Primary desmoid tumor of the mesentery with invasion in the transverse colon: A case report (FULL ARTICLE)

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.

Desmoid tumors (DTs) are a rare and biologically heterogeneous group of locally aggressive fibroblastic neoplasm: their biological behavior spectrum ranges from indolent to aggressive tumors. DTs are classified as intra-abdominal, extra-abdominal, and within the abdominal wall lesions. It is well known that abdominal and extra-abdominal DTs are associated with familial adenomatous polyposis (F.A.P.) and Gardner syndrome. Possible risk factors are prior trauma/surgery, pregnancy, and oral contraceptives.There was a real revolution in the management of DT: from aggressive first-line approach (surgery and radiation therapy) to a more conservative one (systemic treatment and “wait-and-see policy”). In these clinical settings, radiologists play an important role for assessing lesion resectability, evaluating recurrence, monitoring the biological behavior if an expectant management is chosen, and assessing response to systemic treatment as well as to radiation therapy. Awareness of common locations, risk factors, and imaging features is fundamental for a correct diagnosis and an adequate patient management.

We report a case of a 45 year’s old woman with a radial nerve entrapment caused by a desmoid tumor involving the right forearm. This case is outstanding because of the posterior interosseous nerve is entrapped inside the tumor. The marginal excision of the mass while preserving the intratumoral radial nerve was laborious. The radial nerve entrapment caused by a desmoid tumor is very rare and intratumoral radial nerve entrapment is also exceptional. The surgical treatment was challenging and the prognosis is uncertain because of the high rates of recurrence of the desmoid tumor.

This report presents a case of rare right shoulder desmoid type fibromatosis in a 48-year-old male that was missed on an initial workup including EMG/NCS and shoulder MRI, and demonstrates the importance of revisiting the diagnostic process if a former workup has yielded an unclear clinical picture.