Cervical Intradural Extramedullary Melanocytoma , Associated with Nevus of Ito. Recurrence and Long-Term Outcome (original) (raw)
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Intradural extramedullary meningeal melanocytoma: a case report and literature review
Journal of Surgical Case Reports
Primary meningeal melanocytomas are extremely rare, benign tumours arising from the leptomeninges. While they are considered to be benign lesions, there is potential for their growth and transformation into malignant melanomas. They are commonly found in the cervical spine, with a decreased incidence in the thoracic and lumbar regions. We present a case report of a 56-year-old man who presented to our unit with a 4-month history of lower limb weakness and a sensory level at T6. Magnetic resonance imaging shows an intradural extramedullary tumour. The patient underwent a thoracic debulking of the lesion with neurophysiological monitoring. Histopathology confirmed the diagnosis of melanocytoma of meningeal origin, with a low mitotic count. Our patient recovered well post-operatively with no complications. Surgical resection is an effective method to manage this tumour; however, adjuvant radiotherapy is advised due to the risk of recurrence and malignant transformation.
Intramedullary spinal melanocytoma
Rare Tumors, 2010
Meningeal melanocytoma is a benign lesion arising from leptomeningeal melanocytes that at times can mimic its malignant counterpart, melanoma. Lesions of the spine usually occur in extramedullary locations and present with spinal cord compression symptoms. Because most reported spinal cases occur in the thoracic region, these symptoms usually include lower extremity weakness or numbness. The authors present a case of primary intramedullary spinal meningeal melanocytoma presenting with bilateral lower extremity symptoms in which the patient had no known supratentorial primary lesions. Gross total surgical resection allowed for full recovery, but early recurrence of tumor was detected on close follow-up monitoring, allowing for elective local radiation without loss of neurological function. Case reports of such tumors discuss different treatment strategies, but just as important is the close follow-up monitoring in these patients even after gross total surgical resection, since these tumors can recur.
Primary Spinal Melanoma With Intra- and Extradural Extensions: A Rare Case
Cureus, 2021
Primary spinal melanoma (PSM) is a rare primary central nervous system melanoma with limited literature. A 30-year-old male presented with one year of progressive bilateral leg weakness and back pain. Physical examination revealed slightly decreased power and deep tendon reflexes of the lower extremities, decreased sensation at the level of T10, and normal anal sphincter and plantar reflexes. Magnetic resonance imaging (MRI) scan of the thoracolumbar spine revealed a hypointense lesion on T2-weighted and a hyperintense lesion on T1-weighted imaging at the level of T10 with mild extensions. The lesion was causing a mass effect on the spinal cord. The patient underwent laminectomy and near-total excision which showed a black, firm-to-hard, scarcely vascular extradural tumor extending from T10 to T11 that adhered to nerve roots. Histopathological examination and immunostaining with S-100 and Melan-A stains confirmed the diagnosis of malignant melanoma. Other imaging studies like brain computed tomography (CT) and positron emission tomography/computed tomography (PET/CT) scans, and chest X-ray were normal. On follow-up, the patient reported improvement in the power of his lower limbs with intact sensory function and sphincters. The first radiotherapy session was scheduled for six weeks postoperatively. There was no recurrence at a two-year follow-up. The possibility of a melanocytic tumor should be considered for a spinal lesion with paramagnetic properties as early surgical intervention is important for diagnosis and improved survival.
Multifocal spinal meningeal melanocytoma – an illustrated case review
Turkish Neurosurgery, 2011
Primary melanocytic tumors of the central nervous system are rare. In this article the authors describe a case of C1C2 intradural extramedullary melanocytoma in a 43-year-old patient who presented with neck pain. C1-3 laminectomy was performed followed by excision of the lesion and an adjoining satellite nodule, along with the dural attachment. The histopathological features were consistent with a meningeal melanocytoma despite the presence of a satellite nodule. The patient has no evidence of recurrence during the six month follow up period. A brief review of literature pertaining to the radiological features, pathological findings, management and prognosis of this rare tumor is discussed.
