A Rapidly Growing Abdominal Mass: Desmoid Tumor in Pregnancy.
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ABSTRACT:
SUBMITTER: Leon MG
PROVIDER: S-EPMC4502627 | biostudies-other | 2015 Apr
REPOSITORIES: biostudies-other
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Project description:Introduction and importanceDesmoid tumors of the abdominal wall are rare fibroblastic proliferative tissue, included in the group of soft tissue tumors, not metastasizing but locally aggressive as an infiltrating tumor and a source of recurrence.Case presentationThis case report describes a rare case of desmoid tumor of the anterolateral abdominal wall presented with a large mass of the left flank and iliac fossa. The diagnostic was suspected radiologically following an abdominopelvic computed tomography (CT) and magnetic resonance imaging (MRI). An exploratory laparotomy found a large mass infiltrating the left rectus abdominis muscle, a part of the flat left abdominal muscles, and the left iliac crest. The patient underwent a total resection of the mass involving the left rectus muscle with autoplasty by a pedicled flap of the left LATA fascia with the placement of a bifacial mesh.Histologic analysis of the operatory specimen confirmed the diagnosis of a desmoid tumor of the abdominal wall.The patient has been discharged from the hospital on the fifth day post-operatory with an uneventful recovery; she was in good health after a one-year follow-up.Clinical discussionDesmoid tumors of the abdomen are very rare. Although this tumor is histologically benign, it has the potential of invading vital structures and has a high rate of local recurrence.Histology staining confirms the diagnosis, surgery is the gold standard in the management of this pathology.ConclusionWe highlight the importance of radical surgical excision to avoid desmoid tumor complications and to minimize the recurrence risk.
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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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Project description:BackgroundThe efficacy of the classical treatment modalities surgery and radiotherapy in the treatment of aggressive fibromatosis is presently disputed and there is a shift towards a more conservative approach. The aim of the present study is to objectify tumor growth in patients with extra-abdominal or abdominal wall aggressive fibromatosis, while adhering to a "watchful waiting" policy. Other objectives are to investigate quality of life and to identify factors associated with tumor growth, in particular the relation with the presence of a CTNNB1-gene mutation in the tumor.Design and methodsGRAFITI is a nationwide, multicenter, prospective registration trial. All patients with extra-abdominal or abdominal wall aggressive fibromatosis are eligible for inclusion in the study. Main exclusion criteria are: history of familiar adenomatous polyposis, severe pain, functional impairment, life/limb threating situations in case of progressive disease. Patients included in the study will be treated with a watchful waiting policy during a period of 5 years. Imaging studies with ultrasound and magnetic resonance imaging scan will be performed during follow-up to monitor possible growth: the first years every 3 months, the second year twice and the yearly. In addition patients will be asked to complete a quality of life questionnaire on specific follow-up moments. The primary endpoint is the rate of progression per year, defined by the Response Evaluation Criteria In Solid Tumors (RECIST). Secondary endpoints are quality of life and the rate of influence on tumor progression for several factors, such as CTNNB1-mutations, age and localization.DiscussionThis study will provide insight in tumor behavior, the effect on quality of life and clinicopathological factors predictive of tumor progression.Trial registrationThe GRAFITI trial is registered in the Netherlands National Trial Register (NTR), number 4714 .
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Project description:Extra-abdominal desmoid tumors are a significant cause of morbidity in patients with familial adenomatous polyposis syndrome. Understanding of the basic biology and natural history of these tumors has increased substantially over the past decade. Accordingly, medical and surgical management of desmoid tumors has also evolved. This paper analyzes recent evidence pertaining to the epidemiology, molecular biology, histopathology, screening, and treatment of extra-abdominal desmoid tumors associated with familial adenomatous polyposis syndrome.
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Project description: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.
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