Aggressive fibromatosis of the neck in a patient with Gardner's syndrome

T., A.; Nguyen, Thien; Hogg, Jeffery P.; Gabriele, Francesca

doi:10.1007/s002340000530

Cited by 8 publications

(10 citation statements)

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“…Regarding potential etiologic factors, the history in 3% to 33% of patients with head and neck desmoid tumors reveals previous trauma or surgery at the site of the tumor, whereas, occasionally, a desmoid tumor develops in the neck or larynx during pregnancy . A head and neck desmoid tumor typically occurs sporadically, but such a tumor has also been observed in a patient with Gardner syndrome …”

Section: Introductionmentioning

confidence: 99%

Desmoid tumors of the head and neck: A therapeutic challenge

et al. 2014

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Desmoid tumor, or aggressive fibromatosis, is a rare, histologically benign, fibroblastic lesion that infrequently presents in the head and neck. Desmoid tumors often grow locally, invasively, and may, in rare instances, be fatal secondary to invasion into critical structures, such as airway or major vessels. The most common treatment is surgery, but desmoid tumors are characteristically associated with a high local recurrence rate after resection. Although the margin status seems to be of importance, operations that avoid function loss and esthetic disfigurement should be the primary goal. The efficacy of postoperative radiotherapy is controversial. Its potential benefit should be carefully balanced against possible radiation-induced adverse effects. Alternative treatment modalities, such as primary radiotherapy and medical treatment or a wait-and-see policy, may be preferable to mutilating surgery. Considering all the aforementioned, it seems obvious that desmoid tumors of the head and neck present a therapeutic challenge and require an individualized approach.

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Section: Introductionmentioning

confidence: 99%

Desmoid tumors of the head and neck: A therapeutic challenge

et al. 2014

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“…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%

Intracranial Desmoid Tumor with Familial Adenomatous Polyposis Coli

et al. 2008

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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.

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“…Gardner syndrome is a rare autosomal dominant disorder characterized by colonic polyposis and bone and soft tissue manifestations including osteomas, and mesenchymal tumors of the skin and soft tissues (1)(2)(3). To our knowledge, unilateral chest wall anomaly has not been reported in patients with Gardner syndrome.…”

Section: Introductionmentioning

confidence: 99%

“…In general, the cutaneous and bone abnormalities develop approximately 10 years prior to polyposis (4, 6). The gastrointestinal manifestations of Gardner syndrome include extensive adenomatous polyps in the colon, adenomatous polyps in the stomach and small intestine and periampullary carcinomas(1,2,4,7). The colon is the most common site of involvement but other parts of the gastrointestinal tract may be involved(1).…”

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confidence: 99%