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CASE SERIES

BRANCHIAL CYST: A DIAGNOSTIC DILEMMA AND A SURGICAL CHALLENGE Shruti Bansal1, Harshad Nikte2, Arpit Sharma2, J.P. Dabholkar3 1- Medical officer, 2-Assitant Prof., 3- Prof. & HOD, Department of ENT and Head-Neck Surgery, Seth G.S. Medical College and KEM Hospital, Mumbai, Maharashtra, India

ABSTRACT Branchial cleft cysts typically present as a unilateral, fluctuant soft tissue swelling that are mostly localised deep to the anterior border of sternocleidomastoid in the lateral aspect of the neck at the junction of its upper third and lower two third. Diagnosis is usually straightforward and can be made clinically however FNAC facilitates diagnosis.Unusually they can mimic as an abscess or metastatic node and less commonly present at an unusual location. We encountered 3 cases where patients presented with neck swelling either with unusual presentation or atypical location or inconclusive FNA. This report describes a series of patients in which the diagnosis of branchial cysts was not straightforward and posed a diagnostic challenge. Keywords: Branchial cleft cysts, FNAC, Surgical excision Cite this article : Shruti Bansal, Harshad Nikte, Arpit Sharma, J. P. Dabholkar, Branchial Cyst: A Diagnostic Dilemma and a Surgical Challenge, J. Head & Neck phys and surg, Vol 5(1), 2016, Pg-1-8.

INTRODUCTION Lateral cysts of the neck were first described by Hunczovsky in 1785 1, 2. Branchial cleft cysts (BCCs) are the most common cysts in the neck3, 4, 5, 6.The location of a BCC is determined by which branchial cleft failed to obliterate during embryological development, withsecond BCC being the most commonly recognized lesion7.They are mostly located along the anterior border of sternocleidomastoid lying in upper thirds, however the literature does describe unusual locations8. Diagnosis is normallystraightforward and is made by identifying a fluctuant painless swelling in the neck. However atypical presentations of BCClike those mimicking an abscess or rarely 1

a malignant node or unusual location in the neck may pose a diagnostic challenge. Diagnostic procedures like USG, FNAC, CT-scan and sometimes PET-CThelps in establishing the accurate pre-operative diagnosis and thus facilitating the proper treatment. Through this article we take pleasure to report 3 cases of BCC who presented with atypical features. CASE 1: 15-year-old girl presented with right sided neck swelling since 4 months. On examination it was a soft, cystic, non-tender, mobile swelling of approximately 7x 5 cm in size situated along the anterior border of sternocleidomastoid extending anteriorly up-to midline. (Figure 1) Patient underwent sonography from outside before presenting to our institute which was suggestive of hypoechoic lesion with internal echoes and gave the diagnosis of infective pathology suggestive of KOCH’S. On inquiry she had positive history of weight loss, anorexia and evening rise in temperature. Her chest x-ray showed old healed tubercular pathology in bilateral hilar region. At first it looked to be a case of neck abscess mostly tubercular in origin. However, considering the age of the patient and location of the swelling the differential of branchial cyst was considered. CT-scan of neck was done before undertaking any interventional procedure. CT neck showed 7.7x 5.3 x2.9 cm hypodense lesion in right carotid space with splaying of carotid artery and internal jugular vein (Figure 2). The differential diagnosis of cystic schwannoma, infected branchial cyst and abscess were given.

FIGURE 1: 7x5 cm right sided neck swelling along sternocleidomastoid

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Figure 2: 7.7x5.3 x2.9 cm hypodense lesion in right carotid space with splaying of carotid artery and internal jugular vein

FIGURE 3: Intra-Operative Picture of Cystic Swelling Splaying the Great Vessels of The Neck

Figure 4: Excised Specimen of Cystic Swelling 3

Following a course of intravenous antibiotic, patient underwent surgical excision of the lesion. Intraoperatively we found a soft cystic swelling along the sternocleidomastoid with a stump going retrosternally along the innominate artery (Figure 3). However, we were successful in removing the entire cyst in situ (Figure 4). Post-operative histopathology reported it as cystic swelling lined with squamous epithelium with fluid containing macrophages and cholesterol clefts suggestive of branchial cleft anomaly. CASE 2: 45-year-old male presented with left submandibular swelling since 2 months. On examination swelling was approximately 2x 2cm is size, hard in consistency, non-tender and localised to the left submandibular area. Fine needle aspiration cytology from the lesion revealed it to be metastatic squamous cell carcinoma. Detailed history, thorough clinical and endoscopic examination revealed no evidence of any obvious primary in oral cavity, larynx and pharynx. We got a PET-CT done to look for primary lesion. It showed 3x4 cm cystic lesion in left carotid space extending from angle of mandible to thyroid cartilage deep to left sternocleidomastoid likely to be branchial cyst (Figure 5). PET Scan showed focal active disease with low metabolic activity in left submandibular area with no evidence of obvious primary (Figure 6). Patient underwent excision of the lesion. Post-operative histopathology confirmed it to be branchial cyst which was mimicking a metastatic node

