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Mastoraki A, Schizas D, Vassiliu S, Saliaris K, Giagkos GC, Theochari M, Vergadis C, Tolia M, Vassiliu P, Felekouras E. Evaluation of diagnostic algorithm and therapeutic interventions for intra-abdominal desmoid tumors. Surg Oncol 2022; 41:101724. [DOI: 10.1016/j.suronc.2022.101724] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/20/2021] [Revised: 01/03/2022] [Accepted: 02/13/2022] [Indexed: 11/09/2022]
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van Broekhoven DLM, Grunhagen DJ, Verhoef C. Abdominal Desmoid Tumors: Hands Off? Ann Surg Oncol 2016; 23:2128-30. [DOI: 10.1245/s10434-016-5160-0] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/15/2016] [Indexed: 11/18/2022]
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Palladino E, Nsenda J, Siboni R, Lechner C. A giant mesenteric desmoid tumor revealed by acute pulmonary embolism due to compression of the inferior vena cava. AMERICAN JOURNAL OF CASE REPORTS 2014; 15:374-7. [PMID: 25180474 PMCID: PMC4159246 DOI: 10.12659/ajcr.891044] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
Abstract
Patient: Male, 69 Final Diagnosis: Mesenteric desmoid tumor Symptoms: — Medication: — Clinical Procedure: — Specialty: Surgery
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Affiliation(s)
- Elisa Palladino
- Department of General and Digestive Surgery, Chalons en Champagne Hospital, Chalons en Champagne, France
| | - Joseph Nsenda
- Department of General and Digestive Surgery, Chalons en Champagne Hopsital, Chalons en Champagne, France
| | - Renaud Siboni
- Department of General and Digestive Surgery, Chalons en Champagne Hopsital, Chalons en Champagne, France
| | - Christian Lechner
- Department of General and Digestive Surgery, Chalons en Champagne Hopsital, Chalons en Champagne, France
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Jiang D, He D, Hou Y, Lu W, Shi Y, Hu Q, Lu S, Xu C, Liu Y, Liu J, Tan Y, Zhu X. Subsequent intra-abdominal fibromatosis mimicking recurrent gastrointestinal stromal tumor. Diagn Pathol 2013; 8:125. [PMID: 23902675 PMCID: PMC3751073 DOI: 10.1186/1746-1596-8-125] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/06/2013] [Accepted: 07/04/2013] [Indexed: 02/06/2023] Open
Abstract
Abstract Intra-abdominal fibromatosis (IAF) commonly develops in patients who had abdominal surgery. In rare instances, it occurs subsequent to gastrointestinal stromal tumor (GIST). This special situation has clinical significance in imatinib era. About 1000 patients with GIST in our institution from 1993 to 2010 were re-evaluated based on their clinical and pathological data, the treatment strategies and the follow-up information. We identified 2 patients who developed IAF after GIST resection. Patient 1 was a 54 year-old male and had 5 cm × 4.5 cm × 3.5 cm jejunal GIST excised on February 22, 1994. Three years later, an abdominal mass with 7 cm × 6 cm × 3 cm was identified. He was diagnosed as recurrent GIST from clinical point of view. After excision, the second tumor was confirmed to be IAF. Patient 2 was a 45-year-old male and had 6 cm × 4 cm × 3 cm duodenal GIST excised on August 19, 2008. One year later, a 4 cm mass was found at the original surgical site. The patient refused to take imatinib until the tumor increased to 8 cm six months later. The tumor continued to increase after 6 months’ imatinib therapy, decision of surgical resection was made by multidisciplinary team. The second tumor was confirmed to be IAF with size of 17 cm × 13 cm × 11 cm. Although IAF subsequent to GIST is very rare, it is of clinical significance in imatinib era as an influencing factor for making clinical decision. Virtual slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1076715989961803
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Affiliation(s)
- Dongxian Jiang
- Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai 200032, PR China
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Felice FD, Musio D, Caiazzo R, Dipalma B, Grapulin L, Semproni CP, Tombolini V. An unusual case of fatty liver in a patient with desmoid tumor. World J Gastroenterol 2012; 18:3173-6. [PMID: 22791954 PMCID: PMC3386332 DOI: 10.3748/wjg.v18.i24.3173] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/23/2011] [Revised: 03/02/2012] [Accepted: 05/06/2012] [Indexed: 02/06/2023] Open
Abstract
