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Year : 2015  |  Volume : 11  |  Issue : 4  |  Page : 1027

Primary spinal melanoma treated with adjuvant radiotherapy and concurrent temozolomide: A case report and review of literature

Department of Radiation Oncology, Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication15-Feb-2016

Correspondence Address:
Soumyajit Roy
Department of Radiation Oncology, Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.151416

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 > Abstract 

Primary intradural extramedullary melanoma of spinal cord is an extremely rare tumor.It accounts for approximately 1% of the total melanoma cases.We report a case of primary intradural extramedullary spinal melanoma in a 28-year-old gentleman who was managed with surgery followed by postoperative radiation with concurrent temozolomide followed by adjuvant temozolomide. The patient had a disease-free survival of 24 months, after which he developed marginal recurrence. To the best of our knowledge, this is the first case of a primary spinal cord melanoma. (PSCM) treated with postoperative radiotherapy with concurrent temozolomide followed by adjuvant Temozolomide.

Keywords: Adjuvant, primary spinal cord melanoma, radiation, surgery, temozolomide

How to cite this article:
Mallick S, Roy S, Joshi NP, Julka PK. Primary spinal melanoma treated with adjuvant radiotherapy and concurrent temozolomide: A case report and review of literature. J Can Res Ther 2015;11:1027

How to cite this URL:
Mallick S, Roy S, Joshi NP, Julka PK. Primary spinal melanoma treated with adjuvant radiotherapy and concurrent temozolomide: A case report and review of literature. J Can Res Ther [serial online] 2015 [cited 2020 Jan 29];11:1027. Available from: http://www.cancerjournal.net/text.asp?2015/11/4/1027/151416

 > Introduction Top

Primary intradural extramedullary melanoma of spinal cord is an extremely rare tumor accounting for approximately 1% of the total melanoma cases.[1] Primary spinal cord melanoma (PSCM) may arise from melanoblasts within the arachnoid matter, or from pial sheaths of vascular bundles, or from neuroectodermal congenital rests.[1] According to the Hayward classification,[2] the diagnosis of PSCM is based on an absence of melanoma outside the central nervous system (CNS) or an absence of this lesion in other sites in the CNS, and histologic confirmation. The clinical signs and symptoms of PSCM are often nonspecific. Patients usually present with back pain and signs or symptoms of spinal cord compression (such as hemiparesis or sensation impairment). Some patients present with increased intracranial pressure caused by tumor obstruction of the cerebrospinal drainage. Magnetic resonance imaging (MRI) has been the imaging of choice for these malignancies. Complete surgical excision remains the cornerstone of management. However as a result of its rarity, the role of adjuvant treatment modalities in the management of PSCM remains undefined. We describe the clinical and treatment details of a patient of PSCM who underwent combined modality management at our institute. The role of adjuvant treatment in management of such tumor has also been highlighted.

 > Case Report Top

A 28-year-old gentleman presented to our institute with complaints of progressive weakness of both his lower limbs for 4 months along with urinary hesitancy. Clinical examination revealed a power of 4/5 (medical research council grade) bilaterally; tone and planter reflex were noted to be normal. During evaluation, presence of an intradural extramedullary spinal mass in the lower thoracic region was noted in the contrast-enhanced MRI (CEMRI) of the spine. It was extending from the upper border of the eighth dorsal (T8) vertebra to the lower border of the ninth dorsal vertebra (T9). It showed mild hyperintensity on T1-weighted images and hypointensity on T2-weighted images along with moderate post-contrast enhancement [Figure 1]. A provisional diagnosis of a meningioma or a neurogenic tumor was suggested. The patient underwent a D7–D9 laminectomy and durotomy with excision of the mass. The postoperative histopathology revealed a cellular tumor composed of cells with eosinophilic cytoplasm and prominent nucleoli. Mitotic activity was brisk and there were foci of necrosis [Figure 2]. Possibilities considered were epithelioid-schwannoma, amelanotic melanoma, non-Hodgkins lymphoma, and metastatic carcinoma. The tumor cells were immunopositive for S-100 and human melanoma black (HMB)-45, and did not show any immunoreactivity for cytokeratin (CK) and leukocyte common antigen (LCA).
Figure 1: Contrast-enhanced magnetic resonance imaging (CEMRI) of the spine, sagittal sections, showing mild hyperintensity on T1-weighted images (a) and in the lower thoracic region extending from D7 to D9 vertebral level. The lesion is showing moderate enhancement in post-contrast fat saturation sequence (b)

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Figure 2: Photomicrograph at ×400 (a) showing the histopathological finding of cellular tumor composed of cells with eosinophilic cytoplasm and prominent nucleoli. Mitotic activity was brisk and there were foci of necrosis (hematoxylin and eosin (H and E) stain). Photomicrograph at ×400 (b) showing positive staining for human melanoma black (HMB)-45

