Malignant triton tumour of the thigh - A retrospective analysis of nine cases

The Royal Orthopaedic Hospital Oncology Service, Royal Orthopaedic Hospital, Bristol Road South, Northfield, Birmingham B31 2AP, UK.
European Journal of Surgical Oncology (Impact Factor: 3.01). 09/2006; 32(7):808-10. DOI: 10.1016/j.ejso.2006.04.008
Source: PubMed


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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    • "An earlier experiment by Locatelli [3] had shown the growth of neural and muscular elements in the form of a supernumerary limb in the " triton " by transplanting the sciatic nerve of this salamander onto its dorsal surface. An MTT has been seen in the setting of both with and without multiple neurofibromatosis/NF-1 (von Recklinghausen disease) and has been found to behave aggressively [4] [5] [6] [7] [8] [9]. A series of 10 cases of MTT diagnosed over a period of 10 years is described in this article. "
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    ABSTRACT: A rhabdomyoblastic differentiation in a malignant peripheral nerve sheath tumor is unusual and is termed as a malignant triton tumor. A series of 10 such cases with their clinicomorphological features, diagnosed over a 10-year period, is presented. The average age of occurrence was 30 years, with the maximum number of cases in the second decade and with male outnumbering female patients. More cases were seen in the setting of neurofibromatosis. On histology, 80% of the cases were of high grade. Distinct rhabdomyoblastic cells were identified in the areas of malignant peripheral nerve sheath tumor. Immunohistochemistry confirmed the neurogenic differentiation with varying S-100 expression and the rhabdomyoblastic differentiation with desmin and myoglobin positivity in all cases. Surgery with adequate margins constituted the treatment mainstay with adjuvant chemotherapy and/or radiotherapy in individual cases. On follow-up with 7 cases, 3 showed local recurrences, including one that, in addition to another 2 cases, showed lung metastasis. One patient died of the disease. This case along with another high-grade case displayed a diffuse Ki-67 and p53 positivity. Malignant triton tumor is an uncommon tumor associated with an aggressive behavior. Surgery with clear margins is the treatment mainstay. Adjuvant radiotherapy is effective.
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    ABSTRACT: Malignant Triton tumor (MTT) is classified as a variant of a malignant peripherial nerve sheath tumor with a focal rhabdomyosarcomatous differentiation. Nearly one third of described MTTs were located at the head and neck region. In following paper we described a case of MTT of the external nose in a 24 year old man. The tumor was totally exised surgically with the broad margins. Histopathological diagnosis of the tumor was made on the surgical specimen, based on immunohistochemical analysis. Radiation therapy as the next step of treatment of choice was performed in presented case.
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