A desmoid tumor, also known as aggressive fibromatosis, is a rare benign neoplasm that arises from fascial or musculoaponeurotic tissues. It can occur in any anatomical location, most commonly the abdominal wall, shoulder girdle and retroperitoneum. The typical clinical presentation is a painless mass with a slow and progressive invasion of contiguous structures. It is associated with a high local recurrence rate after resection. Many issues regarding the optimal treatment of desmoid tumors remain controversial. Aggressive surgical resection with a wide margin (2-3 cm) remains the gold standard treatment with regard to preserving quality of life. Radiotherapy alone has been shown to be effective for the control of unresectable or recurrent lesions. Desmoid tumors tend to be locally infiltrative, therefore, the fields must be generous to prevent marginal recurrence. The radiation dose appropriate for treating desmoid tumors remains controversial. We present a 25-year-old Caucasian man with local recurrence of a desmoid tumor after repeated surgical resection, treated with radiotherapy. The patient achieved complete tumor regression at 4 mo after radiotherapy, and he is clinically free of disease at 12 mo after the end of treatment, with an acceptable quality of life. The patient developed short bowel syndrome as a complication of second surgical resection. Consequently, radiotherapy might have worsened an already present malabsorption and so led to steatohepatitis.
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Mortality of intra-abdominal desmoid tumors in patients with familial adenomatous polyposis: a single center review of 154 patients. Ann Surg 2012; 255:511-6. [PMID: 22323009 DOI: 10.1097/sla.0b013e31824682d4] [Citation(s) in RCA: 55] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
INTRODUCTION Intra-abdominal desmoid tumors are one of the leading causes of death in patients with familial adenomatous polyposis. Their behavior is unpredictable and their biology is poorly understood, accounting for the lack of a standardized medical and surgical approach. The aim of this study was to evaluate the mortality rate of patients with intra-abdominal desmoid tumors and to identify prognostic factors for the evolution of the disease. MATERIALS AND METHODS A total of 154 patients with intra-abdominal desmoid tumors were included in the study. Each tumor was staged and each patient was categorized according to the stage of their most advanced tumor. Mortality was analyzed and the univariate risk factors associated with survival were included in a multivariable Cox regression model. A scoring system was derived from the multivariate analysis to refine outcomes within stages. RESULTS Five-year survival of patients with stage I, II, III, and IV intra-abdominal desmoid tumor were 95%, 100%, 89%, and 76% respectively (P < 0.001). Severe pain/narcotic dependency, tumor size larger than 10 cm, and need for total parenteral nutrition were shown to further define survival within stages. Five-year survival rate of stage IV patient with all of the above-mentioned risk factors was only 53%. CONCLUSIONS Our study confirmed the validity of the staging system to predict mortality in patients with intra-abdominal desmoid tumors and identified additional risk factors able to better define the risk of death within each stage. Risk stratification is crucial in directing patients with advanced disease and poor prognosis to the most appropriate medical and surgical options.
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Wilkinson MJ, Fitzgerald JEF, Thomas JM, Hayes AJ, Strauss DC. Surgical resection for non-familial adenomatous polyposis-related intra-abdominal fibromatosis. Br J Surg 2012; 99:706-13. [PMID: 22359346 DOI: 10.1002/bjs.8703] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/12/2012] [Indexed: 12/29/2022]
Abstract
BACKGROUND Intra-abdominal fibromatosis (IAF) in the context of familial adenomatosis polyposis (FAP) is associated with significant morbidity and high recurrence rates after surgical resection. Non-surgical treatments are therefore advocated. This study explored outcomes in patients with IAF not associated with FAP who underwent surgical resection. METHODS Data were analysed from a prospectively collected database at a sarcoma tertiary referral centre. RESULTS From 2001 to 2011, 15 patients without FAP underwent primary curative surgical resection of IAF. Their median (range) age was 42 (19-64) years. Median tumour size was 18 (8.5-25) cm and weight 1306 (236-2228) g. Complete macroscopic clearance was obtained in all patients. There were no deaths in hospital or within 30 days and only one patient developed a major complication. Median follow-up was 40 (6-119) months. During follow-up two patients developed a recurrence after a disease-free interval of 12 and 16 months. CONCLUSION In contrast to FAP-associated IAF, non-FAP-associated IAF has a very low recurrence rate after surgical resection. Surgical resection is therefore advocated as first-line treatment in patients with non-FAP-associated IAF when resection can be performed with low morbidity.
