Laparoscopic excision of abdominal wall desmoid tumor.

Open surgical resection is the mainstay treatment for desmoid tumors. Laparoscopic resection is rarely used and not well described in the literature. We report a case of a single, 35-year-old woman who presented with palpable abdominal wall desmoid tumor. The patient had had laparoscopic cholecystectomy 2 years earlier, and the tumor was at the insertion site of the right upper quadrant trocar. The patient underwent successful laparoscopic resection of the tumor. This report aimed to promote laparoscopic resection of abdominal wall desmoid tumors, whenever feasible, and describe the laparoscopic technique.

A new World Health Organization (WHO) Classification of Tumors of the Pleura has recently been published. While the histologic classification of pleural malignant mesothelioma remains the same in the 2015 WHO classification as it was in the 2004 classification, multiple new observations have been recorded. Desmoid type fibromatosis, a locally aggressive tumor that can present in the pleura, has been shown to frequently have CTNNB1 gene mutations and express β-catenin by immunohistochemistry.

We report a case of fibromatosis involving the left infratemporal space in a 35-year-old female who presented with chief complaint of limited mouth opening for the preceding 4 years.

In this study, we isolated three kinds of desmoid cell with different CTNNB1 status, and compared their characteristics.

CTNNB1 gene mutations are the molecular key events in the majority of sporadic desmoid-type fibromatosis (DF). The specific S45F mutation has been reported to be associated with a more aggressive clinical course in DF. For the current study, the CTNNB1 mutation status was analyzed in DF samples from the prospective German Interdisciplinary Sarcoma Group (GISG) phase 2 study evaluating imatinib to induce progression arrest in DF Response Evaluation Criteria In Solid Tumors (RECIST) progressive patients. Mutations at position S45 were overrepresented in the GISG-01 trial recruiting RECIST progressive patients only. The positive correlation of CTNNB1 mutation status with the progression arrest rate after imatinib therapy supports the idea of a potential predictive impact of the mutation status on DF treatment decision making.

The authors report a rare case of small bowel obstruction and perforation secondary to sporadic, synchronous intra-abdominal DTs in a 54-year old man with atypical symptoms and no risk factors or family history. In the non-acute setting patients with sporadic intra-abdominal DTs should be managed in a specialist sarcoma unit by a multidisciplinary team. In the presence of FAP or other polyposis syndromes patients with DTs should be managed at a specialist colorectal unit. Emergent presentations require emergency surgery in suitable candidates.