PK, Misra S, Verma R, Soni N, Lamin JC, Mishra RK, et al. Primary intradural cervical spine melanocytoma: A rare tumor and review of literature. Neurol India 2017;65:653-7.  Back to cited text no. 13
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Halder A, Bera S, Datta C, Chatterjee U, Choudhuri MK. Squash cytopathology of primary meningeal melanocytoma. Neurol India 2016;64:152-4.  Back to cited text no. 14
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Brat DJ, Giannini C, Scheithauer BW, Burger PC. Primary melanocytic neoplasms of the central nervous systems. Am J SurgPathol1999;23:745-54.  Back to cited text no. 15
    
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Ruelle A, Tunesi G, Andrioli G. Spinal meningeal melanocytoma: Case report and analysis of diagnostic criteria. Neurosurg Rev1996;19:39-42.  Back to cited text no. 16
    
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Offiah CJ, Laitt RD. Case report: Intracranial meningeal melanocytoma: A cause of high signal on T1- and low signal on T2-weighted MRI. Clin Radiol2006;61:294-8.  Back to cited text no. 17
    
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Glick R, Baker C, Husain S, Hays A, Hibshoosh H. Primary melanocytomas of the spinal cord: A report of seven cases. Clin Neuropathol1997;16:127-32.  Back to cited text no. 18
    
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Jaiswal S, Vij M, Tungria A, Jaiswal AK, Srivastava AK, Behari S. Primary melanocytic tumors of the central nervous system: A neuroradiological and clinicopathological study of five cases and brief review of literature. Neurol India 2011;59:413-9.  Back to cited text no. 19
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20.
Dirk Rades, Steven E, Schild, Marcos Tatagiba, Hugo A, Molina, et al. Therapy of meningeal melanocytomas. Cancer 2004;100:2442-7.  Back to cited text no. 20
    


    Figures

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

  [Table 1], [Table 2]



 

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