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. 2021 Feb;21(2):120.
doi: 10.3892/ol.2020.12381. Epub 2020 Dec 15.

The importance of surgical margins in malignant Triton tumour of the trunk and extremities

Panagiotis Tsagozis  1   2   3 , Czar Louie Gaston  1   4 , Felix Haglund  5 , Jan Balko  6 , Vaiyapuri Sumathi  6 , Robert Grimer  1 , Michael Parry  1
Affiliations

The importance of surgical margins in malignant Triton tumour of the trunk and extremities

Panagiotis Tsagozis et al. Oncol Lett. 2021 Feb.

Abstract

Malignant Triton tumour (MTT) is a rare variant of malignant peripheral nerve sheath tumour with partial rhabdomyosarcomatous differentiation. To the best of our knowledge, the importance of the surgical resection margins on the outcome of patients with MTT is unknown. The present study is a retrospective review of 24 patients treated for MTT of the trunk and the extremities between 1997 and 2015 in two institutions. The association of surgical margins with overall and tumour recurrence-free survival was analysed. Furthermore, the typical morphological and immunohistochemical characteristics of the tumour were described. In patients treated with curative intent (17/24), a surgical margin exceeding 1 mm was significantly associated with better overall survival and local recurrence-free survival. The oncological outcome was however poor, with only 28% of patients surviving at 5 years. Histopathologically, necrosis was a common feature, and most tumours displayed focal positivity for S100 protein and focal or strong positivity for desmin. The present results highlight the aggressive behaviour of MTTs and underline the importance of adequate surgical treatment.

Keywords: Triton; margin; recurrence; sarcoma; surgery; survival.

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Figures

Figure 1.
Figure 1.
Kaplan-Meier analysis for (A) overall survival and local (B) recurrence-free survival of 24 patients with malignant Triton tumours of the trunk and the extremities. Patients treated in a curative intention had superior local recurrence-free survival, (P<0.001) while the difference in overall survival did not reach statistical significance (P=0.127).
Figure 2.
Figure 2.
Effect of surgical margins on survival as per Kaplan-Meier for patients with malignant Triton tumours of the trunk and the extremities treated in a curative intention. A clear surgical margin below 1 mm (R0, clear close) is comparable to an intralesional one, and inferior to a clear (R0+ 1 mm) margin regarding (A) overall and (B) local recurrence-free survival.
Figure 3.
Figure 3.
Resection with clear margins of at least 1 mm was associated with superior (A) overall survival (P=0.049) and (B) local recurrence-free survival (P=0.045) among patients with malignant Triton tumours treated in a curative intention.
Figure 4.
Figure 4.
Morphological and immunohistochemical pattern of malignant Triton tumours. (A) Haematoxylin and eosin staining showing typical rhabdomyoblasts and (B) immunohistochemistry showing positivity for desmin. A 80 µm scale is shown in both images.
See this image and copyright information in PMC

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