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Primary meningeal melanoma masquerading as neurofibromatosis type 2: illustrative case

BACKGROUND: Primary meningeal melanocytic neoplasms are exceedingly rare tumors, representing only 0.06% to 0.1% of all primary brain tumors and ranging in spectrum from benign localized tumors to highly aggressive malignant lesions. The diagnosis of these tumors is often challenging from clinical,...

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Autores principales: Lang-Orsini, Melanie, Wu, Julian, Heilman, Carl B., Kravtsova, Alina, Weinstein, Gene, Madan, Neel, Arkun, Knarik
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Association of Neurological Surgeons 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9435569/
https://www.ncbi.nlm.nih.gov/pubmed/36061091
http://dx.doi.org/10.3171/CASE21444
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author Lang-Orsini, Melanie
Wu, Julian
Heilman, Carl B.
Kravtsova, Alina
Weinstein, Gene
Madan, Neel
Arkun, Knarik
author_facet Lang-Orsini, Melanie
Wu, Julian
Heilman, Carl B.
Kravtsova, Alina
Weinstein, Gene
Madan, Neel
Arkun, Knarik
author_sort Lang-Orsini, Melanie
collection PubMed
description BACKGROUND: Primary meningeal melanocytic neoplasms are exceedingly rare tumors, representing only 0.06% to 0.1% of all primary brain tumors and ranging in spectrum from benign localized tumors to highly aggressive malignant lesions. The diagnosis of these tumors is often challenging from clinical, radiological, and pathologic standpoints. Equally challenging is the distinction between primary meningeal melanocytic neoplasm and metastatic melanoma. OBSERVATIONS: The authors reported the case of a 41-year-old man with imaging findings diagnostic of neurofibromatosis type 2: bilateral internal auditory canal lesions (most consistent with bilateral vestibular schwannomas), two dura-based lesions presumed to be meningiomas, multiple spinal lesions consistent with peripheral nerve sheath tumors, and one intramedullary spinal lesion consistent with an ependymoma. Biopsy of these lesions revealed melanocytic neoplasms with mild to moderate atypia and a mildly elevated proliferation index, which made the distinction between benign and malignant challenging. In addition, the disseminated nature of these tumors made it difficult to determinate whether they arose from the meninges or represented metastases from an occult primary melanoma. LESSONS: This case illustrated the challenges presented by the diagnosis of meningeal melanocytic neoplasms and highlighted the importance of integrating the clinical and radiographic findings with histologic appearance and molecular studies.
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spelling pubmed-94355692022-09-02 Primary meningeal melanoma masquerading as neurofibromatosis type 2: illustrative case Lang-Orsini, Melanie Wu, Julian Heilman, Carl B. Kravtsova, Alina Weinstein, Gene Madan, Neel Arkun, Knarik J Neurosurg Case Lessons Case Lesson BACKGROUND: Primary meningeal melanocytic neoplasms are exceedingly rare tumors, representing only 0.06% to 0.1% of all primary brain tumors and ranging in spectrum from benign localized tumors to highly aggressive malignant lesions. The diagnosis of these tumors is often challenging from clinical, radiological, and pathologic standpoints. Equally challenging is the distinction between primary meningeal melanocytic neoplasm and metastatic melanoma. OBSERVATIONS: The authors reported the case of a 41-year-old man with imaging findings diagnostic of neurofibromatosis type 2: bilateral internal auditory canal lesions (most consistent with bilateral vestibular schwannomas), two dura-based lesions presumed to be meningiomas, multiple spinal lesions consistent with peripheral nerve sheath tumors, and one intramedullary spinal lesion consistent with an ependymoma. Biopsy of these lesions revealed melanocytic neoplasms with mild to moderate atypia and a mildly elevated proliferation index, which made the distinction between benign and malignant challenging. In addition, the disseminated nature of these tumors made it difficult to determinate whether they arose from the meninges or represented metastases from an occult primary melanoma. LESSONS: This case illustrated the challenges presented by the diagnosis of meningeal melanocytic neoplasms and highlighted the importance of integrating the clinical and radiographic findings with histologic appearance and molecular studies. American Association of Neurological Surgeons 2021-11-15 /pmc/articles/PMC9435569/ /pubmed/36061091 http://dx.doi.org/10.3171/CASE21444 Text en © 2021 The authors https://creativecommons.org/licenses/by-nc-nd/4.0/CC BY-NC-ND 4.0 (http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) ).
spellingShingle Case Lesson
Lang-Orsini, Melanie
Wu, Julian
Heilman, Carl B.
Kravtsova, Alina
Weinstein, Gene
Madan, Neel
Arkun, Knarik
Primary meningeal melanoma masquerading as neurofibromatosis type 2: illustrative case
title Primary meningeal melanoma masquerading as neurofibromatosis type 2: illustrative case
title_full Primary meningeal melanoma masquerading as neurofibromatosis type 2: illustrative case
title_fullStr Primary meningeal melanoma masquerading as neurofibromatosis type 2: illustrative case
title_full_unstemmed Primary meningeal melanoma masquerading as neurofibromatosis type 2: illustrative case
title_short Primary meningeal melanoma masquerading as neurofibromatosis type 2: illustrative case
title_sort primary meningeal melanoma masquerading as neurofibromatosis type 2: illustrative case
topic Case Lesson
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9435569/
https://www.ncbi.nlm.nih.gov/pubmed/36061091
http://dx.doi.org/10.3171/CASE21444
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