Background: Melanotic schwannoma (MS) is a rare variant of peripheral nerve sheath tumor. MS commonly arises along the spinal nerve sheath. Patients most often experience pain along the dermatome of the affected nerve root. Symptoms development is usually insidious. About half of MS cases are associated with Carney complex, a multi-neoplastic disorder. The remaining cases arise spontaneously. About 10-44% of these tumors undergo malignant transformation.
Case Description: We describe a case of hemorrhagic MS presenting as acute chest pain mimicking myocardial infarction, a presentation which has not yet been described in the literature. Neurologic examination did not reveal any abnormalities. Myocardial infarction was ruled out in the ER, and a chest CT angiogram was ordered for evaluation of PE or aortic dissection which revealed an intradural extramedullary dumbbell-shaped mass extending through the left vertebral foramen at the level of T8. MRI revealed a heterogenous mass that was hyperintense with T2 and hypointense with T1-weighted imaging. The patient underwent an open laminectomy of the left T8 and T9 vertebrae and gross total resection (GTR) of a hemorrhagic black tumor. Microscopic examination showed fascicles and nests of plump spindle cells with variable intracellular melanin. Immunohistochemistry showed the cells to be positive for S100, SOX10, HMB-45, and MART-1, confirming diagnosis of MS. Two months after the operation, the patient was doing well and is free of recurrence.
Conclusion: GTR is considered the optimal treatment for MS; radiotherapy and chemotherapy may be considered but have not been shown to improve patient outcomes.
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http://dx.doi.org/10.25259/SNI_786_2020 | DOI Listing |
Eur J Ophthalmol
November 2024
Department of Ophthalmology, SUNY Upstate Medical University, Syracuse, New York, USA.
Introduction: Melanotic schwannoma (MS) is a rare neoplasm composed of Schwann cells with melanosomes in various maturation stages. While MS is typically observed in spinal nerve roots or peripheral nerves, their involvement in intraocular structures is uncommon. Here, we present a case of spontaneous globe rupture as the presenting feature of intraocular extension of a MS.
View Article and Find Full Text PDFInt J Surg Pathol
November 2024
Neuroscience, Reproductive and Odontostomatological Sciences, Division of Neurosurgery, University of Naples Federico II School of Medicine and Surgery, Napoli, Campania, Italy.
Int J Surg Pathol
September 2024
Inova Schar Cancer Institute, Fairfax, VA, USA.
Front Oncol
August 2024
Department of Gynecology, Jinhua Municipal Central Hospital, Jinhua, Zhejiang, China.
Background: Melanotic schwannoma (MS), a rare variant of peripheral nerve sheath tumor, is especially infrequent when originating from the peritoneum. Its definitive diagnosis relies on postoperative histopathological examination and immunohistochemical analysis, while preoperative diagnosis is difficult.
Case Presentation: In the present study, we reported a rare case of giant MS in the retroperitoneum, which was previously misdiagnosed before surgery.
Cureus
July 2024
Pediatric Neurosurgery Division, Department of Pediatric Surgery, King Abdulaziz Medical City, Ministry of National Guard - Health Affairs, Riyadh, SAU.
Intraosseous malignant melanotic nerve sheath tumors are extremely uncommon peripheral nerve sheath tumors that typically present with benign clinical and histopathological features but with more aggressive long-term behavior. These tumors commonly originate from the dorsal nerve roots, sympathetic chain, cranial nerves, and lumbar plexus but may be found throughout the body. It usually presents with gradual compressive symptoms over months to years, like the typical presentation of schwannomas.
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