A Case of Unresectable Basal Cell Carcinoma with Response to Novel Hedgehog Inhibitor Therapy

Abstract

Basal cell carcinoma (BCC) is the most common cancer worldwide. Traditional treatments include surgery and radiation. We present a case of locally advanced BCC with an excellent response to a combination of Hedgehog inhibitor therapy and radiation.A 70 year old gentleman, presented with a disfiguring mass lesion on the nose and cheeks, which had progressively worsened for seven years prior to presentation.The disease was eroding the nose, cheeks, upper lip, and lower eyelids. A biopsy from the left cheek revealed Basal cell carcinoma. His disease was felt unResectable and hence was started on Hedgehog inhibitor therapy, Vismodegib (Erivedge). He tolerated it well with near complete response to the treatment. Subsequently, he had radiation therapy to the affected area and is currently doing well.Vismodegib and Sonidegib, approved now for treatment of locally advanced and metastatic basal cell carcinoma, appear very promising and are changing the paradigm of treatment of this disease. Their efficacy is also being looked at in other solid tumors. Case PresentationA 70-year-old gentleman has self-referred to us for a mass on his nose. Initially the mass was small at the tip of his nose, but unfortunately, he let it grow for seven years before seeking medical care. On clinical exam, the tip of the nose had eroded as well as the majority of the left side of the nose, extending onto the bilateral cheeks, into the inferior eyelids, and into the bilateral lacrimal glands. The mass which measured approximately 8x10 cm also extended into the upper lip. The patient complained of increased tear formation but denied pain or epistaxis. He was a smoker (one pack a day for 50 years), a social alcohol user, and a truck driver by occupation. His left cheek biopsy showed basal cell carcinoma (Figure 1,2). A CT of the neck showed no pathologic lymphadenopathy. CT sinuses showed nasal and paranasal abnormal and irregular contour. Diffuse nasal cavity, maxillary, and frontal sinus mucosal thickening was noted. Clinically, he had T4N0 basal cell carcinoma of the nose/cheek (Figure 3). Due to the un-resectable and locally advanced stage of his cancer, he was started on neoadjuvant hedgehog inhibitor therapy, Vismodegib (Erivedge), for 5 months. He achieved near complete tumor response. He tolerated the Erivedge well with no untoward side effects (Figure 4). He subsequently completed a course of radiation therapy to the nose and cheek. He received 6000cGy/30 fractions, 6MV, and using 3D conformal technique with lateral fields and 0.5 cm bolus on the skin. He tolerated radiation well except for minor nosebleeds, skin erythema and skin tenderness in the radiated field.