[Gastric fibromatosis: a rare tumor in an infrequent location. Case report][article in Spanish]

Desmoid-type fibromatosis is an extremely rare benign tumor with locally aggressive features. It is predominantly developing in soft tissues due to its origin in muscles and aponeurosis. The diagnosis is established by immunohistochemistry with positivity for vimentin, B-catenin and sometimes for smooth muscle actin. The clinical spectrum of presentation is wide, based on this and resectability the treatment is offered. We present the case of a patient with symptoms of gastric outlet obstruction secondary to a subepithelial lesion in the gastric body with conclusive immunohistochemistry for gastric fibromatosis.

We report the case of a 37-year-old woman investigated for left flank pain 1 year after bariatric surgery (Roux-en-Y gastric bypass). Abdominal computed tomography (CT) revealed a solid intra-abdominal lesion measuring 9.3 ? 9.4 ? 10.4 cm, compressing adjacent structures with no signs of invasion. Ileocolectomy with partial mesenteric resection was performed. A histopathological and immunohistochemical analysis confirmed the diagnosis of mesenteric desmoid tumor.

A 40-year-old man with no previous history of abdominal surgery or noteworthy family history presented to our hospital because of a palpable abdominal mass. Abdominal CT revealed a 9 cm diameter mass in the mesocolon. The differential diagnosis included desmoid tumor, and right hemicolectomy with partial resection of the pancreas head and duodenum was performed. Pathologically, the tumor cells were negative for S-100, c-kit, CD34, and desmin but partially positive for a-SMA and slightly for b-catenin. From these findings, desmoid tumor of the mesocolon was diagnosed. Invasion of the pancreas was also found. In this paper, we report a rare case of resected sporadic desmoid tumor in the mesocolon with pancreatic invasion, together with a review of the literature.

In 2001, a Statement was published that described indications for intestinal transplantation in patients with intestinal failure expected to require parenteral nutrition indefinitely. Since 2001, advances in the management of intestinal failure including transplantation and patient survival, both on extended parenteral nutrition and after transplantation, have improved, leading to a reduction in the number of intestinal transplants worldwide from a peak of 270/yr. in 2008 to 149 /yr. in 2017. These changes suggest that the original 2001 Statement requires reassessment. All patients with permanent intestinal failure should be managed by dedicated multidisciplinary intestinal rehabilitation teams. Under care of these teams, patients should be considered for intestinal transplantation in the event of progressive intestinal failure-associated liver disease, progressive loss of central vein access, and repeated life-threatening central venous catheter-associated infections requiring critical care. Additional indications for transplantation include large desmoid tumors and other intra-abdominal tumors with reasonable expectation of posttransplant cure, extensive mesenteric vein thrombosis and intestinal infarction, total intestinal aganglionosis, and nonrecoverable congenital secretory diarrhea. Quality of life typically improves after successful intestinal transplantation and may support the decision to proceed with transplantation when other indications are present. However, the requirement for life-long immunosuppression and its associated side effects preclude intestinal transplantation if motivated only by an expectation of improved quality of life. Increasing experience with intestinal transplantation and critical appraisal of transplant outcomes including graft survival and patient quality of life together with potential advances in immunosuppression can be expected to influence transplant practices in the future.

Herein, we present a 54 year-old male patient who had been previously operated on because of fibromatosis in our clinic and recurred 18 months later with recurrence. As the recurrent tumor infiltrated the chest wall in full layer, the tumor and chest wall including four ribs was en bloc resected.

In this article, we present a case we had
encountered: a Desmoid tumor developing in a patient with a history of GIST 3
years ago. It is the first case of GIST with subsequent development of Desmoid
tumor to be reported in Saudi Arabia. We discuss the two entities of Desmoid
tumor and GIST by comparing their definitions, clinical presentations,
histological features, immunohistochemistry stains, molecular pathogenesis,
prognosis, and treatment. We also discuss the relationship between GIST and the
subsequent development of Desmoid tumors and compare our case with case reports
in literature.