Basic Knowledge in Soft Tissue Sarcoma

Sarcoma is rare and heterogenous with various subtypes having a different prognostic. Desmoid is a tumour with a local aggressiveness; GIST with KIT mutation responds massively to target treatment a IMATINIB, whereas soft tissue sarcoma and leiomyosarcoma are very aggressive with poor response to systemic therapies. Interventional radiology plays an important role in the diagnosis of sarcomas with image-guided percutaneous core needle biopsy being the most commonly used biopsy technique in the diagnosis of sarcomas. Surgery is a mainstay of sarcoma treatment; the resection can be large. Indeed, resection objective is R0 because quality of surgical margins impacts local control and survival. Radiotherapy is possible in neoadjuvant or in adjuvant treatment to improve local control rate. Recently radiotherapy enhancer injected percutaneously in soft tissue sarcoma has proven benefit in increasing the rate of R0 complete surgical resection. Several studies showed better local control rate linked with post-operative radiotherapy. The decision making to use local therapies is complex, depends upon diverse presentations and histologies, and should always be taken in a multidisciplinary discussion. Today, percutaneous image-guided treatments with ablation technologies (radiofrequency ablation, cryotherapy, microwaves ablation) provide high rate of durable local control for small-sized malignant deposit in many organs including lung, liver and bones. Sarcoma must be managed by multimodality treatment in expert reference centres. Such management has a considerable impact on the prognosis.

A retrospective, single-institution review identified 23 patients (ages 16-77) with extra-abdominal desmoid tumors who received CT-guided percutaneous cryoablation as either a first-line (61%) or salvage (39%) treatment in 30 sessions between 2014 and 2018. Median maximal lesion diameter was 69?mm (11-209). Intent was curative in 52% and palliative in 48%. Contrast-enhanced cross-sectional imaging was obtained before and after treatment in addition to routine clinical follow-up. Technical success was achieved in all patients. The median follow-up was 15.4 months (3.5-43.4). Symptomatic improvement was demonstrated in 89% of patients. At 12 months, the average change in viable volume was ?-80% (range -100% to ?+?10%) and response by modified response evaluation criteria in solid tumors (mRECIST) was CR 36%, PR 36%, and SD 28%. No rapid postablation growth or track seeding was observed. Four patients underwent repeat cryoablation for either residual or recurrent disease. Two patients sustained a major procedural complication consisting of significant neuropraxia. Cryoablation for desmoid tumors demonstrates a high degree of symptom improvement and local tumor control on early follow-up imaging with relatively low morbidity.

A girl aged 2.5 years presented to her general practitioner (GP) with a firm lump on her left jaw. The child had not noticed any change; however, her mother had noticed a swelling over the past week with no antecedent trauma. Tha patient did not complain of pain, mastication or airway compromise, paraesthesia or abnormal facial movements. She had a genetically confirmed past medical history and family history of familial adenomatous polyposis (FAP). Clinically she had a firm immobile swelling of the left mandibular body measuring 12 x 9 mm with no soft tissue induration or skin changes.

The use of Ultrasound may be useful to aid in radical resection of the desmoid tumor with adequate margins. In our 30-year-old patient with recurrent abdominal wall desmoid tumor, intra-operative ultrasound was utilized in successful resection of the tumor followed by a minimal but adequate margin. The patient has recovered well and within the 6 months follow-up period and has not reported any functional limitations or recurrence.

Between September 2010 and December 2017, 18 patients with abdominal wall tumors were collected. There were 11 males and 7 females, with an average age of 45.2 years (range 29-68 years). Histologic diagnosis included desmoid tumor in 8 cases, sarcomas in 6 cases, malignant teratoma in 2 cases, and colon adenocarcinoma in 2 cases. All abdominal wall defects were full-thickness defects. Peritoneum continuity was reconstructed with mesh; the lateral vastus muscular flaps were used to fill the dead space and rebuild the abdominal wall strength; the abdominal wall soft tissue defects were repaired with pedicled anterolateral thigh flaps. The size of abdominal wall defects ranged from 15 cm×6 cm to 25 cm×22 cm; the size of lateral vastus muscular flap ranged from 10 cm×8 cm to 22 cm×10 cm; the size of anterolateral thigh flap ranged from 14.0 cm×8.0 cm to 21.0 cm×8.5 cm. The bilateral pedicled anterolateral thigh myocutaneous flaps were harvested to repair the extensive abdominal wall defects in 2 cases. All wounds healed smoothly and all flaps survived totally. The mean follow-up time was 22.5 months (range 11-56 months). No tumor recurrence occurred, the abdominal function and appearance were satisfactory, no abdominal hernia was noted. Pedicled anterolateral thigh myocutaneous flap combined with mesh is fit for large full-thickness abdominal defect reconstruction.