Intermediate grade meningeal melanocytoma of cervical spine
Child's Nervous System, 2009
Background Meningeal melanocytoma is a rare, benign melanotic tumor of the leptomeninges, which occurs anywhere in the cranial or spinal regions but most commonly in supratentorial and thoracic spine regions. The literature on this entity consists of case reports; therefore, there is no agreement on the most effective therapy of this tumor, although total excision seems to be the best therapeutic option. Case history We report a 17-year-old girl with intermediate grade meningeal melanocytoma involving the C6 nerve root with spinal cord compression resulted in progressive tetraparesis. Clinical and radiological examinations suggested the possibility of an intradural extramedullary solid mass. The tumor was removed subtotally through cervical laminotomy followed by rapid improvement of most neurological deficits. This tumor was unusual because of its very hyperintense homogenous signal on T1-weighted images, invasion of the arachnoid membrane, and extension into the neural foramina. Black dots on the surface of the cord were thought to represent an organized blood clot until the frozen section suggested a melanocytic tumor. Discussion We discuss the distinction of meningeal melanocytoma from other melanocytic tumors of the leptomeninges. Conclusion Melanocytic tumors should be considered in the differential diagnosis when a hyperintense lesion of the leptomeninges is identified on T1-weighted images or a very dark mass similar to charcoal or organized hematoma is found in the surgical field. The best management is complete tumor resection, but radiotherapy is reserved in cases of subtotal resection and multiple lesions. Locally aggressive nature of tumor and possibility of recurrence warrant regular follow-up.
Surgical Neurology International, 2014
Background: Primary melanocytic tumors of the central nervous system (CNS) represent only 1% of all melanomas. We report two rare cases of primary diffuse leptomeningeal melanomatosis (PDLM; case 1) and primary melanoma of the thoraco-lumbar spine (case 2). Case Description: In case 1, multiple cerebrospinal fluid (CSF) studies and a brain biopsy were non-diagnostic, with a biopsy of the cauda equina eventually demonstrating malignant melanomatosis. Diagnosis of primary spinal cord melanoma was more straightforward in case 2 with imaging and biopsy. Conclusion: PDLM and primary intramedullary spinal melanoma are rare variants of primary CNS melanoma. This report contrasts the diagnostic challenges between the two entities and alerts the neurosurgeon into considering the diagnosis with appropriate clinical presentation.
Primary Intramedullary Spinal Melanoma: A Rare Disease of the Spinal Cord
Cureus, 2021
Primary malignant melanoma of the intramedullary region of the spinal cord has rarely been reported in the literature. These tumors can have variable appearance on magnetic resonance imaging (MRI) due to different extents of melanin and hemorrhage. Histopathologic confirmation and a comprehensive workup to rule out extra-spinal melanoma are required to make definitive diagnosis. We present a case of a patient diagnosed with primary intramedullary spinal melanoma in his lower thoracic spinal cord who was effectively treated with surgical resection, adjuvant radiation, and adjuvant immunotherapy. Gross total resection (GTR) is most vital in the management of this spinal tumor. Although several studies have established the efficacy of immunotherapy agents in advanced malignant melanoma, the use of these agents has not been studied in primary central nervous system melanomas. This case provides insight into the diagnostic approach and treatment options for this unique malignancy.
Spinal meningeal melanocytoma in a 5-year-old child: a case report and review of literature
The Egyptian journal of neurology, psychiatry and neurosurgery, 2018
Meningeal melanocytoma is considered a rare lesion arising from leptomeningeal melanocytes. Nearly two thirds of meningeal melanocytomas were reported in the intracranial compartment and the remaining one third in the spine. Spinal melanocytomas can be extradural or intradural, with extradural variant being more common, and the majority of cases have been single reports. A 5-year-old male presented with a 4-month history of non-radiating low back pain persistent at rest, with otherwise non-remarkable medical history. The patient was neurologically intact with no deficits. Preoperatively, routine laboratory investigations were non-remarkable. MRI imaging was done and showed a lesion at the level of T11 to L4, hyperintense on T1 and hypointense on T2 with homogenous contrast enhancement. Intraoperatively, the lesion was hemorrhagic, brownish, and rubbery in consistency attached to the ventral dura. Microscopic picture revealed dense cytoplasmic brown melanin pigments, with no signific...