Updates on abdominal desmoid tumors
Bernardino Rampone, Corrado Pedrazzani, Daniele Marrelli, Enrico Pinto, Franco Roviello
World J Gastroenterol 2007 December 7; 13(45): 5985-5988
Intra-abdominal desmoid tumors usually involve the
mesentery and often involve the mesenteric vessels. They
invade the mesentery diffusely, kink loops of bowel and
can cause ureters obstruction This feature
requires a complex surgery and often radical resection
is impossible to achieve]. Therefore the management
of intra-abdominal desmoid tumors is complex and is
dependant on their clinical behavior.
Surgical management of intra-abdominal
desmoid tumours
Smith AJ, Lewis JJ, Merchant NB, Leung DH, Woodruff
JM, Brennan MF.
. Br J Surg 2000; 87: 608-613
Operation can cure patients with intra‐abdominal desmoid tumours, but may result in significant morbidity, especially from loss of small intestine. No other therapy is a predictably good alternative to operation but the natural history of desmoids is often characterized by prolonged periods of stability or even regression. A period of watchful waiting, until significant symptoms develop, may be the most appropriate course in patients who risk mesenteric vascular injury or substantial enterectomy with attempts at resection. © 2000 British Journal of Surgery Society Ltd
Desmoids in familial adenomatous polyposis
- Author: S. K. Clark,R. K. S. Phillips
- Published: Dec 07, 2005
- Pages: 1494-1504
- DOI: 10.1002/bjs.1800831105
Abstract
Clinical desmoid disease affects approximately 10 per cent of patients with familial adenomatous polyposis (FAP); the subclinical rate is unknown. Desmoids are probably neoplastic rather than regenerative in origin and may arise in association with germline or somatic mutations at or beyond codon 1444 of the APC gene. Intra‐abdominal desmoids behave unpredictably but are an important cause of death in those with FAP. Signal intensity on magnetic resonance imaging reflects tumour cellularity, which in part determines progression, and this may help management. Surgical treatment of advanced desmoids is hazardous, but medical treatments have limited success. Chemotherapy with doxorubicin and dacarbazine is currently under evaluation.
Desmoid tumours complicating familial adenomatous polyposis
- Author:S. K. Clark,K. F. Neale,J. C. Landgrebe,R. K. S. Phillips
- Published: Dec 10, 2002
- Pages: 1185-1189
- DOI:10.1046/j.1365-2168.1999.01222
Abdominal wall desmoids caused no deaths or significant morbidity; although recurrence was common after excision, the treatment was safe. Intra‐abdominal desmoids can cause serious complications and treatment is often unsuccessful; in particular, surgery for desmoids at this site is hazardous.
Surgical management of desmoid tumors of the female pelvis
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AndreaMariani, MD Antonio GNascimento, MD Maurice JWebb, MD (FACS) Franklin HSim, MD Karl CPodratz, MD, PhD (FACS
Journal of the American College of Surgeons
Volume 191, Issue 2 , Pages 175-183, August 2000
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Although recent reports demonstrate selective regression of desmoid tumors with antiestrogen therapy, management of symptomatic pelvic desmoid tumors in young women may necessitate an operation. In our series, all primary and recurrent tumors were managed with resection of involved bone and other adjacent soft tissues. The role of adjunctive radiotherapy or hormonal therapy remains to be defined
Case Report
Sporadic, Nontrauma-Related, Desmoid Tumor of the Pancreas: A Rare Disease—Case Report and Literature Review
F. Polistina,1G. Costantin,2E. D'Amore,3 and G.
Volume 2010 (2010), Article ID 272760, 4 pages
doi:10.1155/2010/272760
sporadic pancreatic DT is an extremely rare finding in common clinical practice. There are no symptoms, signs, or imaging features to aid in diagnosis. Fine-needle aspiration should be considered for incidentally discovered small lesions. However, according to current guidelines, surgery must be performed if there is any doubt as to diagnosis. Follow-up is also necessary, regardless of the low-rate of tumor recurrence.
