|Year : 2018 | Volume
| Issue : 3 | Page : 125-128
Primary cervical spine melanoma
Chih-Ming Hu1, Cheng-Neng Chen2, Kuo-Hua Hung1, Ming-Shiang Yang3, Kuan-Ju Lai4
1 Department of Neurology, Mac Kay Memorial Hospital, Taitung, Taiwan
2 Department of Neurosurgery, Mac Kay Memorial Hospital, Taitung, Taiwan
3 Department of Radiology, Mac Kay Memorial Hospital, Taitung, Taiwan
4 Department of Pathology, Mac Kay Memorial Hospital, Taitung, Taiwan
|Date of Submission||09-May-2017|
|Date of Decision||10-Jul-2017|
|Date of Acceptance||24-Oct-2017|
|Date of Web Publication||21-Jun-2018|
Dr. Cheng-Neng Chen
Department of Neurosurgery, Mac Kay Memorial Hospital, Taitung
Source of Support: None, Conflict of Interest: None
Primary spinal melanoma is too rare to have any systemic statistical data on the epidemiology of this disease. We report a 58-year-old woman presented with spastic gait for 10 days, preceded by chronic pain at her left anterior chest that progressively spread to her neck, left shoulder, and arm in half a year. Cervical magnetic resonance imaging (MRI) displayed a well-enhancing focal intraspinal extramedullary tumor at C6/7 level. After surgical resection of the tumor and thorough studies to exclude extraspinal melanoma, the primary spinal melanoma is diagnosed. Postoperative MRI did not disclose any evidence of recurrence at 4 and 21 months later. We present the clinical, radiological, and pathological features of a patient with a primary cervical extramedullary spinal melanoma.
Keywords: Intradural extramedullary, primary melanoma, prognosis, spinal cord tumor
|How to cite this article:|
Hu CM, Chen CN, Hung KH, Yang MS, Lai KJ. Primary cervical spine melanoma. Formos J Surg 2018;51:125-8
| Introduction|| |
Primary spinal melanoma is a rare disease which accounts for 1% of total melanoma cases. Its clinical symptoms are not specific, and progressive myelopathic symptoms are the most common features, which may exist for months before a diagnosis is made. Radiologic studies must be performed, and magnetic resonance imaging (MRI) is the method of choice. As primary spinal melanoma usually develops as an intradural extramedullary tumor, the differential diagnoses usually included meningioma, schwannomas, neurofibroma or melanocytoma. The MRI finding among these neoplasms may be different, which will be explored in the discussion section but are not distinct enough to make an accurate presurgical diagnosis. Pathology is always needed to verify the diagnosis. In general, the primary spinal malignant melanoma is lack of metastatic potential, and a favorable prognosis is expected after complete resection of the tumor. We report a patient with a primary cervical spinal melanoma who are free from recurrence and myelopathic symptoms after surgery for 21 months.
| Case Report|| |
A 58-year-old female, not having any systemic disease or a family history of cancer, presented with left anterior chest pain for the past 6 months. Initially, she visited a pulmonologist and a cardiologist of this hospital, but normal findings were disclosed by chest plain film, echocardiography, and treadmill exercise test. Nevertheless, progressive neck pain happened, which radiated toward her left shoulder and arm, puzzling her a lot. She then visited a local clinic of orthopedist and was diagnosed having cervical spondylosis in addition to myofascial pain syndrome. Despite treating with medication and physiotherapy, her symptoms did not subside.
Gradually, she suffered from progressive numbness of bilateral lower limbs, trunk, and extensor aspects of her arms, but she still paid no attention to it. Ten days before calling at neurologist outpatient department of this hospital, she got acute-onset gait disturbance with much stiffness of her lower limbs. She became hardly able to do household chore or even to ride a motorcycle. Neurologic examination revealed positive Spurling's sign and hypoesthesia at the C6 dermatome on her left side, decreased pinprick sense with sensory level below T3 dermatome, and spastic paraparesis of grade 4 muscle strength. Furthermore, deep tendon reflexes of her lower limbs were brisk and Hoffman's sign was positive bilaterally. These findings indicate a lower cervical radiculopathy with superimposed myelopathy.
MRI [Figure 1] displayed a focal intradural, extramedullary tumor growth around C6/7 level with hyperintensity on T1-weighted images and hypointensity on T2-weighted images, with postcontrast enhancement. Focal long T2 foci could be defined within the cervical cord, and there was some degree of swelling, suggestive of acute myelopathy. A meningioma or a neurogenic tumor was considered initially.
|Figure 1: Preoperative magnetic resonance images of the cervical area showing characteristics of an intradural and extramedullary spinal mass at C6/7 level (a) T1-weighted magnetic resonance images showing the hyperintensity signal lesion. (b) T2-weighted images showing the hypointensity lesion. (c and d) T1-weighted with contrast medium showing homogeneous enhancement of the lesion at intradural extramedullary tumor compressing spinal cord|
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We performed a surgery through a midline incision from suboccipital region to the spinous process of T1. The paraspinal muscle was dissected and separated with self-retaining retractors. After the appropriate levels were identified, total laminectomies from C6 to C7 and partial T1 laminectomy were made thoroughly, revealing bulging of the thecal sac. The thecal sac was opened, and the tumor was about 2.76 cm × 1.32 cm × 1.6 cm in size, brown in color, fragile inconsistency, and lack of hypervascularity. The tumor was carefully dissected and was then removed with cautery under microscope.
