Malignant Triton Tumour: Case report
Keywords:
Malignant peripheral nerve sheath tumour, Malignant triton tumour, FNAC, S-100 protein
Abstract
Malignant triton tumours (MTTs) are a rare subtype of malignant peripheral nerve sheath tumour (MPNST) showing rhabdomyoblastic differentiation, which have no treatment consensus and a poor prognosis. This case report presents case of a 71yrs/ male with swelling in Right Hand over one month. Patient had same swelling 2yrs back which was diagnosed as Peripheral Nerve Sheath Tumour on histopathology following which he underwent surgery followed by radiotherapy. Swelling reappeared at same site after 3 months. FNAC of swelling shows large spindle cells having elongated buckled nuclei, pointed end, with varying nucleus size and coarse nuclear chromatin. Few cells are showing Rabdomyoblastic differentiation.References
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2. Brooks JSJ. Disorders of soft tissue. In: Sternberg SS (ed) Diagnostic surgical pathology, 3rd edn. Lipincott Williams and Wilkins, Philadelphia, 1999: 131–221.
3. Scheithauer BW: Malignant peripheral nerve sheath tumour (MPNST). In: WHO Classification of Tumours of the Central Nervous System. Louis DN (ed). World Health Organization Publ., Corp., Lyon, pp160-162, 2007.
4. McConnell YJ and Giacomantonio CA: Malignant triton tumours-complete surgical resection and adjuvant radiotherapy associated with improved survival. J Surg Oncol 2012:106: 51-56.
5. Brooks JS, Freeman M and Enterline HT: Malignant triton tumour. Natural history and immunohistochemistry of nine new cases with literature review. Cancer 55: 2543-2549, 1985.
6. Masson P: Recklinghausen's Neurofibromatosis, Sensory Neuromas and Motor Neuromas. International Press, New York, NY, 1932.
7. Leroy K, Dumas V, Martin-Garcia N et al (2001) Malignant peripheral nerve sheath tumours associated with neurofibromatosis type 1: a clinicopathologic and molecular study of 17 patients. Arch Dermatol 137:908–913.
8. Aldlyami E, Dramis A, Grimer RJ et al (2006) Malignant triton tumour of the thigh – a retrospective analysis of nine cases. Eur J Surg Oncol 32:808–810.
9. Stasik CJ, Twafik O (2006) Malignant peripheral nerve sheath with rhabdomyosarcomatous differentiation (malignant triton tumour). Arch Pathol Lab Med 130:1878–1881.
10. Daimaru Y, Hashimoto H, Enjoli M (1984) Malignant triton tumours. A Clinicopathologic and immunohistochemical study of nine cases. Hum Pathol 15:768–778.
11. Bhatt S, Graeme-Cook F, Joseph MP and Pilch BZ: Malignant triton tumour of the head and neck. Otolaryngol Head Neck Surg 105: 738-742, 1991.
12. Ren W, Xu X , Yan J , Qian X , Lie B:Malignat triton tumour of anterior mediastinum: A case report.Oncol Lett,2014,7(3),807-810.
Published
2019-06-04
Issue
Section
Case Report
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