Multifocal meningeal melanocytoma of the conus medullaris

Merciadri, Paolo; Secci, Francesca; Sbaffi, Pier Filippo; Zona, Gianluigi

doi:10.1007/s00701-011-1143-x

Cited by 10 publications

(9 citation statements)

References 8 publications

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“…A total of five cases have been described in the combination of a posterior fossa mass with a spinal lesion or multiple spine lesions. [ 1 6 13 18 20 ] The resected lesions were all benign on pathology except in the case described by Foit et al ., where one of the two lesions had an intermediate grade [ Table 1 ].…”

Section: Discussionmentioning

confidence: 92%

Multifocal melanocytoma of the posterior fossa and subcutaneous scalp in the absence of neurocutaneous melanosis

Pierson¹,

Marwaha²,

Guzmán³

et al. 2016

Surg Neurol Int

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Background:Primary leptomeningeal melanocytic neoplasms of the central nervous system are rare. Multifocal lesions typically occur in the setting of cutaneous melanosis. We present the first report of a posterior fossa melanocytoma and subcutaneous melanocytoma of intermediate grade in the absence of cutaneous melanosis.Case Description:We present the case of a 22-year-old male with decreased hearing on the right side, ataxia, nausea, vomiting and a scalp mass. Magnetic resonance imaging (MRI) demonstrated occipital and cerebellopontine (CP) angle masses. The patient underwent gross total resection of the scalp mass and subtotal resection of the CP angle mass. Pathologic examination revealed melanocytoma with intermediate grade. The patient underwent stereotactic radiosurgery to the residual CP angle tumor. This case represents, to the author's knowledge, the first report associating a posterior fossa melanocytoma with a subcutaneous melanocytoma of intermediate grade in the absence of cutaneous melanosis.Conclusion:This case introduces the first report of a new variant of multifocal melanocytoma which is not confined to the central nervous system.

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Section: Discussionmentioning

confidence: 92%

Multifocal melanocytoma of the posterior fossa and subcutaneous scalp in the absence of neurocutaneous melanosis

Pierson¹,

Marwaha²,

Guzmán³

et al. 2016

Surg Neurol Int

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“…The mean age at which patients first presented was 40.6 years (5–72) [ 9 , 10 ]. Common presentations include leg weakness ( n = 8) [ 6 , 7 , 9 , 11–15 ], neck pain ( n = 5) [ 16–20 ] and back pain ( n = 4) [ 10 , 11 , 14 , 21 ]. The average duration of symptoms prior to initial presentation was 8.7 months (0.07–36); 43% ( n = 10) cases were in the cervical region, 43% ( n = 10) were in the thoracic region, 9% ( n = 2) were in the sacral region and 4% ( n = 1) were in the lumbar region of the spinal cord.…”

Section: Discussionmentioning

confidence: 99%

“…The remaining patients (43%, n = 10) underwent STR, with the extent of resection determined by the surgeon; 43% ( n = 10) of patients experienced complete resolution of their symptoms after surgery. Post-operative complications were infrequently reported, however, the most reported was genitourinary dysfunction ( n = 5) [ 6 , 10 , 14 , 22 , 23 ]. Four patients were not followed up, but of those that were, the average follow-up period was 16 months (1–96).…”

Section: Discussionmentioning

confidence: 99%

Intradural extramedullary meningeal melanocytoma: a case report and literature review

Parwez

Ahmed

Baig

et al. 2023

Journal of Surgical Case Reports

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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.

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“…Synchronous presentation of intracranial and extracranial melanocytomas is an exceedingly rare event with only few cases documented in the literature . Cases of synchronous or multifocal melanocytomas published in the English literature are summarized on Table …”

Section: Discussionmentioning

confidence: 99%

Subcutaneous melanocytoma mimicking a lipoma: a rare presentation of a rare neoplasm with histological, immunohistochemical, cytogenetic and molecular characterization

et al. 2016

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Melanocytoma are the melanocytic tumors originating from leptomeningeal melanocytes. Melanocytomas are commonly seen in the central nervous system (CNS) and are often associated with neurocutaneous melanosis (NCM). However, simultaneous presentation of intra-axial and extracranial melanocytoma is a very rare event. Here, we report a unique case of 21-year-old male with intermediate-grade subcutaneous (SC) melanocytoma, mimicking lipoma, occurred synchronously with an intracranial melanocytoma, not associated with NCM. A 21-year-old Caucasian male presented to the emergency department (ED) with severe vertigo and vomiting. A magnetic resonance imaging (MRI) of the brain was performed at the ED, which revealed an SC mass in the right occipital scalp and a right cerebellopontine angle (CPA) mass. Excision of the SC mass revealed a well-circumscribed highly pigmented melanocytic tumor. The SC mass tumor cells were positive for melanocytic lineage markers. The histopathological features were between benign melanocytomas and malignant melanomas. The Ki67 and PHH3 IHCs confirm the intermediate grade of the tumors. An array-CGH (comparative genome hybridization) and next-generation sequencing analysis of the tumor DNA extracted from the formalin-fixed paraffin-embedded tissue reveals chromosome 6p gain and p.Q209P mutation in the GNAQ gene, respectively, consistent with the diagnosis of intermediate-grade melanocytoma.

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Multifocal meningeal melanocytoma of the conus medullaris

Cited by 10 publications

References 8 publications

Multifocal melanocytoma of the posterior fossa and subcutaneous scalp in the absence of neurocutaneous melanosis

Multifocal melanocytoma of the posterior fossa and subcutaneous scalp in the absence of neurocutaneous melanosis

Intradural extramedullary meningeal melanocytoma: a case report and literature review

Subcutaneous melanocytoma mimicking a lipoma: a rare presentation of a rare neoplasm with histological, immunohistochemical, cytogenetic and molecular characterization

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