Malignant Triton Tumor without Von Recklinghausen’s Neurofibromatosis in a 16 year-old Indonesian Girl: A Case Report

Authors

  • Neni Anggraini Departement of Ophthalmology, Universitas Indonesia
  • Nurjati Chairani Siregar Departement of Ophthalmology, Universitas Indonesia

DOI:

https://doi.org/10.35749/journal.v43i1.129

Keywords:

malignant peripheral nerve sheath tumor, malignant triton tumor, s-100 protein

Abstract

Background: To diagnose malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation in malignant triton tumor which has a rare incidence.
Case Illustration: We reported a case in a 16 year-old girl who presented with progressively painless proptosis of the left eye and decreased visual acuity. There was no evidence of Recklinghausen’s disease. She underwent surgery and radiotherapy. The histopatologic findings showed malignant peripheral nerve sheath tumor (MPNST). Four years later, she suffered recurrence proptosis and decreased visual acuity. Histomorphology supported by immunostaining with S-100 protein confirmed the diagnosis of malignant triton tumor. She underwent chemotherapy and enucleation. Conclusion: This case highlights the prudent use of immunohistochemistry that is essential in making an early detection and a correct diagnosis.

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References

1. Statik CJ, Tawfik O. Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation (malignant triton tumor). Arch Pathol Lab Med 2006;130:1878-81.
2. Mae K, Kato Y, Usui K, Abe N, Tsuboi R. A case of Malignant Peripheral Nerve Sheath Tumor with Rhabdomyoblastic Differentiation: Malignant Triton Tumor. Case Rep Dermatol 2013;5:373–378
3. McConell YJ, Giaucomantonio CA. Malignant Triton Tumors-complete surgical resection and adjuvant radiotherapy associated with improved survival. J. Surg. Oncol. 2012; 106:51–56.
4. Tripathy K e al. A Rare Malignant Triton Tumor. Case Rep Neurol 2010;2:69–73

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Published

2017-03-30

How to Cite

Anggraini, N., & Siregar, N. C. (2017). Malignant Triton Tumor without Von Recklinghausen’s Neurofibromatosis in a 16 year-old Indonesian Girl: A Case Report. Ophthalmologica Indonesiana, 43(1), 3. https://doi.org/10.35749/journal.v43i1.129
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