Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2020 Aug 28;15(3):709-712.
doi: 10.4103/ajns.AJNS_142_20. eCollection 2020 Jul-Sep.

A Rare Case of Hemorrhagic Melanotic Schwannoma in a 38-year-old Female

Rakesh Danny Biju  1 , Maria Duignan  1 , Monish Maharaj  1 , Fouzia Ziad  2 , Zakier Hussain  1
Affiliations
Case Reports

A Rare Case of Hemorrhagic Melanotic Schwannoma in a 38-year-old Female

Rakesh Danny Biju et al. Asian J Neurosurg. .

Abstract

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.

Keywords: Carney complex; melanotic schwannoma; nerve sheath tumor; psammoma bodies.

PubMed Disclaimer

Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
Sagittal and axial T2-weighted magnetic resonance imaging of the lumbosacral spine demonstrating the lesion in the left L5/51 neural foramen with some extraforaminal extension. It appears to be a well-circumscribed solid lesion with a central liquid core. Remodeling of the neural exit foramen is noted, suggesting a chronic, benign process
Figure 2
Figure 2
Coronal T2-weighted magnetic resonance imaging of the lumbosacral spine demonstrating the lesion
Figure 3
Figure 3
Psammoma body (orange arrow). Pigmented spindle cells (green arrow) demonstrating nuclear enlargement with vesicular chromatin and distinct nucleoli
Figure 4
Figure 4
Spindle cells demonstrating palisading arrangement (green arrows). Both spindle and epithelioid cells show abundant intracytoplasmic pigment deposition
Figure 5
Figure 5
Immunohistochemistry confirming SOX10-positive tumor cells. SOX10 is a sensitive and specific marker of malignant melanoma
Figure 6
Figure 6
Immunohistochemistry confirming HMB45-positive tumor cells (yellow arrows). HMB 45 is a monoclonal antibody used as a common marker to confirm melanoma
See this image and copyright information in PMC

References

    1. Louis D. WHO Classification of Tumours of the Central Nervous System. 4th ed. France: LARC; 2006. p. 173.
    1. Khoo M, Pressney I, Hargunani R, Tirabosco R. Melanotic schwannoma: An 11-year case series. Skeletal Radiol. 2016;45:29–34. - PubMed
    1. Gulati HK, Joshi AR, Anand M, Deshmukh SD. Non psammomatous melanocytic schwannoma presenting as a subcutaneous nodule: A rare presentation of a rare lesion. Asian J Neurosurg. 2016;11:317–8. - PMC - PubMed
    1. Torres-Mora J, Dry S, Li X, Binder S, Amin M, Folpe AL. Malignant melanotic schwannian tumor: A clinicopathologic, immunohistochemical, and gene expression profiling study of 40 cases, with a proposal for the reclassification of “melanotic schwannoma”. Am J Surg Pathol. 2014;38:94–105. - PubMed
    1. Bernd WS, James MW, Robert AE. Atlas of Tumor Pathology: Tumors of the Peripheral Nervous System. 3rd ed. Washington DC: American Registry of Pathology; 1999.

Publication types