Sporadic desmoid tumors in the pediatric population: A single center experience and review of the literature.

Data were retrospectively collected from 17 children/adolescents treated for sporadic desmoid tumors at a tertiary pediatric hospital in 1988-2016. After a median follow-up of 3.3years, 10 patients were alive in complete remission, 5 had stable disease, and 2 had reduced tumor size. Five-year overall survival was 100%, and event-free survival, 87.5%.Pediatric desmoid tumors are rare, with variable biologic behavior and morbidity. Treatment requires a multidisciplinary approach.

Since December 2014, 14 magnetic resonance-guided focused ultrasound (MRgFUS) treatments for desmoid tumors were performed at our institution in seven patients. MRgFUS is an effective treatment for desmoid tumors of the thigh with a favorable side effect profile, allowing for repeated treatments if necessary. Ablation size and safety can be improved with far-field coupling devices, careful patient positioning, and optimized sonication planning.

Several reports of desmoid tumors arising in the vicinity of the fibrous capsule of a breast implant have been described, but to date, the authors are not aware of any published cases following autologous breast reconstruction. This report describes a desmoid tumor developing after a muscle-sparing free transverse rectus abdominis musculocutaneous (TRAM) flap for breast reconstruction and subsequent surgical management.

There is no report from Iran about the treatment of this tumor (aggressive fibromatosis), and all published articles are single-case reports, therefore in this study, we report our experience from two of the largest referral centers of the South of Iran. During five years (2007-2011), among more than 20000 surgical pathology specimens, 25 cases of fibromatosis were identified. Clinicopathologic findings of desmoid tumor in Iran are very similar to other countries, however, there is still much controversy about the method of treatment for fibromatosis, and there are many challenges for patients, regarding multiple episodes of recurrence and the infiltrative aggressive nature of fibromatosis.

Desmoid-type fibromatosis is defined as an intermediate tumor that rarely occurs in the nuchodorsal region. There is no doubt as to the value of complete surgical excision for desmoid-type fibromatosis. However, surgeons may often be concerned about making a wide excision because of the potential for functional morbidity. Some studies have reported a lack of correlation between margin status and recurrence. We report an unusual case of a 55 y old male who prior to presenting at our hospital underwent debulking surgery. We performed a nuchodorsal tumor resection and achieved incomplete resection. We also reviewed available literature of desmoid-type fibromatosis. We described successful treatment for the refractory case of desmoid-type fibromatosis. The review results showed that some microscopic incomplete resections of tumors in patients with desmoid-type fibromatosis tended to be acceptable with surgical treatment.

According to their anatomical position, desmoid-type fibromatoses can be divided into three groups: extra- abdominal, intra-abdominal, and fibromatoses of the abdominal wall. Mesenteric fibromatoses account for 8% of the intra-abdominal ones. The latter are characterized by myofibroblastic proliferation and infiltration of both the pelvic and abdominal organs. We report a 26-year-old woman who complained of abdominal enlargement, feeling of heaviness, discomfort and reflux, which symptoms dated back 1-2 months prior to hospitalization. The patient underwent laparotomy due to subocclusive symptoms. Intraoperatively, a tumor sized 30 cm in diameter was found. The tumor originated from the mesentery and infiltrated in the jejunum, the entire ileum, and part of the caecum with perforation towards the abdominal cavity. The histological and immunohistochemical examinations are important for clarification of the diagnosis. The treatment requires a multidisciplinary approach, in which the surgical method has the key role.