What is the best front-line approach in patients with desmoid fibromatosis? – A retrospective analysis from a reference center

Desmoid fibromatosis (DF) is a locally aggressive, not metastasizing tumor associated with high local recurrence rates. Surgery was a standard-of-care for DF treatment; however, recently, conservative treatment and active surveillance are preferred. This study aimed to evaluate the real-life outcomes of DF treatment.

To assess the efficacy and safety of sandwich isolation surgery on the local control of progressive desmoid tumors involving neurovascular bundles. A total of 27 patients with progressive desmoid tumors at extremities involving neurovascular bundles who received surgery at our hospital between August 2014 and August 2018 were identified. A total of 13 patients received sandwich isolation surgery, in which R2 resection was performed in neurovasculature-involving regions, and a biomaterial patch was used to envelop involved neurovascular structures and isolate residual tumors. A total of 14 patients received traditional surgery, which included tumor resection without isolation procedure. In sandwich isolation group, tumor progressions and local recurrences occurred in 3 patients outside the isolated neurovasculature-involving regions. However, no progressions or recurrences occurred in any patients in the isolated neurovasculature-involving regions where R2 resection was performed. Sandwich isolation surgery group and traditional surgery group shared similar baseline clinical characteristics. The estimated 3-yr event-free survival rate was 76.9% after sandwich isolation surgery, and 32.7% after traditional surgery (P = .025). Patients who received sandwich isolation surgery were less likely to have local recurrence (hazard ratio: 0.257, P = .040). No complications were noted except intermittent mild pain in operative regions (2 cases). Sandwich isolation surgery is effective and safe for local control of desmoid tumors involving neurovascular bundles.

This paper describes a case of a large desmoid tumor on the abdominal wall that developed in the 10th week of pregnancy in a 29-year-old patient with no history of familial adenomatous polyposis. Ultrasound showed that the tumor had increased around 158% during pregnancy. The patient underwent Cesarean section during which a mass of around 20 cm was detected between the anterior and posterior layer of the aponeurosis of the rectus abdominis muscle. Conservative management (no intervention) was adopted with follow-up imaging. Ultrasound-guided core biopsy was performed four and a half months after delivery, with histopathology revealing a spindle cell neoplasm, with possible musculoaponeurotic fibromatosis. Six months after childbirth, the tumor had decreased spontaneously by 40.5% in thickness and nine months following delivery the patient continues to be monitored under expectant management, with no complaints or symptoms. Although rare, obstetricians should be aware of the possibility of a desmoid tumor during pregnancy and should evaluate the best management option (surgery, pharmacological treatment or expectant management) according to the patientÂ’s profile in order to avoid visceral complications, the need for extensive surgery with possible aesthetic and functional sequelae, and even iatrogenic complications.

fibromatoses (Desmoid-type) tumour is rare benign fibrous tumours account approximately for 0.03% of all neoplasms. The Pancreas origin of this tumour is even more rarely reported subset with around 5% only. Establishing the diagnosis is fundamentally based on characteristic pathological and immunohistochemical studies. Surgical resection of the tumour is the mainstay of intervention. We aim to report the success in cure a rare and the largest pancreatic desmoid-type intraabdominal fibromatosis.

We report a case of a 41-years-old female with a sporadic abdominal wall desmoid tumor. After a period of 12-months observation, chemotherapy was given with Doxorubicin and Dacarbazine to facilitate minimal surgery. The patient received 4 cycles of chemotherapy. Treatment was well tolerated and resulted in a significant reduction of the tumor size from 48mm to 25mm, 48% reduction. The tumor was surgically resected, and the histopathology from the resected specimen revealed a partial pathological response. At 6 months post-surgery, there is no recurrence or surgical complications.

Desmoid tumors (DT) are rare non-metastatic neoplasms that occur through myofibroblast proliferation in musculoaponeurotic or fascial structures of the body, being commonly diagnosed in young women during pregnancy or in the post-partum period. We present the case of a 38-year-old woman, who recently gave birth, manifesting non-specific abdominal symptoms. Computed tomography indicated the presence of a solitary tumor arising from the intestinal wall or from the mesentery. Surgery confirmed the diagnosis, revealing a tumor that was localized at the level of the jejunal mesentery, having about 7 cm in diameter, in tight contact with the duodenum and the mesenteric vessels. “En bloc” resection of the tumor was performed, together with the involved enteral loops followed by end-to-end anastomosis of the jejunum. Histopathological examination of the surgical specimen sustained the diagnosis of desmoid tumor.