Saudi Journal of Medicine and Medical Sciences

CASE REPORT
Year
: 2014  |  Volume : 2  |  Issue : 3  |  Page : 210--212

Bilateral cervical chondro-cutaneous remnants


Sameer A.H. Ansari, Sushma N Ramraje, Shalini R Gupta, Nitin S Deore 
 Department of Pathology, Grant Govt. Medical College and Sir J.J group of Hospitals, Mumbai, Maharashtra, India

Correspondence Address:
Sameer A.H. Ansari
Pathology, Grant Govt. Medical College and Sir J.J group of Hospitals, Mumbai - 400 008, Maharashtra
India

Abstract

Cervical chondro-cutaneous brachial remnant (CCBR) is a developmental anomaly presenting at birth and are usually seen on the lateral aspect of neck, anterior to sternocleidomastoid muscle. Bilateral appearance of this lesion is extremely uncommon. These lesions are painless and lack any inflammation or discharge. Since there is no connection with underlying deeper structures, complete surgical excision is the standard treatment in such cases. It is associated with several other congenital anomalies in 70-80% of cases. Hence, thorough evaluation of patients (clinical and investigation) becomes mandatory in such cases to detect any additional anomaly. We report a case of a 6-year-old female with bilateral cervical chondro-cutaneous remnant located at the lower third of lateral aspect of neck, anterior to sternocleidomastoid muscle, and discuss the embryologic and diagnostic aspects considering the common differential for this lesion at this site.



How to cite this article:
Ansari SA, Ramraje SN, Gupta SR, Deore NS. Bilateral cervical chondro-cutaneous remnants.Saudi J Med Med Sci 2014;2:210-212


How to cite this URL:
Ansari SA, Ramraje SN, Gupta SR, Deore NS. Bilateral cervical chondro-cutaneous remnants. Saudi J Med Med Sci [serial online] 2014 [cited 2019 Sep 21 ];2:210-212
Available from: http://www.sjmms.net/text.asp?2014/2/3/210/142568


Full Text

 INTRODUCTION



Bilateral cervical chondro-cutaneous remnants are very rare congenital lesions with very few cases reported so far in the literature. [1] Knowledge of early embryologic processes from cartilage structures in the head and neck region help us to understand the etiology and consequently histology of this condition. [2] Although benign in nature, their close association with serious underlying congenital anomalies made them a visible indicator for such anomaly. [3] With due written consent from the patient, we present this case of a 6-year-old female with bilateral cervical chondro-cutaneous remnant.

 CASE REPORT



A 6-year-old female, born of nonconsanguineous marriage and uneventful pregnancy presented with bilateral, smooth exophytic mass in the lower half of the neck since birth. The mass was gradually increasing and at present measured about 2.5 × 0.9 cm. The lesion was firm and mobile in relation to its underlying structure. There was no evidence of any inflammation, discharge, epidermal pit, or pore. There was no family history of congenital abnormalities, mental retardation or chromosomal abnormalities. General physical examination revealed no dysmorphic signs like a hypoplastic mandibula or abnormally shaped auricles. Ultrasonography (USG) of the abdomen revealed no other anomaly. The mass was excised. During excision of the tumor, the lesion extended into the neck and connected to the fascia at the anterior part of sternocleidomastoid muscle. Gross examination revealed a completely excised tumor, measuring 2.5 × 0.9 cm [Figure 1], covered by skin, which was normal and intact. Histological examination showed the presence of central zone of chondrocytes covered by unremarkable epidermis [Figure 2] and [Figure 3]. No evidence of glandular, epithelial or cystic tissue was found. No evidence of malignancy seen. Follow up after 6 months showed no signs of recurrence or any complications.{Figure 1}{Figure 2}{Figure 3}

