Abstract
We report the case of a 3-year-old male with a past medical history significant for mild speech and developmental delay, presenting with a stable 1 cm right anterior neck mass present since birth. The clinical differential diagnosis included dermal cysts such as pilomatricoma and branchial cleft cyst. On subsequent excision, the lesion was composed of numerous vellus hair follicles overlying subcutaneous tissue containing a central cartilaginous core with surrounding fibrosis with the appearance of an accessory tragus. However, given the clinical setting and anatomic location, a diagnosis of chondrocutaneous branchial remnant (CCBR) was rendered. CCBR is a rare congenital developmental anomaly typically located in the lateral neck, specifically at the anterior border of the sternocleidomastoid muscle. While the majority of the cases are unilateral, bilateral forms have also been described. Histologically, CCBR has the appearance of an accessory tragus demonstrating a central cartilaginous core with surrounding fibrosis located in the subcutaneous tissue. Elastic fibers are present in the majority of cases, suggesting the cartilage to be of elastic origin; however, occasional cases without elastic fibers have also been reported. Additionally, up to 75% of cases can be associated with a variety of congenital anomalies, particularly involving the auditory, cardiovascular and visual systems. Given that research-based evidence related to CCBR in dermatology literature is sparse, we present this case to raise more awareness about this entity among dermatopathologists, review the different histologic presentations and possible associated congenital anomalies.
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