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Affiliation(s)
- M J Wilkinson
- Sarcoma and Melanoma Unit, Department of Academic Surgery, Royal Marsden Hospital NHS Foundation Trust, Fulham Road, London SW3 6JJ, UK.
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Sinha A, Hansmann A, Bhandari S, Gupta A, Burling D, Rana S, Phillips RK, Clark SK, Goh V. Imaging assessment of desmoid tumours in familial adenomatous polyposis: is state-of-the-art 1.5 T MRI better than 64-MDCT? Br J Radiol 2012; 85:e254-61. [PMID: 22215881 DOI: 10.1259/bjr/42420290] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023] Open
Abstract
OBJECTIVE Desmoid tumour is a common extraintestinal manifestation of patients with familial adenomatous polyposis (FAP) who have undergone prophylactic colectomy. We aimed to determine whether MRI provides equivalent or better assessment of desmoid tumours than CT, the current first-line investigation. METHODS Following ethics approval and informed consent, FAP patients with known desmoid tumour underwent contrast-enhanced 64-slice multidetector CT (MDCT) and 1.5 T MRI (incorporating T(1) weighted, T(2) weighted, short tau inversion-recovery and T(1) weighted with contrast, axial, sagittal and coronal sequences). The number, site, size, local extent, tumour signal intensity and desmoid-to-aorta enhancement ratio were analysed. RESULTS MRI identified 23 desmoid tumours in 9 patients: 9 intra-abdominal desmoid (IAD) tumours, 10 abdominal wall desmoid (AWD) tumours and 4 extra-abdominal desmoid (EAD) tumours. CT identified only 21 desmoids; 1 EAD and 1 AWD were not identified. The two modalities were equivalent in terms of defining local extent of desmoid. Five IAD tumours involved the bowel, six caused ureteric compression and none compromised the proximal superior mesenteric artery. There was no difference in median desmoid size: 56.7 cm(2) (range 2-215 cm(2)) on MDCT and 56.3 cm(2) (3-215 cm(2)) on MRI (p=0.985). The mean MRI enhancement ratio, at 1.12 (standard deviation 0.43), was greater than the CT enhancement ratio, which was 0.48 (0.16) (p<0.0001). High signal intensity on T(2) MRI was associated with increased MRI enhancement ratio (p=0.006). CONCLUSIONS MRI is at least equivalent (and may be superior) to MDCT for the detection of desmoid tumours in FAP. Coupled with the advantage of avoiding radiation, it should be considered as the primary imaging modality for young FAP patients.
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Affiliation(s)
- A Sinha
- Polyposis Registry, St Mark's Hospital, Harrow, UK
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Catania G, Ruggeri L, Iuppa G, Di Stefano C, Cardì F, Iuppa A. Abdominal wall reconstruction with intraperitoneal prosthesis in desmoid tumors surgery. Updates Surg 2011; 64:43-8. [DOI: 10.1007/s13304-011-0109-0] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/05/2011] [Accepted: 08/27/2011] [Indexed: 01/15/2023]
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Johnson PT, Horton KM, Fishman EK. Nonvascular mesenteric disease: utility of multidetector CT with 3D volume rendering. Radiographics 2009; 29:721-40. [PMID: 19448112 DOI: 10.1148/rg.293085113] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Creation of isotropic volumes with submillimeter multidetector computed tomography (CT) has expanded interpretative practice to routinely include two-dimensional (2D) and three-dimensional (3D) postprocessing techniques. Currently, 2D multiplanar reformatting, maximum intensity projection, and 3D volume rendering are available on most workstations. Only volume rendering yields a 3D display that depicts all tissue types from any orientation. Utility is not limited to vascular applications, as 3D volume rendering can be used to evaluate neoplastic, infectious, and inflammatory processes that affect the small-bowel mesentery. Specifically, interactive interpretation of multidetector CT data sets with volume rendering can help characterize nonvascular mesenteric disease, elucidate its extent through more comprehensive display, and facilitate the identification of complications.