The association between Crohn's disease and desmoid tumors: a novel case and review of the literature.
Bungay AW, Smith AJ, Hsieh E, Saibil FG.
Abstract
Desmoids are rare tumors resulting from the proliferation of fibroblasts. They occur in association with familial adenomatous polyposis (FAP), but they may also occur in the post-traumatic peri-partum or post-abdominal surgery setting, and a few present spontaneously. Presenting features of desmoids are protean and largely relate to the anatomical area of involvement. We describe a 50 year old male not known to have Crohn's disease and without FAP who presented with multiple desmoids. Investigation of post-operative diarrhea confirmed a diagnosis of Crohn's disease. This is the first report of a male patient, who had never undergone prior abdominal surgery, presenting with Crohn's disease and abdominal desmoid tumors. The reasons why Crohn's disease and desmoids may be associated are explored, focusing particularly on alternations in the fibrogenic cytokine TGF-β now known to be involved in the pathogenesis of both diseases
.
The surgical management of soft tissue tumours arising in the abdominal wall.
Pencavel T, Strauss DC, Thomas JM, Hayes AJ.
2010 May;36(5):489-95. Epub 2010 Apr 9
Tumours involving the abdominal wall exhibit a wide range of pathologies. Abdominal wall reconstruction can be achieved in the vast majority of cases with mesh reconstruction alone with little surgical morbidity. Sarcomas carry a significant risk of local recurrence. Abdominal wall fibromatosis carries a better prognosis than fibromatosis arising in the extremities.
Unusual presentation of desmoid tumor in the small intestine: a case report.
Chang CW, Wang TE, Chang WH, Yang TL, Chen CK, Hung YC, Shih SC
2011 Mar;28(1):159-62. Epub 2010 Jan 27
Abstract
Desmoid tumor originating from the small intestine is extremely rare. We report a 50-year-old man who presented with the sudden onset of severe abdominal pain. Computerized tomography (CT) demonstrated a huge homogeneous tumor in the lower abdomen that appeared to be in continuity with the distal ileum. The mass adherent to the ileum was resected and proved to be a desmoid tumor. The patient has remained recurrence free on follow-up.
Unusual presentation of an uncommon abdominal pathology.
Jain P, Shah P, Bhansali M.2010 Oct;92(7):W19-21. Epub 2010 Sep 7.Department of General Surgery, Jaslok Hospital and Research Centre, Mumbai, India
Abstract
Desmoid tumour is a non-encapsulated locally invasive tumour, originating from fibroblasts, which has ubiquitous distribution in the body. It has a high tendency for local recurrence, causing deformity in the adjacent organ and consequent organ dysfunction. A case of a 75-year-old man, presenting with high-grade fever, dull aching abdominal pain and weight loss, suspected to be due to malignancy, is reported. Contrast-enhanced computed tomography (CECT) showed a mass in the transverse colon with pericolic collection raising a suspicion of perforation. He underwent a two-stage procedure in the form of defunctioning ileostomy in the initial setting followed by colonic resection and anastomosis 6 weeks later. Histological analysis revealed mesenteric fibromatosis (desmoid tumour). The patient was completely asymptomatic one year after surgery. We report this case, as our patient had none of the predisposing factors. To the best of our knowledge, this is the first case on colonic perforation secondary to desmoid tumour.
Mesenteric Fibromatosis Causing Ureteral Stenosis
Jae Young Choi, Kyung Mo Kang, Bum Soo Kim, and Tae-Hwan Kim
Korean J Urol. 2010 July; 51(7): 501–504.
Mesenteric fibromatosis is a rare benign disease characterized by proliferating fibrous issue in the bowel mesentery. We report a case of aggressive mesenteric fibromatosis with ureteral stenosis arising in a 46-year-old woman who suffered from intermittent right abdominal pain. Computed tomography revealed a right retroperitoneal mass with right ureteral stenosis at the level of the right common iliac vessel. The mass was excised with resection of the affected segment of the ileum, ascending colon, and ureter, and end-to-end ureter anastomosis was performed. Pathological examination confirmed mesenteric fibromatosis.
