Bifocal extra- and intradural melanocytoma of the spine: case report and literature review

Foit, Niels Alexander; Neidert, Marian C.; Woernle, Christoph M.; Krayenbühl, Niklaus

doi:10.1007/s00586-013-2773-x

Cited by 12 publications

(11 citation statements)

References 23 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: 93%

“…Case reports of melanocytomas have sometimes also commented on the presence of diffuse leptomeningeal hyperpigmentation of the surrounding dura, as seen in our case. [ 1 5 6 7 ] These are not considered to be the cases of melanocytosis, which usually occurs with underlying cutaneous melanosis. Of all the case reports describing multifocal disease, Ali et al .…”

Section: Discussionmentioning

confidence: 99%

“…also mentioned the presence of surrounding leptomeningeal hyperpigmentation. [ 1 6 ] The other authors did not specifically comment on the status of the surrounding dura. The clinical significance of underlying leptomeningeal hyperpigmentation in terms of the formation of a de novo melanocytoma, recurrence, leptomeningeal spread, or degeneration to melanoma is not known.…”

Section: Discussionmentioning

confidence: 99%

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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: 93%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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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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“…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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“…[ 2 ] The majority of spinal melanocytomas remain benign and are intradural extramedullary in location with a predilection for the cervical spine due to the increased concentration of melanocytic cells. [ 3 ] This case stands distinct in highlighting the rare occurrence of a lumbar spinal nerve root extradural melanocytoma, emphasizing the remote possibility of its histological progression into malignant melanoma, tackled with the help of adjuvant radiotherapy (RT) and immunotherapy.…”

Section: Introductionmentioning

confidence: 99%

Spinal nerve root extradural melanocytoma progressing to malignant melanoma: A case report with review of literature

et al. 2021

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Melanocytomas are rare benign pigmented tumors arising from the leptomeninges with a very remote chance of progressing to malignant melanoma. They have a predilection for occurring in the posterior fossa or in the intradural extramedullary region of the cervical spine. We report the first case of malignant transformation of a nerve root (extradural) melanocytoma wherein immunotherapy has been added for its treatment. Only four such cases of malignant transformation of central nervous system melanocytoma have been reported in the literature. Definite diagnosis in such cases is based on immunohistochemistry evaluation. Surgical resection with adjuvant radiotherapy and immunotherapy is the recommended treatment.

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Bifocal extra- and intradural melanocytoma of the spine: case report and literature review

Cited by 12 publications

References 23 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

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

Spinal nerve root extradural melanocytoma progressing to malignant melanoma: A case report with review of literature

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