Female Patient with Two Simultaneous Giants Facial Basal Cell Carcinomas Demonstrates a Positive Response to Vismodegib as a Monotherapy

Abstract

Basal cell carcinoma (BCC) is the most frequent histological type of cancer in the world and accounts for approximately 80% of all skin cancers. In the majority of cases, they are slow-growing, low metastatic potential tumors, easy to cure by surgical or nonsurgical procedures. Giant BCC (GBCC) is a rare variant of BCC and according to the American Joint Committee on Cancer, this includes lesions with a diameter larger than 5 cm. GBCC's incidence has been reported to be less than 1%, and it displays a more aggressive behavior with both local invasion and higher metastatic potential. Archodaki et al. specifically reported that metastasis was present in 17.6% of GBCC patients during the primary examination. Patients with GBCC who are not suitable for either surgery or radiotherapy since 2012 seem to have another therapeutic option. Vismodegib is an oral small-molecule inhibitor of the Hedgehog pathway (HPI) that was approved for treating metastatic or locally advanced BCC in patients who are poor candidates for surgery or radiotherapy. In this case, we present a woman with two simultaneous facial GBCCs who was treated successfully using vismodegib as a monotherapy.

A 93-year-old Caucasian woman living in a remote region presented to our hospital with two non-healing sores located on her forehead and nose. Both lesions initially started as red papules that increased in size over a period of 25 and 15 years, respectively.

Her medical history revealed high blood pressure, atrial fibrillation, and coronary artery disease. Due to her bad performance status and her limited access to health providers, the patient sought medical attention ultimately when there was intermittent bleeding and pain of the lesions.

On evaluation, the patient had two well margined ulcerated bleeding tumors: the first one on her forehead measuring 10.5 cm × 8 cm locally extended to her left upper eyelid and the second one measuring 8 cm × 4 cm on her nose [Figure 1]. Physical examination revealed no palpable lymph nodes. Skin biopsy was performed on both lesions, and histopathology confirmed nodular BCC [Figure 2]. Her laboratory tests revealed hypochromic microcytic anemia, and she refused to undergo any imaging studies. Considering the large size and facial location of both lesions, a surgical approach would lead to a substantial deformity and thus was excluded. Her inconvenience of multiple visits to the hospital for treatment made radiotherapy an unfeasible option. Under these circumstances, we agreed to initiate oral vismodegib 150 mg daily. It was well tolerated by the patient as she reported experiencing no other treatment-emergent adverse events than mild dysgeusia and alopecia. On re-evaluation, 6 months later, there was a complete resolution of the nose ulceration and the tumor on the forehead had shrunk to a small-crusted area of 1.7 cm in diameter [Figure 3]. Unfortunately, a new skin biopsy was not possible as the patient did not consent. During a 14-months follow-up, no disease progression has occurred improving her overall quality of life.

Figure 1.

Clinical image of the patient before initiating treatment with vismodegib

Figure 2.

Histopathology revealed a large, rounded, predominantly dermal-based nest with prominent peripheral palisading

Figure 3.

Clinical image of the patient after 6-month treatment with vismodegib

GBCC is a rare variant of the most frequent skin cancer with an occurrence rate between 0.5%–1% out of all types of BCCs.[1,2,3,4,5] To our knowledge, this is the first case of two simultaneous facial GBCC of an elderly woman that were treated effectively with vismodegib as a monotherapy. As in the majority of GBCC cases, this patient's neglect[5,6,7] in addition to her disability to access proper medical care resulted in a very difficult case to treat. Our case represents the burden of treating patients with nonoperable or nonamenable radiotherapy tumors in the era prior to HPIs.[8,9] Herein it is shown that vismodegib can be an efficient treatment alternative for such giant BCC neoplasms. Moreover, the progressive-disease-free period of 16 months may reflect our clinical observations that naive patients for previous treatments (i.e., surgery, cryotherapy, HPIs, etc.) respond faster and have a prolonged positive clinical result.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.