A giant mesenteric fibromatosis involving the muscular layer of the colon wall: A case report

We report a case of giant Mesenteric fibromatosis (MF) in abdominal cavity with abdominal distention as the main symptom. A 26-year-old male presented with 2-month history of abdominal distention, lack of appetite, and symptoms grew progressively more debilitating with time. This patient underwent a contrast-enhanced computed tomography scan which showed a giant (37?×?25?×?13?cm), inhomogeneous enhancing, well-defined, and soft tissue density mass in abdominal cavity, possibly arising in mesocolon, which suggested a high possibility of MF. Exploratory laparotomy was performed, which revealed a large mass involving the transverse colon wall, the root of mesocolon, and encasing the middle colic vessels and the 1st branch of jejunal arteries. The complete surgical resection was performed and the mass weighted 10 kilograms (kg). The patient recovered uneventfully and was discharged 9 days after surgery. Three-month, 6-month, 12-month and 18-month on follow-up after surgery, showed no evidence of recurrence. We consider that surgical resection with negative margins is the goal but not at the expense of damaging the function of vital organs.

This is a retrospective study of 41 patients with extremity fibromatoses who were operated between June 2002 and November 2012. The mean age for all patients was 29.2 years with 30 females and 11 males. The mean duration of follow-up was 4.37 years. Eight patients were margin-positive on final histopathology. Seventeen developed recurrences (41.4%). Ten of these 17 patients underwent repeat surgery. The remaining 7 patients with inoperable recurrence were put on metronomic chemotherapy. The DFS for patients treated with surgical management was 62.6% at 3 years, 54.4% at 5 years and 44% at 10 years. The enigmacy on the tumour biology, natural history and optimal management of fibromatoses continue. Surgery remains the standard treatment and should be attempted only if R0 resection is possible without much morbidity to the patient. Non-surgical modalities also have their role to play in the management of these neoplasms.

Deep fibromatosis of desmoid type comprises rare mesenchymal tumors characterized histologically by proliferation of fibroblasts and myofibroblasts. These lesions are characterized by infiltrative growth and local recurrence but an inability to metastasize. They can be located in the abdominal wall (generally in sutures), intra or extraabdominal. The best imaging modality for evaluation and staging of the deep fibromatoses is MR imaging. These well or ill-defined lesions generally present internal hypointense bands, with lack of enhancement in post contrast images (collagen bundles) and are usually centered in an intermuscular location with a rim of fat (“split fat sign”), although invasion of surrounding muscle is frequent. Linear extension along fascial planes (“fascial tail sign”) is also a frequent manifestation in deep fibromatoses and is uncommon with other soft tissue neoplasms. MR image signal intensity has an implication on tumor recurrence, with a higher recurrence rate in lesions with high T2 signal. In surgically untreated lesions, with undergoing radiation or drug therapy, MR surveillance has been used to assess response to treatment with positive results demonstrating decrease in T2 signal, lesion enhancement and lesion size.

Here, we report the case of a 66-year-old male patient who previously underwent resection of sigmoid colon cancer and its liver metastasis. His follow-up contrast-enhanced CT scan revealed a mass shadow at around the gastrosplenic ligament, which gradually increased in size. Because it could not be pathologically diagnosed by transgastric EUS-FNA, en bloc resection was performed surgically for the tumor in the greater omentum. Hematoxylin-eosin staining of the resected specimen showed fibroblast-like cellswith hyperplasia of bold collagen fibersand spindle-shaped nucleus. While the immunostaining findings denied a diagnosis of mesenchymal neoplasm such as GIST, leiomyosarcoma, or schwannoma, it was pathologically diagnosed as a desmoid tumor. He has been followed up without any recurrence for 2-and-a-half years after the surgical resection. Desmoid tumors tend to be locally invasive; thus, there is the potential for local recurrence, although the frequency of distant metastasis is very low. In cases in which the tumor increases in size, en bloc resection with sufficient surgical margin should be performed. Cases of desmoid tumors originating from the greater omentum are reportedly rare; however, en bloc resection may be useful for both diagnosis and treatment of tumors of the greater omentum showing increased size that are also surgically resectable.

We present the case of a 76-year-old man with no pathological history. admitted in the emergency room due to a 5-day evolution fever associated with abdominal distension and a palpable mass in the hypogastrium.Urgent surgical intervention showed a large tumor of 15×15 cm affecting the jejunum (20 cm from the duodenojejunal angle); intestinal resection was performed with free margins and lateral-lateral mechanical anastomosis was done. Immunohistochemical analysis was negative for CD117, DOG1, ALK1, S100, CD34, desmin and actin and positive for vimentin and beta-catenin, desmoid tumor dependent on the jejunal wall, free neoplasic intestinal margins

We describe the case of a 34-year-old woman who presented with a painful parotid mass. She was found to have an extra-abdominal fibromatosis of her right parotid gland. Medical management with antibiotics and immunosuppression therapy was unsuccessful. Surgical resection was required for both a definitive diagnosis and management. Preoperative findings on computed tomography, magnetic resonance imaging, and both fine-needle aspiration biopsy and surgical biopsy were nonspecific, as is typical in EAF cases.