Large-sized desmoid-type fibromatosis presented in left nuchodorsal region and managed with surgical excision: A case report and review of literature

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.

We evaluated 41 patients with desmoid tumors treated with 49 radiotherapies between 1987 and 2012. Radiotherapy is a valuable option for treating desmoid tumors. Radiotherapy dose appears to be significantly associated to local control.

The targeted nuclease revolution (TALENs, CRISPR/Cas9) now allows Xenopus researchers to rapidly generate custom on-demand genetic knockout models. These novel methods to perform reverse genetics are unprecedented and are fueling a wide array of human disease models within the aquatic diploid model organism Xenopus tropicalis (X. tropicalis). This emerging technology review focuses on the tools to rapidly generate genetically engineered X. tropicalis models (GEXM), with a focus on establishment of genuine genetic and clinically relevant cancer models.

A 49-year-old Pakistani male presented with “heaviness” in his chest. Chest radiograph and computed tomography (CT) confirmed a massive left-sided pleural-based opacity. Histopathology and immunohistochemistry of the resected tumour were consistent for a desmoid tumour. He was followed up for 9 months with no evidence of tumour recurrence. Predominantly pleural-based desmoid tumour is rare but should be included in the differential diagnosis of spindle cell tumours.

Efficacy of liposomal doxorubicin (LD) in treating desmoid fibromatosis (DF) in children has not been well evaluated. This retrospective case series examines five children with progressive DF, treated with LD. We report progression-free intervals (PFIs) and radiographic as well as clinical responses for each medication received. LD was well tolerated, with an average 4.5% reduction in tumor size and median PFI of 29 months. Treatment with LD conferred the longest PFI of all medical therapies pursued. Thus, LD is an important treatment option for DF in pediatrics.