Radiological evaluation of deep soft tissue fibromatosis, the characteristic MR criteria on conventional and corresponding diffusion-weighted images (FULL ARTICLE)

The lesions were growing along the musculoaponeurotic fascia, mostly invaded the muscles, and showed ill-defined margins, low T2 signal bands and areas, and facial tail sign. Diffusion images showed mostly high or high mixed with low signal; only 2 lesions showed a persistent low signal. Post-contrast and DWI detected synchronous lesions and extensions missed on T1 and T2 images. The most frequent MR features of deep fibromatosis are low T2 signal bands and areas, fascial tail sign, ill or partially defined margins, and predominant restricted diffusion pattern in addition to areas of “T2-blackout effect.” Post-contrast and DWI are more valuable in local staging of the tumor.

We reviewed the clinical data from 16 patients who were diagnosed with intra-abdominal aggressive fibromatosis and were admitted to Peking Union Medical College Hospital between March 1983 and September 2018. Among the 16 patients, 11 patients presented with a hard smooth abdominal mass with clear borders and a diameter of 4.3-25.0 cm. Six patients had a history of abdominal surgery and 3 patients had a history of familial adenomatous polyposis. Computed tomography imaging revealed a slightly dense mass with mild-to-moderate enhancement. Of all the 16 patients, 11 patients underwent surgical treatment and no recurrence occurred in 10 case after complete resection while recurrence occurred in 1 case after partial resection. Two patients underwent surveillance and 3 patients received cytotoxic drugs treatment, and no disease progression was observed via imaging during their follow-up.

A 68-year-old man who was on treatment for pulmonary Mycobacterium avium complex complained a worsening of sputum. Although he archived negative sputum culture two months ago, sputum culture tests revealed the newly isolation of Mycobacterium abscessus repeatedly. Chest computed tomography showed newly-appeared extra-pulmonary mass lesion in contact with a cyst at the bottom of his right lung. From the results of contrast-enhanced magnetic resonance imaging, we first suspected loculated pleural effusion due to Mycobacterium abscessus infection. A thoracoscopic examination was performed as the right pneumothorax developed, and the pleural lesion was successfully resected and diagnosed as an intrathoracic desmoid tumor. Intrathoracic desmoid tumor is very rare, and this is the first report of a case with pulmonary Mycobacterium abscessus disease.

Desmoid tumors of the small bowel wall are rare tumors of the gastrointestinal tract. The signs and symptoms ranging from non-specific to severe abdominal pain to the sensation of a mass and abdominal fullness. We present the case of a 48-year-old man who presented 3 years post-one-anastomosis gastric bypass (mini-gastric bypass) with vague abdominal pain and early satiety of one-month duration. A CT scan of the abdomen revealed a well-defined compressive mass in the left hypochondrium. A laparoscopic exploration was performed, but it was converted into a laparotomy due to the huge size of the mass. The desmoid tumor at the gastrojejunal anastomosis was resected, followed by a Roux-en-Y reconstruction. The patient’s postoperative course was uneventful, and he was discharged on the seventh day post-operation. He did not show any signs or symptoms suggestive of complications or recurrence during his follow-up.

The management of desmoid-type fibromatosis has considerably evolved these last years, toward first-line active surveillance then systemic or local symptomatic treatment in case of aggressive tumor. Magnetic resonance imaging is the modality of choice in each of these treatment settings. It needs multiparametric approach taking into account mainly the tumor size, and T2-weighted signal that is correlated with histological composition and clinical behavior. A volumetric approach should be favored for the evaluation of tumor size change. The interest of paramagnetic contrast injection and tumor enhancement requires further investigation.

We present herein an evidence-based, joint global consensus guideline approach to the management of this disease focussing on: molecular genetics, indications for an active treatment, and available systemic therapeutic options. This paper follows a one-day consensus meeting held in Milan, Italy, in June 2018 under the auspices of the European Reference Network for rare solid adult cancers, EURACAN, the European Organisation for Research and Treatment of Cancer (EORTC) Soft Tissue and Bone Sarcoma Group (STBSG) as well as Sarcoma Patients EuroNet (SPAEN) and The Desmoid tumour Research Foundation (DTRF). The meeting brought together over 50 adult and pediatric sarcoma experts from different disciplines, patients and patient advocates from Europe, North America and Japan.