A case of desmoid tumor presenting as intra-abdominal abscess
Yu YH, Son BK, Jun DW, Kim SH, Jo YJ, Park YS, Hong BW, Joo JE.
2009 May;53(5):315-9.
Abstract
Desmoid tumor is a rare benign tumor derived from fibrous sheath or musculoaponeurotic structure. The tumor is benign histologically but considered as malignant clinically because it has high propensity on infiltrative growth with local invasion and tendency to recurrence after local excision. Especially, when this tumor happens to be in the intra-abdomen, the prognosis is worse because it can cause intestinal obstruction, ureter obstruction and, fistula formation. It also can invade major vessels in abdomen. This tumor occurs more frequently in patients with familial adenomatous polyposis (FAP), in post-partume women, and at old surgical incision site. However, in this case, the patient had neither previous surgery nor a FAP history. We report a rare case of the young male patient who presented with an acute abdomen and underwent laparotomy and was found to have an intra-abdominal desmoid tumor with abscess formation
.
Desmoid tumors of the anterior abdominal wall: results from a monocentric surgical experience and review of the literature.
Bertani E, Chiappa A, Testori A, Mazzarol G, Biffi R, Martella S, Pace U, Soteldo J, Vigna PD, Lembo R, Andreoni B.
2009 Jun;16(6):1642-9. Epub 2009 Mar 19
Radical resection aided by intraoperative margin evaluation via frozen sections followed by immediate mesh reconstruction is a safe procedure and can provide definitive cure without functional limitations for patients with desmoid tumors of the anterior abdominal wall.
Intraoperative electron radiotherapy for the management of aggressive fibromatosis.
Roeder F, Timke C, Oertel S, Hensley FW, Bischof M, Muenter MW, Weitz J, Buchler MW, Lehner B, Debus J, Krempien R.
2010 Mar 15;76(4):1154-60. Epub 2009 Aug 3.
Introduction of IOERT into a multimodal treatment approach in patients with aggressive fibromatosis is feasible with low toxicity and yielded good local control rates even in patients with microscopical or gross residual disease
Radiation therapy in the treatment of desmoid tumours reduces surgical indications.
Rüdiger HA, Ngan SY, Ng M, Powell GJ, Choong PF
2010 Jan;36(1):84-8. Epub 2009 Aug 13.
While several modalities have been proposed for the treatment of desmoid tumour/aggressive fibromatosis, high local recurrence rates have been reported. We present a retrospective study of including patients treated with radiation therapy, some of them in combination with surgical resection .Low recurrence rates can be achieved with the use of radiation therapy alone in selected cases. Patients with a metabolic response (decrease) to radiotherapy may be treated with a non-surgical approach. Surgery might be considered in patients with a poor metabolic response to radiotherapy.
Sporadic retroperitoneal aggressive fibromatosis: report of a case.
Ghidirim G, Mishin I, Gagauz I, Vozian M, Zastavnitsky G, Iakovleva
2010 Feb;135(1):79-82. Epub 2009 May 7.
AbstractWe describe herein a case of sporadic retroperitoneal aggressive fibromatosis. A 54-year-old man with a palpable abdominal mass was referred to our hospital for investigation and treatment. Abdominal ultrasonography and computed tomography revealed a solid mass with relatively well-defined borders in the left abdominal retroperitoneum. At surgery, a large tumor (14 x 13 x 11 cm) was found, arising from the retroperitoneal space and involving the wall of jejunum. Complete removal was achieved. Histological examination of the resected specimen revealed the presence of changes consistent with aggressive fibromatosis. The patient was not scheduled for any further treatment. Two years after surgery, the patient is without any signs of recurrent disease. Although extremely rare, aggressive fibromatosis should be considered in the differential diagnosis of retroperitoneal masses. Complete surgical resection with negative pathological margins remains the first line management of theseneoplasms.Careful follow-up is indicated because recurrence may occur every time after surgery.
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