Malignant triton tumour of the thigh - A retrospective analysis of nine cases
ABSTRACT Malignant triton tumour (MTT) is a very rare soft tissue tumour.
To report nine patients diagnosed with malignant triton tumour of the thigh between January 1996 and January 2005 to determine the natural history and factors that may affect survival in this aggressive tumour.
Due to the association of Neurofibromatosis (NF Type I) with malignant triton tumour, two groups of patients were identified: those with NF type I (Group I cases); and those without (Group II).
Group I patients accounted for four cases and arose predominantly in young males, all tumours being high grade, >10 cm in size and all four patients died within two years of diagnosis. By contrast, the five patients of Group II were older, had smaller size and lower grade tumours and three patients are long-term survivors.
Although the number of cases is small, our data supports the views that the natural history of MTT is more aggressive in patients with NF type I. This poor outlook could be attributed to the high frequency of Grade III histology in this disease and the large tumour size.
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ABSTRACT: Rhabdomyoblastic differentiation in a malignant peripheral nerve sheath tumor (MPNST) is termed malignant triton tumor (MTT), a rare neoplasm that poses a diagnostic dilemma in the differential diagnosis of neck masses and portends poor prognosis. We report a sporadic case of MTT of the neck in a 23-year-old female. We present the pathological findings. Immunohistochemistry confirmed the neurogenic origin with S-100 expression and the rhabdomyoblastic differentiation with desmin and vimentin positivity. Radical surgical excision was done. After 4 years there were no signs of recurrence or distant metastasis. The clinical, microscopic, and long-term follow-up of this case are consistent with those of a low-grade malignancy.01/2014; 2014:674094. DOI:10.1155/2014/674094
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ABSTRACT: Background: Malignant triton tumor (MTT) is an histological deviation of malignant peripheral nerve sheath tumor with additional rhabdomyosarcomatous elements. It is very rare, profoundly aggressive, with a tendency to recur locally and metastasize early. If manifests itself more often in individuals with neurofibromatosis type I (NF-1) disease but also sporadically or post radiotherapy. Description of case: A 57-year-old male was admitted with a history of malignant triton tumor of the chest wall. Despite prior aggressive locoregional treatment including wide excision and adjuvant consolidating radiotherapy, the tumor recurred. The patient underwent a new operation and systemic chemotherapy, but expired a few months later due to disease progression. Conclusion: MTT is exceedingly malignant requiring multimodality treatment. The cornerstone of management is radical surgical resection with clear margins. Nevertheless, the overall prognosis remains dismal.Hippokratia 07/2013; 17(3):277-80. · 0.36 Impact Factor
Article: Malignant Triton Tumor: A Rare Case[Show abstract] [Hide abstract]
ABSTRACT: Malignant triton tumor (MTT) is a malignant peripheral nerve sheath tumor (MPNST) with rhabdomyoblastic differentiation. This rare tumor, with fewer than 100 cases reported in the literature, generally affects adult patients with neurofibromatosis 1 (NF-1). We report such a case in a 34-year-old man with NF-1 who presented with a mass over the medial side of the arm. Histopathologically finding of rhabdomyoblasts among malignant Schwann cells in a tumor arising from a peripheral nerve supported by immunostaining with S-100 protein and myogenin confirmed the diagnosis. MTT has a poor prognosis owing to its aggressive biological behavior. The fact that this tumor is extremely rare has prompted us to report this case.Indian Journal of Surgery 06/2013; 75(1). DOI:10.1007/s12262-012-0710-6 · 0.27 Impact Factor