Treatment of periocular basal cell carcinoma with neoadjuvant vismodegib

Abstract

Purpose: To report a case of a locally advanced periocular basal cell carcinoma treated with neoadjuvant Vismodegib therapy prior to surgery.

Observations: A 63-year-old female presented to the oculoplastics clinic with biopsy-proven basal cell carcinoma of the right periorbital region causing significant cicatricial ectropion of the right lower eyelid. The medial canthal lesion involved nearly the entire right lower eyelid with extension onto the cheek, the medial half of the right upper eyelid, the palpebral and bulbar conjunctiva, as well as the right lacrimal system. CT imaging was suggestive of involvement of the extraocular muscles and other post-septal tissues. Fortunately, the patient had no metastatic disease. The extent of the tumor would have necessitated aggressive resection to achieve surgical cure. However, the patient preferred to attempt globe-sparing therapy with a goal of preserving cosmesis as much as possible. Various treatment options were discussed with the patient, including the use of Vismodegib, and the patient elected to pursue this treatment strategy. The goal of Vismodegib treatment was to reduce the tumor size enough to permit surgical resection of all tumor without significantly affecting cosmesis. After 11 months of treatment with Vismodegib, the tumor size had reduced significantly to the point where surgical intervention with minimal disfigurement could be offered. The patient underwent multidisciplinary approach with Mohs micrographic excision of the tumor paired with oculoplastic reconstructive surgery resulting in negative margins and satisfactory cosmetic results.

Conclusions and importance: Although addition study is required regarding Vismodegib as a primary or adjuvant therapeutic approach to periorbital basal cell carcinoma, this case illustrates the potential usefulness of this drug as an option in this context. This case provides information that may help the comprehensive ophthalmologist or oculoplastic specialist in counseling patients with locally advanced periorbital basal cell carcinoma.

Keywords: Basal cell carcinoma; Erivedge; Hedgehog pathway inhibitor; Smoothened homologue; Vismodegib.

Fig. 1

The patient at presentation, both eyes.

Fig. 2

The patient's right eye.

Fig. 3

The patient's right eye, lateral view.

Fig. 4

4A: CT imaging showed soft tissue mass (arrows) measuring 31mm (transverse) x 23mm (antero-posterior) x 26mm (craniocaudal), with involvement of anterior right lamina papyracea. There is an ulcerated soft tissue mass in the medial right orbit which measures up to 31 mm transverse by 23 mm AP by 26 cm craniocaudal. The medial most extent of this mass appears to breech the anterior right lamina papyracea and there is contiguous opacification of a right anterior ethmoid air cell. 4B: The inferomedial portion appears to breech the nasolacrimal duct with concern for tumoral extension into the duct. The superior extent of this lesion extends medially along the orbital wall with obliteration of the fat plane involving the inferior oblique muscular origin, the myotendinous junction of the medial rectus muscle, and the tendon of the superior oblique muscle. 4C: The posterior aspect extends to involve the post septal soft tissues. The lateral most extent abuts the medial sclera, the right globe is otherwise unremarkable.

Fig. 5

5A: The right orbital floor appears intact, but there is extensive mucosal thickening of the right maxillary sinus (also visible in 5B)5B: There is mucosal opacification of the right maxillary ostium, the middle meatus is patent on the right. 5C: There is mild mucosal thickening of the left maxillary sinus, ostiomeatal unit on the left is patent.

Fig. 6

6A: 4 months after initiation of Vismodegib.6B: 10 months after initiation of Vismodegib. 6C & D: Before and after. These figures compare initial presentation (C) to 17 days post-op (D).