Locally Advanced Basal Cell Carcinoma with Intraocular Invasion.

We present a 103 - year - old patient, with duration of complaints of about ten years. The initial complaint had been presented as a small nodule, located on the eyebrow, which subsequently ulcerated and encompassed larger regions of the upper and lower eyelids. For the past three years, the patient also had complaints of a worsening of his vision, without seeking for medical help. Within the dermatological examination, an intraocular and periocular localised tumour was established, characterised by a raised peripheral edge and central ulceration. More careful examination revealed that the bulb was fully consumed. The patient refused further diagnosis and treatment. Advanced basal cell carcinomas with intraocular invasion are rare in general. If the patient refuses surgery, radiotherapy and systemic therapy with modern medications such as Vismodegib or Sonidegib are available as treatment options.

Introduction

Basal cell tumours can be easily treated in their early stages [1]. However, with the larger tumour growth, the more extensive treatment is needed [1]. Some special locations (eyelids, nose flap, lips, auricle) pose a challenge for the surgeon to remove the tumour entirely with proper margins and to retain functionality and satisfactory aesthetic appearance at the same time [1]. Furthermore, large, neglected tumours, especially located in the face region, demand complex reconstructive procedures after excision, i.e. local reconstruction techniques, free grafts, advanced, island, pedicle or microvascular flaps [1].

Case Report

A 103 - year - old male patient, a peasant from the Bulgarian mountain region, presented with a reddish facial lesion. The reported complaints were from the last ten years. The lesion had occurred as a small papule on the medial part of the right eyebrow, followed by gradual enlargement and ulceration on a later stage, leading to complete loss of the upper eyelid (Fig. 1 a, b). The diagnosis of multicentric basal cell carcinoma (BCC) was made clinically and histopathologically. Any treatment was denied.

Figure 1

A) 103-year-old male patient with oozing and crusted periocular lesion; B) Detail with loss of lids and eye

Neglected patients are one of the major contributing factors for the development of mutilating, horrifying and aggressive BCC - a rare, but potentially fatal cutaneous tumour [1]. Basal cell carcinoma occurs in the area of the head in 85 - 90% of cases, often above the line connecting the mouth corner with the ear lobe [2]. They are less often found in the lower part of the face and on the scalp [1].

Discussion

In 10 - 15% of the cases, BCC is found on the neck, trunk and limbs. The affected area is commonly closely related to the exposure to ultraviolet radiation [3]. Although BCC could be rarely metastatic, it has a potential for tremendous and life - threating regional destruction in some locations, leading to severe disfigurations as in the presented case [1][2].

Two systemic treatments options that target the hedgehog pathway have become available recently for patients with advanced BCC: sonidegib (Odomzo; Novartis) and vismodegib (Erivedge; Roche). Based on results from a noncomparative study (the 200 mg arm of the BOLT trial) [3], sonidegib was approved recently in the United States and European Union for the treatment of adults with locally advanced BCC (laBCC) who are ineligible for curative surgery or radiotherapy [4][5]. Similarly, vismodegib is indicated for the treatment of adults with symptomatic metastatic BCC or adults with laBCC who are ineligible for surgery or radiotherapy, approved based on a single-arm trial (ERIVANCE) [6][7].