Aggressive fibromatosisaggressive fibromatosis, aggressive fibromatosis desmoid tumors
Aggressive fibromatosis is a rare condition marked by the presence of desmoid tumors Desmoid tumors can arise in virtually any part of the body, and are tumors that arise from cells called fibroblasts, which are found throughout the body and provide structural support, protection to the vital organs, and play a critical role in wound healing These tumors tend to occur in women in their thirties, but can occur in anyone at any age They can be either relatively slow-growing or malignant However, aggressive fibromatosis is locally aggressive When they are aggressive they can cause life-threatening problems or even death when they compress vital organs such as intestines, kidney, lungs, blood vessels, nerves etc Most cases are sporadic, but some are associated with familial adenomatous polyposis FAP Approximately 10% of individuals with Gardner's syndrome, a type of FAP with extracolonic features, have desmoid tumors1
Histologically they resemble very low-grade fibrosarcomas,2 but they are very locally aggressive and tend to recur even after complete resection There is a tendency for recurrence in the setting of prior surgery; in one study, two-thirds of patients with desmoid tumors had a history of prior abdominal surgery3
Risk factors for desmoid disease amongst FAP patients include female sex, a 3' APC mutation, a positive family history and a history of previous abdominal surgery4
- 1 Classification
- 2 Treatment
- 3 References
- 4 External links
ClassificationeditDesmoid tumor Desmoid fibromatosis, H&E stain Banal fibroblasts infiltrate the adjacent tissue in fascicles Mitoses may be infrequent
Desmoid tumors may be classified as extra-abdominal, abdominal wall, or intra-abdominal the last is more common in patients with FAP It is thought that the lesions may develop in relation to estrogen levels or trauma/operations
A 3' APC mutation is the most significant risk factor for intra-abdominal desmoid development amongst FAP patients5 FAP patients presenting with an abdominal wall desmoid pre-operatively are at an increased risk of developing an intra-abdominal desmoid post-operatively6
Desmoid tumours of the breast are rare Although benign, they can mimic breast cancer on physical examination, mammography and breast ultrasound and can also be locally invasive Even though they occur sporadically, they can also be seen as a part of Gardner's syndrome A high index of suspicion and a thorough triple examination protocol is necessary to detect rare lesions like a desmoid tumour which can masquerade as breast carcinoma Desmoid tumour of the breast may present a difﬁculty in the diagnosis especially where imaging studies are not conclusive and suggest a more ominous diagnosis7
Treatment may consist of watching and waiting, complete surgical removal, radiation therapy, antiestrogens ex Tamoxifen, NSAIDs, chemotherapy or microwave ablation
Patients with desmoid tumors should be evaluated by a multi-disciplinary team of surgeons, medical oncologists, radiation oncologists, geneticists and nurses There is no cure for desmoid tumors and when possible patients are encouraged to enlist in clinical trials8
A biopsy is always indicated as the deﬁnitive method to determine nature of the tumour Management of these lesions is complex, the main problem being the high rates of recurrence in FAP associated disease Conversely, for intra-abdominal fibromatosis without evidence of FAP, although extensive surgery may still be required for local symptoms, the risk of recurrence appears to be lower9 Wide surgical resection with clear margins is the most widely practiced technique with radiation, chemotherapy, or hormonal therapy being used to reduce the risk of recurrence7
Current experimental studies are being done with Gleevec Imatinib and Nexavar sorafenib for treatment of desmoid tumors, and show promising success rates
- ^ Nieuwenhuis MH, De Vos Tot Nederveen Cappel W, Botma A, et al February 2008 "Desmoid tumors in a Dutch cohort of patients with familial adenomatous polyposis" Clin Gastroenterol Hepatol 6 2: 215–9 PMID 18237870 doi:101016/jcgh200711011
- ^ "desmoid" at Dorland's Medical Dictionary
- ^ Lynch HT, Fitzgibbons R December 1996 "Surgery, desmoid tumors, and familial adenomatous polyposis: case report and literature review" Am J Gastroenterol 91 12: 2598–601 PMID 8946994
- ^ Sinha A, Clark SK 2010 "Risk factors predicting desmoid occurrence in patients with familial adenomatous polyposis: a meta-analysis" Colorectal Dis 13 11: no PMID 20528895 doi:101111/j1463-1318201002345x
- ^ Sinha A, Clark SK June 2010 "Risk factors predicting intra-abdominal desmoids in familial adenomatous polyposis: a single centre experience" Tech Coloproctol 14 2: 141–6 PMID 20352275 doi:101007/s10151-010-0573-4
- ^ Sinha A, Clark SK 2010 "Surgical prophylaxis in familial adenomatous polyposis: do pre-existing desmoids outside the abdominal cavity matter" Fam Cancer 9 3: 407–11 PMID 20428953 doi:101007/s10689-010-9342-9
- ^ a b Rammohan A, Wood JJ 2012 "Desmoid tumour of the breast as a manifestation of Gardner's syndrome" Int J Surg Case Rep 3 5: 139–142 PMC 3312056 PMID 22370045 doi:101016/jijscr201201004
- ^ "Archived copy" Archived from the original on 2015-07-27 Retrieved 2015-08-04
- ^ Wilkinson MJ, Fitzgerald JE, Thomas JM, Hayes AJ, Strauss DC 2012 "Surgical resection for non-familial adenomatous polyposis-related intra-abdominal fibromatosis" BJS 99 5: 706–13 PMID 22359346 doi:101002/bjs8703
- Aggressive Desmoid Fibromatosis: A Problem for the Sarcoma Team by Randall Burt, MD and David Viskochil, MD, PhD
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