When a Child has Neck Mass, Consider a Choristoma too! Here’s a Rare Instance: A Cervical Chondrocutaneous Branchial Remnant

Abstract

Cervical chondrocutaneous branchial remnants (CCBR) are rare causes of neck masses in young children. It is commonly associated with genitourinary and cardiac anomalies. We report a case of CCBR in a two-year-old male child who presented with a unilateral painless pedunculated neck mass. Although ultrasonography is noncontributory, histopathology reveals a central core of hyalin cartilage with surrounding skin tissue. A diagnosis of CCBR was made on histopathology, and the patient was further clinically evaluated. The child did not reveal any other anomalies and is currently on follow-up.

Introduction

Cervical chondrocutaneous branchial remnants (CCBR) are choristomas observed around the lower neck [1]. This occurs due to an aberration in the second branchial arch [2]. They are predominantly seen as neck masses, precisely as pedunculated skin auricles/polyp since birth, either unilaterally or, less commonly, bilaterally [3]. To date, fewer than 150 cases have been published in the literature, and many are associated with other congenital syndromes [3]. They are usually located around the lower third of the sternocleido muscle and appear as accessory auricle [2]. The important goal of the diagnosis of this benign congenital lesion is to rule out other congenital anomalies, such as cardiac anomalies, which can lead to significant morbidity.

Case Report

This report describes a 2-year-old male who presented with a unilateral, pedunculated, painless neck mass on the left side of the neck since birth (Fig. 1A). There was no significant family or personal history. The mass exhibited no significant changes in size, discharge, or other noteworthy findings.

Fig. 1.

A: Pedunculated mobile non-tender skin tag on the middle third of the lateral aspect of the right neck. B: Excision specimen with whitish cut surface. C: Sections showing skin-covered polypoidal tissue with a central cartilaginous core (H&E stain, 2X magnification). D: Cartilage surrounded by fibro adipose tissue (H&E stain, 10X magnification)

Ultrasound findings were unremarkable, showing no significant vascularity. The patient was subjected to surgical excision. The excision specimen was a firm, polypoidal tissue covered with skin, measuring 1.5 × 0.8 × 0.7 cm. The outer surface appeared unremarkable, and the cut sections were solid and whitish (Fig. 1B). Initially, the patient was considered to have a fibroepithelial polyp due to its gross appearance. However, microscopic examination revealed polypoidal tissue lined by stratified squamous epithelium, with normal adnexal structures, subcutaneous fat, and a central core of mature cartilaginous tissue (Fig. 1C and D). A diagnosis of a cervical chondrocutaneous branchial remnant was made on the basis of the histological findings.

Upon diagnosis of CCBR, the patient was screened clinically for any associated congenital anomalies; however, no such findings were noted.

Discussion

Cervical chondrocutaneous branchial remnants (CCBR) are rare causes of neck masses in toddlers or infants. In resource-poor countries such as India, many cases are remained undiagnosed and untreated. While believed to be of embryologic origin, the exact mechanism of formation or aberration is still under investigation [4]. The location of the lesion can lead to many differential diagnoses, including dermoid cysts, teratomas, lymphoid hyperplasia, branchial cysts, etc [5]. In our case, the mass was unilaterally located at the lower two-thirds of the sternocleidomastoid muscle, which is a common presentation. In the literature, few cases are familial; [6] however, there was no family history in our case. Histologically, these lesions show central hyaline cartilage surrounded by normal skin tissue. Some cases also exhibit mild epidermal papillomatosis, hyperkeratosis, and subcutaneous fibrosis [7]. To diagnose this entity, Altan et al. [8] provided few criteria that are still very relevant. This includes.

1. predominance in males (11 of 17);

2. high incidence of associated anomalies (76%) involving the auditory (neurosensory deafness, serous otitis media, and malformation of the external ear), respiratory (tracheomalacia), oro-gastrointestinal (cleft palate, oronasal reflux and inguinal hernia), genitourinary (hydronephrosis), cardiovascular (atrial septal defect), musculoskeletal and visual systems;

3. the presence of a cartilage core;

4. A scarcity of bilateral lesions (1 of 17);

5. located in the middle or lower third of the neck; and

6. Increased prevalence anterior to the SCM muscle.

A definite diagnosis is important in this subset of patients, as many patients may have underlying genitourinary or cardiac anomalies, such as vesicoureteral reflux, atrial and ventricular septal defects, and ventricular septal defects [6]. However, upon histopathological diagnosis, our patient did not present any such anomalies in further clinical workup. The patient is on follow-up.

Conclusion

Although rare, this benign entity needs to be considered for clinical differential diagnosis to investigate any associated major congenital anomalies further. Early diagnosis can help screen for associated conditions and prevent catastrophic complications.

Author Contributions

All Authors Contributed equally in the preparation of this manuscript. All authors have read and approved the manuscript.

Funding

No funds were received.

Declarations

Consent to Participate and Ethical Approval

Informed written consent was taken from the patient’s parent. As per institute policy, ethical clearance is not required as no patient intervention was done.

Competing Interests

The authors declare that they have no conflict of interest.