Dural AVM (dAVM) in Cowden disease

Anthony C. Torres, Parth Upadhyaya, Rajneesh Agrawal

Research output: Contribution to journalArticlepeer-review

1 Scopus citations


Cowden disease is a rare autosomal dominant disorder first described by Rachel Cowden in 1963 as one of the phosphatase and tensin homolog (PTEN) gene hamartoma tumor syndromes. Commonly affecting multiple systems, patients typically have an increased risk of breast and thyroid cancers as well as macrocephaly and benign hamartomatous growths. Due to the benign nature of many of the symptoms, true prevalence is likely higher than reported rates. Malformations associated with PTEN gene lead to dysregulation of cell proliferation yielding increased risk of neoplasm and frequently benign growths. Associated dysregulation of angiogenesis may result in arteriovenous malformations (AVMs). Intracranial AVMs may be asymptomatic. These AVMs present a risk of rupture and subsequent morbidity and mortality. Those with Cowden disease must be monitored for development of intracranial AVMs. Patients should be made aware that treatment for intracranial AVMs may be invasive and include serial embolization.

Original languageEnglish (US)
Pages (from-to)155-158
Number of pages4
JournalInterdisciplinary Neurosurgery: Advanced Techniques and Case Management
StatePublished - Dec 2017
Externally publishedYes

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology


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