Figure 5: PET-CT Scan showing 3x4 cm cystic lesion in left carotid space

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Figure 6: PET Scan showing focal active disease with low metabolic activity in left submandibular area CASE 3: 26-year-old male presented with rapidly increasing left sided neck swelling since 10 days. He gave a history of intermittent small swelling in left lateral neck which resolved with medication intermittently. The swelling was associated with pain and tenderness. On examination patient was febrile and had approximately 5x 4 cm soft cystic erythematous swelling in left lateral neck along sternocleidomastoid. USG was suggestive of hypoechoic cystic swelling with internal echoes suggestive of abscess. Considering the huge size of the swelling CT scan was ordered which showed 5x5 hypodense cystic lesion with thick walls,extending from below the ramus of mandible up-to thyroid cartilage. The swelling was situated antero-medial to sternoceidomastoid muscle and superior to carotid bifurcation (Figure 7). Considering the typical CT picture diagnosis of infected branchial cyst was made. Patient was given a course of intravenous antibiotics and was posted for surgical excision under general anaesthesia. Post-operative histopathology confirmed it to be infectedbranchial cleft cyst.

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FIGURE 7: 5x5 hypodense cystic lesion at level of thyroid cartilage causing posterolateral displacement of sternocleidomastoid and lying above carotid bifurcation DISCUSSION: BCC are the most common congenital neck masses usually presenting in the lateral neck 3, 4, 5, 6 . They mostly present as painless fluctuant masses developing gradually over months to years. Fluctuation, due to the fluid contents, is described as an important diagnostic feature, present in more than two thirds of cysts9,10.Although majority of them are located along the anterior border of the sternocleidomastoid muscle lying in the upper thirds, the literature does describe unusual locations.Mostly they occur in second to third decades of life with no sex predilection. Some variations of this presentation are well recognised. 20% of cases may have intermittent swelling which may be associated with pain during pharyngeal or respiratory infections. One third of cases may develop infection of the cyst 9, 10, 11, 12. Occasionally they may mimic a hard mass due to chronic infection thus confusing with Hodgkins, tubercular lymphadenopathy and even metastatic node thus posing a diagnostic dilemma13. Diagnosis may be difficult due to varied location and absence of fluctuation in such cases. This report describes a series of patients in which the diagnosis of branchial cysts was not straightforward The branchial cleft cysts are developmental cysts with disputed pathogenesis. Various theories have been proposed regarding its etio-pathogenesis. 4 most commonly stated theories are 1. Congenital theories: The classic theory that the cyst develops from remnants of the embryonic gill apparatus. 2. Lymph node theories: In 1949, King concluded that the cyst arises from cystic changes in parotid epithelium that becomes entrapped in the upper cervical lymph nodes during embryonic life. 3. Branchial theory: Cyst develops from imperfectobliteration of the pharyngeal cleft. 4. Pre-Cervical Sinus Theory: The cyst develops from investigates of the cervical sinus rather than of the pharyngeal clefts or pouches2. The most accepted theory is development of branchial cleft occurs by second arch overgrowing the other arches to form elongated cavity; cervical sinus His. Second branchial anomalies mostly occur due to incomplete closure of cervical sinus of His.Most frequently, these cysts are identified along the anterior border of the upper third of the sternocleidomastoid muscle8. However, these cysts may develop anywhere along the course of a second branchial fistula, extending from the skin to the lateral neck, between the internal and external carotid arteries, and into the palatine tonsil. In recent years, CT has been shown to be of great value in evaluating solid and cystic neck masses. Diagnosis can be concluded on the basis of location in the neck, displacement of adjacent structures, and characteristics of the cyst itself14, 15, 16, 17. BCC usually lies anterior 6

