S-100-negative, GNA11 mutation-positive intramedullary meningeal melanocytoma of the thoracic spine: A radiographic challenge and histologic anomaly

Flores, Álex; Gadot, Ron; Noorbhai, Ibrahim; Hall, Hayden; Heck, Kent A.; Raper, Daniel Matthew Sholto; Xu, David S.; Karas, Patrick J.; Mandel, Jacob; Ropper, Alexander E.

doi:10.25259/sni_416_2021

Cited by 3 publications

(3 citation statements)

References 27 publications

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“…This, in conjunction with the invasiveness, highlights its locally aggressive behavior despite its benign biology. This may reinforce the notion that less aggressive surgery should be attempted when removing these tumors, with the aim of obtaining the maximal safe resection, as in the case of Flores et al [18]. This is especially important when considering the advancements in targeted therapies, which may prove effective in treating tumor relapse.…”

Section: Discussionmentioning

confidence: 56%

“…Similarly, Wagner et al described tumor control at eighteen months with fractionated RT administered for a relapse following GTR [13]. However, in the other seven reports, a relapse after GTR was noted during FU MRI, and adjuvant RT was deemed unnecessary [15,[17][18][19]21]. In the cases described by Horn et al [19], despite GTR, recurrence was noted.…”

Section: Discussionmentioning

confidence: 96%

“…However, in thirteen cases in which IONM was not employed, a frequent post-operative worsening of neurological function was reported as well. Flores et al [18] reported an unspecified decrease in MEPs during the operation, leading to the decision to interrupt debulking to preserve neurological functioning. Post-operatively, the patient experienced temporary worsening of left leg strength, which recovered after three weeks, and improved right leg strength.…”

Section: Discussionmentioning

confidence: 99%

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

Battistelli,

Grilli,

Rapisarda

et al. 2024

Cancers

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Background: 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. Methods: A systematic review was conducted across the PubMed and Scopus databases including studies published till February 2024. Results: 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. Case presentation: 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. Conclusions: 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.

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Section: Discussionmentioning

confidence: 56%

Section: Discussionmentioning

confidence: 96%

Section: Discussionmentioning

confidence: 99%

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

Battistelli,

Grilli,

Rapisarda

et al. 2024

Cancers

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Intraoperative View of a Multinodal, Paralysis-Inducing Spinal Melanocytoma: Case Report and Literature Review

Clampitt,

Monsour,

Kazelka

et al. 2024

World Neurosurgery

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How Should We Treat Meningeal Melanocytoma? A Retrospective Analysis of Potential Treatment Strategies

Ricchizzi

Gallus

Stummer

et al. 2022

Cancers

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Background: Meningeal melanocytomas (MM) are rare primary melanocytic tumors of the leptomeninges with an incidence of 1:10,000,000. Until now, there has been only sparse information about this tumor entity. Here, we provide a meta-analysis of all cases published in the English language since 1972. Methods: A literature review was performed using PubMed and Web of Science. All published cases were evaluated for location, sex, age, therapeutic approach, and outcome. In total, we included 201 patient cases in our meta–analysis. Results: The majority of MM was diagnosed more frequently in men between the third and fifth decade of life. Surgery is the preferred therapeutic approach, and total resection is associated with the best outcome. Patients with partial resection or tumor recurrence benefit from adjuvant radiotherapy, whereas chemo- or immunotherapies do not improve the disease course. Malignant transformation was described in 18 patients. Of these, 11 patients developed metastasis. Conclusions: We present the first retrospective meta-analysis of all MM cases published in the English language, including an evaluation of different treatment strategies allowing us to suggest a novel treatment guideline highlighting the importance of total resection for recurrence–free survival and characterizing those cases which benefit from adjuvant radiotherapy.

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S-100-negative, GNA11 mutation-positive intramedullary meningeal melanocytoma of the thoracic spine: A radiographic challenge and histologic anomaly

Cited by 3 publications

References 27 publications

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

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

Intraoperative View of a Multinodal, Paralysis-Inducing Spinal Melanocytoma: Case Report and Literature Review

How Should We Treat Meningeal Melanocytoma? A Retrospective Analysis of Potential Treatment Strategies

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