Long-term diagnostic value of MRI in detecting recurrent aggressive fibromatosis at two multidisciplinary sarcoma centers

To assess the diagnostic accuracy of MRI in detecting recurrent aggressive fibromatosis (AF) during long-term follow-up at two multidisciplinary sarcoma centers. Seventy-nine patients from two sarcoma centers were included in this IRB-approved study and were examined postoperatively using 1.5-T MRI. MRI follow-up scans were reviewed for true-positive/-negative and false-positive/-negative results. Available pathological reports and MRI follow-ups were set as reference. The median age of the patients was 38.1 ñ 15.3 years. Of the patients 27.9 % showed recurrent AF lesions. The most common localizations of AF were the axilla/shoulder (n = 15) and the thigh (n = 11). From 498 postoperative MRI follow-ups, 24 true-positive, 16 false-positive, 6 false-negative, and 452 true-negative MRI follow-ups were identified. The overall sensitivity and specificity for detecting recurrent AF was 80 % and 97 %, respectively. There was no significant difference in the diagnostic accuracy at the two sarcoma centers. All false-negative results were found in small lesions. False-positive results mostly mimicked streaky (n = 10) and small ovoid/nodular (n = 5) lesions. The configuration of recurrent AF was significantly most often fascicular (50 %; p = 0.001-0.005). MRI shows a high long-term diagnostic value in detecting AF recurrences. Nevertheless, radiologists should pay close attention when lesions are small, as they may remain undetected. Although the configuration of recurrent AF is most often fascicular, recurrences may also appear in different shapes.

Here, we demonstrate a case of desmoid tumor revealed in tissue pathology. In this case, pelvic mass measuring 15 cm x 12 cm, was noted on abdominal computed tomography (CT). The mass was compressing Rt. ureter, causing Rt. hydronephrosis. The pre-op diagnosis was subserosal uterine leiomyoma on the basis of CT and laparoscopic exploration was done, we found huge intraligamental myoma adhered to Rt. ureter and bladder. Finally, desmoid tumor was diagnosed on permanent tissue pathology. It is a rare condition but should be considered in the differential diagnosis of pelvic mass.

The present review retrieved literature on treatment options for desmoids to summarize the latest treatment modalities and refine their efficacy, as well as their side effects, in order to provide a more comprehensive treatment reference for clinicians.

We present the case of a 60-year-old woman who presented with abdominal pain which began 2 months before the visit to our hospital. Clinical examination identified a tumor, round, mobile located in the epigastric region. A Computer Tomography of the abdominal confirmed the existence of a tumor at the level of the transverse colon. The patient underwent surgery, intraoperative exploration confirmed the existence of a round, smooth tumor which derived from the mesentery with apparent invasion in the transverse colon. A transverse colectomy was done in a single piece with the tumor which was dissected from the mesentery. The pathology report confirmed the mass was a primary desmoid tumor.

Pediatric head and neck desmoid tumors are rare neoplasms that can cause significant morbidity due to infiltration of vital anatomic structures. The goal of this study is to review presentation, evaluation, and management of these tumors. Retrospective study of children with head and neck desmoid tumors treated from 1999 to 2018 and literature review. 11 patients (5 boys, 6 girls) were included. Presentation included firm neck mass (n = 8), trismus (n = 2) and tongue lesion (n = 1). All patients had preoperative imaging with CT (n = 2), MRI (n = 1) or both (n = 8). Five patients underwent needle biopsy, five had open biopsy and one was diagnosed on pathology from primary excision. Seven patients were treated by primary surgical resection, with positive surgical margins in six cases due to proximity to vital neurovascular structures. None needed chemotherapy, had disease recurrence or progression. Three patients with unresectable disease were treated with chemotherapy. One patient was monitored with imaging without any treatment and did not have disease progression. Follow-up ranged from 6 months to 6 years (median 21 months). Ten patients (7 surgical, 2 chemotherapy, 1 observation) were either disease-free or had stable disease at last follow-up.

A man is his 30s had no significant past medical history, including no abdominal surgery. A medical check-up found a large tumor in the right lateral abdomen. After some examinations, a preoperative diagnosis of GIST was made, and open ileocecal resection was performed. However, the final diagnosis based on the pathological findings was desmoid-type fibromatosis. We should consider desmoid-type fibromatosis when we find a large abdominal mass, but it may be difficult to diagnose based only on imaging findings. Immunohistochemical examination of the specimen may make the diagnosis.