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Affiliation(s)
- Pamela T Johnson
- Russell H. Morgan Department of Radiology and Radiologic Sciences, Johns Hopkins School of Medicine, 601 N Caroline St, Room 3140D, Baltimore, MD 21287, USA.
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Groen EJ, Roos A, Muntinghe FL, Enting RH, de Vries J, Kleibeuker JH, Witjes MJH, Links TP, van Beek AP. Extra-intestinal manifestations of familial adenomatous polyposis. Ann Surg Oncol 2008; 15:2439-50. [PMID: 18612695 PMCID: PMC2518080 DOI: 10.1245/s10434-008-9981-3] [Citation(s) in RCA: 170] [Impact Index Per Article: 10.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/12/2007] [Revised: 05/07/2008] [Accepted: 05/08/2008] [Indexed: 12/20/2022]
Abstract
Familial adenomatous polyposis (FAP) is an autosomal dominantly inherited disorder, which results from a germ line mutation in the APC (adenomatous polyposis coli) gene. FAP is characterized by the formation of hundreds to thousands of colorectal adenomatous polyps. Although the development of colorectal cancer stands out as the most prevalent complication, FAP is a multisystem disorder of growth. This means, it is comparable to other diseases such as the MEN syndromes, Von Hippel-Lindau disease and neurofibromatosis. However, the incidence of many of its clinical features is much lower. Therefore, a specialized multidisciplinary approach to optimize health care-common for other disorders-is not usually taken for FAP patients. Thus, clinicians that care for and counsel members of high-risk families should have familiarity with all the extra-intestinal manifestations of this syndrome. FAP-related complications, for which medical attention is essential, are not rare and their estimated lifetime risk presumably exceeds 30%. Affected individuals can develop thyroid and pancreatic cancer, hepatoblastomas, CNS tumors (especially medulloblastomas), and various benign tumors such as adrenal adenomas, osteomas, desmoid tumors and dental abnormalities. Due to improved longevity, as a result of better prevention of colorectal cancer, the risk of these clinical problems will further increase. We present a clinical overview of extra-intestinal manifestations, including management and treatment options for the FAP syndrome. Furthermore, we provide recommendations for surveillance of FAP complications based on available literature.
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Affiliation(s)
- Emma J Groen
- Department of Endocrinology, University Medical Center Groningen, University of Groningen, De Brug 4.069, P.O. Box 30.001, 9700 RB, Groningen, The Netherlands
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Sakorafas GH, Nissotakis C, Peros G. Abdominal desmoid tumors. Surg Oncol 2007; 16:131-42. [PMID: 17719772 DOI: 10.1016/j.suronc.2007.07.009] [Citation(s) in RCA: 87] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/01/2007] [Revised: 06/23/2007] [Accepted: 07/23/2007] [Indexed: 12/11/2022]
Abstract
Desmoid tumors are rare, benign, fibromatous lesions that are the result of abnormal proliferation of myofibroblasts. Desmoid tumors can be classified as extra-abdominal and abdominal. Abdominal desmoid tumors are either superficial or intraabdominal. These tumors are associated with a high recurrence rates, even if their microscopic characters indicate a benign disease; their biologic behavior often indicates rather a "malignant" disease, which can cause even the death. Intraabdominal desmoid tumors can engulf surrounding viscera and vessels, thereby greatly complicating their surgical treatment. Management is multidisciplinary. Simple observation is a reasonable management option for asymptomatic patients; spontaneous regression of these tumors may be observed. Complete excision is the treatment of choice for tumors causing symptoms or complications. Surgery should be minimized as much as feasible, while at the same time achieving free margins. Adjuvant therapy should be considered in selected cases; the role of other management options (including gene transfer therapy) is currently under intensive investigation.
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Affiliation(s)
- George H Sakorafas
- 4th Department of Surgery, Athens University, Medical School, ATTIKON University Hospital, Arkadias 19-21, GR-115 26, Athens, Greece.