and medial to sternoceidomastoid causing displacement of the SCM posteriorly or posterolaterally. Besides a cystic neck mass occurring at or above the level of the carotid bifurcation with extension of the mass between the external and internal carotid arteries is highly suggestive of a second branchial cleft cyst, regardless of its location in relation to the sternocleidomastoid muscle18. In our series one of the patient presented with a neck mass mimicking a metastatic node where PET-CT was highly conclusive in establishing the diagnosis and also ruling out presence of any primary cancer. Thus in such situations imaging modality like PET-CT also has a definitive role. CONCLUSION : Though BCC is a common lesion occurring in neck region, they can sometimes pose a diagnostic challenge by unusual varied presentations.All the 3 cases presented in our series proves this. Abscesses and necrotic adenopathy also can be difficult to distinguish from a BCC, particularly if it has previously been infected. Though investigations like sonography and fine needle aspiration are highly helpful in establishing the diagnosis, one should always correlate it with history and clinical examination. Imaging modalities like CT scan plays crucial rolein establishing the diagnosis of BCC on basis of its location, its relation to adjacent structures and characteristics of cyst itself. In our study even PET-CT was required in one of the case to establish the final diagnosis. Complete surgical excision is must to prevent recurrences. Due to close proximity of the branchial cysts to the carotid arteries and lower four cranial nerves it is imperative to be highly meticulous during dissection to avoid injury to such important structures. Hence, the present series is reported to broaden the varied presentation of branchial cleft cyst occurring in the lateral neck region and to emphasis on the importance of imaging modalities in establishing the correct diagnosis.These findings may act as a warning to junior surgeons treating swellings of the neck. REFERENCES: 1. Thomaidis V, Seretis K, Tamiolakis D, Papadopoulos N, Tsamis I. Branchial cysts. A report of 4 cases. ActaDermatovenerolAlp PannonicaAdriat2006;15:859. 2. Sinha D, Utture SK. Branchial cysts: A case report of a benign lymphoepithelial cyst in the Neck with review of literature. Bombay Hospital Journal 3. Gold B. Second brachial cleft cyst and fistula. AJR Am J Roentgenol1980;134:1067– 1069 4. Lev S, Lev MH. Imaging of cystic lesions. RadiolClin North Am 2000;38:1013–1027 5. Harnsberger H, Manusco A, Muraki A, et al. Branchial cleft anomalies and their mimics: computed tomographic evaluation. Radiology 1984;152:739–748 6. Coppens F, Peene P, Lemahieu SF. Diagnosis and differential diagnosis of branchial cleft cysts by CT scan. J BelgeRadiol1990;73:189–196 7. Anesi A, Pollastri G, Bondì V, Barberini S, Chiarini L. Absence of adjuvant radiotherapy may be an additional criteria in diagnosing a branchiogenic squamous cell carcinoma: A case report. OJST 2012;2:63-7 7

8. Mitroi M, Dumitrescu D, Simionescu C, Popescu C, Mogoantă C, Cioroianu L, et al. Management of second branchial cleft anomalies. Rom J MorpholEmbryol 2008;49(1):69-74 9. Moran AGD, Stell PM. Clinical Otolaryngology. Oxford: Blackwell, 1979. 10. McPhail N, Mustard RA. Branchial cleft anomalies. Can Med Assoc J 1966;94: 1749. 11. Chandler JR, Michell B. Branchial cleft cysts, sinuses and fistulae. OtolaryngolClin North Am 1981;1:175-85. 12. Karlan MS, Michel SL, Snyder WH. Branchogeniccystscongenital or acquired. Am J Surg 1965; 110:615-9. 13. Paley WG, Keddie NC. The aetiology and management of branchial cysts. BrJSurg1970;57:833-4. . 14. Harnsberger HA, Mancuso AA, Muraki AS, et al. Branchial cleft anomalies and their mimics: computed tomographic evaluation. Radiology i984;1 52:739-748 15. Silverman PM, Korobkin M, Moore AV. Computed tomography of cystic neck masses. J Comput Assist Tomogr1983;7:498502 16. Silverman PM, Korobkin M, Moore AV. CT diagnosis of cystic hygroma of the neck. J Comput Assist Tomogr 1983;7 :519-520 17. Reede DL, Whelan MA, Bergeron AT. CT of the soft tissue structures of the neck. Radio! Clin North Am 1984;22 :239-250 18. Jorge E. Salazar,1 Robert A. Duke, and James V. Ellis.Second Branchial Cleft Cyst: Unusual Location and a New CT Diagnostic Sign. American journal of roentgenology. 1985 183.87.255.21 Acknowledgement- None Source of Funding- Nil Conflict of Interest- None Declared Ethical Approval- Not Required CORRESPONDENCE ADDRESS:Dr. Shruti Bansal: Address: House no. 1716, Sector 21 Panchkula, Haryana -134112 Contact: 08097421210 E-mail address : [email protected]

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