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Less-invasive fascia-preserving surgery for abdominal wall desmoid.

ABSTRACT: The mainstay of treatment for desmoid has been shifted to active surveillance (AS). However, surgery is still being performed on abdominal wall desmoid with a wide surgical margin. The purposes of this study are to clarify the treatment results of less-invasive, fascia preserving surgery for patients with abdominal wall desmoid, and to propose a new treatment modality. Since 2009, 34 patients with abdominal desmoid have been treated in our institution. Among them, as a final treatment modality, 15 (44%) were successful with AS, 15 were subjected to less-invasive surgery, and 4 methotrexate and vinblastine treatment. The clinical results of less-invasive surgery were clarified. In the surgical group, although the surgical margin was all microscopic positive (R1), only one patient (6.7%), who has the S45F mutation type of CTNNB1, showed recurrence, at a mean follow-up of 45 months. There were no patients with familial adenomatous polyposis (FAP)-related desmoid in this cohort. Only two patients (13%) required fascia lata patch reconstruction after removal of the tumor. In patients with non FAP-related abdominal wall desmoid, less-invasive, fascia preserving surgery is recommended as a favorable option as active treatment. Based on the results of this study, multi-institutional further research is warranted with an increased number of patients.

SUBMITTER: Nishida Y

PROVIDER: S-EPMC8481551 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:Desmoid fibromatosis is a rare but locally aggressive tumor comprised of myofibroblasts. Desmoids do not have the ability to metastasize but can cause significant morbidity and mortality by local invasion. These tumors may occur throughout the body, but are commonly found on the abdominal wall and within the intestinal mesentery. Desmoids in these areas may cause unique clinical problems for physicians and patients. Mutations in either the β-catenin or the APC genes are usually the cause for the development of these tumors with the former comprising the sporadic development of tumors and the latter being associated with familial adenomatous polyposis syndrome. Surgical resection with histologically negative margins has been the cornerstone of therapy for this disease, but this paradigm has begun to shift. It is now common to accept a microscopically positive margin after resection as recurrence rates may not be significantly affected. An even more radical evolution in management has been the recent movement towards "watchful waiting" when new desmoids are diagnosed. As the natural history of desmoids has become better understood, it is evident that some tumors will not grow and may even spontaneously regress sparing patients the morbidity of more aggressive therapy. Other modalities of treatment for desmoids include radiation and systemic therapy which both can be used adjuvantly or as definitive therapy and have shown durable response rates as single therapy regimens. The decision to use radiation and/or systemic therapies is often based on tumor biology, tumor location, surgical morbidity, and patient preference. Systemic therapy options have increased to include hormonal therapies, non-steroidal anti-inflammatory drugs and chemotherapy, as well as targeted therapies. Unfortunately, the rarity of this disease has resulted in a scarcity of randomized trials to evaluate any of these therapies emphasizing the need for this disease to be treated at high volume multidisciplinary institutions.

Project description:In recent years, three-dimensional reconstruction (3DR) models have become a standard tool in several medical fields such as education, surgical training simulation, patient-doctor communication, and surgical planning. Postoncologic reconstructive surgery in thoracic diseases might benefit from 3DR models; however, limited data on this application have been published worldwide. In this paper, the aim was to report our experience with 3DR modeling to determine resection and plan the surgical reconstruction in a patient with a desmoid tumor of the chest wall. For a better understanding of the case study, we describe all the steps from acquiring computed tomography (CT) scans to the final 3D rendering. A 68-year-old, non-smoking man presented at our outpatient department with painless swelling of the right anterobasal chest wall. A thorax-abdomen-brain CT scan revealed homogenous solid tissue with a dense mass measuring 80 mm × 62 mm. The final 3D model was evaluated by the surgical team (three medical doctors), who found the model to be powerful. Based on the results and the accuracy of the model, the multidisciplinary team decided that the tumor was resectable. Consequently, a surgical plan based on the 3D model was developed to perform chest wall reconstruction after radical resection. The patient underwent right anterolateral thoracotomy at the seventh intercostal space, which confirmed the CT scan findings and revealed infiltration of the serratus muscle and medial portion of the diaphragm. A radical tumor en bloc resection with chest wall and diaphragm resection was performed. The full-thickness chest wall and diaphragm defects were reconstructed using two separate biological patches of a porcine dermal collagen implant (Permacol™ Surgical Implant). Postoperative X-ray revealed unremarkable findings; the patient had an uneventful recovery and was discharged 6 days after surgery. This case study illustrates that 3DR models enable a personalized approach to the treatment of desmoid tumors. Therefore, this approach should be developed further and studied systematically.

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Less-invasive fascia-preserving surgery for abdominal wall desmoid.

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