Optimal radiotherapy strategy for primary or recurrent fibromatosis and long-term results

Herein, we describe our updated institutional experience with radiotherapy(RT) to treat fibromatosis. Forty-seven patients with fibromatosis received RT between 1990 and 2015, and were followed for 12+ months. Eight patients received RT for gross tumors, and 39 received postoperative RT after single/multiple prior surgeries. Seven recurrences were noted, including 2 in-field, 4 marginal, and 1 out-field, after a median follow-up of 60 months. On multivariate analysis, a CTV margin 5 cm and dose >45 Gy were significant predictors of non-recurrence (p = 0.039 and 0.049, respectively). Subgroup analysis showed that patients with both an CTV margin 5 cm and a dose >45 Gy showed a favorable outcome. RT is a valuable option for treating aggressive fibromatosis; doses >45 Gy and a large field produce optimal results. For in-field control, a higher dose is more necessary for gross residual tumors than for totally excised lesions.

To determine the outcome after radiation therapy for desmoid fibromatosis. A retrospective review of 50 patients treated between 1988 and 2016 in a specialised bone and soft tissue tumour clinic. Eleven patients (22%) developed progressive disease after radiation therapy at a median time of 41 months (range 12-113 months). The recurrences were within the radiation therapy field in four patients and outside the field in seven patients. One patient developed a radiation-induced malignancy 20 years after treatment. Radiation therapy is an alternative treatment in the management of desmoid fibromatosis. It should be considered in patients for whom surgical resection is not feasible, or as adjuvant therapy after surgery with involvedmargins where any further recurrences would cause significant morbidity.

One of the commonly used systemic agents for the treatment of aggressive fibromatosis(AF) is the anti-oestrogen drug tamoxifen. However, data on efficacy and optimum methods of response assessment are limited, consisting mainly of small case series and reports. A retrospective database was used to identify consecutive patients diagnosed with AF and treated with tamoxifen plus/minus non-steroidal anti-inflammatory drugs at our tertiary referral centre between 2007 and 2014. No relationship was identified between symptomatic benefit and response by RECIST 1.1 on MRI. Prospective studies in AF should incorporate endpoints focusing on patient symptoms.

Here we present a rare case of a large mesenteric desmoid tumor secondary to familial adenomatous polyposis (FAP) in a 34-year-old man accepted the ex vivo resection, and intestinal autotransplantation. An en bloc resection (R0 resection), and an ex vivo resection followed by intestinal autotransplantation was performed. The patient was discharged from the hospital on the 25th day after the operation, and was regularly followed up after surgery with abdominal ultrasonography and laboratory-biochemical tests every month, and serial CT scans every 3 months which showed no evidence of tumor recurrence, thrombus, intestinal obstruction or abdominal infection so far. An ex vivo resection and intestinal autotransplantation appear feasible for cases with pathological lesions involving the vessels at the root of mesentry, and represents an attractive alternative for the management of mesenteric desmoid tumors.

We describe the diagnosis and successful treatment of a case of hand-foot-skin reaction in a child taking sorafenib for an unresectable desmoid tumor.