Ultrasound-guided peripheral nerve blockade in high-intensity focused ultrasound ablation for extra-abdominal desmoid tumors: a case series

Studies have suggested that HIFU can be used safely and effectively for treating desmoid tumors to eliminate viable tumors or to provide long-lasting control of tumor growth through repeated treatments. The treatment protocol in these studies required general anesthesia or conscious sedation with the intravenous administration of fentanyl and midazolam and caused more severe adverse events, including second-degree skin burns, nerve injury, and off-target heating compared with daily repeated HIFU treatment without sedatives or intravenous analgesics.

US-guided peripheral nerve blockade (USg-PNB) has been widely accepted for providing local anesthesia in surgery and pain management in recent years. Many studies have reported that USg-PNB is a safe alternative that uses minimal amounts of local anesthetic and does not require hemodynamic monitoring or extended postprocedural observation. However, no reports of using USg-PNB during HIFU treatment have been published thus far. In this paper, we describe the usefulness and effectiveness of USg-PNB in the HIFU treatment of four patients with extra-abdominal desmoid tumors.

Desmoid fibromatosis (DF) is a rare and locally aggressive neoplasm. We present a case of a 28-year-old previously healthy multigravida who noticed a lump in her abdomen near the umbilicus two months before becoming pregnant. It underwent rapid growth during pregnancy, causing pain and discomfort. Targeted ultrasound of the area showed an irregular mass measuring 0.9 × 1.7 × 1.4 cm. The origin of the mass was unclear, suggesting a connection with the intra-abdominal contents. An MRI done three weeks later revealed a subcutaneous ovoid mass measuring 3.0 × 2.3 × 3.0 cm, which was significantly larger. Due to pain and rapid growth, surgical resection was done at 25 weeks of pregnancy. Histopathological examination revealed a desmoid tumor. The patient had an uneventful recovery and term vaginal delivery without complications. Hence, our case serves as evidence that DF tumors can be surgically managed during pregnancy with minimal to no complications.

Desmoid tumors are rare tumors with a tendency to infiltrate locally. The lack of a standard treatment approach makes choosing the most appropriate treatment for patients challenging. Most experts recommend watchful observation for asymptomatic patients as spontaneous regression of tumor is observed in up to 20% of patients. Upfront resection of the desmoid tumor has fallen out of favor due to high morbidity and high relapse rates associated with the tumor. Systemic therapy has evolved over several decades. Where chemotherapy, hormonal therapy, and non-steroidal anti-inflammatory drugs were used over the last several decades, tyrosine kinase inhibitors came to the forefront within the last decade. Most recently, gamma-secretase inhibitors have shown significant clinical benefit in patients with desmoid tumors, bringing forth an entirely new mechanistic approach. Several Wnt pathway inhibitors are also under development. Invasive approaches like cryoablation have also shown clinical benefit in patients with extra-abdominal desmoid tumors in recent years. The recent approval of nirogacestat has ushered in a new era of treatment for patients diagnosed with desmoid tumors. Several new molecules are expected to be approved over the coming years.

Desmoid tumours are benign but locally aggressive mesenchymal neoplasms that occur most commonly in the abdomen, with the potential to invade surrounding structures causing significant morbidity. Lateral abdominal wall defects are known to be more challenging and less frequently encountered compared to ventral abdominal wall defects. Asymmetric forces caused by contraction of remnant rectus and contralateral oblique muscles increase the risk of herniation postoperatively. We report a case of a challenging abdominal wall reconstruction after desmoid tumour resection in a 62-year-old male patient who presented to our hospital with a progressively enlarging left upper back lump of 6 months duration. A venous supercharged pedicled anterolateral thigh flap was combined with PROLENE® mesh for reconstruction, and the patient recovered well with good functional and aesthetic outcomes at 2-year follow-up. The pedicled anterolateral thigh flap with venous supercharging can be effectively used for the reconstruction of extensive lateral abdominal wall defects.

Background: Desmoid tumors are fibroblastic lesions which often have an unpredictable and variable clinical course. In the context of familial adenomatous polyposis (FAP), these frequently occur intra-abdominally, especially in the small-bowel mesentery resulting in sepsis, fistulation, and invasion of the abdominal wall and retroperitoneum. In selected cases where other modalities have failed, the most radical option is to perform a total enterectomy and intestinal transplantation (ITx). In this study, we present our center’s experience of ITx for desmoid in patients with FAP.

Methods: We performed a retrospective review of our prospectively collected database between 2007 and 2022. All patients undergoing ITx for FAP-related desmoid were included.

Results: Between October 2007 and September 2023, 144 ITx were performed on 130 patients at our center. Of these, 15 patients (9%) were for desmoid associated with FAP (7 modified multivisceral transplants, 6 isolated ITx, and 2 liver-containing grafts). The median follow-up was 57 mo (8-119); 5-y patient survival was 82%, all with functioning grafts without local desmoid recurrence. These patients presented us with several complex surgical issues, such as loss of abdominal domain, retroperitoneal/abdominal wall involvement, ileoanal pouch-related issues, and the need for foregut resection because of adenomatous disease.

Conclusions: ITx is a viable treatment in selected patients with FAP and extensive desmoid disease. The decision to refer for ITx can be challenging, particularly the timing and sequence of treatment (simultaneous versus sequential exenteration). Delays can result in additional disease burden, such as secondary liver disease or invasion of adjacent structures.

Extra-abdominal desmoid-type fibromatosis (EADTF) is a rare neoplastic condition of monoclonal fibroblastic proliferation characterized by local aggressiveness with a distinct tendency to recur. Although EADTF is a benign disease entity, these tumors have a tendency to infiltrate surrounding normal tissues, making it difficult to completely eliminate them without adjacent healthy tissue injury. Surgical excision of these locally aggressive tumors without clear resection margins often leads to local recurrence. The aim of this thorough review was to assess the current treatment concepts for these rare tumors. A comprehensive search of articles published in the Cochrane Library, MEDLINE (PubMed), and EMBASE databases between January 2008 and February 2023 was conducted. Surgical intervention is no longer the first-line approach for most cases; instead, strategies like active surveillance or systemic therapies are used as initial treatment options. With the exception of EADTFs situated near vital structures, a minimum of 6-12 months of active surveillance is currently advocated for, during which some disease progression may be considered acceptable. Non-surgical interventions such as radiation or cryoablation may be employed in certain patients to achieve local control. The currently preferred systemic treatment options include tyrosine kinase inhibitors, low-dose chemotherapy, and gamma-secretase inhibitors, while hormone therapy is not advised. Nonsteroidal anti-inflammatory drugs are utilized primarily for pain management.