cronkhite- canada syndrome (ccs) considered as a rare and non-hereditary disorder. gastrointestinal polyposis and diarrhea along with some extra signs and symptoms such as hypoproteinemia, and epidermal manifestations are recognized in this syndrome. the pathophysiology of this syndrome is not completely understood and it seems that inflammatory processes may be involved. we present a 50 year-o...

to report a case of buphthalmos with neurofibromatosis (nf-1) who suffered with blindness suffered enucleation. physical examination & histopathology were performed. physical examination revealed buphthalmos & vascularized cornea with lower lid ectropion and orbital deformity. histopathologic findings shows plexiform neurofibroma in orbital tissue with scleral wall involvement. and hamartomatou...

Since 2003, nine squirrels were presented at a South African veterinary dental practice with lesions in the maxilla consistent with the clinical, radiological and histological features of odontomas as described in prairie dogs, rats and mice. These odontoma-like masses have not previously been described in squirrels. This report describes the clinico-pathological features and possible pathogene...

Peutz-Jegher syndrome (PJS) is a rare, autosomal-dominant disorder characterized by hamartomatous polyps in any part of the alimentary tract, and almost always associated with intestinal polyposis and mucocutaneous pigmentation (1). Rarely, solitary PJP arise inpatients without other features of PJS. A review of the English literature revealed only 6 published cases since 1989. Here, we would l...

Cowden syndrome is a rare autosomal dominant familial cancer syndrome with a high risk of breast cancer. The most important clinical features include carcinomas of the breast and thyroid, and hamartomatous polyps of the gastrointestinal tract. There are characteristic mucocutaneous features which allow early recognition of the disease and are generally present before internal malignancies devel...

Cronkhite- Canada syndrome (CCS) considered as a rare and non-hereditary disorder. Gastrointestinal polyposis and diarrhea along with some extra signs and symptoms such as hypoproteinemia, and epidermal manifestations are recognized in this syndrome. The pathophysiology of this syndrome is not completely understood and it seems that inflammatory processes may be involved. We present a 50 year-o...

Cavernous hemangiomas of the orbit are benign, hamartomatous, vascular lesions, more frequent in middle-aged women, representing the most common benign primitive neoplasm of the orbit. Several therapeutic modalities and surgical approaches have been described, in order to preserve the normal orbital structures. We describe the case of a patient with a volumous orbital cavernoma, approached by a...

Capillary hemangiomas, hamartomatous proliferation of vascular endothelial cells, are rare in the central nervous system (CNS). Intracranial capillary hemangiomas presenting with reversible behavioral abnormalities and focal neurological deficits have rarely been reported. We report a case of CNS capillary hemangioma presenting with transient focal neurological deficits and behavioral abnormali...

Granular cell tumor is an uncommon benign hamartomatous lesion occurring in almost any part of the body. Granular cell lesions may be found in other diverse sites, such as the jaw, skin, gastrointestinal tract and respiratory tract. The tongue and buccal mucosa are common intraoral sites. The histogenesis of the lesion remains unknown. In this paper, we report the occurrence of an oral granular...

We report three new mutations in PTEN, the gene responsible for Cowden disease in five patients with Bannayan-Riley-Ruvalcaba syndrome from three unrelated families. This finding confirms that Cowden disease, a dominant cancer predisposing syndrome, and Bannayan-Riley-Ruvalcaba syndrome, which includes macrocephaly, multiple lipomas, intestinal hamartomatous polyps, vascular malformations, and ...