Abstract
A solitary mucosal angiokeratoma is an extremely rare presentation. In this report, we present a 67-year-old woman with a 3 cm solitary angiokeratoma involving the tongue, who was treated with intensity modulated radiation therapy after declining surgery. The patient is alive and free of disease at 1.5 years following radiation therapy.
1
Introduction
Angiokeratomas are rare muco-cutaneous vascular lesions that are defined as one or more dilated vessels lying in the dermis just beneath the epidermis, often accompanied by an epidermal reaction such as acanthosis and/or hyperkeratosis . Although angiokeratomas share similar histologic features, they might differ with regard to anatomical localization and clinical presentation. They can present in either a localized or a generalized form. Treatment strategies for angiokeratomas are governed by cosmetic concerns and by the degree of symptoms such as bleeding and pain. Local excision, cryotherapy or electrocautery could be appropriate for smaller lesions, while larger lesions would be treated with wide excision or laser ablation, although at the expense of significant scarring . An isolated mucosal angiokeratoma involving the oral cavity is an extremely rare presentation. In this case report we describe, for the first time in the literature, the treatment of a solitary angiokeratoma of the tongue in an adult patient with radiation therapy.
2
Case report
A 67-year-old woman presented with severe pain involving the base of her tongue that was accentuated on chewing and speaking. There was no history of local trauma. Her clinical examination revealed a fleshy and exophytic lesion with a rough and corrugated surface, red to violet in colour that involved the right lateral aspect of the middle third of the mobile tongue without crossing the midline. Direct laryngoscopy showed that the lesion extended towards the vallecula ( Fig. 1 A ). There were no other changes in the oral mucosa and cervical lymph nodes were not palpable.
Magnetic resonance imaging (MRI) showed a superficial mass located posteriorly on the right side of the tongue, measuring 2.5 × 2.5 × 3 cm ( Fig. 2 A ). The mass was of isointense signal to the adjacent tongue muscles on T1-weighted images before and after intravenous gadolinium injection. On T2-weighted images it was brighter than the surrounding tissues, yet not as bright as would be expected from a hemangioma or lymphangioma. Curvilinear structures of high T2 signal were also noted, probably representing venous or arterial microvasculature.
The patient underwent biopsy under local anesthesia. Bleeding, which was minimal, was controlled using cauterization. On histopathological examination, the lesion was characterized by non-keratinized squamous epithelium with hyperkeratosis, parakeratosis, acanthosis and dilated vascular spaces in the papillary dermis ( Fig. 3 ). The lesion involved the subepithelial tissue (lamina propria) and reached the muscular tissue. Large dilated vascular spaces lined by normal appearing endothelium and filled with blood were present and surrounded by elongated rete ridges. In the surrounding connective tissue, a chronic inflammatory infiltrate was observed. Based on these findings, the diagnosis of an angiokeratoma was made.
Upon this diagnosis, the patient underwent a thorough dermatological examination that revealed no angiokeratomas involving the skin elsewhere. Given the location and the size of the lesion, standard treatment options including electrocoagulation, cryotherapy and laser ablation were not deemed as viable alternatives due to considerations regarding both treatment adequacy and functional outcome. Since the patient declined extensive surgical excision, the decision was made for the patient to be treated with intensity modulated radiation therapy. The prescribed radiation dose was 46 Gy, delivered in daily fractions of 2 Gy. At 6 months following radiation therapy, the lesion was completely resolved on both clinical ( Fig. 1 B) and radiological ( Fig. 2 B) examination. The patient is alive and free of disease at 1.5 years following radiation therapy.
2
Case report
A 67-year-old woman presented with severe pain involving the base of her tongue that was accentuated on chewing and speaking. There was no history of local trauma. Her clinical examination revealed a fleshy and exophytic lesion with a rough and corrugated surface, red to violet in colour that involved the right lateral aspect of the middle third of the mobile tongue without crossing the midline. Direct laryngoscopy showed that the lesion extended towards the vallecula ( Fig. 1 A ). There were no other changes in the oral mucosa and cervical lymph nodes were not palpable.
