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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.

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The "Pigmented Side" of Nerve Sheaths: Malignant Melanotic Nerve Sheath Tumor.

Int 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.

Article Synopsis
  • - Malignant melanotic nerve sheath tumor (MMNST) is a rare and aggressive tumor that arises in both peripheral and cranial nerves, accounting for less than 1% of all nerve sheath tumors, but its true prevalence is uncertain due to changes in classification.
  • - Unlike previously thought, MMNST is now recognized as a distinct tumor type with unique clinical and biological characteristics, often linked to a specific genetic marker and frequently found in patients with Carney Complex.
  • - This report discusses a patient with MMNST, emphasizing the importance of a multidisciplinary diagnostic approach, and presents the first case of proton beam therapy for this condition while reviewing recent literature to address diagnostic challenges and histopathological pitfalls.
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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.

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Interosseous Malignant Melanotic Nerve Sheath Tumor of the Sacrum Treated With an Innovative Reconstruction Technique.

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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