Clinicopathological characteristics of cervical chondrocutaneous branchial remnant: a single-institutional experience

Abstract

Cervical chondrocutaneous branchial remnant (CCBR) is an uncommon developmental anomaly typically seen on the lateral neck. We recently experienced four cases of CCBR and initiated a comprehensive review of previously published cases. During a 10-year period, four (0.4%) of the 1,096 patients who underwent excision of branchial cleft anomalies were diagnosed as having CCBR at our institution. Patient age ranged from 2-6 years and patients presented with asymptomatic cutaneous masses present since birth measuring approximately 1 cm on the lateral neck. Three patients had congenital thyroid hemiagenesis, subependymal cyst, and tongue tie, respectively. We identified 76 previously published cases of CCBR. The median age of these patients was 18 months. CCBR developed more often in males (48/80; 60.0%). Most of the masses were located on the left (34/80; 42.5%) or right (18/80; 22.5%) lateral neck, whereas 23 (28.75%) involved bilateral lesions. Lesion size ranged from 0.3-3.5 cm. Grossly, the overlying skin of the masses was similar to the surrounding skin of the neck. Histologically, the lesions were covered by keratinizing squamous epithelium and had skin appendages and cartilage. Thirty-nine (48.75%) and 12 (15.0%) patients were found to have elastic and hyaline cartilage, respectively. Twenty-eight patients had single (13/28; 46.4%) or multiple (15/28; 53.6%) congenital anomalies. Forty-four different types of anomalies were reported. The most frequent anomalies were problems with cardiovascular and auditory systems. Our observations suggest that CCBR is a visible marker for more serious associated congenital anomalies. We recommend that clinicians and pediatricians further evaluate patients with CCBR through complete physical examination, abdominal and cardiac ultrasound, karyotyping, and biochemical marker analysis.