Intracranial melanotic schwannoma is quite rare, and involvement of the trigeminal nerve is even rarer. Early diagnosis and surgical excision are the mainstays of management. These tumors have a high tendency to recur and there is high possibility of metastasis. Adjuvant radiotherapy should be considered since the prognosis is uncertain. A 23-year-old man started developing numbness over the left side of the forehead 9 months ago that progressed to involve the ipsilateral cheek. The patient started having diplopia on looking to the left side 8 months ago. His relatives noticed a change in his voice 1 month ago and he developed weakness in the right upper and lower limbs, which was gradually progressive. The patient had slight difficulty swallowing. After examination, we found involvement of multiple cranial nerves with pyramidal signs. Magnetic resonance imaging (MRI) was suggestive of an extra-axial lesion in the left cerebellopontine angle extending into the middle cranial fossa, which was having high T1 and T2 signal loss with contrast enhancement. We achieved near-total excision of the tumor via a subtemporal extradural approach. Trigeminal melanotic schwannoma is a rare occurrence constituting melanin-producing cells and Schwann cells. Rapid progression of symptoms and signs should prompt the suspicion of the possible malignant nature of the pathology. Extradural skull base approaches reduce the risk of postoperative deficits. Differentiating melanotic schwannoma from malignant melanoma is of utmost importance in planning of management.
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http://dx.doi.org/10.1055/s-0043-1768578 | 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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