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Cowden disease: CT findings in three patients

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Cowden disease, also known as multiple hamartoma syndromes, is an autosomal dominant disease characterized by numerous benign mucocutaneous tumors, hamartomas of multiple organs, and malignancies of the breast and thyroid. In this report, we present the computed tomographic findings in three patients with Cowden disease. In addition to the classic findings, the patients were diagnosed with spinal neurinoma (n = 1), meningioma (n = 1), and hepatic hemangioma (n = 3). We also review current clinical and genetic concepts that unify Cowden disease.

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  1. H Hauser B Ody O Plojoux et al. (1980) ArticleTitleRadiological findings in multiple hamartoma syndrome (Cowden’s disease). A report of three cases Radiology 137 317–323

    Google Scholar 

  2. G Marra F Armelao FM Vecchio et al. (1994) ArticleTitleCowden’s disease with extensive gastrointestinal polyposis J Clin Gastroenterol 18 42–47

    Google Scholar 

  3. M Sasaki H Hakozaki T Ishihara (1993) ArticleTitleCowden’s disease with pulmonary hamartoma Intern Med. 32 39–41

    Google Scholar 

  4. KM Lloyd M Dennis (1963) ArticleTitleCowden’s disease: a possible new syndrome with multiple organ involvement Ann Intern Med. 58 136–142

    Google Scholar 

  5. J Burnett R Goldner G Calton (1975) ArticleTitleCowden’s disease Br J Dermatol. 93 329–336

    Google Scholar 

  6. OS Salem WD Steck (1983) ArticleTitleCowden’s disease (multiple hamartoma and neoplasia syndrome). A case report and review of the English literature J Am Acad Dermatol. 8 686–696

    Google Scholar 

  7. TM Starink (1984) ArticleTitleCowden’s disease: analysis of fourteen new cases J Am Acad Dermatol. 11 1127–1141

    Google Scholar 

  8. Cho KC, Sundaram K, Sebastiano LS (1999) Filiform polyposis of the small bowel in a patient with multiple hamartoma syndrome (Cowden disease). AJR pp 501-502

  9. K Hizawa M Iida T Matsumoto et al. (1994) ArticleTitleGastrointestinal manifestations of Cowden’s disease J Clin Gastroenterol 18 13–18

    Google Scholar 

  10. YM Chen DJ Ott WC Wu et al. (1987) ArticleTitleCowden’s disease: a case report and literature review Gastrointest Radiol. 12 325–329

    Google Scholar 

  11. JC Box AL Watne (1995) ArticleTitleInherited syndromes of colon polyps Semin Surg Oncol. 11 394–398

    Google Scholar 

  12. S Schweitzer JP Hogge M Grimes et al. (1999) ArticleTitleCowden disease: a cutaneous marker for increased risk of breast cancer AJR 172 349–351

    Google Scholar 

  13. ED Lynch EA Ostermeyer MK Lee et al. (1997) ArticleTitleInherited mutations in PTEN that are associated with breast cancer, Cowden disease, and juvenile polyposis Am J Hum Genet. 61 1254–1260 Occurrence Handle10.1086/301639

    Article  Google Scholar 

  14. M Kato A Mizuki T Hayashi et al. (2000) ArticleTitleCowden’s disease diagnosed through mucocutaneous lesions and gastrointestinal polyposis with recurrent hematochezia, unrevealed by initial diagnosis Intern Med. 39 559–563

    Google Scholar 

  15. R Koch M Scholz MR Nelen et al. (1999) ArticleTitleLhermitte-Duclos disease as a component of Cowden’s syndrome J Neurosurg. 90 776–779

    Google Scholar 

  16. N Takaya A Maehara S Nishiyama et al. (1999) ArticleTitleTranscatheter embolisation of arteriovenous malformations in Cowden disease Jpn Circ J. 63 326–329 Occurrence Handle10.1253/jcj.63.326

    Article  Google Scholar 

  17. D Liaw DJ Marsk J Li et al. (1997) ArticleTitleGermline mutations of the PTEN gene in Cowden disease, an inherited breast and thyroid cancer syndrome Nat Genet. 16 64–67 Occurrence Handle10.1038/ng0597-64

    Article  Google Scholar 

  18. DJ Marsh V Coulon KL Lunetta et al. (1998) ArticleTitleMutation spectrum and genotype analyses in Cowden disease and Bannayan-Zonana syndrome, two hamartoma syndromes with germline PTEN mutation Hum Mol Genet. 7 507–515 Occurrence Handle10.1093/hmg/7.3.507

    Article  Google Scholar 

  19. C Eng H Ji (1998) ArticleTitleMolecular classification of the inherited hamartoma polyposis syndromes: clearing the muddied water Am J Hum Genet. 62 1020–1022 Occurrence Handle10.1086/301847

    Article  Google Scholar 

  20. HT Lynch A la Chapelle Particlede (2003) ArticleTitleHereditary colorectal cancer N Engl J Med. 348 919–932 Occurrence Handle10.1056/NEJMra012242

    Article  Google Scholar 

  21. T Suzuki M Ichinose Y Matsubara et al. (1997) ArticleTitleCowden’s disease with a defined genetic alteration—chromosomal duplication at 15q11-q13 J Gastroenterol 32 696–699

    Google Scholar 

  22. CJ Lyons CB Wilson JC Horton (1993) ArticleTitleAssociation between meningioma and Cowden’s disease Neurology 43 1436–1437

    Google Scholar 

  23. H Haibach TW Burns HE Carlson et al. (1992) ArticleTitleMultiple hamartoma syndrome (Cowden’s disease) associated with renal cell carcinoma and primary neuroendocrine carcinoma of the skin (Merkel cell carcinoma) Am J Clin Pathol. 88 705–712

    Google Scholar 

  24. MM Entius AM Westerman AMF Velthuysen et al. (1999) ArticleTitleMolecular and phenotypic markers of hamartomatous polyposis syndromes in the gastrointestinal tract Hepatogastroenterology 46 661–666

    Google Scholar 

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Correspondence to J. Rademaker.

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Rademaker, J., Kim, Y., Leibecke, T. et al. Cowden disease: CT findings in three patients. Abdom Imaging 30, 204–207 (2005).

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