Desmoid Tumours: Experience of the Medical Oncology Department of Pierre and Marie Curie Center Algiers

Abstract

Introduction Desmoid tumours, also known as aggressive deep fibromatoses, are soft tissue tumours. Histologically benign, they are characterised by a slow infiltrative course with a high rate of local recurrence. Patients and methods: We report a retrospective series of 10 patients treated in the medical oncology department of the Pierre & Marie Curie Centre over a period of 4 years. 6 were women and 4 men, with a sex ratio of 1.5 in favour of women. The average age was 36 [23-53]. 20% (n=2) of patients had familial adenomatous polyposis. 90% of patients had an extra-abdominal desmoid tumour compared with only 10% in the abdominal wall. 50% of patients underwent initial resection, 60% of whom recurred after 1 year. Only one patient underwent additional radiotherapy. Initial surveillance was proposed in 50% of patients. 60% progressed after 2 years. Only one patient underwent surgical treatment combined with radiotherapy after an initial monitoring period of 9 years. Treatment with IMATINIB 400mg/d was initiated in 60% (n=6) of patients after progression or local recurrence. One patient received tamoxifen for one year. Results: Lesion stability was observed in 6 patients (60%), a partial response (left scapular) in 1 patient and local progression in 1 patient. Tolerability: Grade 3 neutropenia in 1 patient and Grade 2 liver toxicity. Conclusion : Desmoid tumours are rare tumours which progress slowly and are locally aggressive. Their prognosis is closely linked to their recurrence. The current therapeutic approach advocates a "WAIT AND SEE" strategy, particularly in asymptomatic, non-progressive forms. Surgery is only indicated if the immediate vital or functional prognosis is at risk.