Staging Intra-Abdominal Desmoid Tumors in Familial Adenomatous Polyposis: A Search for a Uniform Approach to a Troubling Disease

Church, James M.; Berk, Terri; Boman, Bruce M.; Guillem, José G.; Lynch, Craig; Lynch, Patrick M.; Rodrı́guez-Bigas, Miguel A.; Русин, Л П.; Weber, Tom

doi:10.1007/s10350-005-0018-8

Cited by 85 publications

(54 citation statements)

References 36 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Besides these manifestations, thyroid cancers and central nervous system tumors were also reported in FAP [11]. Although associations of FAP and DT involving mesentery, retroperitoneum, abdominal wall, liver, pelvis, lumbar region, latissimus dorsi muscle, and neck were defined [7,12,13,14,15,16,17,18], only 1 pediatric case of DT with FAP had been reported previously [6]. Cytogenetic analysis of that case demonstrated loss of chromosome region 5 (q21q22) [6], but we did not have the chance to examine our patient and family for this highly probable genetic abnormality.…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…Among patients with FAP, 11–15% of them develop DTs [7]. An inactivating germline mutation in the tumor suppressor gene APC is a dominantly inherited genetic abnormality in these cases [7,8,9]. When FAP is associated with DTs, mesenteric fibromatosis, dental abnormalities, gastric polyps, duodenal polyps, lymphoid hyperplasia of the terminal ileum, and ileal adenomas, it is called Gardner’s syndrome which was first described in 1951 [10].…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Intracranial Desmoid Tumor with Familial Adenomatous Polyposis Coli

et al. 2008

View full text Add to dashboard Cite

Intracranial desmoid tumors are extremely rare. The association of desmoid tumors with familial adenomatous polyposis coli was reported previously, with the tumors involving trunk and extremities. We report a 3.5-year-old girl with intracranial desmoid tumor with familial adenomatous polyposis coli. This condition in a child is rarely reported. Follow-up of the patient after cranial surgery and of the family for this premalignant inherited condition is necessary.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Intracranial Desmoid Tumor with Familial Adenomatous Polyposis Coli

et al. 2008

View full text Add to dashboard Cite

show abstract

“…An attempt has been done by the Collaborative Group of the Americas on Inherited Colorectal Cancer (CGA-ICC) in 2005 for the management of patients with IA DT. 31 Additional variables may interact with outcome, such as FAP, pregnancy associated, or b-catenin status. Larger multi-institutional experience must be gathered in the future for patients with DT; these would be able to provide a more accurate assessment of different sites and clinicopathologic features as it pertains to outcome.…”

mentioning

confidence: 99%

Abdominal Desmoid Tumors: Hands Off?

2016

View full text Add to dashboard Cite

The reported incidence of desmoid tumors (DT) seems to increase from about two cases per million people in 1993 to about five cases per million people in 2013. 1 Because it lacks the ability to metastasize, a desmoid tumor is classified as a benign disorder. Nevertheless, the sequelae of this disease and the applied treatments may be underlined by the fact that patients with familial adenomatous polyposis (FAP) may die from the consequences of DT. Moreover, substantial morbidity due to invasive growth is frequently seen. Because of the poor understanding of the natural history of the disease, its tumor biology, and the lack of randomized studies comparing different treatment options, the recommended treatment options vary widely depending on tumor aggressiveness, location, patient wish, and preferences of the treating physicians.With respect to the existing treatment options, surgery has been the cornerstone in treating DT. The reported recurrence rate after resection varies greatly, from 5 to 63 %, with most studies reporting recurrence rates of approximately 20 %.2-9 The prognostic value of several characteristics has been investigated. The reported results about the importance of age, location, resection margins, and adjuvant radiotherapy are conflicting. 4,7,[10][11][12] Also, radiotherapy has been widely applied for DT, both in primary and adjuvant settings. Results have been ambiguous, especially for adjuvant radiotherapy. 4,8,11,13 A study by Keus et al. addressed the role of radiotherapy in a primary setting. They reported partial and complete response in 50 % of patients and durable stable disease in 41 % of patients, with a local control rate of 81 % at 3 years. 14 Literature on systemic treatment for DT is heterogeneous and limited. Antihormonal drugs, nonsteroid antiinflammatory drugs, chemotherapy, and tyrosine kinase inhibitors have all been applied using different regimens. Most studies consist of relatively small cohorts, rendering it difficult to put results into perspective. Prospective trials are currently running with sorafenib (NCT 02066181), pazopanib (NCT01876082), sirolimus (NCT 01265030), and PF-03084014 (NCT01981551), and their results are eagerly awaited. Isolated limb perfusion is reserved for patients with advanced disease without the possibility of limb preservation in case of surgery. This procedure is only performed in specialized centers. Three European Organization for Research and Treatment of Cancer sarcoma centers reported good results, with limb preservation rates up to 88 %. 15 Key questions in managing DT are when and how to treat. Importantly, expected benefits from therapy should be well balanced against potential treatment-induced untoward effects.Recent years have seen a growing interest in a wait-andsee approach for patients presenting with DT, an approach prompted by observations of spontaneous regressions and durable disease stabilization. 3,4,16 The study by Burtenshaw et al. published in this issue of Annals of Surgical Oncology substantially adds to our knowledge...

show abstract

“…Thesetumorsmayarisefromtheextremities,abdominalwall, intestinalwallandmesentery,headandneck,andbreastand canbesporadicorassociatedwithfamilialadenomatouspolyposis [4][5][6][7][8][9]. Despite being histologically benign tumors, desmoids are locally aggressive and can cause debilitating symptomsanddeath [10].Ithasbeenreportedthataggressive measurestosurgicallyextirpatedesmoidtumorscanresultin significant morbidity and mortality, which has resulted in changesinmanagementoftheselesions [4].Alternativessuch asobservation,radiation,andchemo/hormonaltherapyhave been explored, but with unproven durable success [11,12].…”

Section: Disclosure Statementmentioning

confidence: 99%