Aggressive Fibromatosis of the Left Mesocolon Mimicking a Gastrointestinal Stromal Tumor: A Case Report

Mesenteric fibromatosis (MF) is a proliferative fibroblastic lesion of the intestinal mesentery. It constitutes 8% of all desmoid tumors, representing 0.03% of all neoplasms. It is benign histologically, although it could infiltrate locally and recur following excision; however, it is free from the potential to metastasize. It is spontaneous or associated with familial adenomatous polyposis (F.A.P.]) mutation as a part of Gardner’s syndrome. This case report discusses the radiological, intraoperative, and histopathological findings from a 45-year-old male patient who presented with abdominal pain and a palpable mass in the left hemiabdomen. The pain was dull and aching, extending to the back and unrelated to any other gastrointestinal symptoms. There was no history of severe weight reduction. Furthermore, he is not a smoker. There were no comorbidities, severe medical diseases, or prior surgical procedures. Computerized tomography revealed a well-defined, lobulated, heterogeneously enhancing altered signal intensity mass at the mesocolon. Ultrasonography of the abdomen showed an intra-abdominal mass. Macroscopic mass characteristics include a well-defined mass measuring 22 × 14 × 11 cm connected to a small intestine segment measuring 21 × 2 × 2 cm. Histopathological and immunohistochemical examinations of the resected tumor, including positive nuclear immunostaining for beta-catenin, confirmed a postoperative diagnosis of desmoid-type fibromatosis. Based on its clinical presentation and computed tomography results, this case demonstrated how desmoid-type fibromatosis of the colon might mimic gastrointestinal stromal tumors (GISTs). Due to the varied therapies and follow-up methods used for these lesions, the differential diagnosis between desmoid-type fibromatosis and GIST is clinically significant.

Introduction: Desmoid fibroma (DF) is a disorder characterized by strong clonal proliferation of myofibroblasts and fibroblasts. We describe a case of DF that mimicked a breast tumor, along with a review of the literature on the clinical manifestation, diagnostic process, and course of therapy for this combative disease.

Case report: A 34-year-old female patient with breast lump at the junction of the upper quadrants of the left breast. After the diagnosis of DF, it was decided to perform a sectorectomy of the left breast associated with post-quadrant reconstruction, with immunohistochemistry and findings compatible with DF.

Discussion: Clinically manifests as a solid mass that is often painless and occasionally adherent to the chest wall. A treatment strategy should be idealized for each patient. Thus, there is the possibility of performing radical surgery for resection and/or radiotherapy, and surgery may be followed by radiotherapy.

Capillary leak syndrome is a rare life-threatening disorder of acute endothelial hyperpermeability. It consists of initial fluid extravasation resulting in hypotension, hypoalbuminemia, and hemoconcentration, followed by noncardiogenic pulmonary edema from rapid fluid remobilization into intravascular compartment. Drug-induced etiology is an important diagnostic consideration in cancer patients, particularly with use of antimetabolites, immunostimulants, and monoclonal antibodies. Sorafenib-mediated capillary leak syndrome has never been reported. Here, we present the case of a 29-year-old female patient with a desmoid tumor of the thigh, who was admitted for acute hypoxic respiratory failure after recent initiation of sorafenib. She was found to have extensive pulmonary edema, bilateral pleural effusions, and hemoconcentration, all of which stabilized on supportive care with noninvasive mechanical ventilation and intravenous diuresis. Her infectious and cardiac work-up were negative. Given the temporal relationship between sorafenib use and symptom onset as well as a lack of an alternative etiology of her findings, patient was deemed to have sorafenib-induced acute capillary leak syndrome. Importantly, she did not become hypotensive prior to or during this hospitalization. To our knowledge, we reported for the first time an atypical presentation of acute capillary leak syndrome due to sorafenib use without hemodynamic instability.

Traumatism and surgery are often found in the medical history of patients with sporadic desmoid tumors, as the recent article of the Mannheim Sarcoma Team has shown for 38.3% of patients. Still, it is not possible to establish a direct causality. Decoding the host environment can provide a more precise definition of any causal relationship.

Introduction: Aggressive fibromatosis (AF) is a fibroblastic locally aggressive neoplasm arising from the musculoaponeurotic stroma and has no metastatic potential. The high tendency of recurrence despite complete surgical resections makes the management of the condition onerous. It can result in significant morbidity with major functional loss due to the destruction of adjacent vital structures and organs. AF with hip flexion contracture is a very rare occurrence.

Case report: A 20-year-old male presented with recurrent abdominal AF with severe hip flexion contracture and an unresectable tumor. He underwent deformity correction and he maintains the full correction achieved along with very good functional improvement at the end of 4 years.

Conclusion: This case demonstrates that in a case of AF with an unresectable tumor, good functional outcome can be obtained; it can be maintained over the short term following contracture release with soft-tissue coverage surgery along with chemotherapy with sorafenib.

The patient was a 27-year-old man. He was referred to our hospital because he was aware of a mass in his abdomen. An abdominal ultrasound showed a 70-mm mass lesion. Enhanced computed tomography showed a 70-mm mass with well- defined margins and heterogeneous internal enhancement near the proximal jejunum. The patient was diagnosed with a suspected primary submucosal tumor of the duodenum or small intestine, and surgery was planned to diagnose and treat the tumor. The tumor was located in the upper jejunal mesentery, and tumor resection and partial small bowel resection were performed. Histopathological examination revealed proliferation of spindle-shaped cells without karyomitosis, and mixed collagen fibers in the tissue. Immunohistochemistry showed β-catenin(+), SMA(+), AE1/AE3(-), KIT(-), CD34(-), and S-100(-). Based on these findings, we diagnosed primary desmoid fibromatosis of the small intestinal mesentery. In this report, we describe a case of primary desmoid fibromatosis of the small intestinal mesentery with a review of the literature.