A case report of extra-abdominal desmoid tumor after kidney transplantation

Introduction: Desmoid tumors are rare, locally invasive, non-metastasizing intermediate grade neoplasms. According to the organ procurement and transplantation network report, approximately one million solid organ transplants have been performed globally, from which only three desmoid tumors have been reported.

Case presentation: We present the case of a 61-year-old male who developed an asymptomatic abdominal mass 3 years after kidney transplantation. Computed tomography revealed a 50-mm mass attached to the Hem-o-lok clip beneath the right rectus abdominis muscle. A needle biopsy revealed β-catenin positivity, which raised suspicion for a desmoid tumor. Due to progressive tumor enlargement, surgical excision was performed. The patient was discharged on postoperative day 5 without any surgical complications.

Conclusion: This is the first documented case of an extra-abdominal desmoid tumor after kidney transplantation. Although no recurrence has been observed over a 5-year follow-up period, careful long-term monitoring is required because of the high recurrence rate of desmoid tumors.

none available. Article in Chinese

Approximately 15% of familial adenomatous polyposis (F.A.P.) patients develop desmoid tumors with 40% intra-abdominal desmoids (IAD) which contribute to significant morbidity and mortality due to bowel obstruction, fistulization, sepsis, abdominal bleeding, and/or ureteral obstruction. [1] IADs can lead to intestinal failure (IF), necessitating total parenteral nutrition (TPN) and risk of line sepsis, and intestinal failure-associated liver disease. [2], [3] Although intestinal transplantation (ITx) offers a potential salvage option, there is paucity of data on long-term outcomes and survival in this patient population.

Purpose: Managing the defect after abdominal wall desmoid tumor resection is challenging due to the wide excision required. This report aims to review our institutional experience with mesh-reinforced reconstruction following desmoid tumor resection in the abdominal wall.

Methods: We retrospectively reviewed patients who underwent abdominal wall desmoid tumor resection with mesh-reinforced reconstruction between April 2014 and January 2019. Patient records were analyzed to collect data on demographic characteristics, surgical procedures, and complications.

Results: Sixteen eligible patients were identified, including one patient who underwent simultaneous resection of affected intra-abdominal organs. The mean size of the abdominal wall defect was 88.1 ± 71.6 cm², and the mean mesh size was 160.3 ± 56.5 cm². The mean operative time was 87.5 ± 41.4 min, the mean hospital stay was 15.7 ± 6.0 days, and the mean follow-up period was 83.8 ± 18.0 months. Complications included seroma (n = 1) and abnormal sensation (n = 2). Tumor recurrence was observed in 1 patient, who died due to cancer progression (lung cancer). No surgical site infections or mesh infections were observed. Additionally, no patients developed ventral hernias or abdominal bulging.

Conclusion: Mesh-reinforced reconstruction is a feasible and effective approach for patients requiring abdominal wall desmoid tumor resection.

None Available

This is a plain langauge summary of the DeFi study, published in The New England Journal of
Medicine in March 2023. In the DeFi study, researchers were studying if the drug
called nirogacestat (Ogsiveo®) could slow disease progression and improve
disease symptoms. The study included 142 adults with progressing desmoid
tumors.