Desmoid fibromatosis involving the ureter: A rare presentation with intraoperative challenges

Following failed retrograde and antegrade ureteric stenting, a 35-year-old male patient underwent an elective boari flap for marked proximal hydroureteronephrosis due to a periureteric mass in the right iliac fossa. Intraoperative vascular surgical assistance was required for control of arterial bleeding due to friable vessel wall. Histopathology demonstrated desmoid fibromatosis.

Purpose: Desmoid-type fibromatosis (DF) is clonal fibroblastic proliferation that arises in the deep soft tissues, tends to reoccur, and is locally invasive. Desmoid-type fibromatosis of paranasal sinuses with intracranial extension is a rare condition that is even rarer in a small child. We aim to share with the reader our literature review, decision-making, and endoscopic endonasal operation procedure that combined gained us favorable results against this benign tumor with unpredictable natural history and disease course. Case report: We describe the decision-making process in the management of a 3-year-old boy with a history of sudden vision loss and vomiting. MR showed an expansive well-delineated homogeneous tumor in the sphenoid sinus with intracranial extension and optic nerves compression. The diagnosis of a sporadic form of desmoid-type fibromatosis was made using genetic testing of tumor tissue. A total gross removal was carried out with endoscopic endonasal microsurgical approach. At a 3-month follow-up, the patient is without any signs of recurrance. Conclusion: The treatment of children with desmoid-type fibromatosis requires a multidisciplinary approach by clinicians experienced with the management of pediatric cancer. While the desmoid-type fibromatosis is a benign, locally invasive tumor, observation should be the first step in the management. In case of life-threatening or symptomatic cases, operations that preserve function and structure should be the first choice for this benign tumor with unpredictable natural history and disease course.

Pancreatic collision tumors are rare neoplasm, and cases consisting of ductal adenocarcinoma with a neuroendocrine tumor, intraductal papillary mucinous neoplasm with a neuroendocrine tumor, and solid pseudopapillary neoplasm with a neuroendocrine tumor have been reported. We report a case of a rapidly growing pancreatic collision tumor consisting of desmoidtype fibromatosis and mucinous cystic neoplasm in a 30-year-old pregnant female. To the best of our knowledge, this is the first reported case of a pancreatic collision tumor consisting of desmoid-type fibromatosis and mucinous cystic neoplasm.

Background: Aggressive fibromatosis is a rare histologically benign but locally infiltrative myofibroblastic tumor. Primary intracranial aggressive fibromatosis (IAF) can exhibit a clinically malignant course. Observations: A 22-year-old otherwise healthy woman presented with left painful ophthalmoplegia. Magnetic resonance imaging (MRI) revealed a left sellar tumor with cavernous sinus invasion. Endoscopic transsphenoidal surgery was performed. The lesion could not be totally resected. An inflammatory myofibroblastic tumor was suspected, so steroid pulse therapy was introduced, but it was ineffective. The tumor recurred after a few months, and she complained of visual acuity loss, abducens nerve palsy, trigeminal neuralgia, and panhypopituitarism. The lesion was diagnosed as primary IAF by a pathological review. Gamma Knife radiosurgery was performed, and chemotherapies were introduced but ineffective. Her consciousness was disturbed, and MRI showed hypothalamic invasion of the tumor, occlusion and stenosis of carotid arteries, and cerebral stroke. Palliative care was introduced, and she died 32 months after the onset. The autopsy revealed tumor invasion to the cavernous sinus, optic nerve, hypothalamus, pituitary, and tonsillar herniation due to massive cerebral stroke. Lessons: Radical resection can be impossible in patients with IAF. Radiotherapy and chemotherapy are not always effective for residual lesions. Adjuvant therapy for IAF remains to be explored.

Desmoid fibromatosis is a locally aggressive myofibroblastic neoplasm. In this study, we report a case of desmoid fibromatosis in the paraspinal muscle that was misdiagnosed as intramuscular schwannoma through incisional biopsy at another hospital. We performed total excision of the mass lesion with a clear margin. We found that for an accurate diagnosis, magnetic resonance imaging, incisional biopsy and excisional biopsy were required.

Background: Desmoid fibromatosis (DF) arises in musculoaponeurotic structures, most commonly the rectus abdominis muscle. It is locally aggressive with no propensity for metastasis. The aim was to describe the spectrum of abdominal wall DF and its surgical treatment and outcome at a tertiary institution. Methods: All abdominal wall DF resected from 2007 to 2019 were retrospectively analysed. The tumours had a fullthickness abdominal wall excision. The defect was reconstructed with biological implants covered by either skin edge undermining or split skin graft. Histological diagnosis was based on characteristic morphological and immunohistochemistry parameters. Parameters analysed were demographics, lesion size, procedure performed and outcomes. Results: Eleven female patients with a median age of 29 years (range 21-61) presented with rapid growth over 6-24 months of anterior abdominal wall tumours with a median maximum resected diameter of 163 mm (range 63-200 mm). There was no specific complication related to the abdominal wall reconstruction. With a median follow-up of 5 years (range 1-11 years), all patients displayed an asymptomatic abdominal wall bulge (eventration). One patient has had a small recurrence currently being observed. Conclusion: Striking features of DF tumours at Chris Hani Baragwanath Academic Hospital (CHBAH) were their location, rapid growth, female gender and large size. Biological implant without complex plastic surgery techniques result in good outcomes, though the rate of progression of eventration to hernia is unknown.