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Hence, we reached a final histopathological diagnosis of melanoma. Dermatological and ophthalmological examination for a possible primary melanoma was unrevealing. Hence, a final diagnosis of primary intradural exrtramedullary melanoma of the spinal cord (PSCM) was established. Adjuvant therapy was planned, which consisted of local irradiation with concurrent temozolomide (75mg/m 2 body surface area (BSA) daily) followed by six cycles of adjuvant temozolomide (150mg/m 2; day 1–5; every 4 weeks). Radiation therapy was planned by three-dimensional conformal radiation delivery technique. The planning computed tomography (CT) was done in the prone position with a customized thermoplastic immobilization device. The clinical target volume (CTV) encompassed the postoperative area along with a 1 cm cranio-caudal margin. The CTV was expanded with a uniform margin of 5 mm to generate the planning target volume (PTV). Radiation was delivered using two posterior oblique fields. The dose was prescribed to the 92% isodose line to achieve optimum coverage. The patient was treated to a total dose of 30 Gy in 10 fractions over 2 weeks.

The patient was followed-up with serial imaging at 3monthly intervals; and at 2 years after completion of radiation, he was diagnosed with recurrence at D10 vertebral level. An 18-fluoro-deoxyglucose positron emission tomography (FDG-PET) scan of whole body did not show any metastatic foci. He was advised for re-excision, but subsequently he defaulted and lost to follow-up.

 > Discussion Top

Primary spinal melanoma presents most commonly as solitary pigmented lesions. It occurs commonly in the 5th decade of life, with an age range of 20–80 years. Male and female have equal preponderance. It occurs most frequently in the thoracic segment (42.3%), followed by its occurrence in the cervical (34.6%), thoracolumbar (11.5%), cervicothoracic (7.7%), and the lumbar (3.8%) segments.[3]

MRI has been the imaging of choice for these malignancies. The MRI findings of melanomas vary considerably, depending upon the number of melanin-containing cells and the presence or absence of hemorrhages.[3] The different MRI pattern of spinal cord melanoma includes signal hyperintensity on T1-weighted images and signal iso- or hypointensity on T2-weighted images. On intravenous contrast administration, the lesion shows moderate enhancement. But it is impossible to differentiate melanoma from other melanin-containing lesions of spinal cord such as meningeal melanocytoma, pigmented meningioma, or schwannoma.[1]

Complete surgical excision is the optimum treatment for PSCM. Melanoma is considered a relatively radioresistant tumor. However, some studies have shown that patients with spinal cord compression may achieve shrinkage of the tumor with radiation therapy. Hence, radiotherapy is reserved for palliation of symptoms due to local tumor growth, as sole decompressive modality or as an adjuvant to laminectomy. However, it can be used as an important adjuvant treatment modality during curative management of PSCM.[4],[5] The spinal cord is a late reacting tissue with a low cell turn over rate. Radiobiologically speaking, the alpha/beta ratio is very low for spinal cord, in the order of 3.5. Hence, concern for late toxicity is more for the spinal cord with hypofractionated radiotherapy. Therefore, we chose a radiation fractionation schedule balancing the advantage of hypofractionation in melanomas with the possible late toxicity. The radiation regime closely resembles the dose given by others.[4],[5] Stereotactic radiosurgery can also be expedited as an alternative option for management of PSCM though further studies are necessary for validation of this approach.[6]

Melanoma has also been refractory to most standard systemic therapy for decades. Single-agent or combination chemotherapy drugs in use at this time are of limited value. The two approved treatments, dacarbazine and interleukin-2, have not demonstrated an impact on overall survival.

Temozolomide and dacarbazine has been found to have almost equivalent efficacy as systemic therapy in advanced melanomas.[7] It has shown to induce clinically relevant responses in patients with cerebral metastases of melanoma.[8],[9] It has excellent bioavailability and can efficiently cross blood-brain barrier. Biotherapy-including interferon-α, interferon-γ, interleukin-2, and lymphokine-activated killer (LAK) cells has been attempted in other melanomas and can be tried in primary CNS melanoma.[10] Currently MD Anderson Cancer Center has started a trial evaluating the role of ipilimumab and temozolomide combination in metastatic melanoma. [Table 1] is showing the available treatment details and outcome of patients of PSCM reported since 1999.
Table 1: Showing treatment details of reported patients of primary spinal cord melanoma treated since 1999

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Our patient showed a disease-free survival of 2 years with use of adjuvant temozolomide and radiation. However, the present dose fractionation schedule which corresponds to 35 Gy at 2 Gy per fraction (biological effective dose) is far less than the spinal cord tolerance of 46 Gy at 2 Gy per fraction. This might be attributed as a potential reason for marginal failure in this patient. With advancements in radiation delivery techniques leading to better sparing of critical organs, higher dose can be recommended for achieving superior disease control in an aggressive malignancy like melanoma.

