Aggressive Fibromatosis of the Pancreas: A Rare Cause of Incomplete Duodenal Obstruction

The pancreas is an extremely rare location for aggressive fibromatosis (AF), and the preoperative diagnosis of pancreatic AF is rather difficult. A 26-year-old man was referred to our hospital with a 3-week history of abdominal distension, intermittent abdominal pain, nausea, and vomiting. He had no history of abdominal surgery, trauma, or chronic hepatitis virus infection, nor did he ever suffer from pancreatitis. The contrast-enhanced computed tomography scan showed a solid mass, approximately 3 × 4 cm in size, located in the uncinate process of pancreas. The mass was hypodense in the arterial phase, isodense in the portal phase, and hyperdense in the delayed phase. The mass invaded and compressed the descending and horizontal part of the underlying duodenum and caused the dilatation of the descending duodenum. Colonoscopy examination excluded signs of polyposis. Malignant pancreatic tumor was suspected, and operation was performed. During the surgery, a solid mass, which invaded the adjacent duodenum, was detected within the uncinate process of pancreas. Pancreaticoduodenectomy was performed successfully, and the patient recovered uneventfully.

We report a case of an extra-abdominal desmoid fibromatosis that was successfully treated with computed tomography (CT)-guided percutaneous cryoablation. A 36-year-old female with familial adenomatous polyposis who had intractable 6–10/10 pain despite opioid use secondary to a biopsy-proven inoperable right rectus abdominis desmoid fibromatosis measuring 13.2 x 13 x 9.5 cm was referred to our institution. She underwent chemotherapy with imatinib and sorafenib followed by radiation therapy without clinical or radiologic improvement.

A 44-year-old woman was referred to the department of head and neck surgery for a 12-month history of decubitus dyspnea in the context of a painless mass of the right side of the neck, blocking the homolateral shoulder joint. Medical history reported the realization of a biopsy of the mass 4 months ago in Chechnya with a diagnosis of rhabdomyoma of the right side of the neck. The patient had never underwent other surgical procedures. Since then, the size of the mass continued to expand rapidly, leading to a recent decubitus dyspnea. The ear, nose, and throat examination revealed a very painful mass located in the right side of the posterior cervical area next to the cervical scar of the previous biopsy. Magnetic resonance imaging (MRI) showed an extensive tumor of the neck basis measuring 20 × 15 cm and infiltrating the nerve roots of the brachial plexus, the scalene muscles, the right carotid, the right phrenic nerve, and the adjacent adipose tissue. The chest X-ray found a right-side elevation of the right diaphragmatic cupola highlighting the paresis of the right side of the diaphragm. A fine-needle aspiration biopsy was performed but the result was noncontributory. Regarding the risk of malignancy, a debulking of the mass was performed with a frozen examination that could not found the histopathological nature of the lesion. Vascular and nerve structures have been conserved and there was no postoperative defect. The postsurgery histopathological examination of the mass revealed a desmoid-type fibromatosis of the neck. The reconstruction was made with a right pectoralis major musculomyocutaneous flap. Following the discussion of the oncological multidisciplinary group, patient received complementary radiation for the residual tissue. The 4-year follow-up was unremarkable.

A 74-year-old woman underwent laparoscopic sigmoid colectomy for colon cancer with pathological stage I. Follow-up computed tomography (CT) 18 months after primary surgery showed a nodular and enhanced soft tissue density mass, 20 mm in size, in the mesentery at the left side of the abdomen. Another CT scan, done 1 month later, revealed that the tumor had enlarged to 25 mm in size. Although the pathological diagnosis was not obtained, we suspected recurrence of the sigmoid colon cancer and applied chemotherapy using capecitabine, oxaliplatin, and bevacizumab. After 3 cycles of chemotherapy, however, the tumor had enlarged further. Therefore, the surgical resection of the tumor was performed to determine the diagnosis and to achieve possible curative resection of the tumor. The tumor existed in the mesentery of the jejunum, 100 cm from the ligament of Treitz, and showed invasive growth. We resected 40 cm of the jejunal segment together with the tumor. Microscopically, the tumor was composed of fibroblast, myofibroblast, and infiltrating the inflammatory cell and diagnosed as desmoid tumor by immunostaining (desmin+/-, beta-catenin+, CD117-, vimentin+). At 33 months after the resection of the desmoid tumor, neither the sigmoid colon cancer nor desmoid tumor has had a recurrence. After surgery for gastrointestinal cancer, it is difficult to differentiate between intra-abdominal desmoid tumor and recurrence. The possibility of intra-abdominal desmoid should be considered along with tumor recurrence during postoperative surveillance after resection of gastrointestinal cancer, especially when the risk of recurrence is low.

Desmoid-type fibromatosis (DF) is a rare monoclonal fibroblastic proliferation that is characterized by locally infiltrative but rarely metastatic lesions. Tyrosine kinase and y-secretase inhibitors are primarily used in the targeted therapy of DF. The use of these drugs, however, is mainly based on the recommendations of retrospective studies with small sample sizes. Previous studies that focused on the mechanism, efficacy, and safety of targeted therapy for DF were reviewed to provide references for clinical applications and research. The efficacy and safety of targeted therapy were compared with those of other systemic therapy options. Targeted therapy does not provide considerable advantages in efficacy and safety over other medical treatments and is usually applied after the failure of antihormonal therapies, nonsteroidal anti-inflammatory drugs, and chemotherapy. Further studies are required to explore the mechanism, indications, and appropriate drug dosage of the targeted therapy of DF.

Treatment of intra-abdominal located desmoid-type fibromatosis(DTF) is difficult because of the close relationship with vital organs. A healthy young male presents with an asymptomatic palpable mass in the lower right abdominal quadrant. A computed tomography shows a 10 × 7 sq cm pear-shaped mass, and pathological examination revealed DTF. A watchful waiting approach was initiated, as the patient was asymptomatic and surgery would imply a significant amount of intestinal resection. After a follow-up of 2 years, the tumor has regressed spontaneously and the patient is still without symptoms. DTF is a difficult to treat condition where individualized management is appropriate. An asymptomatic patient could be treated with a watchful waiting approach, even with intra-abdominal location. Thereby sparing unnecessary morbidity as the tumor can be stable for many years or even regress spontaneously.