Case Report

A Rare Case of Malignant Triton Tumor with Pluridirectional Differentiation

Authors: Konstantinos Ballas MD, PhD, Theodoros M. Kontoulis MD, Athanasios Papavasiliou MD, Dimitrios Pissas MD, Theodoros Pavlidis MD, PhD, Evangelia Katsiki MD, Ioannis Venizelos MD, PhD, Athanasios Sakadamis MD, PhD

Abstract

Abstract:An enlarging soft tissue mass was resected from the leg of a young man with neurofibromatosis type 1. Rhabdomyosarcomatous elements admixed with islands of osteoid and chondroid matrix was seen on microscopy. Based on immunohistochemistry, a malignant triton tumor, an uncommon subtype of peripheral nerve sheath tumor with rhabdomyosarcomatous elements, was diagnosed. The important feature of this neoplasm was that it showed pluridirectional differentiation to osteosarcoma and chondrosarcoma. This pathologic finding is rare and seen in only a few cases of all malignant triton tumors.

This content is limited to qualifying members.

Existing members, please login first

If you have an existing account please login now to access this article or view purchase options.

Purchase only this article ($25)

Create a free account, then purchase this article to download or access it online for 24 hours.

Purchase an SMJ online subscription ($75)

Create a free account, then purchase a subscription to get complete access to all articles for a full year.

Purchase a membership plan (fees vary)

Premium members can access all articles plus recieve many more benefits. View all membership plans and benefit packages.

References

References1. Wander JV, Das Gupta TK. Neurofibromatosis. Curr Probl Surg 1977;14:1–81.WanderJV]]Das GuptaTKNeurofibromatosis.Curr Probl Surg1977141-812. Ducatman BS, Scheithauer BW. Malignant peripheral nerve sheath tumor with divergent differentiation. Cancer 1984;54:1049–1057.DucatmanBS]]ScheithauerBWMalignant peripheral nerve sheath tumor with divergent differentiation.Cancer1984541049-10573. Ducatman BS, Scheithauer BW, Piepgras DG, et al. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer 1986;57:2006–2021.DucatmanBS]]ScheithauerBW]]PiepgrasDG&etal;Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases.Cancer1986572006-20214. Stasik CJ, Tawfik O. Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation (malignant triton tumor). Arch Pathol Lab Med 2006;130:1878–1881.StasikCJ]]TawfikOMalignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation (malignant triton tumor).Arch Pathol Lab Med20061301878-18815. Kar M, Deo SV, Shukla NK, et al. Malignant peripheral nerve sheath tumors (MPNST)—clinicopathological study and treatment outcome of twenty-four cases. World J Surg Oncol 2006;4:55.KarM]]DeoSV]]ShuklaNK&etal;Malignant peripheral nerve sheath tumors (MPNST)—clinicopathological study and treatment outcome of twenty-four cases.World J Surg Oncol20064556. Woodruff JM, Perino G. Non-germ-cell or teratomatous malignant tumors showing additional rhabdomyoblastic differentiation, with emphasis on the malignant triton tumor. Semin Diagn Pathol 1994;11:69–81.WoodruffJM]]PerinoGNon-germ-cell or teratomatous malignant tumors showing additional rhabdomyoblastic differentiation, with emphasis on the malignant triton tumor.Semin Diagn Pathol19941169-817. Guccion JG, Enzinger FM. Malignant schwannoma associated with von recklinghausen’s neurofibromatosis. Virchows Arch A Pathol Anat Histol 1979;383:43–57.GuccionJG]]EnzingerFMMalignant schwannoma associated with von recklinghausen’s neurofibromatosis.Virchows Arch A Pathol Anat Histol197938343-578. Huang L, Espinoza C, Welsh R. Malignant peripheral nerve sheath tumor with divergent differentiation. Arch Pathol Lab Med 2003;127:e147–e150.HuangL]]EspinozaC]]WelshRMalignant peripheral nerve sheath tumor with divergent differentiation.Arch Pathol Lab Med2003127e147-e1509. Malerba M, Garofalo A. [A rare case of nerve-sheath sarcoma with rhabdomyoblastic differentiation (malignant triton tumor).] Tumori 2003;89:246–250 [in Italian].10. Woodruff JM, Chernik NL, Smith MC, et al. Peripheral nerve tumors with rhabdomyosarcomatous differentiation (malignant “triton” tumors). Cancer 1973;32:426–439.WoodruffJM]]ChernikNL]]SmithMC&etal;Peripheral nerve tumors with rhabdomyosarcomatous differentiation (malignant “triton” tumors).Cancer197332426-43911. Hörstadius S. The Neural Crest; Its Properties and Derivatives in the Light of Experimental Research. London, NY, Oxford University Press, 1950.HörstadiusSThe Neural Crest; Its Properties and Derivatives in the Light of Experimental Research.London, NYOxford University Press195012. Masson P, Martin JF. Rhabdomyomes des nerfs. Bull Assoc Fr Cancer 1938;27:751–767.MassonP]]MartinJFRhabdomyomes des nerfs.Bull Assoc Fr Cancer193827751-767