 > Conclusion Top

Postoperative chemoradiation should be explored in patients with PSCM especially after inadequate dissection. Temozolomide can be an option as adjuvant therapy for managing PCSM. It is less toxic, can be given orally, and thus associated with better compliance. To the best of our knowledge, this is the first case of a PSCM treated with postoperative radiotherapy with concurrent temozolomide followed by adjuvant temozolomide which induced a superior disease-free survival in such aggressive malignancy.[23]

 > References Top

Farrokh D, Fransen P, Faverly D. MR findings of a primary in tramedullary malignant Melanoma: Case report and literature review. AJNR Am J Neuroradiol 2001;22:1864-6.  Back to cited text no. 1
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. 2
Kim MS, Yoon DH, Shin DA. Primary spinal cord melanoma. J Korean Neurosurg Soc 2010;48:157-61.  Back to cited text no. 3
Salame K, Merimsky O, Yosipov J, Reider-Groswasser I, Chaitchik S, Ouaknine GE. Primary intramedullary spinal melanoma: Diagnostic and treatment problems. J Neurooncol 1998;36:79-83.  Back to cited text no. 4
Kanatas AN, Bullock MD, Pal D, Chakrabarty A, Chumas P. Intradural extramedullary primary malignant melanoma radiographically mimicking a neurofibroma. Br J Neurosurg 2007;21:39-40.  Back to cited text no. 5
Benzil DL, Saboori M, Mogilner AY, Rocchio R, Moorthy CR. Safety and efficacy of stereotactic radiosurgery for tumors of the spine. J Neurosurg 2004;101:413-8.  Back to cited text no. 6
Middleton MR, Grob JJ, Aaronson N, Fierlbeck G, Tilgen W, Seiter S, et al. Randomized phase III study of temozolomide versus dacarbazine in the treatment of patients with advanced metastatic malignant melanoma. J Clin Oncol 2000;18:158-66.  Back to cited text no. 7
Quirt I, Verma S, Petrella T, Bak K, Charette M. Temozolomide for the treatment of metastatic melanoma: A systematic review. Oncologist 2007;12:1114-23.  Back to cited text no. 8
Katalinic D, Anic B, Stern-Padovan R, Mayer M, Sentic M, Cikes N, et al. Low back pain as the presenting sign in a patient with primary extradural melanoma of the thoracic spine-A metastatic disease 17 years after complete surgical resection. World J Surg Oncol 2011;9:150.  Back to cited text no. 9
Wöll E, Bedikian A, Legha SS. Uveal melanoma: Natural history and treatment options for metastatic disease. Melanoma Res 1999;9:575-81.  Back to cited text no. 10
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. 11
Bidziński J, Kroh H, Leszczyk C, Bojarski P. Primary intraspinal cervical melanoma. Acta Neurochir (Wien) 2000;142:1069-70.  Back to cited text no. 12
Kounin GK, Romansky KV, Traykov LD, Shotekov PM, Stoilova DZ. Primary spinal melanoma with bilateral papilledema. Clin Neurol Neurosurg 2005;107:525-7.  Back to cited text no. 13
Jeong DH, Lee CK, You NK, Kim SH, Cho KH. Primary spinal cord melanoma in thoracic spine with leptomeningeal dissemination and presenting hydrocephalus. Brain Tumor Res Treat 2013;1:116-20.  Back to cited text no. 14
Cetinalp NE, Yildirim AE, Divanlioglu D, Belen D. An uncommon intramedullary tumor: Primary spinal cord melanoma. Asian Spine J 2014;8:512-5.  Back to cited text no. 15
Yu J, Zhao DD, Chen S, Zhang JM, Xu J. Primary melanoma of the cervical spine with cerebral metastases: Case report and review of the literature. J Int Med Res 2012;40:1207-15.  Back to cited text no. 16
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. 17
Kolasa M, Jesionek-Kupnicka D, Kordek R, Kolasa P. Primary spinal cord melanoma-a case report. Folia Neuropathol 2010;48:212-6.  Back to cited text no. 18
Lee CH, Moon KY, Chung CK, Kim HJ, Chang KH, Park SH, et al. Primary intradural extramedullary melanoma of the cervical spinal cord: Case report. Spine (Phila Pa 1976). 2010;35:E303-7.  Back to cited text no. 19
Vij M, Jaiswal S, Jaiswal AK, Behari S. Primary spinal melanoma of the cervical leptomeninges: Report of a case with brief review of literature. Neurol India 2010;58:781-3.  Back to cited text no. 20
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Fuld AD, Speck ME, Harris BT, Simmons NE, Corless CL, Tsongalis GJ, et al. Primary melanoma of the spinal cord: A case report, molecular footprint, and review of the literature. J Clin Oncol 2011;29:e499-502.  Back to cited text no. 21
Yan L, Chang Z, Liu Y, He BR, Hao DJ. Primary spinal melanoma: A case report and literature review. Chin Med J (Engl) 2012;125:4138-41.  Back to cited text no. 22
Sinha R, Rizvi TH, Chakraborti S, Ballal CK , Kumar A. Primary melanoma of the spinal cord: A case report. J Clin Diagn Res 2013;7:1148-9.  Back to cited text no. 23


  [Figure 1], [Figure 2]

  [Table 1]


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