Cowden syndrome is an autosomal dominant disorder characterized by hamartomas, as
well as benign and malignant neoplasms that may present in organ systems throughout
the body. It also poses an increased lifetime cancer risk in those with the disorder.
Its clinical presentation is often variable, and diagnosis can be challenging. In
the head and neck region, it can present as thyroid enlargement or mass formation,
cutaneous and mucocutaneous lesions of the skin and the oral cavity. The most typical
oral manifestations of Cowden syndrome are oral papillomatosis and a cobblestone appearance
of the mucosa. We present a case of vascular malformation of the tongue in a patient
with Cowden syndrome. This lesion was similar in appearance to a cutaneous hamartoma
on the patient's upper extremity. He had received prior surgical intervention for
this tongue mass, and complete resection was recommended subsequently. However, in
search of a less invasive treatment to minimize impact on speech and oral function,
sclerotherapy was performed, resulting in resolution of the lesion. Sclerotherapy
is a well-documented treatment for head and neck vascular malformations, but it is
not universally employed. In our patient with atypical oral manifestation of Cowden
syndrome, bleomycin sclerotherapy was employed, resulting in resolution of the lesion,
as well as preservation of speech articulation and oral function.
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Article info
Publication history
Published online: July 04, 2020
Accepted:
May 31,
2020
Received in revised form:
May 13,
2020
Received:
March 17,
2020
Identification
Copyright
© 2020 Elsevier Inc. All rights reserved.
