Effective treatment of locally advanced periocular basal cell carcinoma with oral hedgehog pathway inhibitor?
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LETTER TO THE EDITOR
Effective treatment of locally advanced periocular basal cell carcinoma with oral hedgehog pathway inhibitor? Xiaoyi Hou 1 & Alexander C. Rokohl 1 & Monika Ortmann 2 & Ludwig M. Heindl 1,3 Received: 29 April 2020 / Revised: 29 April 2020 / Accepted: 1 June 2020 # The Author(s) 2020
Keywords Basal cell carcinoma . Eyelid carcinoma . Eyelid . Hedgehog pathway inhibitor . Sonidegib
Dear Editor, Basal cell carcinoma (BCC) is the most common malignant tumor in the periocular region [1, 2]. The vast majority of periocular BCCs can be effectively treated by complete histopathology-controlled excision, the gold standard for BCC treatment [3]. However, in some cases, alternative approaches are required due to advanced stage of the BCC, reduced general condition of the patient prohibiting general anesthesia, unreasonable cosmetic changes after surgery, or multiple BCC lesions such as in Gorlin-Goltz-syndrome [4]. These alternative approaches include among others systemic therapy with immune checkpoint inhibitors such as sonidegib [4, 5]. Sonidegib (Odomzo®), an oral hedgehog pathway inhibitor (HPI), is indicated for the treatment of adults with locally advanced BCC (laBCC) that are not candidates for surgery or radiation therapy or adults with recurrent laBCC following surgery or radiation therapy [6, 7]. Until today, there is no report of sonidegib for treating periocular laBCC, and evidence regarding optimal management of this condition is still limited. Hence, we demonstrate a case of a laBCC on the lower eyelid margin which was successfully treated with sonidegib. A 73-year-old man with a 20-year history of multiple BCCs at his back, chest, nose, and ear presented with a morphea-like BCC of the left lower eyelid along with an
* Ludwig M. Heindl [email protected] 1
Department of Ophthalmology, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany
2
Department of Pathology, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany
3
Center for Integrated Oncology (CIO) Aachen-Bonn-Cologne-Duesseldorf, Cologne, Germany
ectropion (Fig. 1a). Local biopsy revealed histopathologically confirmed BCC (Fig. 1b). The patient refused any further surgical or radiotherapeutic interventions and preferred systemic treatment. After oral treatment with sonidegib (capsule 200 mg; Odomzo ®) once a day for 6 months, the lesions on the eyelid margin showed no significant clinical remission, and the lower eyelid ectropion persisted (Fig. 1c). Due to the suspicion of a persistent malignant process and in order to treat the ectropion, a full-thickness biopsy of the lower eyelid was performed. The histopathology revealed no manifestation of a BCC anymore but only nonspecific inflammation and slight parakeratosis (Fig. 1d). Ninety percent of BCCs have a pathologic activation of the sonic hedgehog pathway; vismodegib is the first HPI been applicated in the treatment of periocular laBCC [8]. However, during treatment with vismodegib, new
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