Pathological examination of the tissue shows compact proliferation of epithelioid and spindle cells with prominent deposition of melanin pigments. The tumor cells have abundant eosinophilic cytoplasm, prominent vesicular nuclei, and large nucleoli in addition to mitotic figures. Spinal melanoma is the pathological diagnosis [Figure 2].
|Figure 2: Hematoxylin and eosin staining shows a highly cellular malignant tumor with dense deposition of melanin granules (a) x100 (b) x200 (c) x400|
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After complete removal of the tumor, the patient underwent several studies including brain MRI, chest, and abdominal computed tomography with contrast, and whole-body fluorodeoxyglucose-positron emission tomography. Other foci of melanoma were excluded, and the diagnosis of primary intradural melanoma was finally made.
Progressive improvement of gait strength and extremity paresthesia has been found postoperatively. She regularly visited neurosurgery outpatient service and followed up MRI scan of her cervical spine [Figure 3] did not display disease recurrence at 4 and 21 months after the operation. She becomes symptomatically free.
|Figure 3: Postoperative (a) T1- and (b) T2-weighted magnetic resonance imaging scan|
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| Discussion|| |
According to Hayward, three criteria are required to diagnose a primary central nervous system (CNS) melanoma: (a) absence of melanoma outside of the CNS, (b) absence of this lesion in another areas of CNS, and (c) histologic confirmation of melanoma. We reported a patient with primary cervical spinal melanoma, located in intradural extramedullary space, who met the criteria. She had nonspecific chest and back pain for half a year before the onset of myelopathic signs and symptoms. The symptoms have subsided, and no recurrence has been found after surgical excision.
No specific symptoms or signs are established for diagnosing primary spinal melanoma. Primary spinal melanoma is less to be located in the cervical segments if comparing with other segment. Patients may have chronic anterior chest pain, upper back pain, or radiation pain and are always attributed to angina pectoris, myalgia, or spondylosis. It may take weeks or months to develop progressive myelopathic symptoms with paresthesia, hemiparesis, or spasticity. One study found the mean symptom duration was 15 months, ranging from 0.3 to 96 months. In the present case, she had chronic anterior chest pain and back pain for half of a year and neurologic deficits appeared 10 days before the diagnosis.
There is a research using the term of “cervical angina” to describe chest pain induced by cervical radiculopathy or myelopathy. They found that compression of the C4–C8 nerve roots or cervical myelopathy would produce the sensation of anginal pain. The majority of cervical angina is attributed to cervical root compression, which may be related to irritating lesion on roots that innervated the anterior chest wall through the medial and lateral pectoral nerves. In cervical myelopathy, the pain may be secondary to lesions of the dorsal horns or disruption of the ascending cardiac spinothalamic tracts. For patients with chest pain and normal studies of respiratory and cardiovascular systems, we should evaluate for possible cervical spinal problems.
Spinal MRI is the radiological tool of choice for diagnosing a spinal tumor. A spinal melanoma displays hyperintensity on T1-weighted images and iso- or hypo-intensity on T2-weighted images, which presentation depended on the paramagnetic properties of melanin, fat deposits, or the hemorrhagic elements in the tumor. However, these findings are not specific for primary melanomas. Meningioma, melanotic schwannomas, and neurofibroma containing melanotic pigment may also show hyperintensity lesions in T1-weighted images. Reviewing literature, a document stated spinal melanoma mimicking spinal meningioma was found. It attributed hyperintensity signal on T1-weighted images to atypical meningioma with intratumoral bleeding preoperatively. Indeed, an accurate diagnosis relies on histological and immunohistochemical analyses of the excised tissue, which is essential for selecting appropriate therapy and predicting patients' outcomes.
Although surgical resection is traditionally the standard treatment for primary spinal cord melanoma, most experts recommended a complete surgical resection as the only effective treatment for a curative outcome. For those patients who have incompletely resected tumor, chemotherapy or radiotherapy may be combined as a complementary treatment. However, the efficacies of radio- and chemotherapies remain controversial. A meta-analysis study showed that chemo- and biological therapies could reduce recurrence rate, but they increase the 5-year survival by only 3%.
The confirmation of spinal melanoma is based only on the pathologic study. There are several methods of staining tumor cells including immunohistochemical (e.g., HMB-45 and S-100) markers and histopathological examination (e.g., hematoxylin and eosin stain). In our case, the pathology shows a highly cellular malignant tumor with dense deposition of melanin granules by hematoxylin and eosin stain which indicated malignant melanoma.
There is no potent statistical data to verify the recurrent rate or prognosis of the disease. Most of the information is collected through case studies. Documents show variations in the mean survival times.,, According to the studies reported by Kim et al., and Liu et al., patients who received total excision of primary cervical melanoma seem to have potentially favorable prognosis., These may be explained by the absence of lymphatic system and presence of blood–brain barrier of spines. These restrict the tumor from lymphatic or hematogenous spreading.
| Conclusion|| |
Primary spinal melanoma is a very rare disease, and spinal MRI is an important tool for preoperative diagnosis. This neoplasm should be considered in patients with intradural extramedullary tumors with the following characteristics: hyperintensity onT1W signals, Iso- or hypo-intensity on T2W signal, and varying contrast enhancements. The pathological examination is essential for diagnosis, and the neoplasm should be differentiated from metastatic melanoma through serial studies to exclude other foci of melanoma. The potential outcome is favorable if the tumor is totally removed.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that her names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]