Intramedullary Melanocytoma Research Articles

Unsatisfactory Neurological Outcome in an Intramedullary Thoracic Intermediate-Grade Melanocytoma-Systematic Review and Illustrative Case.

Intramedullary melanocytomas are exceedingly rare, with only twenty-four cases reported up to now. They present as local invasive tumors despite their benign biological behavior. Attempting a complete safe resection often results in severe post-operative neurological deficits, as in our case presented here. A systematic review was conducted across the PubMed and Scopus databases including studies published till February 2024. A total of 19 studies were included, encompassing 24 cases. A similar distribution between sexes was noted (M:F 13:11), with ages ranging from 19 to 79 years. The thoracic segment was most affected, and intermediate-grade melanocytoma (19 cases) was the most common histotype. Radiographically, intramedullary melanocytomas usually appear as hyperintense hemorrhagic lesions peripheral to the central canal with focal nodular enhancement. Intraoperatively, they are black-reddish to tan and are tenaciously adherent lesions. In the sampled studies, IONM employment was uncommon, and post-operative new-onset neurological deficits were described in 16 cases. Adjuvant RT was used in four cases and its value is debatable. Recurrence is common (10 cases), and adjuvant therapies (RT or repeated surgery) seem to play a palliative role. A 68-year-old woman presented with a three-year history of worsening spastic paraparesis and loss of independence in daily activities (McCormick grade 4). An MRI revealed an intramedullary tumor from Th5 to Th7, characterized by T1-weighted hyperintensity and signs of recent intralesional hemorrhage. Multimodal neuromonitoring, comprising the D-Wave, guided the resection of a black-tan-colored tumor with hyper-vascularization and strong adherence to the white matter. During final dissection of the lesion to obtain gross total resection (GTR), a steep decline in MEPs and D-Wave signals was recorded. Post-operatively, the patient had severe hypoesthesia with Th9 level and segmental motor deficits, with some improvement during neurorehabilitation. Histopathology revealed an intermediate-grade melanocytoma (CNS WHO 2021 classification). A four-month follow-up documented the absence of relapse. This literature review highlights that intramedullary T1 hyperintense hemorrhagic thoracic lesions in an adult patient should raise the suspicion of intramedullary melanocytoma. They present as locally aggressive tumors, due to local invasiveness, which often lead to post-operative neurological deficits, and frequent relapses, which overwhelm therapeutic strategies leading to palliative care after several years.

Cervical Intradural Extramedullary Melanocytoma , Associated with Nevus of Ito. Recurrence and Long-Term Outcome

Background: Melanocytoma is a rare pigmented central nervous tumor. In the spine, it may develop in meninges, extramedullary or intramedullary. It usually has good outcome after total excision.
 Case report:
 A 40-year-old male patient was admitted to our hospital 12 years ago with a 3-month history of progressive neck pain and difficulty walking. On clinical examination, he showed upper dorsal bilateral gray blue hyperpigmentation, quadriparesis and bilateral hypoesthesia below C4. Cervical magnetic resonance imaging (MRI) T1-weighted images without contrast showed a C3-C4 intradural, extramedullary lesion. The patient underwent total microsurgical resection of the lesion. Histological and immunohistochemical analyses confirmed the diagnosis of melanocytoma. Another skin biopsy of the abnormal area showed histological findings of a blue skin nevus. Eight years after resection the patient presented with recurrent symptoms, which were similar to his initial presentation. Cervical MRI showed an intradural extramedullary tumor anterior to the spinal cord at the same C3-C4 level. The patient underwent a second complete tumor excision and melanocytoma was confirmed histologically. The patient significantly improved and remained well at last follow-up.
 Conclusions: Intradural extra- or intramedullary melanocytoma is a rare benign tumor that frequently recurs but usually has good outcome following total excision.
 Keywords: Spinal cord, Meningeal, Melanocytoma, Extramedullary, Skin Nevus of Ito, Outcome

