Primary Malignant Melanotic Nerve Sheath Tumour of the Neck: A Diagnostic Predicament

Uthra, K. S.; Parasuraman, Lakshminarasimman; Soundararajan, Jagadesh Chandra Bose; Rajendiran, S

doi:10.1007/s12070-022-03304-w

Cited by 2 publications

(2 citation statements)

References 8 publications

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“…Patients should be followed up for a long time postoperatively, including screening for Carney syndrome, especially in younger patients [ 3 ]. The efficacy of chemoradiotherapy for this disease is uncertain, but there is some literature suggesting that stereotactic radiotherapy can be used to treat this disease, but more evidence-based evidence is lacking [ 8 , 9 ].…”

Section: Discussionmentioning

confidence: 99%

“…However, after 7 months, the tumor recurred, and it was only then that we realized that the disease was not benign. This case report hopes to provide you with more information about MMNST, on the one hand, to reduce the misdiagnosis of the disease, to pay more attention to the complete removal of the lesion during surgery, and on the other hand, to provide a pathological tissue for those who need to be studied [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

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Malignant melanotic schwannoma of the cervical spinal cord: a case report

Chen,

Wei

2024

BMC Neurol

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Spinal cord malignant melanotic schwannoma (MMNST) is a rare central nervous system tumor that originates from the spinal cord or spinal myelin sheath cells and can produce melanin. This type of tumor is usually highly aggressive and malignant, with a poor prognosis. The clinical manifestations of spinal cord MMNST are mainly pain, paresthesia, muscle weakness, muscle atrophy, etc., and symptoms of spinal cord compression, such as intestinal and bladder dysfunction, paraplegia, etc. Early detection of tumor lesions can facilitate tumor removal, improve patients’ quality of life, and prolong patients’ survival. In this case report, a 27-year-old young woman was diagnosed with MMNST of the cervical spinal cord due to weakness of her limbs in our hospital, and underwent surgical resection. The patient’s limbs returned to normal after surgery. It is worth mentioning that the patient visited our hospital 7 months ago for “right upper limb pain for 3 days” and was diagnosed with a cervical spine space-occupying lesion at the same position this time, but the pathology report was “hemosiderosis”. The patient’s limbs returned to normal after surgery. It is worth mentioning that the patient visited our hospital 7 months ago for “right upper limb pain for 3 days” and was diagnosed with a cervical spine space-occupying lesion at the same position this time, but the pathology report was “hemosiderosis”. This case report aims to raise awareness of the problem of spinal cord MMNST and contribute to greater knowledge of this rare tumor. This case report aims to raise awareness of the problem of spinal cord MMNST and contribute to greater knowledge of this rare tumor.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Malignant melanotic schwannoma of the cervical spinal cord: a case report

Chen,

Wei

2024

BMC Neurol

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The “Pigmented Side” of Nerve Sheaths: Malignant Melanotic Nerve Sheath Tumor

Franca,

Di Crescenzo,

Ugga

et al. 2024

Int J Surg Pathol

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Malignant melanotic nerve sheath tumor (MMNST) represents a highly aggressive neoplasm arising both in peripheral and cranial nerves. It accounts for < 1% of all nerve sheath tumors, but the real incidence may not be well defined yet because of the evolution of its nomenclature. To date, it is considered a distinct tumor type, no longer as the pigmented variant of schwannoma, with a different clinical course and biological behavior. MMNSTs exhibit a specific genetic hallmark related to the PRKAR1A gene, which explains the major incidence in Carney Complex-affected patients. One of the more frequent localizations is the paravertebral region, where it poses diagnostic concerns with both primary tumors arising from soft tissues and the meningeal covering, as well as metastatic ones (ie, melanoma). Herein we present a patient with an MMNST accompanied by the main clinical, radiological, histopathological, and molecular findings, stressing the need for a multidisciplinary diagnostic approach. To the best of our knowledge, this is the first report of proton beam therapy for MMNST. We also performed a literature review to collect and compare the more recent data in English literature and to highlight the “keep-in-mind” concepts to apply in a multidisciplinary diagnostic algorithm, with a focus on histopathology and related pitfalls.

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Primary Malignant Melanotic Nerve Sheath Tumour of the Neck: A Diagnostic Predicament

Cited by 2 publications

References 8 publications

Malignant melanotic schwannoma of the cervical spinal cord: a case report

Malignant melanotic schwannoma of the cervical spinal cord: a case report

The “Pigmented Side” of Nerve Sheaths: Malignant Melanotic Nerve Sheath Tumor

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