Magnetic resonance-guided focused ultrasound treatment of extra-abdominal desmoid tumors: a retrospective multicenter study

Abstract

Objectives To assess the feasibility, safety and preliminary efficacy of magnetic resonance-guided focused ultrasound (MRgFUS) for the treatment of extra-abdominal desmoid tumours. Methods Fifteen patients with desmoid fibromatosis (6 males, 9 females, age range 7-66 years) were treated with MRgFUS, with 7 patients requiring multiple treatments (25 total treatments). Changes in viable and total tumour volumes were measured after treatment. Efficacy was evaluated using an exact one-sided Wilcoxon test to determine if the median reduction in viable tumour measured immediately after initial treatment exceeded a threshold of 50% of the targeted volume. Median decrease after treatment of at least two points in numerical rating scale (NRS) worst and average pain scores was tested with an exact one-sided Wilcoxon test. Adverse events were recorded. Results After initial MRgFUS treatment, median viable targeted tumour volume decreased 63%, significantly beyond our efficacy threshold (P=0.0013). Median viable total tumour volume decreased [105mL (IQR=217mL) to 54mL (IQR=92mL)] and pain improved (worst scores: 7.5±1.9 vs. 2.7±2.6, P=0.027; average scores: 6±2.3 vs. 1.3±2, P=0.021). Skin burn was the most common complication. Conclusions MRgFUS significantly and durably reduced viable tumour volume and pain in this series of 15 patients with extra-abdominal desmoid fibromatosis.

Identification and assessment of health-related quality of life issues in patients with sporadic desmoid-type fibromatosis: a literature review and focus group study

“…n  = 2 pain with use of medicationTESS[28]14 (1) a UESGNA n  = 1 MSTS 50 (pain 3, function 2, acceptance 5, hand positioning 0, dexterity 5, lifting 0), TESS 62 a EORTC QLQ-C30[19]14AWSGNAMean global health status b : 97 (± 5.9); physical functioning 93 (± 11.1); role functioning 89 (± 16.7); emotional functioning 87 (± 19.1); cognitive functioning 94 (± 8.3); social functioning 93 (± 14.7)MDASI – [32]17ASGSINAMean symptom severity: partial responders ( n  = 5): 1.65 point improvement, stable disease ( n  = 5): 0.8 point improvement, no response/dropout ( n  = 7) 0.04 point improvementModified Johnstone scale – [34]40 (24)ASSGARTxCT n  = 24 amputation required (grade 0): n  = 1 (4%), severe functional deficit (grade 1): n  = 1 (4%), major functional limitations (grade 2): n  = 4 (17%), mild functional limitations (grade 3): n  = 7 (29%), no functional limitations (grade 4): n  = 9 (38%), NA: n  = 2 (8%) n  = 24 grade 0: n  = 5 (21%); grade 1: n  = 0; grade 2: n  = 6 (25%); grade 3: n  = 3 (13%); grade 4: n  = 8 (33%) not recorded: n  = 2 (8%)NRS – [37]15 (6)EAMRgFUSCTCA n  = 6 NRS 7.5 (± 1.9) (worst daily NRS); n  = 6 NRS 6 (± 2.3) (average daily NRS) n  = 6 NRS 2.7 (± 2.6) (worst daily NRS); n  = 6 NRS 1.3 (± 2) (average daily NRS) – [38]44ASSTMedian NRS 6 (IQR 2–7) group B: median NRS 7/10; n  = 7 moderate pain, n  = 17 severe pain; group C: n  = 8 severe pain c Group B n  = 12 (75%) pain improvement, n  = 1 (6%) pain worsening, n  = 3 (19%) stable symptoms; group C: n  = 8 (100%) pain relief c Other scoresFunctional outcome[43]21HN<...>…”

“…Functional scores like the DASH score [26], the Enneking score/MSTS [24, 27, 28, 30], the TESS [28], and the Johnstone scale [34] are used for extremity diseases but are not suitable for patients who have sites of disease other than the extremities. Symptoms scores including the MDASI score [32] and the NRS [37, 38] are quite specific for measuring the severity of symptoms, and could be useful in combination with HRQL tools measuring issues like emotional or social well-being. The EORTC QLQ-C30 [19] is designed to cover issues relevant for cancer patients and may be a good generic measure to be completed by an item list consisting of the key DTF-specific issues identified in our focus groups, in order to create a more holistic perspective of HRQL issues in patients with DTF.…”

Concurrent Imatinib and Radiation Therapy for Unresectable and Symptomatic Desmoid Tumors

“…For example, we have employed noninvasive treatment modalities such as MR-guided high-intensity focused ultrasound to avoid surgery for extremity desmoid tumors [4, 5]. We consider surgery and/or radiation therapy for desmoid tumors that are symptomatic and not responsive to medical therapy.…”

Effect of Sonication Duration and Power on Ablation Depth During MR‐Guided Focused Ultrasound of Bone

“…The technique is US Food and Drug Administration (FDA)‐approved for treatment of uterine fibroids, palliation of bone metastases, and essential tremor . In addition to these indications, MRgFUS is actively used for a wide range of focal musculoskeletal conditions, including benign bone tumors (eg, osteoid osteomas), soft‐tissue tumors (eg, desmoid tumors), and joint pain, for example, lower back pain secondary to facet joint arthropathy …”