Intradural Intramedullary Primary Spinal Melanocytoma: A Rare Case Report

Spinal melanocytomas are rare pigmented tumors of the central nervous system commonly affecting females in their fifth decades. It has been reported in various locations with intradural intramedullary being the rarest. Patients can present with varied symptomatology from mild backache to frank myelopathy. Here we report a case of 34-year-old man with no medical comorbidities with vague backache associated with numbness of anterior aspect of right thigh for 1 year aggravated by activities and relieved by rest. His neurologic examination revealed normal power and sensation in both lower limbs except for decreased temperature sensation in right L2 dermatome. MRI of dorsolumbar spine revealed a well-defined lesion that was hyperintense in T1- and hypointense in T2-weighted image at D11–12 region with uniform bright enhancement in gadolinium-enhanced T1 image. The patient underwent laminectomy of D11 and D12, and an en bloc excision was done. Diagnosis of primary intradural intramedullary melanocytoma was made from histopathologic examination. Spinal melanocytomas should be considered as a differential diagnosis for intradural space-occupying lesions. MRI remains the investigation of choice for diagnosis, but histopathologic examination is required to confirm and differentiate from other pigmented tumors and malignant melanomas. Total surgical excision of the tumor remains the standard treatment with radiotherapy reserved for cases of incomplete excision and recurrences.

An Extremely Rare Case of Thoracic Intramedullary Melanocytoma: Case Report and Review of Literature

An Extremely Rare Case of Thoracic Intramedullary Melanocytoma: Case Report and Review of Literature

Cervical intramedullary melanocytoma: A case report and review of literature

Meningeal melanocytomas are rare tumours of the central nervous system, most commonly found in the intracranial compartment (posterior cranial fossa). Spinal lesions are uncommon, with the majority being intradural, extramedullary lesions. Intramedullary melanocytomas are extremely rare and are most commonly found in the thoracic region. We report a patient with cervical intramedullary melanocytoma and review the available literature regarding this rare pathology.

Primary Intramedullary Melanocytoma: A Case Report

We report herein a case of primary intramedullary melanocytoma in a 28-year-old female. She presented with a 3-year history of back pain with paresthesia and dysesthesia of the right lower extremity. Magnetic resonance imaging showed an intramedullary mass located at the T11-12 level. The tumor was removed totally. The histopathological diagnosis was melanocytoma. Intramedullary melanocytomas are rare lesions, and this is only the 20th case described in the literature. Though known as benign tumors, local recurrence and the aggressive behavior of melanocytomas must be kept in mind, even following total resection.

Malignant Transformation of Intramedullary Melanocytoma

Meningeal melanocytomas are low-grade primary melanocytic tumors with benign histological features and a favorable clinical prognosis. Transition from meningeal melanocytoma to primary melanoma of the central nervous system is exceptionally rare, with only 5 cases having been previously reported. Here, we discuss a case of malignant transformation of an intramedullary melanocytoma to primary melanoma and review the pertinent literature. A 79-year-old woman presented with progressive paresis in the lower limbs followed by sphincter dysfunction. Magnetic resonance imaging scans disclosed an intramedullary lesion located at the T10-T11 level. The patient underwent subtotal resection of an intermediate-grade melanocytoma. Two years later, the tumor recurred locally, and the patient underwent additional surgery to remove the intramedullary mass. The histological findings of the tumor were consistent with an intramedullary malignant melanoma. The malignant transformation of melanocytic tumors of the central nervous system may occur years after surgical treatment, and its incidence remains unknown. Emphasis should be placed on the importance of careful and continued follow-up monitoring of the tumor.

Intramedullary Melanocytoma: Case Report and Review of Literature

We report on a very rare case of dorsal intramedullary melanocytoma in a 62-year-old man. The tumor was resected and gross total removal was achieved. During a 2-year follow-up period in which no radiotherapy was given, the tumor did not recur. We review the literature on these tumors and present disease criteria to distinguish melanocytoma from primary malignant melanoma. Patients and investigators should be cautioned that an intramedullary melanocytoma may recur and give rise to metastatic spread via the cerebrospinal fluid.

Thoracic intramedullary melanocytoma with long-term follow-up

Melanocytomas are tumors of the neuraxis seen predominantly in adults. The authors report a case of a thoracic intramedullary melanocytoma with long-term follow-up of 8 years postsurgery. Melanocytomas, although rare, must be included in the differential diagnosis of intramedullary spinal cord tumors.