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Latchford AR, Sturt NJH, Neale K, Rogers PA, Phillips RKS. A 10-year review of surgery for desmoid disease associated with familial adenomatous polyposis. Br J Surg 2006; 93:1258-64. [PMID: 16952208 DOI: 10.1002/bjs.5425] [Citation(s) in RCA: 75] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
BACKGROUND Desmoid tumours affect 10-25 per cent of patients with familial adenomatous polyposis and represent a major cause of morbidity and mortality. Surgery for intra-abdominal desmoids has traditionally been used as a last resort or to manage obstructive complications. The aim was to review 10 years of desmoid surgery in patients with familial adenomatous polyposis from a single centre. METHODS Patients who had surgery for desmoid disease between 1994 and 2004 were identified from the Polyposis Registry database and their hospital notes reviewed. RESULTS Twenty patients had surgery to remove 32 desmoid tumours (16 intra-abdominal, 12 abdominal wall, four extra-abdominal). Complete clearance was achieved in 19 tumours and, of these, clinically significant recurrence occurred in eight. There was no difference in recurrence rates for site or sex. There was no operative mortality. Intra-abdominal desmoid resection was associated with a mean resection of 45.55 (range 10-200) cm of small bowel. One patient required long-term parenteral feeding. Median follow-up was 5 (range 0.6-10) years. During this period, one patient died (metastatic duodenal cancer); there was no mortality from desmoid disease. CONCLUSION Surgery for intra-abdominal desmoids in selected patients is less hazardous than previously reported. Surgery for abdominal wall and extra-abdominal tumours is safe. However, disease recurrence remains a major problem.
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Affiliation(s)
- A R Latchford
- Polyposis Registry, St Mark's Hospital, Northwick Park, Harrow HA1 3UJ, UK
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Abstract
Desmoid tumors are nonencapsulated, locally invasive tumors of fibrous origin. Although lacking the ability to metastasize, they are notorious for recurrence. A wide variety of locations have been documented in the literature with respect to the origin of these tumors. Intra-abdominal desmoids are commonly found in the mesentery. We present the report of a case in which a sporadic desmoid tumor originated from the wall of the ileum. The patient was successfully treated with surgery alone, and has remained recurrence free on follow-up. Modalities of treatment documented in the literature include surgery, nonsteroidal, anti-inflammatory drugs such as sulindac, and chemotherapeutic drugs such as vinblastine and methotrexate, radiotherapy, and, recently, tyrosine kinase inhibitors such as Imatinib (gleevec). Of all the options described, surgery with tumor-free margins has been defined as the best treatment modality.
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González MA, Menéndez R, Ayala JM, Herrero M, Cuesta J, Domínguez A, Martínez M, Graña JL, Pozo F. [Intra-abdominal desmoid tumor]. Cir Esp 2006; 77:362-4. [PMID: 16420952 DOI: 10.1016/s0009-739x(05)70872-1] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/21/2022]
Abstract
Aggressive fibromatosis (desmoid tumor) are rare connective tissue tumors that occur sporadically or in association with familial adenomatous polyposis. The etiology is unknown and clinical findings depend on growth into neighboring structures. Biopsy is required to establish the diagnosis. The treatment of choice is surgery. We report a case with unusual localization in this form of presentation. The patient remains asymptomatic 15 months after surgery.
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Affiliation(s)
- M A González
- Servicio de Cirugía General y del Aparato Digestivo, Hospital Vital Alvarez-Buylla, Mieres, Asturias, España.
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Church J, Berk T, Boman BM, Guillem J, Lynch C, Lynch P, Rodriguez-Bigas M, Rusin L, Weber T. Staging intra-abdominal desmoid tumors in familial adenomatous polyposis: a search for a uniform approach to a troubling disease. Dis Colon Rectum 2005; 48:1528-34. [PMID: 15906134 DOI: 10.1007/s10350-005-0018-8] [Citation(s) in RCA: 74] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
INTRODUCTION Desmoid tumors are a clinical problem in 12 to 15 percent of patients with familial adenomatous polyposis. There is no predictably effective treatment for intra-abdominal desmoid tumors, which sometimes cause significant complications by their effects on the ureters or bowel. The relative rarity and the clinical heterogeneity of intra-abdominal desmoid tumors make randomized studies difficult to do. In this article a staging system is proposed to make multi-institutional studies easier. METHODS Intra-abdominal desmoid tumors can be staged according to their size, clinical presentation and growth pattern. CONCLUSION A way of staging intra-abdominal desmoid tumors is proposed to facilitate stratification by disease severity during collaborative studies of various treatments.
