Mesenteric desmoid tumor: De novo occurrence or recurrence following appendectomy?

Desmoid type fibromatosis (DF) is a rare, locally aggressive but benign proliferation of fibrous tissue which produces a fibroblastic mass that can cause a wide range of symptoms secondary to mass effect. When resected, these masses most commonly recur in the first 2 years. We present a case of a 33-year-old male with a history of an appendectomy 2 years prior, though his pathology report did not identify inflammation in the appendix, who presented with gradual onset of abdominal pain, and radiographs that demonstrated a large mass in the right lower abdomen. Given his symptoms the mass was resected and pathologic evaluation revealed a desmoid tumor. This case presents a unique possibility of a recurrent desmoid tumor in which the patient’s surgical history and radiographic findings can contribute to the overall management strategy of the patient given the evolving options for treatment of desmoid fibromatosis.

Case description: A 21-year-old healthy female presented with severe left lateral hip pain beginning suddenly two weeks prior. Physical examination revealed zero degrees of left hip external rotation passive range of motion with a firm end feel and pain severity and irritability out of proportion to an expected musculoskeletal presentation. She was referred to her physician with a recommendation for imaging to determine the source of pain and appropriateness of physical therapy. Outcome: Magnetic resonance imaging revealed a foreign mass in her left gluteus medius muscle which biopsy revealed to be a desmoid fibromatosis. Conclusion: This case demonstrates a thorough differential diagnostic process leading to medical imaging referral in a patient with a non-musculoskeletal source of pain. Physical therapists must be diligent in their differential diagnostic process to ensure appropriateness of their treatments or the need for referral.

Objective: The aim of this study was to determine the prognostic factors for recurrence in patients with extra-abdominal desmoid tumors (EDTs) treated surgically. Methods: This single-institution, retrospective study included patients with a histologically-proven extra-abdominal desmoid tumor between 2007 and 2018. The demographic characteristics (age, sex) of the patients, tumor characteristics (region, size, proximity to neurovascular structures, margins), treatment management (surgery and/or adjuvant radiotherapy), and clinical results were analyzed. The effects of these possible prognostic factors on overall and disease-free survival rates and the risk of local recurrence were evaluated. Results: Evaluation was made of 22 patients (16 females, 6 males) with a mean age at diagnosis of 34.7 years (range = 22-76 years). The mean follow-up was 104 months (range = 4.8-168). Tumor localization was in the upper extremity in 4 patients (18.1%), the lower extremity in 11 (50.0%), and the trunk in 7 (31.8%). The mean tumor size (maximum diameter) was 5.2 cm (range = 0.6-13 cm; median = 5.8 cm), and the mean tumor volume was 181.3 ± 531.4 ml. All the 22 patients were treated surgically along with adjuvant radiotherapy (RT) administered to 8 in addition to surgery for the primary treatment of the tumor. Following primary surgery, resection margins were R0 in 11 patients, R1 in 9 and R2 in 2. Local recurrence (LR) developed in 6 patients (27.2%) during the follow-up period. Recurrence-free survival rate (RFS) was 90.9% at one year, 74.1% at 5 years, and 61.7% at 10 years. During the follow-up, no patient died, and distant metastasis was not detected. Tumor length, resection margins, and adjuvant RT were observed to influence the risk of local recurrence (P < 0.05). Conclusion: The results of this study have demonstrated that tumor size ? 5 cm and the presence of microscopic or macroscopic positive surgical margins can increase the risk of LR, and adjuvant RT can reduce the development of LR in the management of EDT.

Background: Desmoid-type fibromatosis is a rare disease that can result in hydronephrosis. Hydronephrosis associated with desmoid-type fibromatosis often requires surgery or ureteral stent insertion. Although radiation therapy is recommended for inoperable cases of desmoid-type fibromatosis, there has been no report of treatment for hydronephrosis associated with desmoid-type fibromatosis by radiation therapy alone. We herein report a case of successful treatment for inoperable recurrence of desmoid-type fibromatosis with unilateral hydronephrosis by radiation therapy alone. Case presentation: A 43-year-old Japanese female underwent resection of desmoid-type fibromatosis in the right inguinal region and combined resection of the right external iliac vein 5 years before. Other treatment was not performed because of her pregnancy. Four years after surgery, desmoid-type fibromatosis recurred in the right pelvic wall. Cyclooxygenase-2 selective inhibitor treatment was given for 1 year, but her desmoid-type fibromatosis enlarged to more than 10 cm, and she had swelling of her right leg and hydronephrosis of her right kidney. The patient received 50.4 Gy in 28 fractions of prophylactic irradiation using 10 MV X-ray and 9 Gy in five fractions of a sequential boost for the recurrent desmoid-type fibromatosis. Although there was temporary tumor progression at 1 month after radiation therapy, slow regression of the tumor was seen. At 5 years after radiation therapy, there was no disease progression or severe complications. Conclusion: We experienced successful treatment for an inoperable case of desmoid-type fibromatosis with hydronephrosis. Moderate-dose radiation therapy alone is an effective and feasible approach for the management of hydronephrosis associated with desmoid-type fibromatosis.

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.