 DISCUSSION



Cervical chondro-cutaneous brachial remnants (CCBR) are uncommon benign congenital lesions found in the lateral aspect of the neck anterior to sternocleidomastoid muscle. [4],[2] Previously it was described under variety of names including tags, wattles, appendages, polyps, vestiges, accessory auricles or tragi, rests, choristomas, hamartomata, papillomata, fibromata, etc. [4],[2] This confusion was due to the fact that their origin was not clearly understood. [5] It was Atlan et al. who described in a series of 17 patients of congenital neck anomalies proposed the term chondro-cutaneous branchial remnants. [6] They originate during transformation of branchial embryonic structure that usually disappears during maturation. Two theories have been proposed for the same. Some authors postulated that it arises from ectopic auricular tissue. [7],[8],[9] Others believe that it originates from branchial tissue contributing to the formation of most cervical tissues. [9],[10] Depending upon the presence of type of cartilage, the origin of lesion is determined. Presence of elastic cartilage would suggest an auricular (first or second branchial arch) origin or derivation from lower neck, [11] whereas hyaline cartilage favor cervical spine as seen in our case. Cervical chondro-cutaneous remnants are commonly seen in males and are present from birth as described by Atlan et al.; [6] our patient is a female. A painless protruding cervical mass without discharge or inflammation is the most common manifestation in such cases. [1] Our patient presented with similar complaints. Family history is significant as it is familial in origin. [6] Grossly, they are skin covered masses showing cartilaginous tissue in deeper layer or core. Microscopic examination showed lining by keratinised stratified squamous epithelium with deeper tissue showing hair follicles along with cartilage (hyaline or elastic depending upon origin). [3],[6] USG plays pivotal role in such cases. It helps define the size and extent of mass and also to detect the associated anomalies. [6],[12] Associated anomalies are common in 70-80% of cases. They include anomalies of auditory, respiratory, gastrointestinal, genitourinary, cardiovascular, musculoskeletal, and visual system. [6],[12] Hence if skin tags are detected, it becomes necessary for a detailed examination with imaging modalities of the patient. CCBR have, despite their clear clinical markings, a broad differential diagnoses. [2],[13] Which can be easily differentiated on histopathology. It includes thymic cysts and thyroglossal duct cysts, branchial cleft, hair follicle nevus, congenital midline hamartoma, fibroepithelial polyp, epidermoid cyst, and squamous papilloma. Thymic cysts are differentiated by lining with stratified squamous epithelium and cyst lumen filled with fluid. Thyroglossal duct cysts are located anteriorly and contain thyroid follicles. Branchial cleft cysts, although located laterally, are differentiated by the fact that they are lined by upper respiratory epithelium and they have a shallow epidermal pore and contain seromucinous glands. Congenital midline hamartomas are located on the anterior mandibular and have prominent skeletal muscle component. Epidermoid cysts, squamous papillomas, and fibroepithelial polyps do not usually present differential diagnostic difficulties. [2],[13] The definitive treatment of this remnant is simple excision extending up to the muscle, which was done in our case and it proves to be adequate. [3],[6] The prognosis in CCBR is favorable as recurrence and malignant transformation in it has not been reported so far. [3],[6] Atlan et al. stated the average age of surgery to be12 months . [6] In our case, the patient presented at 6 years of age, as she was from low socioeconomic background and also by the fact that she was hailing from a remote area. We propose the excision to be done as early as possible so that the patient is spared of psychological complications of having bilateral visible neck masses.

 CONCLUSION



CCBR is a rare congenital lesion with favorable prognosis and no malignant transformation. Since it is associated with underlying serious congenital anomaly, hence it becomes necessary to do a thorough evaluation of the patient with imaging modalities.

References

1Hemmaoui B, Fejjal N, Nadour K, Benariba F. Cervical chondrocutaneous brachial remnants: Two cases report. J Pediatr Dermatol 2013;14:33-5.
2Rouas L, Tahri R, Lamalmi N, Alhamany Z, Cherradi N. Congenital Cervical Cartilaginous Rest. Canadian J Pathol 2013;5:23-5.
3Altin G, Erdogan BA, Paksoy M, Sanli A, Eken M, Yavuzer D, et al. Cervical chondrocutaneous branchial remnant: A case report. J Pediatr Sci 2012;4:e161.
4Nasser HA, Iskandarani F, Berjaoui T, Fleifel S. A case report of bilateral cervical chondrocutaneous remnants with review of the literature. J Pediatr Surg 2011;46:998-1000.
5Tamir S, Nidal M, Constantin R, Perez R, Sichel JY. Bilateral cervical chondrocutaneous branchial remnants. Int J Pediatr Otorhinolaryngol 2008;3:117-9.
6Atlan G, Egerszegi EP, Brochu P, Caouette-Laberge L, Bortoluzzi P. Cervical chondrocutaneous branchial remnants. Plast Reconstr Surg 1997;100:32-9.
7Kim SW, Moon SE, Kim JA. Bilateral accessory tragi on the suprasternal region. J Dermatol 1997;24:543-5.
8Atherton DJ, Rook A. Naevi and other developmental defects. In: Rook A, Wilkinson DS, Eblings FJ, (editors). Textbook of Dermatology, 4 th ed. Boston: Blackwell Scientific; 1986. p. 167-84.
9Clarke JA. Are Wattles of auricular or branchial origin? Br J Plast Surg 1976;29:238-44.
10Hogan D, Wilkinson RD, Williams A. Congenital anomalies of the head and neck. Int J Dermatol 1980;19:479-86.
11Sperling LC. Congenital cartilaginous rests of the neck. Int J Dermatol 1986;25:186-7.
12Vladareanu R, Zvanca M, Andrei C. Pathology of fetal neck. In: Kurjak A, Chervenak FA (editors). Donald School Textbook of Ultrasound in Obstetrics and Gynecology, 3 rd ed. New Delhi: Jaypee Brothers Medical publisher; 2011. p. 286-7.
13Rund CR, Galyon SW, Fischer EG. An anterior neck mass in a 5-month-old female infant. Arch Pathol Lab Med 2004;128:1453-4.