Cowden syndrome surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vamsikrishna Gunnam M.B.B.S [2]

Overview

Surgery is not the first-line treatment option for patients with cowden syndrome. Surgery is usually reserved for patients with gastrointestinal polyps, Dysplastic Gangliocytoma and facial papules.

Surgery is not the first-line treatment option for patients with cowden syndrome. Surgery is usually reserved for patients with the following:

Dysplastic Gangliocytoma:
- Keeping in mind about the potential effects of hydrocephalus like fatal herniation, resection of dysplastic gangliocytoma of the cerebellum should be considered.^[1]^[2]
Gastrointestinal polyps:
- Patients with cowden syndrome should consider resection of gastrointestinal polyps endoscopically which includes adenomatous polyps which are risk for developing colon cancer.^[3]^[4]
- Endoscopic techniques like narrow band imaging should be considered for removing large polyps (≥1 cm).^[5]

↑ Robinson, Shenandoah; Cohen, Alan R. (2006). "Cowden disease and Lhermitte–Duclos disease: an update". Neurosurgical Focus. 20 (1): 1–6. doi:10.3171/foc.2006.20.1.7. ISSN 1092-0684.
↑ Shinagare, Atul B.; Patil, Nirupama K.; Sorte, S. Z. (2009). "Case 144: Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease)". Radiology. 251 (1): 298–303. doi:10.1148/radiol.2511071390. ISSN 0033-8419.
↑ Levi Z, Baris HN, Kedar I, Niv Y, Geller A, Gal E, Gingold R, Morgenstern S, Baruch Y, Leach BH, Bronner MP, Eng C (November 2011). "Upper and Lower Gastrointestinal Findings in PTEN Mutation-Positive Cowden Syndrome Patients Participating in an Active Surveillance Program". Clin Transl Gastroenterol. 2: e5. doi:10.1038/ctg.2011.4. PMC 3365666. PMID 23238744.
↑ Heald B, Mester J, Rybicki L, Orloff MS, Burke CA, Eng C (December 2010). "Frequent gastrointestinal polyps and colorectal adenocarcinomas in a prospective series of PTEN mutation carriers". Gastroenterology. 139 (6): 1927–33. doi:10.1053/j.gastro.2010.06.061. PMC 3652614. PMID 20600018.
↑ Howe JR, Mitros FA, Summers RW (December 1998). "The risk of gastrointestinal carcinoma in familial juvenile polyposis". Ann. Surg. Oncol. 5 (8): 751–6. PMID 9869523.