Gardner's syndrome, colon cancer, and sarcoma

Johnson, Jerome G.; Gilbert, Enid F.; Zimmermann, Bernard; Watne, Alvin L.

doi:10.1002/jso.2930040406

Cited by 16 publications

(6 citation statements)

References 8 publications

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“…Another very important observation by Jarnum and Jensen was that histologically, the polyps only showed cystic dilatation of glands with no areas of adenomatous epithelium67. In 1972, Johnson reported that the stomach and large bowel are not carpeted with adenomatous polyps as originally described, but with hamartomatous polyps75, which confirmed the original description of Jarnum and Jensen in 1966. All the above clinical characteristics have been described by multiple authors, as well as the fact that this disease spares the esophagus15,30,103.…”

Section: Cronkhite-canada Syndrome (Ccs)supporting

confidence: 66%

Hamartomatous Polyposis Syndromes

Calva

Howe

2008

Surgical Clinics of North America

127

116

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SynopsisSince the histological description of the hamartomatous polyp in 1957 by Horrilleno et al., several different syndromes have been described with the propensity to develop these polyps in the upper and lower GI tracts. These include Juvenile Polyposis, Peutz-Jeghers syndrome, hereditary mixed polyposis syndrome, and the PTEN hamartoma tumor syndromes (Cowden and Bannayan-RileyRuvalcaba syndromes), which are autosomal-dominantly inherited, and Cronkhite-Canada syndrome, which is acquired. The clinical aspects, the molecular pathogenesis, the organ systems affected, the risks of cancer, and the management of these hamartomatous polyposis syndromes will be reviewed in this paper. Although the incidence of these syndromes is low, it is important for clinicians to recognize these disorders in order to prevent morbidity and mortality in these patients, and to perform presymptomatic testing in patients at risk.

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Section: Cronkhite-canada Syndrome (Ccs)supporting

confidence: 66%

Hamartomatous Polyposis Syndromes

Calva

Howe

2008

Surgical Clinics of North America

127

116

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“…In 1972, Johnson et al [3] published that the polyps in the stomach and large intestine are hamartomas and confirmed the description of Jarnum and Jensen.…”

Section: Historymentioning

confidence: 65%

Cronkhite-Canada Syndrome: Review of the Literature

Kopáčová

Urban

Cyrany

et al. 2013

Gastroenterology Research and Practice

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Cronkhite-Canada syndrome is a rare disease characterised by diffuse polyposis of the gastrointestinal tract, diarrhoea, weight loss, abdominal pain, cutaneous hyperpigmentation, dystrophic changes of fingernails, and alopecia. The etiology is probably autoimmune and diagnosis is based on history, physical examination, endoscopic findings of gastrointestinal polyposis, and histology. The disease is very rare; about 450 cases have been described in the literature so far. We present a review of the literature with our own picture documentation of this rare condition.

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“…110 Trauma, as from surgery, may worsen DTF, and DTF has a high risk of local recurrence after surgery ranging from about 25% to 60% at 5 years. 19,31,59,94,[111][112][113][114][115][116][117][118][119][120] Inflammation from other types of trauma also may augment or stimulate recurrence; however, DTF does not metastasize. Although a marginal resection is associated with a worse outcome than a complete resection, the nature of the surgical procedure is strongly influenced by tumor location and associated anatomical and functional consequences.…”

Section: Evidence For Clinical Treatments Of Dtfmentioning

confidence: 99%

Biology and Treatment of Aggressive Fibromatosis or Desmoid Tumor

Skubitz

2017

Mayo Clinic Proceedings

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Aggressive fibromatosis, also known as desmoid-type fibromatosis (DTF) or desmoid tumor, is an uncommon locally invasive tumor. Because of its low incidence and variable behavior, DTF is often first seen by physicians who are not familiar with it, and recent advances in understanding this disease have led to changes in treatment approaches. The Wnt (β-catenin) pathway appears to play a key role in DTF pathogenesis, and recent studies of DTF biology suggest a possible model of DTF pathogenesis. Histologically, DTF shows a poorly circumscribed proliferation of myofibroblast-like cells with variable collagen deposition, similar to the proliferative phase of wound healing, and DTF has been associated with trauma and pregnancy. Desmoid-type fibromatosis may be a useful model of the tumor stroma in carcinomas as well as other fibrosing diseases such as progressive pulmonary fibrosis. The clinical course of DTF can vary greatly among patients, complicating the determination of the optimal treatment approach. Treatment options include surgery, nonsteroidal anti-inflammatory drugs with or without hormonal manipulation, chemotherapy, radiation therapy, and other forms of local therapy. Many treatments have been used, but these are not without toxicities. Because of the variable nature of the disease and the potential morbidity of treatment, some cases of DTF may do better without treatment; simple observation is often the best initial treatment. This review used a PubMed search from January 1, 1980, through October 31, 2016, using the terms fibromatosis and desmoid and discusses DTF disease characteristics, pathophysiology, and treatment options as well as examines several cases illustrating key points in the biology and treatment of this heterogeneous disease.

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Gardner's syndrome, colon cancer, and sarcoma

Cited by 16 publications

References 8 publications

Hamartomatous Polyposis Syndromes

Hamartomatous Polyposis Syndromes

Cronkhite-Canada Syndrome: Review of the Literature

Biology and Treatment of Aggressive Fibromatosis or Desmoid Tumor

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