Melanotic schwannoma is a rare form of nerve sheath tumor composed of melanin-producing neoplastic Schwann cells. Less than 200 cases have been reported worldwide. The entity has been associated with Carney complex, a rare genetic disorder characterized by multiple benign tumors. A 38-year-old female presented to our unit with sudden-onset lower back pain and radiculopathy triggered by a mechanical injury. Imaging demonstrated a lesion within the left L5/S1 neural exit foramen with remodeling of bony architecture typical of a chronic, benign process. She proceeded for resection and histology revealed a psammomatous melanotic schwannoma. The patient recovered well with improvement in symptomology. Due to the aggressive nature of the disease, she remains under surveillance for local recurrence and distant metastasis. Clinicians should be aware of this malignant entity, despite its possible presentation with radiological features of a chronic, benign process. Unusual characteristics such as hemorrhage should be treated with a high index of suspicion.
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http://dx.doi.org/10.4103/ajns.AJNS_142_20 | 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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