Desmoid tumor of the abdominal wall: A case report and literature review

A 31-year-old Caucasian woman with a mass in the middle third of the left rectus abdominal muscle, which has risen for about a month. An ultrasound and an MRI of the abdomen pose the suspicion of a desmoid tumor of the abdominal wall. Indication is given to the surgical treatment of lesion enucleation and reconstruction of the muscular planes without the aid of prosthetic networks. The follow-up performed at 1 year did not show any relapse.

The characteristics of desmoid tumors(DTs) associated with familial adenomatous polyposis(FAP) and relationships between the development of DTs and the sites of APC germline mutation have not closely been examined in Japan. This retrospective study was performed to address these issues by examining patients with FAP who underwent proctocolectomy between 1981 and 2015. The frequency of DTs development and genotype-phenotype relationship of FAP patients seems to concur with those reported from Western countries. Since the DOX plus DTIC therapy for severe DTs is valid but toxicity is high, the development of less toxic regimens are warranted.

To evaluate the efficacy and safety of doxorubicin (ADM) combined with thalidomide (THA) as a first-line treatment for patients with refractory aggressive fibromatosis (AF). No patient had a complete response, but five patients had partial responses, resulting in a RR of 33%. Eight patients (53%) had stable disease and two patients (13%) had progressive disease, and the disease control rate was 87%. ADM plus THA was well-tolerated and effective as a first-line treatment for patients with refractory AF. However, patients with hypoalbuminemia at baseline had inferior clinical outcomes, and further studies are needed to investigate this issue.

We report the case of a 57-year-old man presenting with a desmoid tumor arising from his chest wall. By computed tomography-guided biopsy, he was diagnosed as having a desmoid tumor. He underwent right-sided chest wall resection and reconstruction.

Treatment of recurrent aggressive fibromatosis (AF) following surgical resection is a clinical challenge. Non-steroidal anti-inflammatory drugs(NSAIDs) have been reported to be an effective option for controlling the disease. However, long-term NSAID use can result in unfavorable complications. This study was a trial of the use of intralesional steroid injection (ILSI) including investigation of safety margins and clinical outcomes of high-dose steroids for local use treatment of AF. A prospective cohort study was conducted to evaluate the safety and efficacy of particulate corticosteroids for AF. Intralesional steroid injections of Kanolone(R) guided by ultrasound were given monthly for three consecutive months with 1 mg/kg/episode (a total of 3 mg/kg). Eight recurrent AF patients completed the full 6-month evaluation process. The highest number of complication events, all of which were mild or detectable only by laboratory analysis, occurred during the month following the second injection. Tumors were stabilized in 83.3% of the cases during the study period, and pain and functional ability scores improved significantly. Intralesional steroid injection appears to be a safe and effective alternative treatment for recurrent AF.

Infantile desmoid fibromatosis of the postcricoid area is a rare disease and is characterized by a proliferation of fibrous tissue with non-metastasis, local infiltration, and a high rate of recurrence after surgical resection. Currently, ultrasound is scarcely used in the hypopharynx and larynx area. A 4-year-old boy presented with hoarseness, deep voice and snoring for 2-4 years without any surgical history. On sonography, the lesion was found in the postcricoid area, and the left larynx showed impaired mobility in real time observation. Complete excision with a negative margin in this pivotal anatomic area is impossible, and necessitates a long-time surveillance. Local excision was carried out to relieve the upper airway narrowing. Relieved hoarseness and snoring were reported on the latest follow-up. A residual lesion was seen in the surgical bed and maintained a stable extent on ultrasound and MR imaging after a year. Considering the non-radiation merit and diagnostic ability, ultrasonography is advocated as a valuable supplementary imaging method to CT, MR and laryngoscopy in the juvenile larynx and hypopharynx.