Desmoid tumors: who, when and how to treat?

PURPOSE OF REVIEW: The objective of this article is to summarize new treatment strategies of desmoid tumors. RECENT FINDINGS: Desmoid tumor has an unpredictable evolution that may spontaneously regress or stabilize. A shift toward an initial frontline active surveillance has been acknowledged by experts. Surveillance monitoring should be performed frequently after the diagnosis to avoid missing a significant progression and then spaced in case of stabilization. Treatment is based on significant tumor growth or symptoms. Recent guidelines recommend commencing medical treatment. Kinase inhibitors and cytotoxic agents are the two classes of drugs where studies included progressive desmoid tumors and should be selected to guide medical practice. In a randomized trial, 2 years progression-free survival (PFS) was significantly better in the sorafenib group (81 versus 36% in the placebo group). In another randomized phase 2, 6 months PFS was 83.7% with pazopanib versus 45% with methotrexate and vinblastine. In a retrospective study, including progressive desmoid tumors, methotrexate + vinca alkaloids achieved 75 months median PFS. Cryotherapy is an alternative option in desmoid tumors with compatible locations and tumor sizes. Following medical treatment or cryotherapy failure, superficial sites represent the best indications for surgery in cases of continuous progression. In the event of a contra-indication or failure of medical treatment, in locations where surgery would be mutilating and incomplete, radiotherapy is an effective option. SUMMARY: Active surveillance with planned imaging has become the first-line management in desmoid tumor.

Two Different Types of Sarcoma Occurring Synchronously: The Impact of Molecular Biology on Therapeutic Strategy.

Sarcoma is a rare type of tumor that can arise in the different types of connective tissues. Symptoms vary depending on the size, type, and location of the tumor. Management and surgery should be performed in a referral sarcoma center with a molecular biology platform and a dedicated medical staff. A preoperative percutaneous core needle biopsy (CNB) is required to tailor the medical and surgical strategies. In this report, we describe the case of a well-differentiated retroperitoneal liposarcoma (WD LPS) discovered in the context of recurrent cystitis on an abdominal CT scan, synchronously occurring, with another different type of sarcoma, undifferentiated pleomorphic sarcoma (UPS) developing in the thigh, discovered because of sciatic pain. This extremely rare condition was confirmed by the molecular analysis and justifies a specific strategy taking account of the differential risk. This event should also encourage genetic counseling.

Desmoid Tumors Arising on the Mesenteric Surgical Scar of Abdominal Sarcomas.

A sporadic desmoid tumor (DT) is a rare type of tumor of the mesenchymal connective tissues is now considered an intermediate disease or locally aggressive. They may develop on scars or after traumatism, favored by growth factors released during the initial phase of wound healing. Most of the abdominal DT arising on a scar is described on the wall incision. In this report, we describe two cases of DT arising on the intraperitoneal surgical scar, shortly after the resection of a low-grade retroperitoneal liposarcoma and a low-risk gastric gastrointestinal stromal tumor (GIST). Inconsistency between low risk according to the classification of the primary sarcoma and early local recurrence (LR) should raise the possibility of DT. Core needle biopsy (CNB) should be performed when it is feasible, including on local recurrences (LR). Surveillance has become the first-line treatment for DT. In case of progression between two imaging during the surveillance phase, surgery, when it's not mutilating, is indicated for selected cases as second-line treatment.

Laparoscopic Sleeve Gastrectomy for Adolescents Under 18 Years Old with Severe Obesity.

INTRODUCTION: Laparoscopic sleeve gastrectomy (LSG) is a widely accepted stand-alone bariatric operation. Data on adolescent patients undergoing LSG are limited. The aim of this study was to demonstrate that LSG is safe and effective for patients strictly under 18 years old with severe obesity. METHODS: Prospectively collected data from consecutive patients undergoing LSG were retrospectively analyzed. Patients with more than 1-year follow-up were included in the analysis for weight loss and comorbidity evaluation. Quality of life (QoL) was evaluated using the Short-Form 36 questionnaire. RESULTS: Eighty-four patients under 18 years old (range: 15-17 years) underwent LSG. Median weight was 128 kg and median body mass index (BMI) 43.7 kg/m2. Median duration of surgery was 68.5 min. One major complication was recorded: a patient developed severe pneumonia that necessitated ventilatory support in intensive care unit and intravenous antibiotic treatment. Mortality was null. Median length of hospital stay was 4 days. Six, 12, and 24 months after LSG, median BMI decreased significantly to 34.3, 29.8, and 28.8 kg/m2, respectively (p < 0.001), with a mean percentage of total body weight loss of 29.1% at 2 years. Obesity-related comorbidities improved at 1 year, while all SF-36 scale scores of QoL assessment improved significantly. CONCLUSION: This study suggests that LSG is safe and effective for patients under 18 years old, resulting in significant weight loss, comorbidity remission, and QoL improvement. Careful patient selection after adequate risk versus benefit evaluation by an expert multidisciplinary team is essential.