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Affiliation(s)
- James Church
- Department of Colorectal Surgery, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA
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Azizi L, Balu M, Belkacem A, Lewin M, Tubiana JM, Arrivé L. MRI Features of Mesenteric Desmoid Tumors in Familial Adenomatous Polyposis. AJR Am J Roentgenol 2005; 184:1128-35. [PMID: 15788583 DOI: 10.2214/ajr.184.4.01841128] [Citation(s) in RCA: 54] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022]
Affiliation(s)
- Louisa Azizi
- Department of Radiology, Hôpital Saint-Antoine, 184 rue du faubourg Saint-Antoine, Paris 75012, France.
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Chew C, Reid R, O'Dwyer PJ. Evaluation of the long term outcome of patients with extremity desmoids. Eur J Surg Oncol 2004; 30:428-32. [PMID: 15063897 DOI: 10.1016/j.ejso.2004.01.010] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/21/2004] [Indexed: 10/26/2022] Open
Abstract
BACKGROUND The optimum management and long-term outcome for patients with extremity desmoids remains unclear. The aim of this study was to evaluate the long-term outcome of patients with extremity desmoids and to compare patient episodes treated with surgery alone with those treated by radiotherapy either alone or with surgery at any stage of the patient's disease. METHODS All patients with extremity desmoids followed at an oncology clinic with an interest in soft tissue tumours and treated between 1959 and 1996 were identified. All were traced back to their primary presentation and first treatment episode. RESULTS During the study period 42 patients, 29 females and 13 males were identified. Median follow-up of the patient population was 12.5 years (range 3-30.9). Median age at presentation was 27 years (range 1-69). The lesion was in the upper limb or shoulder in 18, lower limp or pelvis 17 and head and neck 7. Forty patients underwent operation as their primary treatment, 25 of whom developed recurrent tumour. In all there were 41 recurrences following 78 episodes of surgery alone. This compared to 10 recurrences following 16 episodes of radiotherapy or radiotherapy with surgery (p = 0.467). There was no association between primary, first or second recurrence and the development of a further relapse. The median interval to recurrence after surgery alone was 12 months (interquartile range (IQR) 6-13) as opposed to 52 months (IQR 33-62) for patients treated with primary or adjuvant radiotherapy (p= 0.004). CONCLUSION Results are poor after both radiotherapy and surgery for extremity desmoids and research into other methods of managing this rare condition need to be considered.
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Affiliation(s)
- C Chew
- University Department of Surgery, Western Infirmary, Glasgow G11 6NT, UK
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Abstract
Desmoid tumours (DT) are rare benign tumours that do not metastasise, but tend to invade locally. DT are frequently seen in patients with familial adenomatous polyposis (FAP), and diagnosis and treatment are often difficult. Surgical trauma, genetic predisposition and hormonal factors are considered to be correlated with the development and growth of DT. In patients with FAP, 50% of the tumours are localised intra-abdominally, and 85-100% of these are mesenteric. DT frequently present as non- tender, slowly growing masses. The symptoms are abdominal pain, vomiting, diarrhoea or haematochezia. Mesenteric DT can cause small bowel obstruction or ischaemia, hydronephrosis or form fistulas. Diagnosis is obtained through biopsy and the extension is determined by a CT-scan. Surgical excision is recommended in patients with DT in the abdominal wall. First line treatment of mesenteric DT is a NSAID in combination with tamoxifen. Surgery may be considered in case of a small and well-defined DT with no signs of invasion of vital structures, and in cases of imminent bowel ischaemia or obstruction. The prognosis in mesenteric DT is serious, and improvement of the therapeutic strategy awaits current international studies.
