Desmoid type fibromatosis of the distal pancreas: A case report

A 23-year-old Korean female presented epigastric pain of two-months’ duration. She had a laparoscopic ovarian cyst excision 8 months previously. An abdominal computed tomogram (CT) demonstrated a 10-cm solid mass in the distal pancreas, with signs of splenic artery and vein occlusion, gastric and transverse colon invasion. Operative findings showed a mass involving distal pancreas, invasive to the posterior wall of the antrum of the stomach and transverse colon and 4th portion of the duodenum without lymph node involvement. The surgery consisted of a distal pancreatectomy, splenectomy and combined partial resection of the stomach, transverse colon and 4th portion of the duodenum. The immunohistochemistry and histopathological features were consistent with a confirmed diagnosis of intra-abdominal desmoid type fibromatosis (DTF). The prognosis of pancreatic DTF is not known and she showed no recurrence or distant metastasis during a 3 year follow-up. Herein we report a rare case with an isolated, sporadic, and non-trauma-related DTF, located at the pancreatic body and tail.

Current treatment modalities show high levels of recurrence and comorbidities for extra-abdominal desmoids. Cryo-surgery as an alternative was subsequently investigated. In this retrospective, single center study 11 patients showing symptomatic tumors were treated with individualized cryo-surgery. Treatment protocol included preoperative planning using computer rendered 3D models, intraoperative navigation and execution using cone beam guidance, and postoperative magnetic resonance imaging image analysis using a gaussian mixture model software. Subjective outcomes were reported using Short Form Health Survey (SF-36) questionnaires. Conclusion: Cryo-surgery using the three-phase protocol as described may improve the overall outcome of future ablation procedures.

A 22-year-old male was presented with a recurrence of lower abdominal wall mass(LAWM) after multiple operations and a new mass in the skin flap donor area of the right abdominal wall. Magnetic resonance imaging (MRI) revealed multiple soft tissue masses and nodules in the subcutaneous fat layer of the lower abdomen and right abdomen wall. Confirmed diagnosis of desmoid-type fibromatosis in combination with pathological characteristics of the resected specimens. In general, DTF did not have the ability to metastasize, but we extracted paraffin sections and performed full exon sequencing. After personalized analysis, the results showed that there was a possibility of implantative metastasis. Due to the aggressive growth and recurrence of the tumor, the patient was subsequently treated with radiotherapy and no recurrence was observed 6 months later. Continued follow-up is ongoing. We report the first case of DTF that occurs in the superficial layer of fat with the possibility of surgically induced implantative metastasis, for such patients, diagnosis of DTF is more difficult and surgical treatment should be treated with greater caution.

Desmoid fibromatosis (DF) is a locally aggressive, not metastasizing tumor associated with high local recurrence rates. Surgery was a standard-of-care for DF treatment; however, recently, conservative treatment and active surveillance are preferred. This study aimed to evaluate the real-life outcomes of DF treatment.

To assess the efficacy and safety of sandwich isolation surgery on the local control of progressive desmoid tumors involving neurovascular bundles. A total of 27 patients with progressive desmoid tumors at extremities involving neurovascular bundles who received surgery at our hospital between August 2014 and August 2018 were identified. A total of 13 patients received sandwich isolation surgery, in which R2 resection was performed in neurovasculature-involving regions, and a biomaterial patch was used to envelop involved neurovascular structures and isolate residual tumors. A total of 14 patients received traditional surgery, which included tumor resection without isolation procedure. In sandwich isolation group, tumor progressions and local recurrences occurred in 3 patients outside the isolated neurovasculature-involving regions. However, no progressions or recurrences occurred in any patients in the isolated neurovasculature-involving regions where R2 resection was performed. Sandwich isolation surgery group and traditional surgery group shared similar baseline clinical characteristics. The estimated 3-yr event-free survival rate was 76.9% after sandwich isolation surgery, and 32.7% after traditional surgery (P = .025). Patients who received sandwich isolation surgery were less likely to have local recurrence (hazard ratio: 0.257, P = .040). No complications were noted except intermittent mild pain in operative regions (2 cases). Sandwich isolation surgery is effective and safe for local control of desmoid tumors involving neurovascular bundles.

This paper describes a case of a large desmoid tumor on the abdominal wall that developed in the 10th week of pregnancy in a 29-year-old patient with no history of familial adenomatous polyposis. Ultrasound showed that the tumor had increased around 158% during pregnancy. The patient underwent Cesarean section during which a mass of around 20 cm was detected between the anterior and posterior layer of the aponeurosis of the rectus abdominis muscle. Conservative management (no intervention) was adopted with follow-up imaging. Ultrasound-guided core biopsy was performed four and a half months after delivery, with histopathology revealing a spindle cell neoplasm, with possible musculoaponeurotic fibromatosis. Six months after childbirth, the tumor had decreased spontaneously by 40.5% in thickness and nine months following delivery the patient continues to be monitored under expectant management, with no complaints or symptoms. Although rare, obstetricians should be aware of the possibility of a desmoid tumor during pregnancy and should evaluate the best management option (surgery, pharmacological treatment or expectant management) according to the patientÂ’s profile in order to avoid visceral complications, the need for extensive surgery with possible aesthetic and functional sequelae, and even iatrogenic complications.