Transcatheter embolization of arteriovenous malformations in Cowden disease.

A patient with Cowden disease and multiple arteriovenous malformations (AVMs) that resulted in high output heart failure is described. Cowden disease is a familial syndrome characterized by endodermal, mesodermal and ectodermal dysplasia causing benign and malignant tumors of the skin, breast, gastrointestinal tract, and thyroid gland. Our patient had gastrointestinal polyposis, a right renal tumor, a left lung tumor, an adenomatous goiter, and typical dermatologic findings such as facial papules, acral keratosis, gingival papillomatosis and hemangiomas. AVMs were observed in the pelvis, cervical vertebra, liver, and right supraclavicular area. Transcatheter embolization was performed 7 times for the pelvic AVMs, but the effect decreased with repetition and the patient died of heart failure 2 years after the first embolization. The serum levels of tissue plasminogen activator (t-PA), platelet-derived growth factor (PDGF), hepatocyte growth factor (HGF), vascular endothelial growth factor (VEGF), and transforming growth factor beta1 were high, suggesting that these angiogenic molecules may play a role in the pathogenesis of AVMs in Cowden disease.

[1]  E. Battegay,et al.  Platelet-Derived Growth Factor and Angiogenesis , 1996 .

[2]  E. Mariman,et al.  Localization of the gene for Cowden disease to chromosome 10q22–23 , 1996, Nature Genetics.

[3]  M. Hallisey Vascular Diseases: Surgical and Interventional Therapy , 1994 .

[4]  N. Hayashi,et al.  A CASE OF COWDEN'S DISEASE WITH PELVIC ARTERIOVENOUS MALFORMATIONS , 1994 .

[5]  J. Fryns,et al.  Cowden syndrome: report of a large family with macrocephaly and increased severity of signs in subsequent generations , 1993, Clinical genetics.

[6]  R. Spetzler,et al.  Cowden syndrome and lhermitte‐duclos disease , 1992, Cancer.

[7]  R. R. Murray,et al.  Congenital arteriovenous malformations: tailored embolotherapy. , 1988, Radiology.

[8]  F. Arwert,et al.  The Cowden syndrome: a clinical and genetic study in 21 patients , 1986, Clinical genetics.

[9]  Schwartz Mz,et al.  Management of congenital arteriovenous malformations. , 1983 .

[10]  H. Aram,et al.  Multiple hamartoma syndrome (Cowden's disease) , 1983, Journal of the American Academy of Dermatology.

[11]  A. Gomes,et al.  Congenital Arteriovenous Malformations: The Role of Transcatheter Arterial Embolization , 1983 .

[12]  W. Steck,et al.  Cowden's disease (multiple hamartoma and neoplasia syndrome). A case report and review of the English literature. , 1983, Journal of the American Academy of Dermatology.

[13]  M. Flye,et al.  Management of congenital arteriovenous malformations. , 1983, Surgery.

[14]  T. H. Newton,et al.  Particulate intraarterial embolization in pelvic arteriovenous malformations. , 1981, AJR. American journal of roentgenology.

[15]  T. Ishikawa Congenital Arteriovenous Malformations Involving the Pelvis and Retroperitoneum: A Case Report , 1979, Angiology.

[16]  Bernatz Pe,et al.  Surgical treatment of congenital pelvic arteriovenous malformation. , 1978 .

[17]  P. E. Bernatz,et al.  Surgical treatment of congenital pelvic arteriovenous malformation. , 1978, Mayo Clinic proceedings.

[18]  P. Weary,et al.  Multiple hamartoma syndrome (Cowden's disease). , 1972, Archives of dermatology.

[19]  Bernatz Pe,et al.  Arteriovenous fistulas: a review and ten-year experience at the Mayo Clinic. , 1970 .

[20]  K. Lloyd,et al.  Cowden's disease. A possible new symptom complex with multiple system involvement. , 1963 .