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Affiliation(s)
- A L Knudsen
- The Danish Polyposis Register, Department of Surgical Gastroenterology, Hvidovre University Hospital, Hvidovre, Denmark
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Overhaus M, Decker P, Fischer HP, Textor HJ, Hirner A. Desmoid tumors of the abdominal wall: A case report. World J Surg Oncol 2003; 1:11. [PMID: 12890284 PMCID: PMC169179 DOI: 10.1186/1477-7819-1-11] [Citation(s) in RCA: 46] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/16/2003] [Accepted: 07/09/2003] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND: Desmoid tumors are slow growing deep fibromatoses with aggressive infiltration of adjacent tissue but without any metastatic potential. CASE PRESENTATION: We report on two female patients with desmoid tumor of the abdominal wall who underwent primary resection. Both patients had a history of an earlier abdominal surgery. Preoperative evaluation included abdominal ultrasound, magnetic resonance imaging and computed tomography. The histology in both cases revealed a desmoid tumor. CONCLUSION: Complete surgical resection is the first line management of this tumor entity.
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Affiliation(s)
- Marcus Overhaus
- Department of General-, Visceral-, Thoracic- and Vascular Surgery, Sigmund Freud Str. 25, 53105 Bonn, University of Bonn, Germany
| | - Pan Decker
- Department of General-, Visceral-, Thoracic- and Vascular Surgery, Sigmund Freud Str. 25, 53105 Bonn, University of Bonn, Germany
| | - Hans Peter Fischer
- Department of Pathology, Sigmund Freud Str. 25, 53105 Bonn, University of Bonn, Germany
| | - Hans Jochen Textor
- Department of Radiology, Sigmund Freud Str. 25, 53105 Bonn, University of Bonn, Germany
| | - Andreas Hirner
- Department of General-, Visceral-, Thoracic- and Vascular Surgery, Sigmund Freud Str. 25, 53105 Bonn, University of Bonn, Germany
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Zlotecki RA, Scarborough MT, Morris CG, Berrey BH, Lind DS, Enneking WF, Marcus RB. External beam radiotherapy for primary and adjuvant management of aggressive fibromatosis. Int J Radiat Oncol Biol Phys 2002; 54:177-181. [PMID: 12182989 DOI: 10.1016/s0360-3016(02)02926-7] [Citation(s) in RCA: 76] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
Abstract
PURPOSE To review a large single-institution experience in the management of aggressive fibromatosis to determine the effectiveness of external beam radiotherapy (EBRT) and identify the presentation and treatment variables predictive of locoregional control. METHODS AND MATERIALS Between 1975 and 2000, 72 patients were treated with EBRT for a pathologically confirmed diagnosis of aggressive fibromatosis. Thirty patients were treated at the primary presentation and 42 at the time of a locoregional recurrence. Minimal 2-year follow-up data were available for 65 patients (median 6 years). Megavoltage irradiation with 60Co to 20 MV photons or electron therapy was used for all patients. Most patients were treated after attempted complete surgical resection; 16 patients underwent pretreatment biopsy alone. The prescribed treatment was standard (1.8 Gy) daily fractions in 42 cases and 1.2 Gy fractions b.i.d. in 23 cases. The median prescribed dose was 54 Gy. The prognostic variables and treatment results were evaluated by Kaplan-Meier actuarial analysis. RESULTS Locoregional control was achieved in 52 of 65 patients. The 5-year actuarial locoregional control was 83%. Locoregional failure occurred in 13 patients (11 in patients with recurrent tumors). Only two failures occurred within the irradiation fields; nine failures occurred at the field margins. Eleven patients were salvaged by surgery: wide excision in nine and amputation in two. The only prognostic factor significant for locoregional control was primary vs. recurrent presentation (p = 0.0193). The 5-year locoregional control rates for irradiation at initial presentation and at recurrence were 96% and 75%, respectively. The variables without significance for locoregional control included primary tumor location, surgical procedures performed, resection margins, and gross vs. microscopic residual tumor at irradiation. Lymphedema was the most common late effect, occurring in 7 patients, 5 with prior treatment. Bone fracture occurred in 3 patients; all 3 had fibromatosis involving the bone at presentation but without recurrence at the time of fracture. CONCLUSION EBRT is effective treatment for aggressive fibromatosis. The probability of locoregional control decreases with multiple prior recurrences.
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Affiliation(s)
- Robert A Zlotecki
- Department of Radiation Oncology, Health Science Center, University of Florida College of Medicine, Gainesville, FL 32610-0385, USA.
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