Surgical management of a giant abdominal wall desmoid tumour

Desmoid tumours are clonal fibroblastic proliferations in soft tissues, characterised by infiltrative growth and local recurrence, but not metastasis. Various treatment strategies for desmoid tumours exist, varying from observation, medical and systemic therapy to radiotherapy and surgery. A 25-year-old woman with a background of familial adenomatous polyposis was referred with an enlarging abdominal desmoid tumour measuring 40?40?40 cm despite repeated radiofrequency ablation, surgical debulking and hormone therapy. The patient had a two-stage operation. The first stage involved excision of the desmoid tumour with full-thickness abdominal wall. The abdominal wall was not closed, and a topical negative pressure seal was applied. After 2 days, she underwent the second stage: reconstruction of the abdominal wall defect with a large porcine mesh which was covered with anterolateral thigh flaps. Postoperative complications included ileus and a fall which required further surgery. The patient was discharged 1 month after the first operation. Abdominal MRI scans were performed at 3 and 7 months postdischarge and showed no recurrence of diseaseBackground.

We present an unusual case of a 6-year-old boy who presented with the sudden presence of left neck mass and acute hypoxemic respiratory failure, whose subsequent imaging demonstrated a previously undiagnosed anterior mediastinal mass (AMM) extending into the left neck. Biopsy of the mass was consistent with a desmoid tumor, which is a rare cause of AMM in children. The sudden growth of the neck mass suggests a very aggressive desmoid tumor, causing an unexpected respiratory compromise. Anterior mediastinal masses may cause symptoms by compressing the heart, great vessels, and airways. However, the patient may adapt and develop compensatory mechanisms to counter the compressive effects. Emergency care of the patient with an AMM who presents with acute respiratory distress includes optimizing oxygenation through promoting a calm environment, oxygenating while minimizing positive end-expiratory pressure, maintaining the patient’s compensatory mechanisms by minimizing sedation and muscle relaxation, positioning the patient to minimize compressive effects of the mass on the vital thorax structures, and early consultation with pediatric specialists to develop a shared-emergency treatment strategy and to secure an expedited disposition to the appropriate venue of care.

Background: Studies suggest that surgical breast augmentation with implants is a risk factor for breast desmoid tumors. The statistical strength of this correlation is unknown, as evidence is limited to anecdotal reports. Methods: Patients with breast desmoid tumors and a history of breast implants seen at a single center between 2000 and 2021 were identified via radiology, breast, and sarcoma databases. The standardized incidence ratio (SIR) was calculated to assess the correlation between breast desmoid tumors and breast implants. The cases were pooled with published cases for analyses. Progression-free survival curves and hazard ratios were estimated using the Kaplan-Meier method and Cox proportional-hazards modeling. Results: Fourteen patients from one institution and 66 cases in the literature were identified. All patients were female, and the mean age was 38 years old (range 20-66). 63 patients (82%) underwent resection, 9 (12%) received chemotherapy, 3 (4%) received sorafenib, 11 (14%) received hormonal therapy, and 3 (4%) underwent active surveillance. After resection, the 2-year recurrence-free survival rate was 77% (95% CI 65%-89%). The recurrence risk was lower for resection with no residual tumor (R0) compared to microscopic (R1) or macroscopic (R2) residual tumor (HR: 0.15; 95% CI 0.02-0.8; p < 0.05). The SIR was 482 (95% CI 259-775) to 823 (95% CI 442-1322), suggesting a 482-823 times higher risk of developing a breast desmoid tumor after breast augmentation than the general population. Conclusion: We present a nonrandom association between breast implants and desmoid tumors. Whether the tumors arise from the surgical trauma or the implant’s biomaterial is unknown. When surgery is indicated, negative margins reduce the risk of recurrence.

Objective: To evaluate the safety and efficacy of ultrasound-guided high-intensity focused ultrasound (USgHIFU) ablation for desmoid tumors (DTs). Method: A total of 111 patients with histologically proven DTs were included and treated by USgHIFU ablation. Adverse events were continuously evaluated according to the Common Terminology Criteria for Adverse Events (CTCAE) v5.0 until 3 months after treatment. The incidence of non-perfused areas within the treated tumors, non-perfused volume rate (NPVR) and tumor volume reduction were evaluated using contrast-enhanced MRI before and one week and 3 months after the procedure. Results: The enrolled patients (32 male, 79 female, mean age 29.5 ± 1.0 years) with 145 DTs (118 extra-abdominal, 16 abdominal wall, 11 intra-abdominal; median maximum diameter: 9.6 cm, range: 3-34.5 cm) underwent 188 sessions of HIFU ablation, and the mean number of ablations was 1.7 (range, 1-7) per patient. In majority of cases (143/145 cases, 98.6%), no serious adverse events were observed. There was no significant difference in the incidence of adverse events between patients who received a single treatment and those who received multiple treatments. Non-perfused area was observed within every treated tumor, and the median NPVR was 84.9% (range, 1.9-100%). The tumor volume reduction rate was 36.1 ± 4.2% at 3 months after treatment. Conclusion: USgHIFU ablation, as a noninvasive and easily repeatable local treatment, is a promising treatment for DTs.

The extra-abdominal deep fibromatoses also called as desmoid tumor (DT) are rare musculoaponeurotic, histologically benign tumoral soft tissue lesions which are locally aggressive with high rate of recurrence and no metastatic potential. Here, we describe a rare case of postoperative paraspinal fibromatosis in female child after aciurgy of intraspinal cervical meningioma. The lesion was incidentally detected in routine follow-up study and showed significant interval growth over a period of 1 year. Surgical resection was performed after histopathological confirmation; however, recurrence was observed. Hitherto only seven cases of postoperative paraspinal fibromatosis have been reported in the literature. To the best of authors’ knowledge, this report is the first case of postoperative fibromatosis in cervical spine after resection of meningioma. The aim of the case report is to emphasize the clinical scenario for suspicion of fibromatosis, magnetic resonance imaging clues, and multidisciplinary treatment strategy which has shifted toward initial active surveillance.

A 14-year-old girl with cerebral palsy presented for bilateral lower limb surgery for spasticity. A lumbar epidural catheter was sited under general anaesthesia on the third attempt and used intra-operatively with good effect. A local anaesthetic infusion was used for postoperative analgesia but was noted to be leaking under the dressing with a patchy, unilateral block. The catheter was therefore removed on the second postoperative day. Following discharge, the patient progressively developed new back and leg pain for which she was re-admitted seven weeks later. This was investigated and initially thought to be myositis of the erector spinae muscles on magnetic resonance imaging. When the patient failed to respond to treatment, a muscle biopsy demonstrated desmoid fibromatosis. Trauma may cause or accelerate the development of desmoid fibromatosis, which has also been theorised to arise from scar tissue in previously injured areas. We hypothesise that challenging epidural placement or the leakage of the local anaesthetic agent into the surrounding muscular tissue, inducing local myonecrosis, could have been the triggering or accelerating event in tumour development. This may be the first reported case of extra-abdominal desmoid fibromatosis in association with epidural placement.