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All Journal SAINS MEDIKA : JURNAL KEDOKTERAN DAN KESEHATAN
Muchtar Hanafi
Departement of Radiology, Faculty of Medicine, Universitas Sebelas Maret, Surakarta, Central Java
Health Professions Medicine & Pharmacology

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Malignant triton tumour of the distal femur: imaging features of a rare entity Reni Ibrahim; Muchtar Hanafi; Amelia Tjandra Irawan
Sains Medika: Jurnal Kedokteran dan Kesehatan Vol 17, No 1 (2026): June 2026
Publisher : Faculty of Medicine, Universitas Islam Sultan Agung (UNISSULA), Indonesia

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.30659/sainsmed.v17i1.40815

Abstract

Malignant Triton tumour (MTT) is a rare rhabdomyoblastic variant of malignant peripheral nerve sheath tumour (MPNST), characterised by striated muscle differentiation, with an incidence of approximately 0.001%. We report a 40-year-old woman with a rapidly painful, enlarging mass of the left distal femur and associated palpable lesions in the left calf and abdomen. Multislice CT demonstrated multiple solid lesions with peripheral contrast enhancement infiltrating adjacent tissues. MRI showed a large (>5 cm) heterogeneous lesion measuring 9.4 × 8.4 × 9.2 cm with lobulated margins, heterogeneous contrast enhancement, and mass effect on surrounding structures. Diagnosis and treatment: Histopathology and immunohistochemistry confirmed the diagnosis of MTT. The patient underwent two wide tumour excisions followed by adjuvant chemotherapy. Despite treatment, chest radiography one and a half years later revealed pulmonary metastases. MTT is an aggressive tumour with rapid growth and a high propensity for early metastasis. Radiologic features that should raise suspicion include large size, lobulated margins, peripheral calcifications, and internal heterogeneity. Correlation of imaging with clinical and histopathological findings is essential for timely diagnosis and treatment planning.
Co-Authors Amelia Tjandra Irawan Reni Ibrahim